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Biomedical subjects

C Matuchansky

Publications and source records attributed to C Matuchansky.

At least 37 records · Page 2Linked to original sources
PubMed1986

Digestive tract and renal small vessel hyalinosis, idiopathic nonarteriosclerotic intracerebral calcifications, retinal ischemic syndrome, and phenotypic abnormalities. A new familial syndrome.

A new familial syndrome that affected 3 of 7 siblings is described. All 3 patients were young women with a very peculiar phenotype, poikilodermia and hair greying, and idiopathic nonarteriosclerotic cerebral calcifications. Pathological studies demonstrated a marked and progressive hyalinosis involving capillaries and often arterioles and small veins of the digestive tract, kidneys, and calcified areas of the brain. Using electron microscopy, we found that the hyalin substance in the intestinal capillaries consisted of several concentric layers of basal membrane-like deposits within a finely granular fluffy material. Huge deposits of this material were present in the subepithelial and mesangial spaces of the kidneys. Endothelial cells and, in the kidneys, mesangial cells were markedly abnormal, and a true mesangiolysis pattern was present in 2 patients. The clinical and biologic expression of these vascular changes was variable. Diarrhea, rectal bleeding, malabsorption, and protein-losing enteropathy were the main and lethal clinical problems in the proband. Hypertension appeared in the early stage of a second pregnancy in 1 sister, and mild proteinuria was found in all 3 affected patients. Peripheral retinal ischemic syndrome and chorioretinal scars were found in the ocular fundi of both affected sisters of the proband. A subarachnoid hemorrhage, due to a right sylvian aneurism, also occurred in both sisters and was lethal in 1 sister. None of the known causes of distal vessel hyalinosis could be ascertained.

Adult↗
PubMed1986

Parenteral nutrition in inflammatory bowel disease.

Nutritional support, administered via the enteral or parenteral routes, has been widely introduced in the treatment of inflammatory bowel disease over the past decade. The precise place of total parenteral nutrition, however, as a sole or adjunct treatment of inflammatory bowel disease, has yet to be defined.

Colitis, Ulcerative↗
PubMed1986

[Continuous ambulatory enteral feeding in hospitalized adults: prospective experience in 98 patients].

The goal of this study, which describes a personal technique of continuous enteral nutrition (CEN) in hospitalized adults on an ambulatory basis, was: to prospectively evaluate, over a 2-year period, its efficacy and tolerance in 98 patients requiring CEN for at least 15 days; to compare its efficacy and tolerance with those of conventional non-ambulatory CEN on a prospectively randomized basis in 16 patients. Ambulatory CEN was given at the rate of 35-45 kcal/kg/d (lipids: 35 p. 100; carbohydrates: 45 p. 100); during day-time, a portable system, including pump, tubes and low-viscosity nutrient solutions, allowed ambulation. Ninety-eight consecutive patients with a minimal level of physical autonomy were treated for intestinal (n = 47), pancreatic (n = 20), esophagogastric (n = 17) diseases, or for malnutrition of other causes for an average of 38 days (15 to 141). The average weight gain (m +/- SD) was 1.2 +/- 5.5 p. 100 of ideal body weight (IBW) and the average nitrogen gain was 0.7 +/- 3.8 g/24 h; weight gain proved significantly lower in patients with inflammatory bowel disease receiving steroids. The clinical tolerance proved excellent, except for 5 cases of transient diarrhea and 9 cases of reposition of the nasogastric tube. A decrease in cholesterolemia below 3.9 mmol/l was noted in 25 p. 100 of patients during CEN. No significant difference between ambulatory and non-ambulatory CEN was observed in terms of evolution of body weight and other anthropometric variables, nitrogen balance, albuminemia, and oxygen consumption; conversely, the CEN experience, evaluated by patients on analogical visual scales, was significantly better endured in the ambulatory group.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1985

[Relations between physical exertion and protein-energy metabolism. Application to nutritional support of hospitalized patients].

The relationship between physical activity, nutritional requirements and protein-energy metabolism is well-established. However, and contrary to bedside practice at home, non-ambulatory techniques of enteral or parenteral nutrition are often used in hospitalized patients, even those who are able to move about. The purpose of the present study is: (a) to review the relationship, as we know it at present, between physical activity and protein metabolism in both normal subjects and patients with denutrition, and (b) to demonstrate that using from the start, or as early as possible, ambulatory techniques of enteral or parenteral nutrition does represent, both physiologically and psychologically, a decisive step forward in the care of hospital patients.

Amino Acids↗
PubMed1985

Effects of cyclic (nocturnal) total parenteral nutrition and continuous enteral nutrition on circadian rhythms of blood lipids, lipoproteins and apolipoproteins in humans.

