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-0001-11-30

TRENDS IN PERINATAL MORTALITY


TRENDS IN PERINATAL MORTAL ITY AT KING FAHD HOSPITAL OF THE UNIVERSITY, AL-KHOBAR, SAUDI ARABIA: A TEN YEARS STUDY

Fathiya M. Al-Mejhim, CAB(O/G), Sulaiman S. Al-Najashi, M.MED(O/G)

Department of Obstetrics and Gynaecology, Collegeof Medicineand Medical Sciences, King Faisal University, Dammam, Saudi Arabia

هدف الدراسة: الهدف من هذه الدراسة هو تحديد الأسباب الرئيسية لوفيات الأطفال، وتحديد العوامل الرئيسية المتعلقة بالأم التي تؤثر على هذه الوفيات، في مستشفى الملك فهد الجامعي بالخبر بالمملكة العربية السعودية.

طريقة الدراسة:دراسة استعادية أجريت خلال فترة عشر سنوات، بدءًا من شهر يناير 1987م إلى شهر ديسمبر 1996م. كل الوفيات ومجموعها 548 حلة وفاة، والتي حدثت خلال تلك الفترة تم تحليلها حسب طريقة ويجلزورث.

نتائج الدراسة:أظهرت الدراسة أن معدل الوفيات كان (19.2 في الألف) من الولادات. كانت التشوهات الخلقية مسئولة عن 116 حالة (21.1% من مجموع الوفيات). أما الباقي من الوفيات وهم 432 حالة، فإن 214 منهم ( 49.5%) حدثوا خلال الحمل، و 71 حالة ( 16.5%) خلال الولادة، و147 حالة ( 34%) خلال الأسبوع الأول بعد الولادة. ومن بين المواليد الطبيعيين شكل ناقصو الوزن النسبة الأكثر في الوفيات (29%) وتلاهم وفيات المواليد بسبب أمراض الأم الحامل (14.8%) بينما كان سبب الوفيات كان غير معروفا في (17.3%) من المواليد كاملي الوزن. كانت الأمهات اللاتي لم يراجعن عيادات الحمل مسئولات عن 72% من مجموع الوفيات في الأطفال.

الاستنتاج:لن يتم تقليل نسبة الوفيات بين المواليد إلا بوعي جميع النساء بضرورة الاستشارة الطبية قبل الحمل في العيادات الاستشارية ثم الالتزام بمتابعة زيارتهن لعيادات الحمل أثناء فترة الحمل.

الكلمات المرجعية:وفيات المواليد، رعاية الحوامل.

Objective:The aim of the study is to identify the major causes of perinatal mortality and to determine the main maternal factors which affect perinatal mortality at the King Fahd Hospitalof the University (KFHU), Al-Khobar, Saudi Arabia.

Methods: A retrospective study was conducted covering a period of ten years from January 1987 to December 1996. All 548 perinatal deaths, which occurred during that period, were analyzed according to Wigglesworth classification.

Results: The study revealed a perinatal mortality rate of 19.2:1,000 births. Lethal malformations accounted for 116 (21.1%) of fetal deaths. Of the remaining 432 deaths, 214 (49.5%) occurred antenatally, 71 (16.5%) in labour and 147 (34%) within the first week of delivery. Amongst the normally formed babies, low birth weight was the commonest cause of death (29%) followed by the consequences of maternal diseases (14.8%). The cause of death was unknown in 17.3% of mature babies who died. Unbooked patients were responsible for 72% of the total perinatal deaths.

Correspondence to:

Dr. Sulaiman S. Al-Najashi, Department of Obstetrics and Gynaecology, King Fahd Hospital of the University, P.O. Box 40023, Al-Khobar 31952, Saudi Arabia

Conclusion:Reduction of the perinatal mortality rate is possible when all women begin to value preconception counselling and attendance at antenatal clinics.

Key Words:Perinatal mortality, Antenatal care


 

INTRODUCTION

A course  but valid  parameter  for the assessment of antenatal care is perinatal mortality rate. It varies with the quality and degree of utilization of antenatal care and perinatal services. This is important for identifying problems in obstetric and early neonatal care. Though most perinatal mortality studies have been done in developed countries, a few published data from developing countries suggest that perinatal mortality rate (PMR) is still high in comparison to developed countries.1-3

Though improvements in antenatal and intrapartum care have reduced perinatal mortality worldwide, perinatal morality in Saudi Arabiaremains high despite the vast improvement in the perinatal care over the past decade. No national  statistics on perinatal mortality  in Saudi  Arabia  are available but, those reported from hospitals in the various regions showed that the rate of decline in fetal mortality has slowed down in the last few years. Despite the substantial reductions, perinatal mortality in the Kingdom remains much higher than that  in developed countries.4-6

The aim of this study is to identify the major causes of perinatal mortality and to determine the main maternal factors which affect it.

PATIENTS AND METHODS

This study included all perinatal deaths occurring at the King Fahd Hospitalof the University, Al-Khobar, between January 1987 and December 1996. King Fahd Hospitalof the University (KFHU) is a referral center in the Eastern Province of Saudi Arabia with an average delivery rate of 2500 to 3000 births per year. The majority of our patients 67% of whom have no antenatal care in the hospital are at high risk. The study included all stillbirths and all live babies who weighed 500 g or more and died in the first week of life. Information about each perinatal death was tabulated on a specially designed form.

                                Gestational age was calculated from the first day of the last menstrual period and rounded to the nearest week. Where the dates were doubtful, ultrasound, clinical findings and paediatric  scores at birth using the Dubowitz method were employed for all live babies weighing  500 g  and  above. Both obstetricians and neonatologists attended all perinatal mortality meetings. Post mortem examination was not carried out on any infant, since no parental consent was given and routine autopsy is not permitted in Saudi Arabia. The causes of  death  were  classified according to the Wiggelsworth classification (Table 1).

                                The following definitions were used: Low birth weight babies, newborn weight less than 2500 g; dysmature baby: weight below 10th centile for gestational age; macrosomia birth: weight in excess of the 90th centile; preterm birth, newborn at less than 37 weeks of gestation; postmaturity: gestational age of 42 weeks or more.

RESULTS

During the period of study, there were 28,507 deliveries and 548 perinatal deaths. Of the deliveries, 28,186 were singleton births and 321 multiple pregnancies, including one set of quadruplets, eleven sets of triplets and 142 sets of twins. There were 512 perinatal deaths in the singletons and 36 in the multiple


 

Table 1:Classification of perinatal deaths – Wigglesworth classification

Birthweight in grams

Normally formed, macerated SB

Congenital malformation

Conditions associated with immaturity (NDD)

Asphyxial conditions developing

in labour

Other specific conditions e.g. abruption of placenta

Total

SB

NND

SB

NND

SB

NND

500-1000

46

13

  1

63

5

1

26

7

162

1001-1500

24

11

18

26

7

4

9

7

106

1501-2000

23

9

12

5

1

6

9

2

  67

2001-2500

25

3

13

1

1

3

6

3

  55

2501 and over

74

7

29

-

7

10

22

9

158

TOTAL

192

43

73

95

21

24

72

28

548

SB=Stillbirth; NND=Neonatal death

Table 2:Comparative table for ten years

1987

1988

1989

1990

1991

1992

1993

1994

1995

1996

Total mothers delivered

2380

2993

3128

3114

2514

2884

2713

2777

2677

2538

Total infants born

2855

3017

3154

3148

2549

2920

2740

2826

2718

2580

Total perinatal deaths

   45

   46

   60

   44

   53

  64

   47

  65

   58

   66

Corrected perinatal deaths

  36

  35

   45

   32

   45

  50

   44

  50

   46

  49

Congenital malformations

   9

  11

   15

  12

     8

  14

   3

   15

   12

   17

Mortality rate

(excluding malformation)

12.6

11.6

14.2

10.1

17.6

17.1

16

17.6

16.9

18.9

Table 3:Perinatal mortality in relation to maternal age

Maternal age

Perinatal deaths

Total mothers delivered

Perinatal mortality per 1000 total births

Leas than 20

  65

3197

21.8

20-24

106

6743

14.7

25-29

118

7185

15.7

30-34

  98

5421

17.8

35-40

  78

3318

29.0

More than 40

  47

2322

28.5

Table 4:Perinatal mortality in relation to maternal parity

Parity

Perinatal deaths

Total mothers       delivered

Perinatal mortality per 1000 total births

0

98

4340

24.0

1

76

4211

17.9

2

68

4229

14.8

3

59

4221

11.5

4

54

4127

10.0

5

56

3537

14.0

5

101

3521

35.7


 

pregnancies. During this  period,  the number of births at KFHU increased from 2,855 in 1987 to a peak of 3,154 births in 1989, but decreasing to 2,580 births in 1996. However, perinatal mortality rate fell from 12.6 per thousand in 1987 to a low of 10.1 per thousand in 1990, but again rose to a maximum level of 18.9 per thousand in 1996. The reduction in the number of deliveries was due to the restriction on  the  expatriates receiving antenatal care in KFHU. This resulted in a dramatic increase in the perinatal mortality rate during the last six years as compared with the first four years (Table 2).

                                The perinatal mortality rate was 19.2 per thousand total births, and 18.1 per thousand in singleton deliveries. Among the 548 perinatal deaths, 116 babies (21.1%) died from lethal malformations. The corrected perinatal mortality rate from the total births was 15.1 per thousand deliveries. Of the  432  babies who were normally formed, 214 (49.5%) died antepartum, 71 (16.5%) during labor and 147 (34%) after delivery. With regard to those who had no antenatal care, the perinatal mortality rate was 20.7 per thousand compared with 16 per thousand in booked patients. This means the former were responsible for 72% of the total perinatal deaths compared with 28% in the latter.

Age and parity

The effect of age on the perinatal mortality rate is shown in Table 3. Perinatal mortality was highest in maternal age groups less than 20 years and more than 35 years. Table 4 shows the effect of parity on the perinatal  mortality.  Low  parity  in   particular primigravida and high parity, more than 5 gave the highest perinatal mortality.

