Diagnostic values of lipid and lipoprotein levels in late onset neonatal sepsis.

Serum lipids/lipoproteins were assayed to evaluate the diagnostic values of serum lipids/ lipoproteins in neonatal late onset sepsis (NLS) in 36 episodes of NLS (15 of the 36 in preterm newborns and 21 of the 36 in term newborns) while 36 healthy newborns were used as controls. On d 0, levels of total cholesterol (TC), triglyceride (TG), lipoprotein-a (Lp-a), high-density lipoprotein (HDL) and apolipoprotein-A (Apo-A) and B (Apo-B) were found to be significantly lower in the NLS group than in the control group (p=0.001). The sensitivity and specificity values were 61.5% and 69.4% for TC, 96.2% and 44.4% for HDL, 73% and 50% for Lp-a, 69.2% and 83.3% for triglyceride, 73% and 97.2% for Apo-A and 77% and 69.4% for Apo-B, respectively, at diagnosis. In conclusion, Apo-A appeared to be a useful marker for detection of NLS. Low TG levels may be due to impaired triglyceride-related neutralization of lipopolysaccharides in NLS.

Clinical and pathological aspects of ARC (arthrogryposis, renal dysfunction and cholestasis) syndrome in two siblings.

We describe the first family report of ARC syndrome (arthrogryposis multiplex congenita, renal dysfunction, and cholestasis) diagnosed in Turkey. ARC syndrome is a rare cause of cholestatic jaundice and skeletal abnormalities in the neonatal period. Fanconi-like renal tubular dysfunction completed the clinical picture. Consanguinity and affected membership are the other typical components of this rare disorder, and possibility of autosomal recessive transmission was considered. A broad spectrum of histopathological abnormalities have been described in the liver and kidney. In this report, we describe two male siblings with ARC syndrome who had cholestatic jaundice, arthrogryposis multiplex congenital-like joint contractures and renal involvement with additional clinical features. Clinical and pathological aspects of the syndrome are discussed and compared with the other cases in the literature.

Oral acitretin treatment in severe congenital ichthyosis of the neonate.

Two newborn infants with ichthyosis, one with lamellar ichthyosis and one with nonbullous ichthyosis form erythroderma, who presented at birth with a collodion baby appearance, were treated with acitretin (1 mg/kg/day). Clinical improvement was achieved shortly after treatment. The second case received oral retinoid for 3.5 months and was followed for nine months. The result was excellent. The treatment resulted in a satisfactory improvement in the skin condition of the first case. The tolerance to the drug was good. Side effects were not observed. It was concluded that early management of severe ichthyosis cases could prevent life-threatening events such as hyperthermia, disturbance in electrolyte and fluid balance, and infection.