First-year profile of biomarkers for early detection of renal injury in infants with congenital urinary tract obstruction.

BACKGROUND: Diagnosis of renal function impairment and deterioration in congenital urinary tract obstruction (UTO) continues to be extremely challenging. Use of renal biomarkers in this setting may favor early renal injury detection, allowing for a reliable choice of optimal therapeutic options and prevention or minimization of definitive renal damage. METHODS: This longitudinal, prospective study analyzed the first-year profile of two serum renal biomarkers: creatinine (sCr) and cystatin C (sCyC); and six urinary renal biomarkers: neutrophil gelatinase-associated lipocalin (NGAL), kidney injury molecule-1 (KIM-1), transforming growth factor beta-1 (TGF-ß1), retinol-binding protein (RBP), cystatin C (uCyC), and microalbuminuria (µALB) in a cohort of 37 infants with UTO divided into three subgroups: 14/37 with unilateral hydro(uretero)nephrosis, 13/37 with bilateral hydro(uretero)nephrosis, and 10/37 patients with lower urinary tract obstruction (LUTO), compared with 24 healthy infants matched by gestational age and birth weight. RESULTS: All urine biomarkers showed significantly higher values at the first month of life (p ≤ 0.009), while NGAL (p = 0.005), TGF-ß1 (p < 0.001), and µALB (p < 0.001) were high since birth compared to controls. Best single biomarker performances were RBP in bilateral hydronephrosis and LUTO subgroups and KIM-1 in unilateral hydronephrosis subgroup. Best biomarker combination results for all subgroups were obtained by matching RBP with TGF-ß1 or KIM-1 and NGAL with CyC ([AUC] ≤ 0.934; sensitivity ≤ 92.4%; specificity ≤ 92.8%). CONCLUSIONS: RBP, NGAL, KIM-1, TGF-ß1, and CyC, alone and especially in combination, are relatively efficient in identifying surgically amenable congenital UTO and could be of practical use in indicating on-time surgery.

Flow-through peritoneal dialysis in neonatal enema-induced hyperphosphatemia.

Fleet enemas are hypertonic solutions with an osmotic action and a high concentration of phosphate. When retained in the human body they have a great toxic potential, causing severe hydro-electrolyte disorders in children, especially in newborns. We report the case of a previously healthy 8-day-old newborn who needed neonatal intensive care treatment after the inadvertent administration of an osmotically active hypertonic phosphate enema. Taking into account that phosphate removal by peritoneal dialysis (PD) strongly depends on total dialysate turnover, we chose continuous flow PD (CFPD) as the treatment option, with a successful outcome. Clinical experience with this dialytic modality is limited to a few case reports in pediatric and adult patients. To the best of our knowledge, we report here the first description of CFPD in the setting of acute phosphate nephropathy in the neonatal period. The modality of PD described here has potential as an alternative management option as it is a highly efficient, methodologically simple, and low-cost method without any need for sophisticated equipment. Physicians and parents should be aware of the adverse effects of a hypertonic phosphate enema and should never use these medications in infants and newborns.

Arterite de Takayasu em uma lactente com história de dois familiares acometidos / Takayasu's arteritis in an infant with a family with three affected siblings

Objetivos: Por um período consecutivo de 27 anos, 5367 pacientes foram acompanhados na Unidade de Reumatologia Pediátrica do Instituto da Criança. Destes, 17 (0,003%) casos tiveram o diagnóstico de arterite de Takayasu. Uma delas (0,0001%) apresentou antecedentes familiares desta vasculite primária e será descrita. Descrição: Os autores reportam uma lactente do sexo feminino, com história familiar de três irmãos acometidos por arterite de Takayasu. Esta paciente, com 8 meses de idade, apresentou acidente vascular encefálico, hipertensão arterial com diferença de pressão arterial nos quatro membros e redução dos pulsos periféricos. As provas de atividade inflamatória foram elevadas: velocidade de hemossedimentação 37 mm/1ª hora e proteína C reativa 19 mg/L. A angiorressonância evidenciou estenoses de aorta infra-renal, artérias carótidas, artérias cerebral anterior direita e renais, preenchendo os novos critérios de arterite de Takayasu. O tratamento foi realizado com corticoterapia, metrotrexate, pulsoterapia com ciclofosfamida e anti-hipertensivos, com melhora discreta da hipertensão. Conclusão: Arterite de Takayasu é uma vasculite rara em um serviço terciário de reumatologia pediátrica e este diagnóstico deve ser considerado em lactente com doença vascular e redução dos pulsos arteriais...

Objectives: For a period of 27 consecutive years, 5079 patients were followed at the Pediatric Rheumatology Unit of the Instituto da Criança. Of these, 17 (0.003%) cases had a diagnosis of Takayasu’s arteritis. One (0.0001%) had family history of primary vasculitis and will be described. Description: The authors report a female infant with familial history of three siblings affected by Takayasu’s arteritis. This patient’s at 8-month-old had stroke, hypertension with blood pressure differences in all four limbs and reduction of peripheral pulses. The evidence of inflammatory activity was high: erythrocyte sedimentation rate 37 mm/1st hour and C-reactive protein 19 mg/L. The magnetic resonance imaging showed stenosis of the infrarenal aorta, carotid arteries, right anterior cerebral arteries and kidney, filling the new criteria of Takayasu’s arteritis. The treatment was carried out with steroids, methotrexate, intravenous cyclophosphamide and anti-hypertensive drugs, with a slight improvement in hypertension. Conclusion: Takayasu’s arteritis is a rare vasculitis in a tertiary pediatric rheumatology and this diagnosis should be considered in children with vascular disease and reduction of arterial pulses...