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Biomedical subjects

G Frau

Publications and source records attributed to G Frau.

33 records · Page 2Linked to original sources

[Neuromuscular and vascular hamartoma of the small intestine. Report of a clinical case and review of the literature].

We present the case of a 50-year old patient, operated on for three adjoining annular strictures in the jejunum with proximal dilatation. He presented with a one-year history of recurrent cramping in the upper abdomen and vomiting, with two episodes of intestinal obstruction. Microscopic examination of the stricture revealed, in the submucosa, disorganised fascicles of smooth muscle derived from the muscularis mucosae, bundles of non-myelinated nerve fibres with scattered abnormal ganglion cells and haemangiomatous vessels. The pathological findings observed were similar to those described as neuromuscular and vascular hamartoma, a rare stricturing condition of the small intestine. Many authors have questioned the hamartomatous nature of this disorder, since identical features may be seen in Crohn's disease, in ischaemic enteritis, in radiation enteritis and in non-steroidal antiinflammatory drug-induced small intestinal strictures. On the basis of a review of the 5 previously described cases and of our own experience, we believe that neuro muscular and vascular hamartoma of the small bowel should be considered as a distinct entity if histological hallmarks of Crohn's disease are absent, in patients with no history of gastrointestinal disease, or of chronic ingestion of non-steroidal antiinflammatory drugs.

Hamartoma↗

[Acute disseminated histiocytosis X. Presentation of a case].

The AA. report a case of acute, diffuse histiocytosis X with an unfavourable course observed in a child of 33 months. The histological diagnosis, formulated on the basis of specimen taken from a skin nodule, was confirmed by ultrastructural observation of Birbeck bodies in the cytoplasm of proliferating Langerhans cells.

Acute Disease↗

[Osteomatosis of the skin. Presentation of 2 cases].

Osteomatosis cutis. Report of two cases. The AA. reported two cases of osteomatosis cutis in a child three years old and in a girl sixteen years old. In both cases there was no history of previous trauma and acne. Microscopic study revealed islands of true bone, having lamellar configuration, periosteum, marrow cavities with endosteum, lacunae and canaliculae. No other calcified tissue and cartilage were seen.

Adolescent↗

[Osteoclastoma-like giant cell tumor of the lung].

Osteoclastoma-like giant cell tumor of lung. A case of osteoclastoma-like giant cell tumor of the lung is reported. This is an unusual tumor too described in the heart, thyroid, skin, soft tissues and pancreas. AA. studied the tumor's structure and are of opinion that it concerns a primitive nonepithelial neoplasm of the lung.

Adult↗

[Syndactylia associated with multiple malformation syndromes. Observation of a new symptomatologic complex in 3 brothers].

Hand and feet malformations are often part of complex malformation associations. The present paper reports on a family whose three sibs (two males and one female) are affected with symmetric soft tissue syndactyly involving both fingers and toes, fifth finger clinodactyly, a pattern of dysmorphism including down slanting palpebral fissures, long flat nasal saddle, out turned nostril openings ("Greek warrior helmet"-like profile), dysplastic teeth, and, in addition, severe growth retardation, microcephaly, severe mental deficiency with immaturity of cerebral activity of EEG, hypergonadotropic hypogonadism and some skeletal anomalies. All cases show large secondary constriction in one of the chromosome 1 pair (1qh+).

Abnormalities, Multiple↗

Congenital hepatic dysplasia from cytomegalovirus.

The Authors report on a case of liver dysplasia in a four-month-old infant affected by prenatal cytomegalovirus infection. Immunologic, histologic and ultrastructural studies suggest an embryopathic origin of the lesions.

Cholestasis, Intrahepatic↗