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Biomedical subjects

A Szeinberg

Publications and source records attributed to A Szeinberg.

At least 19 recordsLinked to original sources
PubMed1991

[Pneumothorax in cystic fibrosis: intrapleural instillation of Atabrine].

Intrapleural instillation of quinacrine HC1 (Atabrine) was used to treat spontaneous pneumothorax in 2 young men, aged 26 and 36 years, respectively, with advanced pulmonary disease due to cystic fibrosis. Pneumothorax did not recur until 1 year later in 1 case and 2 years later in the other. This mode of therapy for pneumothorax provides a valuable alternative to surgery for the patient with cystic fibrosis, severe lung disease and marginal pulmonary reserve. Quinacrine sclerosis should be considered for management of pneumothorax in cystic fibrosis because of the high rate of recurrence. However, caution should be exercised since heart-lung transplantation cannot be performed after pleural sclerosis.

Adult↗
PubMed1989

Hypercapnia in relation to pulmonary function in Duchenne muscular dystrophy.

Arterialized blood gases were analyzed in 143 patients with Duchenne muscular dystrophy (DMD) to assess the relationship between forced vital capacity (FVC) and hypercapnia. The majority of patients studied had PaCO2 values in the low or normal range. Only six older patients had hypercapnia (PaCO2 greater than or equal to 45 mm Hg), and all these patients had FVC values less than or equal to 40% predicted. We conclude that hypercapnic respiratory failure occurs as a late preterminal event in DMD.

Blood Gas Analysis↗
PubMed1988

Assessment of maximal expiratory pressure in healthy adults.

Maximal static expiratory pressure developed at the mouth (PEmax) provides a useful clinical index of expiratory muscle function; however, the range of normal values among laboratories shows considerable variation. We examined the hypothesis that the wide variability could be attributable to the differences in technique among laboratories. We measured PEmax at functional residual capacity (PEmax FRC) in 28 healthy subjects using the following five techniques: 1) using a scuba-type mouthpiece with the cheeks supported by the hands ("hands on"), 2) without supporting the cheeks ("no hands"), 3) using a rigid, circular mouthpiece (2.8 cm ID, "tube"), 4) using the scuba-type mouthpiece but with the cheeks supported by an observer ("other hands"), and 5) using a large-bore circular mouthpiece (4.1 cm ID, "new tube"). Mean PEmax FRC obtained with hands on was significantly higher than no-hands and tube methods. PEmax FRC values obtained by the other-hands and new-tube maneuvers were similar to the hands-on maneuver. We conclude that the technique used to measure PEmax FRC can significantly affect the results and suggest that it should be measured using a large-bore circular mouthpiece or a scuba-diving mouthpiece with the cheeks supported.

Adult↗
PubMed1988

Cough capacity in patients with muscular dystrophy.

Cough capacity was evaluated in 22 patients with muscular dystrophy (MD) using subjective cough assessment, cough flow-volume curves, maximum expiratory pressures (MEP), forced vital capacity (FVC), and peak expiratory flow rates (PEFR). In ten of the 22 patients transients of peak flow were generated during cough flow-volume maneuvers, indicating dynamic compression of the airways, which is considered important in the physiology of an efficient cough. Patients who could not generate peak flow transients had significantly reduced PEFR, FVC, and MEP values. Measurement of MEP was the most sensitive predictor of flow transient production during coughing; all of the patients who exhibited transients had MEP values of above 60 cmH2O, whereas the highest value of MEP recorded in patients without transients was 45 cmH2O. Three of the 12 patients who were unable to generate flow transients were considered to have an adequate cough by subjective assessment. We concluded that the measurement of MEP is extremely useful for assessment of cough strength in patients with MD.

Adolescent↗
PubMed1987

Genetic polymorphism of delta-aminolevulinate dehydrase in several population groups in Israel.

The genetic polymorphism of red cell delta aminolevulinate dehydrase (ALADH) has been investigated in several population groups in Israel: Ashkenazi Jews, non-Ashkenazi Jews from North Africa, Egypt, Turkey, Iraq, Iran, Yemen and the Balkans, and Arabs. The distribution of the ALADH genes was not homogeneous (chi 2 = 36.83; d.f. = 8; p less than 0.0005). A significantly higher frequency of the ALADH2 gene was observed among the Ashkenazi Jews (0.2021) than among the non-Ashkenazi Jews and Arabs (gene frequencies 0.0825-0.1290) or all the other population samples so far studied (Liberia, Japan, Italy, Germany and Spain).

