Assuntos
Doença de Crohn/diagnóstico , Edema/etiologia , Hiperplasia Gengival/etiologia , Doenças Labiais/etiologia , Síndrome de Melkersson-Rosenthal/diagnóstico , Dor Abdominal/etiologia , Criança , Doença de Crohn/tratamento farmacológico , Diagnóstico Diferencial , Diarreia/etiologia , Edema/tratamento farmacológico , Hiperplasia Gengival/tratamento farmacológico , Humanos , Doenças Labiais/tratamento farmacológico , Masculino , Síndrome de Melkersson-Rosenthal/tratamento farmacológico , Prednisolona/uso terapêuticoRESUMO
After allogeneic hematopoietic stem cell transplantation (HSCT), viral infections/reactivations are a frequent complication, sometimes with fatal outcome. Thus, early diagnosis is recommended by screening of whole blood or plasma preparations using highly sensitive molecular techniques that test for the most common viral pathogens, such as Epstein-Barr virus, cytomegalovirus, and adenoviruses (ADVs). Despite this approach, not every reactivation/infection can be adequately detected or excluded, even with highly sensitive polymerase chain reaction. Particularly after toxic treatment, uncommon infections or infections resistant to first-line treatment can occur, even in unusual locations. Herein, we present the case of a child with Philadelphia chromosome-positive acute lymphoblastic leukemia after allogeneic HSCT who suffered from 5 different viral reactivations/infections, including acyclovir-resistant herpes simplex virus type 1 esophagitis, human herpesvirus 6 encephalitis, rotavirus gastroenteritis, respiratory syncytial virus pneumonia, and ADV esophagitis, despite routinely performed blood examinations for viral pathogens remaining unrevealing at all times.
Assuntos
Transplante de Células-Tronco Hematopoéticas/efeitos adversos , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Viroses/diagnóstico , Viroses/virologia , Adenovírus Humanos/isolamento & purificação , Adolescente , Feminino , Herpesvirus Humano 1/isolamento & purificação , Herpesvirus Humano 6/isolamento & purificação , Humanos , Vírus Sinciciais Respiratórios/isolamento & purificação , Rotavirus/isolamento & purificação , Transplante Homólogo/efeitos adversos , Ativação Viral , Viroses/patologiaRESUMO
Ornithine transcarbamylase deficiency (OTCD) is the most common inborn urea cycle disorder. Patients with OTCD are at risk of acute metabolic decompensation with hyperammonemia and subsequent encephalopathy, coma and death. Symptoms may be triggered by infections, drugs and stress, evoked by trauma, pain, fear, surgery and anaesthesia or by episodes of protein catabolism, i.e. fasting-induced, post partum or during gastrointestinal bleeding. Several specific considerations must be made for anaesthetic and intensive care management in patients with this disease in order to avoid metabolic decompensation. We report the intensive care management of the first manifestation of late-onset OTCD in a 16-year-old girl and a course of inconspicuous general anaesthesia with midazolam, s-ketamine, fentanyl and isoflurane in a 22-year-old girl with known OTCD.