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Biomedical subjects

Y Naveh

Publications and source records attributed to Y Naveh.

At least 55 records · Page 3Linked to original sources
PubMed1984

Serum IgG antibodies to gliadin in children with celiac disease as measured by an immunofluorescence method.

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.

Animals↗
PubMed1984

Progressive diaphyseal dysplasia: genetics and clinical and radiologic manifestations.

Progressive diaphyseal dysplasia was found in a three-generation family including 13 affected individuals, the largest family reported to date. Our study confirms that progressive diaphyseal dysplasia, also known as Engelmann's or Camurati-Engelmann disease, is an autosomal dominant disorder with variable osseous and muscular manifestations. Disease distribution among patients, within a given patient, or even in individual bones is unpredictable. The femur is the most commonly and severely affected bone and hence most useful for radiographic screening of possible patients. Radiographs provide a meaningful assessment of disease activity and extent. The severity of symptoms is generally proportionate to severity of involvement shown by roentgenography. Exophthalmos due to osteosclerotic dysplasia of the skull occurred in more than half of the patients with progressive diaphyseal dysplasia. Twelve-year follow-up of this family, with affected individuals ranging in age from 6 months to 12 years, indicates that progressive diaphyseal dysplasia may progress or become quiescent and be remarkably inactive despite advanced osteosclerosis and structural deformity.

Adolescent↗
PubMed1984

Vitamin K deficiency presenting with hemarthrosis.

A breast-fed 25-day-old infant was hospitalized because of swelling and tenderness of the left leg, developed after mild rotary motion of the leg by his brother. Radiographic examination showed widening of the left articular hip joint space. On the day of admission, a presumptive diagnosis of septic arthritis was entertained, and antibiotic therapy was instituted. Following profuse bleeding from sites of skin punctures, coagulation studies were performed. Prothrombin time and partial thromboplastin time were prolonged. Administration of phylloquinone (vitamin K1) resulted in rapid normalization of coagulation. Differential diagnosis between hemarthrosis resulting from vitamin K deficiency and septic arthritis with disseminated intravascular coagulation is a matter of great importance in such patients.

Hemarthrosis↗
PubMed1983

Primary sclerosing cholangitis associated with immunodeficiency.

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.

Bile Ducts↗
PubMed1983

Papular acrodermatitis with cytomegalovirus hepatitis.

The syndrome of papular acrodermatitis of childhood with hepatitis (Gianotti-Crosti syndrome) is classically considered to be associated with hepatitis B surface antigen (HBsAg) infection. We report an infant with the syndrome, but with serological evidence of infection by cytomegalovirus.

Acrodermatitis↗
PubMed1981

Electron microscopic studies in lipid storage disease.

Biopsy and autopsy specimens from patients with Gaucher's disease and Niemann-Pick disease were studied ultrastructurally. The morphological features were compared with those obtained from paraffin-embedded tissues after reprocessing the tissue for electron microscopy. With routine glutaraldehyde-osmium fixation, both surgical and autopsy specimens from Gaucher's disease showed cytoplasmic bodies containing elongated tubular structures with a translucent appearance. Tissue processed from paraffin-embedded blocks showed moderate preservation of the cytoplasmic glucocerebroside-containing bodies in Gaucher's disease. Routine fixation for electron microscopy of surgical of surgical and autopsy specimens from Niemann-Pick disease showed characteristic cytoplasmic vacuolated formations which were frequently multilaminated. These sphingomyelin-containing vacuoles were poorly preserved in tissue reprocessed from paraffin blocks.

Adult↗
PubMed1981

Copper deficiency with cow's milk diet.

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.

Anemia, Hypochromic↗
PubMed1980

Foreign body in the airway. A review of 200 cases.

Two hundred children aged 6 months to 12 years were admitted to the hospital with a foreign body in the airway. Food materials constituted 93% of all foreign bodies. A positive history of foreign body aspiration was obtained in 88% of the cases. The most common symptoms of laryngotracheal foreign bodies were dyspnea, cough, and stridor, whereas those of bronchial foreign bodies were cough, decreased air entry, wheezing, and dyspnea. Chest fluoroscopy contributed to the diagnosis in 90% of the cases of bronchial foreign bodies, but only 32% of those in the laryngotracheal area. Of the foreign bodies removed, 98 1/2% were done by laryngoscopy, tracheoscopy, and/or bronchoscopy. Complications were involved in 6% of the cases, including one death. History of recurrent intractable pneumonia should make one consider a foreign body in the airway. Removal of one foreign body does not exclude the existence of another. The condition may be fatal; thus immediate removal of the foreign body is mandatory.

Airway Obstruction↗
PubMed1980

Intrafamilial meningococcal meningitis.

Two couples of sibs presented with meningococcal meningitis. In both families, immediate preventive measures were not taken due to a negative cerebrospinal fluid (CSF) smear in the first case from Family A, and a negative CSF smear and culture in the first case from Family B. All the children were treated successfully. Our recommendations are: a) contacts of patients with pyogenic meningitis should have close clinical surveillance, and b) contacts of meningococcal infection should have nasopharyngeal cultures and should be given a prophylactic antibiotic such as rifampicin or minocycline.

Adolescent↗
PubMed1980

The value of cardiac catheterization and cineangiography in infantile lobar emphysema.

Lobar emphysema is an uncommon cause of respiratory distress in infancy. Congenital heart disease is seen in about 20% of the patients with infantile (congenital) lobar emphysema. We described six infants with lobar emphysema. In three of them a congenital heart disease was demonstrated by cardiac catheterization and cineangiography; two had a tetralogy of Fallot with right aortic arch and the third infant a ventricular septal defect. The pulmonary angiography showed stretching of the arteries with very poor filling of the peripheral arteries and a characteristic smaller pulmonary vein in the affected lobe. In all the six patients the pulmonary artery pressure was normal. All the patients underwent lobectomy with good results. We feel that a preoperative cardiac catheterization and cineangiography is of value in this very sick group of infants.

Cardiac Catheterization↗
PubMed1980

Congenital hepatic fibrosis with congenital heart disease. A family study with ultrastructural features of the liver.

A family with congenital hepatic fibrosis (CHF) and congenital heart disease (CHD) is presented. The consanguineous healthy parents gave birth to 12 children of whom 10 survived. One son had CHF and CHD, one daughter had CHF and a second daughter had CHD. Three other siblings probably had small a ventricular septal defect and another one probably had mild pulmonary valve stenosis. Development of portal hypertension and hypersplenism necessitated performing shunt operation on both siblings suffering from congenital hepatic fibrosis. Ultrastructural findings were giant mitochondria with large laminar inclusions in hepatocytes, and excess of villi and whorls of membranes and collagen fibrils between hepatocytes.

Adolescent↗