Clinical profile of sickle cell trait.

OBJECTIVES: Although sickle cell trait is considered a harmless condition in ordinary circumstances, a large number of pathological conditions have been attributed to it often without a scientific basis. Many patients visit this centre with various complaints and on testing are found to be sickle cell trait. Hence it was thought necessary to analyse these cases to find out the nature of their ailments. METHODS: Two hundred cases of sickle cell trait diagnosed by sickling test and hemoglobin electrophoresis on CAM, and 150 age and sex matched control cases with normal hemoglobin phenotype from a survey were studied. Hemoglobin estimation was done in all by cyanmethemoglobin method. Besides history and clinical examination other relevant investigations were done as necessary to arrive at the diagnosis. Seven cases of sickle cell trait were asymptomatic while the rest were suffering from different conditions. 51% of sickle cell trait and 86% of control cases had mild to severe anaemia which improved with iron therapy in trait cases. Hepatomegaly (11% vs 4.6%), epistaxis (5% vs 0.66%) and cholelithiasis (3% vs 0%) was seen in significantly more number in sickle cell traits than the control cases. Splenomegaly and midsystolic murmurs were present in higher percent of cases but was not statistically significant. There was one case of epilepsy with multiple small infarcts in the brain and another with focal fits with epileptogenic focus in the left cerebral hemisphere where no other cause could be found except sickle cell trait. CONCLUSION: The ailments of sickle cell trait cases are like persons with normal hemoglobin. Anaemia is not more frequent and can improve with iron therapy. However, hepatomegaly, epistaxis, cholelythiasis are seen more frequently and minor cerebral infarcts probably can occur in sickle cell trait. These require more elaborate studies to decide their pathogensis.

Splenectomy in sickle cell disease.

INTRODUCTION: Since role of splenectomy in sickle cell disease still remains controversial, this paper evaluates the same in 32 cases of splenectomy in sickle disease patients observed by the author. MATERIAL AND METHODS: After proper diagnosis by standard methods the cases were observed for 2-3 years before deciding on splenectomy. Though acute splenic sequestration (53%) and chronic hypersplenism (21.8%) were the main indication. Two cases of splenic abscess and one case with frequent pain attacks were also subjected to splenectomy. There was one immediate post-operative death. RESULTS: The cases were followed up for 1 to > 8 years. While Acute splenic sequestration disappeared, in chronic hypersplenism cases as well as others besides a sense of general well being, steady state hemoglobin rose by > 2G/dl in 81% and significant increase in height and weight was seen in 90% and 61% cases respectively. Frequency of pain attack, fever, anaemia and need for blood transfusion improved in 79% patients. CONCLUSION: Splenectomy in selected cases appears to be an acceptable procedure in sickle disease.

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.

Clinical profile of sickle cell disease in Orissa.

Children comprised 52% of patients with Sickle Cell Disease (SCD). Types of Sickle Cell Disease encountered were SS (92.7%). SB thalassaemia (6.7%) and SD disease (0.7%). The disease was widespread in almost all castes and communities in the society; largest number of patients (20%) belonging to scheduled castes and only 1.4% were from scheduled tribes. Maximum number of cases were in the age group 2-4 and 4-6 years, many of whom died around this age. Besides attacks of pain, jaundice and anemia, frequent attacks of fever with anemia or only anemia in childhood were a predominant presenting feature. Splenic sequestration was frequent (10.1%). The patients usually had a steady state hemoglobin level of 6-10 g/dl, with which they thrived well. Fetal hemoglobin was 5-30%. Blood transfusion was not a frequent requirement, but prophylactic long acting penicillin was helpful in preventing frequency of crisis.

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.

Sickle cell disease in India.

A screening programme involving 9,822 hospitalised patients revealed the frequency of individuals with S gene to be 11.1 per cent. A population survey of 1,000 randomised subjects from amongst about 70,000 people in one block of the area showed the frequency to be 15.1%. The gene is not confined to tribal peoples, but is prevalent throughout the society, being more frequent in scheduled castes and some caste Hindus. With the available Indian data a sickle cell belt can be mapped out in the country. Analysis of clinical data on the first 700 cases of sickle cell disease seen in the Sickle Cell Research Centre (ICMR) at Burla shows patients of all ages, even beyond 40 years, though many patients tend to die by 20 years of age. Genetically, while most patients are SS and 8.1% are S-beta thalassaemia, cases of SD disease and SE disease were also encountered. A frequency of 0.32% of alpha thalassaemia gene was noted in SS patients against 0.28% in sickle cell trait and 0.12% in AA controls. The disease was found to manifest as early as 3 months or may remain asymptomatic till adult life. Though generally running a milder course, moderate to severe anaemia, vaso-occlusive attacks (86.5-89.36%), splenic sequestration (8.43%-12.76%), crippling avascular bone necrosis (5.7%-35.08%), osteomyelitis (5/700), and epistaxis (28.92%-35.08%) remain a few clinical events deserving competent and urgent medical management.(ABSTRACT TRUNCATED AT 250 WORDS)

Sickle haemoglobin, G-6PD deficiency and malaria in western Orissa.

Sixty cases of malaria were screened for sickle haemoglobin and G-6PD deficiency. Plasmodium vivax was detected in 40 (66%) and Plasmodium falciparum in 21 (35%) cases, with six of the latter having cerebral manifestation. Sickle Hb was found in 7 (11.5%) patients and G-6PD deficiency in 3 (5%) cases. Both patients with SS disease had vivax malaria, while of 5 with sickle cell trait 3 had only vivax, one only falciparum and one mixed infection. Amongst G-6PD deficient patients one had vivax and two falciparum malaria. One of the latter had both SC trait and G-6PD deficiency. Thus, adult persons with SS disease or SC trait were not found to be resistant to either vivax or falciparum malaria. A high frequency (5%) of G-6PD deficiency amongst malaria patients warrants a caution against indiscriminate use of 8-aminoquinoline drugs.

Yellow oleander (cerbera thevetia) poisoning with jaundice and renal failure.

Jaundice and renal failure in yellow oleander poisoning have not been reported previously. Similarly no post-mortem report has shown renal tabular necrosis and glomerular pathology, liver and brain changes in this poisoning. Four cases of yellow oleander poisoning with jaundice and renal failure and the postmortem findings in another three cases are reported.