Congenital  Malformations

Lethal fetal malformations accounted for 116 of the perinatal deaths (21.1%). The types of anomalies are listed in Table 5. Of the deformities, 43.1% were of the central nervous system, and multiple deformities were present in 35.3% of the babies that died. The perinatal mortality rate due to fetal anomalies was 4:1000 singleton births.

Table 5: Perinatal  deaths  due  to fetal malformations

Malformation

Total deaths (%)

Anencephaly

30 (25.8)

Hydrocephaly

20 (17.3)

Multiple deformities

41 (35.3)

Potter’s syndrome

8 (6.9)

Achondroplasia

1 (0.8)

Trisomies

3 (2.6)

Polycystic kidney

4 (3.5)

Congenital Heart disease

7 (6)

Diaphragmatic hernia

2 (1.8)

Total

116

Low birth weight

A total of 159 perinatal deaths (29%) were due to low birth weight of less than 2500 g among the normally formed babies. Of these, 64 (40%) were stillbirths, 48 died antepartum and 16 died intrapartum. Of the 48 stillbirths who died antepartum, 28 had severe growth retardation. Of the total 159 low birth weight babies, 95 (60%) were neonatal deaths. The causes of death for  48 were  respiratory  distress syndrome, 22 had intrapartum asphyxia, 8 had pneumonia and septicemia, hemolytic disease accounted for nine and pneumothorax -eight.

Pre-eclampsia

Maternal pre-eclampsia was the cause of deaths in 14 perinatal deaths (2.5%) and 11 stillbirths, 8 of which were antepartum and 3 intrapartum deaths. Unbooked mothers accounted for 10 of the stillbirths; 8 of which were associated with abruptio placentae. Of the 14 deaths due to pre-eclampsia, 10 were delivered spontaneously and 4 had forceps deliveries.

Antepartum hemorrhage

Fifty-two perinatal deaths occurred as a result of antepartum hemorrhage (9.5%), 5 from placenta previa and 47 were caused by placenta abruption. Forty-three of the babies were stillborn and the other 9 died within 24 hours of birth as a result of the effects of severe hypoxia. Of the 52 perinatal deaths, 38  occurred  in  grand multiparae, 32 of whom had no antenatal care.

Maternal diseases

Maternal disease was the predisposing factor in 81 perinatal deaths (14.8%). Seventy-eight were stillbirths and 3 were neonatal deaths. Diabetes mellitus was responsible for 64 of them, 61 of whom had never had antenatal  care. In  42  mothers,  the  gestational  age  was calculated as 40-43 weeks  when they were admitted in labour.

Essential  hypertension  with   superimposed pre-eclamptic toxemia resulted in 8 stillbirths at 36-48 weeks. Two of the  3  neonatal deaths were  due to   neonatal hypoglycemia and the third one was  due  to renal hypertension. Of the remaining stillbirths, 2 were due to renal hypertension; 2 to idiopathic thrombocytopenic purpura and 3 were due to severe asthmatic attacks.

Mechanical causes

Mechanical factors accounted for 32 of the perinatal deaths (5.8%) among the normally formed babies, 15 stillbirths and 17 neonatal deaths. Of the stillbirths, 5 of the mothers had a prolapsed non-pulsating  umbilical  cord  on   admission. Three patients underwent caesarian section for cord prolapse but the fetuses were dead by the time they  were delivered. Of  the  remaining 7 stillbirths 3 resulted from a delay in extracting the head of the fetuses of breech delivery, 2 from difficult shoulder dystocia and 2 were due to a delay in the delivery of  the  abdomen  due to fetal abdominal ascites.

Among neonatal deaths, 7 occurred after  breech  deliveries,  5  following   caesarian section, 3 after forceps delivery and 2 after vacuum extraction.

Normal birth weight babies with no maternal complications

Ninety-four perinatal deaths (17.3%) occurred in normally formed babies with no maternal complications. All the babies in this group weighed over 2500 g, the largest being 4100 g. Eighty-two were stillbirths and 12 died in the first week of life. No specific cause of death  was  discovered, and  there  was no congenital malformation in any of them. The mothers of 60 babies of these babies had had no antenatal care, and it was difficult to find out if there was any antenatal cause of death.

DISCUSSION

Perinatal mortality has been used as a monitor of perinatal care and the health services. In developed countries, it has a major influence in outlining areas of deficiency and stimulating efforts toward its reduction. Data on perinatal mortality and maternal risk factors in Saudi Arabiahave appeared in literature, but unfortunately, with the varying definitions of perinatal mortality from different author, there is the occasional discrepancy in the figures presented from the same institution.7-10

This study identifies the major causes of perinatal mortality in King Fahd Hospitalof the University in the Eastern Province of Saudi Arabia. This may be representative of the causes of perinatal deaths in the region. In recent  years  there  has been  a  marked reduction in perinatal mortality in babies of all weight groups with the exception of the lowest (500-999 g)  and  babies  with lethal malformations. The reduction in overall perinatal mortality in this hospital from 20.5 per 1000 in 1985 (El-Zibdeh et al, 1988) to 10.1 per 1000 total births in 1990 (Al-Najashi 1991) is mainly due to   antenatal attendance, in addition to  better  facilities for antepartum and intrapartum fetal monitoring of high risk pregnancies and improved neonatal care. Stillbirth accounted for two-thirds of the perinatal deaths in this study. Patients who did  not  have  antenatal care were responsible for 72% of the total perinatal deaths compared with 28% in patients who had had antenatal care.11,12

The major clinical causes of perinatal mortality found in this study were congenital fetal malformations, low birth weight, maternal disease, particularly diabetes mellitus and antepartum hemorrhage in normal birth weight babies.

Fetal malformations accounted for 21.1% of all perinatal deaths, an incidence of 4 per 1000 total births. The high rate in the present study could be explained by the high incidence of consanguineous marriages among Saudis. A  similar  high  incidence of congenital anomalies had been reported from Riyadh (Mesleh 1985)13 and from Bahrain (El Shafei et al 1986)14 where 25% of the patients attending antenatal clinics of the Armed Forces Hospital, were  married to their first cousins. Consanguinity was 27% among our patients. However, the overall rate of major fetal anomalies among our population was more or less similar to that reported in several studies from other regions in Saudi Arabia.15-17 Early detection of major fetal malformations is pointless in this country since abortion is illegal. The alternative means of prevention of these malformations is through the reduction of consanguineous marriages. This may be achieved by a nationwide publicity and the education of the girls in high school on the risks of such marriages and also the importance of a balanced diet. It has been shown that periconceptional folic acid and multivitamin supplements reduce the recurrence of neural tube defect and other congenital anomalies in women who have previously had an affected baby. Periconceptional prophylactic folic acid and multivitamin supplements in all women with a history of fetal anomalies, especially neural tube defects and in women with low serum folate levels, estimated before conception may be worthwhile.18-20

Low birth weight babies accounted for 29% of the perinatal deaths. Of the total 159 low birth weight babies, 95 (60%) were neonatal deaths and 64 (40%) were stillbirths. Among the factors contributing to perinatal death, immaturity and antepartum or intrapartum deaths due to asphyxia accounted for about two-thirds of the cases. This finding is similar to the findings from a study in Riyadh. The improvement in prenatal and obstetric care as well as the provision of more neonatal intensive care would significantly reduce mortality associated with these factors.21,22

Sixty-four perinatal deaths (11.6%) occurred with mothers who had diabetes mellitus. The incidence of diabetes mellitus in the pregnant population of the hospital was estimated as 3.6%, gestational diabetes constituting 72% of this. Sixty-one of these mothers had never had antenatal care. Preconception counselling, adherence to the advice of the  physician regarding regular antenatal attendance, diet control, insulin regimen and timely admission to hospital for delivery are necessary if perinatal deaths resulting from this to be reduced.

Nearly one-fifth of normal birth weight babies, who died, had no obvious maternal or fetal  complications. Perinatal  autopsy  is proscribed in Saudi Arabiafor social and religious reasons. Had this been possible, the exact cause of death could have been determined in most of these cases.

Much needs to be done to achieve a rate comparable with those quoted for developed countries. Three factors seem to be significant with regard to this high rate of perinatal  deaths: congenital  malformations, lack of adequate antenatal care, and a high percentage of  low birth weight babies. However, the overall rate of perinatal mortality among the population under study was comparable to that reported in several studies from other  regions in  the Kingdomof Saudi  Arabia.4,5

Reduction of the perinatal mortality rate will only be possible when all women begin to value preconception counselling and attendance at antenatal clinics. This is possible with a well-planned mass-media campaign by the health authorities on the importance of antenatal care. With the availability of skilled obstetric care of high-risk  patients  and  more  skilled neonatal care to cope with very low birth weight babies, it is hoped that this objective would be achieved.

REFERENCES

1.     Abu-Heija AT. Causes and factors affecting perinatal mortality at Princess Basma Teaching Hospital in North Jordan. Asia OceaniaJ Obstet Gynaecol 1994;20:415-8.

2.     Howell EM, Vert P. Neonatal intensive care and birth weight-specific perinatal mortality in Michiganand Lorraine. Pediatrics 1993;91: 464-9.

3.     Mavalankar DV, Trivedi CR, Gray RH. Levels and risk factors for perinatal mortality in Ahmedabad, India. Bull Wld Hlth Org 1991;69:435-42.

4.     Al-Faraidy A, Dawodu A, Al-Umran K, Magbool G. Survey of perinatal mortality in the Kingdom of Saudi Arabia. Saudi Medical Journal 1993;14:307-11.

5.     Hashim TJ, Anokute CC. Imbalances in perinatal mortality in health regions of Saudi Arabia. Saudi Medical Journal 1994;15:376-9.

6.     Kayani AK, Akiel AS.Trends in fetal mortality in the Kingdomof Saudi Arabia: Lessons from 15 years of experience. Annals of Saudi Medicine 1988;8:267-73.

7.     Settatree RS, Watkinson M. Classifying perinatal death: experience for a regional survey. B J Obstet Gynaecol 1993;100:110-21.

8.     Stanley  FJ, Watson L. Trends in perinatal mortality and cerebral palsy in Western Australia, 1967 to 1985 BMJ 1992;304:1658-63.

