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We reviewed epidemiological data and outcome of 134 children with abdominal tumors (AT) among a total of 460 children with solid tumors (ST) seen between 1971-1989. There were no significant differences in ethnic distribution or sex ratio between children with AT and ST. The mean age was younger in those with AT than with ST (4.7 vs 7.2 years). The 3 major histologic AT types were Burkitt lymphoma, Wilms' tumor and neuroblastoma, with a relative increase after 1978 in those with abdominal Burkitt lymphoma. A comparison of our therapeutic results in AT treated before and after 1981 shows significant improvement in the actuarial survival at 5 years: 71.1% of those treated after 1981 were then alive with no evidence of disease, vs only 32.8% of those treated before 1981. Since 1981 the actuarial survival at 5 years in our children with Wilms' tumor and abdominal Burkitt lymphoma is about 80%.

A 3-month old female infant was transferred from another hospital where she had been hospitalized from the age of 1 month for protracted secretory diarrhea. The diarrhea had begun at birth and was unresponsive to various therapeutic formulas and to total parenteral nutrition (TPN). The parents were consanguineous. There were 6 normal siblings, while 3 siblings had died in infancy, including a sister who had succumbed to protracted diarrhea at the age of 6 months. In our patient duodenal biopsy showed flattening of villi and proliferation of mononuclear cells in the lamina propria. Specific circulating IgG antibodies against gut epithelium were found, as well as thyroglobulin antibodies. Repeated trials of oral feeding were unsuccessful and TPN was required for 8 months. Complications included septicemia, osteomyelitis and acute renal failure. Therapeutic trials with intravenous hydrocortisone, zinc sulphate and metronidazole were unsuccessful and the infant died at the age of 11 months. Intestinal tissue taken postmortem showed nearly absolute flattening of intestinal villi. This is the first report in Israel of intractable infantile diarrhea due to autoantibodies to intestinal epithelium.

The development of optical fibers capable of transmitting laser energy has encouraged the experimental use of laser irradiation for the treatment of acquired cardiovascular disorders. One of the key questions is which combination of laser source, energy parameters, and transmitting fiberoptic would be best suited for intravascular use. In most experiments argon, neodymium-YAG, and excimer lasers, coupled to suitable optical fibers, have been used. We now describe the use of a carbon dioxide fiberoptic laser catheter for the creation of an atrial septal defect. Silver halide infrared transmitting fibers were inserted into standard 6 French cardiovascular catheters. This laser catheter system, capable of transmitting several watts of pulsed CO2 laser energy, was initially used to create atrial septal defects in isolated dog hearts to determine the best energy parameters. Atrial septostomy was later performed successfully in four of five anesthetized dogs. The thermal damage extended 50 to 60 micron beyond the "holes" created by the laser irradiation in the interatrial septum. Thus, pulsed CO2 laser irradiation, delivered through optical fibers, can create an atrial septal defect.

An infant, first admitted at the age of 5 months with diarrhea (which was adequately treated with formula), was readmitted at the age of 1 year with poor weight gain, steatorrhea, and hepatomegaly. Liver function test results were compatible with cholestatic jaundice, and hepatobiliary scintigraphy visualized dilated bile ducts and evidence of hepatocellular disease. Exploratory laparotomy, liver biopsy, and cholangiography disclosed pathologic and roentgenographic findings of primary sclerosing cholangitis (PSC). The patient proved to be immunodeficient, pointing to the possible pathogenetic role of immunodeficiency in causing PSC in some patients. It is important to look for the disease in immunodeficient children and in patients with ulcerative colitis, and to consider PSC in the differential diagnosis of cholestatic jaundice.

