Risk factors for mortality in infants and young children on dialysis.

The factors associated with a greater mortality risk in infants and young children undergoing dialysis have not been clearly determined. We report the results of a North American Pediatric Renal Transplant Cooperative Study designed to assess risk factors in patients aged younger than 6 years at initiation of dialysis therapy. Sixty-four nonsurvivors were matched with 110 survivors for age at dialysis initiation, primary renal disease, and year of entry onto the database. Questionnaires on 137 patients (51 nonsurvivors, 86 survivors) were completed by participating centers. Seventy-five percent (103 of 137 patients) of the patients were aged younger than 2 years at dialysis initiation; 42% (58 of 137 patients) had renal aplasia, dysplasia, and/or hypoplasia or obstructive uropathy; 62% were boys; and 62% were white. One-year patient survival rates were 83% in infants beginning dialysis at younger than 3 months of age, 89% in 3- to 23-month-olds, and 95% in 2- to 5-year-olds (P = 0.001). Comorbid nonrenal disease occurred in 37 of 51 nonsurvivors (74%) versus 46 of 84 survivors (55%; P = 0.027). Nonsurvivors had pulmonary disease and/or hypoplasia more often (14 of 37 nonsurvivors; 37.8% versus 8 of 46 survivors; 17.4%; P = 0.04). Oliguria or anuria was present in 23 of 33 nonsurvivors (70%) aged younger than 2 years versus 26 of 64 survivors (41%; P = 0.007). Infection accounted for 15 of 51 deaths (29.4%). In summary, these results suggest that age at dialysis initiation; presence of nonrenal disease, particularly pulmonary disease and/or hypoplasia; and oliguria or anuria in children aged younger than 2 years are identifiable as risk factors for mortality in these young patients.

Terminal deletion of chromosome 10q at band 26.1: follow-up in an adolescent male with high-output renal failure from congenital obstructive uropathy.

We report on the clinical findings in an adolescent male with a de novo terminal deletion of chromosome 10 del(10)(q26.1). This young man is one of the oldest known patients reported with this condition. His condition is compared with that of 11 reported cases of de novo terminal deletion of 10q at band 26. Individuals with chromosome 10q26 deletion have some findings and medical complications in common. Our patient has chronic renal failure due to urinary tract obstruction from posterior urethral valves. Similar anomalies have been reported in cases of 10q26 deletion, suggesting a careful renal/urinary tract evaluation should be completed in individuals with this condition.

Splenorenal arterial bypass in a child with Takayasu's disease: a case report.

In adults splenorenal arterial bypass is a highly effective treatment for renovascular hypertension, but in children the procedure has been less successful because of the small size of the splenic artery. However, with the improvement in microvascular techniques the procedure is now possible in children. A 2-year-old child with Takayasu's arteritis, previous right nephrectomy and severe renovascular hypertension required revascularization to salvage his remaining left kidney. A splenorenal arterial bypass was performed through a left retroperitoneal flank incision. Postoperatively his creatinine level returned to normal and his requirements for antihypertensive medication were markedly diminished. The advantages of a splenorenal arterial bypass for left renal revascularization in a developing child are discussed.

Prognostic implications of circulating immune complexes and Pseudomonas aeruginosa-specific antibodies in cystic fibrosis.

We re-examined the role of circulating immune complexes (CIC) in cystic fibrosis, by their serial measurement in 16 patients who had advanced lung disease and persistent specific antibodies to Pseudomonas aeruginosa in their serum. CIC were detected by 125I C1q-BA (IgG + IgM-IC) and Raji RIA (IgG-IC), and IgA-IC by a modification of the Raji RIA method. Serum precipitins to Ps. aeruginosa were detected by crossed-immunoelectrophoresis (XIE) and antibodies to alkaline protease (AP), elastase (EL) and exotoxin-A (EA) of Ps. aeruginosa were detected by ELISA technique. CIC were positive in greater than 50% of the samples in 8 patients (group A) and in less than 25% in the other 8 (group B); follow-up averaged 22 mo in group A and 26 mo in group B. The numbers of precipitins to Pseudomonas were equally high, and levels of antibodies to AP, EL, and EA of Ps. aeruginosa were similarly positive, in the 2 groups. In group A the CIC were mostly IgG-IC by Raji RIA (76%) and IgA-IC, whereas in group B C1q-binding IC (79%) and IgA-IC were more prevalent than IgG-IC by Raji RIA. Four group A children died during follow-up, together with one group B child in whom the CIC level had suddenly risen precipitously. We postulate that a high level of CIC in association with persistent specific-antibody response to Ps. aeruginosa heralds a poor prognosis in cystic fibrosis.

Combined evaluation of circulating immune complexes and antibodies to Pseudomonas aeruginosa as an immunologic profile in relation to pulmonary function in cystic fibrosis.

We developed a solid-phase radioimmunoassay with a reference standard pseudomonas antigen and used this with 125I-labeled anti-human immunoglobulin to evaluate specific antibodies to Pseudomonas aeruginosa, qualitatively and quantitatively, in sera from children with cystic fibrosis (CF) whose lungs were colonized by this bacterium. The results of this IgG assay correlated with the number of precipitin antibodies to the standard reference antigen determined by cross-immunoelectrophoresis in the same sera. Forced expiratory volume (FEV1; percentage predicted), determined as an indicator of lung injury in CF, was evaluated as an immunologic response to pseudomonas, against a profile derived from combined serial data on both the circulating immune complexes (CIC) and the Ps. aeruginosa antibodies (N = 25 CF patients; 108 sera). This revealed that in CF patients who had no specific IgG antibodies to Ps. aeruginosa and no IgG-CIC had the best pulmonary function (FEV1 = 115 +/- 14.52%) and those with high levels of antibodies to this organism and high IgG-CIC levels had the poorest lung function (FEV1 = 69.75 +/- 10.99%) (P less than 0.05). We believe that this indicates an immunologic basis for lung injury in cystic fibrosis.

Haemolytic uraemic syndrome: evidence of multiple viral infections in a cluster of ten cases.

During July 1979, ten patients were admitted to the hospital with bloody diarrhoea followed by manifestations of haemolytic uraemic syndrome (HUS): acute microangiopathic haemolytic anaemia, intravascular coagulopathy, and impaired renal function. Ages ranged from 13 months to 58 yr, with only two patients more than 5 yr old. In a household that included seven children born to three sisters who married three brothers, six children required hospitalization for bloody diarrhoea and four developed HUS; the father of one case and the maternal grandmother also developed bloody diarrhoea. Echovirus type 11 was isolated from the pharyngeal secretions or faeces of all members of the household with bloody diarrhoea, with the exception of the grandmother. Picornavirus-like particles were seen by direct electron microscopy (E/M) in faeces from four other HUS patients and an adenovirus in one, but these viruses failed to replicate in cell cultures. Parvovirus-like particles were seen by E/M in faeces from six patients. Serological examination indicated recent infection with one or more enteroviruses (echovirus 11, coxsackieviruses A4, B2, B4) in nine cases. Combined viral studies revealed presumptive evidence of recent infection with two or more viruses in all of the patients with HUS. Stools were negative for bacterial pathogens including campylobacter, salmonella, shigella, and yersinia organisms. Only one of nine patients tested had circulating immune complexes. Our data support the concept that the pathology seen in HUS may be due to a Shwartzman-type reaction provoked by concurrent infection with two or more viral agents.