Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024523 (malabsorption)
 7,319 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We report on a 56-year-old caucasian female, 154 cm, 38 kg, who was admitted to the hospital in extremely poor, cachectic condition with pretibial edemas, ascites and pleural effusion with a history of severe watery diarrhea up to 15 times daily. The patient's history with permanent meteorism, recurrent episodes of watery diarrhea and dystrophy dates back to her early childhood. Multiple biopsies from the distal part of the duodenum showed a complete atrophy of villi with an extensive subepithelial layer of collagenous fibers. No antigliadin or antiendomysial antibodies could be detected. Under a strictly gluten-free diet, the patient's symptoms quickly improved. During the follow-up of one year, no recurrence of diarrhea and meteorism has been seen. The patient showed a continuous improvement of her general condition and nutritional state with a weight gain to 50 kg. Repeated control biopsies still revealed a pronounced villous atrophy. However, no subepithelial collagenous fibers were detectable. A diagnosis fitting to the patient's clinical symptoms of severe malabsorption, would be the rare "collagenous sprue", which is considered to be gluten-insensitive. However, the patient's good response to a gluten-free diet raises the possibility, that the collagenous layer represents a manifestation of more severe forms of common celiac disease. In this case, it would not be necessary to regard collagenous sprue as a distinct entity.
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PMID:[Long-term collagenous sprue--remission with a gluten-free diet]. 1066 40

A 52-yr-old white female presented with worsening low back and hip pain, associated with lower limb proximal muscle weakness and a waddling gait. Her laboratory evaluation revealed hypocalcemia, hypophosphatemia, a very low 25-hydroxyvitamin D level of less than 5 ng/mL, and a bone mineral density in the osteoporotic range. Her laboratory studies were consistent with osteomalacia, although this diagnosis was not established by histomorphometry. She avoided dairy products, spent little time outdoors, and when she went out, she covered her face, arms, and legs. She was on no medication. Her workup for malabsorption including sprue was negative. She was treated with calcium plus high-dose vitamin D 600,000 IU intramuscularly twice witihin 2 mo and had an impressive clinical improvement. Her difficulty with ambulation improved within 1 wk of start of therapy. Her bone mineral density increased by 40% at the spine and 35% at the hip at 4 mo of therapy, by 63% and 39% at 10 mo, and by 62% and 52% at 15 mo at these sites, respectively. Treatment of osteomalacia is extremely rewarding, with dramtic clinical improvement and normalization of bone mineral density.
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PMID:Nutritional osteomalacia: substantial clinical improvement and gain in bone density posttherapy. 1074 6

The vast majority of patients with celiac disease respond to a gluten-free diet; yet, a small number of refractory patients do not respond and have persistent malabsorption and residual mucosal abnormalities of the small intestine. The histologic features of refractory/unclassified sprue have been published as case reports, often without long-term follow up, and no clear histologic picture has emerged. We present the results of a long-term study of the clinical and histologic features of 10 patients with refractory/unclassified sprue. The histologic features of small bowel biopsies in this group of patients were compared with those of 10 patients with responsive celiac disease and with 10 patients without malabsorption who had normal duodenal biopsies. Five of the 10 refractory patients ultimately developed collagenous sprue as a distinct histologic marker of refractory disease. Additional distinctive findings found in small bowel biopsies in the refractory group were subcryptal chronic inflammation (10 of 10) and marked mucosal thinning in three patients. Other nonspecific findings included acute inflammation and gastric metaplasia. One patient with collagenous sprue developed a B-cell lymphoma of the ileum, and in general collagenous sprue was associated with a poor prognosis. Two of five patients died whereas two others require total parenteral nutrition for survival. Pathologists evaluating small bowel biopsies in the setting of malabsorption should be aware of the subtle histologic changes described here that may portend a refractory course.
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PMID:The histologic spectrum and clinical outcome of refractory and unclassified sprue. 1125 32

Clinical investigation of the small bowel at The Mount Sinai Hospital began with David Adlersberg's arrival in 1931. His research interests were in bile acids, cholesterol, carotene, and vitamin A. In 1952, he was given a Nutrition Laboratory and later, a Nutrition Clinic. His vitamin A tolerance test and interest in malabsorption led him to a comprehensive study of sprue, the separation of the tropical and non-tropical forms, and their different etiologies and treatments. Adlersberg's work was complemented by (a) Marshak and Wolf's radiologic examination of the small bowel (especially in sprue and other malabsorption disorders); (b) Gerson s perfusion experiments; and (c) Friedman, Waye and Wolf's motility studies. Lieber and his colleagues explored the deleterious effects of alcohol on the function and structure of the small intestine. Gerson explored the nutrition of patients with Crohn's disease of the small intestine, especially after extensive resection or bypass leading to ascorbic and folic acid deficiencies and hypergastrinemia.
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PMID:The small intestine. 1082 9

