[Hereditary renal adysplasia. Apropos of 2 families].
Two families with hereditary renal adysplasia are reported. Transmission is dominant autosomal. There were no genital abnormalities.
Biomedical subjects
Publications and source records attributed to C Pignodel.
Two families with hereditary renal adysplasia are reported. Transmission is dominant autosomal. There were no genital abnormalities.
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Discovery of Dirofilaria (Nochtiella) repens during a pancreatectomy in the adipose tissue of the pancreas. Brief description of the worm and transverse sections which show longitudinal ridges of the cuticule (photo no 4). Clinical history, pathological anatomy study of liver; mesenteric infarction and patient's death two weeks after the operation. Parasitosis known in the South of France near at hand littoral pools but any visceral topographical diagnosis had never been signaled in France.
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Report of a 54 year-old man with a carcinoid tumor in the larynx and cervical lymph node metastasis. This left sided and dum-bell shaped tumor was spreading from the vocal cord to the piriform sinus. It was smooth, reddish and rubbery. Microscopic study showed, near by the mucosa, the tumor cells arranged in a trabecular pattern, but more disseminated and infiltrating in the deepness. Grimelius stain was positive, Masson Fontana stain negative. Electron microscopy showed neuro-secretory type granules. Immuno-histo-chemical study revealed irregularly scattered cells which were positive for ACTH, but negative for somatostatin, calcitonin and gastrin. Review of literature for this extremely rare tumor.
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Based on the observation of a cyst compressing the popiteal artery, the authors discuss the characteristic clinical picture and radiology of this benign neoformation. They emphasize two possible causes: cystic degeneration of the arterial median or a cyst of articular derivation. Histological data from this observation supports the second hypothesis.
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Yersinia infections are exceptionally responsible for liver damage, the hepatic diseases most commonly encountered being liver abscess consecutive to Y. enterocolitica or Y. pseudo-tuberculosis septicaemia or, more rarely, hepatic granulomata or hepatitis with necrosis and cholestasis. Two new cases are reported. One patient had hepatic granulomata caused by Y. pseudo-tuberculosis and the other, necrotizing hepatitis caused by y. enterocolitica. The course of the disease was favourable in both cases.
Oat cell carcinomas of the lung are sometimes associated with endocrine secretion. Resulting syndromes, first regarded as "paraneoplasic", are in fact imputable to secretory activity of the tumoral cells, as demonstrated by electron microscopy and immunofluorescence studies. Such ascertainments are explained by the existence in the bronchial tree of a diffuse endocrine system sector, from which carcinoid and oat cell carcinoma originate. However, the authors consider other histogenetic hypotheses and rapidly survey the extra-pulmonary oat cell carcinomas.
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The authors report a case of anaplastic carcinoma with amyloid stroma, accompanied by the secretion of calcitonin, detected in a cervical lymph node and initially interpreted as being a metastasis from a medullary carcinoma of the thyroid. The actual pancreatic origin of the malignant tumour was proved only during the advanced stages. Ultrastructural study confirmed the endocrine nature of the tumour (neurosecretory granules). Despite its exceptional character, the existence of such a tumour is not surprising, if one accepts the concept of a diffuse endocrine system.
On two personal cases of left diaphragmatic congenital hernia, the authors study the innervation of the diaphragm. They describe a lower number of fibres in the phrenic nerve on the side of the malformation and don't find any branches coming from the intercostal nerves. This malformation is probably of neurogenic origin.
The authors report an analysis of 154 true gastric duplications published up to now, and a personal case. This malformation occurs exceptionally and is generally discovered during the first year of life. Diagnosis is rarely performed before laparotomy, clinic signs being various. They insist on investigation by barium enema and fibroscopy. They try to oppose tubular to cystic forms, according to a different embryogenesis. Surgical treatment is specific to each case.
Portal hypertension consecutive to hypervitaminosis A has seldom been well documented. Two cases are reported here. The first case concerns a 39-year old woman who had taken meladinine (8-methoxypsoralen) for the purpose of tanning. In the second case, a 43-year old woman had absorbed Plethoryl (a combination of tiratricol, cyclovalone and retinol) in order to lose weight. Both patients had histological hepatic lesions, such as hypertrophic Ito cells, perisinusoidal cirrhosis and spontaneous autofluorescence, suggestive of vitamin A overload, associated with a hepatic vein pressure gradient of 10 mmHg or more and high liver concentrations of vitamin A. The responsibility of Plethoryl in case 2 is beyond any doubt, but the mechanism of hypervitaminosis in case 1 is controverted. A review of the literature has provided detailed information on the signs of vitamin A overload, the frequency of which is probably underestimated. Patients with portal hypertension of unknown origin should be investigated for hypervitaminosis A.
A 26 year-old man presented a hepatic cyst complicated by intracystic bleeding. Clinical and biological features (chronic pancreatitis), ultrasound and computed tomography findings apparent hepatic and splenic location), macroscopic aspects (inflammatory changes, brown-colored fluid) as well as microscopic characteristics (thickened cyst-wall, absence of epithelium) of both cysts suggested the diagnosis of pancreatic pseudocysts. The diagnosis of hepatic cyst was carried out on histological examination showing Meyenburg and neuro-fibrillar complexes. Occurrence of complications (bleeding, size enlargement) may modify typical histological aspect of hepatic cyst.
A case of schwannosarcoma associated with a pleural neurofibroma and a parathyroid adenoma is presented. The neuroectodermal origin and the relationship with neurofibromatosis are discussed. The sarcomatous relapse of a schwannoma previously classified as benign confirms the difficulty of microscopic analysis and the malignancy power of the tumor. A long survival is obtained by a large intestinal and mesenteric resection as long as hepatic metastases are not present or can be resected. The pleural neurofibroma needs symptomatic treatment while parathyroid adenoma must be early removed as soon as malignant hypercalcemia is detected.