Trichosporon beigelii, a new neonatal pathogen.

Trichosporon beigelii is an uncommon cause of sepsis in low-birth-weight infants. We present two cases of neonatal trichosporonosis and two cases of neonatal trichosporon colonization to familiarize neonatologists with this entity and to discuss management considerations. A 23-week-gestation male developed clinical evidence of sepsis on day 10 and was found to have "yeast" growing in a blood culture on day 12. Despite receiving amphotericin B, he expired within 2 days, at which time the organism was identified as T. beigelii. A 23-week gestation female developed fungal septicemia in the second week of life, while being treated for persistent bacterial sepsis. Candida albicans grew from blood culture, while T. beigelii grew from suprapubic urine, tracheal aspirate, and umbilical catheter tip cultures. She died 2 days later despite therapy with amphotericin B, at which time the fungal isolates were correctly identified. Two other infants were found to have colonization of central vascular catheters, without evidence of invasive disease. Trichosporon infections in neonates have been almost uniformly fatal. Most strains of T. beigelii are relatively resistant to amphotericin B and may be confused with Candida sp. on initial culture examinations. Therefore, delays in appropriate treatment may occur. We discuss treatment options, including alternative antifungal drugs, as well as possibilities for combination therapy.

Cerebral glycogenosis, alpha particle type: morphologic and biochemical observations in an infant.

A 3-month-old infant with congenital hypotonia suffering from an unusual form of glycogenosis is reported. The most striking neuropathologic findings were vacuolation of neuropile and glycogen accumulation, especially in the cerebral cortex and cerebellar molecular layer. Ultrastructurally, glycogen accumulation was present mainly in neurites and astrocytic processes, and mostly appeared as rosettes (alpha glycogen particles). Biochemical analysis of glycogen in various regions of the central nervous system showed an increase of up to 100-fold. The cerebral cortex, deep nuclei, and cerebellar cortex had the highest glycogen elevations, while the cerebral white matter glycogen level was normal. Among other tissues, the heart showed a several-fold increase in glycogen content. Muscle, liver, and kidney glycogen levels were not elevated. Findings in this case and in three other reported patients with cerebral glycogenosis of alpha particle type are discussed.

Lipoprotein-X and other tests in the diagnosis of obstructive jaundice in the infant.

The infant with elevated direct-reacting bilirubin levels requires an early specific diagnosis to identify those who would require early surgical intervention and those in whom the bilirubin levels will eventually return to normal. This study compares the accuracy of three tests: the serum lipoprotein-X (LP-X), the I131 rose bengal (IRB) excretion and the serum alpha-fetoprotein (AFP) in making a specific diagnosis in 15 patients. When used individually the accuracy of the tests varies from 56-100%. The LP-X and IRB excretion are more specific and when in agreement are 100% acurate in the diagnosis of the neonatal hepatitis syndrome (NHS) or extrahepatic biliary obstruction (EHBO). This study suggests that both the LP-X and IRB excretion should be used in the investigation of the infant with conjugated hyperbilirubinemia.

Chylothorax in two mongoloid infants.

Two newborns with stigmata of Down syndrome required resuscitation at birth because of unilateral congenital chylous pleural effusion. After thoracentesis, ventilation was improved. Congenital chylous pleural effusion in newborns with Down syndrome has not previously been described. Pleural effusion at birth must be promptly diagnosed and aspirated.