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Biomedical subjects

M Gabriel

Publications and source records attributed to M Gabriel.

At least 91 records · Page 5Linked to original sources

[Surgical therapy of primary hyperparathyroidism in newborn infants].

This is a report on a neonatal primary hyperparathyroidism (NPHPT) treated by total parathyroidectomy and autotransplantation of parathyroid tissue into the forearm musculature. Histologically, the glands were composed of a "chief cell hyperplasia" of the parathyroid tissue. Clinical manifestation in neonates and differential diagnosis of HPT are discussed. Indication for operation and surgical procedure are described.

Humans↗

Class-specific antibody response to pneumococcal capsular polysaccharides in men infected with human immunodeficiency virus type 1.

We characterized the effect of infection with human immunodeficiency virus type 1 (HIV) on levels of total immunoglobulins and pneumococcal vaccine-specific immunoglobulins in 28 heterosexual and 25 homosexual men seronegative for HIV; 27 asymptomatic, seropositive homosexual men; and 21 patients with AIDS. Total serum IgG levels were increased in both HIV-seropositive groups compared with the HIV-seronegative men (P less than .001). Total IgM levels, however, were elevated only in the asymptomatic, HIV-seropositive men (P less than .08); total IgA levels were elevated only in the patients with AIDS (P less than .05). Vaccine-specific serum IgG, IgM, and IgA significantly increased over baseline three and six weeks after immunization in all groups (P less than .05). Responses to vaccine among the HIV-seronegative groups were similar but were greater for all antibody classes than were responses among the HIV-seropositive groups (P less than .05).

Acquired Immunodeficiency Syndrome↗

Long-term follow-up of patients treated with VVI pacing and sequential pacing with special reference to VA retrograde conduction.

The aim of this prospective study is comparing long-term prognosis in patients implanted with a VVI pacemaker (group A) with those implanted with a sequential pacing device, AAI or DDD, (group B). Both groups of 45 patients each, were comparable as regards to age, sex, pacing indications, underlying heart disease, and technical conditions of implantation and were followed-up over 55 months. Atrial arrhythmias (A.A.) incidence was higher in group A: 24.4% than group B: 8.8% (P less than 0.05). Arterial embolisms (A.E.) occurred in group A patients only. Worsening or occurrence of exercise limitation was more frequent in group A: 35.6% as compared to group B: 13.3% (P less than 0.05) and deaths related to these complications, occurred in seven cases in group A versus four cases in group B. In group A, all patients who experienced a worsening or occurrence of an A.A. or an A.E., had a ventriculoatrial conduction (VAC). No statistical difference was observed in worsening or occurrence of exercise limitation between patients with VAC and those without VAC: nine (42.8%) and seven (29.2%) but they respectively experienced at least one complication in 16 cases (76.2%) and seven cases (29.2%) (P less than 0.01). In conclusion, long-term prognosis in patients implanted with VVI pacing as compared to patients implanted with sequential pacing is poorer. The presence of VAC in patients treated with permanent VVI pacing is a major factor for complications and deaths related to A.E. and cardiac failure. Thus VVI pacing should be avoided in patients with VAC.

Arrhythmias, Cardiac↗

[Van der Woude syndrome in combination with ring chromosome 18].

7-month-old girl with a combination of van der Woude syndrome and ring chromosome 18 showed the following malformations: medial palate cleft, fistulas of the lower lips, hypertelorism, epicanthus, antimongoloid position of the eyelids, a small hymenal appendix, plantar placing of the third toe, atrial septum defect, stenosis of the auditory canal. The combination of fistulas of the lower lip and cleft lip or palate already established the diagnosis of Van der Woude Syndrome. Because of the additional malformations observed a chromosome analysis was performed which demonstrated a ring chromosome 18. The phenotypic features of ring-chromosome 18, a 18p-/18q- syndrome, vary according to the extend of deletion of the short or long arm. However, all patients show craniofacial dysmorphism, intellectual and developmental retardation, defective hearing, speech disorder, and more than 50% have stenosis or atresia of the auditory canal.

Abnormalities, Multiple↗

Posterior cingulate cortical lesions eliminate learning-related unit activity in the anterior cingulate cortex.

