First case of childhood Takayasu arteritis with renal artery aneurysms

Takayasu arteritis is a large vessel systemic granulomatous vasculitis characterized by stenosis or obliteration of large and medium sized arteries. It commonly involves elastic arteries such as the aorta and its main branches. Renal artery involvement is rare and has not been reported in a child. We report a 12-year-old boy with Takayasu arteritis who developed severe hypertension, proteinuria, microscopic hematuria and renal dysfunction. Conventional angiography demonstrated aneurysms of both renal arteries and multiple microaneurysms of the superior mesenteric artery. This case report illustrates that the children with Takayasu arteritis can develop renal involvement resulting in hematuria, proteinuria and even renal failure

Case Report - Nephritis and Cerebellar Ataxia: Rare Presenting Features of Enteric Fever

Enteric fever is a common infectious disease of the tropical world, about 80% of these cases occur in Asian countries. Enteric fever presenting with isolated cerebellar ataxia or nephritis is rare. We report three cases of enteric fever that presented with these complications. Isolated cerebellar ataxia usually occurs in the second week, whereas in our cases it presented within first four days of fever. The common complications of enteric fever related to the urinary tract are cystitis, pyelitis, and pyelonephritis. Glomerulonephritis is uncommon. Most patients with enteric glomerulonephritis present with acute renal failure, hypertensive encephalopathy, or nephritic syndrome. In comparison, our case had milder manifestations. All three patients were treated with parenteral ceftriaxone and showed a prompt recovery

Incomplete Kawasaki Disease with Recurrent Skin Peeling: A Case Report with the Review of Literature

Kawasaki disease (KD) is an acute systemic vasculitis of unknown aetiology that has largely replaced rheumatic heart disease as a cause of acquired heart disease in children of many developed countries. We report a case of incomplete KD in a five-year-old girl. The diagnosis of incomplete KD was made after exclusion of conditions with similar presentation. She was treated with intravenous immunoglobulin following which she made an uneventful recovery but demonstrated thrombocytosis in the second week of convalescence. During the six-month follow up period, she had two episodes of recurrent skin peeling a phenomenon, which is recently reported with KD but not with atypical or incomplete KD. It is important for the treating physicians to become aware of the incomplete KD as prompt diagnosis and early treatment of these patients with intravenous immunoglobulin is vital for the prevention of lethal coronary complications. Physicians need to have a "high index of suspicion" for KD and even, higher for IKD. (J Postgrad Med 2003;49:72-74

Case Report - Ovarian Dysgenesis with Balanced Autosomal Translocation

Autosomal translocations are rare in the patients with ovarian dysgenesis. An 18-year-old female who presented with primary amenorrhoea had hypergonadotropic hypogonadism and streak ovaries with hypoplastic uterus. Karyotype analysis revealed a balanced autosomal translocation involving chromosomes 1 and 11. The probable role of autosomal translocations in ovarian dysgenesis has been discussed

Study of Takayasu's Arteritis in Children: Clinical Profile and Management

AIMS: To study clinical features, pattern of involvement and treatment modalities of Takayasu's arteritis (TA) in children. MATERIAL AND METHODs: Retrospective analysis of hospital records of children admitted over a period of 4.5 years. Results: 17patients in the age group of 5 to 11 years (M: F = 1.1: 1) were diagnosed to have TA on the basis of Ishikawa's criteria. One child was diagnosed post-mortem. The commonest presenting features were hypertension (64.7%), congestive cardiac failure (47%), weak or absent peripheral pulses, cardiomyopathy (41.1% each) and cardiac valvular affection (35.2%). Retinopathy, hypertensive encephalopathy and abdominal bruits were uncommon. None presented in the prepulseless phase. No patient had an active tuberculous lesion, although Mantoux or BCG test was positive in 6 (35.2%). The predominant pattern of angiographic affection was Type II (52.9%). Nephrotic syndrome and portal cavernoma seen in one patient each were incidental associations. Anti-hypertensive drugs, oral steroids and drugs to control congestive heart failure were the mainstays of medical management. Antitubercular therapy was started in six patients. Angioplasty was attempted in 15 cases and proved to be partially beneficial in six. Three patients who failed to respond to medical management had to undergo surgical procedures, either bypass, nephrectomy or both. In-hospital mortality was 11.7%. Conclusions:of TA in this study presented acutely in the pulseless phase, with hypertension and its complications. Type II involvement was the commonest pattern. Anti-hypertensive agents and steroids along with angioplasty were partially successful in controlling symptoms in 35.2%. Surgical procedures were reserved for a minority with poor response to drugs and angioplasty

Case Report - Neurofibromatosis-Noonan Syndrome or LEOPARD Syndrome? A Clinical Dilemma

Neurofibromatosis (NF), Noonan syndrome (NS), and LEOPARD syndrome are all autosomal dominant conditions, each being a distinct clinical entity by itself. Rarely, one encounters cases with features of NF and NS and is termed as the 'Neurofibromatosis-Noonan syndrome' (NF-NS). The authors report a clinical dilemma with major clinical features of the NF-NS syndrome and LEOPARD syndrome co-existing in the same patient. Also, features of Noonan syndrome and LEOPARD syndrome are compared with the case reported