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.
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.
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+).
The IgA antigliadin antibodies AGA title was detected in 37 patients with IDDM, mean age 32.59 +/- 14.71, where mean duration of disease was 8.76 +/- 9.62 years, and 29 patients with NIDDM, mean age ...55.31 +/- 14.71, where disease lasted 11.5 +/- 5.55 years. A group of 51 normal pts. was employed as control. In IDDM group 2 cases on 37 showed high AGA title (case n. 1 and n. 2) but just the case n. 1 where IDDM lasted 16 years, showed an histologic picture of coeliac disease (partial villous atrophy), while in the case n. 2 where IDDM was at the onset, the histologic picture was normal. The increase of AGA title in the IDDM at the onset is rarely associated with coeliac disease, but it seems to be an aspecific response. Viceversa an increased AGA title is in IDDM for greater than 1 years often associated with coeliac disease. In NIDDM no high AGA title was found. The prevalence of coeliac disease in our patients with IDDM was 1:37 and we suggest that diabetics be screened routinely for antigliadin antibody.
Case report of microencephalia Santa Cruz, G; Frau, G; Faa, G
Zentralblatt fur allgemeine Pathologie u. pathologische Anatomie,
1983, Volume:
127, Issue:
3-4
Journal Article
A case of microencephalia diagnosed in a 34-year-old Sardinian woman is reported. The macroscopic and histologic features of this rare lesion are described in detail. Some interesting histologic ...patterns to the hypothesis of a very early encephalic developmental arrest are discussed.