9.     Millat WA, Florey CDUV. Perinatal mortality in Jeddah, Saudi Arabia. Int J Epidem 1992;21:82-90.

10.   Owa AJ, Abusrair H. Trends in perinatal mortality in Qatif Central Hospital, Saudi Arabia. Saudi Medical Journal 1995;16:206-9.

11.   El-Zibdeh MY, Al-Suleiman SA, Al-Sibai MH. Perinatal mortality at King Fahd Hospitalof the University, Al-Khobar, Saudi Arabia. International Journal of Gynaecology and Obstetrics 1988;26:399-407.

12.   Al-Najashi SS. Perinatal outcome in a University Hospitalin Saudi Arabia. J of Obstetrics and Gynaecology 1991;11:427-30.

13.   Mesleh R. Perinatal mortality at the Riyadh Armed Forces Hospital, Riyadh. Saudi Medical Journal 1985;6:135-44.

14.   El-Shafei A, Rao PSS, Sandhu AK. Congenital malformations and consanguinity. Australian and New ZealandJournal of Obstetrics and Gynecology 1986;26:168-72.

15.   Refat MYM, Al-Moghanem M, McDonald P, Reyes L. Major birth defects at King Fahd Hofuf Hospital, prevalence, risk factors and outcome. Annals of Saudi Medicine 1995;15:339-43.

16.   Nisar M, Galuzek WC, Soni A. Easily identifiable congenital malformations in children. Survey of incidence and pattern in 32,332 live born neonates. Annals of Saudi Medicine 1992;12:366-71.

17.   Khan SMA, Ahmed GS, Abu-taleb A, Ellis J, Yaish H. Are congenital anomalies more frequent in Saudi Arabia? Annals of Saudi Medicine 1990;10:488.

18.   Czeizel AE. Prevention of congenital abnormalities by periconceptional multivitamin supplementation. BMJ 1993;306:1645-8.

19.   Werler MM, Shapiro S, Mitchell AA. Periconceptional Folic Acid Exposure and Risk of Occurent Neural Tube Defects. JAMA 1993;269:1257-61.

20.   Czeizel AE, Dudas I. Prevention of the first occurrence of neural tube defects by peri-conceptional vitamin supplementation. N Engl J Med 1992;327:1832-5.

21.   AlTassan F, Khan M. Multiple pregnancies – RKH experience. Abstract: International Symposium on Neonatal Intensive Care, RiyadhArmed Forces Hospital, Riyadh, Saudi Arabia29-30 January 1989.

22.   Dawodu AH, Al-Faraidy A. Major causes of perinatal death in Saudi Arabia. Postgrad Doctor 1990;13:409-14.


-0001-11-30

PREVALENCE OF INDICES


THE PREVALENCE OF INDICES OF HEPATITIS C AND B INFECTION, AND ELEVATED AMINOTRANSFERASE ENZYMES IN PATIENTS WITH ORAL LICHEN PLANUS (OLP) IN EASTERN SAUDI ARABIA

Abdulnasser M. El-Rifaei, PhD*, Sami E. Fathalla, PhD†, Iman H. Al-Sheikh,FCP‡, Mukund B. Tinguria, MD†, Yousef A. Qadry, MD§

* Dammam  Central Hospital, †Dammam Regional Laboratories and Blood Bank,

‡ King Faisal University, Dammam, Saudi Arabia

§Faculty of Medicine, Zagazig University, Egypt

هدف الدراسة:تحديد احتمال وجود ارتباط بين الالتهاب الكبدي الفيروسي ب ، ج  وارتفاع إنزيم أمينو ترنسفريز ومرضى الحزاز المنبسط الفموي في المنطقة الشرقية بالسعودية.

طريقة الدراسة:تم تجميع أمصال الدم من 34 من مرضي الحزاز المنبسط الفموي من المنطقة الشرقية بالمملكة العربية السعودية لاختيار HBsAgومستوى الإنزيمات ALT/AST   وتم فحصهم إكلينيكيا في قسم أمراض اللثة بمستشفى الدمام المركزي وتم إجراء اختبارات الأنسجة في قسم الأنسجة بالمختبر الإقليمي بينما تمت الاختبارات الفيروسية في معمل تشخيص الفيروسات بالمختبر الإقليمي وبنك الدم بالدمام ، واختبارات إنزيم أمينوترنسفريز في معامل مستشفي الدمام المركزي. واستمرت الدراسة من يناير 1995- مارس 1997. واختيرت عينة أخرى من 32 من الأصحاء من نفس العمر والجنس كعينة مقارنة للدراسة.

نتائج الدراسة:أوضح مرضى الحزاز المنبسط الفموي حدوث HBsAG ومضاد HBsAg+HCV  وارتفاع إنزيم أمينو ترنسفريز كان 8.8%، 14.7%، 2.94% ، 47.05% على التوالي، بينما نتائج مجموعة المقارنة كانت 6.25% ، 3.12%، 0.0%، 3.12% على التوالي.

الاستنتاجات والتوصيات: يوجد ارتباط واضح بين الحزاز المنبسط الفموي وفيروس الالتهاب الكبدي C. لا يوجد دليل واضح لهذه العلاقة مع فيروس الالتهاب الكبدي B. كل العينات التي بها إنزيم أمينوترانسفريز مرتفع كانت إيجابية مع التهاب الكبدي C     حيث أعطت دليل أكبر على الارتباط بين الالتهاب الكبدي C المزمن مع الحزاز المنبسط الفموي. يمكن استعمال ارتفاع إنزيم الأمينو ترانسفيريز كمفتاح للعلامات الإكلينيكية لحالات الالتهاب الكبدي الخالية من الأعراض وكدليل لاختبار حالات الحزاز المنبسط الفموي لفيروسات الكبد ذات الصلة بالموضوع. بالرغم من قلة عدد حالات الحزاز المنبسط الفموي في هذه الدراسة إلا أن النتائج تلقى بعض على هذه المشكلة في هذه المنطقة الجغرافية من العالم. تعتبر هذه الدراسة مبدئية ويوصي بإجراء دراسة موسعة تشمل عدد أكبر من هذه الحالات.

الكلمات المرجعية:مرض الحزاز المنبسط ، الإلتهاب الكبدي C ، التهاب الكبدي B، أمينوترانسفريز  والمملكة العربية السعودية.

Objective:To determine the possibility of any association between HBV, HCV, elevated aminotransferase enzymes and Oral Lichen Planus ( OLP)patients in Eastern Saudi Arabia.

Correspondence to:

Dr. Sami E. Fathalla, P.O. Box 4103, Dammam 31491

Design: Sera were collected from OLPpatients, to be tested for HbsAg, anti-HCV and ALT/AST levels.

Settings: All the patients who were diagnosed clinically in periodontal section, Dammam Central Hospitalwere from Eastern Saudi Arabia. The histopathological diagnosis was done in Histopathology Section, Dammam Regional Labs, the virological studies in the Virus Diagnosis Lab of Dammam Regional Labs and Blood Bank, and the aminotransferase tests done in the Dammam Central HospitalLabs.

Subjects:34 serum specimens were collected from OLPpatients, and 32 other samples from healthy populations of the same age and sex as the controls of the study.Results:Incidence of HBsAg, anti-HCV, HBsAg+HCV, and elevated aminotransferase enzymes among OLPpatients were 8.8%, 14.7%, 2.9%, and 47.05% respectively and the results from the control subjects were 6.25%, 3.12%, 0%, and 3.12% respectively.

Conclusion:There is a significant association between OLPand HCV infection. No clear evidence of this relationship appeared with HBV. All the aminotransferase elevated samples were positive to HCV, giving a clear evidence of the association of chronic HCV infection with the OLP. Aminotransferase elevated results could be used as a clue to clinical signs of asymptomatic hepatopathies, and as a marker to check the OLPcases for the relevant Hepatic Viruses. Despite the limited number of OLPpatients in this study, the results could highlight the problem in this geographical area of the world. We recommend a comprehensive study to be carried out using this current study as a preliminary one.

Key Words:Oral Lichen Planus ( OLP), Hepatitis C Virus (HCV), Hepatitis B Virus (HBV), Aminotransferase, Saudi Arabia.


 

INTRODUCTION

Lichen Planus (LP) was first described in 1869 by Eramous Wilson.1 It is a pruritic eruption of violaceous polygonal papules topped by characteristic white lines called Wickham’s striae.1-3 LP is known for its sub-epidermal lymphocytic infiltration that involves skin, hair, nails, oral and/or genital mucosa,2-4 such infiltration gives a saw-tooth appearance at the dermo-epidermal junction.2,3 However, oral LP is diagnosed by the presence of Wickham’s striae which are more common on the buccal mucosa, tongue, gingiva, lips, floor of the mouth and the palate. About 30% of oral LP patients have extra-oral lesions, while about 10-50% of all the LP patients has oral lesions.2,4,5 Nevertheless, LP is generally a  benign  disease. However, erosive or atrophic LP may progress to malignancy.2-4 Despite many studies on LP, its cause is still unknown, though viral, and/or autoimmune pathogenesis has been proposed and there is some association with hepatitis viruses, especially with Hepatitis C Virus (HCV), as a good percentage of cases are associated with HCV infection.6-18 There is an elevation of the liver enzymes (aspartate amino transferase AST/GOT), and (alanine amino transferase ALT/GPT) in many cases of LP,12,13 and it is well known that the LP patients with normal hepatic enzyme levels are usually HCV negative.2-4,9

The aim of  this  study is to show the incidence of  HCV/HBV among   histopathologically diagnosed Oral Lichen Planus ( OLP) and to study the levels of the hepatic enzymes in these cases. Such a study could explain the association of the hepatitis virus with the oral LP disease in this part of the world. We used hepatitis B surface antigen (HBsAg), and antibody to hepatitis C virus (anti-HCV) as our indices for Hepatitis B Virus (HBV) and Hepatitis C  Virus (HCV)  infection  respectively. However, the basis of our OLP histopathological diagnosis was the presence of acanthosis and hyperkeratosis of the lining of the stratified squamous epithelium with dense chronic inflammatory  cell  infiltration  in association with the destruction of the basal cell layer.

