Idiopathic midaortic syndrome: normalization of blood pressure on medication.

Midaortic syndrome (MAS) is a rare, idiopathic condition in children usually presenting with severe hypertension. We report a case of a 13-year-old girl who presented with severe hypertension (200/110 mmHg) associated with renal artery stenosis and normal renal function (creatinine clearance 110 ml/min/1.73m(2)). Percutaneous angioplasty (PTA) was first performed, but early recurrence of hypertension occurred. Subsequent imaging evaluation demonstrated association of aortic narrowing, proximal stenosis of the left renal artery, and wall thickening of superior mesenteric artery and right common carotid artery. Although previous large-vessel arteritis cannot be absolutely excluded, a diagnosis of idiopathic MAS was made, given the absence of any other clinical signs of inflammation (C-reactive protein <0.5 mg/dl; erythrocyte sedimentation rate 5 mm/h). Medical treatment was undertaken without repeat PTA or surgery. Blood pressure control was good, and antihypertensive therapy was stopped 4 years later. At age 22, the patient was still normotensive and receiving no antihypertensive therapy; normalization of Doppler velocities in the proximal left renal artery was confirmed. In the absence of renal dysfunction or target-organ damage, medical management of hypertension in MAS is feasible without intervention if blood pressure is well controlled on two antihypertensive agents.

Long-term follow-up of Epstein-Barr virus viremia in pediatric recipients of renal transplants.

The common observation of Epstein-Barr virus (EBV) viremia in pediatric recipients of renal transplants and the occurrence of an EBV-related pulmonary leiomyoma prompted us to intensify the follow-up of EBV infections from 1995 to October 2000. Follow-up included serology and detection of viral DNA in blood using a semi-quantitative nested polymerase chain reaction (PCR) and later a real-time PCR with higher sensitivity. The aim of this study was the early detection of primary infections or reactivations. We obtained 250 samples from 32 patients. EBV DNA detection was consistently negative in 14 patients. There were 5 patients that were considered at risk for post-transplant lymphoproliferative disease, as they were EBV seronegative and were given a kidney from a positive donor. Of these, 4 had at least one episode of high-level EBV viremia. During these episodes, an absence of noticeable symptoms that could be related to EBV was noted for all but 1 patient. This child presented with severe neutropenia 1 month after grafting and, 28 months later, several nodules of pulmonary leiomyoma, which were found to be EBV related. Four episodes of high-level viremia were observed before the discovery of the leiomyoma. Viral DNA detection is important for the follow-up of such patients that are especially at risk of serious complications of EBV infections.

Final height in children with chronic renal failure who have not received growth hormone.

Final height (FH), growth velocity after 16 and 18 years of age, and factors predictive for FH were assessed in 60 patients (21-36 years old), whose chronic renal failure (CRF) started before the age of 16 years (28 girls and 32 boys). At 16 years of age, 22 had conservative treatment (CT, group A) and 38 end-stage renal failure [ESRF, group B, which includes 19 receiving hemodialysis (HD) and 19 with a functional renal transplant (RTx)]. None received recombinant human growth hormone (rhGH) treatment. FH was lower than in a normal population: 161.6+/-8 cm for males [-2.06+/-1.3 standard deviation score (SDS)] and 154.3+/-8.1 cm for females (-1.4+/-1.4 SDS). FH in group A (-1.15+/-1.4 SDS) was significantly higher than in group B (-2.1+/-1.3 SDS); 45% of all patients (56% of males and 23% of females) had a final height below -2 SDS (41% in group A and 47% in group B). FH was reached at 20.2+/-1.8 years in males and 18.8+/-2 years in females. A continuation of growth after 18 years of age was observed in 23 males (71.8%): +5.2 cm (+0.87 SDS) and in 14 females (50%): +1.75 cm (+0.3 SDS). However, this partial recovery concerned mainly patients with an important growth deficiency. A higher height at enrolment or at ESRF was significantly associated with a higher FH, whereas a longer period of ESRF had a significantly negative effect. In conclusion, all efforts should be made to diagnose CRF as early as possible and to try to improve growth before ESRF and RTx. Early institution of rhGH therapy should improve FH and improve the chance of achieving near-normal adult height in most patients.