ABSTRACT
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.
Subject(s)
Lysosomal Storage Diseases , Fatal Outcome , Female , Humans , Hydrops Fetalis/etiology , Infant, Newborn , Lysosomal Storage Diseases/complications , MaleABSTRACT
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.
Subject(s)
Adrenal Cortex Hormones/therapeutic use , Autoimmune Diseases/immunology , Immunity, Maternally-Acquired , Immunoglobulins, Intravenous/therapeutic use , Immunosuppressive Agents/therapeutic use , Pregnancy Complications, Hematologic/immunology , Purpura, Thrombocytopenic, Idiopathic/immunology , Thrombocytopenia/therapy , Adult , Cerebral Hemorrhage/prevention & control , Evaluation Studies as Topic , Female , Humans , Immunoglobulin G/immunology , Infant, Newborn , Methylprednisolone/therapeutic use , Platelet Count , Pregnancy , Thrombocytopenia/congenital , Thrombocytopenia/etiology , Treatment OutcomeABSTRACT
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.