The assumption that fresh cow's milk may have a direct effect on jejunal mucosal macromolecular permeability was tested in 14-day-old suckling guinea pigs, by in situ luminal perfusion of a proximal jejunal segment with horseradish peroxidase administered either in 0.9% NaCl or in cow's milk. For positive control, a group of guinea pigs previously sensitized to beta-lactoglobulin was similarly perfused with horseradish peroxidase in 0.9% NaCl or in cow's milk at 14 days of age. A segment of the wall of the perfused jejunal loop was processed for peroxidase staining and for examination of the extent and routes of macromolecular absorption by light and electron microscopy. Mucosal exposure to cow's milk was associated with penetration of tracer material across the jejunal epithelium only in the lactoglobulin-sensitized guinea pigs. In guinea pigs with a first-time exposure to cow's milk, no entry of tracer material beyond the brush border surface of the enterocytes was seen. Thus, whereas we could observe absorption of bystander antigens during antigenic challenge of the mucosa in sensitized animals, we found no evidence of a presumptive "permeability factor" in cow's milk that can disrupt the mucosal macromolecular barrier by an acute direct action.

To clarify the possible role of renal prostaglandins (PGs) in the phenomenon of bile-induced diuresis in the dog, we studied the effect of in situ unilateral infusion of bile on kidney function and PGs excretion, before and after PG-synthetase inhibitor administration in anesthetized dogs. The contralateral intact kidney served as control. In the first group of 6 dogs, infusion of bile diluted 1:20 resulted in a significant increase in urinary flow (117%; p less than 0.05), sodium (61%; p less than 0.01), potassium (26%; p less than 0.05), PGE2 (240%; p less than 0.05) and PGF2 alpha (137%; p less than 0.05) excretion rates. Further significant increases in urinary flow, sodium and PGE2 excretion rates were noted with infusion of bile diluted 1:10. All parameters returned to basal levels upon cessation of bile infusion. Significant linear correlation coefficients (p less than 0.005) were found between PGE2 excretion rates and urinary flow (r = 0.72), sodium (r = 0.91) and potassium (r = 0.88) excretion rates. In a second group of 6 dogs, intravenous administration of PG-synthetase inhibitor abolished the increase in renal PGs excretion and the increments in the rates of urinary flow and solute excretion in response to bile infusion. These findings support the notion that the acute diuretic and natriuretic effect of bile, and presumably that of cholemia is mediated, in part, through stimulation of renal PGs synthesis.

A 6-month-old full-term infant had severe anemia and neutropenia. The patient was being fed cow's milk and a diet of corn flour. Thorough investigation revealed low serum iron concentration, severe hypocupremia, low ceruloplasmin, retardation of bone age, and metaphysial irregularities and spurring. Bone marrow aspirate revealed cytoplasmic vacuolization in precursors of the erythroid and myeloid series and ringed sideroblasts. Therapy with oral iron, folic acid, and vitamin C was futile. Administration of copper sulfate resulted in brisk increase in neutrophils and reticulocytes. The child maintained normal levels of hemoglobin, neutrophils, serum copper and ceruloplasmin, and serum iron one year after copper therapy was discontinued. The probable role of unrecognized copper deficiency in causing anemia in infants more than 6 months of age is discussed, and the importance of serum copper examination in refractory iron deficiency anemia and neutropenia is stressed. To the best of our knowledge, no such case has previously been described in the literature.

Hematogenous Candida osteomyelitis is described in a two-month-old infant, as a complication of Candida septicemia which occurred during a parenteral hyperalimentation regimen. Treatment with flucytosine led to full recovery. The scarcity of reports on hematogenous Candida osteomyelitis in infants, despite an increased incidence of Candida septicemia, and the non-specific symptomatology which the disease may assume in this age group, indicate the need for greater awareness of this complication. Flucytosine is an antifungal drug which can also be given by mouth and carries relatively low toxicity. We found flucytosine to be extremely effective in the treatment of disseminated infantile Candida osteomyelitis.

