Grebe syndrome: a very severely affected case.
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Biomedical subjects
Publications and source records attributed to M L Kulkarni.
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A hospital based study of skeletal dysplasias was conducted over a period of 2 years in Davangere, Karnataka, in which 169 cases of skeletal dysplasias were studied. One hundred were osteochondrodysplasias and were grouped according to international classification of osteochondrodysplasias. Among the individual cases, osteogenesis imperfecta (13 cases) had the maximum representation. Several cases of rare disorders were also identified. Eighty eight cases of skeletal dysplasias were in the pediatric age group and of these 41 were newborns. The incidence of skeletal dysplasia among newborns was 19.6 per 10,000 deliveries and lethal dysplasias 5.2 per 10,000 deliveries. In 7 cases of skeletal dysplasia, an antenatal diagnosis was possible by ultrasonography.
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Snake venom poisoning is a common medical emergency and the epidemiological features vary from region to region. We conducted a prospective study to review the epidemiology, treatment and outcome of snake venom poisoning in central Karnataka. Six hundred and thirty three cases of snake bite, seen in a teaching hospital, upto the age of 18 years, over a period of 8 years from 1985 to 1992 constituted the material for the study. Detailed history with special reference to the type of snake, circumstances leading to the bite and clinical consequences were studied and final outcome was noted. Males (n = 433) were bitten more often than females (n = 200). Two hundred and fifty six (40.4%) cases were in the age range of 11-15 years. The cases were seen during two periods, i.e., Oct, Nov, Dec (n = 210) and Apr, May, June (n = 199). Most (n = 506) were encountered in the lower limbs. Viper was the most common poisonous snake. Five hundred and seventy (90%) cases were from rural area. Coagulation time was prolonged in 371 (58.6%) cases, hemorrhagic syndrome was noticed in 354 (55.9%) cases, neurological involvement in 79 (12.5%) cases. Polyvalent anti snake venom (ASV) was given to 479 cases. Hypersensitivity to ASV was noted in 8 cases. Blood transfusion was given to 33 cases for the management of excessive bleeding. The death rate among snake victims was 5.2% (33 cases). The morbidity and mortality can be reduced substantially by increasing and maintaining confidence in good medical care and providing health education.
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The usefulness of single Widal test in the diagnosis of typhoid fever was investigated. The test was done on 50 normal children, 50 children with non typhoidal fevers and 30 culture proved typhoid cases. Twenty one (70%) and nine (30%) of thirty typhoid fever cases had 'O' and 'H' agglutinin titer levels of more than or equal to 1:160, respectively as compared to only 3 (3%) and to 1 (1%) among controls. These differences were significant (p < 0.001). Twenty two (73.3%) out of thirty typhoid fever cases had either an 'O' or 'H' agglutinin titer of more than or equal to 1:160 as compared to only 3 (3%) among controls. An 'O' agglutinin titer of 1:160 had a specificity of 97%, a sensitivity of 70% and an accuracy of 90%. An 'H' agglutinin in titer of 1:160 was had a specificity of 97%, a sensitivity of 30% and an accuracy of 83.1%. Based on the above analysis, 'O' or 'H' titers of 1:160 or more were indicative of typhoid fever. Similarly, when the 2 titers were considered together, either 'O' or 'H' titers of 1:160 or more were suggestive.
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Forty cases of hereditary bleeding disorders, who are the members of Karnataka Hemophilia Society (a State Chapter of the Hemophilia Federation of India) were studied to learn their problems. Twenty-four cases were thoroughly investigated including factor assay and in 16 cases the diagnosis was based on classical clinical presentation and pattern of inheritance. There were 16 cases of hemophilia A, 3 cases of hemophilia B, 2 cases of von Willebrand's disease, 1 case of factor XIII deficiency and 2 cases of hereditary afibrinogenemia. The age group varied from 3 months to 36 years; 39 were male. The age of onset of symptoms, type of bleeding, type of joints involved, disabilities experienced by these patients, severity of the disorder, presence of antibodies to factor, presence of HBsAg and HIV, the availability of treatment for these patients, treatment received and the financial burden faced by these patients were studied. Thirty-three cases (82.5%) had joint bleeds, 60% of the patients experienced various type of disabilities, 11 (27.5%) had epistaxis, 10 (25%) had GI bleeds, 5 (12.5%) had dental bleeding, 8 (20%) had haematomas, 3 (7.5%) had umbilical stump bleeds and 19 cases (47.5%) had previous blood transfusions.
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