SUBJECTS AND METHODS

Subjects

Between January 1995 and March 1997, 34 specimens were collected from 21 female and 13 male patients with a mean age of 40 years (range 30-50 years) and histologically diagnosed as Oral Lichen Planus ( OLP). The subjects were from the main cities of the Eastern Province of Saudi Arabia. Thirty-two serum samples were collected from healthy individuals of the  same age  and sex  distribution as control subjects. The samples collected were either run the  same  day, or  kept at –70oC till processing.

Methods

Anti-HCV screening: Antibodies to HCV proteins expressed by C-100-C and C-22-3 clone regions of the HCV genome were detected by Enzyme Linkage Immuno-Sorbent-Assay (ELISA) kits with sensitivity of 55.64% - 81.62% and specificity of 94.04%-100% Abbott’s HCV EIA 2nd generation screening test kits (Abbott Diagnostics, Germany).

Confirmatory HCV tests:The specimens that were repeatedly reactive (RR) by the screening test  were  re-evaluated   by  Recombinant Immuno-Blotting Assay Riba IV with sensitivity, and specificity around 100%. RIBA IV kits HCV Blot 3.0 kits were used (Genelabs Diagnostics Pte Ltd, Singapore– under license from: Ortho Diagnostics Systems Inc., and Chiron Corporation, USA). The reactive positive specimens from RIBA IV were considered positive to anti-HCV.

HBsAg screening tests:ELISA technique was used to detect HBsAg (Ayzyme Monoclonal Kits from Abbott’s Diagnostics, USA). The RR specimens were reevaluated by the confirmatory test for HBsAg.

HBsAg confirmatory assays:We used confirmatory Abbott’s Diagnostics for HBsAg, from Abbott’s USA. The confirmed HBsAg by this method was considered as positive for HBsAg.

Hepatic enzymes assay:The Hitachi717 autoanalyser was used to evaluate the following enzymes: serum aspartate amino-transferase (AST)/Glutamic Oxaloacetic Transaminase (GOT), serum alanine amino- transferase (ALT)/Glutamic pyruvic transaminase (GPT). The normal range for this technique was 0-40 IU/L for both enzymes.

The techniques

All the methodology used in this study followed the instructions of the kit manufacturers.

RESULTS

The outcome of  our  study  was  tabulated (Table 1) as follows: 14.7% (5 of 34) patients with OLPwere positive with HCV antibodies, while in the controls onlyone out of 32 (3.12%) was diagnosed with HCV: HBsAg positive cases represented 8.8% (3 of 34), and 6.25% (2 of 32) of the OLPcases and the controls respectively; the double-infected cases (HBsAg and anti-HCV) represented 2.94% (1 of 34), and 0% (none of 32) among the OLPcases and the controls; aspartate amino transferase (AST/GOT) and Alanine amino transferase  (ALT/GPT)  elevation  was


 


 

Table 1:Incidence of HCV, HBV, elevated hepatic enzymes among Oral LP patients and controls

Parameters

Oral LP (34)

+ve (%)

95% CI*

Controls (32)

+ve (%)

95% CI

anti-HCV†

5/34 (14.70)

2.79-26.6

1/32 (3.12)

   - 2.9-9.14

HBsAg‡

   3/34 (8.80)

-0.72-18.32

2/32 (6.25)

-2.14 -14.64

anti-HCV and HBsAg

   1/34 (2.94)

   -0.27-8.62

0/32 (0.00)

00

AST/ALT§

16/34 (47.05)

30.22-63.67

1/32 (3.12)

-2.90-9.14

*95%CI 95confidence interval     †-anti Hepatitis C Virus    ‡Hepatitis B surface antigen   §More than 40 IU/L

Odd ratio:5.345, 1.45, 27.55 for anti-HCV, HBsAg and AST/ALT respectively.

Y2 (Dispersion factor): 2.68, 0.139, and 16.63 for anti-HCV, HBsAg and AST/ALT respectively.

95%CI (for ODD ratio): 0.589-48.42, 0.22-9.21, and 3.35-225.88 for anti-HCV, HBsAg, and AST/ALT respectively.


 

found in 47.05% (16 of 34) and 3.12% (1 of 32) among the OLPcases and the controls respectively.

DISCUSSION

Although the exact causes of LP are still unknown, some evidence suggests that it could be a multifactorial disease or a syndrome caused by more than one etiological agent.

Hepatitis B and C viruses and their relationship to OLP have been a subject to extensive studies6-18 and  this paper is  reporting  a comparative study of HBV and HCV and their supposed  relationship   to  OLP. HBsAg   incidence among healthy populations was previously recorded as 8.5%,19 while the incidence among OLP patients was 6.25% (not statistically significant difference) suggesting that HBV is less likely to be a direct cause of OLP, at least in this limited study.

On the other hand, there was 14.7% incidence of Hepatitis C virus among the Oral Lichen Planus (OLP) patients, while the incidence among healthy population in the same geographical area was 2.8%.20,21

The double infections (HBV+HCV) among the OLPcases were 2.94% and 0% among the controls.

In this  study,  elevated serum aminotransferase enzymes (ALT&AST) were found in 47.05% of the OLP cases, but in only 3.12% of the control subjects. This finding suggests a relationship between ALT/AST and OLP. However, some authors have reported a direct relationship between the aminotransferases and the OLP, especially  in  errosive  OLP patients,9,10,17 and that oral lesions become more aggressive as the liver enzymes increase.9,10 However, OLP patients with normal hepatic enzymes were mostly negative to HCV infection. This finding needs to be confirmed in a next study.

The cause of OLPis not restricted to hepatic C virus infection alone, or to the elevation of aminotransferase enzymes (as a sign of chronic active/chronic persistent hepatitis. Other factors such as cell mediated immune reactions could possibly play a role. Just as an association between OLP and some systemic diseases of immunological origin such  as  ulcerative colitis, alopecia areata, diabetes mellitus has been suggested by some authors.

To the best of our knowledge, this current study is the first from Eastern Saudi Arabia, or perhaps from the whole Kingdom.

CONCLUSION

In conclusion, our results suggest an association between OLPand HCV infection, especially in patients with high amino transferase enzyme levels. However, HBV does not appear to play a major role in the causation of OLP. However, this is yet to be confirmed. We also conclude that OLPas a disease could be used as a clinical sign of the usually symptomless hepatopathies, and may play a role in the diagnosis of asymptomatic liver diseases.

ACKNOWLEDGMENT

The authors thank Mrs. Paz Duplon F BS (Statistics) of Iba, Zambales, The Philippines, for the statistical analysis of this study. We also extend our thanks to the staff of Virology Diagnostic Labs of Dammam Regional Labs & Blood Bank for their technical help during making this research.

REFERENCES

1.   Burkhart NW, Burker EJ, Burkes J, Wolfe L. Assessing the characteristics of patients with oral lichen planus. JADA 1996; 127:648-62.

2.   Van ML, Parks ET. Prevalence of Oral Lichen Planus in patients with Diabetes Mellitus. Oral Surg Oral Med Oral Radiol Endod 1995; 79:696-700.

3.   Bunker CB, Doiod PM. Skin Disease. In: John Axford Medicine, Blackwell, Oxford, London, UK1996:14.14-14.68.

4.   Lamey PJ, McCartan BS, MacDonald DG, Mackie RM. Basal cell cytoplasmic autoantibodies in Oral Lichenoid reaction. Oral Surg Oral Med Oral Path Oral Radiol Endod 1995;79:44-9.

5.   Jabrt C, Pawlotsky TM, Puget F, Andre C, De-Forges L, Bretagne S, et al. Lichen Planus and Hepatitis C Virus (HCV) – related chronic active hepatitis. ARCH Dermatol 1994; 130:73-6.

6.   Boisnic S, Ouhayoun JP, Branchet MC, Francis C, Beranger JY, Le-Charpentier Y, Szpirglas H. Alteration of Cytokeratin expression in OLP. Oral Surg Oral Med Oral Path Oral Radiol Endod 1995;79:207-15.

7.   Porter S. OLP and chronic liver disease. Oral Surg Oral Med Oral Pathol 1995;79:267-8.

8.   Sanchez-Perez J, De-Castro M, Buezo GF, Fernandez-Herrer J, Borque MJ, Garcia-Diaz A. Lichen Planus and HCV: Prevalence and clinical presentation with LP and HCV infection. Br Jour Derm 1996;134:715-9.

9.   Bagan JV, Aguirre JM, Del-Olmo JA, Milian A, Penarrocha M, Rodrigo JM, et al.  Oral Lichen Planus and chronic liver disease: A clinical and morphometric study of the oral lesions in relation to transaminase elevation. Oral Surg Oral Med Oral Path 1994;78:337-42.

10.Amichai B, Lazarov A, Halevy S. HCVinfection and oral erosive LP. J Dermatol 1994;21(10):783-4.

11.Gondolfo S, Carbone M, Carrozzo M, Gallo V. OLP and HCV infection: is there a relationship? J Pathol Med 1994;23(3):119-22.


-0001-11-30

REFERRAL FROM PRIMARY CARE


REFERRAL FROM PRIMARY CARE TO HOSPITALS IN SAUDI ARABIA: 1) QUALITY OF REFERRAL LETTERS AND FEEDBACK REPORTS

Jamal S. Jarallah, MRCGP, Department of Family & Community Medicine, King Khalid University Hospital, Riyadh, Saudi Arabia

خلفية الدراسة: للإحالة بين المراكز الصحية والمستشفيات أهمية قصوى في متابعة رعاية المرضى رعاية جيدة، ومن هنا وجب الاهتمام بجودة وسيلتها وهي رسائل الإحالة والردود من المستشفيات.

هدف الدراسة: أجريت هذه الدراسة لتقييم جودة نماذج الإحالة من المراكز الصحية إلى المستشفيات العامة في أربع مناطق بالمملكة العربية السعودية.

