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1.
J Pediatr ; 202: 315-319.e2, 2018 11.
Artículo en Inglés | MEDLINE | ID: mdl-30057141

RESUMEN

We describe 2 children with cobalamin G disease, a disorder of vitamin B12 metabolism with normal serum B12 levels. They presented with megaloblastic anemia progressing rapidly to severe thrombotic microangiopathy. In infants presenting with acute thrombotic microangiopathy, cobalamin disorders should be considered early as diagnosis and targeted treatment can be lifesaving.


Asunto(s)
Anemia Megaloblástica/diagnóstico , Anemia Megaloblástica/tratamiento farmacológico , Progresión de la Enfermedad , Hidroxocobalamina/uso terapéutico , Microangiopatías Trombóticas/tratamiento farmacológico , Microangiopatías Trombóticas/etiología , Anemia Megaloblástica/sangre , Anemia Megaloblástica/complicaciones , Análisis Químico de la Sangre , Transfusión Sanguínea/métodos , Preescolar , Diagnóstico Precoz , Insuficiencia de Crecimiento , Pruebas Hematológicas , Humanos , Lactante , Inyecciones Intramusculares , Masculino , Pronóstico , Medición de Riesgo , Índice de Severidad de la Enfermedad , Resultado del Tratamiento , Deficiencia de Vitamina B 12/sangre , Deficiencia de Vitamina B 12/diagnóstico
3.
Pediatr Nephrol ; 31(11): 2013-5, 2016 11.
Artículo en Inglés | MEDLINE | ID: mdl-27270721

RESUMEN

Peritoneal dialysis (PD) is generally considered the preferred extracorporeal therapy for neonates with acute kidney injury (AKI). However, there are situations when PD is not suitable, such as in patients with previous abdominal surgery, hyperammonemia and significant ascites or anasarca. Additionally, with a need to start PD soon after catheter placement, there is increased risk of PD catheter leak and infection. Extracorporeal continuous renal replacement therapy (CRRT) is challenging in severely ill neonates as it requires obtaining adequately sized central venous access to accommodate adequate blood flow rates and also adaptation of a CRRT machine meant for older children and adults. In addition, ultrafiltration often cannot be set in sufficiently small increments to be suitable for neonates. Although CRRT practices can be modified to fit the needs of infants and neonates, there is a need for a device designed specifically for this population. Until that becomes available, providing the highest level of care for neonates with AKI is dependent on the shared experiences of members of the pediatric nephrology community.


Asunto(s)
Lesión Renal Aguda , Terapia de Reemplazo Renal , Niño , Humanos , Lactante , Recién Nacido , Riñón , Diálisis Peritoneal , Diálisis Renal
6.
Front Pediatr ; 12: 1417724, 2024.
Artículo en Inglés | MEDLINE | ID: mdl-39359743

RESUMEN

Common variable immunodeficiency (CVID) can be complicated by granulomatous disease, often granulomatous lymphocytic interstitial lung disease (GLILD). Granulomatous interstitial nephritis represents an atypical presentation in pediatrics. Our patient is a previously healthy 13-year-old white male with a recent diagnosis of CVID. He presented with a rash and laboratory findings included pancytopenia (white blood cells 2.6 cells × 103/µl, hemoglobin 11.8 g/dl, platelets 60 × 103/µl), hypercalcemia (14.9 mg/dl), elevated Vit D 1,25 OH level (>200 pg/ml), hyperuricemia (8.8 mg/dl), and acute kidney injury (AKI) (serum creatinine 1.1 mg/dl; baseline 0.64 mg/dl). A broad infectious workup was unremarkable. The rash improved with empiric doxycycline. Hypercalcemia and hyperuricemia were managed with fluid resuscitation, calcitonin, and zoledronic acid. Evaluation for malignancy including a positron emission tomography scan, revealed multiple mediastinal hypermetabolic lymph nodes and pulmonary ground glass opacities, later reported as small pulmonary nodules by computed tomography (CT). Splenomegaly was confirmed by ultrasound and CT. Peripheral smear, bone marrow biopsy, and genetic testing were non-revealing. His angiotensin-converting enzyme level was elevated (359 U/L), raising concerns for sarcoidosis. Given Stage 1 AKI, a renal biopsy was pursued and identified non-caseating granulomatous interstitial nephritis. Treatment with 60 mg of prednisone began for presumed sarcoidosis for 4 months, causing steroid-induced hypertension and mood changes. Zoledronic acid minimally reduced serum creatinine. Pneumocystis jirovecii pneumonia prophylaxis was initiated due to T-cell cytopenia. Chest CT findings showed a suboptimal response to steroids. A bronchoalveolar lavage demonstrated >50% lymphocytes (normal <10%) and the lung biopsy exhibited non-caseating granulomas, indicating GLILD. Rubella was identified by staining. Following a fever, he was found to have elevated liver enzymes and confirmed hepatitis with portal hypertension on CT. A liver biopsy revealed epithelioid non-caseating granuloma and HHV6 was detected by PCR. He was treated with four cycles of rituximab and granulocyte-colony stimulating factor for persistent neutropenia. Subsequent treatment with mycophenolate led to the resolution of the granulomatous lesions and cytopenias. The rare complication of granulomatous interstitial nephritis in CVID illustrates the intricate nature of diagnosis. This case underscores the necessity for a holistic view of the patient's clinical and immune phenotype, including distinctive radiological presentations, for precise diagnoses and tailored management of CVID.

