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Biomedical subjects

V Dubowitz

Publications and source records attributed to V Dubowitz.

At least 217 records · Page 12Linked to original sources
PubMed1984

Correlation of neurologic assessment in the preterm newborn infant with outcome at 1 year.

A prospective study was undertaken of the outcome at 1 year in 129 preterm infants of less than 34 weeks gestation (range 27 to 34 weeks) who underwent detailed neurologic assessment and ultrasound scanning in the neonatal period and again at 40 weeks postmenstrual age, and an independent neurodevelopmental assessment at 12 months chronologic age. Of the 129 infants, 37 (29%) had ultrasound evidence of periventricular hemorrhage. At 40 weeks postmenstrual age the infants were classified as neurologically normal, abnormal, or borderline on the basis of the neurologic examination. Of the 62 infants considered normal at 40 weeks, 57 (91%) were assessed as normal at one year, compared to only 14 (35%) of the 39 infants considered abnormal (P less than 0.001). Ten (85%) of the 12 normal infants with associated periventricular hemorrhage were normal at 1 year, compared to 47 (94%) of the 50 normal infants without periventricular hemorrhage, whereas 5 (25%) of 20 abnormal infants with associated periventricular hemorrhage and 9 (47%) of the 19 without periventricular hemorrhage were normal at 1 year. There was no direct correlation in individual cases between the severity of neurologic deficit and the presence or severity of periventricular hemorrhage. Infants with a cluster of abnormal signs were more likely to have later dystonia or cerebral palsy than those with marked hypotonia but no other abnormality.

Cerebral Hemorrhage↗
PubMed1984

Fasciculation of the eyelids: an additional clue to clinical diagnosis in spinal muscular atrophy.

Fasciculation of the eyelids was observed in five of eleven children with spinal muscular atrophy (SMA); two had severe SMA and three were of intermediate severity. Three other children with intermediate SMA and the two children with mild SMA did not show eyelid fasciculations. This could provide an additional clinical sign to tongue fasciculation and hand tremor in the diagnosis of SMA in childhood.

Adolescent↗
PubMed1984

Monoclonal antibodies detect a spectrin-like protein in normal and dystrophic human skeletal muscle.

Spectrin is the major protein of the erythrocyte membrane skeleton, which is bound to the cytoplasmic surface of the membrane's lipid bilayer and is responsible for cell shape and membrane elasticity. Inability to identify spectrin in other cell types led to the assumption that this protein was unique to erythrocytes. However, spectrin-like proteins have been demonstrated recently in a variety of cell types, including skeletal and cardiac muscle, in several species. We used monoclonal antibodies against human erythrocyte spectrin subunits in an immunocytochemical study to detect related proteins in normal and diseased human skeletal muscle. Six of seven monoclonal antibodies against beta-spectrin determinants were bound at the cytoplasmic surface of muscle fiber plasma membranes, whereas none of six monoclonal antibodies against alpha-spectrin determinants was bound. Muscle fibers of patients with neuromuscular diseases showed similar distribution and specificity of antibody binding to those of normal subjects, but the intensity of binding was increased. In contrast, probable regenerating fibers in muscle of patients with muscular dystrophies showed reduced binding of antibodies, but reduced binding was not seen in fetal muscle fibers nor in those of a patient with a myotubular myopathy. We conclude that human skeletal muscle fibers possess a spectrin-related protein associated with their plasma membrane that shows extensive beta-chain similarities to erythrocyte spectrin but differs significantly with respect to the alpha-subunit. Its function may be associated with the maintenance of membrane and myofibril integrity during contraction, and the increased antibody binding in diseased muscle may reflect a structural rearrangement of spectrin or a compensatory increase in spectrin abundance in response to increased stress on these systems.

Antibodies, Monoclonal↗
PubMed1984

Diagnostic needle muscle biopsy. A practical and reliable alternative to open biopsy.

The technique of needle muscle biopsy using the Bergstr öm needle has been in routine use in our muscle clinic since 1978. In an initial feasibility study 24 children had a needle and an open biopsy performed simultaneously through extension of the same incision and 22 had identical interpretation of the needle and open biopsies. Needle biopsies have subsequently been performed in 674 children and have been satisfactory for diagnostic assessment in 656. The samples have been of adequate size and comparable in quality to our previous open biopsies, with good preservation and orientation. Needle muscle biopsy under local anaesthetic is quicker and less traumatic than open biopsy and leaves only a very small scar. Sufficient muscle can be obtained for routine histological, histochemical, and electronmicroscopic diagnosis, as well as for specialised biochemical and research purposes. There seems little justification for the continued use of open biopsy for routine investigation of neuromuscular disease.

Adolescent↗
PubMed1983

Use of nerve conduction velocity to determine gestational age in infants at risk and in very-low-birth-weight infants.

Nerve conduction velocity was used to measure gestational age in at risk and very-low-birth-weight neonates. The method gave highly significant correlations with gestational estimates by the Dubowitz score and with confirmed maternal dates; 86% of the estimates agreed within two weeks with confirmed maternal dates. The method is valid in babies 30 weeks old or younger and is reproducible after the first postnatal week. There was no difference in babies small for gestational age. The technique was estimated to predict gestation with a standard deviation of +/- 1.14 weeks.

Gestational Age↗
PubMed1983

Auditory brainstem responses in neurologically abnormal infants.