The frequency of multi-unit neuronal firing in response to tonal conditional stimuli increases, and the neuronal responses become discriminative in character, in the anterior and posterior subfields of the cingulate cortex (Brodmann's Area 24 and 29, respectively) during the course of discriminative avoidance conditioning in rabbits. Bilateral electrolytic lesions of the dorsal subicular complex eliminated the training-induced excitatory response in both cortical subfields, suggesting that subiculocortical projection fibers are essential for this effect. Yet, available data indicate that the subicular projection reaches only Area 29, not Area 24. Here we test the hypothesis that the subicular influence attains Area 24 via a synaptic relay in Area 29. The results showed in keeping with the hypothesis, that bilateral aspirative or electrolytic lesions in Area 29 eliminated the training-induced neuronal responses in Area 24. As in the case of subicular lesions, the Area 29 lesions increased the amplitude of the macropotentials in Area 24. These results are discussed in relation to a model of the interactions of the cingulate cortex, hippocampus and the limbic thalamic nuclei during learning.

Animals↗

Hippocampal control of cingulate cortical and anterior thalamic information processing during learning in rabbits.

Past studies of the neural determinants of discriminative avoidance conditioning in rabbits have fostered a theoretical model that describes the interactive functioning of the cingulate cortex (Brodmann's Areas 24 and 29), the anterior ventral and medial dorsal thalamic nuclei (AVN and MDN) and the hippocampus. Here we test hypotheses of the model concerning the influence of the hippocampus on cortical and thalamic information processing. The rabbits learned to perform locomotory conditioned responses (CRs) in an activity wheel in response to an acoustic (pure tone) positive conditional stimulus (CS+). A shock unconditional stimulus (US) was given 5 s after CS+ onset, but locomotion during the CS+ - US interval prevented the US. The rabbits also learned to ignore a second tone (a negative conditional stimulus, CS-) of different auditory frequency than the CS+, that did not predict the US. Multi-unit activity and intracranial macropotentials were recorded in the cingulate cortex and the AVN during acquisition, overtraining, extinction, reacquisition and reversal training. Data were obtained in intact rabbits and in rabbits with bilateral lesions of the subicular complex, the origin of projections of the hippocampal formation to the cingulate cortex and AVN. In addition, the activity in the AVN was recorded in a separate group of rabbits with posterior cingulate cortical (Area 29) lesions. Subicular and Area 29 lesions were associated with an enhancement of the training-induced CS+ elicited neuronal response in the AVN. The frequency of CRs was enhanced in animals with subicular lesions. CS elicited unit responses in the cingulate cortices were attenuated in rabbits with subicular lesions. Both of the lesions were associated with significantly increased amplitudes of the CS elicited average cortical and thalamic macropotentials. These results suggested the following conclusions: subiculocortical afferents provide an enabling influence that is essential for CS elicited excitation in the cingulate cortex; the cingulate cortical excitatory response in intact animals exerts a limiting influence on the activity in the AVN; the enhanced AVN neuronal response in rabbits with lesions is due to the absence of the limiting influence and it contributes to the increased CR frequency in those animals. It is hypothesized that the hippocampus via subiculocortical projections, governs the flow of CR-inducing thalamocortical excitatory volleys. This governance determines the timing of CR output. The results of hippocampal processing of contextual information acting through the subiculocortical projection determines the moment most appropriate for the CR.

Acoustic Stimulation↗

Melioidosis: a serological survey in a tuberculosis sanatorium in Hong Kong.

A serological survey of 275 Chinese patients with underlying pulmonary diseases in a tuberculosis sanatorium in Hong Kong showed that 39 (14%) had haemagglutinating antibody (HA) against Pseudomonas pseudomallei in a titre of 1: 80 or above. Only 9 of these 39 patients had travelled to endemic areas, suggesting that at least 30 patients (11%) had been exposed to Ps. pseudomallei locally. Females are affected as often as males, and the seropositive rate is the same whether patients are immunosuppressed or not. Because subclinical melioidosis is prevalent and HA may persist for a long time, even at a high titre, after infection, determination of HA alone cannot differentiate between active melioidosis and its masquerade--active tuberculosis.

Adolescent↗

Unexpected findings amongst the skin test responses to mycobacteria of BCG vaccinated Kuwaiti school children.

