Abstract
OBJETIVO: Relatar uma série de casos de hiperfosfatasemia transitória benigna da infância (HTBI). DESCRIÇÃO: São descritas quatro meninas. A faixa etária variou de 11-45 meses (mediana: 13 meses). Ao diagnóstico, a fosfatase alcalina sérica estava aumentada de 1,1-6,1 vezes (mediana: 1,36 vezes) o valor de referência. O retorno à normalidade ocorreu entre 7-11 meses (mediana: 9 meses). Não havia evidência de doenças ósseas, hepáticas, endócrinas, ou uso de medicamentos associados à elevação da fosfatase alcalina. Uma paciente apresentou infecções de vias aéreas superiores precedendo o diagnóstico da hiperfosfatasemia. Alanina aminotransferase, aspartato aminotransferase, cálcio, fósforo e magnésio estavam normais em todos. O paratormônio foi dosado em três crianças, estando normal em todas. Em dois pacientes, a investigação para hepatites A, B e C foi negativa. A fosfatase alcalina estava normal em três dos quatro pares de pais testados. COMENTÁRIOS: HTBI é uma patologia autolimitada, benigna e de resolução espontânea, que acomete crianças abaixo de cinco anos, sem evidência clínica ou laboratorial de doença óssea, hepática ou endócrina subjacente. A etiologia é desconhecida. Esta possibilidade deve ser considerada no diagnóstico diferencial da hiperfosfatesemia para evitar exames e procedimentos desnecessários.
Highlights
It is speculated that the excessive synthesis of alkaline phosphatase in Benign transient hyperphosphatasemia of childhood (BTHC) occurs as part of the inflammatory response at the acute phase or of the reduced hepatic clearance due to an increased sialination.[6,7]
The traditional BTHC diagnosis uses the propositions by Kraut et al.[11], which require the presence of the following criteria: age < 5 years, absence of evident hepatic, bone or kidney disease; alkaline phosphatase rise in apparently healthy children about 2-50 times higher than the normal level and return to normal values within 4-6 months, and increased liver and bone fraction of alkaline phosphatase.[2,3,12]
Except for the identification of alkaline phosphatase isoenzymes, which was not performed in this study, all the criteria for diagnosing BTHC have been met
Summary
Benign transient hyperphosphatasemia of childhood (BTHC) affects children younger than five years, with no evidence of bone, liver or endocrine disease, being characterized by a sudden and transient rise of serum alkaline phosphatase in an otherwise healthy child.[1,2,3] Alkaline phosphatase rise may reach serum levels 2-50 times higher than reference values for the age group.[1,3] The return to normality occurs in an average period of four months.[1,3] Its etiology is still unknown, but the reduced enzyme clearance on blood stream has been regarded as the most likely mechanism.[2,3] There are reports in literature correlating alkaline phosphatase transient rise to upper respiratory tract infections or fever accompanied by diarrhea.[4]. In view of the benign nature of this condition and its various differential diagnoses, which can be very expensive and invasive, this article was designed to describe a series of BTHC cases
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