ABSTRACT
Background: Haemophilia A and B are the most common severe bleeding disorders and are inherited as X linked recessive pattern. The main clinical manifestations include bleeding into musculoskeletal sites or soft tissues mainly causing joint impairment and thus resulting in various morbidities. Prophylaxis therapy and inhibitor management have contributed a lot to the management of haemophilia cases. However in resource poor setting countries, like India, availability of factors, prophylactic therapy is farfetched leading to joint abnormalities, decreased physical activity and thus leading to different nutritional states. Studies from developed countries reveal obesity and overweight instances in children with Hemophilia. However not many studies have been undertaken to evaluate the nutritional status of such children in India.Methods: This study was conducted in the Comprehensive hemophilia Care Centre, Victoria Hospital, attached to Bangalore Medical College. A total of 50 children were included in the study. Children aged between 4 and 18 years attending the hemophilia Clinic were included in the study. Observations and review of relevant documents were done.Results: Among 50 children of haemophilia, 18(36%) children were aged less than 10 years and 32(64%) children were aged more than 10 years. The mean age of onset of disease in haemophilia A was 27.5 months (SD of 24.84; range 6-120) and in haemophilia B was 8 months (SD of 2.72; range 3-12). The mean BMI among children aged more than 10 years was more (21.35; SD= 4.02) compared to the children less than 10 years (16.87; SD= 3.41).Conclusions: The prevalence of overweight and obesity among children with Haemophilia is more in adolescent age group compared to children in the first decade.
ABSTRACT
The Kunze-Riehm syndrome also called as Michelin tire baby syndrome (MTBS), is a rare genodermatosis, characterized by multiple symmetric circumferential folding of excess skin with the various phenotypic abnormality. The diagnosis is made on the basis of the characteristic clinical features in the literature there are approximately 31 cases reported and to the best of our knowledge, this would be the sixth case published from India. Herein authors report a rare case of Kunze- Riehm syndrome in a neonate.
ABSTRACT
To date, the College of Radiology (CoR) does not see any clear benefit in performing whole body screening computed tomography (CT) examinations in healthy asymptomatic individuals. There are radiation risk issues in CT and principles of screening should be adhered to. There may be a role for targeted cardiac screening CT that derives calcium score, especially for asymptomatic medium-risk individuals and CT colonography when used as part of a strategic programme for colorectal cancer screening in those 50 years and older. However, population based screening CT examinations may become appropriate when evidence emerges regarding a clear benefit for the patient outweighing the associated radiation risks.