طريقة الدراسة: هذه الدراسة مقطعية لعينة عشوائية من نماذج الإحالة من المراكز الصحية. كانت وحدة الاعتيان (أخذ العينة) الأولى هي المستشفيات العامة التي تحيل إليها المراكز. وقد أخذت عينة عشوائية على أساس بعدها عن المستشفيات وتبع ذلك أخذ ثلاثين نموذج إحالة من كل مركز صحي بطريقة عشوائية. وقد تم دراسة نماذج الإحالة المشتملة على رسائل الإحالة الصادرة من المراكز الصحية والردود من المستشفيات من حيث جودتها بتطبيق نظام الإحراز على محتوياتها. وأدخلت البيانات على الحاسب الشخصي باستخدام برنامج  . STAT PACK GOLDوللمقارنة بين المناطق استخدم اختبار مربع كاي كما تم استخدام T- TEST  لمقارنة نقاط الإحراز مجتمعة.

نتائج الدراسة: تمثلت هذه في مدى جودة رسائل الإحالة وتقارير الردود من المستشفيات بعد مقارنة محتوياتها بمحتويات قياسية (معيارية) نموذجية باستخدام نظام الإحراز. كانت أكثر المحتويات تسجيلا في النماذج هي المعلومات الديموغرافية بنسبة 100%، ثم التخصص المحال إليه المريض   ( 93.3%)، وسبب الإحالة ( 82.7%)، وتم إغفال معلومات أخرى مهمة. أما بالنسبة للردود فقد أغفلت معلومات مهمة مثل من هو الطبيب المعالج(81.8%)، التشخيص(86%)، والقرار بالنسبة لمتابعة المريض(80%). لقد كانت جودة رسائل الإحالة متدنية في 23%، وبالمقابل فإن 81% من الردود كانت كذلك، مع اختلاف بين المناطق.

الاستنتاجات: إن جودة رسائل الإحالة والردود متدنية وتحتاج إلى تحسين ويمكن ذلك من خلال تطبيق نظام الجودة النوعية.

الكلمات المرجعية: رسائل الإحالة، تقارير الردود، الرعاية الأولية، المملكة العربية السعودية.

Background: Referral between primary care and hospitals is of great importance for patient care and follow-up. This study was conducted because of the importance of the quality of referral letters and feedback reports.

Objectives:To evaluate the quality of referrals from primary health care (PHC) centers to general hospital in four regions in Saudi Arabia.

Methods:This was a cross-sectional study of a random sample of referrals from the PHC centers. The first sampling unit was the general hospitals to which the health centers refer. A random sample of health centers was then selected based on their distance  from the hospitals. This  was followed  by  randomly  selecting 30 referrals

Correspondence to:

Dr. Jamal S. Jarallah, Associate Professor and Director of Primary Health Care Clinics, Department of Family & Community Medicine, King Khalid University Hospital, College of Medicine, P.O. Box 2925, Riyadh 11461, Saudi Arabia

from each health center. The referral letters and the corresponding feedback reports were then studied for quality by scoring the components of each. The data was entered in a personal computer using the Stat Pack Gold Statistical Package. The chi-square was used to compare the different scores across the regions and T-test was used to compare the cumulative scores.

Main outcome measures:The quality of referral  letters and  feedback reports   was defined according to the standardized components using a scoring system.

Results:The most frequently mentioned items in the referrals were demographic data (100%), specialty referred to (93.3%) and reason for referral (82.7%). Other important items in the feedback reports including the name of the treating physician (81.8%), diagnosis (86.0%), and decision on follow-up (80%) were missing. The quality of referral letters was poor in 23%, with 81% as the corresponding figure for the feedback reports with some variation between the different regions.

Conclusions: The quality of referral letters and feedback reports is poor and needs to be improved. This can be achieved through implementing the quality assurance programme.

Key Words: Referral letters, feedback reports, quality, primary care, Saudi Arabia.


 

INTRODUCTION

Referral from primary care to hospitals is an important daily activity that safeguards good medical care to patients. The main objective of  building  and  improving a referral system is to improve the quality of patient care.

Referral is a two-way communication process between primary care physicians and specialists in hospitals, both of whom have an important role to play. It is the responsibility of the primary care physician to convey a clear message about the need and reason(s) for referring a patient. On the other hand, the specialist in a hospital is responsible for conveying a clear feedback on his evaluation of the patient’s condition and a plan of management. However, problems in the referral process arise from primary care or hospitals when the primary care physician fails to clarify the reason(s) for referral,1 or conveys  inappropriate  or  incomplete  information.2 The specialist may also not address the physician’s reason for referral or may fail to communicate his finding to the referring physician.3

Several authors have stressed the importance of good referral letters as the best mode of transmitting information to promote the understanding of a problem or patient. This not only improves management, but also aids the appropriate use of resources.4-9

In Saudi Arabia, the referral system given to primary care physicians is based on clear guidelines detailing the referral process. This includes  the  use of a  pre-designed standardized referral form with important relevant clinical and social information. However, a small scale study on referral letters in two health centers in Riyadhhave shown the inadequacy of both referral letters as well as the feedback received from specialists.10 Thus, there is a need to explore further the practices within the referral system in Saudi Arabiafurther.

The current study is part of a national project undertaken to evaluate the quality of the referral system in Saudi Arabia. In this paper, we report the quality of referrals in four regions of the Kingdom.

METHODS

The study was conducted in four regions in Saudi Arabia(namely Riyadh, Gazan, Northern Region and Hail). These regions were chosen because of the assumed variation in many health service characteristics such as the total population served, the number of health centers and hospitals, geographic characteristics, and other facilities.

Sampling Procedures

The sampling units for the study were the general  hospitals in the region. These hospitals were first listed in a stratified sampling frame based on bed capacity, as follows: large hospitals – more than 150 beds, medium hospitals – from 100-150 beds, small hospitals – less than 100 beds. A random sample from each stratum was chosen, using a lottery method. The final number of hospitals was: 3 hospitals each from Riyadhand Gazan, and 2 hospitals each from the Northern Region and Hail (there were no hospitals of 100-150 beds in these two regions).

The next step was the sampling of the health centers. First, the health centers referring patients to these hospitals were identified and numbered, and the distance from hospitals (in kilometers) specified. The health centers were stratified according to the distance from hospitals to study the effect of this on the process of referral. A sample of two health centers that were near and two distant health centers was taken randomly using the random tables after pre-pointing a random start. The total number of selected health centers was 39 centers (one health center from Hail was excluded because there was no record of referrals for the whole year).

Sampling of the referral letters

The selected health centers were visited and the referral records of one year were identified and numbered. Thirty referrals from each health center were then randomly selected using random tables, and the information gathered was recorded on pre-designed and tested data sheets. The data sheets included demographic data of the patient referred, place of referral, specialty to which he was referred and other items that reflect the quality of the referral letters.

Scoring of the referrals

The important items in the referral letter were identified and the investigator and two other experienced family physicians undertook the scoring for referrals from the health centers. The team assessed the quality of referral letters on the basis of the following scoring system: patient’s age (not present=0, present=1), patient’s sex  (not present=0, present=1),  present complaint (not specified=0, specified=1), duration of complaint (not mentioned=0, mentioned=1), physical examination (not present=0, present but not clear=0, present and clear=1),  differential  diagnosis (not mentioned=0, mentioned  but  not clear=0,  mentioned and clear=1), name of the referring physician (not mentioned=0, mentioned but not clear=0, mentioned and clear=1), reason for referral (not specified=0, specified=1), specialty to which the patient was referred (not specified=0, specified=2), and date, file number, health center and hospital in the same way and legibility of writing (not legible=0, legible=1). This gave a total score of 14.

Scoring was also done for the feedback from hospitals according to the following manner: result of evaluation (not available=0, available and not clear=0, available and clear=1), name of the attending physician (not written=0, written and not clear=1, written and clear=2), decision on follow-up (not clear=0, clear=1), plan of management (not clear=0, clear=1), and recommendations to the referring physician (not clear=0, clear=1) final diagnosis, response to the questions of the PHC physician, physical examination and file number were scored in the same way. This  gave  a total score of 9 for the feedback from specialists.

Data analysis

The  data  were  entered in a personal computer using the Statpack Gold Statistical Package. Chi-square test was used to compare the variables and the  different  scores of quality of referral letters in the different regions.

RESULTS

Of the 1060 referral letters with feedback reports studied, 1026 (97%) were suitable for the final analysis, 34 forms were found to be difficult to evaluate and were excluded.

The specialists to whom the patients were  referred from the primary care centers are presented in Table 1. The majority of patients were referred to General Medicine (15.7%), Ophthalmology (14.7%), Obstetrics and Gynecology (14.3%), and Pediatrics (13.1%). In a few of the referrals (2.9%) the specialty was not defined.

The frequency of important and relevant components of the referral letter is shown in Table 2. The most frequently mentioned items were age and sex (100%), the specialty to which the patient was referred (96.5%), the present complaint (90.8%) and reason for referral (99.8%). Important and relevant items such as duration of complaint, physical examination, name of the referring  physician,  and differential diagnosis were not mentioned or specified in 46% of the referral letters.

The most frequently mentioned components of the feedback report (from hospitals) were patient’s record number (69.6%), recommendations to PHC physicians (76.0%), and result of examination (58.5%) (Table 3). Response to specific questions from PHC doctors was mentioned in only 41.7%. Other important items such as the name of the treating physician, diagnosis, decision on follow-up and arrangement for follow-up were mentioned in less than 20% of the reports.

The frequency of scoring for the relevant items as components of referral letters is shown in Table 4. The mean score was 10.7 ± SD 2.2 and the majority were in the higher scores (more than 70% scored 10 or more).

In Table 5, the scores for the relevant items of the feedback reports are shown. Only about half of the reports scored 5 or more of the scale (the mean score ± SD was 3.9 ± 2.1).

Assessment of the quality of referral letters and feedback reports based on the scores in different regions is shown in Table 6. The majority of letters (77.1%) were found to be of good quality (scored 10 or more). There was  a  significant  difference between the regions. Riyadhand Northern regions were the best and Gazan was the worst (chi-square for trend = 18.95, p=0.0000).

However, the quality of a vast majority (80.8%) of feedback reports was poor. There  was   a  significant difference between the regions. Hail and Gazan were the best and the Northern region was the worst (chi-square for trend = 24.1, p=0.000).