A multiple skin test survey was carried out in Kuwait on 1200 school children aged 8-11 years, and on 1228 children aged 12-16 years. With only 15 exceptions, all these children had received vaccination with Japanese BCG just before they started school, 5 years and 9 years earlier respectively. Tuberculin positivity was almost 90% in both groups, with a mean response size of 8.7 mm. This was associated with remarkably high responsiveness to many of the other mycobacterial species investigated. Since this high reactivity was also to Mycobacterium ulcerans, a species most unlikely to be present in Kuwait, it is proposed that this might be due to responsiveness to group ii antigen which is present in all slow growing species. Only M. flavescens and M. rhodesiae amongst the fast growing species, were absent as sensitising organisms. After correction for the supposed reactivity to group ii antigen, M. avium B, M. gordonae, M. ulcerans and M. xenopi amongst the slow growing species, also appeared to be absent from the Kuwait environment. The species most commonly encountered were M. leprae, M. chitae, M. neoaurum, M. diernhoferi, and M. vaccae in this order. This was a remarkable finding for a country assumed to be poor in contact with environmental species, and known to have a very low prevalence of leprosy. As previously reported from Iran, but not confirmed in other places, there was a 95% correlation between responsiveness to Leprosin A and Vaccin. Amongst the slow growing species M. avium A, M. intracellulare, and M. kansasii appear to be frequent sensitising agents, in common with many other places.

Adolescent↗

Anterior thalamic discriminative neuronal responses enhanced during learning in rabbits with subicular and cingulate cortical lesions.

The neuronal discharge that develops in the anterior ventral thalamic nucleus (AVN) in response to a task-relevant stimulus during learning is enhanced in rabbits with damage in the cingulate and subicular (hippocampal) cortical areas that project to this nucleus. These results indicate that the corticothalamic projections limit the anterior thalamic response but they do not appear to contribute causally to its development. Thus, the discriminative neuronal response is owed to interactions that occur wholly within the subcortical domain of the AVN. The production of this neural code for significant stimuli may be the principal function of the AVN. The enhanced thalamic discharges may be a neural cause of behavioral hyperactivity in animals and amnesia in humans with hippocampal damage.

Action Potentials↗

Neuropathologic and morphometric studies in hereditary motor and sensory neuropathy type II with neurofilament accumulation.

Histological, electron microscopic and morphometric data on sural nerve, muscle, and skin biopsies of three patients affected by autosomal dominant hereditary motor and sensory neuropathy type II with neurofilament accumulation, whose neurological, cardiological and electrophysiological data have been provided in a previous paper disclosed focally enlarged myelinated axons, due to aggregation of neurofilaments in sural nerves of all 3 biopsied patients, as well as densely packed clusters of filaments in occasional non-myelinated axons without axonal enlargement, in several fibroblasts and endothelial cells in muscle and particularly in skin. This accumulation of filaments was less pronounced in our patients' tissues than in autosomal-recessive GAN. No ultrastructural differences concerning the accumulated filaments appear to exist between the affected cells of our patients and GAN. Taken together, these findings best fit a hereditary motor and sensory neuropathy type II with focal accumulation of intra-axonal neurofilaments.

Axons↗

Effect of age on homovanillic and 4-hydroxy-3-methoxymandelic acid levels in plasma.

The plasma levels of homovanillic and 4-hydroxy-3-methoxymandelic acids have been determined in 524 normal subjects ranging from birth to 49 years of age using a mass fragmentographic method. The mean concentration of homovanillic acid in plasma declines exponentially from the age of 1 day (X = 2342.0 nmol/l, 426.6 ng/ml) to adulthood (X = 60 nmol/l, 10.9 ng/ml). 4-Hydroxy-3-methoxymandelic acid behaves in a similar although not so extreme manner (392 nmol/l, 77.6 ng/ml-50 nmol/l, 10 ng/ml).

Adolescent↗

Spinal tuberculosis presenting with abdominal symptoms--a report of two cases.

We present two unusual cases of tuberculosis of the spine presenting with abdominal symptoms; both patients underwent exploratory laparotomy before the true nature of the diagnosis became known. The diagnosis of spinal tuberculosis was evident on the pre-operative abdominal radiographs but this was overlooked.

