PREVALENCE AND PREVENTIVE MEASURES FOR THALASSAEMIA IN HAZARA REGION OF N.W.F.P., PAKISTAN

Authors

  • Mumtaz Khan Burki
  • Iftikhar Qayum
  • Noaman Siddiqui

Abstract

65 cases of thalassaemia major were selected out of 300 cases of anaemia(21.667c) admitted to Paediatric Unit A in
Women and Children Hospital, Abbottabad since June 1995, based on their family history clinical data, laboratory
investigations and X-ray analyses. Most of them were between 1-5 years of age. No difference was noted between the
two sexes. Parental consanguinity was present in 49.237c of cases and non-consanguinity in 24.617c, whereas data
was not available in 26.157c. of cases.
All the patients had moderate to severe anaemia and failure to thrive as their presenting symptoms. Other
predominant associated features were splenomegaly(907c), typical facies (607c), skull changes on X-rays(607c),
hepatomegaly (45%), jaundice(107c) and repeated infections such as gastroenteritis, pneumonia, etc. Some of them,
particularly older children having received multiple blood transfusions, presented with complications such as viral
hepatitis, congestive cardiac failure (4.617c) and diabetic ketoacidosis (1.537c). A variety of problems were
encountered in diagnosis and management of these cases and are listed with suggested improvements in patient care.

References

Weatherall DJ. Chapter 50. The thalassemias. In:

Williams WJ, Beutler E, Erslev AJ & Lichtman MA

(Eds.) Haematology, 3rd edition, McGraw Hill Book

Co., Singapore; 1986: 493-521.

Saleem M. Thalassaemia in Pakistan. In: Proceedings

of the symposium on thalassaemia in Pakistan, Nov.

Eframov GD, Huisman THJ. Wrightstone RN and

Schroeder WA. Micro chromatography of

haemoglobins. II. A rapid method for the determination

of Hb A2. J. Lab. Clin. Med. 83:657, 1974.

Eframov GD. An evaluation of the methods for

quantitation of haemoglobin A2: Results from a survey

of 10,663 cases. Haemoglobin 1(8):845, 1977.

Schmidt RM and Holland S. Standardization in

abnormal haemoglobin detection. An evaluation of

haemoglobin electrophoresis kits. Clin. Chem. 20:591.

Fairbanks VF. Haemoglobinpathies and

Thalassaemias: Laboratory Methods and Case Studies.

Brian C Decker, New York, 1980, p 85.

Gan YY, Lai CM, Lee SH & Gan SH. Production of

monoclonal and polyclonal antibodies against various

haemoglobins for the detection of thalassaemias. Ann

Acad Med Singapore, Jan 1996; 25(l):75-8.

Tuzmen G, Tadmouri GO, Ozer A, et al. Prenatal

diagnosis of beta-thalassaemia and sickle cell anaemia

in Turkey. Prenat Diagn, Mar 1996; 16(3): 252-8.

Cao A, Rosatelli MC & Galanello R. Control of betathalassaemia by carrier screening, genetic counselling

and prenatal diagnosis: The Sardinian experience. Ciba

Found Symp, 1996; 197: 137-51: discussion 151-5.

Araujo A, Kosaryan M, MacDowell 1A et. al. A novel

delivery system for continuous desferrioxamine

infusion in transfusional iron overload. Br J Haematol,

Jun 2, 1996; 93(4): 835-7.

International News - The 1996 ASH Conference. In:

TIF News, March 1997: Issue no. 21: pp. 2-3.

Porter John B. Scientific News - News from the

American Society of Haematology. In: TIF News,

March 1997: Issue no. 21: p. 8.

Hoffbrand AV. Advances in treatment of thalassaemia

major. In: Proceedings of the symposium on

thalassaemia in Pakistan, Nov. 1991.

Anapliotis-Vazeos I. Chapter 1. A comparative review

of laws regarding selective abortion. In: Dimitris

Loukopoulos (Ed.). Prenatal diagnosis of thalassaemia

and the haemoglobinpathies. CRC Press, Boca Raton,

Florida, USA, 1988, pp. 1-28.

Antsaklis A. Chapter 9. First trimester prenatal

diagnosis chorionic villi sampling. In: Dimitris

Loukopoulos (Ed.). Prenatal diagnosis of thalassaemia

and the haemoglobinpathies. CRC Press, Boca Raton,

Florida, USA, 1988, pp. 131-143.

Petrou M. Role of prenatal diagnosis in control of the

thalassaemia. In: Proceedings of the symposium on

thalassaemia in Pakistan, Nov. 1991.

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How to Cite

Burki, M. K., Qayum, I., & Siddiqui, N. (1998). PREVALENCE AND PREVENTIVE MEASURES FOR THALASSAEMIA IN HAZARA REGION OF N.W.F.P., PAKISTAN. Journal of Ayub Medical College Abbottabad, 10(1), 28–31. Retrieved from https://jamc.ayubmed.edu.pk/jamc/index.php/jamc/article/view/4804

Issue

Vol. 10 No. 1 (1998): JOURNAL OF AYUB MEDICAL COLLEGE, ABBOTTABAD

Section

ORIGINAL ARTICLE

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