In view of the known toxicity of aluminum, we studied the effects of CaCO3 as an alternative phosphate binder in 12 chronic renal failure (CRF) children during 152 patient-months. Mean (+/- SD) serum creatinine concentration rose during that period from 3.7 +/- 1.8 to 5.1 +/- 3.0 mg/dl. 8 patients received CaCO3 from the start, and 4 were switched from A1(OH)3 after 2 months of interruption. In addition to CaCO3 (0.1-0.3 mg/kg BW) all patients received NaHCO3, and all but two received 1 alpha-hydroxyvitamin D3 [1 alpha(OH)D3] or dihydrotachysterol (DHT). Urine and blood variables were checked every 4-6 weeks and medication dosages were adjusted accordingly, aiming to keep serum Ca at 10.4-10.8 mg/dl, serum Pi at 3.5-5.5 mg/dl, and serum HCO-3 above 18 mEq/l. Bone X-rays were obtained every 6-9 months. With treatment, mean serum Ca increased from 8.9 +/- 0.7 to 10.3 +/- 0.4 mg/dl (p less than 0.01), serum Pi decreased from 6.3 +/- 0.9 to 4.2 +/- 0.5 mg/dl (p less than 0.01), and the mean Ca X P product decreased slightly and insignificantly. Mean serum alkaline phosphatase levels decreased significantly from 486 +/- 251 to 168 +/- 28 IU (p less than 0.01). Bone X-rays at the end of the study showed either healing of renal osteodystrophy or its prevention. Only one episode of mild hypercalcemia (serum Ca 11.7 mg/dl) was observed in 1 patient, but his Ca X P product remained low.(ABSTRACT TRUNCATED AT 250 WORDS)

The value of long-term prophylactic treatment of chronic granulomatous disease (CGD) in childhood cannot be established with certainty, as controlled studies are not available. We describe a boy, presently 15 years old, who suffered from CGD since early infancy. By the clinical, laboratory and genetic features, this case appeared to be a new variant of CGD, combining elements of the "childhood" type with others that characterize the "adult" type of the syndrome. For years, the patient had been almost continuously ill and needed frequent and prolonged hospitalizations because of severe bacterial infections. At age 13 years, long-term prophylactic treatment with trimethoprim-sulfamethoxazole (TMP-SMX) was instituted. With this regimen, the patient was maintained practically infection-free, relapsing only in those instances when he neglected to comply with the prophylactic regimen for 1 to 2 weeks. Thus, the patient served as his own control in demonstrating the efficacy of antimicrobial prophylaxis in CGD. The rationale for employing TMP-SMX for the prophylactic regimen is discussed.

We studied the acute effects of phototherapy (PT) on bile flow and on the biliary excretion of bilirubin pigments and of bile salts in male homozygous Gunn rats (120-150 g). 13 rats received PT and 10 rats were kept in the dark. Bile was collected by cannulation of the common bile duct at hourly intervals from 1 h prior to PT till after 4 h of 'lights on'. Before treatment, all values were similar in both groups. After 4 h of lights on, mean plasma bilirubin fell from 145.3 +/- 4.3 to 99.2 +/- 2.7 mumol/l (p less than 0.01) in the PT rats, but did not change in the controls. During the lights-on period, PT rats had a significantly higher hourly bile volume, and a higher excretion of biliary bilirubin and bile salts than the controls (p less than 0.005). Over the total 4-hour lights-on period, the PT group had a higher mean output of bile than the controls (0.93 +/- 0.17 vs. 1.52 +/- 0.34 ml/4 h; p less than 0.005) and an increased excretion of bilirubin (0.08 +/- 0.016 vs. 0.148 +/- 0.01 mumol/4 h; p less than 0.005) and bile salts (35.1 +/- 3.7 vs. 55.2 +/- 12.5 mumol/4 h; p less than 0.005). The results show that PT of the Gunn rat is associated with a rise in bile flow and with an increased excretion of bile salts, in addition to an increased biliary bilirubin output.

Acute hepatic failure (AHF) combined with acute renal failure (ARF) is a well-known complication of open-heart surgery in adults. The occurrence of this complication in two children after open-heart surgery for correction of congenital heart disease is reported. Hypotension occurred during the operation and was treated by catecholamine vasopressors. AHF set in during the postoperative course; it was manifested by impaired consciousness, hypoglycemia, hyperbilirubinemia, hyperammonemia, elevated liver enzymes and prolongation of the prothrombin time with failure of hemostasis. ARF also developed in both children. One of the patients survived the acute episode of hepatic failure. The importance of early diagnosis, routine close monitoring, and appropriate selection of vasopressors is emphasized.

