Systemic lupus erythematosus associated with acute Epstein-Barr virus infection.

Systemic lupus erythematosus (SLE) is a multisystem disease of unknown origin, characterized by a variety of autoimmune phenomena. Viruses have long been postulated to play a role in its pathogenesis. Several observations suggested a link between Epstein-Barr virus (EBV) and SLE. We describe a 14-year-old girl who presented with acute onset of SLE concurrently with clinical and laboratory findings consistent with EBV-induced infectious mononucleosis (IM). Evidence for acute EBV infection was confirmed by serological studies and detection of specific EBV antigens on kidney biopsy. This close association between EBV and SLE suggests a possible role of the virus in the pathogenesis of SLE in this patient.

Excessive dietary protein and suboptimal caloric intake have a negative effect on the growth of children with chronic renal disease before and during growth hormone therapy.

Although diet and nutrition are an integral part of the management of individuals with chronic renal failure (CRF), little has been written on the effect of nutrition on the growth response to growth hormone (GH) in CRF. We studied the GH axis and nutritional status of 31 prepubertal children aged 8.7 +/- 0.5 years with a height standard deviation score (SDS) of -3.2 +/- 0.2 (mean +/- SEM) with CRF. Sixteen CRF patients on hemodialysis and 15 on peritoneal dialysis were studied. Forty-four age-matched normal short children without GH deficiency served as controls. Spontaneous 12-hour GH and stimulated GH values were significantly higher and GH binding protein (GHBP) was significantly lower in the CRF patients than in the normal short children. Both before the initiation of GH therapy and after the first year of treatment, the growth velocity (SDS) was inversely correlated with dietary protein intake and positively correlated with caloric intake. GH was administered at a dosage of 28 and 21 IU/m2/wk to the CRF group and the normal short children, respectively, divided into seven daily doses. The growth response of the normal short children was significantly greater than that of the CRF patients. GH therapy induced a smaller increment in GHBP and IGF-I in the CRF patients versus the normal short children (8.8 +/- 2.2 and 10.2 +/- 2.7 v 24.8 +/- 1.3 and 27.6 +/- 2.5 nmol/L, respectively, P < .01). The 1-year growth velocity of the CRF children was most closely correlated with dietary protein and caloric intake. The nutritional status of CRF patients is concluded to be a major factor in growth both before and during GH therapy.

Pseudohypoaldosteronism.

Three children, two of whom were siblings, had pseudohypoaldosteronism. The features of this condition include failure to thrive, hyperkalemia, metabolic acidosis, salt wasting, elevated peripheral renin activity, and increased plasma aldosterone concentration. Hyperplasia of juxtaglomerular apparatus was seen in a renal biopsy specimen from one of these patients. Administration of large quantities of salt normalized the serum electrolyte abnormalities and permitted normal growth. Furthermore, the serum electrolyte abnormalities were prevented by administration of increased amounts of salt to one of these children from birth.

Neonatal neutrophilia: possible role of a humoral granulopoietic factor.

During the first days of life, newborn infants have leucocytosis with marked neutrophilia and a "shift to the left," the mechanism for which is as yet unknown. In an attempt to elucidate whether humoral granulopoietic factor(s) plays a role in this phenomenon, serial measurements of urinary and serum colony-stimulating activity levels were made in healthy newborn infants and normal older controls. Twenty-four-hr urine collections, serum samples, and complete blood counts were obtained from 30 full-term normal infants 24 hr and 4 days after delivery and in 13 of them on the 14th and 28th days of life as well. Specimens were assayed for their colony stimulating activity levels by their ability to stimulate bone marrow cells from C3HB mice to grow into colonies in soft agar. Elevated neutrophil, band form, and monocyte counts were found during the first day of life, which gradually decreased thereafter. Serum and urinary colony-stimulating activity levels were significantly increased (3- to 5-fold) over the controls on the first and fourth days of life, but declined to normal values by the 14th and 28th days.

Blood pressure determinations in Israeli schoolchildren aged 5 to 14 years.

Blood pressure (BP) determinations were made by three physicians in 1,554 healthy Israeli schoolchildren aged 5 to 14 years, of whom 783 wer boys and 771 were girls. Subjects were divided into four groups according to ethnic origin: Yemenite--560, North African--357, Iraqi-Iranian--246, and European-American--391. There were no statistically significant differences in either systolic (S) or diastolic (D) BP between ethnic groups or between sexes. Comparing our data with those compiled by the United States Task Force on Blood Pressure Control in Children, we found that the SBP tended to be lower than in American children by about 10 mm Hg, while the DBP was lower by about 7 mm Hg. An additional 4,460 children were examined by public health nurses, and 25 (0.6%) children were found to have a DBP above the 97th percentile for their age, and 46 (1.0%) children were found to have an SBP above the 97th percentile for their age. None of the children had an SBP or DBP exceeding the mean for their age by 2.5 SD, nor had any child any sign or symptom of high BP. Based on these results, we doubt the usefulness and cost-effectiveness of BP screening of children.

Growth effect of human chorionic gonadotrophin in 2-8-year-old boys with undescended testes.

