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The human complement system is vital for host defense and plays a role in a number of inflammatory disorders. Inherited deficiency or dysfunction of most of the individual complement components occurs uncommonly. The phenotype displayed by such patients varies with the specific component deficiency and ranges from recurrent infections to autoimmune diseases. Most of the latter are associated with glomerulonephritis. The onset of severe lupus erythematosus in a young child, with prominent cutaneous and renal manifestations, especially if a similar disorder is present in another family member, is a clue to the presence of a complement component deficiency. The distinguishing of acquired deficiencies from inherited deficiencies in complement components is sometimes difficult and may require sophisticated laboratory testing. [ABSTRACT FROM AUTHOR]

The neonatal Bartter syndrome (NBS) is associated with a complex disorder of mineral metabolism in children, including hypercalciuria, nephrocalcinosis, and diminished bone mineral density. Although cyclooxygenase inhibition usually brings about improvement in these findings, there is a variable component which is resistant to such therapy in many children. The factor mediating this disorder has not been identified. Blood and urine from 12 children with NBS were examined. When compared with samples from normal children and adults, all (NBS) sera reduced bone calcium uptake in a bone disc bioassay. This effect persisted in the presence of parathyroid hormone (PTH) antibody and PTH receptor blockade, indicating that neither PTH nor PTH related peptide was responsible. It was eliminated by indomethacin, suggesting that prostanoid generation was essential. Protamine was also inhibitory, as was the addition of ecteola, an anion binder. Activity could be recovered from ecteola by elution with hypertonic buffer. Urine samples from children with NBS had the same calcitropic effect. The agent was removed by ecteola and recovered by hypertonic elution. Activity was eliminated by protamine and by heparinase, but not by trypsin digestion. Size exclusion centrifugation showed that the activity was associated with a material between 10 and 30 kilodaltons. Finally, urine ecteola eluates from NBS patients raised serum concentrations of calcium after intraperitoneal injection in rats. These data suggest that children with NBS have a calcitropic substance in their serum and urine which is not found in normal individuals. The substance is heparin like, and mediates its effects through prostanoid production. These studies provide additional evidence against a direct renal cause of the urinary calcium disturbance characteristic of the disorder. [ABSTRACT FROM AUTHOR]

Children with neonatal Bartter syndrome (NBS) have hypercalciuria, nephrocalcinosis, and osteopenia. A complex of basic-fibroblast growth factor (b-FGF) and a naturally occurring glycosaminoglycan has been identified in the serum and urine of NBS patients. This complex increases bone resorption in a bone disc bioassay system. Angiotensin II (AT II), which is increased in Bartter syndrome, increases the synthesis of b-FGF by cultured endothelial cells. Addition of 10[sup -8 ]M AT II to the bioassay, a concentration reported in Bartter syndrome patients, significantly decreased calcium uptake into bone discs [E/C 0.60 (0.04), P< 0.001 compared with buffer, normal E/C >0.90]. Adding b-FGF monoclonal antibody at 10 μg/ml [E/C 0.90 (0.06), P=NS] or indomethacin [E/C 1.00 (0.03), P=NS] to 10[sup -8 ]M AT II neutralized this effect. In separate experiments, newborn rats were given intraperitoneal injections of AT II. Bone discs from these animals were used in the bioassay system and calcium uptake was markedly reduced compared with discs from rats injected with phosphate-buffered saline [AT II 6.6×10[sup -9], E/C 0.10 (0.04), P<0.001, AT II 3.3×10[sup -8], E/C 0.10 (0.05), P<0.001]. AT II decreases calcium uptake in the bone disc bioassay system. This effect can be abrogated by antibody to b-FGF or prostaglandin synthetase inhibition. These results support the hypothesis that in children with NBS, elevated levels of AT II stimulate local skeletal b-FGF synthesis, with a resultant increase in bone resorption via a prostaglandin-dependent pathway. [ABSTRACT FROM AUTHOR]

Decades after the first report of a complement-activating factor in the serum of some people with chronic glomerulonephritis, its causal role in the disease is still debated. In a new study, mice engineered to lack a regulatory protein, factor H, uniformly develop glomerulonephritis, and have ongoing consumption of serum complement similar to that seen in humans. [ABSTRACT FROM AUTHOR]

Presents a case of peritoneal pseudocyst. Medical history; Diagnosis; Pathophysiology.