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التكيس الليفي في البحرين Incidence of cystic fibrosis in Bahrain

 
 

الزيارات : 78

التاريخ : 27-10-2009

 
 

عن الملف :

المرفقات : Incidence of cystic fibrosis in Bahrain.doc

 
 
   

Incidence of cystic fibrosis in Bahrain

Shaikha Salem Al Arrayed, MBChB, DHCGm PhD.*

Foud Abdulla, MBBS, DCH.**

 

 

ABSTRACT

 

 

التكيس الليفي هو مرض وراثي متعدد يتم توريثه بصبغات متنحية مؤديا إلى مرض رئوي مزمن ونقص في الأنزيم البنكرياسي, واضطراب في الشوارد السكرية. ويعتبر هذا المرض سائد بين العرق القوقازي المنحدر من العرق الأوروبي.

وتعود دراسة المراجعة هذه إلى بحث مكثف لهؤلاء المرضى عن طريق المعلومات السريرية وسجلات المستشفى في مركز السلمانية الطبي وذاك بهدف تحديد نسبة الإصابة بالتكيس الليفي في البحرين.

يحتوى التقرير على 27 إصابة مؤكدة بالتكيس الليفي لأطفال ولدوا بين عام 1987 و1994 وقد تأكد التشخيص من وجود نسب عالية لشوارد الصوديوم والكلورايد السكرية (>70mml/L).بلغت نسبة الإصابة خلال هذه الفترة إصابة واحدة بين كل 7700 وقد تم تشخيص جميع الحالات خلال السنة الأولى بعد الولادة.  كما تم تشخيص 60% من الحالات خلال الثلاثة شهور الأولى.  بلغت نسبة الذكور إلى 14/13(1:1).. وبلغت نسبة العلوص العقي (16%) . أما نسبة الوفيات في فترة ما بعد الولادة(60%). كما باغت نسبة التزاوج بأبناء الخالات والعمات والعموم والأخوال بين هذه العائلات نسبة (63%).

 

Cystic Fibrosis (CF) is a hereditary multi system disease transmitted as an autosomal recessive, leading to chronic pulmonary disease, pancreatic enzyme deficiency and abnormally high sweat electrolytes. It is considered predominantly a disorder of Caucasians of European descent.  The following study refers to an intensive retrospective search for patients with cystic fibrosis from clinical data, hospital record in Salmanyia Medical (SMC), with the aim of determining the incidence of cystic fibrosis in Bahrain.  The survey included 27 confirmed cases of cystic fibrosis born during the period 1978-1994.  diagnosis was established by sperence of a high sweat sodium and chloride (70 mmol/1).  The mean incidence during this period was found to be one in 7,700, all cases were diagnosed during the first year of life, and 60% were diagnosed in the first three months of life.  Male and female ratio was found to be 14/13 (1:1).  The incidence of meconium ileus was 16%. Mortality in the neonatal period was 60%.  First cousin marriage rate among these families was 63%.

 

 

Introduction

 

The State of Bahrain consist of a group of islands with a total area of approximately 695.26 square kilometers. Kilometers.  It is roughly half-way down the Arabian Gulf.  It has a population of 519,000 (medium projection for 1992), approximately 36% of the population is non-Bahraini.  The overall crude birth rate is 26.7% per 1000, and the infant mortality rate is 20.5% per thousand of newborns1. Cystic fibrosis is the most frequent lethal genetic disease among white Caucasian children. The commonest syndrome is a triad of chronic lung disease, pancreatic insufficiency, and elevated sweat electrolytes. The disease was first described in 1936, and its prevalence among Caucasian in Europe and North America is estimated between 1 in 1600 to 1 in 2000, it reported to be very rare amongst American Blacks (1in 17,000) and Orientals (1 in 90,000)2-8.

 

The first Arab child with the disease was documented by Salem from Lebanon in 1962 9. Recently cases were reported from Iraq, Kuwait, Palestine and Jordan. Saudi Arabia and Bahrain. But the incidence in the Arab population is still unknown 10-16. This study is an attempt to find the incidence of cystic fibrosis in Bahrain. It is a retrospective study which include 17 years review case notes of patients diagnosed for cystic fibrosis from SMC, which is the main hospital in Bahrain with a bed capacity of nearly 700.

