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Biomedical subjects

J Kasturi

Publications and source records attributed to J Kasturi.

10 recordsLinked to original sources

Hereditary sideroblastic anaemia in 4 siblings of a Libyan family--autosomal inheritance.

Most of the hereditary sideroblastic anaemias are inherited as x-linked recessive traits and are often pyridoxine responsive. The present paper describes the classical features of sideroblastic anaemia in 2 male and 2 female siblings of a Libyan family. All 4 children had severe anaemia and moderate hepato-splenomegaly. The equal severity of the disease in all 4 family members suggests autosomal inheritance.

Anemia, Sideroblastic↗

Congenital dyserythropoietic anaemias: splenectomy as a mode of therapy.

3 cases of congenital dyserythropoietic anaemia (type I, case 3; type III, cases 1 and 2) are reported with a follow-up of 8-10 years. Conventional therapy with vitamins, steroids and/or anabolic hormones was ineffective. Regular blood transfusions were required to maintain the haemoglobin levels around 8 g/dl. Splenectomy, however, resulted in a sustained rise in haemoglobin without blood transfusions. During the follow-up period varying from 40 to 77 months after splenectomy, diabetes mellitus was detected in all 3 patients. Liver biopsy showed haemosiderosis in 2 patients with cirrhosis in one of them. It is suggested that splenectomy could be an effective mode of therapy to reduce the need for blood transfusion in congenital dyserythropoietic anaemias.

Adult↗

Platelet functions in dysproteinaemia.

Platelet functions were studied in 16 patients with multiple myeloma (MM) and 4 with primary macroglobulinemia (PM). The fall in immunoglobulins on therapy was correlated with the status of platelet function. Effect of incubation of normal platelets with immunoglobulins derived from these patients on kaolin-induced platelet factor 3 (PF3) release was studied to elucidate the mechanism of platelet function defect. Results show increased bleeding time, absence or poor platelet adhesion and aggregation, poor PF3 availability and reduced total PF3 in platelets more consistently in PM but to a lesser extent in MM. Normalisation or partial reduction in globulins on therapy was associated with improvement in platelet functions. Incubation of normal platelets with immunoglobulins enhanced the kaolin-induced PF3 availability. It is suggested that in vivo platelet activation may bring about the PF3 release which subsequently manifests as poor PF3 availability and reduction in its total contents.

Blood Platelets↗

Pulmonary vascular changes associated with isolated mitral stenosis in India.

Pulmonary vascular changes were studied in 100 cases of isolated mitral stenosis; these included 90 patients in whom lung biopsies were obtained at valvotomy and 10 patients who came to necropsy. Medial thickness of the pulmonary arteries was measured in each case and in 12 cases was correlated with the haemodynamic data. Most patients were young, 78 being 30 years of age or less and 42 under 20 years or less. Males predominated 2:1. All patients with mitral stenosis showed varying degrees of vascular and other associated parenchymal changes. The most conspicuous were those observed in the muscular branches of the pulmonary artery in which the media was thickened in all cases, moderately in 44 and considerably in 28 cases. Dilation lesions representing grade 4 lesions of hypertensive pulmonary vascular disease (Heath and Edwards, 1958), hitherto not described in mitral stenosis, were observed in 4 cases. The intima was found to be frequently abnormal, showing oedema, fibrosis, and, more importantly, variable degrees of muscularization, often suggesting the incipient formation of a second media. Arteries and arterioles were often occluded by thrombi in various stages of organization, and the freshly formed channels tended to acquire a muscular lining. Arterioles were muscularized in all cases, and in many there was a pronounced intimal proliferation. Other changes included medial hypertrophy in the veins and and occasional muscularization and dilatation of the lymphatics. A notable feature was hypertrophy of the musculature of the bronchiolo-alvelar system seen in a majority of cases. The alveolar walls showed variable degrees of thickening and fibrosis, intimal proliferation of alveolar capillaries, and "epithelialization" of alveoli. Haemosiderosis was present in 70 cases. On the whole the more severe changes were observed more often in the younger subjects, further supporting the observation that rheumatic mitral stenosis in India commonly affects the juvenile age groups and is characterized by association with severe pulmonary hypertension. Medial hypertrophy was proportional to the level of pulmonary artery pressure.

Adolescent↗