Abnormal auditory brainstem responses (ABR) were recorded in 33 of 59 infants with neurological abnormality in the newborn period compared with a 5 per cent incidence in infants who were neurologically optimal. Abnormality of ABR was most common in infants with intraventricular hemorrhage (17/21) but showed no correlation to size of hemorrhage. Abnormal ABRs were also frequent in infants with apnoeic syndrome (5/8) and in infants with abnormal tone patterns in the absence of intraventricular hemorrhage (7/13). All the infants with birth asphyxia had normal ABRs. Although some infants with midbrain signs had abnormal ABRs, the degree of abnormality did not correlate with the severity of clinical signs. ABR may be a useful adjunct in assessing function in neurologically abnormal infants and in confirming early major hearing deficit. Further studies should show whether the abnormal ABR reflects a primary neurological defect or is secondary to recognisable neurological lesions such as intraventricular hemorrhage. Follow-up studies are also needed to establish whether infants who are clinically abnormal and have abnormal ABR will have a worse prognosis than those without associated abnormality in ABR.

Apnea↗
PubMed1983

Drug treatment of juvenile dermatomyositis.

A series of 29 children with dermatomyositis has been reviewed and the outcome compared between cases treated by us initially with a low dosage short duration course of corticosteroids, and those referred late and having had various different therapeutic regimens and usually more active and higher dosage drug schedules. There were fewer relapses and less morbidity in the low dosage short term group. It was noted also that there was no relation between the initial mode of onset or severity and the eventual outcome or course of the disease. However, pronounced skin vasculitis appeared to reflect severity of disease.

Acute Disease↗
PubMed1983

Hemorrhagic periventricular leukomalacia in the neonate: a real-time ultrasound study.

Periventricular leukomalacia is an important complication of hypoperfusion of the brain in immature newborn infants. In a real-time ultrasound study with frequent scanning of 120 infants of birth weight less than 1,501 g, hemorrhagic periventricular leukomalacia was observed in nine (7.5%), and in five of these infants subsequent development of cystic degeneration was noted. The overall incidence of intraventricular hemorrhage in the same population was 48%.

Brain Diseases↗
PubMed1982

Nuclear magnetic resonance imaging of the brain in children.

A preliminary study of nuclear magnetic resonance imaging of the brains of four normal children (36 weeks' postmenstrual age to 5 years) showed long T(1) areas in the periventricular region of the neonate as well as evidence of progressive myelinisation with increasing age. Study of 18 patients of 40 weeks' postmenstrual age to 4 years showed an apparent deficit in myelinisation in an infant with probable rubella embryopathy and another with ventricular dilatation of unknown cause. Abnormal scans were obtained in an infant with congenital muscular dystrophy, and abnormalities were visualised at the lateral ventricular margins in a case of acute hydrocephalus after shunt blockage. Periventricular regions of increased T(2) were seen in a term infant aged 4 days after severe birth asphyxia and convulsions.Nuclear magnetic resonance imaging appears to provide a unique demonstration of myelinisation in vivo and shows changes in pathological processes of importance in paediatric practice.

Brain↗
PubMed1982

A comparison of neurological function in growth-retarded and appropriate-sized full-term newborn infants in two ethnic groups.

A comparative neurological study was undertaken of growth-retarded or small-for-dates (SFD) and appropriately grown (AFD) full-term infants in a Cape Coloured and a White (London, UK) population. There were no associated complications of pregnancy or labour. Consistent differences were found between SFD and AFD infants in both ethnic groups. In the SFD infants there was an increase in visual and auditory orientation, alertness, startle reflexes, tremors and tone, and some reduction in habituation. The difference between our results and those of earlier authors can probably be accounted for by factor such as use of drugs in labour, perinatal events and different causes of growth retardation, all of which may influence neurological behaviour.

Black or African American↗
PubMed1982

Erythrocyte-ghost Ca2+-stimulated Mg2+-dependent adenosine triphosphatase in Duchenne muscular dystrophy.

The Ca2+-stimulated Mg2-dependent ATPase activities (Ca2+-ATPase) of erythrocyte-ghost membranes from patients with Duchenne muscular dystrophy (DMD) and carriers of DMD were compared with activities of normal controls. The Ca2+-ATPase activity of DMD-patient ghost preparations was found to follow the same pattern of activation by Ca2+ as the control membranes. However, the Ca2+-ATPase activity in DMD and some DMD-carrier preparations was substantially elevated compared with controls. To characterize further the elevated Ca2+-ATPase activity found in DMD-patient ghost membrane preparations, we estimated kinetic parameters using both fine adjustment and weighting methods to analyse our experimental data. It was established that in both DMD and DMD-carrier preparations the increase in Ca2+-ATPase activity was reflected by a significant increase in Vmax. rather than by any change in Km. The response of the membrane Ca2+-ATPase activity to changes in temperature was also investigated. In all preparations a break in the Arrhenius plot occurred at 20 degrees C, and in DMD and DMD-carrier preparations an elevated Ca2+-ATPase activity was detected at all temperatures. Above 20 degrees C the activation energy for all types of preparation was the same, whereas below this temperature there appeared to be an elevated activation in DMD and DMD-carrier preparations compared with normal controls. The concept that a generalized alteration in the physicochemical nature of the membrane lipid domain may be responsible for the many abnormal membrane properties reported in DMD is discussed.

Adolescent↗