 

Patients and Methods

 

This survey included all cystic fibrosis cases born in Bahrain in the years 1978-1994  and registered in the record department in SMC. The survey included statistical information on the births, date of confirmed diagnosis, date of death where applicable , sex, nationality, family data including consanguinity and affected sibships, clinical picture including meconium ileus, and information about investigation and treatment. All the data were confirmed from the original medical record for each patient. The clinical diagnosis of cystic fibrosis was confirmed by a sweat chloride test. The mean incidence of cystic fibrosis in Bahrain was calculated indecently for each year from the ratio of the number of children born in each year who were ascertained to have CF to the total number of live births in that year.

 

 

Results

 

The survey included a total of 27 patients (14 male/13 females) born between January 1978- and December 1994 and confirmed to have cystic fibrosis. The series included 13 families each having one affected child and 7 families each having , two affected children. Thirteen of those families had history of early child hood deaths(28 infants) these cases were not included in the  study as they were not confirmed to have CF. the minimum incidence of CF was found to be one in 7,700 over the 17 years period (table 1).  All these patients were Bahraini except two, one was Saudi the other Syrian.

 

The sex ratio was found to be M/F 1:1 (14:13), all patients were diagnosed within the first year of life, and 60% were diagnosed by 3 month of age. The consanguinity rate was high among the parents (80%).  First cousin marriage accounted for 63% of the total (Fig 2). Average birth weight was 1.5 kg. Six out of 27 (22%) had sickle cell trait one patients had sickle beta thalasaemia and 98% of patients had glucose six phosphate dehydrogenase deficiency. Mean hemoglobin concentration was 9.8 gm/dl. Meconium ileus was reported in 4 of the 27 patients giving an overall incidence of meconium of 15% in patients with cystic fibrosis. The most common pathogens found in the sputum were pseudomonas, Klebsiella and Candida.  Table 2 shows the common signs and symptoms found in these patients. The mortality rate was60% as only eleven patients are still alive. The eldest patient is now nine years old.

 

 

TABLE 1

Incidence of Cystic fibrosis in Bahrain

Between 1978 and 1994

 

 

 

 

 

Year

No. of birth

No. of cases

Incidence per Thousand

1978

9,398

22

¼,700

1979

9,395

0

0

1980

10,097

0

0

1981

11,248

1

1/1,100

1982

11,248

2

1/5,600

1983

11,633

3

1/3,900

1984

12,254

2

1/6,100

1985

12,394

1

1/12,000

1986

893

2

1/6,400

1987

12,699

3

¼.000

1988

12,555

0

0

1989

13,611

1

1/13,600

1990

13,370

1

1/13,000

1991

13,229

3

¼,000

1992

13,874

2

1/7,000

1993

14,234

3

¼,700

1994

13,941

1

1/13,000

Total

208,663

27

1/7,700

        

 

 

TABLE 2

Clinical picture & Complications Observed

In Cystic fibrosis Cases in Bahrain

Between 1978 and 1994

 

________________________________________________________

SIGN & SYMPTOMS

NO.

PERCENT

Failure to thrive

 

18

66%

Pneumonia

7

62%

Hypochloremic alkalosis

12

44%

Anaemia

10

37%

Malabsorption

10

37%

Oedema

6

22%

Meconium ileus

4

16%

Hydrocoele

2

7%

Pneumothorax

2

7%

Bleeding diathesis

2

7%

Inguinal hernia

1

3%

   

  Discussion

 

From this study the incidence of CF in Bahrain is estimated to be 1/7,700.  this represents a minimum incidence for Bahrain.  Not accounted for in this estimate are undiagnosed patients, patients who died before diagnosis and patients with milder forms of the disease who may not be diagnosed until later in life. Until a widespread screening programme is established for CF, it will not be possible to determine the actual incidence of this disease in Bahrain or elsewhere.  Warwick 1980 has proposed that the actual incidence of cystic fibrosis may be tow to three times greater than the minimum incidence.  We found that the consanguinity rate was high among the families studied. First cousin marriages accounted for 63% of the total as compared to 21% in the general population.  This is consistent with an autosomal receive inheritance pattern. the common presenting clinical was failure to thrive 66%, pneumonia62%, hypochloremic alkalosis 44%, and anemia 37% (table 2).

 

Our study chows that the incidence of meconium ileus in cystic fibrosis is 16% which is similar to other studies, as earlier reports describe the incidence of meconium ileus in a range from 7-25% of CF subject 19. Sixty percent of CF patients died (16 patients) and (70%) of these died before the first year of life (11 patients). While 11 are still alive. Pneumonia in the first place as a cause of death which is expected 20-21. these patients were treated by using chest physiotherapy, Salbutamol, Hydrocortisone, Panox capsules 10% Propylene glycol, and vitamins and different types of antibiotics.

