Desensibilización con Ketocal® en lactante con dieta cetogénica y alergia a proteínas de leche de vaca / Oral desensitization with Ketocal® in an infant with ketogenic diet and cow's milk protein allergy

Introducción: la dieta cetogénica es un tratamiento con eficacia demostrada en la epilepsia infantil refractaria a fármacos. La alergia a la proteína de leche de vaca puede ser una limitación para tratar a lactantes con dieta cetogénica, ya que necesitan un producto que contiene proteínas de leche de vaca (Ketocal®). Caso clínico: presentamos el caso de un lactante con una encefalopatía epiléptica refractaria a fármacos y alergia a las proteínas de leche de vaca IgE mediada al que se quiere instaurar una dieta cetogénica clásica. Se consigue la desensibilización con Ketocal 3:1®, pudiendo utilizarlo en la dieta y logrando una mejoría clínica con el control de las crisis. Discusión: un paciente con epilepsia y alergia a las proteínas de leche de vaca puede beneficiarse de la dieta cetogénica, ya que es posible realizar una inmunoterapia oral con Ketocal® y conseguir, además, una probable resolución de su alergia. (AU)

Introduction: ketogenic diet is a treatment with proven efficacy in drug-refractory childhood epilepsy. Cow's milk protein allergy may be a limitation for treating infants with ketogenic diet, as they need a product that contains cow's milk protein (Ketocal®). Case report: we report the case of an infant with a drug-refractory epileptic encephalopathy and IgE-mediated cow's milk protein allergy, who started a classic ketogenic diet. Oral desensitization was achieved with Ketocal 3:1®, allowing its use in the diet and achieving a clinical improvement with seizure control. Discussion: a patient with epilepsy and cow's milk protein allergy can benefit from the ketogenic diet, since it is possible to perform an oral immunotherapy with Ketocal®, also achieving a probable resolution of his/her allergy. (AU)

[Oral desensitization with Ketocal® in an infant with ketogenic diet and cow's milk protein allergy]. / Desensibilización con Ketocal® en lactante con dieta cetogénica y alergia a proteínas de leche de vaca.

Introduction: Introduction: ketogenic diet is a treatment with proven efficacy in drug-refractory childhood epilepsy. Cow's milk protein allergy may be a limitation for treating infants with ketogenic diet, as they need a product that contains cow's milk protein (Ketocal®). Case report: we report the case of an infant with a drug-refractory epileptic encephalopathy and IgE-mediated cow's milk protein allergy, who started a classic ketogenic diet. Oral desensitization was achieved with Ketocal 3:1®, allowing its use in the diet and achieving a clinical improvement with seizure control. Discussion: a patient with epilepsy and cow's milk protein allergy can benefit from the ketogenic diet, since it is possible to perform an oral immunotherapy with Ketocal®, also achieving a probable resolution of his/her allergy.

Introducción: Introducción: la dieta cetogénica es un tratamiento con eficacia demostrada en la epilepsia infantil refractaria a fármacos. La alergia a la proteína de leche de vaca puede ser una limitación para tratar a lactantes con dieta cetogénica, ya que necesitan un producto que contiene proteínas de leche de vaca (Ketocal®). Caso clínico: presentamos el caso de un lactante con una encefalopatía epiléptica refractaria a fármacos y alergia a las proteínas de leche de vaca IgE mediada al que se quiere instaurar una dieta cetogénica clásica. Se consigue la desensibilización con Ketocal 3:1®, pudiendo utilizarlo en la dieta y logrando una mejoría clínica con el control de las crisis. Discusión: un paciente con epilepsia y alergia a las proteínas de leche de vaca puede beneficiarse de la dieta cetogénica, ya que es posible realizar una inmunoterapia oral con Ketocal® y conseguir, además, una probable resolución de su alergia.

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

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.

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.