1887
Volume 2003, Issue 2
  • ISSN: 0253-8253
  • EISSN: 2227-0426

Abstract

Hyperbilirubinemia is a common problem of term and preterm neonates. Two hundred four jaundiced neonates admitted to Basrah Maternity and Childrens Hospital over a 6-month period were studied to determine the frequency of pyruvate kinase (PK) deficiency, glucose-6-phosphate dehydrogenase (G6PD) deficiency and glutathione reductase (GSSG-R) deficiency. Forty two neonates (20.5%) had PK deficiency, 68 (33.3%) G6PD deficiency and 40 (19.6%) GSSG-R deficiency. Interaction of more than one enzymopathy was found in 36 neonates (17.6%). Other hemolytic causes of jaundice were ABO incompatibility in 50 (24.5%) of neonates and Rh. incompatibility in 18 (8.8%). In 38 neonates no cause of jaundice was identified. There was a statistically significant increase in the frequency of G6PD deficiency and more than one enzymopathy with increasing severity of jaundice. The highest frequency of kenricterus was found in those with more than one enzymopathy. Red cell enzymopathies are an important cause of jaundice in Iraqi neonates and the presence of more than one enzymopathy carries a greater risk of developing severe jaundice.

Loading

Article metrics loading...

/content/journals/10.5339/qmj.2003.2.8
2003-12-01
2024-03-28
Loading full text...

Full text loading...

References

  1. Segel GB. Disease of the blood. In: Behrman REKliegman RMJenson HB, eds. Nelson textbook of pediatrics. 16th edition. philadephia: WB Saunders company 2000;:14881489.
    [Google Scholar]
  2. King DJ. Disorders of the blood and reticuloendothelial system. In: Campbell AGMcintosh N, eds. Forfar and ArneU's textbook of pediatrics. 5th edition: Churchill 1998;:859860.
    [Google Scholar]
  3. Valentine WN, Tanaka KR, Paglia DE. Pyruvate kinase and other enzyme deficiency disorders of the erythrocyte. Metabolic basis of inherited disorders of the erythrocyte. 6th edition. New York: McGraw Hill Company 1989;:23412365.
    [Google Scholar]
  4. Millar DR, Bachner RL. Blood disease of infancy & childhood. 6th edition: The CV Mosby Company 1989;:318322.
    [Google Scholar]
  5. Gracia SC, Moragon C, Lopes-Fernandez ME. Frequency of glutathione reductase, pyruvate kinase and glucose-6- phosphate dehydrogenase deficiency in Spanish population. Hum. Hered. 1979; 29::310313.
    [Google Scholar]
  6. Tanaka KR, Palgia DE. Pyruvate kinase deficiency. Sem. Hematol. 1971; 8::367396.
    [Google Scholar]
  7. El Hazmi MA, Al Swailem AR, Al Faleh FZ, et al., Frequency of glucose-6-phosphate dehydrogenase, pyruvate kinase and hexokinase deficiency in the Saudi population. Hum Hered. 1986; 36::4549.
    [Google Scholar]
  8. Zi-Liang Wu, Wei-Dong Yu, Shun-Cun Chen. Frequency of erythrocyte pyruvate kinase deficiency in Chinese infants. Am. J of Hematology. 1985; 20::139144.
    [Google Scholar]
  9. Valentine WN, Tanaka KR, Paglia DE. Hemolytic anemias and erythrocyte enzymopathies. Annals of Internal Medicine. 1985; 103::245257.
    [Google Scholar]
  10. Warsy AS, El Hazmi MAF. Glutathione reductase deficiency in in Saudi Arabia. East Mediterranean Health J. 1999; 5::12081212.
    [Google Scholar]
  11. Al Naama MM, Al Naama LM, Al Sadoon TA. Frequencies of G6PD, PK and hexokinase deficiencies in Basrah population in Iraq. Screening. 1995; 5::2734.
    [Google Scholar]
  12. Salman KA, Al Naama MM, Al Naama LM. Glucose- 6- phosphate dehydrogenase phenotypes in Basrah. Dirasat. 2000; 27::9095.
    [Google Scholar]
  13. Beutler E, Mitchell M. Special modifications of the fluorescent screening method for G6PD. Blood. 1968; 32::816818.
    [Google Scholar]
  14. Al Naama LM. Efficiency of screening methods used in detecting erythrocytes G6PD deficiency. The Med J Basrah University. 1995; 13::3142.
    [Google Scholar]
  15. Organization World Health. Standardization of procedures for the study of glucose-6-phosphate dehydrogenase, Tech Rep Ser. No. 366. Geneva: WHO 1969.
    [Google Scholar]
  16. Beutler E. Red cell metabolism « A manual of biochemical methods». 2nd ed. New York: Grune & Stratton 1975.
    [Google Scholar]
  17. Beutler E, Blume KG, Lohr GW, et al., International committee for standardization in Hematology recommended methods for red cell enzyme analysis. Br J Haematol. 1977; 32::331340.
    [Google Scholar]
  18. Augustine MC. Hyperbilirubinemia in the healthy term newborn. Nurse pract. 1999; 24::2426.
    [Google Scholar]
  19. Rahman Khan MA, Hameed A, et al., Erythrocyte G6PD deficiency and neonatal jaundice. J pak. Med. Assoc. 1995; 45::259260.
    [Google Scholar]
  20. Tanphiachitr VS, Pung-amritt P, Yodthong S. G6PD deficiency in the newborn: its prevalence and relation to neonatal jaundice. Southeast Asian J Trop. Med. Pub Health. 1995; 26:sup 1 (10):137141.
    [Google Scholar]
  21. Dawodu A, Qureshi MM, Moustafa IA, et al., Epidemiology of clinical hyperbilirubnemia inAl Ain, United Arab Emirates. Ann. Trop. Pedia. 1998; 18::9399.
    [Google Scholar]
  22. Casado A, Casado C, Lopez-Fernandez E, et al., Enzyme deficiency in neonates with jaundice. Panminerva Med. 1995; 37::175177.
    [Google Scholar]
  23. Chuu WM, Lin DT, Lin KH, et al., Can severe neonatal jaundice be prevented by neonatal screening for glucosesphosphate dehydrogenase deficiency? A review of evidence. Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi. 1996;:333341.
    [Google Scholar]
  24. Al Naama LM, Al Sadoon IA, Al Naama MM. Neonates jaundice and G6PD deficiency in Basrah. Annal Trop. Pedia. 1987; 7::134138.
    [Google Scholar]
  25. Chen F, Zhang Y, Wu Z. The role ofNADPH in the development of neonatal jaundice with G6PD deficiency. Zhonghua Yi Xue Za Zhi. 1997; 77::278281.
    [Google Scholar]
  26. Frischer H. Erythrocytic glutathione reductase deficiency in a hospital population in the United States. Am J Hematol. 1977; 2::327334.
    [Google Scholar]
  27. Wu ZL, Chen FX, Lai YH. Mechanism and prevention of hemolysis in jaundiced infants in phototherapy. Chung-Hva-IHsueh-Tsa-Chih. 1994; 74::364366.
    [Google Scholar]
http://instance.metastore.ingenta.com/content/journals/10.5339/qmj.2003.2.8
Loading
  • Article Type: Research Article
This is a required field
Please enter a valid email address
Approval was a Success
Invalid data
An Error Occurred
Approval was partially successful, following selected items could not be processed due to error