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Biomedical subjects

R Zass

Publications and source records attributed to R Zass.

14 recordsLinked to original sources
PubMed1998

Bilateral oedema of the basal ganglia in an echovirus type 21 infection: complete clinical and radiological normalization.

A 4-year-old girl with bilateral striatal oedema in association with an echovirus type 21 infection is reported. In the course of a prolonged upper respiratory-tract infection, the patient developed muscular hypotonia, resting tremor, ataxia, sleepiness, hyperaesthesia, and indistinct speech. T2-weighted cranial MRI revealed bilateral oedema of the basal ganglia and the cerebellar peduncles. At follow-up after 3 months MRI changes and clinical symptoms had fully resolved.

Basal Ganglia↗
PubMed1997

Progression of cerebral white matter abnormalities in early treated patients with phenylketonuria during adolescence.

In this study of cranial MRI a group of 15 adolescents with classical phenylketonuria and permanent blood phenylalanine (phe) checks from infancy was investigated twice with an interval of three years in between. Cranial MRI revealed a progression of white matter abnormalities in patients with moderate and poor control of blood phe levels, however not in well controlled patients. Nevertheless results indicate an individual vulnerability of the brain against elevated phenylalanine levels in phenylketonurics.

Adolescent↗
PubMed1996

Untreated non-phenylketonuric-hyperphenylalaninaemia: intellectual and neurological outcome.

The intellectual, neurological, and neuropsychological outcome of patients with non-phenylketonuric-hyperphenylalaninaemia (PKU-HPA) (serum phenylalanine levels under free diet < 600 mumol/l) has not been systematically studied so far. We therefore tested 28 patients (mean age = 21.8, SD = 4.2 years) for IQ (WAIS-R/WISC-R), school performance, job career, clinical neurological examination, fine motor performance (motor performance task), and selective and sustained attention (stroop task, Dot Pattern Exercise from the Sonneville visual attention task). In addition, cranial MRI (1.5 T unit) was obtained in 10 of these patients. Clinical-neurological examination revealed no significant abnormalities in the non-PKU-HPA patients. They also had a normal IQ (mean = 101.9, SD = 13.6). Compared to their healthy siblings, they attended a normal school and had a normal job career. The motor performance task revealed no deficits in fine motor abilities. The patients performed normally in the stroop task and the dot pattern exercise. Their MRIs were normal. Our results indicate that patients with non-PKU-HPA are not at risk for developing intellectual, neurological, and neuropsychological impairment, as described for patients with treated mild or classical phenylketonuria. From this point of view a dietary treatment is not necessary in patients with hyperphenylalaninaemia.

Adolescent↗
PubMed1996

The first isolation, in vitro propagation, and genetic characterization of Ehrlichia canis in Israel.

Ehrlichia canis, the etiologic agent of canine ehrlichiosis, was isolated in Israel from a naturally infected dog with acute signs of the disease. The organism designated E. canis 611, was passaged experimentally to a beagle, from which it was propagated in primary canine monocytes. The organism was then grown in vitro in a continuous canine cell line, DH82. Nine beagles subsequently injected with whole E. canis-infected blood all developed typical symptoms of ehrlichiosis. An indirect immunofluorescence antibody test to E. canis was developed and compared with a commercial kit, revealing a good correlation between the two assays. Transmission electron microscopy of DH82 cells infected with the Israeli strain of E. canis (611), revealed organisms similar to those described in the literature: two different forms of morulae appeared, one tightly, the other loosely, packed. The 16S rRNA gene sequence obtained from the Israeli Ehrlichia isolate was compared with other isolates, E. canis Oklahoma and E. canis Florida. The Israeli strain 16S rRNA had three nucleotide differences from the Oklahoma isolate, and four nucleotide differences from the Florida isolate, in addition to one nucleotide gap in each. The Israeli isolate was found to be 0.54% different from the Oklahoma strain, and 0.61% different from the Florida strain. There are the same magnitudes of differences displayed by the other most closely related group in the phylogenetic tree, namely Ehrlichia equi, Ehrlichia phagocytophilia and the human granulocytic ehrlichia.

