Rabdomiólisis grave secundaria a deshidratación hipernatrémica / Severe rhabdomyolysis secondary to severe hypernatraemic dehydration

Introducción: La rabdomiólisis es una enfermedad poco frecuente en pediatría. El objetivo es presentar un paciente en el que se desarrolló secundario a una deshidratación hipernatrémica grave tras una diarrea aguda. Caso clínico: Lactante de 11 meses que consultó por fiebre, vómitos, diarrea y anuria. Presentó convulsión tónico-clónica autolimitada. Ingresó en mal estado general, severamente deshidratado, con escasa reactividad. En las pruebas complementarias destacó acidosis metabólica grave, hipernatremia e insuficiencia renal prerrenal. Al tercer día apreció leve hipotonía axial y elevación de creatín fosfokinasa 75.076 UI/l, interpretado como rabdomiólisis. Se inició hiperhidratación y alcalinización sistémica, con buena respuesta clínica y bioquímica, siendo dado de alta sin secuelas motoras. Conclusiones: La hipernatremia grave está descrita como causa rara de rabdomiólisis e insuficiencia renal. En pacientes críticos es importante un alto índice de sospecha de rabdomiólisis y determinación seriada de la creatín fosfokinasa para su detección y tratamiento precoz.

Introduction: Rhabdomyolysis is a rare paediatric condition. The case is presented of a patient in whom this developed secondary to severe hypernatraemic dehydration following acute diarrhoea. Case report: Infant 11 months of age who presented with vomiting, fever, diarrhoea and anuria for 15 hours. Parents reported adequate preparation of artificial formula and oral rehydration solution. He was admitted with malaise, severe dehydration signs and symptoms, cyanosis, and low reactivity. The laboratory tests highlighted severe metabolic acidosis, hypernatraemia and pre-renal kidney failure (Sodium [Na] plasma 181 mEq/L, urine density> 1030). He was managed in Intensive Care Unit with gradual clinical and renal function improvement. On the third day, slight axial hypotonia and elevated cell lysis enzymes (creatine phosphokinase 75,076 IU/L) were observed, interpreted as rhabdomyolysis. He was treated with intravenous rehydration up to 1.5 times the basal requirements, and he showed a good clinical and biochemical response, being discharged 12 days after admission without motor sequelae. Conclusions: Severe hypernatraemia is described as a rare cause of rhabdomyolysis and renal failure. In critically ill patients, it is important to have a high index of suspicion for rhabdomyolysis and performing serial determinations of creatine phosphokinase for early detection and treatment.

Treatment of BK virus-associated hemorrhagic cystitis with low-dose intravenous cidofovir in patients undergoing allogeneic hematopoietic cell transplantation

BACKGROUND/AIMS: BK virus (BKV) has been associated with late-onset hemorrhagic cystitis (HC) in recipients of hematopoietic stem cell transplantation (HSCT). Cidofovir has been used at higher doses (3 to 5 mg/kg/wk) with probenecid prophylaxis; however, cidofovir may result in nephrotoxicity or cytopenia at high doses. METHODS: Allogeneic HSCT recipients with BKV-associated HC are treated with 1 mg/kg intravenous cidofovir weekly at our institution. A microbiological response was defined as at least a one log reduction in urinary BKV viral load, and a clinical response was defined as improvement in symptoms and stability or reduction in cystitis grade. RESULTS: Eight patients received a median of 4 weekly (range, 2 to 11) doses of cidofovir. HC occurred a median 69 days (range, 16 to 311) after allogeneic HSCT. A clinical response was detected in 7/8 patients (86%), and 4/5 (80%) had a measurable microbiological response. One patient died of uncontrolled graft-versus-host disease; therefore, we could not measure the clinical response to HC treatment. One microbiological non-responder had a stable BKV viral load with clinical improvement. Only three patients showed transient grade 2 serum creatinine toxicities, which resolved after completion of concomitant calcineurin inhibitor treatment. CONCLUSIONS: Weekly intravenous low-dose cidofovir without probenecid appears to be a safe and effective treatment option for patients with BKV-associated HC.

Aplicação local de cidofovir como tratamento adjuvante na papilomatose laríngea recorrente em crianças / Local application of cidofovir as adjuvant therapy in recurrent laryngeal papillomatosis in children

OBJETIVO: Avaliar a eficácia da aplicação local de cidofovir em associação com o tratamento cirúrgico na papilomatose laríngea recorrente (PLR) em crianças. Desenho do estudo: Prospectivo. MÉTODOS: Quatorze pacientes, com idade média de 4.7 anos e com duas ou mais recidivas após tratamento cirúrgico, foram submetidos à ressecção dos papilomas e injeção de 22.5 mg de cidofovir (7,5 mg/ml) no tecido de onde as lesões foram removidas. Após intervalos de 2-3 semanas, a mesma dose de cidofovir foi repetida duas ou três vezes. Em caso de recidiva, um novo ciclo de cirurgia seguido de aplicações locais de cidofovir era reiniciado. Cinco crianças apresentavam HPV-6 e cinco HPV-11; em quatro casos a tipagem não foi realizada. RESULTADOS: Antes do início do estudo, os pacientes eram submetidos, em média, a duas cirurgias por ano para o controle das recidivas; após o tratamento com cidofovir, a taxa anual de cirurgia diminuiu para 1,1 (p = 0,013). O intervalo médio entre as recidivas antes do início do estudo era de 1.6 meses; ao final do estudo, o intervalo aumentou para 4,4 meses (p = 0,014). Os pacientes com HPV-6 não apresentaram alteração significante nos intervalos entre as recidivas após o tratamento com cidofovir, enquanto 60 por cento das crianças com HPV-11 encontravam-se livres de doença ao final do estudo. CONCLUSÃO: O cidofovir é um adjuvante eficaz no tratamento da PLR em crianças, quando utilizado sob a forma de aplicações locais em associação com a ressecção cirúrgica das lesões. O HPV-11 pode ser mais susceptível aos efeitos benéficos do cidofovir.