Celiac disease is a gluten-sensitive enteropathy, characterized by villous atrophy, which is reversed by gluten withdrawal. A minority of patients with celiac-like enteropathy are resistant to gluten-free diet, so-called refractory sprue, or unclassified sprue. Refractory sprue is a diagnosis of exclusion; all other causes of a celiac-like enteropathy must be eliminated before a diagnosis of refractory sprue can be made. Recent evidence suggests that refractory sprue comprises a heterogenous group of patients with diverse underlying causes. A small proportion of these patients seem to have an adult form of autoimmune enteropathy, characterized by the presence of antienterocyte antibodies. However, a larger group of patients with refractory sprue now seem to have a cryptic intestinal T-cell lymphoma, characterized by the presence of phenotypically abnormal, monoclonal intraepithelial lymphocytes, despite benign cytology. Current therapeutic options include nutritional support and immunosuppressive therapy, but response is variable. The prognosis of refractory sprue may be poor; patients may die of severe malabsorption, or through synchronous or metachronous development of an enteropathy-associated T-cell lymphoma. Based on this recent evidence, patients with refractory sprue should be screened for antienterocyte antibodies and have T-cell receptor and monoclonal antibody studies performed; this could facilitate identification of cases of adult-onset autoimmune enteropathy and those of cryptic T-cell lymphoma. Moreover, early recognition of the malignant nature of the intestinal infiltrate in some cases of refractory sprue could permit the development of novel chemotherapeutic regimens for this condition.
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PMID:Refractory celiac disease. 1088 75

In 1970 a case of malabsorption with flat small intestinal mucosa with subepithelial collagen deposition was described. There was no response to a gluten-free diet, and the condition was termed collagenous sprue. We report a case of coeliac disease with subepithelial deposition of collagen in duodenal biopsy, which responded to a gluten-free diet.
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PMID:[Celiac disease with subepithelial deposition of collagen]. 1101 88

The objective of the paper is to draw attention to the not very frequent and thus omitted form of sideropenic anaemia in selective iron malabsorption as the only manifestation of malabsorption syndrome in coeliac sprue. This type should be suspected when examination of blood losses is futile and oral iron administration which is usually administered empirically produces no effect. The authors present the example of two patients with severe sideropenic anaemia where the diagnosis of malabsorption was confirmed only several years after the diagnosis of sideropenic anaemia was established. Evidence of a correct conclusion was permanent normalization of haemoglobin values when the patients adhered to a gluten-free diet.
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PMID:[Sideropenic anemia as a manifestation of selective iron malabsorption]. 1150 23

Psychiatric symptoms and psychological behavioral pathologies are common in patients with untreated coeliac disease. There are several case reports of coexistence of coeliac sprue and depression, schizophrenia and anxiety. Views on association between coeliac disease and psychiatric disturbances and results of the most important studies are discussed. Biological background is referred. Malabsorption and deficiency of aminoacids and vitamins implicate reduction of synthesis of neurotransmitters in the central nervous system. Psychiatric symptoms could also be linked to immunological disregulation in coeliac patients. Psychological pathologies do appear in treated and untreated coeliacs, the need of psychological support is stressed. Coeliac disease should be taken into consideration in patients with psychiatric disorders, particularly if they are not responsive to psychopharmacological therapy, because withdrawal of gluten from the diet usually results in disappearance of symptoms. In recent years, an increased incidence of subclinical/silent coeliac disease has been reported. Psychiatric symptoms and psychological behavioral pathologies could be the only clinical manifestation of coeliac disease, but the epidemiological aspects need further investigation.
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PMID:[Psychiatric symptoms and coeliac disease]. 1229 86

Celiac disease is a gluten-sensitive enteropathy characterized by villous atrophy that is reversed by gluten withdrawal. A minority of these patients is resistant to a gluten-free diet or, after a period of remission, they experience relapse despite continued adherence to treatment, which is called unclassified sprue or refractory sprue.The prognosis of refractory sprue may be poor: patients may die of severe malabsorption or from the development of an enteropathy-associated T-cell lymphoma. We report a 72-year-old-woman with a diagnosis of refractory sprue who responded well to treatment with corticosteroids and a gluten-free diet.
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PMID:[Diagnosis and treatment of refractory sprue]. 1245 21

Lower duodenal biopsies (LDB) are not taken at every oesophago-gastro-duodenoscopy (EGD). In the present study, biopsies from the endoscopic normal lower duodenum were checked as a measure of quality assurance. From 1996 to 2000, 9,955 EGD were performed and 4,199 LDB were taken (42.2 %). Of these, 667 showed pathological histology (15.9 %). A non-specific inflammation was seen in 537 cases and lymphangiectasia in 30 cases. Signs of indigenous sprue were described histologically in 6 LDB. In 4 of the 6 first diagnoses, the LDB was taken owing to clinical suspicion of malabsorption syndrome. Giardia lamblia could be detected in 22 patients. Only 6 of the 22 patients had diarrhoea. A total of 18 clinically relevant first diagnoses were made by LDB in asymptomatic patients with normal endoscopic findings in the duodenum. In order to make a relevant first diagnosis, 233 LDB had to be taken. LDB can be dispensed within EGD when there is neither diarrhoea nor loss of weight, and no anemia, iron deficiency, vitamin deficiency, macrocytosis, hypoproteinaemia, meteorism, joint symptoms or fever.
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PMID:[4199 biopsies from the endoscopic normal lower duodenum]. 1254 Nov 78


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