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Biomedical subjects

P Babin

Publications and source records attributed to P Babin.

At least 91 records · Page 5Linked to original sources
PubMed1982

Jejunal IgA and C3 deposition in adult Henoch-Schönlein purpura with severe intestinal manifestations.

An adult male patient with Henoch-Schônlein purpura and severe intestinal involvement is reported. Henoch-Schönlein purpura was documented by microscopic and immunofluorescent studies of the skin and kidney. At duodenojejunoscopy there were multiple ulcerations and thickening of mucosal folds. Microscopic and immunofluorescent studies of duodenum and proximal jejunum showed submucosal vasculitis and granular deposits of immunoglobulin A and complement components (C3) in the vessel walls, similar to those observed in the skin and kidney. This patient exhibited severe and prolonged intestinal lesions of Henoch-Schölein purpura, and was resistant to a 10-day course of total parenteral nutrition. However a dramatic clinical response was observed within 24 h after the initiation of plasma exchange therapy; steroids were not used. Intestinal involvement in anaphylactoid purpura may be associated with local immunologic abnormalities similar to those observed in cutaneous vessels and glomerular mesangium.

Adult↗
PubMed1980

[Oculo-cutaneous tyrosinosis (Richner-Hanhart syndrome). Histo-pathological study of a case].

The Richner-Hanhart syndrome corresponds to a tyrosine elevation in serum due to deficit in soluble tyrosine aminotransferase in liver cells. This new enzymopathy which is transmitted in an autosomal recessive mode is called oculocutaneous tyrosinosis. It is curable by a poor diet in tyrosine and its precursors. The diagnosis has been invoked in a 18 months old girl, on the association of punctuate palmar and plantar keratosis, dentritic ulcerated keratitis, and mental retardation. The diagnosis is confirmed by elevation of tyrosinemia to 52 mgs/100 mls associated with a high urinary elimination of tyrosine and plenylcetonic acid. Absences of anomaly in the metabolism of methionin and hepatorenal absence of disturbance of hepatorenal system is characteristic. The keratosis accompany orthokeratotic hyperkeratosis. The keratinocytes show 2 types of anomaly ranged in strates in the epiderm. Intracytoplasmic vacuoles which include or lead to pseudomyelinic formations extend progressively from the mitochondrial alterations in the epidemial basal layers. Bulky polyhedral electron dense particles are found in the cytoplasm of the superficial keratinocytes. Most of these aspects have been demonstrated anteriorly in the keratinocytes and the cornea; on the other hand, signs of mitochondrial sulferance had not been observed. The genesis of these cellular alterations based on the liberation of lysosomial enzymes by the action of crystals of tyrosine has been suggested by Goldsmith from experimental facts. However, it seems the mitochondrial defect occurs outside this mechanism.

Female↗
PubMed1980

Nodular lymphoid hyperplasia of the small bowel associated with primary jejunal malignant lymphoma. Evidence favoring a cytogenetic relationship.

A nonimmunodeficient patient with diffuse nodular lymphoid hyperplasia of the small bowel and a jejunal malignant lymphoma of mixed lymphocytic-histiocytic type is reported. Surface marker and immunohistologic studies of the malignant lymphoma and of histologically benign lymphoid nodules proximal to the tumor showed a similar pattern of monoclonality (IgM-kappa) and gave suggestive evidence of a cytogenetic relation of the lymphoma to nodular lymphoid hyperplasia. It is suggested that intestinal nodular lymphoid hyperplasia may be a condition leading to lymphoid malignancy; its exact incidence in patients with both malignant lymphoma of the gut and a primary immunodeficiency syndrome should warrant further consideration.

Adolescent↗
PubMed1979

Peutz-Jeghers syndrome with metastasizing carcinoma arising from a jejunal hamartoma.

The association of Peutz-Jeghers (P-J) syndrome and gastrointestinal carcinoma is well documented, but an unequivocal histologic demonstration that malignancy may originate in a hamartomatous polyp has been very rarely given. A patient with the P-J syndome is described, in whom a definite intestinal adenocarcinoma with metastases to omentum and celiac lymph nodes was shown to originate in a jejunal hamartoma. Evidence that malignancy was derived from hamartomatous structures was given by the following observations: (a) Adenocarcinoma was intimately intricated with smooth muscle bands, and well-defined transitional zone of malignant cells could be observed in several glands of the degenerated P-J polyp; and (b) close to malignant areas, glands of this polyp exhibited a less-differentiated epithelium, but were still intermixed with nonstriated muscle bundles, which strongly suggest dedifferentiation of hamartomatous structures.

Adenocarcinoma↗
PubMed1978

[Hypocomplementaemic leucocytoclasic vasculitis. (Mac Duffie's syndrome). One case with bacterial lymphadenitis (author's transl)].

Mac Duffie's syndrome includes a hypocomplementaemic allergic vasculitis with essentially cutaneous and articular manifestations. Its pathogenesis, still incompletely elucidated, involves the precipitation of immune complexes in the walls of the all vessels. The problem remains as to whether the alteration in the complement system is merely a reflection of this formation of immune complexes, or if it is primary, favourising the chronic infections which produce such complexes. The significance of the presence of low molecular weight precipitins reacting with C1g in the serum of a number of patients remains uncertain. The case reported, with its new clinical features, does not provide an answer to these questions but emphasises the possible role of chronic infections in the origin of this syndrome.

Adult↗
PubMed1978

[Massive digestive hemorrhage caused by arterial malformation of the jejunum. Diagnostic value of emergency arteriography].

A case of massive digestive bleeding due to an arterial abnormality of the jejunal wall is reported. Its interest is twofold: a) After a complete endoscopic and operative examination of the GI tract proved negative, an emergency superior mesenteric angiography was the only investigation which allowed to recognize the site and mechanism of the bleeding; b) from a nosologic point of view, the clinico-pathological picture was entirely similar to that of "Dieulafoy's erosion", a syndrome which has been only described, up to now, in the gastric mucosa.

Adult↗
PubMed1978

Jejunal bleeding from a solitary large submucosal artery. Report of two cases.

Two young patients with massive intestinal bleeding resulting from a solitary submucosal arterial abnormality of the jejunum are reported. In one case, the bleeding lesion was localized only by emergency abdominal angiography, while a previous exploratory laparotomy proved negative. The vascular lesion encountered in both patients consisted of a wide caliber artery lying in the jejunal submucosa; there was no evidence of true aneurysm on serial sections. The clinicopathological picture closely resembles that of lesions previously described in the stomach under the name of "gastric submucosal aneurysms" in the English literature, or of "gastric Dieulafoy's erosion" in the French literature. It is suggested that the vascular lesions herein reported should be called "solitary large submucosal artery" or "Dieulafoy-like erosion" of the jejunum.

Adult↗
PubMed1976

[Diabetes mellitus in pheochromocytoma].

The frequency of latent disorders of glucose regulation during pheochromocytoma, is evaluated at 75% of cases. Detailed analysis of 83 cases with a diabetic state, gave the following results: insulin dependent diabetes, 37 cases. Non-insulin dependent, 14 cases. Latent diabetes, 32 cases. The characteristics of the insulin-dependent diabetes were not always suggestive. Insulin dependency was, however, unusual above a certain age. We noted loss of weight in spite of good control of the diabetes, the absence of acidosis and ketosis contrasting with rapid loss of weight. In fact, it is above all the hypertension which should lead to diagnosis. Surgical operation, cures or improves considerably the diabetic state, thus proving the symptomatic nature of this diabetes.

Catecholamines↗