10.
PLoS One ; 12(8): e0182134, 2017.
Artículo en Inglés | MEDLINE | ID: mdl-28792509

RESUMEN

Pediatric liver failure patients frequently develop multiple organ failure and require continuous renal replacement therapy (CRRT) as part of supportive therapy in the pediatric intensive care unit. While many centers employ no anticoagulation for fear of bleeding complications, balanced coagulation disturbance predisposes these patients to clotting as well as bleeding, making maintenance of longer circuit life to deliver adequate dialysis clearance challenging. Regional citrate anticoagulation (RCA) is an attractive option as it avoids systemic anticoagulation, but since citrate metabolism is impaired in liver failure, concerns about toxicity has limited its use. Pediatric data on RCA with liver failure is very scarce. We aimed to establish safety and efficacy of RCA in pediatric liver failure patients on CRRT. Retrospective review of pediatric patients with liver failure receiving CRRT over 30 months. Demographic data and CRRT related data were collected by chart review. Citrate accumulation (CA) was defined as total calcium (mg/dl) /ionized calcium (mmol/L) ratio >2.5 for > 48 hours. Efficacy was assessed by filter life. Safety was assessed by frequency of adverse events ((AEs) defined as bleeding, hemodynamic instability, arrhythmias). Fifty-one patients (median age 3.5 (IQR 0.75-14.2) years) received 861 CRRT days; 70% experienced at least one episode of CA, only 37% were recorded as such in the medical record. AE rate was 93/1000 CRRT days and did not differ between CA days and others. Median filter life was 66 hours (IQR 29-74); 63% filters lasted longer than 48 hrs. Though common, CA was not associated with increased AEs on in pediatric liver failure patients on CRRT receiving RCA. Filter life was adequate. RCA appears an effective anticoagulation for CRRT in pediatric liver failure. Application of a structured definition would increase recognition of CA to allow timely intervention.


Asunto(s)
Anticoagulantes/uso terapéutico , Ácido Cítrico/uso terapéutico , Fallo Hepático Agudo/tratamiento farmacológico , Terapia de Reemplazo Renal , Adolescente , Anticoagulantes/efectos adversos , Calcio/metabolismo , Niño , Preescolar , Ácido Cítrico/efectos adversos , Femenino , Mortalidad Hospitalaria , Humanos , Lactante , Fallo Hepático Agudo/metabolismo , Fallo Hepático Agudo/mortalidad , Fallo Hepático Agudo/cirugía , Trasplante de Hígado , Masculino , Análisis Multivariante , Terapia de Reemplazo Renal/efectos adversos , Terapia de Reemplazo Renal/instrumentación , Estudios Retrospectivos , Factores de Tiempo
11.
Pediatr Nephrol ; 24(3): 613-7, 2009 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-18839218

RESUMEN

We present the details of three children with hypercalcemia-induced acute kidney injury (AKI). After traditional therapy with fluids, loop diuretics, steroids and calcitonin had failed to correct the hypercalcemia, they were given treatment with low doses of intravenous (i.v.) pamidronate, which resulted in normalization of serum calcium and kidney function. In one child Doppler renal ultrasound revealed dampened arterial blood flow, which resolved with normalization of serum calcium. On the basis of cumulative data and our experience, we suggest that i.v. application of bisphosphonates be moved from the second to the first line of treatment of hypercalcemic AKI.


Asunto(s)
Conservadores de la Densidad Ósea/uso terapéutico , Difosfonatos/uso terapéutico , Hipercalcemia/tratamiento farmacológico , Enfermedades Renales/etiología , Enfermedad Aguda , Adolescente , Niño , Preescolar , Difosfonatos/administración & dosificación , Femenino , Humanos , Hipercalcemia/complicaciones , Inyecciones Intravenosas , Pamidronato
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