William E. Segar

Diffuse Glomerulonephritis Associated with Infected Ventriculoatrial Shunt

Abstract Of six patients with diffuse glomerulonephritis and associated infected ventriculoatrial shunt, three had improved renal function after removal of the infected shunt, and three died from Central-nervous-system disease. The renal disease probably resulted from an immunologic response to the staphylococcal infection rather than from direct bacterial embolization of the kidney.

Wegener granulomatosis in childhood: prolonged survival following cytotoxic therapy.

1. Jones KL, and Smith DW: The Williams elfin facies syndrome, J PEDIATR 86:718, 1975. 2. Williams JCP, Barratt-Boyes BC, and Lowe JB: Supravalvular aortic stenosis, Circular 24:1311, 1961. 3. Wiltse HE, Goldbloom RB, Antia AU, Ottesen OE, Rowe RO, and Cooke RE: Infantile hypercalcemia syndrome in twins, N Engl J Med 275:1157, 1966. 4. Illig R, and Prader A: Kasuistische Beitrage zur Idiopathischen Hypercalcemie und Vitamin D Intoxication, Helv Paediatr Acta 14:618, 1959. 5. Forfar JO: Clinical and metabolic studies in idiopathic hypercalcemia of infancy, M.D. Thesis, Scotland, 1958, University of St. Andrews, p 78 (cited in reference 6). 6. Kenny FM, Aceto T Jr, Purisch M, Harrison HE, Harrison HC, and Blizzard RM: Metabolic studies in a patient with idiopathic hypercalcemia of infancy, J P~DIATR 62:531, 1963. 7. Antia AU, Wiltse HE, Rowe RD, Pitt EL, Levin S, Ottesen OE, and Cooke RE: Pathogenesis of the supravalvular aortic stenosis syndrome, J PEDIATR 61:431, 1967.

Peritoneal Dialysis in the Treatment of Boric Acid Poisoning

THE purpose of this communication is to demonstrate that peritoneal dialysis can be performed safely and successfully on small infants and that the removal of boric acid and, presumably, other wate...

Secondary Oxalosis as a Complication of Parenteral Alimentation in Acute Renal Failure

Secondary oxalosis is a complication of renal failure. Since oxalate is excreted by the kidney, the severity of oxalate deposition has been directly related to the duration of renal failure. We report a patient with acute renal failure on hemodialysis who received daily ascorbic acid (an oxalate precursor) via parenteral alimentation. He developed widespread secondary oxalosis, which was especially prominent in the kidneys and pancreas. This oxalate burden may have contributed to the complications seen during his hospital course. The provision of ascorbic acid to patients with renal failure should be carefully monitored to avoid accelerated development of secondary oxalosis.

Peripheral gangrene complicating idiopathic and recessive hemolytic uremic syndromes

Abstract Three patients with hemolytic uremic syndrome (HUS) developed peripheral gangrene. Bilateral carotid artery thromboses occurred in one of these patients after recovery from HUS. One patient had a long history of juvenile rheumatoid arthritis. In the second patient, a flu-like illness preceded the onset of HUS. The third was one of two sisters, with the HUS appearing more than 1 year apart. None had evidence of disseminated intravascular coagulation or infection with Streptococcus pneumoniae. The patient with rheumatoid arthritis had renal cortical necrosis but recovered moderate renal function after treatment with dialysis and plasmapheresis for 6 months. The child with a genetic form of HUS died of renal failure and had massive cortical necrosis and vascular thrombosis at autopsy. This is the first report of peripheral gangrene in children with idiopathic HUS and autosomal recessive HUS.

Lack of inhibition of vasopressin release in midfacial hypoplasia

asymptomatic carrier among household contacts. However, this number may be an underestimate, because bacteriologic monitoring of asymptomatic contacts was not continued if initial stool cultures were negative. In vitro antibiotic susceptibilities of Campylobacter have been described by ,Butzler et al. 1 Erythromycin or aminoglycosides are uniformly active and could be prescribed for oral or parenteral therapy in children. There is no evidence, however, that these drugs improve the clinical or bacteriologic course of illness; the indications for antibacterial treatment are undefined. Because of its slow growth and special gaseous requirement, Campylobacter will not be recognized by routine diagnostic procedures for enteric pathogens. Clinical laboratories, particularly in pediatric hospitals, should be aware of this seemingly important enteric pathogen, and include special isolation techniques for Campylobacter in their routine diagnostic procedures.

