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Biomedical subjects

M Toppet

Publications and source records attributed to M Toppet.

At least 37 records · Page 2Linked to original sources
PubMed1990

Corticosteroids in primary tuberculosis with bronchial obstruction.

The usefulness of prednisolone in combination with the modern potent antituberculous drugs has been studied in 29 children with primary lung tuberculosis and hilar adenopathy causing bronchial obstruction. These children were divided at random in two groups of 15 and 14 patients. Both groups were treated similarly except that one group received prednisolone. Both groups were very similar before the onset of treatment for most variables. Tuberculous infection healed in both groups but the group on steroids improved earlier and had significantly fewer complications, both on radiography and bronchoscopy. Only two of the patients on steroids still had progressive lesions: a very young baby probably because he developed two severe viral infections consecutively, and another infant of 7 months whose treatment was unreliable, as the parents were not very compliant. Some patients initially not treated with prednisolone improved only after it was given. Prednisolone treatment is not recommended when the reliability of the treatment cannot be guaranteed, as the hazard of harm would exceed the expected benefit.

Adolescent↗
PubMed1989

[Nanism with short limbs, dysmorphism, renal dysplasia, growth hormone deficiency with pituitary hypoplasia and psychomotor delay: a new syndrome?].

The authors present the case of a male infant affected with short limbed dwarfism already detected in utero by ultrasound. In addition, facial dysmorphism, bilateral hypoplasia and dysplasia of the kidneys with altered renal function, hypotonia and non-evolutive developmental delay are noted. Endocrine tests show a severe and isolated growth hormone deficiency. Metabolic investigations are negative, including peroxisomal functions. Prometaphase chromosomes are normal. Bone x-rays reveal generalized osteoporosis with absence of post-natal osseous maturation and the presence of wormian bones. Major pituitary hypoplasia is demonstrated by nuclear magnetic resonance. The present observation is compared to the case reported by Stratton & Parker (Am. J. Med. Genet., 1989, 32, 169-173). Available data do not allow either to affirm or to exclude the identity of the syndromes presented by these 2 isolated cases.

Abnormalities, Multiple↗
PubMed1988

Unusual ventilation-perfusion patterns in primary lung tuberculosis.

We report two cases of primary lung tuberculosis in children with unusual perfusion ventilation scintigraphic patterns. In the first case, a mismatch in the right upper lobe suggests an elective compression of the bronchi by the mediastinal lymph nodes; in the second case, the total absence of ventilation and perfusion of the left lobe at scintigraphy illustrates the discrepancy sometimes encountered between chest x-ray and lung scintigraphy.

Airway Obstruction↗
PubMed1988

[Sequential development of vitamin D metabolites under isoniazid and rifampicin therapy].

A sequential study of 25-hydroxy vitamin D (25-OH-D), 1.25 dihydroxy vitamin D [1.25 (OH)2-D], PTH, alkaline phosphatase and gammaglutamyl transpeptidase (gamma GT) was undertaken in a series of 46 children with asymptomatic tuberculosis treated by isoniazid (INH) alone or associated with rifampin (RMP). These parameters were measured before treatment, 1 month, 3 months after the onset and at the end of treatment (6 months). In order to reduce the influence of the time of the year on the 25-OH-D levels, 22 patients were selected for whom the whole treatment took place between October and May of the following year. In this group, 13 children were treated by INH and RMP, 9 by INH alone. A statistically significant decrease in 25-OH-D levels could be demonstrated after 3 months of treatment in 13 patients under INH and RMP as well as a significant increase in alkaline phosphatase and gamma GT levels. In 9 patients given INH alone, 1.25 (OH)2-D levels decreased after 3 months without significant changes in 25-OH-D, alkaline phosphatase or gamma GT levels. These results emphasize the need for regular biochemical supervision, even if no sign of rickets is observed in these patients.

Adolescent↗
PubMed1987

Neutrophil dysfunctions in thalassaemia major: the role of cell iron overload.

The susceptibility to infections was recorded in 13 patients with beta thalassaemia major (T.P.). The following parameters were also investigated in their polymorphonuclear neutrophils (PMN): nitro blue tetrazolium (NBT) reduction, heated yeast and Escherichia coli phagocytosis, Escherichia coli killing and myeloperoxydase activity. These results were compared to those obtained in healthy controls (H.C.). The Perls's reaction was performed on PMN and graded according to a scoring system with the aim of quantifying the iron intoxication of PMN. Phagocytosis and Perls's reaction of PMN from H.C. were also studied after 20 h of incubation with thalassaemic serum. 6 T.P. out of 13 developed septicaemia during their lifetime and in all 9 septicaemic episodes were noted. Phagocytosis was greatly impaired, disclosing both cellular and serum abnormalities. The mean percentage of Perls's positive PMN was 13% in T.P., contrasting with the constant negative reaction in H.C. The incubation of PMN from H.C. with serum from T.P. induced the simultaneous appearance of a phagocytosis defect and of a positive Perl's reaction. It was concluded that in beta thalassaemia major the phagocytosis of PMN was altered due to a combination of serum and cellular abnormalities and that both may be related to the iron overload.

