Cetoacidose diabética e pancreatite secundárias à peg-asparaginase / Diabetic ketoacidosis and pancreatitis secondary to peg-asparaginase

RESUMO: Objetivos: Apresentar um caso raro de cetoacidose diabética (CAD) e pancreatite secundários ao uso de PEG-asparaginase em paciente pediátrico em tratamento para leucemia linfoblástica aguda (LLA) e alertar quanto aos sinais que remetem a esses diagnósticos. Descrição do caso: Adolescente do sexo feminino, 10 anos e 11 meses, em tratamento para LLA e uso prévio de PEG-asparaginase há seis dias da internação, admitida com choque hipotensivo grave e encaminhada à Unidade de Terapia Intensiva. Inicialmente o quadro foi interpretado como choque séptico. Em seguida a anamnese detalhada e os exames laboratoriais direcionaram para os diagnósticos de CAD e pancreatite, iniciando-se as intervenções específicas. Recebe alta hospitalar após 30 dias, sem necessidade de insulinoterapia, mas com reposição de enzimas pancreáticas. Comentários: Geralmente, às crianças com LLA gravemente enfermos e leucopênicos, atribui-se apenas o diagnóstico de sepse, que é um diagnóstico prioritário. Entretanto, no grupo em uso de PEG-asparaginase, o pediatra emergencista deve estar alerta ao raciocínio diferencial envolvendo CAD e pancreatite, o que pode ser bem difícil inicialmente. O alerta dos diagnósticos diferenciais do choque séptico, mesmo que raros, na assistência a pacientes oncológicos pediátricos, além da correta e pronta identificação do quadro e seu manejo apropriado, correlacionam-se diretamente ao sucesso terapêutico e, em algumas situações, à sobrevivência do paciente. (AU)

ABSTRACT: Objectives: We present a rare case of diabetic ketoacidosis (DKA) and pancreatitis secondary to the use of PEG-asparaginase in a pediatric patient being treated for acute lymphoblastic leukemia (ALL) and draw attention to the signs that refer to these diagnoses. Case description: A female adolescent, aged 10 years and 11 months, undergoing treatment for ALL, used PEG-asparaginase for 6 days prior to admission. She was hospitalized due to severe hypotensive shock and was then referred to the intensive care unit. Initially, the clinical condition was interpreted as septic shock. However, detailed anamnesis and results of laboratory tests led to the diagnoses of DKA and pancreatitis; hence, appropriate interventions were initiated. She was discharged after 30 days without the need for insulin therapy but received pancreatic enzyme replacement therapy. Comments: Generally, diagnosing severely ill and leukopenic children with ALL is only attributed to sepsis, which is a priority diagnosis. However, in the group treated with PEG-asparaginase, the pediatric emergency specialist should consider differential reasoning in patients with DKA and pancreatitis, which can be quite difficult to assess initially. Alertness towards the differential diagnoses of septic shock, although rare, in the care of pediatric oncology patients, in addition to the correct and prompt identification of the condition and provision of appropriate management, directly correlates with treatment success and, in some situations, the improvement in patient's survival. (AU)

Neuromuscular synapse degeneration without muscle function loss in the diaphragm of a murine model for Huntington's Disease.

