Hypomorphic variant of C3, arthritis, and chronic glomerulonephritis.

Decreased synthesis (hypomorphism) of the fast variant of the third component of complement was detected in three generations of a family in which the propositus has an immune complex-type glomerulonephritis, arthritis, and a false positive test for syphilis. An affected sibling has bursitis, hematuria, and proteinuria. Decreased serum C3 protein was detected in three of four and decreased C3H50 in four of four family members with this hypomorphic variant (C3f). This is the first association between C3f and immune complex-type disease.     

Effect of antibiotic therapy on the clinical course of streptococcal pharyngitis

We examined the effect of antibiotic therapy on the clinical course of group A beta-hemolytic streptococcal (GABHS) pharyngitis in 260 children. After a throat culture had been obtained, each child was evaluated for the presence of predetermined signs and symptoms, and was then randomized in a double-blind manner to receive penicillin V, cefadroxil, or placebo. Of the 194 children with throat cultures positive for GABHS, 68 received penicillin V, 70 received cefadroxil, and 56 received placebo. Approximately 18 to 24 hours later, each patient returned for reevaluation. Significantly fewer children who had received either penicillin or cefadroxil had persistence of each of the three objective signs and each of the three subjective symptoms than did children who had received placebo. In addition, the evaluating physician, parents, and patients all believed that significantly fewer of the patients given antibiotic failed to demonstrate overall clinical improvement.     

Reevaluation of Russell-Silver syndrome

Russell-Silver syndrome was reevaluated 2.9 to 13.0 years after initial diagnosis in 15 patients. At follow-up, five of the 15 patients exhibited late catch-up growth and had normal height, six had developmental delays or mental retardation, and asymmetry was present in five. Given the great variability in the long-term prognosis for growth and development in patients with Russell-Silver syndrome, there is a need to reevaluate this syndrome and its clinical implications.     

Elevated serum 1,25 dihydroxyvitamin D concentrations in rickets of very low-birth-weight infants

Elevated 1,25 dihydroxyvitamin D concentrations were found in five VLBW infants who developed rickets at two to three months postnatal age or term postconceptual age; 25 hydroxyvitamin D concentrations were low. Bone mineralization was found to be extremely low as measured by infant-adapted direct photon absorptiometry. After treatment with a formula supplemented with additional Ca and P, there was a rapid improvement in bone mineralization with a concomitant decrease of 1,25(OH)2D to normal adult values, whereas 250HD values increased and parathyroid hormone values decreased. In the VLBW infants studied, we suggest that rickets may be caused by Ca and P deficiency rather than by a deficiency of vitamin D metabolism.     

Necrotizing enterocolitis in low-birth-weight infants fed an elemental formula.

The incidence of necrotizing enterocolitis in the newborn infant has increased within the same time period that increasing emphasis has been placed on oral alimentation of very small infants. A prospective investigation was conducted to determine the nutritional efficacy as well as the incidence of necrotizing enterocolitis of a standard cow milk formula compared with an elemental formula. Sixteen infants who weighed less than 1,200 gm were randomized and fed one of the two formulas. The clinical status of the two groups was similar. Seven of eight (87.5%) infants fed the elemental formula and two of eitht (25%) fed the standard cow milk formula developed necrotizing enterocolitis (p less than 0.02). The hypertonicity of the elemental diet may have contributed to the increased incidence of necrotizing enterocolitis in infants fed this formula.     

Postheparin lipolytic activity and Intralipid clearance in very low-birth-weight infants

The effect of heparin (10 U/kg) on serum lipolytic activity, triglyceride and FFA levels, during four hours infusion of 0.5 gm/kg Intralipid was measured in 18 AGA infants, 25 to 32 weeks' gestational age. PHLA, TG, and FFA were measured at 0, 10, 30, 120, and 240 minutes of infusion of Intralipid, before and following a bolus of 10 U/kg heparin iv. Lipolytic activity, measured by hydrolysis of activated tri-3H-oleate and expressed in mumol FFA released per milliliter serum per hour, was not detected in serum before heparin administration. Ten minutes after heparin administration peak PHLA was significantly higher in infants of 27 to 32 weeks' gestation than in infants of 25 to 26 weeks' gestation. There was no significant difference in peak PHLA between infants of 27 to 28 and 29 to 32 weeks' gestation. PHLA returned to baseline (zero) two hours after heparin administration in all infants. Infants of 25 to 26 weeks' gestational age had significantly higher concentrations of serum triglycerides before and during Intralipid infusion than in infants of 27 to 32 weeks' gestational age. Although there was a transient rise in FFA 10 and 30 minutes after heparin administration, the levels of FFA and triglycerides were not different at the end of infusion with or without heparin in either group, suggesting that a single bolus of heparin has only a transient effect on Intralipid clearance.