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Biomedical subjects

Y Shapira

Publications and source records attributed to Y Shapira.

At least 253 records · Page 14Linked to original sources
PubMed1981

Vitamin E deficiency in Werdnig-Hoffmann disease.

Vitamin E levels were measured in the plasma of infants and children with various neuromuscular disorders. Seven of 8 infants with Werdnig-Hoffmann disease (WHD) had a significantly lower plasma vitamin E level (p less than 0.01) than age-matched normal controls, children with congenital myopathies, or children with muscular dystrophy. Vitamin E deficiency in WHD is not caused by malabsorption. A therapeutic trial of vitamin E in 3 patients with WHD did not change the natural course of the disease. Vitamin E deficiency may play a role in the pathogenesis of WHD.

Child↗
PubMed1981

An approach to rotational relapse.

The tendency of rotated teeth to relapse after treatment following retention is partially because of the supra-alveolar fibers which are stretched during rotational correction. Early treatment, overrotation, a long retention period, and proper contouring of contact points have been used to reduce relapse. With several surgical methods, it has been shown that releasing the tension in the periodontal fibers by severing the supracrestal and transseptal fibers significantly reduced relapse tendencies following rotation. This study was designed to test a simple surgical method (Vanarsdall) and to examine the effect of transsecting supra-alveolar fibers in orthodontically rotated teeth. Twelve cases in which a tooth and its antimere were rotated in excess of 10 degrees prior to orthodontic treatment were chosen for this study. One of the two rotated teeth was chosen for the gingival fibrotomy, while its antimere was retained by conventional orthodontic means and served as a control. The incision was made at a 45-degree angle to the long axis of the tooth, on the labial and lingual aspects of each tooth, 2 months prior to debanding. A significant decrease in the amount of relapse up to 30 days following removal of appliances in the fibrotomy group as compared to the control group (0.42 degrees and 5.75 degrees, respectively) suggests that the procedure be considered for overcoming rotational relapse.

Gingivoplasty↗
PubMed1981

Reye's syndrome; diagnosis by muscle biopsy?

Three children with Reye's syndrome are described. One child died, the second had mild and transient illness, and the third had recurrent episodes. In all 3 children a muscle biopsy showed pronounced infiltration of the myofibres with fat microdroplets as shown by the oil red O stain and by electron microscopical examination. We suggest that needle biopsy of muscle may be a quick and safe aid to the diagnosis of Reye's syndrome, and may be preferable to liver biopsy in view of the pronounced tendency to bleed in Reye's syndrome.

Biopsy, Needle↗
PubMed1981

Myopathy in hyperornithinemic gyrate atrophy of choroid and retina.

Five patients in two families with hyperornithinemia and gyrate atrophy (HOGA) of the choroid and retina are reported. All patients had marked muscle wasting. Biochemical studies revealed high levels of plasma ornithine and low levels of plasma lysine. Muscle biopsies were performed in four patients and showed subsarcolemmal accumulation of pleomorphic mitochondria and tubular aggregates. These findings suggest that muscle wasting in HOGA is associated with a myopathy that is probably secondary to hyperornithinemia and/or hypolysinemia.

Adolescent↗
PubMed1980

Transposition of canines.

Eleven cases of canine transposition, in both the maxillary and the mandibular arches, have been reported. Canines were involved in every case. Alignment of the teeth in their transposed position or orthodontic movement to their normal position in the arch are alternatives in treatment. However, should an involved tooth be seriously affected by caries, developmental defects, or trauma, extraction may be the preferred treatment, with alignment of the remaining tooth and closure of the space.

Adolescent↗
PubMed1979

Myotonia congenita (Thomsen's disease). Early diagnosis in infancy.

A family with myotonia congenita. (Thomsen's disease) is reported in which the father and his two offspring are affected. The course was characterized by the early onset of clinical manifestations in both the father and his two children. In one child, a clinical and electrical diagnosis of the disease was made as early as two weeks of age. Early manifestations were breathing difficulty and eye closure myotonia. The importance of early recognition of the disorder is emphasized.

Adult↗
PubMed1979

Autosomal recessive sudden unexpected death in children probably caused by a cardiomyopathy associated with myopathy.

The propositus, who died suddenly at the age of 22 months, was investigated because of an unusual myopathy. Family history revealed two sisters and four cousins who had also died suddenly and unexpectedly. The finding of asymmetric septal hypertrophy by echocardiography in the propositus suggested that the cause of the sudden death in the relatives was an undetected cardiomyopathy accompanying a mild and often subclinical myopathy. The affected children were in two sibships and both sets of parents were first cousins. The mother of one sibship was the sister of the father of the other. It is suggested that a gene causes a mild autosomal recessive myopathy with cardiomyopathy that is often undiagnosed and usually ends in sudden unexpected death in the second year of life. The same gene may manifest on echocardiogram in some heterozygotes as asymmetric septal hypertrophy.

Cardiomyopathies↗
PubMed1978

Myopathy with multiple central cores. A case with hypersensitivity to pyrexia.

An eight-year-old male suffered from long-standing proximal muscle weakness, dramatically aggravated by febrile episodes. Neuromuscular work-up disclosed a myopathy with multiple central cores of non-familial nature. The presence of central cores in this patient as well as in the myopathy of malignant hyperpyrexia might suggest a pathophysiological basis common to both conditions.

Child↗
PubMed1978

Neuromotor development in relation to birth weight in rabbits.

The development of neuromotor patterns in relation to birth weight was studied in the rabbit, a perinatal brain developer. In order to induce intrauterine growth retardation and to increase the number of low birth weight rabbits, experimental ischemia to half the fetuses in each doe was achieved by total ligation of approximately 30% of spiral vessels to the placenta, during the last trimester of gestation. Following natural delivery, the rabbit pups were periodically observed for the appearance of eye-opening and righting reflex, and for the cessations of falling, circling and dragging of hind limbs. An index of neuromotor development was assigned to each rabbit by summing up the age (in days) of appearance of each of the neuromotor milestones. An association was found between low birth weight and delayed neuromotor development at 2 weeks of age. The most significant correlation was found between low birth weight and delayed disappearance of falling. The latter may represent incoordination as an expression of cerebellar dysfunction.

Age Factors↗