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Biomedical subjects

E Katzuni

Publications and source records attributed to E Katzuni.

At least 19 recordsLinked to original sources
PubMed1991

[Familial infantile myasthenia gravis].

The several forms of myasthenia gravis that occur in infancy include transient neonatal myasthenia, congenital myasthenia, and familial infantile myasthenia gravis. The latter is inherited in an autosomal recessive pattern. Severe episodes of respiratory distress are frequent in infancy, and are often provoked by mild respiratory infections. 2 sisters with congenital myasthenia gravis are described. Probably 2 other sisters died of the same disease, but no definite diagnosis was made in their cases.

Female↗
PubMed1990

Imerslund syndrome with dolichocephaly.

Imerslund syndrome is a rare autosomal recessive disorder of megaloblastic anemia as a result of selective vitamin B12 malabsorption associated with proteinuria. An Arabic Muslim family is described, with three children who had inherited selective vitamin B12 malabsorption with proteinuria. Dolichocephaly was noted in all the male children of this family in association with congenital megaloblastic anemia and proteinuria. The findings of this anemia are compatible with Imerslund-Gräsbeck syndrome, and coexistence of this syndrome with dolichocephaly in a single family has not been previously reported.

Anemia, Megaloblastic↗
PubMed1990

[Toxocariasis in Emek Israel].

Toxocariasis is a rare zoonotic disease in Israel. It usually affects children under the age of 10. Toxocara canis and Toxocara catis are common parasites among dogs and cats which affect man when he ingests the eggs of these helminths. We describe 3 children with different clinical presentations of the infestation. A 6-year-old boy had pain in the muscles of the limbs and diffuse patches in the right lower lobes on X-ray; a 7-year-old girl presented with a limp; and a 3-year-old boy had abdominal pain and a maculopapular rash covering the whole body. All 3 had eosinophilia. They illustrate the importance of toxocariasis in the differential diagnosis of eosinophilia.

Child↗
PubMed1989

[Hepatitis A and pleural effusion in children].

Pleural effusion associated with hepatitis A is rare and has not previously been reported in children. A 5-year-old girl who recovered from this association of diseases is presented. An immunological mechanism is suggested.

Child, Preschool↗
PubMed1989

Congenital dysgranulopoietic neutropenia in two siblings: clinical, ultrastructural, and in vitro bone marrow culture studies.

Two siblings with congenital neutropenia are reported. The first patient, female, died after Pseudomonas sepsis. The second patient male, suffered from recurrent pyogenic infections, with a more benign course. Bone Marrow (BM) and Peripheral Blood (PB) analysis in the second patient revealed a reduced number of granules and myelin bodies in the PB neutrophils, suggesting a developmental defect of primary and secondary granules. BM promyelocytes were almost normal, but the myelocytes and metamyelocytes showed defective granulogenesis. The BM in vitro granulocyte-macrophage-colony-forming cell (GM-CFC) growth and the PB white blood cells (WBC) granulocyte-macrophage-colony-stimulating factor (GM-CSF) production, which were analyzed in the second patient, showed normal numbers of GM-CFC, with differentiation mostly toward monocytes and a defect in the GM-CSF production capacity. The second patient's PB mononuclear cells or serum did not inhibit normal GM-CFC when added to control BM cells. We suggest that in this specific form of congenital neutropenia, which is probably an autosomal recessive disorder, the abnormal neutrophil granule production and the defective provision of GM-CSF by PB WBC are unique pathognomonic characteristics, possibly associated with the overt neutropenia.

Agranulocytosis↗
PubMed1987

Right ventricular cardiac dysfunction in beta-thalassemia major.

In patients with iron overload associated with severe, transfusion-dependent beta-thalassemia, congestive heart failure develops during the second decade of life. Biventricular heart function was studied by multigated radionuclide angiography in 22 patients with beta-thalassemia major. Six patients were symptomatic. Congestive heart failure developed in five patients at the time of blood transfusions, and one other patient had been treated for multiple ventricular extrasystole. The mean (+/- SD) left ventricular ejection fraction was normal (63.0% +/- 7.6%). Only one patient had a left ventricular ejection fraction under the normal level (less than 50%). The mean (+/- SD) right ventricular fraction (RVEF) was 33.3% +/- 9.4%. In only three patients was the RVEF normal (greater than or equal to 40%); an RVEF under 30% was registered in six patients. We suggest that the early right ventricular dysfunction in patients with beta-thalassemia may be due to pulmonary hypertension secondary to iron overload and iron deposits in the ventricles.

Adolescent↗
PubMed1984

Bone infarction in children with sickle cell disease: early diagnosis and differentiation from osteomyelitis.

