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G Perie

Publications and source records attributed to G Perie.

13 recordsLinked to original sources

Histological demonstration of haemosiderin deposits in lungs and liver from victims of chronic physical child abuse.

In the context of chronic physical child abuse, two entities have been described based on macroscopical and radiological criteria: the battered baby syndrome and the shaken baby syndrome. However, in some autopsy cases, clinico-radiological information may not be available. In these cases, histological examinations are necessary to look for sequelae of repeated haemorrhages, particularly in organs likely to have suffered traumatisms such as the lungs, or in organs belonging to the mononucleated macrophage resorption system, such as the liver and the spleen. We examined a series of 15 young children who died from proven chronic child abuse and compared them with 15 sex and age-matched control subjects who died from natural causes with no history of child abuse. Using Perl's stain for iron, we identified haemosiderin deposits in pulmonary, hepatic and splenic samples and the deposits were evaluated qualitatively and quantitatively. Haemosiderin deposits were significantly (P < 0.001) more abundant in the lungs and liver of the chronic abuse victims than in those of the control subjects. However, they were not significantly more abundant in the spleens of child abuse victims than in controls. We conclude that haemosiderin deposits in lungs and liver could be proposed as a marker for chronic physical child abuse. This study stresses the importance of systematic histological examination to look for pulmonary and hepatic haemosiderin deposits in cases in which chronic child abuse is suspected.

Battered Child Syndrome↗

Testicular fibroma of gonadal stromal origin with minor sex cord elements: clinicopathologic and immunohistochemical study of 2 cases.

OBJECTIVE: To report the histologic and immunohistochemical features of 2 cases of intratesticular fibromatous tumors. RESULTS: Microscopically, these tumors were composed of short, randomly interweaving fascicles of spindle cells dispersed within a fibrocollagenous stroma. A sex cord component was detected in one case by microscopic examination and in both cases by immunohistochemical study using MIC2 and anti-inhibin antibodies. CONCLUSIONS: The presence of minor sex cord elements, morphologically or by immunohistochemistry, suggests that these fibromatous tumors are related to and are a subset of sex cord-stromal tumors. Intratesticular fibromatous tumors, of which 11 other cases lacking sex cord elements have been reported, could be considered as the testicular equivalent of ovarian fibroma. These tumors could then be referred to as testicular fibroma of gonadal stromal origin, with or without minor sex cord component.

Adolescent↗

[Angiokeratoma and fucosidosis. Immunohistochemical and ultrastructural study].

INTRODUCTION: Angiokeratoma can lead to diagnoses other than Fabry's disease. We report a case of angiokeratoma in a child with fucosidosis. CASE REPORT: A 7-year-old child with psychomotor retardation presented angiokeratoma located on the penis. Uptake of type I Ulex Europaeus Agglutinin antilectin antiserum was intense in the endothelial structure. This antibody is specific for alpha-L-fucose residues which were thus found in large quantities in the vacuoles of the ultrastructure. The patient also had a major deficiency in leukocyte, serum and fibroblast alpha-fucosidase. COMMENTS: This is a typical case of fucosidosis, a rare hereditary disease with autosomal recessive transmission due to generalized deficiency in alpha-L-fucosidase. Diffuse angiokeratosis should suggest, other than Fabry's disease, fucosidase and other enzyme deficiencies including sialidase, GM1 gangliosidase as well as Kanzaki's disease.

Angiokeratoma↗

[Familial form of bulbo-spinal amyotrophy in the adult].

Three siblings, a male and two females, respectively 47, 51 and 60 years-old, were investigated, since they had, like their father, labioglossopharyngeal paralysis, fasciculations and predominant cervico-facial muscular atrophy. No pyramidal signs were present. The disorder was fatal within 9, 5 and 14 months. In two cases the pathological examination showed a selective involvement of the peripheral motor neuron whereas the central motor neuron was normal. The main abnormalities consisted of abundant chromatolytic neurons and cell loss and were mostly present in the brain stem motor neurons. Immunocytochemical studies with antibodies against neurofilaments and protein-tau, and electron microscopic technics were performed on cervical motor neurons. Neurogenic muscular atrophy was massive in the tongue, less predominant in the diaphragm and psoas and were similar to lesions present in the peroneus brevis muscle. Transmission was autosomal dominant. We suggest that this clinico-pathological syndrome should be considered as a variety of the adult bulbo-spinal muscular atrophies.

Brain Stem↗

[Papaverine hepatitis].

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Chemical and Drug Induced Liver Injury↗