Animals↗
PubMed1996

Detection of ehrlichial antigen in plasma of beagle dogs with experimental acute Ehrlichia canis infection.

Six beagles were experimentally infected with Ehrlichia canis. All dogs developed typical clinical signs of ehrlichiosis and sero-converted. Ehrlichial antigenemia in the plasma of the infected dogs was detected using a sandwich enzyme-linked immunosorbent assay (ELISA). Ehrlichial antigen was present starting 15-20 days post-infection, after the development of clinical signs and antibody titre to Ehrlichia canis. The appearance of ehrlichial antigen in the plasma for a relatively short and variable period after the clinical and haematological signs, limits its potential as an early diagnostic prognosticator of canine ehrlichiosis.

Animals↗
PubMed1996

No fine motor deficits in patients with untreated non-phenylketonuria hyperphenylalaninaemia.

Twenty-four untreated adolescent and adult patients with non-phenylketonuria (PKU) hyperphenylalaninaemia (HPA) (serum phenylalanine levels < 600 mumol l-1) and 24 healthy controls matched for age, sex and IQ were investigated for their neurological outcome, especially for fine motor abilities by the Motor Performance Task. No pathological findings could be revealed by clinical neurological examination. Patients with HPA and healthy controls did not significantly differ in their fine motor performances. These performances were not significantly influenced by serum phenylalanine concentrations. Our results indicate that untreated patients with non-PKU HPA are not at clinically significant risk for developing fine motor deficits and severe neurological impairment. From this point of view a dietary treatment is not necessary in patients with HPA, as recommended most recently.

Adolescent↗
PubMed1994

Simultaneous determination of betaine and N,N-dimethylglycine in urine.

A method is described for the determination of betaine and its metabolite N,N-dimethylglycine in human urine. The method involves a deamination step and a solid-phase extraction to isolate both substances from urine followed by high performance liquid chromatography (HPLC) separation on a 5-microns SCX column with UV absorbance detection. Limit of detection is about 0.9 micrograms for both substances. Recoveries were > 85%. The method appears to be suitable for monitoring betaine and its metabolite in urine of patients undergoing therapy with betaine.

Betaine↗
PubMed1993

Contribution of odd-numbered fatty acid oxidation to propionate production in neonates with methylmalonic and propionic acidaemias.

Newborns with disturbed propionate metabolism (propionic and methylmalonic acidaemias) accumulate during fetal life large amounts of odd-numbered long-chain fatty acids in adipose tissue (8% to 10% of total fatty acids vs 1% in normal controls) and other body lipids. During periods of acute catabolism, such as in the early days of life, mobilization and oxidation of odd-chain fatty acids from adipose tissue yield extensive amounts of toxic propionyl-CoA in the mitochondria and thus might contribute to the severe illness of the patients. Our data suggest that not only catabolism of protein but also that of adipose tissue has to be avoided in order to alleviate the neonatal illness of patients with propionic and methylmalonic acidaemias. It seems prudent to avoid prolonged fasting during the 1st year of life.

Acyl Coenzyme A↗
PubMed1993

[Hyperthyroidism caused by inadequate TSH secretion].

At 5 years of age inappropriate thyrotropin secretion was diagnosed in a girl now 11 years old. She had clinical signs of hyperthyroidism, and circulating thyroid hormones were elevated, but the TSH-level was within normal range. There was no evidence of a pituitary tumor. Therapy with bromocriptine, dexamethasone, and D-thyroxine had no measurable effect. The treatment with methimazole led to normal levels of circulating thyroid hormones, however, the TSH-level increased and the thyroid gland became diffusely enlarged. By the administration of 3,5,3'-triiodothyroacetic acid (TRIAC) (at present 20 micrograms/kg body weight) the TSH-level was lowered and a long-term remission of hyperthyroidism was achieved.

Antithyroid Agents↗
PubMedDate not supplied

[Abuse of artificial sweetener as differential diagnosis of lactose intolerance].

A two year old dystrophic boy with chronic diarrhea was described. At the age of 6 month the boy developed a severe gastroenteritis with a secundary lactase deficiency. Dietary treatment was however not successful and consequently the boy became dystrophic. At last, an abuse of sweetener was diagnosed.

Child, Preschool↗