Breastfeeding follow-up in the treatment of children with phenylketonuria.

Phenylketonuria (PKU) is the inability to convert phenylalanine into tyrosine, causing toxic effects to the central nervous system. Traditionally, in the treatment of PKU, breastfeeding is replaced by formula milk. This study verified the effects of breastfeeding as a source of phenylalanine on the development of children with PKU. Participants were ten infants with PKU who started treatment with the introduction of formula before 30 days of life, and maintained breastfeeding for at least 30 days after the start of procedures. The procedures were based on estimating breast milk intake, with a safe margin of phenylalanine concentration, calculating stomach volume, and initially offering formula, then breastfeeding on free demand, at every feeding. Breastfeeding duration ranged from one month and five days to 14 months. Blood controls were tested weekly. If the serum level of phenylalanine was >2 mg/dL and <6 mg/dL, the prescription was kept; if it was >2 mg/dL, the formula was decreased by 25%, indirectly increasing breastfeeding; if it was <6 mg/dL the formula was increased by 50%. The phenylalanine levels were assessed, and the Early Milestone Scale and the Basic Steps of Development were applied. Those who had normative index in all evaluations were considered adequate. Eighty percent of infants were able to keep safe concentrations of phenylalanine and development within normal indices. Continued breastfeeding is viable in the treatment of children with PKU, provided that phenylalanine levels are strictly controlled and the effects of breastfeeding on child development are monitored.

Acompanhamento do aleitamento materno no tratamento de crianças com fenilcetonúria / Breastfeeding follow-up in the treatment of children with phenylketonuria

A fenilcetonúria (PKU) ocorre na incapacidade para transformar fenilalanina em tirosina, trazendo efeitos tóxicos para o sistema nervoso central. Tradicionalmente, no tratamento da PKU, o aleitamento materno é substituído por fórmula láctea. Este estudo verificou os efeitos do aleitamento materno como fonte de fenilalanina no desenvolvimento de crianças com PKU. Participaram dez lactentes com PKU, que iniciaram o tratamento com a introdução de fórmula láctea antes dos 30 dias e que mantiveram o aleitamento materno por no mínimo 30 dias de vida após o início dos procedimentos. Os procedimentos basearam-se em estimar a ingestão de leite materno, com margem segura da concentração da fenilalanina, calculando o volume gástrico e oferecendo inicialmente fórmula láctea, seguida do aleitamento materno em demanda livre, em todas as mamadas. O tempo de amamentação variou de um mês e cinco dias a 14 meses. Os controles sanguíneos foram semanais. Se o nível sérico da fenilalanina estivesse >2 mg/dL e <6 mg/dL mantinha-se a prescrição; se estivesse <2 mg/dL, diminuía-se a fórmula láctea em 25%, aumentando indiretamente o aleitamento materno; se estivesse >6 mg/dL, aumentava-se a fórmula em 50%. Avaliou-se os níveis de fenilalanina, aplicou-se a Early Language Milestone Scale e Passos Básicos do Desenvolvimento. Foram considerados adequados aqueles lactentes que apresentaram índices normativos em todas as avaliações. Dos lactentes, 80% conseguiram manter limites seguros da fenilalanina e desenvolvimento nos índices normativos. Há viabilidade da continuidade do aleitamento materno no tratamento de crianças com PKU desde que os níveis de fenilalanina sejam rigorosamente controlados e que os efeitos do aleitamento materno para o desenvolvimento infantil sejam verificados.

Phenylketonuria (PKU) is the inability to convert phenylalanine into tyrosine, causing toxic effects to the central nervous system. Traditionally, in the treatment of PKU, breastfeeding is replaced by formula milk. This study verified the effects of breastfeeding as a source of phenylalanine on the development of children with PKU. Participants were ten infants with PKU who started treatment with the introduction of formula before 30 days of life, and maintained breastfeeding for at least 30 days after the start of procedures. The procedures were based on estimating breast milk intake, with a safe margin of phenylalanine concentration, calculating stomach volume, and initially offering formula, then breastfeeding on free demand, at every feeding. Breastfeeding duration ranged from one month and five days to 14 months. Blood controls were tested weekly. If the serum level of phenylalanine was >2 mg/dL and <6 mg/dL, the prescription was kept; if it was >2 mg/dL, the formula was decreased by 25%, indirectly increasing breastfeeding; if it was <6 mg/dL the formula was increased by 50%. The phenylalanine levels were assessed, and the Early Milestone Scale and the Basic Steps of Development were applied. Those who had normative index in all evaluations were considered adequate. Eighty percent of infants were able to keep safe concentrations of phenylalanine and development within normal indices. Continued breastfeeding is viable in the treatment of children with PKU, provided that phenylalanine levels are strictly controlled and the effects of breastfeeding on child development are monitored.

Hanseniase tuberculoide em paciente com aids / Tuberculoid leprosy in aids patients

Os autores apresentam o caso de uma crianca de 8 anos, portadora de aids por transmissao vertical, que desenvolveu as primeiras manifestacoes da hanseniase tuberculoide, antes do inicio do tratamento anti-retroviral. As lesoes iniciais percebidas eram ulceras de bordas endurecidas, que cicatrizavam e ulceravam novamente. Nesta epoca apresentava 0,3 celulas CD4+. As lesoes permaneceram com esta evolucao ate o momento em que foram realizados os primeiros exames para confirmacao do diagnostico de hanseniase. Nesta ocasiao estava fazendo tratamento anti-retroviral, apresentava 270 celulas CD4+. Foi realizada uma biopsia da lesao cutanea. Quando retornou, as ulceras estavam cicatrizadas, podendo se notar mais nitidamente o aspecto sarcoidico das lesoes. A reacao de Mitsuda foi de 8,5mm, havia hipoestesia em perna esquerda, e o diagnostico de hanseniase foi definido, principalmente atraves da imunoistoquimica anti-proteina S100, que demonstrou fragmentos de ramos nervosos no interior dos granulomas. A incidencia da hanseniase nao aumentou com o advento da aids e tambem nao ha modificacoes na apresentacao clinica, nem resposta a terapeutica nos casos de hanseniase associado a aids. Tambem nao observamos neste paciente o desenvolvimento da reacao tipo 1, como resultado da reconstituicao imunologica em decorrencia do tratamento anti-retroviral