DISCUSSION

The results of this  study as reflected by the quality of the referral letters and feedback reports show  that communication between the primary care physicians and specialists in hospitals is poor. The lack of basic clinical information such as duration of complaints, findings on clinical examination, and the like in the referral letter, is worrying.  This observation which was documented in previous international8,11,12 and local studies,10,13 was found to be a major determinant of the quality of the process of referral and the feedback from the specialist.14 Taking  into  consideration  the costly hospital consultations as compared to PHC, the “gate keeping” provided


 

Table 1: Specialties to which patients were referred in the different regions

REGIONS

Specialty

Riyadh

No (%)

Northern

No (%)

Hail

No (%)

Gazan

No (%)

Total

No (%)

Internal Medicine

65 (20.8)

29 (11.5)

21 (15.2)

46 (14.2)

161 (15.7)

Ophthalmology

40 (12.8)

40 (15.9)

12 (8.7)

59 (18.3)

151 (14.7)

Obstetrics

(Antenatal,Postnatal)

30 (9.6)

32 (12.7)

53 (38.4)

32 (9.9)

147 (14.3)

Pediatrics

46 (14.7)

27 (10.7)

7 (5.1)

54 (16.7)

134 (13.1)

General Surgery

32 (10.2)

15 (6.0)

1 (0.7)

41 (12.7)

89 (8.7)

Dermatology

26 (8.3)

19 (7.5)

3 (2.2)

24 (7.4)

72 (7.0)

Orthopedics

15 (4.8)

25 (9.9)

6 (4.3)

23 (7.1)

69 (6.7)

ENT

35 (11.2)

23 (9.1)

7 (5.1)

21 (6.5)

86 (8.4)

Others

22 (7.0)

33 (13.1)

11 (8.0)

21 (6.5)

87(8.5)

Not defined

2 (0.6)

9 (3.6)

17 (12.3)

2 (0.6)

30 (2.9)

Total

313 (30.5)

252 (24.6)

138 (13.5)

323 (31.5)

1026 (100)

Table 2:Frequency distribution of referral letters from health centers according to relevant items

Components of referral letter

Not present and/or not clear

No (%)

Present and clear

No (%)

Present complaint

94 (9.2)

925 (90.8)

Duration of complaint

489 (48)

530 (52.0)

Physical examination

637 (62.5)

383 (37.5)

Differential diagnosis

471 (46.2)

548 (53.8)

Name of the referring physician

464 (45.5)

556 (54.5)

Specialty to which patient is referred

36 (3.5)

938 (96.5)

Patient demographic data

Age

0 (0.0)

1020 (100.0)

Sex

0 (0.0)

1020 (100.0)

Reason for referral

2 (0.2)

1024 (99.8)

Date of referral

243 (23.8)

776 (76.2)

Patients’ file number

287 (28.2)

732 (71.8)

Health Center

6 (0.6)

1013 (99.4)

Hospital

36 (3.5)

983 (96.5)

Table 3: Frequency of components of referral feedback reports

Components of feedback reports

Percentages (%)

Not present/not clear

Present and clear

Name of the attending physician

81.8

18.2

Result of examination

41.5

58.5

Diagnosis

86.0

14.0

Plan of management

51.7

48.3

Recommendations

24

76.0

Decision on follow-up

80.5

19.5

Answering specific questions from PHC doctors

58.3

41.7

Patient record number

30.4

69.6

Follow-up appointment

83.3

16.7

Table 4: Frequency of scores for the contents of referral letters by region (n=1019)

REGIONS

Scores

Riyadh

No (%)

Hail

No (%)

Gazan

No (%)

Northern

No (%)

Total

No (%)

1

0 (0)

0 (0)

0 (0)

0 (0)

0 (0)

2

0 (0)

0 (0)

0 (0)

0 (0)

0 (0)

3

0(0)

0 (0)

0 (0)

1 (0.1)

  1 (0.1)

4

0 (0)

8 (0.8)

0 (0)

8 (0.8)

16 (1.6)

5

0 (0)

11 (1.1)

0 (0)

10 (1.0)

21 (2.1)

6

   1 (0.1)

12 (1.2)

  1 (0.1)

6 (0.6)

20 (2.0)

7

   6 (0.6)

  1 (0.1)

10 (1.0)

2 (0.2)

19 (1.9)

8

17 (1.7)

  1 (0.1)

20 (2.0)

11 (1.1)

49 (4.9)

9

24 (2.4)

4 (0.4)

68 (6.7)

11 (1.1)

107 (10.5)

10

60 (5.9)

15 (1.5)

83 (8.1)

23 (2.3)

181 (17.8)

11

59 (5.8)

7 (0.7)

52 (5.1.)

59 (5.8)

177 (17.4)

12

53 (5.2)

34 (3.3)

63 (6.2)

78 (7.7)

228 (22.4)

13

48 (4.7)

25 (2.5)

26 (2.6)

40 (3.9)

139 (13.6)

14

20 (2.0)

12 (1.2)

10 (1.0)

19 (1.9)

61 (6.0)

Total

288 (28.3)

130 (12.8)

333 (32.7)

268 (26.3)

1019 (100)

Table 5: Frequency of scores for the components of feedback reports (n=896)

REGIONS

Scores

Riyadh

No (%)

Hail

No (%)

Gazan

No (%)

Northern

No (%)

Total

No (%)

1

5 (0.6)

   9 (1.0)

  1 (0.1)

  6 (0.7)

21 (2.3)

2

18 (2.0)

  5 (0.6)

26 (2.9)

12 (1.3)

61 (6.8)

3

56 (6.3)

12 (1.3)

54 (6.0)

43 (4.8)

165 (18.4)

4

50 (5.6)

17 (1.9)

57 (6.4)

73 (8.1)

197 (22.0)

5

54 (6.0)

11 (1.2)

72 (8.0)

65 (7.3)

202 (22.5)

6

21 (2.3)

16 (1.8)

18 (2.0)

29 (3.2)

84 (9.4)

7

15 (1.7)

7 (0.8)

39 (4.4)

15 (1.7)

76 (8.5)

8

10 (1.1)

18 (2.0)

16 (1.8)

  5 (0.6)

49 (5.5)

9

7 (0.8)

2 (0.2)

32 (3.3)

  0 (0.0)

41 (4.5)

Total

236 (26.3)

97 (10.8)

315 (35.2)

248 (27.7)

896 (100)

Table 6: Assessment of referral letters and feedback report according to the scores in different regions

Quality

Riyadh

No (%)

Northern Province

No (%)

Hail

No (%)

Gazan

No (%)

Total

No (%)

Referral letter (n=1019)

0-9 (poor)

48 (16.7)

49 (18.3)

37 (28.5)

99 (29.7)

233 (22.9)

10-14 (Good)

240 (83.3)

219 (81.7)

93 (71.5)

234 (70.3)

786 (77.1)

Chi-square for trend = 18.93

Odd Ratios

1.00

1.12

1.99

2.12

Feedback reports (n=896)

0-6 (poor)

204 (86.4)

222 (89.5)

70 (72.2)

228 (72.4)

724 (80.8)

7-9 (Good)

32 (13.6)

26 (10.5)

27 (27.8)

87 (27.6)

172 (19.1)

Chi-square for trend=24.1

Odd Ratios

0.41

0.3

1.0

0.99

p-value=0.000


 

by primary health care should be efficient and cost-effective.  This certainly should include providing the specialist in hospital with adequate clinical information about the patient, the initial evaluation of his condition, and the specific questions which need to be addressed.

The feedback from specialists to PHC doctors was found to be deficient in some respects. For  example, there  should  be responses to the referring physician’s questions and an indication of a clear and complete management plan to be followed. In  this  study, the specialists in hospitals did not address nearby 60% of the questions. Ignoring such questions widens the gap  between  the   PHC physician and the specialist, and may endanger patient care.

It seems regrettable that a significant proportion (62%) of the letters and reports were not legible. Hardly any benefit can be derived from letters or reports that are illegible. Such letters will be ignored and new information about  the patient’s  problem will be sought and workup done resulting in unnecessary waste of resources and time of both the PHC physician and the specialists in hospitals.

Both the PHC physicians and the specialists should recognize that referral is a bi-directional  process  which does not imply permanent transfer of responsibility for patient care to either party. It is obvious that inadequate  or unclear information will affect the decision and the attitude of the recipient. Byrd and Moskwitz have reported that the overall satisfaction of the referring physician was dependent on the clarity and promptness of the consultant’s reply, and on the clarity of his responses to specific questions.2

The educational benefits to the referring physician from referral needs some emphasis. The additional useful information provided to him helps him to improve the management of the referred patients, and improves the continuity of care. Poor quality feedback reports leaves the PHC physician with limited options on how to evaluate and manage the patient. The results of the referral also enable him to draw up an effective management  plan. Not  communicating  these results to the PHC physician, has a negative effect on decision in future referrals. The referral threshold may increase to the detriment of the patient.

The very high rate of poor feedback reports (80%) is striking. Despite the fact that the facilities in hospitals are expected to be better than those in PHC, and the fact that the referral system has reduced the workload in hospitals,14 the specialists still do not seem to be aware of the importance of providing adequate information to PHC doctors, and the effect of this formation on patient care and its continuity. It is also possible that specialists consider referral as a permanent transfer of patient care and, consequently do not find it necessary to provide detailed information to the referring physicians. The referral system and the patient’s care at large  would benefit if this situation is clarified in formal administrative meetings in the hope of opening up communication channels between PHC doctors and hospital staff. Standardization of the referral forms was recommended by a group of general practitioners in a previous study.15 It is interesting to note that although the referral forms in the  present  study were standardized, this did not guarantee good quality of the contents of the letters and reports. The problem faced may reflect the attitude of the physicians rather than  their knowledge  about  the components of a standard referral letter or feedback report.

The variation between the regions as regards the overall quality of referral letters is noteworthy. There were fewer letters and reports suitable for analysis from Hail region, and there were more poor quality letters from Hail and Gazan than from other regions. Gazan is remote, densely populated and has the highest workload (consultation load) among the regions under study but Hail is the reverse, which means that little effort is done to maintain quality referrals in Hail region.

One possible solution for this is to build a system of individual feedback on referrals and feedback reports to physicians involved, within the referral system in Saudi Arabia. Moreover, the involvement of the hospital and its staff in PHC tasks and activities might help in understanding the role of PHC and encourage the specialists in hospitals to respond more appropriately to the PHC doctor’s requests.

