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Biomedical subjects

V Dubowitz

Publications and source records attributed to V Dubowitz.

At least 235 records · Page 13Linked to original sources
PubMed1982

Quantitation of muscle function in children: a prospective study in Duchenne muscular dystrophy.

A protocol has been developed for the quantitative assessment of muscle function in children with muscle disease. It includes total muscle strength (% MRC) based on a clinical assessment of strength of 32 groups using the 6-point MRC grading; the force of 8 selected muscle groups measured with a specially designed electromyometer; a motor ability score based on 20 consecutive motor activities; walking times over 28 and 150 feet, and recording of muscle contractures. A 3-year sequential study of 61 boys with Duchenne dystrophy showed progressive decline of muscle strength with age, a close correlation of total strength and the motor ability score (r = 0.89), and a curvilinear relationship of muscle strength with walking times over 28 and 150 feet (r = 0.78 and 0.79, respectively). A profile of the natural progression of Duchenne dystrophy has been established which could serve as a reference base for the assessment of cases at varying ages and their response to therapy and management.

Aging↗
PubMed1982

Collagen types in neuromuscular diseases.

The striking proliferation of connective tissue characteristic of the muscular dystrophies can be attributed predominantly to an increase in endomysial and perimysial type III collagen. Carriers of muscular dystrophy occasionally revealed a slight increase in anti-type III collagen fluorescence, but no abnormalities in collagen disposition were observed in foetuses "at risk" for DMD. In contrast, the proportion of collagen types in neurogenic atrophies appeared normal although anti-type IV and V staining, which delineated the basement membrane, was very intense around atrophied fibres, as was also the case in small fibres in myopathic diseases. The detection of staining with anti-type III, IV and V collagens in splits which are sometimes observed in hypertrophied fibres in the muscular dystrophies supports the suggestion that abnormalities in collagen production, perhaps involving a defective modulation of myoblast-fibroblast expression, may be involved in the pathogenesis of these diseases.

Adolescent↗
PubMed1982

Cytochemical studies of lectin binding by diseased human muscle.

The binding characteristics of lectins with varying sugar specificities were investigated in muscle biopsies from normal individuals and from patients with neuromuscular disorders. Horseradish peroxidase (HRPA) and fluorescein isothiocyanate (FITC)-conjugated lectins were used for light microscopy and ferritin-conjugated Concanavalin A (Con A) for electron microscopy. In normal and diseased muscle a lectin specific for alpha-D-mannosyl residues (Con A) and a group of lectins specific for beta-D-galactosyl residues were found to bind to the perimysial and endomysial connective tissue, blood vessels and capillaries and clearly demonstrated the perimeter of each muscle fibre. Wheat germ agglutinin (WGA), specific for N-acetylglucosamine and N-acetyl-neuraminic acid residues, had a similar distribution of staining although it appeared to stain the capillaries more strongly. In contrast, lectins specific for alpha-L-fucose and N-acetyl-galactosamine did not stain specifically any structures in normal or diseased muscle. In biopsies from dystrophic patients Con A, WGA and the beta-D-galactose specific lectins were always associated with splits and in biopsies from a variety of disorders discontinuities in staining were observed at the periphery of occasional fibers. These were not found in normal muscle. Electron microscopy showed Con A bound to the basement membrane of muscle fibres and capillaries and the connective tissue. The plasma membranes themselves were unstained. These preliminary investigations of lectin binding in muscle have shown important differences in diseased muscle and demonstrate the application of lectin chemistry to the study of membrane structure.

Adolescent↗
PubMed1982

Ultrasound imaging in the diagnosis of muscle disease.

A comparative study has been done of the static B-scan ultrasound appearance of the quadriceps muscle of the thigh in 60 new patients attending our muscle clinic and in 60 control children. In the control subjects there was good visualization of bone and fascia, which stood out clearly against the background of echo-free muscle tissue. Striking change was found in children with neuromuscular disease. Muscular dystrophies were associated with an increase in the intensity of echo reflected from the muscle substance, with corresponding loss of bone echo. Spinal muscular atrophies and neuropathies also showed an increase in muscle echo along with atrophy of the muscle and increase in depth of subcutaneous tissue. Various congenital myopathies also showed changes. Infants with hypotonia from nonneuromuscular causes had normal scans. Severity of change on the scan did not relate to functional disability, and some children had good function yet strikingly abnormal scans. Three degree of change on the scan correlated with the degree of disruption of muscle architecture on biopsy. Ultrasound imaging has proved to be a useful, noninvasive screening tool in the investigation of children with neuromuscular disease.

Adolescent↗
PubMed1982

Progressive neurological disorder associated with obstructive jaundice and vitamin E deficiency.

A ten-year-old girl had obstructive jaundice in the newborn period which persisted for 4 years despite choledochojejunostomy at 6 weeks. From the age of 6 years she developed a progressive neurological syndrome characterized mainly by dysarthria and ataxia. A causal relationship with her profound vitamin E deficiency seemed likely. Treatment with vitamin E over a 2 1/2 year period appeared to arrest the progression of the neurological deficit and subsequent increase in dosage produced some improvement in her ataxia.