Antigliadin antibodies (AGAs) were studied in sera from 190 patients divided into five clinical groups. Group I included 28 sera from children with newly diagnosed celiac disease on a normal diet. Group II consisted of 43 sera from children with celiac disease who were fed a gluten-free diet (GFD). Group III included 25 sera from children with celiac disease who had been in remission but exposed to a gluten-containing diet (GCD). Group IV consisted of 46 sera from children with chronic diarrheal disorders other than celiac disease. Group V included 43 sera from healthy children. The observed p values proved that (a) mean titer levels of AGAs in Groups I and III were significantly higher than the mean values for all other groups (p less than 0.001), and (b) the mean titer level of AGAs in Group II was significantly higher than the mean values for Groups IV and V. A good correlation between the AGA titers and the morphology of the duodenal mucosa was found in children with celiac disease. The examination of IgG AGAs by the immunofluorescence technique used in our study appears to be a useful tool in the follow-up of individual patients to determine adherence to a GFD.

We report a 10-year-old boy who has Fanconi's anemia and was admitted because of acute hemiplegia of the left side. Internal carotid arteriography disclosed a moyamoya cerebrovascular pattern on the right side. Although this condition may be acquired, it is suggested that in this case the moyamoya might pertain to the array of congenital malformations associated with Fanconi's anemia. Intracranial accidents occurring in Fanconi's anemia are generally ascribed to the bleeding tendency; however, the possibility of an underlying vascular anomaly should also be considered.

A 2 1/2-month old preterm infant had failure to gain weight with high caloric intake, and had generalized persistent dermatitis and mild diarrhea. The patient was being fed zinc-supplemented cow's-milk-based formula (Osterfeed). High caloric intake of 8 weeks' and topical treatment of 11 weeks' duration were futile. A thorough investigation revealed low serum zinc concentration. Administration of zinc sulfate 150 mg/day resulted in brisk weight gain and complete clearing of skin lesions. The infant maintained normal levels of zinc 4 months after zinc therapy was discontinued, while being fed unmodified cow's milk and a diet of corn flour. The probability that zinc-supplemented formulas do not meet the high zinc requirements of premature infants is raised. The importance of plasma or serum zinc examination in preterm infants with slow growth velocity or failure to gain weight despite adequate caloric intake, with or without skin lesions and diarrhea, is emphasized.

We describe five children in whom symptoms and signs fully consistent with a diagnosis of acute rheumatic fever arose in association with infection by Mycoplasma pneumoniae. With the decline of streptococcal disease as a causative agent of acute rheumatic fever, other etiologic possibilities capable of reproducing the clinical syndrome - especially Mycoplasma pneumoniae - should be considered, as this may have therapeutic and prognostic implications.

Calcium and creatinine concentrations were analyzed in urine samples of 42 chronically institutionalized bedridden children, with neurologic disorders. Their ages ranged from 2 to 16 (mean 8.7) years. Hypercalciuria, defined as UCa/UCr ratio greater than 0.21, was recognized in 18 children (42.8%), the mean UCa/UCr ratio of this group was 0.40 +/- 0.18. Twenty-four children were normocalciuric, with a mean UCa/UCr ratio of 0.08 +/- 0.03. There were no significant differences between the two groups with regard to age, sex, length of institutionalization, the basic neurologic disorder, diet, anticonvulsive medications, exposure to sunlight and weekly hours of physiotherapy. Age- and sex-matched percentiles for fat and muscle areas were similar in both groups. Seven limb fractures had occurred in the hypercalciuric group during the last three years, but only in one of the normocalciuric patients (p less than 0.02). The hypercalciuric children were treated with a hydrochlorothiazide-amiloride preparation for three weeks, which resulted in reduction of their mean UCa/UCr ratio by 57.7%, to 0.17 +/- 0.13 (p less than 0.005); only four children remained hypercalciuric. We conclude that resorptive hypercalciuria is common among chronically immobilized bedridden children. Hypercalciuria in such children should be specifically looked for, because of its association with deranged bone metabolism and increased frequency of limb fractures. Treatment with thiazides was found to be efficient in reducing urinary calcium excretion in our hypercalciuric patients. This therapeutic modality for the reduction of morbidity in bedridden chronically immobilized children should be further explored.

The case of a newborn female in congestive heart failure due to a cerebral A-V fistula is presented. The cardiac manifestations in the presence of this condition are described, and the diagnostic importance of auscultation of the skull is stressed. Because of the precarious condition of most of these neonates, diagnostic procedures should be minimized if the clinical picture is compatible with this diagnosis, and cerebral angiography should be performed before any heart studies are undertaken.