Adolescent↗
PubMed1985

[Course of serum alkaline phosphatase during treatment with isoniazid and rifampicin].

In a population of 132 tuberculous children coming from immigrant families, statistical analysis of data showed a significant increase in serum alkaline phosphatase levels. This phenomenon is more significant when several antibiotics are used (Isoniazid and Rifampicin) than when monotherapy is used (Isoniazid). These results demonstrate a high tendency to occult osteomalacia in tuberculous patients treated by tuberculostatic drugs.

Adolescent↗
PubMed1983

Circulating vitamin D metabolite concentrations in children with nutritional rickets.

Serum calcidiol, calcitriol, and 24,25-dihydroxyvitamin D concentrations were measured in 20 children with vitamin D-deficiency rickets. Vitamin D metabolite concentrations were measured in 17 of 20 patients before treatment and in 14 of 20 patients after vitamin D administration. Conclusions are as follows. (1) Before treatment, serum calcidiol seems to be the best criterion of D deficiency, as it was low (less than 8 ng/ml) in 15 of 17 studied children, whereas calcitriol and 24,25-dihydroxyvitamin D concentrations ranged from undetectable to high values (350 pg/ml and 5.9 ng/ml, respectively). (2) Low calcidiol concentrations may occur despite recent vitamin D intake: low serum values were found in children given vitamin D2 up to two months after the onset of therapy (50 micrograms/day). (3) Elevated calcitriol serum concentrations were observed in all children after initiation of vitamin D therapy; these high concentrations persisted for four weeks or more, even after normalization of serum calcium, phosphorus, and parathyroid hormone values. (4) Healing of biochemical abnormalities can occur even in children with low circulating concentrations of calcidiol and 24,25-dihydroxyvitamin D.

25-Hydroxyvitamin D 2↗
PubMed1982

Fanconi's anaemia. Simultaneous onset in 2 siblings and unusual cytological findings.

Fanconi's anaemia is reported in 2 siblings. The simultaneous onset of pancytopenia after possible exposure to common external agents suggest that both a frail genotype and environmental factors may be etiologically involved in the disorder. One of the children died after having developed a monoblastic leukaemia. This patient disclosed very striking cytological abnormalities, including a dyserythropoietic pattern and ropalocytosis. The physiopathology of the latter abnormality, which has hitherto not been noticed in Fanconi's anaemia, is uncertain.

Adolescent↗
PubMed1981

Significance of persisting IgM anti-HBc antibodies in hepatitis B virus infection.

Igg and IgM antibodies to the core antigen of hepatitis B virus (HBV) were measured in 136 patients who developed acute HBV hepatitis and who were followed prospectively. After acute hepatitis all the patients developed transiently IgM anti-HBc lasting for two to five months. In contrast, IgM anti-HBc persisted 8 and 9 months in two patients who developed persistent hepatitis and were continuously detected for two years in nine patients who developed aggressive hepatitis. The results suggest that the determination of IgM anti-HBc might be useful to predict the outcome of chronic hepatitis B infection.

Acute Disease↗
PubMed1978

Farber's disease as a ceramidosis: clinical, radiological and biochemical aspects.

A case of Farber's disease associated with athyreosis is reported in a Belgian infant born from consanguineous parents. A detailed clinical observation made from the early onset of symptoms until death of the patient at age of 22 months, together with radiological, morphological and biochemical data confirmed the diagnosis of Farber's disease and its specific storage process. Cultured fibroblast studies disclosed an abnormal catabolism of ceramides, presumably related to the deficiency in lysosomal ceramidase. Family history confirms that the disease is inherited as an autosomal recessive trait.

Ceramides↗
PubMed1977

Oculocraniosomatic neuromuscular disease with hypoparathyroidism.

During a six-year period, an adolescent girl developed a polyglandular disease characterized by hypoparathyroidism, chemical diabetes, growth failure and pubertal delay, hypercholesterolemia, and hypomagnesemia. A slowly progressive neurological disorder occurred simultaneously, consisting of progressive external ophthalmoplegia, mitochondrial myopathy, ataxia, neural deafness, mental subnormality, atypical retinitis, corneal dystrophy, cataract, and increased protein level in the cerebrospinal fluid. An intracardiac conduction defect was also found. This disorder, the cause of which is uncertain, is termed oculocraniosomatic disease. Our patient is apparently unique in that there was an associated hypoparathyroidism.

Adolescent↗