Huntington's disease (HD) is an autosomal dominant neurodegenerative disease characterized by chorea, incoordination and psychiatric and behavioral symptoms. The leading cause of death in HD patients is aspiration pneumonia, associated with respiratory dysfunction, decreased respiratory muscle strength and dysphagia. Although most of the motor symptoms are derived from alterations in the central nervous system, some might be associated with changes in the components of motor units (MU). To explore this hypothesis, we evaluated morphofunctional aspects of the diaphragm muscle in a mouse model for HD (BACHD). We showed that the axons of the phrenic nerves were not affected in 12-months-old BACHD mice, but the axon terminals that form the neuromuscular junctions (NMJs) were more fragmented in these animals in comparison with the wild-type mice. In BACHD mice, the synaptic vesicles of the diaphragm NMJs presented a decreased exocytosis rate. Quantal content and quantal size were smaller and there was less synaptic depression whereas the estimated size of the readily releasable vesicle pool was not changed. At the ultrastructure level, the diaphragm NMJs of these mice presented fewer synaptic vesicles with flattened and oval shapes, which might be associated with the reduced expression of the vesicular acetylcholine transporter protein. Furthermore, mitochondria of the diaphragm muscle presented signs of degeneration in BACHD mice. Interestingly, despite all these cellular alterations, BACHD diaphragmatic function was not compromised, suggesting a higher resistance threshold of this muscle. A putative resistance mechanism may be protecting this vital muscle. Our data contribute to expanding the current understanding of the effects of mutated huntingtin in the neuromuscular synapse and the diaphragm muscle function.

Intravesical and transperitoneal laparoscopy in the management of tumor in the residual ureter.

The occurrence of tumor in the residual ureter after an incomplete nephroureterectomy required by a tumor of renal collecting system is an uncommon but a well described situation. The recommended treatment in this situation is the radical excision of the remaining ureter, being the open technique the most used approach. The aim of this video is to demonstrate a new approach using intravesical and transperitoneal laparoscopy to remove the residual ureter following the oncological concepts. A 67 year-old male patient underwent an incomplete open right radical nephroureterectomy for a transitional cell carcinoma of the renal collecting system. After 16 months, the cystoscopy diagnostic revealed a recurrence of it in the residual ureter. An intravesical approach followed by a transperitoneal laparoscopy has removed the remaining ureter. Operative time was 110 minutes, blood loss 100 mL, the patient was discharged on the first postoperative day and the Foley catheter was removed on the seventh one. Pathological examination revealed low grade transitional cell carcinoma and free surgical margins, no recurrence was observed after six months. To our knowledge, this is the first treatment description of a tumor in the residual ureter with these techniques. This approach can be a minimal invasive alternative in this unusual situation.

Prevalência de hemoglobinopatias e talassemias em crianças de 6 meses a 7 anos de idade no laboratório escola do departamento de biomedicina (CBB) - UCG / Prevalence of hemoglobinopathies and thalassemias in children from 6 months to 7 years old at the school laboratory from the departament of biomedicine (cbb) - ucg

Foram estudadas 197 amostras de sangue de crianças com idade variando entre 6 meses a 7 anos, de ambos os sexos. O objetivo do trabalho foi avaliar a prevalência de hemoglobinopatias e talassemias em crianças na faixa etária de 6 meses a 7 anos de idade. As amostras foram obtidas por punção venosa e submetidas à análise laboratorial, empregando os seguintes procedimentos: eritrograma, estudo citológico, teste de resistência globular, teste de falcização, eletroforese de hemoglobina em pH alcalino, ácido e HPLC. Os resultados obtidos mostraram que 36,5 porcento das crianças estudadas portavam alterações da hemoglobina. Estes resultados enfatizam a necessidade de programas com maior abrangência na população para estudo da epidemiologia das hemoglobinopatias no estado de Goiás.

Carcinoma primário de mama acessória axilar: relato de caso / Primary carcinoma of axillary accessory breast: case report

A polimastia é uma ocorrência comum nos ambulatórios de Mastologia e a possibilidade de malignização deve ser considerada, assim como em qualquer tecido mamário. Neste trabalho é relatado o caso de uma paciente de 48 anos que foi submetida à exérese da mama axilar acessória esquerda por motivos estéticos, tendo sido encontrado, no exame histopatológico, um carcinoma ductal invasor, associado a um componente intraductal extenso, com margens livres. A paciente foi submetida à excisão ampla com esvaziamento axilar à esquerda e radioterapia. Neste trabalho são discutidos aspectos referentes à freqüência da ocorrência de câncer em mama acessória, às dificuldades diagnósticas, ao prognóstico e às estratégias terapêuticas.