Hiperfosfatasemia transitoria benigna de la infancia, reporte de un caso / Benign transient hyperphosphatasemia of childhood: a case report

La Hiperfosfatasemia Transitoria Benigna (HTB) es la causa más frecuente de elevación aislada de la Fosfatasa Alcalina (FA) en la población pediátrica. Es relevante tener la sospecha de esta entidad dada su frecuencia, carácter auto limitado y fácil diagnóstico, a pesar de esto, es poco conocida y estudiada en la Pediatría. Su clínica se asocia a niños sanos como a infecciones virales respiratorias, gastrointestinales y al retraso ponderal. El presente trabajo tiene como objetivo reportar un caso clínico y revisar el diagnóstico de la HBT.

Benign Transient Hyperphosphatasemia (BTH) is the most frequent cause of isolated elevation of Alkaline Phosphatase (AF) in the pediatric population. It is relevant to have the suspicion of this entity given its frequency, self limited character and easy diagnosis, despite this, it is little known and studied in Pediatrics. Its symptoms are associated with healthy children, such as viral respiratory, gastrointestinal infections and delayed weight gain. The objective of this work is to report a clinical case and review the diagnosis of HBT.

Tetania severa por uso de enemas fosfatados, reporte de caso / Tetany secondary to phosphate enema toxicity, case report

Los enemas fosfatados son utilizados frecuentemente en el tratamiento de la constipación. Errores en la posología pueden producir complicaciones graves. Objetivo: Reportar un caso de toxicidad grave por enema fosfatado en un pre escolar sin factores de riesgo. Caso clínico: Paciente de 2 años con constipación funcional, evaluada en servicio de urgencia por dolor abdominal a quién se le diagnosticó un fecaloma impactado. Recibió 2 dosis de enema de fosfato, “medio frasco” de Fleet® adulto (Synthon, Chile) por vez, sin resolución de su fecaloma, decidiéndose hospitalización para proctoclisis. Posterior al ingreso presentó un cuadro clínico de tetania. Ingresó a la Unidad de Paciente Crítico donde se confirmó una hiperfosfemia e hipocalcemia secundaria. Se realizó corrección electrolítica progresiva, retiro de enema fosfatado residual del recto e hiperhidratación forzando diuresis. La tetania cedió 2 horas después del ingreso sin otras complicaciones. Se realizó proctoclisis y fue dada de alta a los 3 días. Conclusión: Los enemas fosfatados pueden presentar complicaciones graves en niños sin factores de riesgo. Errores en la posología son la causa más frecuente de toxicidad en este grupo, pero esta puede estar favorecida también por una administración y eliminación inadecuadas. Pediatras y personal de salud que atiende a niños deben conocer factores de riesgo, signos y síntomas de intoxicación por enemas fosfatados.

Phosphate enemas are frequently used in the treatment of constipation. Errors in dosage and administration can lead to severe complications. Objective: To report a case of severe toxicity of phosphate enemas in a child with no risk factors. Case: 2 years old female, with functional constipation, was brought to emergency department because abdominal pain. She was diagnosed with fecal impaction and received half a bottle of Fleet Adult® (Laboratorio Synthon, Chile) two times, with no clinical resolution, deciding to start proctoclisis in pediatric ward. Soon after admission, she presented painful tetany, but alert and oriented. Patient was transferred to PICU where severe hyperphosphatemia and secondary hypocalcemia were confirmed. Her treatment included electrolyte correction; removal of residual phosphate enema and hyperhydration. Tetany resolved over 2 hours after admission and no other complications. Proctoclisis was performed and patient was discharged three days after admission with pharmacological management of constipation. Conclusion: Phosphate enemas may cause serious complications in children with no risk factors. Errors in dosage, administration and removal of the enema are causes of toxicity in this group. Pediatricians and health personnel must be aware of risks and signs of toxicity of phosphate enema.

Dieta e quelantes como ferramentas para o manuseio do hiperparatireoidismo secundário / Diet and phosphate binders in the management of secondary hyperparathyroidism

O fósforo é um elemento fundamental no metabolismo celular e sua homeostase é mantida pelo sistema digestivo, remodelação óssea e rins. Uma dasprincipais alterações no metabolismo do fósforo, a hiperfosfatemia, pode se tornar uma situação de grave morbidade para pacientes com doença renalcrônica (DRC), sendo considerada atualmente uma responsável indireta pela alta taxa de mortalidade dessa população. Cerca de 60% dos pacientes em diálise apresentam níveis de fósforo elevados. O excesso de ingestão de fósforo, o uso inadequado de seus quelantes intestinais, a inadequação dialítica e o status da remodelação óssea compõem o caráter multifatorial da hiperfosfatemia, tornando seu tratamento um dilema ao nefrologista. Na fase não-dialítica, a restrição de fósforo é mais facilmente implementada já que normalmente os pacientes são orientados a ingerir reduzida quantidade de proteína, o que, conseqüentemente, acarreta uma diminuição no conteúdo de fósforo. Na fase dialítica, em função da elevada necessidade protéica, a restrição significativa de fósforo quase nunca pode ser empregada, o que na maioria das vezes, implica na utilização de quelantes de fósforo. Os quelantes devem ser ingeridos junto com a alimentação, de forma a permitir a melhor mistura com os alimentos. Dentre os tipos mais comumente utilizados estão os quelantes à base decálcio ou aqueles livres de cálcio ou metal, como o sevelamer. A dose de cálcio elementar proveniente de quelantes não deve exceder a 1500 mg/dia ou 2000 mg/dia, se considerado o cálcio da dieta. Pacientes com hipercalcemia não devem utilizar quelantes que contêm cálcio. Finalmente, é importanteressaltar que o sucesso do tratamento da hiperfosfatemia da DRC requer o envolvimento de toda a equipe multiprofissional, particularmente do nutricionista.

Phosphorus, an essential element for cell metabolism, has its homeostasis maintained in the body by the integrated actions of intestine, bone and kidneys.Hyperphosphatemia, mainly due to derangements in phosphorus metabolism, is a serious complication of chronic kidney disease (CKD) responsible for thehigh rates of mortality in this population. Elevated serum phosphorus is found in about 60% of the patients on maintenance dialysis. Several factors can contribute to hyperphosphatemia, including high phosphorus intake, inappropriate use of phosphate binders, poor dialysis efficiency and the bone turnover condition. For these reasons the treatment of hyperphosphatemia is still a challenge for nephrologists. In CKD stages 2 to 4 a low phosphorus intake is often achieved since dietary protein restriction, with consequent phosphorus reduction content is usually employed for these patients. In contrast, considering the elevated protein requirement of patients on dialysis it is not possible to reduce phosphorus intake in a significant manner without harmful consequences inthe nutritional status. Thus, the use of phosphate binders is always necessary for these patients. For better results, however, the binders must be takentogether with the meals to guarantee a satisfactory mixture with food. Calcium based phosphate binders or those binders free of calcium or metals such assevelamer are among the most used ones. Calcium intake provided by phosphate binders should not exceed 1500 mg/day or 2000 mg/day, considering the calcium provided by the diet. However, for patients with hypercalcemia, calcium based phosphate binders should be avoided. Finally, it is important to address that the success of the treatment relies on the involvement of all members of health care team in particular the nutritionist.