 

Conclusion

 

 The minimum incidence of CF in Bahrain is 1/7,700 and the mortality rate is high. Registration for theses cases together with molecular genetic studies to identify the defect causing the disease in our population is needed. Early diagnosis and management together with early genetic counseling may improve the outcome for CF patients.

 

Reference

 

1.         Bahrain Health Information Centre, Health statistical abstract 1992, Ministry of Health.State of Bahrain.

 

2.         Sturgess JM,Czgiedy-Nagy E, Corey M. Thompson M. Cystic Fibrosis in Ontario. American journal of Medi­cal Genetics 1985:22:383-393.

 

3.         Kolberg H. Incidence and Survival curves of cystic fibrosis in Sweden. Acta Paediatr Scan l982;71:197-202.

4.         Allan IL, Phelan PD. Incidence of cystic fibrosis in ethnic Italians and Greeks and in Australians of pre­dominantly British origin. Acts Paediatric Scan1985 74:286-289.

 

5.         Heffer ET Cystic fibrosis in black children I Ped1977;90:324-5.

 

6.         Komi N. Fibrocystic chances of the pancreas its Japa­nese infant. Bull Tokyo Med Dent Univ 1967l4:29-36.

 

7.         Macdoueall LG. Fibrocystic disease of pancreas in African children. Lancet 1962:2:409-10.

 

8.         Reddy CR. Sita Devic-Anees Am. Prasantha Murthy 0, Eswar reddy G. Cystic fibrosis of pancreas in India j Trop Med Hyg 1970:73:59-62.

 

9.         Salem M. ldresh H. Cystic fibrosis in the Middle East. j Med Liban 1962:15:61-74.

 

10.       Katsnelson D. Cystic fibrosis in the Middle East. Ian-eel 1982;1:11-2.

 

11.       AI-Hassani M.Cystic fibrosis in Iraqi children j Trop Ped 1977:23:136-7.

 

12.       Aluwihare APR, Au SM, Ahmad MS. et al. Cystic fibrosis in Kuwait Lancet 1981 :ii:1056.

 

13.       Khan IM. Mohammed AM. Mucoviscidosis . Cystic fibrosis of pancreas in Bahrain. Rub Med Bull 1985:7:17-23.

 

14.       Al Umran K. Lamabadusuriva SF. Cystjc fibrosis in Saudi Arabia: a diagnosis not to he missed. j Trop Ped1989:35 289-290.

 

15.       Karjoo M. Bahremand M. Mihansoust G. Cystic fibro­sis in Southern Iran j Trop Ped 1984:30:195-6.

 

16.       .Abdullah MA. Katugampala M. Karrar ZAR. Cystic fibrosis in Saudi Arabia, A case report. Saudi Med j 1986 :7(2): 189-191.

 

17.       Wurwick WJ The incidence of Cystic fibrosis in Caucasian population. Helv  Paediatr .Acta I 978:33:117-25.

 

18.       Al-Arrayed S. The frequency  of Consanguineous  marriages in the state Of Bahrain. Bah Med Bull. 1995;17(2):63-66

 

19.       Crawfurd MDA Prenatal diagnosis of common genetic disorders Br Med j 1988;297:502-6.

 

20.       Danks DM. Allan jL. Phelan PD. Chapman C Mutations at more than one locus may be involved in cystic fibrosis evidence based on first cousin data and direct counting of cases. Am j Humun Genet 1983;35:838.44.

 

21.       Wilcken B. Towns Sj .Mellis CM. Diagnostic delay in cystic.. fibrosis lessons from new born screening. Arch Dis Child 1983:58:863-66.

 

 

 

Journal of the Bahrain Medical Society, Volume 8, No. 3 December 1996

 
     
 

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Features of sickle-cell disease in Bahrain

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Review of the spectrum of genetic diseases in Bahrain

23-01-2012 119
 

Clients' Satisfaction of the Premarital Counseling Service in Bahrain

22-10-2009 112
 

Knowledge, attitude, and practice of reproductive behavior in Iranian minor thalassemia couples

11-08-2009 247
 

طبيعة فقر الدم المنجلي لدى البحرينيينTHE NATURE OF SICKLE CELL DISEASE IN BAHRAIN

27-10-2009 85
 

التكيس الليفي في البحرين Incidence of cystic fibrosis in Bahrain

27-10-2009 78
 

Spectrum of b thalassemia Mutation in Bahrain

23-09-2007 71
 

Incidence of genetic disorders of haemoglobins in the hospital population of Bahrain

23-09-2007 191

الصفحات :1-2-3-4