Experience with multiple stent implantations in primary antiphospholipid syndrome in childhood: a case report.

The antiphospholipid syndrome (APS) is an autoimmune condition characterized by the persisting presence of antiphospholipid antibodies in association with thrombosis and/or pregnancy morbidity. Primary APS is quite rare in childhood and exact prevalence is not known. However, substantial proportion of thrombotic events in children is being attributed to APS. We herein present a 9-year-old boy presented with impending pericardial tamponade and large pleural effusions likely secondary to transudation of fluid from his gradually developed collateral circulation which was resulted from almost completely occluded vena caval system due to primary APS. He was treated with multiple angioplasty-stenting which offered symptomatic relief and better quality of life. To our knowledge, this is the first reported paediatric case of primary APS presented with extensive occlusive lesions in both caval systems and treated with repeated endovascular stent placements.

Impact of dialysis adequacy on cardiac function in pediatric CAPD patients.

BACKGROUND: Left ventricular hypertrophy is a major cause of morbidity and mortality among patients with chronic renal failure. Uremia-related risk factors play a fundamental role in its occurrence, thus better prognosis and prolonged survival can be attained by successful dialytic therapies. OBJECTIVE: To investigate whether dialysis adequacy has a beneficial effect on cardiac structure and function in children receiving continuous ambulatory peritoneal dialysis (CAPD). DESIGN: Cross-sectional study in the Pediatric Peritoneal Dialysis Unit of a university hospital. PATIENTS: Eighteen children, aged 13.3 +/- 2.8 years, being treated with CAPD, and 20 healthy age- and sex-matched control subjects were enrolled in this study. MAIN OUTCOME MEASURES: Echocardiographic evaluation was performed in all subjects. Dialysis adequacy indices [weekly urea (Kt/V) and creatinine clearance (TCCr)] were calculated in the dialysis group. RESULTS: Interventricular septal thickness, left ventricular (LV) posterior wall thickness, LV mass index (LVMI), and LV end systolic and diastolic dimensions were all found to be significantly higher in the CAPD group compared to the control subjects (p < 0.01). Ejection fraction and fractional shortening of the LV were not significantly different between the two groups. Mean Kt/V was 2.02 +/- 0.71 and mean TCCr was 58 +/- 33 L/wk/1.73 m2. There were significant negative correlations between dialysis adequacy indices and LV end systolic and diastolic dimensions (p < 0.05 and p < 0.001). Ejection fraction and fractional shortening were positively correlated with Kt/V (p < 0.01). Systolic and diastolic blood pressures were positively correlated with LVMI (r= 0.501 and r = 0.523). Significant inverse correlations between mean arterial pressure and both Kt/V and TCCr (r = -0.555 and r = -0.520) were detected. CONCLUSION: These data clearly document that cardiac structure and function are remarkably influenced by the uremic state and dialysis therapy in pediatric CAPD patients. The close relationships between echocardiographic findings and dialysis adequacy indices suggest that adequate dialysis has a beneficial effect on cardiac function via effective removal of toxic substances.

Severe renal impairment in the case of classic polyarteritis nodosa.

A 14-year-old boy with classic polyarteritis nodosa (cPAN) and a clinical picture resembling rapidly progressive glomerulonephritis (RPGN) is described. He had severe hypertension, malaise, weight loss, fever, myalgia, and rapid deterioration of renal function. Renal biopsy revealed acute necrotizing vasculitis. Angiography showed small saccular aneurysmatic dilatations in the intrarenal branches of the right renal artery and the intrahepatic branches of the hepatic artery. cPAN was diagnosed and pulse methylprednisolone (MP), pulse cyclophosphamide (CYC) and subsequently oral prednisolone were given. Clinical and laboratory findings improved dramatically and remission was attained rapidly. The patient has remained in remission for the last 11 months. cPAN should be considered in patients who present with severe systemic symptoms and hypertension. Progressive renal insufficiency can occur during the acute course of cPAN due to renal vascular involvement without glomerulonephritis. Prompt and aggressive corticosteroid and cytotoxic therapy is essential to suppress disease activity and to maintain remission.

Mumps interstitial nephritis: a case report.

Mumps is still a common childhood disease in rural areas where mumps vaccination is not widespread. A 14-year-old girl with fatal interstitial nephritis as a complication of mumps is reported. The patient had not been vaccinated with mumps vaccine and had contracted mumps during a village epidemic. The illness began with parotitis, and renal insufficiency developed within a week. The patient's renal function rapidly deteriorated and the outcome was fatal. The postmortem renal necropsy sample demonstrated interstitial mononuclear cell infiltration, edema, and focal tubular epithelial cell damage, confirming the clinical diagnosis. In developing countries routine mumps vaccination may help to prevent possible fatal complications of mumps. Furthermore, patients with mumps, especially complicated cases, should be followed closely.