An early differential diagnosis between bone infarction and osteomyelitis in sickle cell patients is practically impossible using routine laboratory methods. Twenty radioisotope studies in sickle cell patients during vaso-occlusive crises, were analyzed. A three stage process can be described. In the first stage a decreased uptake can be demonstrated by Tc 99m methylene diphosphonate (MDP) bone scanning. In osteomyelitis, an increased uptake area is usually seen at this early stage, corresponding to increased uptake in Ga-67 citrate scanning. At the second stage, approximately a week later, normal uptake can be seen. Two to four weeks later an area of increased uptake is recorded that corresponds to the healing process, stage three. We recommend therefore Tc 99m MDP bone scanning in the early stages if clinical signs and symptoms suggest a vaso occlusive crisis or osteomyelitis in a sickle cell patient. This study can be followed by a Ga-67 citrate scintigraphy in doubtful cases. Later studies should be used for the assessment of the healing process. Two illustrative case reports are included.

Anemia, Sickle Cell↗
PubMed1984

Further study of the inhibition of premature labor by indomethacin. Part I.

Prematurity still remains one of the unsolved problems in obstetrics and is responsible for a majority of cases of perinatal morbidity and mortality. The use of indomethacin to stop uterine contractions and prevent premature delivery is based on the observation that indomethacin inhibits the release of prostaglandin which is assumed to play a role in the induction and continuation of labor. The effect of indomethacin as an antagonist to prostaglandin was evaluated in a series of 297 women in premature labor. The gestational age at admission varied between 24 and 34 weeks of pregnancy (120 primiparas and 177 multiparas). In 83% of cases there was complete cessation of labor for a period of 1 to 12 weeks, in 10% of cases from 2 to 7 days and in 7% there was no effect. The delay of premature labor for 2 to 7 days allowed the administration of betamethasone in an attempt to improve fetal lung maturity. The total daily dose needed for successful treatment was between 200-300 mg indomethacin. Dilation of cervix beyond 4 cm was associated with successful treatment in 58% compared to 90% if cervix was dilated 3 cm or less. In comparing women with intact membranes to women with ruptured, the success rate in suppressing premature labor was significant; 88% versus 53%. In 49 patients delivery was delayed 11-12 weeks. Fifty-one babies were born in spite of therapy, and of these 15 with birth weights of 700-1500 g suffered from respiratory distress syndrome and died. All the rest (36 premature and 246 mature infants) showed no ill effects related to the treatment.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1984

Further study of the inhibition of premature labor by indomethacin. Part II double-blind study.

In the etiology of premature labor prostaglandins fulfill a significant role. It is known that indomethacin is a strong inhibitor of prostaglandin synthesis. The effect of indomethacin on premature labor was studied in a prospective randomized double-blind study in 36 patients. Eighteen patients received indomethacin and eighteen received placebo. 200-300 mg of indomethacin was the total dosage in a 24 hours period. The activity of the uterus was monitored with a cardiotocograph. The mean duration of pregnancy and the mean birth weight in indomethacin group (36.4 weeks, 2833 g) were both significantly greater (p less than 0.001) than that in placebo group (31.2 weeks, 2028 g). In the indomethacin group 3 children weighted less than 2500 g compared with 14 in placebo group. In 15 of 18 indomethacin treated patients (83.3%) premature labor was arrested after indomethacin treatment compared with 4 of 18 in the placebo group (22.2%). The indomethacin group had a mean 1 minute APGAR score of 9.3 +/- 0.2 whereas the placebo group showed a score of 7.8 +/- 0.5 (p less than 0.01). Three infants died from respiratory distress syndrome; one in the indomethacin group (1810 g) and two in the placebo group (600 and 1450 g). Autopsies in the infants demonstrated a typical picture of pulmonary atelectasis and hyaline membranes. There was no evidence of premature closure of the ductus arteriosus or pulmonary hypertension. 2 mothers in the indomethacin group suffered minor discomfort i.e. nausea, vomiting and vertigo.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1984

Serum immunoglobulin levels in children after splenectomy. A prospective study.

Serum immunoglobulin concentrations (IgG, IgA, and IgM) were measured in 34 children before and after splenectomy. Splenectomy was performed either after trauma or for an underlying hematologic disorder. In the patients with a hematologic disorder, we found a significant decrease in the IgM level after splenectomy. The IgM concentration remained low for a two-year period. Those patients who were "afebrile" during the study period had the lowest IgM levels. No significant changes were observed in the IgM levels in the group that underwent a splenectomy because of trauma. No significant changes were found in IgG or IgA levels in either group. A low IgM level in patients with hematologic disorders may have contributed to a defect in opsonization and to a high incidence of overwhelming infection soon after splenectomy in this group. In the group that underwent a splenectomy because of trauma, the normal IgM level may have protected them and contributed to the lower incidence of infection in this group.

Adolescent↗