Pelargonium zonate spot virus is transmitted vertically via seed and pollen in tomato.

In autumn 2007, a new disease with unknown etiology was observed in open-field tomato (Solanum lycopersicum) in the Lachish region of Israel. The symptoms included mild mosaic, leaf malformation, and severe stunting of the plants. The causal agent was readily transmitted mechanically from the sap of infected plants to indicator plants. Viral particles were purified from infected plants and cDNA was synthesized from RNA isolated from the particles. Cloning and sequencing of the cDNA showed 95% identity to RNA 3 of Pelargonium zonate spot virus (PZSV). Using reverse-transcription polymerase chain reaction, PZSV was detected in both seed and pollen grains of infected tomato plants. Attempts to disinfect seed by using hydrochloric acid and trisodium phosphate failed to eliminate this PZSV detection. Seed from infected tomato plants gave rise to infected seedlings with a seed-transmission rate of PZSV of 11 to 29%. Pollen grains collected from flowers of infected plants were used to hand pollinate healthy mother tomato plants. Although none of the pollinated mother plants became infected with PZSV, 29% of the seedlings produced from seed harvested from these plants were found to be infected. This is the first demonstration that PZSV is transmitted vertically via both pollen and seed in tomato plants.

Further delineation of cerebro-osteo-nephrosis syndrome.

We describe an Israeli Jewish child of Yemenite origin who may be affected with "cerebro-osteo-nephrosis." She is short of stature (height below 3rd centile) due to skeletal abnormalities. She has minor anomalies and borderline intelligence. There is marked proteinuria and she is in kidney failure. Opitz et al. [1985: Am J Med Genet 22:521-529] described 2 Hutterite sisters in America who were suffering from a condition which greatly resembles that of our patient. We question whether these conditions in the two families are the same syndrome with pleiotropic expression, as suggested by Opitz et al., or whether they represent two distinct genetic entities.

Accidental busulfan overdose: enhanced drug clearance with hemodialysis in a child with Wiskott-Aldrich syndrome.

A 4.6 kg infant with Wiskott-Aldrich syndrome received an accidental overdose of busulfan during preparation for allogeneic stem cell transplantation. Pharmacokinetic analysis of plasma busulfan levels alerted staff to the dosing error. Hemodialysis was immediately performed and resulted in accelerated clearance of busulfan. There were no acute neurologic and hepatic side-effects of the busulfan overdose, and despite 2 months of cough accompanied by rales, the patient is now free of pulmonary symptoms. Stable partial donor chimerism occurred after transplantation. At present, the patient is thriving and infection-free 12 months after transplantation, although his platelet count remains at the lower limit of normal.

Renal function in full-term neonates with hyperbilirubinemia.

The purpose of this study was to investigate the effect of unconjugated hyperbilirubinemia on endogenous creatinine clearance and urinary excretion of sodium, phosphorus, lysozyme, and amino acids in full-term infants. Thirty-seven healthy, breast-fed newborns who were not exposed to phototherapy were studied on their third to fifth day of life. Twenty had neonatal hyperbilirubinemia with a mean indirect bilirubin value of 16.4 mg/dl, whereas 17 who were used as controls had a mean indirect bilirubin value of 7.8 mg/dl. Urine was collected, and samples were taken for examination of creatinine, lysozyme, sodium, and phosphorus concentration. Urinary sediment, glucose, and amino acid levels were also measured. Serum total and direct bilirubin, creatinine, sodium, and phosphorus measurements were taken at the beginning of urine collection. Calculations were made for creatinine clearance, fractional excretion of sodium (FENa), and tubular reabsorption of renal phosphate per deciliter glomerular filtrate (TP/GFR). The means (+/-1 SD) of creatinine clearance, FENa, and TP/GFR were 27.0 +/- 14.2 ml/min/1.73 m2, 0.53% +/- 0.49%, and 5.72 +/- 1.16 mg/dl GF, respectively, in the hyperbilirubinemic group compared with 21.1 +/- 9.4 ml/min/1.73 m2, 0.4% +/- 0.47%, and 6.01 +/- 0.51 mg/dl GF, respectively, in the controls. No statistically significant differences were found between the groups for any of the examined parameters of either glomerular or tubular function. Neonatal hyperbilirubinemia < 20.8 mg/dl has no detrimental effect on renal function of healthy, breast-fed, full-term newborns, and no modification in the approach regarding renal function is necessary in these babies.

[Group A beta-hemolytic streptococcal sepsis in childhood].