OBJECTIVE: Evaluate the efficacy of local application of cidofovir in association with surgical treatment of recurrent laryngeal papillomatosis in children. Study design: Prospective. METHODS: Fourteen patients, with an average age of 4.7 years and with two or more relapses after surgical treatment, were submitted to resection of the papillomas and injection of 22.5 mg of cidofovir (7.5 mg/ml) in the tissue where the lesions had been removed. After 2 to 3 week intervals, the same dose of cidofovir was repeated two or three times. In the case of relapse, a new cycle of surgery followed by local applications of cidofovir was repeated. Five children presented HPV-6 and five HPV-11, while in four, the type was not determined. RESULTS: Before beginning of the study, patients were submitted, on the average, to 2 operations a year for control of relapses. After treatment with cidofovir, the annual rate for surgery dropped to 1.1 (p = 0.013). The average interval between relapses before beginning of the study was 1.4 months; at the end of the study, the interval reached 4.4 months (p = 0.014). Patients with HPV-6 did not show a significant change in the intervals between relapses after treatment with cidofovir, while 60 percent of the children with HPV-11 were disease free at the study end. CONCLUSION: Cidofovir was found to be an effective adjuvant in the treatment of recurrent laryngeal papillomatosis in children, when used in the form of local applications in association with surgical resection of the lesions. HPV-11 may be more susceptible to the beneficial effects of cidofovir.

Endoftalmitis candidiásica: presentación de dos casos / Candida endophthalmitis: report of two cases

Se denomina endoftalmitis a un proceso inflamatorio que afecta a la cavidad ocular y a las estructuras adyacentes. En su etiología intervienen agentes infecciosos (hongos, virus, bacterias, protozoos y parásitos), neoplasias, sangre intraocular y material retenido de lentes, luego de la cirugía de cataratas. Se presentan dos casos de endoftalmitis candidiásica que afectaron a dos pacientes jóvenes inmunocompetentes. En los dos casos se realizó tratamiento con anfotericina B y 5 fluorcitosina. A uno de ellos se le realizó una vitrectomía bilateral, quedando con una secuela visual permanente. Sólo el diagnóstico precoz y el tratamiento oportuno y efectivo reducen las posibilidades de lesiones retinianas permanentes en estos pacientes

Leucemía mieloide aguda en niños del Hospital Universitario de Caracas (Período 1979-1989) / Acute myeloid leukemia in children at the University Hospital of Caracas (period 1979-1989)

En el Hospital Universitario de Caracas 52 nuevos casos de Laucemia Mieloblástica aguda (LMA) fueron tratados en dos series consecutivas entre 1979-1989. Concomitantemente se intentó correlacionar los hallazgos clínicos y de laboratorio al ingreso, y la respuesta al tratamiento, con los subtipos morfológicos (FAB) de LMA. Serie I (1979-1985): comprendió 37 niños que recibieron principalmente la terapia de inducción con Citosina Arabinosa 100 mg/m2/día, en una infusión continua por 7 días, además de Daunomicina 45 mg/m2/día, en los 3 primeros días del tratamiento. La serie II incluyó solo 15 pacientes tratados en igual forma, excepto por la Citosina Arabinosa que se administró durante 10 días. Los pacientes que alcanzaron remisión en ambas series recibieron luego 5 ciclos con el esquema de COAP como terapia de consolidación. Posteriormente, fueron a un tratamiento de mantenimiento semanal con 6-Thioguanima por 4 días más Citosina Arabinosa el 5to. día durante 24 meses en remisión contínua. el tratamineto de inducción se realizó en 44 niños, dado que 7 murieron durante la fase de inducción en aplasia y otro caso se descartó por Síndrome de Down. La remisión completa fue de 64 por ciento (21/33) en la Serie I y de 36 por ciento (4/11) en la Serie II; observandose en ambas series un 42 por ciento de LMA con un componente monocítico, la mayoría del tipo M4 y 80 por ciento (20/25) de los casos que alcanzaron remisión, presentaban diferenciación mieloide FAB M1-M4. La duración de la remisión fué corta, lo que sugiere fallas en el tratamiento citotócico recayendo el 60 por ciento en los primeros 6 meses. La sobrevida libre de enfermedad fue menor de 20 por ciento al año y de sólo 6 por ciento a los 30 meses, sin diferencias estadísticas significativas en las 2 Series. Se estima que la problemática asistencial crónica de nuestro hospital pudo haber influido en la alta tasa de mortalidad temprana. Esta es la primera casuística de niños con LMA reportada en Venezuela