Growth-promoting effect of human chorionic gonadotrophin (HCG) was studied in 40 boys of 2-8 years with unilateral undescended testes. A transient acceleration of height and weight increase was noted that exceeded rates found in normal puberty. No significant advance in bone age was noted following treatment. On the basis of this study we conclude that short-term HCG treatment does not change the growth pattern or bone age of 2-8-year-old boys.

Organophosphate poisoning presenting as diabetic ketoacidosis.

A 3-year-old boy was admitted to hospital following rapid-onset coma. Laboratory tests demonstrated hyperglycemia, glycosuria and keto-acidosis. Organophosphorus poisoning was the cause of the coma since he had been in contact with Parathion, serum cholinesterase activity was undetectable and his condition returned to normal under atropine therapy.

Clinical outcome and follow-up of prenatal hydronephrosis.

Between 1987 and 1991, 160 hydronephrotic kidneys were diagnosed prenatally and confirmed postnatally in 100 infants. The aim of the study was to describe the natural history and management of primary hydronephrosis detected prenatally. We devised a new classification of obstructive uropathy outcome using ultrasonography and the diethylenetriamine penta-acetic acid scan. Accordingly, we classified the patients as having mild, moderate or severe hydronephrosis. Nine patients had pyeloplasties and 5 had ureteric reimplantations. We conclude that in most cases there is no need for immediate surgery, and that the initial approach to the management of congenital hydronephrosis should be conservative. We suggest that anti-bacterial prophylaxis be conventionally given to infants with vesicoureteral reflux and for the first 6 months of life to infants demonstrating moderate to severe newborn primary hydronephrosis.

Muscle extract infusion in rabbits. A new experimental model of the crush syndrome.

Previous studies provide inconclusive data concerning the nephrotoxicity of myoglobin following muscle injury. We investigated the possibility that released muscle constituents other than myoglobin may be associated with renal damage, and studied accompanying hematological and coagulation changes. An extract of homologous or autologous muscle was infused intravenously in rabbits in a dose of 100 mg of muscle extract protein/kg; equine myoglobin was given to control animals. Experimental animals developed proteinuria, cylindruria, and a 50% reduction in glomerular filtration rate. Leukopenia, thrombocytopenia and evidence of intravascular coagulation also were seen. The muscle extract was shown to have thromboplastic activity; however inhibition of this by phospholipase C did not prevent the changes induced by muscle extract infusion possibly because the intrinsic changes coagulation pathway still was activated. Although moderate hypotension and ECG changes developed in some rabbits, these were not consistent and the renal functional changes appeared to be independent of these factors. Pulmonary and glomerular microthrombi were seen in experimental animals and there was vacuolation of the renal proximal tubular cells. The studies indicate that a number of biological systems are activated following muscle extract infusion and that these may be more important than the nephrotoxicity of myoglobin in the pathogenesis of the renal injury.

Effect of growth hormone therapy on IGF-I, bone GLA-protein and bone mineral content in short children with and without chronic renal failure.

Chronic renal failure (CRF) in the young is complicated by, among other conditions, growth retardation, hyperparathyroidism and uremic osteodystrophy. Many children with CRF are now being treated with growth hormone (GH). Since GH has a direct mitogenic effect on osteoblasts in culture, we studied the effects of GH therapy on osteoblastic activity, such as serum alkaline phosphatase (AP), bone GLA-protein (BGP) and bone mass density (BMD) in poorly growing children with and without CRF. Fifteen (4 girls, 11 boys) healthy children with short stature (SS) and 10 (3 girls, 7 boys) children with end-stage renal failure (CRF) 4.5-12.4 years of age were treated with daily subcutaneous injections of GH in a dose of 0.1-0.125 IU/kg/day for 1 year. IGF-I, BGP and BMD of the spine were determined before and after the year of treatment. During GH therapy, a similar increase in height velocity and IGF-I were noted in SS and CRF groups: 3.8 +/- 0.77 to 8.38 +/- 1.25 (p < 0.001) vs. 4.0 +/- 0.6 to 7.14 +/- 1.3 cm/year (p < 0.001) and 7.8 +/- 2.6 to 21.8 +/- 7.5 (p < 0.01) vs. 7.9 +/- 1.3 to 21.5 +/- 5.6 nmol/l (p < 0.01), respectively. AP increased from 205 +/- 27 to 274 +/- 50 IU/l (p < 0.01) in the SS group but not in CRF patients (223 +/- 58 pre- 218 +/- 51 IU/l post-GH therapy).(ABSTRACT TRUNCATED AT 250 WORDS)

Evaluation of prenatally diagnosed hydronephrosis by morphometric measurements of the kidney.

A large number of hydronephrotic kidneys (108) were diagnosed prenatally in 69 infants between 1987 and 1991 and subsequently confirmed postnatally. Prenatal morphometric measurements were done in order to find reliable parameters for the detection of a group at risk for surgical treatment. A second aim of the study was to describe the natural history and management of hydronephrosis detected prenatally. We devised a classification of postnatal obstructive uropathy using ultrasonography and the renal scan. Accordingly, we classified the patients as having mild, moderate or severe hydronephrosis. A renal pelvic antero-posterior diameter (APD) of 9 mm or more, and a pelvic-to-renal APD ratio of 0.45 before 32 weeks of gestation and 0.52 thereafter, were found to be useful for the detection of severe outcome. Our new parameter, a pelvic-to-renal volume ratio of greater than 0.08, can also be used for this purpose.