Hereditary hemolytic disorders among the Ashram school children in Mayurbhanj district of Orissa.

Hemoglobinopathy and allied hemolytic disorders are important genetic and public health problems in Orissa. These cause high degree of hemolytic anemia, morbidity and mortality in the vulnerable populations. A total of 465 Ashram School children aged 6-15 years belonging to Bathudi, Bhumiz, Kolha and Santal tribes in six localities of Mayurbhanj district of Orissa were screened for hemoglobinopathy, glucose-6-phosphate dehydrogenase (G-6-PD) deficiency, ABO and Rhesus blood groups serology and any other hereditary condition. The sickle cell trait (Hb AS) was detected in Santal (1.0%), Bathudi (1.0%) and Bhumiz (0.9%) tribals. No case of homozygous sickle cell disease was detected among the tribes of Mayurbhanj district. The beta-thalassemia trait was detected in Santal (8.0%), Kolha (2.0%), Bhumiz (1.7%) and other tribal (3.8%) students. Sickle cell hemoglobinopathy and beta-thalassemia are prevalent in this district among the tribes, but the frequency is very low. The prevalence of G-6-PD deficiency is considerably high (7.7-9.8%) among the tribes of Mayurbhanj district in Orissa. Out of total 43 G-6-PD deficient subjects, there were 32 males, 9 heterozygote females and 2 homozygous females. This shows that the antimalarial drugs should be administered with caution as these cause hemolytic anemia, sometimes fatal also. The distribution of ABO and Rhesus blood groups shows the preponderance of B blood group (33.8%) over O (29.6%) and 2.1% cases of Rhesus negativity were detected among the Bathudi tribe. This pattern is consistent with the characteristic features of tribal populations in India.

Cholelithiasis in sickle cell disease in India.

Eighty cases (63 Males and 17 Females) of sickle cell disease were searched for gall stone by ultrasonography, 8 (10%) cases had gall stone. Gall stones were more common in males (11.1%) than females (5.8%). It was not found below 13 years of age. Three cases had single and five cases had multiple gall stones. Three of them had thickened gall bladder wall. Typical biliary colic was uncommon (1 case). There was no case of obstructive jaundice. HbF% level was significantly lower in cases with gall stone (12.31 +/- 3.95) than without gall stones (16.73 +/- 6.30). Episodes of aggravated anaemia and total serum bilirubin was significantly higher (1.27/pt and 4.12 +/- 1.34 mg%) in cases with gall stone than without gall stone (0.31/pt and 2.74 +/- 1.47 mg%). The above findings suggest association of greater degree of haemolysis with formation of gall stone in sickle cell disease cases.

Fetal outcome and childhood mortality in offspring of mothers with sickle cell trait and disease.

The sickle cell hemoglobinopathy is a major public health problem which causes high morbidity and mortality in India. Although the hematological and clinical profile of the patients is extensively studies. The reproductive outcome of mothers afflicted with sickle cell trait and disease is still unknown in India. In a retrospective study, we have examined the reproductive profile of 190 mothers afflicted with sickle cell, attending Medical Out-Patient Department at V.S.S. Medical College Hospital, Burla in Western Orissa, India during the year 1991-1992. Seventy-three mothers who were found normal after medical examination and were free from hemoglobinopathic disorders, anemia, jaundice, iron deficiency, etc. constituted the control group and 66 mothers with sickle cell trait and 51 with sickle cell disease formed the study group. The reproductive history was recorded for number of conceptions, fate of offspring, live birth, surviving children and childhood mortality. Hematological investigations and hemoglobin electrophoresis were done as per the standard procedure. There was no difference in mean number of livebirths per mother between controls and sickle cell trait mothers. But between the controls and sickle cell homozygotes (p < 0.01), and sickle cell trait and disease (p < 0.01) mothers, this mean number was significant. For abortions/miscarriages, the difference between controls and sickle cell homozygotes (p < 0.001), and sickle cell trait and disease (p < 0.01) mothers was highly significant. The number of stillbirths per mother in homozygous sickle cell mothers was higher (p < 0.01) as compared to controls. There were significantly higher childhood deaths in sickle cell trait (p < 0.05) and disease (p < 0.05) mothers than in the controls. It seems that the sickle cell heterozygote and hemoglobin E heterozygote mothers are genetically better fit than the sickle cell homozygotes. Further, the sickle cell disease is clinically severer than the hemoglobin E disease in India probably due to molecular diversity.

Serum immunoglobulins in sickle cell disease.

Immunoglobulins IgG, IgA and IgM were estimated in 20 cases of sickle cell disease in steady state, eleven cases of sickle cell disease in crisis and/or infection, 20 cases of sickle cell trait and 14 normal healthy controls. Immunoglobulin deficiency has not been observed in sickle cell disease. Significantly raised IgG but normal IgA and IgM found in sickle cell disease cases was probably due to chronic stimulation of reticuloendothelial cells secondary to chronic hemolysis. IgM levels were significantly higher in patients with splenic enlargement > 3 cm. There was no alteration in immunoglobulin levels in sickle cell trait.