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Biomedical subjects

R Jean

Publications and source records attributed to R Jean.

At least 253 records · Page 14Linked to original sources
PubMedDate not supplied

[Autoimmune panhemopathy in lymphoid hyperplasia in a child].

The case reported concerns a 12 year old boy, admitted in 1970 with a clinical picture evoking a malignant hemopathy: thrombocytopenic purpura, leukopenia, multiple lymphadenopathies, enlarged spleen and, at histological examination, lympho-reticular hyperplasia of the spleen and lymphe nodes, simulating at the beginning, a nodular lymphoma. Immuno-hematological investigations revealed a complex auto-immune picture, which concerned essentially hematopoiesis. In fact, the pseudo-tumoral aspect of lymphenodes, was related to a dysfunction of the immune system, the pathogeny of which is presently better known.

Autoimmune Diseases↗
PubMedDate not supplied

[Persistant pubertal gynecomastia. Biological study (author's transl)].

17 cases of peristant pubertal gynecomastia (which differs from physiologic breast enlargement by their volume and duration) were studied. Plasma estrogens and urinary phenol steroids excretion were found higher than in normal boys. The main biological finding in these subjects was a decrease of the plasma testosterone/estradiol ratio, and a moderate but transient increase in plasma prolactin. LH and FSH were within the normal range. In the mammary tissue, no estrogen receptor was detectable and aromatase activity was in the same range as in adipose tissue. Pathological examination showed a simulataneous proliferation of galactophoric and fibroblastic stroma. Thus, persistant pubertal gynecomastia could be related to an elevated sensitivity of the mammary tissue to a moderate increase of plasma estrogens. Such a sensitivity could be due to an insufficient masculinization of the mammary anlage during the intra-uterine life.

Adolescent↗
PubMedDate not supplied

[Congenital neuropathy caused by hypomyelination].

A 9 year-old girl with congenital hypomyelination neuropathy is reported. Clinical features were delayed motor development, disturbances of gait, deep sensory defect, areflexia and mild distal palsy with claw feet. E.M.G. revealed dramatically diminished motor conduction velocities. C.S.F. protein was normal. Histological examination of the right sural nerve revealed a severe loss of myelinated fibers. On electron microscopy, poorly myelinated and large amyelinated axons were enclosed by normal Schwann cells and surrounded by multiple single or double layered basement membranes which were concentrically arranged in an onion bulb pattern. This case of congenital hypomyelination neuropathy is compared with ten similar cases in the literature.

Child↗
PubMedDate not supplied

[Recurrent hematuria in children].

Twenty-three children with recurrent macroscopic hematuria have been observed. Six children presented with glomerular disorder with mesangial deposits of IgG and IgA (Berger's disease). The hematuria episodes occurred in the 48 hours following an infection, more often respiratory. Renal functions were not impaired. On light microscopy, glomerular lesions were minimal, or were lesions of focal and segmental glomerulonephritis. Ten children seemed to have hereditary nephritis. In 5 cases, nephritis was accompanied by deafness (Alport's syndrome). In 5, nephritis was hereditary but isolated. The electron microscopy examination of basement membranes showed lesions consistent with Alport's syndrome. In cases of hereditary nephritis without deafness, basement membranes were less impaired. In 7 children, no specific etiology was found.

Child↗
PubMedDate not supplied

Lung function after neonatal respiratory distress syndrome.

Functional residual capacity (FRC), dynamic lung compliance (CLdyn) and lung resistance (RL) were measured in a group of 51 healthy children aged 18 months to 6 years (group A) and in a group of 69 survivors of the respiratory distress syndrome (RDS) of the same age (group B). Forty-four per cent of the latter had clinical and radiological abnormalities. In the healthy children significant correlations were found between FRC and height (p less than 0.01), CLdyn and height (P less than 0.01) and CLdyn and FRC (p less than 0.01) but a poor correlation existed between RL and height (p greater than 0.05). There was no significant difference between groups A and B in the slopes of the regression lines for FRC, CLdyn and RL against height, but the intercept for the regression lines relating to CLdyn and RL was lower in group B than in group A. Group B was divided into two subgroups according to the length of exposure to artificial ventilation with an FIO2 in excess of 60 %. A lower value for CLdyn was found in the sub-group with the longer exposure (greater than 120 hours) to a high FIO2, suggesting that this is an additional factor in causing lung damage.

Airway Obstruction↗