Erythrocytes↗
PubMed1986

Studies on the effect of intralipid on human monocyte functions in vitro.

Intralipid (IL) particles were ingested by human monocytes in culture. These particles remained within the cells for periods of up to 3 weeks in culture. The presence of IL particles did not alter the normal secretion of lysozyme or prostaglandin E2 (PGE2), nor was the normal process of biochemical activation altered, as evidenced by the expected increase in PGE2 secretion and superoxide anion production by stimulated monocytes. Phagocytosis of zymosan particles was increased in monocytes that had ingested IL. These results indicate that in this in vitro system essential monocyte functions were not altered following ingestion of IL.

Dinoprostone↗
PubMed1985

Maximal inspiratory and expiratory pressures are reduced in hyperinflated, malnourished, young adult male patients with cystic fibrosis.

We measured maximal inspiratory and expiratory pressures (MIP and MEP, respectively) in 23 male patients with cystic fibrosis (CF), 16 to 35 yr of age (22.1 +/- 3.7), and in a control group of 33 male volunteers, 17 to 39 yr of age (22.5 +/- 6.8), to evaluate the effects of chronic hyperinflation and malnutrition on MIP and MEP in the patients with CF. Routine pulmonary function tests and skeletal muscle indexes, such as the force generated by the adductor pollicis muscle with supramaximal ulnar nerve stimulation at a frequency of 10 Hz as percentage of force at 100 Hz (F10/100) and midarm muscle circumference as a percentage of predicted (MAMC), were also measured in the patients with CF. Severe hyperinflation in this study was defined as a ratio of residual volume to total lung capacity above 50% and malnutrition as a ratio of actual weight to the ideal weight for the patient's age and height of 90% or less. The severely hyperinflated subgroup of patients with CF had significantly reduced MIP values in comparison with those in the other patients with CF. The malnourished subgroup, which was also severely hyperinflated, differed from the well-nourished one in both skeletal muscle indexes; MAMC was reduced, whereas F10/100 was elevated, and respiratory muscle pressure generation, MIP, and MEP were reduced. We conclude that patients with CF who are malnourished and/or severely hyperinflated have reduced maximal respiratory pressures.

Adolescent↗
PubMed1985

Malignant phenylketonuria due to defective synthesis of dihydrobiopterin.

A defect in the synthesis of dihydrobiopterin was detected in an Arab girl, ascertained through high blood phenylalanine level on neonatal screening. An oral loading test with tetrahydrobiopterin (BH4) caused a significant fall in her blood phenylalanine and a rise in tyrosine concentrations. Her blood biopterin levels were low. In urine and cerebrospinal fluid (CSF) very high neopterin and low biopterin levels were observed. A deficiency of metabolites of neurotransmitters, serotonin and dopamine, was observed in CSF and urine. The patient was given replacement therapy of BH4, 5-hydroxytryptophan, and L-dopa with carbidopa starting from the age of 16 to 18 weeks. On this treatment the blood phenylalanine levels dropped to the desired range, while in urine and CSF a satisfactory rise of neurotransmitter metabolites was observed. In spite of this biochemical control, the patient developed neurological symptoms with myoclonic jerks and changes in muscle tone and presented severe cerebral damage with mental retardation. She died suddenly at the age of 38 weeks.

5-Hydroxytryptophan↗
PubMed1982

alpha 1-antitrypsin deficiency in Israeli children: a five-year survey.

In order to elucidate the importance of alpha 1-antitrypsin (AAT) deficiency as a cause of liver disease in children in Israel, we conducted a retrospective 5-yr study. The screening of 51 liver biopsies for the presence of AAT inclusion bodies was performed using the immunoperoxidase technique. Serum AAT concentrations of 300 pediatric patients determined during the same period were reviewed. No case of AAT deficiency was detected. We conclude that AAT deficiency is rare in Israel.

Adolescent↗
PubMed1982

Early blood sampling in neonatal programs for the detection of phenylketonuria.