HEGENER'S GRANULOMATOSIS (WG) IN CHILDHOOD WITH PROLONGED SURVIVAL FOLLOWING CYTOTOXIC THERAPY

Wegeners granulomatosis is a systemic disease of unknown etiology involving mainly the kidney and respiratory tract in a necrotizing and granulomatous vasculitis, though other organ systems may be affected. Only 6 cases have been reported in children under age 16 years and each had a rapidly fatal outcome despite supportive therapy and corticosteroids. Although several studies have reported a good response in adults to cytotoxic therapy, this form of therapy has not been tried in children with WG. We have treated 2 girls, aged 10 and 13 years, with a combination of prednisone and cyclophosphamide therapy for generalized WG with renal, ocular, nasopharyngeal, sinus and cutaneous involvement. One patient also had middle ear pulmonary and laryngeal granulomata. Multiple biopsies of involved tissues confirmed the diagnoses. Cyclophosphamide was capable of inducing remission in disease activity initially and during subsequent relapses. Proteinuria decreased in one patient from 5.2 to 0.8 gm/24 h and her serum creatinine concentration fell from 2.1 to 1.3 mg/dl. Pulmonary granuloma regressed in the second patient. Hemorrhagic cystitis was the only complication of therapy, but did not recur with readministration. These patients have survived 2½ and 5 years respectively. Both are in good health and attending school at present. These are the first children of which we are aware to receive this form of therapy for WG.

EVALUATION OF AN EFFICIENT INEXPENSIVE TEACHING MODEL FOR VENOUS CUT DOWN

Because of its life-saving potential, teaching of emergency intravenous access including saphenous cutdown technique demands high priority in pediatric training programs. An inexpensive porcine foot cutdown model has been developed and tested on two pediatric housestaff populations in Syracuse, NY and Madison, WI (n=50).Residents were instructed to place a 2 cm transverse incision 2-3 cm superior to the intersection of the first and second digits on segments of pig extremities split midline in the saggital plane. Veins may be located superficially or within deep fascial compartments. Although nonviable tissue is utilized, characteristics of the skin, connective tissue, fascia, tendons, bones, and veins provide a reasonable approximation of structures encountered in the child. Cannulation by direct insertion, by Seldinger technique, and by surgical flap and vein dilator are all serially compatible utilizing this model.Residents exposed to this teaching device have readily completed cutdowns with minimal difficulty, with little cost, with satisfaction for the model, and without the need for anesthetized live animals.Most pediatricians are likely to encounter situations where saphenous vein cutdown may be lifesaving. Initial practice utilizing the porcine foot model provides an inexpensive but realistic teaching device for this fundamental procedure.

1064 CHRONIC HYPONATREMIA AND MIDFACIAL HYPOPLASIA (MFH)

Persistent hyponatremia (Na 126 mEq/L) was found in a 30 month old girl with typical features of MFH. Hyponatremia, serum hypoosmolality (250 mOsm/L), urine hyperosmolality (U/P osm 3:1) and continued urinary sodium excretion suggested the syndrome of inappropriate antidiuretic hormone secretion (SIADH). Renal, adrenal, pulmonary and drug etiologies for SIADH were excluded. 5 mEq/kg hypertonic Na Cl resulted in less than expected rise in serum Na (115 to 120 mEq/L) and marked increase in urine Na excretion (26 to 95 mEq/L). Water loading (25 ml/kg D5W) leading to serum osmolality fall from 250 to 227 mOsm/L or volume expansion (25 ml/kg 0.9% Na Cl iv) did not lead to formation of dilute urine (lowest U osm 686 mOsm/L). Also plasma ADH measured by radioimmunoassay did not fall to 0 (lowest value 1.4 μlU/ml). These findings suggested resistance of hypothalamic center to afferent stimuli from volume and osmoreceptors which normally inhibit ADH release. Only fluid restriction (40 ml/kg/24h) successfully raised serum Na to normal. Serum Na returned to hyponatremic levels on 3 days ad lib diet. MFH has been associated with deficiencies of anterior and posterior pituitary hormones. This report documents persistent ADH excess and further suggests hypothalamic abnormalities in MFH.