Edmund C. Burke

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.

Surgical Treatment of Coarctation of the Aorta in a Ten Week Old Infant: Report of a Case

Although most patients with coarctation of the aorta survive to early adult life, it has been adequately demonstrated that some patients die in infancy and early childhood as a result of their coarctation. In selected cases, then, operation is advisable at a very early age. Cardiac failure in a 10 week old infant necessitated operation at this age in the case reported.

Hemolytic-Uremic Syndrome in Two Siblings

RECENT reviews1 , 3 do not mention the hemolytic-uremic syndrome of children in siblings except for the fatal outcome in six-month-old, hyperlipemic monozygotic twins.3 We encountered this syndrome...

Long-term Prognosis for Children With Nephrotic Syndrome

Follow-up survival and health information were obtained, after a median of 27.5 years, from 132 patients who had been seen originally as children with nephrotic syndrome between 1951 and 1967. Ninety seven patients were alive. Recurring edema or proteinuria, or both, persisted in 15 percent of those still alive. Eight of 11 parous women reported relapses during pregnancy. There was no apparent increase in malignancies, atopic diseases, clinical defects in cell-mediated immunity, or cardiovascular diseases. Twenty two patients (17%) died of renal causes between 3 months and 8 years after the onset of nephrotic syndrome. Steroid resistance was the presenting feature universally predictive of a poor outcome; nine of the 11 such patients died and the other two are now receiving hemodialysis. Hematuria was present initially in 41 percent of the patients who died of renal causes, compared with 14 percent of those still alive. Hypertension was noted on the first examination in 22 percent of those who died of renal causes, compared with 10 percent of those alive.

Identification of Heterozygous Carriers of Gargoylism

Summary The carbazole and naphthoresorcinol methods for hexuronic acid determination have been used to characterize the partially purified acid mucopolysaccharides obtained from urine of normal persons, from patients with gargoylism and from their relatives. The results, expressed as a ratio of carbazole to naphthoresorcinol hexuronic acid, demonstrated a similar abnormal qualitative excretion of chondroitin sulfate B by patients with gargoylism. by one or both parents, and by some of their other relatives. Occurrence of an abnormal qualitative excretion of chondroitin sulfate B in the clinically normal relatives of patients with gargoylism probably indicates the heterozygous carrier state of the disease.

Familial nephrotic syndrome with nephrocalcinosis and tubular dysfunction

Five children in two families, three boys in one family and two girls in another, had the onset of nephrotic syndrome in early infancy. The proteinuria and edema formation were not altered by steroid therapy. Growth retardation, renal osteodystrophy, and tetany appeared at an early age. Glycosuria, aminoaciduria, and renal tubular acidosis developed during the course of illness, and nephrocalcinosis was noted. A renal biopsy specimen from a child in each family showed glomerular proliferative changes, glomerular scarring, interstitial inflammation, and microcalcinosis, together with extensive tubular atrophy. Three of the five children have died so far, all at about 8 years of age. The similarity of the clinical course suggests that the children in each of these families has a distinct inherited disease, a form of familial nephrotic syndrome.

Juvenile xanthogranuloma with ocular involvement: Report of case

Summary “Juvenile xanthogranuloma” issuggested as the best diagnostic term for the condition characterized by yellowish-brown nodules of the skin present at birth or shortly thereafter. The blood lipids are normal and the general health is unaffected. Spontaneous involution of the lesions occurs. The histologic picture consists of proliferation of histiocytes and xanthomatous giant cells. One case of nevoxantho-endotheliomawith involvement of the eye is reported. Inasmuch as the lesions are benign and tend to regress, the ocular globe should be left intact with the hope of eventual return of vision.

Proteinuria in Children Review and Evaluation

Current information relating to the clinical significance of proteinuria in children through adolescence was reviewed. Proteinuria may be transient, orthostatic, or fixed. The data indicate that only fixed proteinuria—that is, proteinuria on multiple urine examinations without pos tural exaggeration—may indicate serious glomerular disease. The patient should be followed with periodic but infrequent examinations, avoiding unnecessary, complicated, and hazardous investigations.

Familial nephrotic syndrocalcinosis and tubular dysfunction

We have studied 5 children in 2 families, 3 boys in family A and 2 girls in family B, who had the onset of nephrotic syndrome in early infancy. The syndrome proved to be steroid-resistant, although there was no evidence of tubular failure initially. Growth retardation, renal osteodystrophy, and tetany appeared in several at an early age, and all manifested these symptoms eventually. Glycosuria, aminoaciduria, and nephrocalcinosis also appeared. Renal biopsies from 2 children in each family showed glomerular proliferative changes, glomerular scarring, interstitial inflammation, and microcalcinosis together with marked tubular atrophy. Autopsy studies were completed on 2 boys. Each boy died when about 8 years old. One girl is preterminal with renal failure at 8 years of age. Hypertension was present only terminally. The youngest boy in family A has not yet developed nephrocalcinosis. Total-body composition studies on 1 boy and 2 girls showed increased exchangeable sodium, increase in extracellular water, decrease in total body potassium, and decrease in intracellular potassium. Whereas tubular failure previously has been reported in patients with long-standing nephrotic syndrome, we believe these cases differ in that they are familial and have interstitial nephritis and nephrocalcinosis.

Procedure for Needle Biopsy of the Kidney

any premedication, relying on local anesthesia with procaine. Reassuring the child and allowing adequate time for the procaine to take effect are two key factors. The child is placed with his abdomen on a small, long, narrow sandbag that is situated just below the rib cage. With an applicator dipped in gentian violet, three lines are drawn on the patient’s back (Fig. 3): the first over the dorsal processes of the lumbar spinal