A prospective study of blood phenylalanine levels during the early days of life of infants in 11 families with classical phenylketonuria (PKU) and 2 families with variant hyperphenylalaninemia has been performed. Four affected children were detected. In 3 infants with PKU, blood specimens obtained on the second day demonstrated levels of 8--10 mg% (480--600 micrometers/l) phenylalanine. In one infant with a variant type of abnormality (high phenylalanine requirement) only slightly elevated levels of 2--4 mg% (120--240 micrometers/l) were observed on the second to fourth days, though later very high levels were reached, warranting dietary treatment. In the routine neonatal screening program several variant cases were detected with borderline blood phenylalanine levels (2--4 mg%) in samples obtained on the third or fourth day of life. These results suggest that though probably very few cases of classical PKU might be missed by examination of blood samples obtained on the second postnatal day, the findings in variant cases justify a recommendation to obtain repeat blood specimens under such circumstances.

Age Factors↗
PubMed1981

Phosphoglycolate phosphatase in several population groups in Israel.

The genetic polymorphism of phosphoglycolate phosphatase (PGP) found in red blood cells has been investigated in several population groups in Israel: Ashkenazi Jews, non-Ashkenazi Jews from Iraq, Yemen, Turkey, Iran, Balkan, North Africa and Arabs. The distribution of the PGP genes was not homogeneous (chi 2 = 40.545; d.f. = 20; p less than 0.005). The PGP2 gene frequency varied between 0.0185 in the Yemenite and 0.0688 in the Iranian Jews. PGP3 gene frequency ranged between 0.0062 in the Iranian and 0.0547 in the Moroccan Jews. Depsite this heterogeneity all the Israeli population groups showed some unifying characteristics which differentiated them from a random European population sample, namely higher frequencies of PGP1 gene (92-97% as opposed to 82% in th European sample) and lower frequencies of PGP2 gene (1.8-6.8% compared to 12.9% among Europeans).

Erythrocytes↗
PubMed1979

Decreased erythrocyte glutathione peroxidase activity in multiple sclerosis.

The mean activity of glutathione peroxidase (GSH-PX) in erythrocytes of 22 Israel-Jewish patients with multiple sclerosis (19.3 +/- 4.5 U/gHb) was significantly lower than in a control group of 30 Jewish patients with various neurological disorders (24.3 +/- 5.1 U/gHb). This observation confirms a similar finding of a decreased activity of GSH-Px in erythrocytes of multiple sclerosis patients in Denmark (Shukla et al. 1977). These results are discussed in relation to the possibility of selenium deficiency and to the recently described genetic polymorphism and ethnic variation of GSH-Px activity in human red cells. It is concluded that additional investigations are required in order to elucidate the cause of the decreased activity of this enzyme in red cells of patients with multiple sclerosis.

Erythrocytes↗
PubMed1979

Erythrocyte glyoxalase I polymorphism in several population groups in Israel.

The genetic polymorphism of red blood cell glyoxalase I (GLO) has been investigated in 9 population groups in Israel: Ashkenazi Jews, non-Ashkenazi Jews from Iran, Iraq, Balkan, North Africa, Yemen, Turkey and Egypt as well as Arabs living in Israel. The distribution of GL01 and GLO2 genes in the 9 communities was not homogeneous (x2 = 14.48; d.f. = 8; p less than 0.0005). Jews from Iran were found to have the lowest GLO1 frequency (0.2294), while those from Egypt had the highest gene frequency (0.3968). The other investigated communities were shown to have intermediate values for this gene frequency. No significant difference has been found between Ashkenazi and non-Ashkenazi Jews (with the exception of those from Egypt) or Arabs living in Israel.

Erythrocytes↗
PubMed1978

Abetalipoproteinemia treated with parenteral and oral vitamins A and E, and with medium chain triglycerides.

An 11-year-old girl with abetalipoproteinemia was treated with parenteral vitamin A and vitamin E for two and a half years. Some improvement in neurological and visual deficits was noted. On changing to oral vitamin E and later with addition of medium chain triglycerides (MCT) to the diet, a considerable improvement in her general wellbeing, neuromuscular lesions and ophthalmological symptoms was noted. This regimen is being adhered to for five and a half years. The condition is stable with no further improvement.

Abetalipoproteinemia↗