Mesangial C4d Deposits in Early IgA Nephropathy.

BACKGROUND AND OBJECTIVES: The prognostic value of mesangial C4d deposits in IgA nephropathy has been analyzed in patients with reduced GFR but has not been analyzed in those with normal kidney function. The main objective of the study was to analyze the prognostic value of C4d deposits and association with response to treatment in patients with IgA nephropathy and normal GFR. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: This retrospective cohort study included 190 patients with idiopathic IgA nephropathy diagnosed by kidney biopsy between 1988 and 2005. The patients had GFR≥80 ml/min per 1.73 m2 at the time of diagnosis, and they had a paraffin-embedded kidney biopsy with eight glomeruli available. RESULTS: In total, 170 (89%) and 20 (11%) patients were >18 and <18 years old, respectively; median (interquartile range) follow-up was 15 (12-22) years. Mesangial C4d deposit prevalence was 20% (38 of 190). At diagnosis, C4d-positive versus -negative patients had higher protein-to-creatinine ratio (median [interquartile range]: 1.94 g/g [0.9-3.1] versus 1.45 g/g [0.9-2.2]; P=0.04). During follow-up, C4d-positive patients showed a higher number of nephritic flares (median [range]: 1.4 [0-5] versus 0.9 [0-2]; P=0.04), had a higher protein-to-creatinine ratio (median [interquartile range]: 1.32 g/g [0.7-1.7] versus 0.89 g/g [0.1-1.3]; P<0.01), were more prone to receive repeated treatment with corticosteroids (45% versus 24%; P<0.01), and showed a larger reduction in eGFR (-1.6 versus -0.8 ml/min per 1.73 m2 per year; P=0.04). Furthermore, the presence of mesangial C4d deposits was an independent predictor of long-term kidney survival. CONCLUSIONS: C4d deposits may be one of the earliest poor prognostic variables available for patients with idiopathic IgA nephropathy and normal kidney function at the time of diagnosis. However, Cd4 deposits alone are not associated with the response to angiotensin blockers or corticosteroid treatment.

Kidney transplantation in children weighing 15 kg or less is challenging but associated with good outcome.

OBJECTIVE: Pediatric kidney transplantation (KT) in small children is assumed to be related to potential surgical complications that may cause severe morbidity and graft loss. The aim of our study was to analyze the outcome of KT recipients weighing ≤15 kg, focusing on surgical complications, associated morbidity and mortality, as well as allograft loss. METHODS: We reviewed our retrospective institutional database for recipients of KT between January 2000 and December 2014 with body weight ≤15 kg. RESULTS: Forty-four children weighing ≤15 kg, out of a total of 164 children (26.8%), received a deceased donor KT at our center during the study period. Mean weight was 10.10 ± 2.9 kg (3-15 kg), and weight was ≤10 kg in 23 patients (52.3%). The allograft was implanted intraperitoneally in two cases (4.5%) and extraperitoneally in the remaining 42 (95.5%). Two patients received a simultaneous double liver-kidney transplant. Postoperative complications appeared in 10 patients (22.7%) and eight required reintervention. Five allografts (11.4%) were lost secondary to surgical complications. No statistically significant differences in surgical complications were observed when compared with patients weighing >15 kg. Actuarial graft survival was 81% and 73% at 1 and 5 years, respectively. No significant differences in graft survival were observed compared with patients >15 kg. Mean follow-up was 84.95 ± 50 months (1-190 months). CONCLUSIONS: Our results demonstrate that KT in children weighing ≤15 kg is challenging but not associated with increased risk of surgical complications or early graft loss.

Intracranial Hypertension in Cystinosis Is a Challenge: Experience in a Children's Hospital.

BACKGROUND: Cystinosis is a rare systemic lysosomal disease affecting mainly the kidney and eye. Ocular involvement in cystinosis is universal being the presence of cystine crystals in the cornea a diagnostic criterion and one of the earliest manifestations of the disease. Neuro-ophthalmologic manifestations are considered a rare and late complication in these patients. The aim of this article is to report the unexpectedly high incidence of intracranial hypertension in children with cystinosis at our centre. METHODS: This study included eight children (0-16 years of age) with cystinosis seen at the paediatric ophthalmology department, Hospital Universitari Vall d'Hebron (Barcelona, Spain), a tertiary hospital, over the last 5 years. RESULTS: Three girls and five boys, mean age: 9.6 years (range: 5-14 years), were studied. During follow-up, 4 out of 8 developed papilledema and confirmed high cerebrospinal fluid (CSF) pressure. The only symptomatic child presented an Arnold-Chiari anomaly with enlarged ventricles, whereas the other three, all asymptomatic, were diagnosed by scheduled fundoscopy and had normal neuroimaging studies. All four patients had at least one known risk factor for developing intracranial hypertension: initiation of growth hormone therapy, tapering of corticosteroids, acute renal failure and Arnold-Chiari malformation. Two of them required a ventriculoperitoneal shunt. CONCLUSIONS: Our results show that intracranial hypertension can occur more frequently than expected in patients with cystinosis. Furthermore, visual prognosis depends on early diagnosis and prompt treatment. A multidisciplinary approach is necessary, and we recommend fundoscopic examinations in all paediatric patients with cystinosis whether or not they present symptoms.