RESUMEN
INTRODUCTION: Renal stone disease diagnosed in the first year of life is relatively uncommon. While risk factors such as low birth weight, furosemide exposure, and metabolic disorders are well established, there exists little information regarding resolution rates and need for surgical intervention. Our study objective was to evaluate urolithiasis and renal calcification resolution rates, time to resolution, and need for surgical intervention in children diagnosed in their first year of life. MATERIAL AND METHODS: REB approved retrospective chart review of children younger than 12 months of age (corrected for prematurity) diagnosed with nephrolithiasis and/or nephrocalcinosis in a tertiary pediatric hospital between April 2000 and August 2015 with a minimum 1-year follow-up period. Exact logistic regression was performed to assess the relationship between size of the largest stone (on either side) and the need for surgical intervention. Kaplan-Meier curves were constructed to examine time to stone resolution among those not requiring surgical intervention. RESULTS: 62 patients (61% male) were diagnosed with stones or nephrocalcinosis by ultrasound at a median age of 2.9 months. Of these, 37% had been admitted to the NICU because of prematurity, low birth weight or comorbidities. A total of 45 patients were found to have stones (Table); 35 of these had a stone at initial ultrasound and 10 initially diagnosed as nephrocalcinosis were later confirmed to have a stone. 67% of all stones were asymptomatic on presentation. Metabolic anomalies were present in 56% (35/62), and 16% (10/62) required medical treatment. Seven patients ultimately required surgical intervention. Stone size was found to predict the eventual need for surgical intervention (OR 3.52, 95% CI 1.47-12.78) for each 0.1 mm increase in diameter). Among patients not requiring surgical intervention (n = 38), the estimated median time to spontaneous resolution of urolithiasis was 1.1 years (95% CI 0.89-1.53, range 2 months-6 years) and 1.2 years for nephrocalcinosis (95% CI 0.59-2.13). CONCLUSIONS: Spontaneous resolution was a common outcome for newborns and infants diagnosed with urolithiasis in the first year of life, but high variability in time-to-resolution was observed. Only a small proportion who had confirmed stones on ultrasound required surgical intervention (15%), and large stone size was a predictive factor for surgery.
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Nefrocalcinosis/diagnóstico , Nefrocalcinosis/cirugía , Nefrolitiasis/diagnóstico , Nefrolitiasis/cirugía , Femenino , Humanos , Lactante , Masculino , Selección de Paciente , Estudios Retrospectivos , Resultado del Tratamiento , UltrasonografíaRESUMEN
BACKGROUND: We analyzed the impact of immunoglobulin M (IgM) positivity on the relapse-free interval post completed course of cyclophosphamide (CYC) treatment in patients with steroid-dependent nephrotic syndrome (SDNS) and minimal change disease (MCD). METHODS: This was a retrospective chart review of all children who received CYC for SDNS and MCD between 1988 and 2009. Patients were divided into three groups based on kidney biopsy: MCD without immunoglobulin M (IgM) positivity (IgM-), MCD with IgM-positive immunofluorescence (IF) only (IgM+), and MCD with IgM-positive IF and electron-dense deposits on electron microscopy (IgM++). The relapse-free time interval to the first relapse post-CYC therapy or up to 48 months of follow-up (if no relapse occurred) was used for survival analysis. RESULTS: Forty children aged 1.5-12.3 years (15 were IgM-, 16 were IgM+, 9 were IgM++) received a cumulative CYC dose of 175 ± 30 mg/kg. The overall relapse-free survival time was 75 % at 12 months, 64 % at 24 months, 59 % at 36 months, and 56 % at 48 months, with no significant differences between the IgM groups (p = 0.80). CONCLUSIONS: Based on our results, we conclude that more than 50% of our SDNS patients with MCD remained relapse-free 4 years post-CYC treatment. No significant difference in the response to CYC was observed between patients with or without IgM positivity.
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Ciclofosfamida/uso terapéutico , Inmunosupresores/uso terapéutico , Nefrosis Lipoidea/tratamiento farmacológico , Síndrome Nefrótico/tratamiento farmacológico , Niño , Preescolar , Supervivencia sin Enfermedad , Femenino , Humanos , Inmunoglobulina M/inmunología , Lactante , Masculino , Nefrosis Lipoidea/inmunología , Nefrosis Lipoidea/mortalidad , Síndrome Nefrótico/inmunología , Síndrome Nefrótico/mortalidad , Estudios Retrospectivos , Resultado del TratamientoRESUMEN
TH of infancy and early childhood is characterized by transiently increased S-ALP, predominantly its bone or liver isoforms. There are neither signs of metabolic bone disease or hepatopathy corresponding to the increased S-ALP, nor a common underlying/triggering disease. TH may also occur in children post-renal Tx, which may raise significant concerns and anxiety. We describe four patients aged 2.8-7 yr in whom the TH occurred at 11-34 (median = 28) months after Tx and lasted from 40 to 105 (median = 63) days. No obvious cause/trigger of TH could be found; the clinical status and bone turnover were not altered. In cases of TH post-Tx, we recommend the evaluation of basic biochemical indices and wrist X-ray. If these results are normal, TH is most likely the diagnosis and the S-ALP can be monitored over the next three months without further testing. In patients with persisting TH for more than three months and/or in children with pre-existing or suspected metabolic bone disease, further evaluation may be indicated. In conclusion, TH is a benign disorder in patients post-Tx. Detailed investigation including bone biopsy is only indicated in patients with persisting TH.
