Defective yeast opsonisation and functional deficiency of complement in sickle cell disease.

Opsonisation of heat-killed baker''s yeast, functional activity of the total alternative pathway of complement, and factor B detected functionally and immunochemically were significantly reduced in 72 children with sickle cell disease compared with 40 age-matched black control children. There was significant correlation between functional activity of the total alternative pathway and functionally measured factor B, but not between factor B measured functionally and immunochemically. The opsonisation defect could be corrected in vitro by normal serum, and factor B-depleted serum, and was qualitatively similar to that seen in patients with primary yeast opsonisation deficiency. Serial studies showed that these serum defects were persistent. Reduction in the activity of components of the alternative pathway of complement and opsonisation was found in 4 patients who had recovered from pneumococcal meningitis and in one who developed osteomyelitis. Defects of yeast opsonisation and complement which are common in patients with sickle cell disease, may partly explain the children''s increased susceptibility to infection, and might help to identify individuals especially at risk.     

Anthropometry underestimates body protein depletion in haemodialysis patients

The body composition of 62 haemodialysis patients (41 males) and 63 controls (30 males) was assessed using anthropometry and in vivo neutron activation analysis of body nitrogen. There was no significant difference between patients and controls in body mass index (BMI) and percentage body fat. Arm muscle circumference was significantly reduced in males. Lean body mass was strongly correlated with body nitrogen in controls (r = 0.951) but less so in patients (r = 0.876). The mean standardised body nitrogen index (NI) was reduced in male patients by 13% (95% confidence interval -9 to -17%) and in females by 4% (95% confidence interval +4 to -12%). Of the 16 patients with a NI below the control range, arm muscle circumference was below the control range in only 3 and BMI less than 18 kg/m2 in 2. NI was correlated negatively with the duration of renal replacement therapy, duration of haemodialysis, the number of previous failed transplants and the total dose of steroids received but not with current energy or protein intakes. Steroid dose was the only significant independent variable. Anthropometry underestimates body protein depletion in haemodialysis patients and the degree of protein loss is related to the cumulative dose of corticosteroids previously received.     

Hypercholesterolemia in children: risk and management

Hypercholesterolemia in children is currently a controversial issue. Screening and management recommendations are inconsistent due to the lack of longitudinal data currently available to support specific long-term interventions. Children who are generally at risk and the options for management are discussed.     

Intravenous digital subtraction renal angiography: use in screening for renovascular hypertension

Intravenous digital subtraction renal angiography (DSRA) has been compared with conventional angiography only in small, selected series of hypertensive patients. The authors prospectively examined with intravenous DSRA 94 patients at increased risk for renovascular hypertension and compared these studies with conventional angiography. A stenosis of at least one main renal artery was identified with intravenous DSRA in 22 patients and confirmed in 20 patients. No significant stenoses were seen with conventional angiography in any of the 64 patients in whom lesions were not seen with intravenous DSRA. Since inadequate DSRA studies were considered positive for renal artery stenosis, the sensitivity of intravenous DSRA was 100% (25 of 25); specificity, 93% (64 of 69); positive predictive value, 83% (25 of 30); and negative predictive value, 100% (64 of 64). The authors conclude that intravenous DSRA is a sensitive test for identifying stenosis of the main renal arteries and is appropriate to use as a screening test among patients at increased risk for renovascular hypertension.     

Causes of jaundice during hepatic artery infusion chemotherapy

Jaundice develops in many patients with liver metastases from colorectal adenocarcinoma during hepatic arterial infusion chemotherapy (HAIC). The usual cause is thought to be hepatotoxicity from the chemotherapeutic agent or biliary obstruction from progressive neoplastic disease. The authors evaluated the abdominal computed tomography and ultrasound examinations performed on 49 patients who were jaundiced during long-term HAIC. In only one patient was diffuse intrahepatic biliary dilatation caused by an obstructing mass in the porta. Two patients had metastatic hepatic lesions causing focal biliary obstruction. Intrahepatic dilatation without an obstructing mass occurred in 20 patients. Percutaneous or endoscopic cholangiograms were commonly interpreted prospectively as showing extrinsic compression by metastases, but no mass was confirmed on imaging studies. Seven patients had focal intrahepatic ductal dilatation from stricture without an associated mass. The remaining 19 patients had normal-caliber ducts; their jaundice was caused by chemical hepatitis. This series suggests that the most common causes of jaundice in these patients are chemical hepatitis and common bile duct stricture, complications of intraarterial chemotherapy, rather than neoplastic obstruction. Stricture formation may be confused with extrinsic compression on direct cholangiograms.