Rare manifestations of sirenomelia syndrome: a report of five cases.

Five cases of sirenomelia presented with rare manifestations are discussed. Three neonates were born alive and died within 2 to 12 hours after birth. One case was the offspring of a triple in vitro fertilization pregnancy with history of early intrauterine death of one of the triplets. The main features included fusion of lower extremities (five of five), renal agenesis (three of five), polycystic renal dysplasia (two of five), anal atresia with large bowel hypoplasia (three of five), pulmonary hypoplasia (four of five), and single umbilical artery (five of five). Other features that have only rarely been associated with sirenomelia included concurrence of congenital heart disease and neuroblastoma, gallbladder agenesis, and upper extremity defects.

In vivo effect of rhGM-CSF And rhG-CSF on monocyte HLA-DR expression of septic neonates.

OBJECTIVE: To investigate the effect of rhGM-CSF and rhG-CSF on the monocyte HLA-DR expression of septic neonates. SUBJECTS: 60 septic neonates and 41 healthy ones. Septic neonates were randomly assigned into three treatment groups, the GM-CSF group [n=20, rhGM-CSF 5 mcg/kg/d for 4 days, intravenously over 2h (IV)], the G-CSF group (n=20, rhG-CSF 10 mcg/kg/d for 4 days, IV) and the placebo group (n=20, normal saline for 4 days, IV). MEASUREMENTS: Serial (days 0,1, 3 and 5 after the onset of sepsis) measurements of the percentage of HLA-DR positive monocytes (%HLA-DR+ monocytes) and mean fluorescence intensity (MFI) by flow-cytometry as well as the absolute monocyte counts (AMC). MAIN RESULTS: On day 0, the HLA-DR expression of the septic neonates (%HLA-DR+ monocytes: 38%+/-1.8% (mean+/-SEM) and MFI: 73+/-3.4) was significantly lower than the healthy control values (%HLA-DR+ monocytes: 68%+/-2% and MFI: 123+/-4.6) (P<0.0001, for both parameters). On follow up (days 1, 3 and 5), a significant increase of HLA-DR expression was observed in all the groups of septic neonates. Healthy control values of %HLA-DR+ monocytes were reached by day 1 in the GM-CSF group and by day 3 in the G-CSF and placebo groups. Healthy control values of MFI were reached by day 3 in all groups of septic neonates. The AMC showed a significant increase in the GM-CSF group (during the whole follow up period) and in the G-CSF group (for the first 3 days of follow up). CONCLUSIONS: The monocyte HLA-DR expression is depressed on the onset of neonatal sepsis and is progressively restored during the following days. Treatment with rhGM-CSF results in an earlier increase of the number of monocytes expressing the HLA-DR.