The aim of the present study was to investigate the occurrence and characteristics (acrophase, amplitude) of circadian rhythms of serum total cholesterol, free-fatty acids (FFA), triglycerides, lipoproteins (HDL-, LDL-, and VLDL-cholesterol), apolipoproteins A and B, glucose and total proteins in hospitalized patients fed with 12 h nocturnal total parenteral nutrition (TPN) (from 8 PM to 8 AM) including lipids, patients fed with continuous enteral nutrition over 24 h daily spans, and patients eating 3 meals a day serving as controls. All the subjects were synchronized with diurnal activity and nocturnal rest in the hospital routine. The results showed the following: 1) circadian rhythms of total cholesterol, triglycerides, FFA, HDL-, LDL-cholesterol, apolipoprotein A and total proteins were detected in both TPN patients and controls, rhythms of apolipoprotein B and glucose in TPN patients only; in enteral nutrition patients, rhythms were detected for total proteins, glucose and triglycerides only; 2) a significant shift in triglyceride and FFA acrophases was observed in TPN patients, as compared with controls; 3) 24 h mean of both triglyceride and cholesterol concentrations remained unchanged after one month, in both TPN and enteral nutrition patients. The present approach, by extending results of previous investigations, leads one to conclude that, on both a metabolic and a chronobiological basis, cyclic nocturnal TPN is well-tolerated.

Administration, Oral↗
PubMed1984

[Familial occurrence of cancer of the colon and the rectum: results of a 3-year case-control survey].

A case-control study of the familial prevalence (first-degree relatives) of colorectal carcinoma (CRC) was performed from October 1979 through March 1983 in: (a) 170 consecutive patients with histologically proved rectal (n = 64) or colonic (n = 106) adenocarcinoma; cases of familial polyposis coli and cancer family syndrome were systematically excluded from the study; (b) 170 control subjects, who were free of CRC or colorectal adenoma(s) and matched to patients according to sex and age; (c) 170 consecutive patients with common rectal or colonic adenoma(s), and no evidence of polyposis coli; (d) 100 patients with cancer of various organs, excluding CRC and primary tumours known to be epidemiologically related to CRC. Results of family studies were expressed as "proved" (when the pathological report was received) or "probable" CRC. Eighteen (10.6 p. 100) of the 170 patients with CRC had at least one first-degree relative with past or present proved CRC, and 14 (8.2 p. 100) with probable CRC; the corresponding figures were 3 (1.7 p. 100) proved and 3 (1.7 p. 100) probable CRC in the control group, 14 (8.2 p. 100) proved and 11 (6.5 p. 100) probable CRC in the "adenoma" group and 1 (1 p. 100) and 3 (3 p. 100) in the non-colonic carcinoma group. The relative risk of CRC in close relatives was 6.3 and 4.7 in the CRC and adenoma groups respectively. For the variables studied no significant differences were found between males and females.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1984

Cavitation of mesenteric lymph nodes, splenic atrophy, and a flat small intestinal mucosa. Report of six cases.

This study describes, in 6 patients with a flat small intestinal mucosa and splenic atrophy, a particular lesion of the mesenteric lymph nodes termed "cavitation." In 4 women and 2 men with abdominal mass, intestinal obstruction, or suspected celiac disease-associated lymphoma, unusual pseudocystic lymph node lesions were found in the jejunal or jejunoileal mesentery. These lesions consisted histologically of a large central cavity occupied by hyaline-type material and surrounded by fibrous tissue and remnants of lymph node structures. There was no histologic evidence of malignant lymphoma or mesenteric panniculitis. Diffuse subtotal villous atrophy involving at least the jejunum was found in each case, together with unequivocal biological and morphological evidence of splenic atrophy, severe malabsorption, and a history of chronic or childhood diarrhea. HLA B8 or DR3, or both, was present in 4 of 4 cases; dermatitis herpetiformis was present in 1 case. An unequivocal mucosal response to a gluten-free diet was observed in 2 cases. Four patients died of cachexia or hyposplenism-related infections. We conclude that cavitation of mesenteric lymph nodes is an original feature which may be associated with splenic atrophy and a flat small intestinal mucosa; some of these patients may have celiac disease. Pathogenesis is unknown.

Adult↗
PubMed1983

[Chronic radiation-induced esophagitis after treatment of oropharyngolaryngeal cancer: a little-known anatomo-clinical entity].

Chronic esophagitis induced by radiation therapy (CRE) for carcinoma of the oropharynx and larynx, and involving an initially normal esophagus, is poorly known, and often confused with a locally recurrent malignancy. By reporting 8 consecutive cases, the aim of this study is to contribute to proving that cervical CRE does indeed exist. Eight men, aged 58 to 76 years, developed complete aphagia (7 cases) or severe dysphagia (1 case), 2 to 56 months (median 8.5 months) after radiation therapy for carcinoma of the laryngopharyngeal area. In 5 patients, the primary tumour and/or chains of cervical lymph nodes were removed before radiation therapy; in the 8 patients, the esophagus was endoscopically free of disease. The total dose of radiation exceeded 60 grays in 6 patients. Although they were initially considered as suffering from a locally recurrent malignancy on a clinical basis, none of the 8 patients had patent signs (either endoscopic or histologic) of recurrence. In each case a severe lesion of the cervical esophagus was documented (stenosis 7 cases, giant ulceration 1 case): the diagnosis of CRE was based on endoscopic, histological, and evolutive criteria. Six patients were treated by prolonged nutritional support (via enteral route, 5 cases; parenteral route, 1 case); endoscopic dilatations were used as a single treatment or in association with nutritional support in 4 cases. Three patients died of CRE-induced regional complications; post mortem examination, performed in 2 cases, confirmed CRE without evidence of locally recurrent malignancy.(ABSTRACT TRUNCATED AT 250 WORDS)

Aged↗