One of two children admitted with septicemia due to group A beta-hemolytic streptococcus died following a very fulminant course. This organism may cause overwhelming disease in newborn infants, as well as in children with diseases which compromise the immune system. Group A streptococcus, though very sensitive to penicillin, can cause severe and rapidly progressive illness even in previously normal children, unless recognized and treated promptly.

Renal functional reserve after acute poststreptococcal glomerulonephritis.

We evaluated renal functional reserve (RFR) in 36 patients aged 5-21 years, who had recovered from an acute episode of poststreptococcal glomerulonephritis (PSGN) 1-16 years previously, without apparent sequelae, as evidenced by normal serum creatinine, blood pressure, and urinary sediment. The control group consisted of 12 children aged 2-12 years with recurrent urinary tract infections or nocturnal enuresis, without active infection or anatomical anomalies. The basal creatinine clearance was similar in the PSGN and control groups: 140.0 +/- 27.4 ml/ min per 1.73 m2 and 142.9 +/- 15.5 ml/min per 1.73 m2, respectively. The RFR in the PSGN group was significantly reduced compared with that of the control group: 18.6 +/- 12.9 ml/min per 1.73 m2 and 41.1 +/- 25.3 ml/min per 1.73 m2, respectively (P < 0.02). In 7 PSGN patients (19.4%), no RFR was found. In 69% of patients who had recovered from PSGN more than 10 years before the protein loading tests, a significantly reduced RFR (less than 10% of baseline) was found. The same degree of reduction in RFR was found in only 26% of patients who had suffered from PSGN less than 10 years ago.

Ultrastructural changes suggestive of focal segmental glomerulosclerosis in atypical minimal change nephrotic syndrome.

In an attempt to recognize early stages of focal segmental glomerulosclerosis (FSGS) in patients with a clinical course suggesting a diagnosis other than minimal change disease (MCD) and normal histology, or minor, nondiagnostic changes on light microscopy (LM), we used a protocol for systematic and extensive electron microscopy (EM) examination of kidney biopsies obtained from such patients. By this method ultrastructural pathology was found in 8 patients. These changes were localized, involving only portions of single glomerular segments. The findings included mild to moderate increase of the mesangial matrix, focal wrinkling of the capillary basement membrane, and early obliteration of the normal architecture of individual capillary loops, as well as electron-dense deposits in a mesangial and subendothelial distribution. Of these 8 patients, 2 are at present in remission without therapy (in 1, following therapy with cyclophosphamide); 3 are in remission on steroid therapy; 1 developed massive proteinuria during pregnancy, after a spontaneous remission lasting almost 2 years; 1 patient advanced to terminal renal failure 3 1/2 years after biopsy; and 1 died of sepsis 1 month after biopsy. We believe that the ultrastructural changes found may represent early or mild FSGS and that the protocol described can add valuable information in clinically worrisome patients in whom renal histology appears normal.

Gaucher's disease and mesangiocapillary glomerulonephritis in childhood--a coincidence?

A 6-year-old boy, presenting with a nephritic syndrome, was diagnosed as suffering from Gaucher's disease (GD) and mesangiocapillary glomerulonephritis (MCGN). GD was suspected because of aseptic necrosis of the femoral heads on X-ray and later confirmed by bone marrow aspiration and a lack of glucocerebrosidase activity in white blood cells; MCGN was documented on renal biopsy. The child was treated with prednisone, dipyridamole and aspirin, and recovered completely clinically. A second biopsy was not performed. The connection between these two rare diseases, and between nephritis and GD in general, is discussed.

Severe tubular resistance to aldosterone in a child with familial juvenile nephronophthisis.

A 9.5-year-old girl, whose early symptoms were polyuria and growth retardation, is described. During the progression of her disease, hyperkalaemia developed out of proportion to the degree of renal insufficiency. Her fractional excretion of sodium increased from 3.3% to 35%, and her fractional excretion of potassium decreased from 55% to 22%. The plasma aldosterone level and plasma renin activity (PRA) were very high--290 ng/ml and 100 ng/dl per hour, respectively (normal range for this age 2.6-20.8 ng/ml and 1.2-2.7 ng/ml per hour, respectively). In an attempt to reduce these hormone levels, an acute and sustained saline load, captopril and peritoneal dialysis were used. Only the sustained saline load normalized the PRA, and only peritoneal dialysis sufficiently suppressed the plasma aldosterone level. Successful renal transplantation normalized both plasma aldosterone and PRA. This girl presents the unusual occurrence of pseudohypoaldosteronism type I, during the course of familial juvenile nephronophthisis.