The implementation of the quality assurance program for referrals established by the national committee of quality assurance16 would  guarantee  better  monitoring and quality of the referrals. However, this should go hand in hand with better provision of relevant diagnostic and therapeutic facilities in health centers and more importantly, communication facilities in both health centers and hospitals. Both hospitals and PHC facilities should have adequate resources to maintain referral support functions.

In conclusion, the quality of referral based on quality of referral letters and feedback reports is poor and needs to be improved. This can be achieved by making the PHC physicians and specialists in hospitals aware of the problem, and understand their role in the process. The implementation of the quality assurance programme on referral will improve the situation and is highly recommended.

ACKNOWLEDGMENT

I would like to thank the Ministry of Health, Saudi Arabiafor sponsoring this work, the primary health care personnel in all the regions, and the family physicians who helped in scoring the referral letters. The  editorial comments of Prof. A. Gader are highly appreciated and my gratitude to Mr. Ejaz for his secretarial assistance.

REFERENCES

1.Lee T, Pappius EM, Goldman L. Impact of inter-physician communication on the effectiveness of medical consultations. Am J Med 1983; 74:109-12.

2.Byrd JC, Moskowitz AM. Outpatient consultation: Interaction between the general internist and the specialists. J Gen Intern Med 1987;2(2):93-8.

3.Sears CL, Charlson ME. The effectiveness of a consultation: compliance with initial recommendations. Am J Med 1983;74:870-6.

4.Epstein O. The transformation of letter writing with information technology. Br J Hosp Med 1989;41:177-81.

5.Hart JT. What sort of letters do GPs want to receive from hospital specialists? Br J Hosp Med 1989;41:175-6.

6.Blaney D,  Pullen I. Communication between psychiatrists and general practitioners: what style of letters do psychiatrists prefer? J R Coll Gen Pract 1989;39:67.

7.Marinker M, Wilkin D, Metcalfe DH. Referral to hospital: can we do better? BMJ 1988;297:461-4.

8.Scaffardi RA. Study of written communication between general practitioners and a community physiotherapist. J R Coll Gen Pract 1989;39:375-6.

9.Kentish R, Jenkins P, Lask B. Study of written communication between general practitioners and departments of child psychiatry. J R Coll Gen Pract 1987;37:162-3.

10.Jarallah JS. The quality of referral letters in two health centers in Riyadh. Annals of Saudi Medicine 1990;10(5):585.

11.Cumminus RO, Smith RW, Inui TS. Communication failure in primary care failure of consultants to provide follow up information. JAMA 1980;24(16):1650-2.

12.Hansen JP, Brown SE, Sullivan RJ, Muhlabaier LH. Factors related to an effective referral and consultation process. J Fam Pract 1982;15(4):651-6.

13.Al-Kashif Mr. Evaluation of efficiency of the referral system in rural primary health care. Annals of Saudi Medicine 1990;10(5):585.

14.Al Mazroa YY, Al-Shammari SA, Siddique M, Jarallah JS. A preliminary report on the effect of referral system in four areas in the Kingdomof Saudi Arabia. Ann Saudi Med 1991;11:663-8.

15.Newton J, Eccles M, Hutchinson A. Communication between general practitioners and consultants: What should their letter include. BMJ 1992;304:821-4.

16.Jarallah JS. Referral system; In: National Committee for Quality Assurance (ed.) Quality Assurance in PHC, MOH 1994.


-0001-11-30

HEREDITARY BLINDNESS


HEREDITARY BLINDNESS: A GENEALOGICAL STUDY AMONG AL-GHARAHIR, A JORDAN VALLEY TRIBE

Mohanna Y. Haddad, PhD, Mahmoud Y. El-Najjar, PhD

Department of Anthropology, Yarmouk University, Irbid, Jordan

مقدمة: يشكل المرض الوراثي مشكلة مستعصية في المجتمعات العربية عامة والمجتمعات الريفية العربية على وجه الخصوص.

هدف الدراسة: تعالج هذه الدراسة العمى الوراثي في عشيرة الغراغير في منطقة وادي الأردن وعلاقته بالزواج الداخلي، أي الزواج بين أبناء الأقارب.

طريقة الدراسة:عن طريق إجراء مسح ميداني في قرية الصوالحة ( الأغوار الشمالية- دير علا- المملكة الأردنية الهاشمية) باستخدام الملاحظة بالمشاركة، والاستجواب المباشر المكثف الهادف، وبناءا عليه تم جمع معلومات عن التسلسل النسبي، ودرجات القرابة بين الأزواج، ثم تم تحليل المعطيات بناءا على المعلومات الشفوية التي جمعت من أفراد عينة البحث.

النتائج والخلاصة:لقد أوضح التحليل الجينيالوجي ( الانحدار النسبي) أن العمى بين أفراد هذه العشيرة وراثيا بسبب الزواج بين الأقارب من درجات مختلفة، وان الزواج بين أجيال الأسلاف الأول كان زواجا خارجيا تحول إلى زواج داخلي بعد الجيل الثالث تنازليا، وبذلك تحول الانحدار النسبي من أحادي إلى ثنائي. ربما أن ثنائية الانحدار النسبي في العلاقة بين الأزواج تساعد على رفع نسبة الاحتمالية في تسبب العاهات الوراثي، والدليل على ذلك هو معامل الزواج الداخلي الذي يصل إلى 0.0687.

الكلمات المرجعية: العمى الوراثي، الزواج الداخلي، الأقارب، الأردن.

Background:Hereditary diseases form an omnibus problem in Arab societies in general and in rural Arab societies in particular.

Aim:Study of hereditary blindness and its relation to endogamous marriage among the Gharaghir tribe in the Jordanvalley.

Methodology: The researchers carried out a fieldwork among the Gharaghir tribe in Al-Sawalha (the  northern  valley  region,  Deir  Alla, the Hashemite Kingdomof Jordan) using participant observation, and intensive focussed interviews, in addition to the genealogical method. We gathered data on endogamous marriages, blindness, genealogy and the degrees of kinship between the spouses.

Results and Discussion:The results show that blindness among the Gharaghir was found to be associated with endogamous marriage. Marriage among the established ancestors of the clans used to be exogamous and kinship was unilineal-patrilineal. Through the shift to endogamy (marriage between close kin) there was a shift from uniliny (patriliny) to bilineal descent. This may have increased the probability of a recessive gene transmission from both parental sides. This is evidenced by the high inbreeding coefficient of 0.0687, one of the highest recorded.

Key Words:Hereditary, blindness, endogamous marriage, spouses, Jordan.

Correspondence to:

Dr. Mahmoud Y. El-Najjar,Department of Anthropology, Instituteof Archeologyand Anthropology, Yarmouk University, Irbid, Jordan


 

INTRODUCTION

Consanguineous marriage is the dominant marriage pattern in whole Jordan, reaching up to 63% of all marriages.1 Within this pattern, marriage between cousins and particularly first cousins predominant reaching an average of 38%.2 Variations are found between urban and rural areas.3 Continuity  of  marriage  between cousins throughout the successive generations seems to have led to genetic impoverishment resulting in various kinds of mental and genetic physical deformation.

                                There is a lot of published scientific information on marriages among relatives and the resultant negative effects including their generation of genetic illnesses. Cavali Sforza and Bodmer4 affirm that endogamy, particularly the marriage of close cousins is an important social factor in causing the appearance of genetically determined illnesses. This is particularly evident in the case of recessive alleles so that the genetic units existing in single forms are transmitted through marriage in the form of pairs. Some studies5,6 show that marriage among relatives is still encouraged in certain societies resulting in the emergence of genetic illnesses, such as disorders of the ear, blindness, mental retardation and malformation.7-10 Other studies provide evidence that endogamous marriage in the Third World forms one of the main reasons for various health problems. El-Najjar11 reported that endogamous marriage, especially marriage between cousins which is preferred among Kuwaitis was the cause of mental retardation and physical deformities such as deafness and blindness, which is as much as 59.1% of the sample, as compared with 5.1% among foreign residents of Kuwait. Other studies from nearby Saudi Arabiashowed a strong  correlation between  consanguinity and hereditary hearing disease among the newborn.12

Hypotheses

On the basis of the information we gathered in the field showing that endogamous marriage (particularly patrilineal cousins marriage) was the most common marriage pattern, the following hypotheses were formulated: Endogamy lead to genetic isolation; Genetic distance increases proportionally with the increase of exogamous marriage and decreases proportionally with increasing endogamy which at a given stage, leads to coincidence of patriliny and matriliny; In the latter case  the  probability  that genetically determined deviance (genetically determined illness), in this case blindness, increases more than in normal kin marriage relations as either patriliny or matriliny. Genetic studies made it obvious that genetic diseases are transmitted to children from their parents. Where these diseases exist they are transmitted through the recessive genes or alleles, especially when two mating individuals carry the same allele on the same locus.

METHODS

In this study, the genealogical method was used to trace descent and the degree of relationships in marriages among Al-Gharahgir tribe. Through several repeated visits during the summer months of 1996, the researchers (a biological and a social anthropologist) made comprehensive genealogical survey among the tribesmen by means of personal interviews with key informants selected carefully on the basis of their knowledge of the oral history of the tribe. This tribe, inhabiting the Sawalha village was chosen after a survey on possible hereditary diseases was conducted by the authors in the Jordan Valley, a large area known for the high incidence of consanguineous marriages. At the end, a genealogical tree of the whole tribe was designed and the researchers  went  back  to check on correctness, validity and completeness.

                                Afterwards, cases of blindness were traced back in ascendance to outline the degree of consanguineous marriages as in the six samples shown  in this  paper. The inbreeding coefficient for each case was then calculated based on Cavalli-Sforza and Bodmer.13 In addition, a counting process was applied to draw the distribution of marriages by clan exogamy and endogamy, by endogamous marriages, by clan and degree of kinship between spouses.

