RESUMO
Congenital sialidosis is a rare disease resulting from the absence of neurominidase and presenting with hydrops fetalis, hepatosplenomegaly, dysmorphic features, vacuolated lymphocytes and extensive vacuolation of the connective tissue. Elevated levels of sialooligosaccharides in the urine is characteristic. We describe a newborn baby with congenital sialidosis and discuss the difficulties in reaching the diagnosis.
Assuntos
Doenças por Armazenamento dos Lisossomos , Evolução Fatal , Feminino , Humanos , Hidropisia Fetal/etiologia , Recém-Nascido , Doenças por Armazenamento dos Lisossomos/complicações , MasculinoRESUMO
OBJECTIVE: This study was performed to test whether corticosteroids were superior to intravenous immunoglobulins (IVIG) in the treatment of neonatal autoimmune thrombocytopenia (NAT). METHODS: All cases received IVIG, and unresponsive cases received corticosteroids. RESULTS: Of 7 babies who received IVIG, only 1 responded. The 6 remaining cases received corticosteroids thereafter, and all of them responded well to this therapy. CONCLUSIONS: Corticosteroids may be more effective than IVIG in NAT.
Assuntos
Corticosteroides/uso terapêutico , Doenças Autoimunes/imunologia , Imunidade Materno-Adquirida , Imunoglobulinas Intravenosas/uso terapêutico , Imunossupressores/uso terapêutico , Complicações Hematológicas na Gravidez/imunologia , Púrpura Trombocitopênica Idiopática/imunologia , Trombocitopenia/terapia , Adulto , Hemorragia Cerebral/prevenção & controle , Estudos de Avaliação como Assunto , Feminino , Humanos , Imunoglobulina G/imunologia , Recém-Nascido , Metilprednisolona/uso terapêutico , Contagem de Plaquetas , Gravidez , Trombocitopenia/congênito , Trombocitopenia/etiologia , Resultado do TratamentoRESUMO
The outcome of congenital and nosocomial septicemia has been documented in infants who were admitted to a neonatal intensive care unit over a four-year period. The overall incidence of neonatal septicemia in the neonatal intensive care unit was 5.4 percent. Common causes of neonatal septicemia were gram-negative bacilli and staphylococci. Gram-positive microorganisms were the major causative agents for early-onset septicemia. Since the most common pathogen in cases of nosocomial sepsis was gram-negative bacillus, higher mortality rates were observed in nosocomial sepsis. The overall mortality rate in neonatal sepsis was 44.2 percent. The mortality rate in infants in whom nosocomial septicemia developed was significantly higher than in the infants in whom early-onset septicemia developed. However, as gram-negative colonization of the nursery recently changed to gram-positive microorganisms, the mortality rate is hoped to decrease.