Adult↗

Brain stem reticular formation neuronal correlates of stimulus significance and behavior during discriminative avoidance conditioning in rabbits.

Multiple-unit activity in the reticular formation of the midbrain and pons was recorded in rabbits during discriminative conditioning of locomotor (wheel-running) avoidance behavior. The conditional stimuli (CS+ and CS-) were pure tones of different auditory frequency, and the unconditional stimulus (US) was a constant-current footshock (1.5-2.5 mA) delivered through the grid floor of the wheel. The pontine, but not the midbrain, sites manifested development during behavioral acquisition, of brief-latency (10-40-ms) discriminative neuronal discharges (i.e., greater discharges to the CS+ than to the CS-). The greatest magnitude of the discriminative discharges in the rostral pontine loci occurred in the first conditioning session. The discriminative response in the caudal loci developed more slowly, and it persisted to the criterial stage of training. Both rostral and caudal pontine loci, during the interval from CS onset to US onset, manifested a progressive build-up of neuronal firing in anticipation of the behavioral response. The occurrence in the rostral and caudal pontine areas, respectively, of early- and late-developing discriminative discharges is analogous to effects observed in past studies in the limbic mesocortical and thalamic systems. These findings provide a basis for establishing the possible functional relatedness or independence of these analogous effects.

Animals↗

[Angelman syndrome].

An eight-year-old boy with Angelman-(Happy Puppet-)Syndrome is described. Nearly all typical symptoms of the syndrome, especially severe psychomotoric retardation with spontaneous outbursts of laughing and protrusions of the tongue, athetoid movements, typical electroencephalogram and microcephaly, could be found in our patient. The incidence of the Angelman-syndrome may be underestimated.

Abnormalities, Multiple↗

Choledochal cyst type I: successful endoscopic balloon dilatation of the distal common bile duct and sphincter of Oddi: a case report.

A 12-year-old boy with a choledochal cyst type I is reported with clinical features of abdominal pain, postprandial vomiting, weight loss and fatigue since 3 months. Diagnosis was made by ultrasound and confirmed by computer-assisted tomography (CAT) and endoscopic retrograde cholangiography (ERC). Instead of the usual surgical treatment balloon dilatation of the sphincter of Oddi and the distal common bile duct was performed by endoscopic means. The patient was relieved from his complaints, gained weight and felt healthy again. Sonographic and endoscopic reexamination had proven that the diameter of the common bile duct had diminished from 3,0 to 2,6 cm. Endoscopic balloon dilatation of choledochal cysts type I may be a valuable alternative to surgical treatment.

Ampulla of Vater↗

Cryptic miliary tuberculosis.

Fifteen patients with cryptic miliary tuberculosis seen over a six-year period in a large teaching hospital were reviewed. This form of tuberculosis tended to be difficult to diagnose and was most common in older people and those with underlying diseases such as malignancy or blood dyscrasias. Bacteriological investigation was of the little help in diagnosis while bone marrow and liver biopsies were more useful. The diagnosis was made during life in only seven (47 per cent) of the 15 cases, and the overall mortality was 80 per cent. Tuberculosis should be considered in all cases of pyrexia of unknown origin, and investigations performed to establish or exclude the diagnosis. A prompt and adequate therapeutic trial with antituberculous treatment even in the absence of definite evidence of tuberculosis can be life-saving.

Adolescent↗

Hereditary motor sensory neuropathy type II with neurofilament accumulation: new finding or new disorder?

Peroneal muscular atrophy is now known to be heterogeneous and to be due to various underlying genetic mechanisms. Exploring this heterogeneity further, we report on a German kinship with the clinical, genetic, and nerve conduction features of hereditary motor and sensory neuropathy type II (HMSN type II) but whose sural nerves on biopsy were found to show infrequent axonal swellings with neurofilament accumulations not previously described. The dominant inheritance and absence of kinky hair set this disorder apart from giant axonal neuropathy. There was no history of toxic exposure to industrial chemicals. We conclude that the disorder either is a new type of HMSN or is HMSN type II with previously unencountered neurofilament accumulations. Neurofilament accumulation indicates that the axon could be a site for primary derangement and may implicate an abnormality of slow axonal flow. In addition, some of the patients exhibited features suggestive of a cardiomyopathy.

Adolescent↗