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Biomedical subjects

K Yalaz

Publications and source records attributed to K Yalaz.

At least 19 recordsLinked to original sources
PubMed1992

Selective involvement of the quadriceps muscle in congenital muscular dystrophies: an ultrasonographic study.

Muscle ultrasound scanning is a non-invasive and painless technique for evaluating muscle disorders in childhood. We have performed ultrasound scans of the quadriceps muscle in 26 children with various forms of congenital muscular dystrophies. There were 8 patients clearly showing selective involvement within the components of the quadriceps. In all cases showing selective involvement, the rectus femoris was spared and the vastus muscles were the affected group. Our findings support the concept of heterogeneity, often encountered in congenital muscular dystrophies.

Adolescent↗
PubMed1992

Intraventricular interferon and oral inosiplex in the treatment of subacute sclerosing panencephalitis.

We treated 22 patients with subacute sclerosing panencephalitis (SSPE) with intraventricular alpha-interferon (IFN) and inosiplex PO and followed them for 2 to 54 months. Three deaths occurred. Clinical improvement, demonstrated by decreasing scores on the Neurological Disability Index, occurred in 11/22 (50%); five patients became stable, and the progression rate of the disease decreased in three. The remission rate was significantly higher than untreated controls from the same institution. Patients who had a slowly progressive disease responded best to treatment. Serious side effects were rare. We recommend intraventricular IFN, combined with oral inosiplex, in the treatment of SSPE.

Administration, Oral↗
PubMed1991

Occidental type cerebromuscular dystrophy: a report of eleven cases.

Occidental type cerebromuscular dystrophy (OCMD) forms a substantial distinct group within congenital muscular dystrophy (CMD). These patients invariably present with amyotrophy, multiple joint contractures, facial muscle involvement, normal or nearly normal intelligence, leukodystrophic appearance on CT scan, and dystrophic changes in muscle.

Biopsy↗
PubMed1990

Congenital muscular dystrophy with cerebral involvement--report of a case of "occidental type cerebromuscular dystrophy"?

Cerebral CT scan abnormalities have been seen to be afflicted with some cases of classic occidental type congenital muscular dystrophy (CMD) with normal or borderline intelligence without neurological abnormality. A case is presented with early hypotonia, joint contractures, muscle biopsy features of CMD, normal intelligence and diffuse white matter hyperlucency on CT scan. Every CMD case should be screened with cerebral CT and magnetic resonance (MRI) scans to reach more aspects of this heterogenous disorder.

Brain↗
PubMed1990

N-acetylaspartic aciduria in Canavan disease: another proof in two infants.

Increased amounts of urinary N-acetyl-aspartic acid was found in two infants with biopsy proven Canavan disease. The aspartoacylase assay is a new tool for determining both the prenatal and antenatal diagnosis of Canavan disease. This assay should be screened in patients with early onset of psychomotor deterioration, macrocephaly, spasticity/hypotonia and white matter hyperleucency at CT scan.

Amidohydrolases↗
PubMed1990

Neurologic complications in chronic renal failure: a retrospective study.

We have retrospectively examined 324 patients with chronic renal failure and evaluated the probable underlying causes of neurologic complications, laboratory data and therapeutic interventions. The common neurologic problems in our patients were alterations in consciousness (40.7%) and convulsions (35.1%). When BUN concentration was above 135 mg/dl and creatinine clearance was below 8 m/min/1.73 m2, alteration of consciousness was observed and when BUN concentration was 200 mg/dl and creatinine clearance was below 7 m/min/1.73 m2, abnormal convulsives appeared. Changes in deep tendon reflexes and pathologic reflexes were associated with hypertension. All of the patients with cortical atrophy using computerized cranial tomography aluminum hydroxide at least for 18 months, and six of them had hemodialysis. Fourteen patients who underwent dialysis developed convulsions and were thought to have disequilibrium syndrome. These findings are consistent with the suggestion that the metabolic and biochemical derangements associated with CRF may be particularly detrimental to the still developing CNS of the child.

Adolescent↗
PubMed1989

Long-term prognosis after neonatal tetanus.

Twenty-four Turkish children who had had neonatal tetanus were evaluated by means of physical and neurological examinations and psychometric tests at four to 15 years of age. Enuresis, mental retardation and growth retardation were frequent findings. The prevention of neonatal tetanus is important not only because of the high mortality rate but also because of its sequelae.

Adolescent↗
PubMed1988

[Symptoms and clinical signs, laboratory data in 80 cases of subacute sclerosing panencephalitis].

Clinical signs, laboratory data and electroencephalography were studied in 80 cases of subacute sclerosing panencephalitis. Myoclonus with or without intellectual deterioration was the most frequent symptoms (56,2 p. 100). EEG was one of the more specific tests; diazepam injection made it more sensitive. Level of complement fixing antibodies in the CSF was sometimes low, even in advanced cases (29 out of 70; 41 p. 100). CT was normal in 70 p. 100 of cases, showed cerebral atrophy in 17 p. 100 and some other (uncommon) signs in 13 p. 100 of cases.

Adolescent↗
PubMed1984

Cell mediated immunity in patients with subacute sclerosing panencephalitis.

Cell mediated immunity was assessed on the basis of total lymphocytes (TL), total T lymphocytes (TTL) counts, delayed skin test responses and in vitro leucocyte migration inhibition test (LIF) production in 25 patients with SSPE, classified according to the clinical stages of the disease. The patients in stage I of the disease did not show any defect in cellular immunity while the patients in stage II showed decreased TL and TTL counts, more negative skin test responses to PHA, SKSD and PPD, and unresponsiveness to SKSD of LIF production. When the patient group was evaluated as a whole, only the TL counts and the skin test responses against SKSD differed from those in the controls. These results suggest that the defects in cellular immunity demonstrated in the patients with SSPE may be due to SSPE or the measles virus itself rather than to a genetic factor predisposing patients to SSPE.

Adolescent↗
PubMed1984

Urban Turkish children's performance on the Denver Developmental Screening Test.

Normative data for 1176 healthy, urban Turkish children were analysed for sex and social-class differences in performance of the Denver Developmental Screening Test (DDST). Sex differences were minor, and were similar to those found in other countries. Social-class differences, however, in contrast to findings in other DDST studies, were consistently in favour of the more advantaged children in all areas of functioning from about the age of 10 months. These differences became more marked with age, particularly in language and fine-motor skills. These findings indicate considerable cultural differences within urban Turkey and suggest that the use of only one set of norms may lead to misdiagnosis. Further, they question the predictive validity of this test for lower-class urban Turkish children, particularly for language and fine-motor tasks.

Child Development↗
PubMed1981

Hyperlysinemia without clinical findings.

A three-year-old asymptomatic boy with hyperlysinemia is presented. The patient's plasma lysine levels have been constantly high (685-1370 mumol/l) and excessive urinary excretion of ornithine, arginine and cystine have been noted. There was no detectable activity of lysine-ketoglutarate reductase nor saccharopine dehydrogenase in skin fibroblast culture. Review of the reported cases and this patient with serious biochemical defect but without symptoms indicate clinical heterogeneity in hereditary hyperlysinemia.

Amino Acid Metabolism, Inborn Errors↗