Erythrocyte transketolase activity and thiamin pyrophosphate effect were examined in the offspring of streptozotocin-diabetic rats. Thiamin reserve was found to be significantly reduced in litters of untreated diabetic rats as compared to control and to insulin-treated diabetic rats. Supplementation of the untreated diabetic dams throughout pregnancy with oral thiamin was associated with a significantly improved thiamin status of the litters. We conclude that, due to enhanced fetal glucose turnover during diabetic gestation, a fetal thiamin deficiency state may evolve; this condition can be remedied with maternal thiamin supplementation.

Tuberous sclerosis is an inherited neurocutaneous disorder characterized by seizures, mental retardation, cutaneous lesions and visceral hamartomas. We describe a 17-year-old boy in whom polycystic kidneys of the adult type were fortuitously detected on routine check-up. The patient enjoyed good health and had no evidence of renal dysfunction. Closer scrutiny of his past history and his physical and laboratory findings disclosed that he had tuberous sclerosis. Our case adds to the scant reported experience with the association of tuberous sclerosis and adult-type polycystic kidneys, and suggests that a search of additional manifestations of tuberous sclerosis is warranted in children in whom adult-type polycystic renal disease is detected.

We studied the effects of increased circulating levels of bile salts on jejunal mucosal function in dogs. In situ luminal perfusion of a 30-cm proximal jejunal segment was performed while deoxycholate, cholate, taurodeoxycholate, or taurocholate solutions were directly added to the mesenteric arterial supply, reaching the intestinal wall at successive concentrations of 5, 8, 12, and 22 mumol/L. The transport rates of water, sodium, glucose, fructose, glycine, and lysine were measured. The mucosa of the experimental loop was assayed for ATPase activity, and examined by light and electron microscopy. Deoxycholate at 8 microM in the blood supply of the perfused jejunal segment was associated with a significant (p less than 0.02) reduction in the absorption rates of water, sodium, glucose, and glycine, and inhibition of mucosal Na+, K+-ATPase. The absorption of fructose and lysine, and brush border enzyme activities, were not affected. Cholate had a similar effect at 12 microM. There were no obvious histological alterations, but electron microscopy showed swelling of mitochondria in the enterocytes. The reduction in mucosal transport, the inhibition of mucosal Na+, K+-ATPase, and the mitochondrial swelling were reversed after discontinuation of the bile salt infusion. The taurine conjugates at 22 microM depressed transport of water and sodium only, and did not inhibit Na+, K+-ATPase. Our study indicates that increased circulating concentrations of unconjugated bile salts, particularly deoxycholate, may impair Na+, K+-ATPase-related jejunal mucosal function.

A prospective blind study comparing the findings of ultrasonography, intravenous pyelography, and voiding cystourethrography was conducted on 81 patients to examine the place of ultrasonography in the initial radiologic evaluation of children with urinary tract infection. The patients' mean age was 4.8 years; 15 were male. Forty-eight were inpatients (mean age, 3.2 years) and 33 were outpatients (mean age 7.2 years). In 29 patients (35.8%) abnormality of the urinary system was detected by one or more of the three imaging procedures; 21 were inpatients and eight were outpatients. The most frequent finding was vesicoureteral reflux, occurring in 62.1% of the pathologic cases. The findings at ultrasonography correlated well with those of intravenous pyelography in 73 of the 81 studies (90.1%), but they failed to demonstrate double collecting systems and several of the minor changes. However, ultrasonography in combination with cystourethrography identified all patients who had abnormal urinary systems, except for two children with negligible findings. Moreover, ultrasonography and cystourethrography together identified all 11 patients, nine of them inpatients, in whom surgical treatment was indicated. It is concluded that ultrasonography can successfully replace intravenous pyelography as a screening imaging procedure for the urinary system, but because of the superiority of intravenous pyelography in the detection of some types of lesions, intravenous pyelography will be required whenever ultrasonography or cystourethrography results are abnormal.(ABSTRACT TRUNCATED AT 250 WORDS)