Malignant hypertension secondary to renovascular disease during infancy--an unusual cause of failure to thrive.

An 11-month-old girl presented with a history of failure to thrive, vomiting, polydipsia, polyuria and visual inattention. She was found to have malignant hypertension due to unilateral renal artery stenosis. This was successfully treated with percutaneous transluminal balloon angioplasty. Nearly 10 years following this initial presentation, she remains normotensive on no anti-hypertensive medications.

Blood pressure control and left ventricular mass in children with chronic kidney disease.

BACKGROUND AND OBJECTIVES: Heart disease is a major cause of death in young adults with chronic kidney disease (CKD). Left ventricular hypertrophy (LVH) is common and is associated with hypertension. The aims of this study were to evaluate whether there is a relationship between LVH and BP in children with CKD and whether current targets for BP control are appropriate. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: In this single-center cross-sectional study, 49 nonhypertensive children, (12.6 ± 3.0 years, mean GFR 26.1 ± 12.9 ml/min per 1.73 m²) underwent echocardiographic evaluation and clinic and 24-hour ambulatory BP monitoring. LVH was defined using age-specific reference intervals for left ventricular mass index (LVMI). Biochemical data and clinic BP for 18 months preceding study entry were also analyzed. RESULTS: The mean LVMI was 37.8 ± 9.1 g/m²·7, with 24 children (49%) exhibiting LVH. Clinic BP values were stable over the 18 months preceding echocardiography. Patients with LVH had consistently higher BP values than those without, although none were overtly hypertensive (> 95th percentile). Multiple linear regression demonstrated a strong relationship between systolic BP and LVMI. Clinic systolic BP showed a stronger relationship than ambulatory measures. Of the confounders evaluated, only elemental calcium intake yielded a consistent, positive relationship with LVMI. CONCLUSIONS: LVMI was associated with systolic BP in the absence of overt hypertension, suggesting that current targets for BP control should be re-evaluated. The association of LVMI with elemental calcium intake questions the appropriateness of calcium-based phosphate binders in this population.

Drug-induced lupus and antiphospholipid syndrome associated with cysteamine therapy.

Serological evidence of drug-induced lupus (DIL) and antiphospholipid syndrome (APS) were detected in a paediatric patient with nephropathic cystinosis during work-up for live related renal transplantation. Cysteamine was considered the most likely cause. Antinuclear (ANA) and antihistone antibodies disappeared after stopping cysteamine. ANA became positive after reintroduction of cysteamine. The patient's post-transplant course was complicated by severe thrombosis, with histological findings in her native nephrectomy consistent with APS. This is the first reported case of DIL and APS secondary to cysteamine therapy. Clinicians should exclude autoimmune abnormalities in patients with cystinosis, especially if patients report non-specific, unusual or unexplained symptoms.

Management of congenital nephrotic syndrome.

We reviewed the medical records of seven children with congenital nephrotic syndrome (CNS) treated by unilateral nephrectomy, captopril, and indomethacin since 1990. Clinical response to the treatment was analyzed using the Students' t-test. After a median period of 54 months (range 36-88 months) follow-up, five patients were alive at a median age of 74 (range 43-88) months. Median (range) plasma albumin rose from 11 (6-17) g/l at the start of treatment to 18 (15-22) g/l and 21 (18-25) g/l after 6 and 12 months treatment, respectively ( P=0.001 and P=0.0006). Albumin infusions per patient per month decreased from 7 (0-18) to 0 (0-30) in the 6 months post treatment ( P=0.017). The median (range) height standard deviation scores at 12 months and 30 months from onset of treatment were -1.56 (-2.96 to 0.41) and -1.43 (-2.40 to 0.90), respectively. In conclusion, management of CNS with captopril and indomethacin therapy in combination with unilateral nephrectomy achieves significant improvements in plasma albumin and reduces the need for albumin infusions and time in hospital, while growth is maintained. Second nephrectomy, dialysis, and transplantation can be delayed until the 3rd year of life or longer.

The outcome of pediatric cadaveric renal transplantation in the UK and Eire.

An analysis of all pediatric cadaveric renal transplant recipients in the UK and Eire was undertaken to review the outcomes of pediatric cadaveric renal transplantation and to consider the implications for organ allocation procedures for pediatric recipients. Factors influencing the outcome of 1,252 pediatric cadaveric renal transplants in the UK and Eire in the 10-yr period from 1 January 1986 to 31 December 1995 were analyzed by Cox proportional hazards regression, including analysis of four distinct post-transplant epochs (0-3 months, 3-12 months, 12-36 months, and beyond 36 months). At the time of analysis (December 2000), 113 (11%) recipients had died and 47% of grafts had failed. In the multi-factorial modelling, the factors significantly affecting transplant outcome were cold ischaemia time, donor and recipient age and human leucocyte antigen (HLA) matching. Epoch analysis demonstrated that these factors operated at different times post-transplant. Cold ischaemia time had a strong influence on outcome at 3 months. A highly significant increased risk of graft failure was associated with donors under 5 yr of age. Young recipients had an increased risk of failure in the short term, but beyond 1 yr post-transplant there were few failures in young recipients while a steady rate of graft loss persisted in the older children. In terms of HLA matching, the worst outcome was observed for two HLA-DR mismatched grafts, while 000 and favorably matched kidneys (100, 010, 110 HLA-A, -B, -DR mismatches) survived longest. Hence, a policy of exchanging organs on the basis of HLA matching is justified for 000 mismatched and favorably matched kidneys. The poor outcome associated with very young donors should discourage pediatric units from transplanting kidneys from such young donors. The reasons for late losses in older recipients need investigation.