Ataxia↗
PubMed1982

Neurological and neurobehavioural differences between preterm infants at term and full-term newborn infants.

A detailed neurological and neurobehavioural examination was done in 80 preterm infants of 27 to 35 weeks gestation in the first week of life and again at 40 weeks postmenstrual age, and in 40 appropriately-sized full-term infants on the first and fifth days of life. There were several consistent differences between the preterm infants reaching term and the newborn full-term infants. The preterm infants reaching 40 weeks showed less flexion in their posture, and less arm traction, arm recoil and leg recoil than the full-term infants on day 1, although this difference was less apparent by day 5. The preterm infants appeared not to develop the flexor tone of the full-term newborn. The preterm infants demonstrated better visual and auditory orientation and alertness than the full-term infants. The significance of these findings, both for initial neonatal examinations and for subsequent assessments, is evaluated.

Arousal↗
PubMed1982

The development of visual function in normal and neurologically abnormal preterm and fullterm infants.

Pattern preference for four different pairs of patterns, and visual acuity based on the ability to distinguish black and white stripes of different widths, were compared in neurologically normal and abnormal preterm infants at 36 and 40 weeks postmenstrual age and in normal and abnormal fullterm infants in the newborn period and again at four and six weeks of age. The study aimed to chart the maturation process of these visual functions in the neonatal period and to assess their predictive value in the neurologically abnormal infant. Part I of the study deals with the normal infant and Part II with the abnormal infant. In Part I, the maturation process for both visual functions in newborn preterm infants of increasing gestational age is compared with longitudinal assessment of postnatal maturation of these functions in preterm infants up to 40 weeks postmenstrual age. Up to 36 weeks postmenstrual age the functions were comparable in the maturing preterm infants and the newborn infants of comparable postmenstrual age, but at 40 weeks the preterm infants did less well than the fullterm newborn infants. In Part II, the abnormal fullterm infants demonstrated a significantly poorer pattern preference at the initial and subsequent examination. Visual acuity was significantly poorer at the initial examination but less marked at follow-up. The abnormal preterm infants showed poorer pattern preference and visual acuity at both 36 and 40 weeks postmenstrual age. Compared with neurologically abnormal infants without intraventricular haemorrhage (IVH), preterm infants with IVH showed no significant difference in pattern preference at 36 and 40 weeks, but a significant deficit in visual acuity.

Cerebral Hemorrhage↗
PubMed1982

Developmental and neurological progress of preterm infants with intraventricular haemorrhage and ventricular dilatation.

A prospective neurological and developmental assessment was completed at ages 6, 9, and 12 months on 39 preterm infants under 34 weeks' gestation. In the newborn period each infant had an assessment of gestation and sequential neurological and ultrasound examinations and was placed in one of three groups: intraventricular haemorrhage (IVH) (n = 14), IVH followed by ventricular dilatation (n = 11), and control infants with no evidence of IVH (n = 14). When corrected for prematurity the Griffiths's developmental quotients (DQs) were normal at 6, 9, and 12 months for every infant except one aged 12 months. In contrast, the uncorrected DQs at 12 months were under 80 in only one of the 14 preterm infants without haemorrhage, compared with 2 of the 14 with IVH, and with 7 of the 9 with IVH and dilatation. There was also a higher incidence of neurological abnormality at each follow-up age in the infants with IVH plus ventricular dilatation, compared with those with IVH alone, or with infants without IVH. Similar differences were also demonstrated in 5 milestones reflecting gross motor, fine motor, and social or verbal development in the three groups at 6, 9, and 12 months. The neurological and developmental deficits seemed to relate more closely to the presence of post-haemorrhagic ventricular dilatation than to the size of the initial haemorrhage itself. These results may have important implications for therapeutic intervention in the management of newborn infants with IVH and ventricular dilatation.

Brain Diseases↗
PubMed1982

C-reactive protein in childhood dermatomyositis.

Serum levels of C-reactive protein (CRP) were determined in 9 patients with childhood dermatomyositis. Four children were seen during clinical relapse and all had serum CRP levels less than 1 mg/l. In addition direct immunofluorescent staining of muscle biopsies from 4 patients showed no evidence of CRP deposition in muscle tissue. Such patients appear to be able to produce CRP in response to acute infections, and it is suggested therefore that the pathological process in childhood dermatomyositis may not induce a significant CRP response.

Adolescent↗
PubMed1981

Activity of creatine kinase in sera from healthy women, carriers of Duchenne muscular dystrophy and cord blood, determined by the "European" recommended method with NAC-EDTA activation.