                                Our example comes from the Jordanvalley. The research was carried out among Al-Gharaghir, a tribe living in a town called Al-Sawalhah lying midway between the Dead Seaand Tiberias Lake. This is  a tribe  of  about 2000 whose livelihood comes primarily from agriculture. Their common ancestor, known as Mohammed Waheed is said to have come to the region in the first half of the 19th century, married into the tribes living there and settled permanently. His nickname was Gharghur (singular) and his sons got the same nick name Gharaghir, (in the plural). He, Gharghur had three sons: Ibrahim, Qassem and Abdullah who became the grandfathers of the three clans of Al-Gharaghir tribes.14-16 Endogamous (consanguineous) marriage among them appears to have led to genetically caused blindness as a result of genetic deformation (according to medical reports) in the iris and in the retina.

                                This paper aims  to  describe consanguineous marriage showing how it leads through continuous occurrence, to a shift from a patrilineal descent to bilineal one. In biliny, patrilineal and matrilineal descent coincide, increasing the possibility of genetically determined deformations in the offspring  of  marriage  and mating between carriers of the same recessive allele.

RESULTS

The number of children in the Gharagir tribe is 823, twenty-two of them are blind (27/1000). In fact, all blind children were examined clinically by Professor Mahmoud Al-Salim, Head of the Department of Ophthalmology at the Jordan University of Science and Technology and found that a recessive gene effecting both the iris and the retina inherited the disease.


 

Table 1:Distribution of marriages by clan and type of marriages in generations

Endogamous

Marriage

F (%)

Exogamous Marriage

F (%)

Total

F (%)

Clan 1

90 (60.4)

61 (39.6)

151 (100)

Clan 2

23 (51.1)

22 (48.9)

45 (100)

Clan 3

25 (55.5)

20 (44.5)

45 (100)

Total

138 (55.6)

123 (44.3)

241 (100)

                                Table 1 shows  clearly  that the average endogamous marriage through the seven generations that were traced genealogically in an ascending order is 60.4% for clan 1, 51% for clan 2 and 55.5% for clan 3. The average was 55.6%.

                                Table 2 shows that there had been a shift from exogamous to endogamous marriage. In the clan, the first three generations had exogamous marriages while in the fourth generation, the tribe members started to intermarry and continued to do so in the succeeding generations. This seems to have led to genetic enclosure, when there was a shift from unilineal (partilineal) to bi-lineal kinship where patriliny and materliny conjoined.

                                Table 3 shows that marriage between cousins of the first degree is the highest while that between cousins of the second degree occurs less frequently than marriage between cousins of the third and fourth degrees. Marriage between cousins forms 39.1% of marriages between relatives while it is 20.3% of all occurring marriages.

                                The two blind people B1 and B2 (Figure 1) are the result of a marriage between cousins


 

Table 3:Distrbution of Endogamy anong the Gharaghir Tribe by clan and degree of relationship

Clan

C1*

C2

C3

C4

C5

Total

Ibrahim

31

5

16

22

10

92

Qassim

11

1

3

3

5

23

Abdullah

12

1

2

3

5

23

Total

54

7

21

28

20

138

%

39.1

5.1

15.2

20.3

20.3

100

*C1=Clan1

Figure 1: Genealogies of the blind B1-B8

of the first degree. The father and mother are the result of marriages between cousins of the 3rd and 4th degrees respectively. They are related bilineally. So were the parents (in the third ascending genealogically related clans) who trace descent back to the same common ancestor  (Figure 2). Marriage  between cousins of 3rd degree produced blindness just as marriage between cousins in the 4th degree did in the case of  blind  B4-B8. Yet the marriage between cousins of the first degree which produced B3 had spouses who were descendents of parents themselves cousins of the 1st degree once removed in the mother’s line. Heredity in all the three examples was not only matrilineal, but also bilineal. Both the father and mother trace their descent back to one common ancestor in the 5th ascending generation from ego B. Both spouses in all three generations in the majority of marriages are traceable bilineally to one common ancestor. The shift from patrilineal exogamy to bilineal endogamy is seen clearly in this model.

                As in the previous example, this model shows exogamous marriage in the first three generations, except for one marriage between cousins in the third generation, which is a marriage between cousins of the 1st degree. In the fifth generation, there is one marriage between cousins of  the 1st degree, which  produced the  three  blinds B9-B11. It  seems evident that the consanguinal relations are mostly  bilineal  for  both  clans A and B in the formally  classified  partilineal   group. Marriages in  the  first   three   ancestral generations were exogamous and the shift to bilineal kinship  is  very  clear  in  the  later  generations.

                Figure 3 shows clearly that there are no consanguineous marriages in the first four ancestral generations. In the fifth generation, we found  one   consanguineous  marriage between 1st degree cousins, which produced the blinds B12-B13. Similarly, the exogamous marriage in the first four generations  did   not  prevent the

Figure 2: The Geneaology of B9-B11

manifestation of blindness in the children resulting from the marriage of cousins of the 1st degree in the sixth generation. At first sight, this diagram may appear to contradict our hypothesis but further analysis proves that because the recessive gene is transmitted patrilineally, the shift to bilineal descent within the patrilineal group through endogamous marriage is likely to  increase  the  probability  of  malformation.

                In figure 4, marriage between cousins of the 1st degree in the sixth generation, which resulted from successive marriages between 1st degree cousins in previous marriages produced blindness in B14 -17. This example makes  it  clear that the genetic defect that ran through partilineal descent in both clans A and B was enhanced  by the shift  to  bilineal descent. The shift in this diagram starts from the second descending generation.

Figure 3: Genealogy of the blind B12-13

In this model we find the marriage of two cousins once removed in the fifth and seventh generation. Despite the fact that the wife in one of the marriages in the fifth generation is  a foreigner (exogamous marriage), the cousins’  marriage  once  removed produced the blinds B18-B20. As in the previous models, consanguinity passed patrilinealy, while the shift to bilineal descent started from the second descending generation.

Figure 6  shows   one  consanguineous  marriage in the third generation and the marriage of two cousins in  the fourth generation,

one of which is of the first degree and the other of the second  degree. The fifth  generation contains a marriage between a fourth degree cousins. In the sixth generation, the marriage between cousins of the 1st degree produced two blind infants B21 and 22. The shift to bilineal descent starts in the third descending generation.

DISCUSSION

Consanguineous marriages are still widely practiced in many countries of the third world particularly in Moslem Arab countries.18-21 This type of  mating, especially marriage between cousins of the 1st degree remains an important  social  cause of  hereditary   abnormalities particularly in the case of recessive genetic traits.4 Consanguineous mating is known to cause an increase in recessively transmitted diseases, congenital malformation,  infant mortality and mental retardation.8-9 For example, Freudlich and Hino17 reported a percentage of  consanguineous marriages among Arabs living in the rural areas of Galilee in northern Israel, of up to 37% including those between cousins of 1st and 2nd degrees. Another sample, consisting of 1,546 children from the same region, Gevi et al18 found 32% of the offspring the product of consanguineous marriages between cousins of the 1st and 2nd degrees. El-Alfi et al  report  the  percentage of consanguineous marriage among Kuwaitis to be as high as 53.9%.22 Hamamy et al19 put nearby Iraq among the highest in the world (ca 70%) except for perhaps strongly isolated societies with a high genetic load. The incidence of consanguinity in recent generations has greatly declined in many parts of the world. This is particularly true of the United Statesand Western Europe, where the incidence of marriages between first cousins in the general population is approximately 0.5%. A large amount of published information show the adverse effects of consanguinity on offspring. However, genetic and demographic studies show that within many large human populations, consanguineous marriages are still strongly favored.23 For example, in the mainly Moslem countries of North Africa and Western Asia, including India and middle Asian countries of the former Soviet Union,  marriages  contracted  between persons who are second cousins or closer, account for 20% to 55% of the total number of marriages.24

In Jordan, consanguineous marriages are still predominant  reaching up to 63%.1-2 Marriages between cousins of the 1st degree account for 38.7% of this. In the sample of Algharaghir, it is clear that the occurrence of consanguineous marriages in successive generations has strongly affected the inbreeding coefficient. Khory and Massad3 and Al-Salem and Rawashdeh1 report an inbreeding coefficient for the general Jordanian population of 0.0283. The inbreeding coefficient in our sample is far higher and varies from 0.037 to 0.09374 giving an average of 0.0687.

The literature so far reviewed speaks of actual consanguineous mating. The question arises as to whether the actual accidental marriages between cousins is responsible for such harmful effects as malformations of different types. Our sample shows clearly that the generation of genetic malformation of inbreds is more or less conditioned by successive 1st degree cousin marriages for many generations transforming patriliny or matriliny to bilineal non-prescribed rule. Our view is that this transformation  increases  the likelihood of the disease manifesting when two people carrying the same recessive alleles mate.

REFERENCES

1.   Al-Salem M, Rawashdeh N. Consanguinity in North Jordan: Prevalence and pattern. J Biosoc Science 1993;25:553-6.

2.   Al-Zaghal A. Al-taghayyur fi-al-khasa’us ak-Bunyawiya Lil-Usra fi shamal al-Urdun: Dirasa Maydaniya (Change in the structural characteristics of the family in North Jordan). Irbid; Jordan; Irbid: Yarmouk UniversityPress, 1989.

3.Khory SA, Massad D. Consanguineous mariage in Jordan. American J of Med Generics 1992;43:769-75.

4.Bodmer WH, Cavali-Sforz LL. Genetics, evolution and man. San Fransisco: WH Freeman and Co., 1976.

5.Stern C. Principles of Human Genetics. San Francisco: WH Freeman and Co. 1964.

6.Cook R, Handlip A. Mortality among offspring of consanguineous marriage in a rural area of East Jordan. J Trop Pedia 1985;2:95-9.

7.Underwood JH. Human variation and micro-evolution. New York; Prentice Hall Inc., 1979.

8.Sharockh N. Congenital abnormalities in newborns of consanguineous and non-consanguienous parents. Obstet  Gynec. New York; 1989;53:1915.

9.Feaser FC, Biddle CG. Estimating the risks for offspring of first cousin mating: An approach. Am J Hum Genetics 1976; 28:522-6.

10.Hartle DL. Our certain heritage: genetics and human diversity. Philadelphia; JB Lippencott Co., 1977.

11.El Najjar MY. Consanguinity in Kuwati. Collegium Antrhopol 1996;20:275-82.

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