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Hiperfosfatemia/etiología , Trasplante de Riñón/métodos , Insuficiencia Renal/terapia , Fosfatasa Alcalina/sangre , Biopsia , Huesos/patología , Niño , Preescolar , Femenino , Humanos , Hiperfosfatemia/terapia , Masculino , Síndrome Nefrótico/terapia , Isoformas de Proteínas , Radiografía , Resultado del Tratamiento , Muñeca/diagnóstico por imagenRESUMEN
Acute renal failure (ARF) is a rare but potentially fatal complication of diabetic ketoacidosis (DKA). Early recognition and aggressive treatment of ARF during DKA may im-prove the prognosis of these patients. We present a case report of a 12 year old female admitted to the hospital with severe DKA as the 1s t manifestation of her diabetes mellitus. She presented with severe metabolic acidosis, hypophosphatemia, and oliguric ARF. In addition, rhabdomyolysis was noted during the course of DKA which probably contributed to the ARF. Management of DKA and renal replacement therapy resulted in quick recovery of renal function. We suggest that early initiation of renal replacement therapy for patients with DKA developing ARF may improve the potentially poor outcome of patients with ARF associated with DKA.
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Lesión Renal Aguda/etiología , Diabetes Mellitus Tipo 1/complicaciones , Cetoacidosis Diabética/complicaciones , Lesión Renal Aguda/terapia , Niño , Diabetes Mellitus Tipo 1/diagnóstico , Diabetes Mellitus Tipo 1/terapia , Cetoacidosis Diabética/etiología , Cetoacidosis Diabética/terapia , Femenino , Humanos , Hipofosfatemia/etiología , Oliguria/etiología , Terapia de Reemplazo Renal , Rabdomiólisis/etiología , Índice de Severidad de la Enfermedad , Resultado del TratamientoRESUMEN
Few studies report Ganciclovir or Valganciclovir levels in children. Single-center, retrospective study of all available Ganciclovir levels in transplanted children. Ganciclovir monitoring was performed as previously described [G. Filler (1998); Pediatric Nephrology, 12, 6]. For the normalization of dosing to GFR and target trough levels, we assumed first-order kinetics. We analyzed 57 Ganciclovir levels in 20 children (mean age 8.6 +/- 5.5 yr) treated with intravenous or oral Ganciclovir or oral Valganciclovir. Ganciclovir levels were drawn after IV therapy (n = 9), during oral Ganciclovir (n = 5), or during oral Valganciclovir (n = 15). Oral bioavailability of Valganciclovir was 42.0 +/- 21.8%. The dose-normalized intrapatient Valganciclovir variability was 83%. Mean GFR was 92 +/- 22 mL/min/1.73 m(2). Mean Ganciclovir concentration at last available measurement was 0.60 +/- 0.09 mg/L. While target trough Ganciclovir levels have not been established, possibly subtherapeutic Ganciclovir levels <0.5 mg/L on recommended IV doses were found in eight patients. This subset of patients was significantly younger (4.5 +/- 3.1 vs. 11.4 +/- 5.0 yr). Levels <0.5 mg/L were found in 24/57 instances and 10 patients subsequently had their dose increased. The last Valganciclovir dose adjusted to a GFR of 100 mL/min/1.73 m(2) was 842 +/- 323 mg/m(2)/day. A high proportion of patients had low Ganciclovir levels both on intravenous and oral therapy. The oral bioavailability of Valganciclovir was 42%. Our data suggest substantial inter- and intrapatient variability of Ganciclovir levels after pediatric renal transplantation and may support the need for pharmacokinetic monitoring of Ganciclovir and Valganciclovir therapy for the prevention and treatment of CMV disease after pediatric transplantation. It is currently unclear what target trough level would be most suitable.
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Antivirales/farmacocinética , Ganciclovir/farmacocinética , Adolescente , Disponibilidad Biológica , Niño , Preescolar , Infecciones por Citomegalovirus/prevención & control , Ganciclovir/análogos & derivados , Humanos , Estudios Retrospectivos , Trasplantes , ValganciclovirRESUMEN
OBJECTIVES: To assess the efficacy of the anti-estrogens tamoxifen and raloxifen in the medical management of persistent pubertal gynecomastia. STUDY DESIGN: Retrospective chart review of 38 consecutive patients with persistent pubertal gynecomastia who presented to a pediatric endocrinology clinic. Patients received reassurance alone or a 3- to 9-month course of an estrogen receptor modifier (tamoxifen or raloxifene). RESULTS: Mean (SD) age of treated subjects was 14.6 (1.5) years with gynecomastia duration of 28.3 (16.4) months. Mean reduction in breast nodule diameter was 2.1 cm (95% CI 1.7, 2.7, P <.0001) after treatment with tamoxifen and 2.5 cm (95% CI 1.7, 3.3, P <.0001) with raloxifene. Some improvement was seen in 86% of patients receiving tamoxifen and in 91% receiving raloxifene, but a greater proportion had a significant decrease (>50%) with raloxifene (86%) than tamoxifen (41%). No side effects were seen in any patients. CONCLUSION: Inhibition of estrogen receptor action in the breast appears to be safe and effective in reducing persistent pubertal gynecomastia, with a better response to raloxifene than to tamoxifen. Further study is required to determine that this is truly a treatment effect.