Creatine kinase activity has been measured at 37 degrees C in sera from healthy women, carriers of Duchenne muscular dystrophy and cord blood, with activation by N-acetyl cysteine (NAC) and EDTA as recommended by several European committees on standardisation. The upper limit of the reference range for healty women was found to be 170 U/l. The distributions of creatine kinase activities in healthy and carrier women have been used to calculate probability of carrier status as a function of creatine kinase activity. Although the range of creatine kinase activities in normal cord blood is wide, the data provide a basis for interpretation when Duchenne muscular dystrophy is suspected.

Acetylcysteine↗
PubMed1981

Needle muscle biopsy for carrier detection in Duchenne muscular dystrophy. Part 1. Light microscopy--histology, histochemistry and quantitation.

Needle muscle biopsies from 14 genetically proven carriers of Duchenne muscular dystrophy (DMD), 14 possible carriers with elevated serum creatine kinase activity (SCK) and 37 possible carriers with normal SCK were compared with similar samples from 10 healthy volunteer females with the aim of identifying subtle changes that might aid carrier detection. Qualitative differences were similar to those previously reported and included variation in fibre size, an increase in the number of internal nuclei and fragmentation of the myofibrillar network. By visual assessment, only 11% of the biopsies were classified as unequivocally abnormal but quantitation of fibre type proportions, fibre diameters and the number of internal nuclei showed that 71% of definite carriers, 86% of possible carriers with elevated SCK and 78% of possible carriers with normal SCK had at least one feature that was significantly different from controls. The commonest abnormalities were an increase in the proportion of type 1 fibres, an alteration in the mean diameter ratio of type 1:type 2 fibres, an increase in the fibre diameter variation coefficient and an increase in the number of internal nuclei. The changes in definite carriers were not related to elevated SCK and several with normal SCK showed morphological differences. It was concluded that quantitation of morphological features is essential for a full appraisal of biopsies from DMD carriers and that this approach is a useful aid to carrier detection.

Adenosine Triphosphatases↗
PubMed1981

Neurologic signs in neonatal intraventricular hemorrhage: a correlation with real-time ultrasound.

A comprehensive neurologic assessment was applied sequentially in 100 consecutive unselected newborn infants in our neonatal unit in parallel with independent sequential real-time ultrasonic examination of the head. The results were analyzed in three separate gestational groups: Group I, 31 weeks and below (n = 29); Group II, 32 to 35 weeks (n = 39); Group III, 36 weeks and above (n = 25). In the remaining seven infants the ultrasonic examination was technically unsatisfactory. Deviant neurologic signs were correlated in each group with the presence of intraventricular hemorrhage recognized by ultrasound. Impaired visual tracking, an abnormal popliteal angle, and the later development of roving eye movements correlated strongly with the presence of IVH in Groups I and II. Decrease in tone and poor motility correlated with IVH in Group II infants only. In Group III the number of cases with IVH was small and none of the clinical signs reached statistical significance. The results suggest that our system of careful sequential neurologic examination in the newborn period is a sensitive means of detecting the development of intraventricular hemorrhage in the majority of immature infants, and of following its progress and resolution.

Cerebral Hemorrhage↗
PubMed1981

Intellect and behaviour in Duchenne muscular dystrophy.

A study was made of intellectual, educational and emotional functioning of 57 boys with Duchenne muscular dystrophy, aged between three and 13 years, using standard intelligence tests, a reading test and the Rutter Behaviour Questionnaires. Their intelligence test scores were about 1 SD below the mean, the majority functioning better on performance than on verbal tasks. Reading levels were variable, but averaged about 1-5 SD below the mean; a large proportion of the boys read at a very low level. Both of the Behaviour Questionnaires showed a high rate of emotional disorder. The cognitive tests showed no change with age and the general pattern of functioning remained constant. This study confirms the view that intellectual impairment, and particularly verbal impairment, is associated with Duchenne muscular dystrophy, but it is non-progressive and does not affect all children. A high rate of emotional disturbance is also associated with the disease.

Adolescent↗
PubMed1981

Cerebral structure and intraventricular haemorrhage in the neonate: a real-time ultrasound study.

A method for systematic scanning of the neonatal brain with real-time ultrasound is described, and the linear array is compared with mechanical sector scanners. Anatomical landmarks recognised on axial, coronal, and parasagittal scans are verified by comparison with brain slices at necropsy. A prospective study of 50 infants admitted consecutively to the neonatal unit at this hospital showed intraventricular haemorrhage in 18 (36%). These 18 infants included 10 (43%) out of 23 of birthweight less than or equal to 1500 g, and 3 (27%) out of 11 of birthweight 1501-2000 g. An unexpected feature was the recognition of intraventricular haemorrhage in 5 of the 13 infants greater than 2000 g birthweight. On sequential daily scans intraventricular haemorrhage was diagnosed most often in the first 2 days of life and abnormal ventricular echoes persisted for up to 12 days thereafter. Late development of hydrocephalus was recorded in 2 infants. Real-time ultrasound provides the neonatologist with a practical method for diagnosis and monitoring of intracranial lesions in the ill neonate and is a valuable, non-invasive, and safe tool for studying the pathophysiology of neurological handicap in infancy.

Cerebral Hemorrhage↗