Recurrent bacteremia with enteric pathogens in recessive polycystic kidney disease.

Eight children with autosomal recessive polycystic kidney disease (ARPKD) and recurrent bacteremia with enteric pathogens are described. Typical clinical features of bacterial cholangitis were absent, although in five patients histological and/or microbiological data indicated that the bacteremic episodes originated in the biliary tree. Bacteremia with enteric pathogens or recurrent culture-negative febrile illness in a child with ARPKD should raise suspicion of cholangitis, even in the absence of typical clinical findings.

Aggressive respiratory support and unilateral nephrectomy for infants with severe perinatal autosomal recessive polycystic kidney disease.

Newborn infants with severe autosomal recessive polycystic kidney disease often receive minimal intervention because poor respiratory and renal outcomes are anticipated. We describe two patients whose respiratory failure was successfully treated with aggressive intervention. Massive kidneys restricted gastrointestinal capacity and limited feedings. Uninephrectomy allowed adequate enteral feedings and preserved sufficient renal function for homeostasis and growth.

An analysis of the approach to management of childhood nephrotic syndrome by pediatric nephrologists.

The value of a renal biopsy for a child with frequently relapsing corticosteroid-responsive or corticosteroid-dependent nephrotic syndrome is unresolved. This was the subject of two independent surveys done by North American pediatric nephrologists. In one study, 59% of the respondents indicated that they would nearly always perform a biopsy prior to starting cytotoxic therapy, while 23% would do so rarely. Less experienced pediatric nephrologists were more inclined to recommend a biopsy (P < 0.05). The indications for a renal biopsy were to provide prognostic information and to make decisions concerning further therapy. In the second survey, 33% of pediatric nephrologists said they would perform a renal biopsy in children with frequent relapses, while 91% would recommend a biopsy in children with corticosteroid resistance (P < 0.001). Once a biopsy was performed, therapy was based on the histopathologic findings regardless of the previous clinical response to corticosteroids. At this time, there is no standard approach to the evaluation and management of children with frequently relapsing, corticosteroid-dependent nephrotic syndrome. Some physicians rely on their clinical acumen, whereas others depend on the histopathologic findings.

Anaphylaxis to cutaneous exposure to milk protein in a diaper rash ointment.

A 12-month-old boy, with a strong history of cow's milk allergy, developed two episodes of anaphylaxis following cutaneous application of a casein containing ointment to an inflamed diaper area. RAST testing showed a significant elevation in specific IgE antibodies to milk and milk proteins. This experience suggests that children who are highly sensitive to milk may have severe allergic reactions on cutaneous exposure to milk or milk products.

Phenytoin clearance by continuous ambulatory peritoneal dialysis.

Phenytoin clearance by continuous ambulatory peritoneal dialysis (CAPD) was studied in a three-year, 8-month-old child. This dialysis resulted in a clearance rate less than 1 ml/min. This is different from that reported in the literature for regular peritoneal dialysis for phenytoin overdose. Unlike regular peritoneal dialysis, CAPD may not require phenytoin adjustment.

Congenital nephrotic syndrome detected by hypothyroid screening.

An infant was evaluated for a low thyroxine value, detected on routine neonatal screening for congenital hypothyroidism. She was found to have the congenital nephrotic syndrome, and to be euthyroid with low thyroid binding globulin. Congenital nephrosis can be detected by neonatal screening programs which use thyroxine as the primary screening test, but not by those which use TSH. This may be important in those countries in which the incidence of congenital nephrosis approaches that of congenital hypothyroidism.

Summer camp hemodialysis for children with chronic renal failure.

A hemodialysis unit was established at a rural summer camp for children. Required medical treatment was planned so as to interfere as little as possible with normal camp programs. Campers who require dialysis were mixed fully into the population of normal campers. Twenty-two children participated during the first summer of operation. Our experience indicates that children on maintenance hemodialysis can be integrated with normal peers in a recreational program and can improve their self-image and self-confidence. The program also demonstrates that chronic pediatric hemodialysis can be safely performed in a rural satellite unit.

Hemodialysis at a summer camp.

A successful pediatric hemodialysis program was established at a summer camp. The dialysis campers had an opportunity to develop peer relationships and improve their self image by mastery of new skills and by increasing their sense of independence. Families were able to gain perspective on their child's development. The program will be continued and expanded. Further efforts will be made to determine how a vaction dialysis program aids in the rehabilitation of a dialysis patient,

Recurrent hyperosmolar nonketotic episodes in a young diabetic.

A 15-month-old girl was successfully treated for substantial hyperosmolarity in the absence of ketosis at the onset of permanent insulin-requiring diabetes mellitus. Hypotonic solutions containing small amounts of glucose and subcutaneous administration of low doses of insulin were empolyed. Potassium was added to the hydrating solutions during the second hour of treatment. In the next three months, two recurrences of this syndrome were verified and successfully treated in a similar manner.