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)

During the period 1985-88, 30 children with a chronic blistering dermatosis were studied. Of these 25 were found to have chronic bullous dermatosis of childhood (CBDC) and five had bullous pemphigoid (BP). No case of dermatitis herpetiformis (DH) was seen in the same period. Except for the difference in immunofluorescence (IMF) there were no definite clinical, histological or therapeutic differences between the two groups. All the children were Africans with the exception of one Indian girl. Their ages ranged from 1 year to 12 years with a mean of 5 years. The females outnumbered the males in a ratio of 3:2. All children had a generalized eruption consisting of large tense blisters arising on normal skin. The blisters were more profuse on the lower trunk, pelvic region and limbs. Face and scalp were also affected. Histological features of BP and DH were seen. Direct IMF in the CBDC patients showed linear deposits of IgA at the basement membrane zone (BMZ) while linear deposits of IgG were seen in the BP group. Complement and IgM were also seen in some cases in both groups. Sixty per cent of the CBDC patients showed IgA BMZ antibodies by indirect IMF. There were no symptoms or signs of malabsorption. Serum vitamin B12 and folate levels were normal. HLA studies showed the B-8 antigen in five of the 20 patients studied. Therapy was difficult in most cases. All patients haemolysed on therapeutic doses of dapsone, sulphapyridine and/or prednisone had to be added. Follow-up was generally poor as six patients failed to return after discharge from hospital.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Chronic bullous dermatosis of childhood--clinical and immunological features seen in African patients. 193 64

A pair of monozygous male twins concordant for dermatitis herpetiformis (DH) and gluten-sensitive enteropathy (GSE) are reported. Presentation of DH in the twins was asynchronous, with one affected at the age of 20, the other at the age of 27. Neither twin had symptoms of malabsorption but duodenojejunal villous atrophy was confirmed histologically.
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PMID:Dermatitis herpetiformis in monozygous twins--concordance for dermatitis herpetiformis and gluten-sensitive enteropathy. 202 37

We report four patients (two children, one adolescent, and one adult) having normal small bowel mucosa shown on a biopsy specimen taken before the initial diagnosis of coeliac disease was made. The first biopsy was undertaken in two cases because of suspected malabsorption, in the third because of suspected dermatitis herpetiformis, and in the fourth as part of a coeliac disease family study. After a further 2.6 to 9 years on a diet containing gluten, small bowel villous atrophy with crypt hyperplasia compatible with coeliac disease was found on a second biopsy specimen. The HLA type of the patients was that typical for coeliac disease; all were DR3 positive. Within the families three other patients with coeliac disease have been diagnosed, two earlier and one at the time the first biopsy was undertaken. Four other HLA-DR3 positive haploidentical first degree relatives were found and had biopsies. All four had normal small bowel villous architecture, one had an increased intraepithelial cell count, and another was positive for reticulin and endomysium antibodies. Coeliac disease may exist latent in patients having normal mucosa when eating a normal diet containing gluten.
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PMID:Normal small bowel biopsy followed by coeliac disease. 224 6

The association of coeliac disease and partial lipodystrophy is described. The patient also had deficiencies of serum IgA and C3 complement (the latter associated with partial lipodystrophy). In addition, there was subclinical dermatitis herpetiformis confirmed by skin biopsy. The facial wasting of fully developed partial lipodystrophy may be misinterpreted as a sign of malabsorption but the facial, upper limb, and truncal lipodystrophy contrasts with normal pelvic and lower limb appearances.
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PMID:Partial lipodystrophy in coeliac disease. 237 78

Dietary intakes of energy and nutrients were calculated from diet history interviews in 30 patients with dermatitis herpetiformis, before and after 18 months on a gluten-free diet. In spite of great changes in the intake of different foods, the mean intake of dietary fibre did not decrease. There was only a small decrease in the intake of iron in women, while the intake in men did not change. Patients with a previous high intake of gluten had indirect evidence of malabsorption.
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PMID:Changes in food consumption and its nutritional quality when on a gluten-free diet for dermatitis herpetiformis. 299 Nov 72

Computerised image-analysis was used to quantitate small intestinal mucosae from celiac sprue and dermatitis herpetiformis patients, Gambian children with tropical-sprue-like malabsorption, first-degree celiac sprue relatives, and treated celiac sprue patients during challenge with a peptic-tryptic digest of gluten. A wide range of mucosal appearances was observed. Typically, 'flat' lesions (Type 2) revealed a reduced number of epithelial lymphocytes that were large and mitotically active. At the other extreme, mucosal architecture was relatively well preserved (Type 1) but surface epithelium contained an expanded population of small, non-mitotic lymphocytes, with or without crypt hyperplasia. Similar changes were observed in one-third of celiac relatives and following small dose gluten challenge. Larger dose challenges revealed a transition from Type 1 to Type 2 lesions over a 5-day period. Studies in a few patients over 2-4 years showed a similar type of progression. A major feature of this sequence was early appearance of crypt hypertrophy while villi persisted, indicating a role for factors other than increased loss of enterocytes from surface epithelium. These changes parallel the T lymphocyte-mediated events in graft-versus-host reactions in animals. It is thus concluded that the spectrum of immunopathologic changes observed in gluten sensitivity is fundamentally a cell-mediated effect, the degree of change being controlled by host genetic factors. In becoming flat, it appears obligatory for the mucosa to evolve through the earlier Type 1 lesion in which crypt hypertrophy is a prominent response.
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PMID:Studies of intestinal lymphoid tissue. XI--The immunopathology of cell-mediated reactions in gluten sensitivity and other enteropathies. 320 Dec 2

Nutritional status of 86 patients with dermatitis herpetiformis (DH) was defined by anthropometric measurements and hematological and biochemical laboratory tests to establish prevalence of malabsorption and malnutrition. Anthropometric measurements in DH patients were comparable to normal control patients. Four individuals were of short stature; two had had diarrhea and failed to thrive in childhood. Abnormalities attributable to nutritional deficiency were detected in only 6 of the 86, whereas drug-associated hematological or biochemical changes were present in 36 of 55 subjects treated with dapsone or sulfapyridine. Twenty patients had hemolytic anemia or macrocytosis related to drug therapy. Only two had anemias attributable to malabsorption; one was iron deficient, the other folate deficient. Two other patients were mildly Fe deficient and two had slight folate deficiency; they lacked other stigmata of malabsorption. Drug-induced hematological and biochemical abnormalities were more common than changes that suggest nutritional disease, even though most DH patients had an enteropathy at presentation.
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PMID:Nutritional status in patients with dermatitis herpetiformis. 340 14

The absolute oral bioavailability of dapsone (DDS) was established in dogs and healthy volunteers by comparing AUC's after oral and intravenous administration. Five female inbred beagles each received 100 mg DDS orally and intravenously as a bolus injection in a randomized cross-over study. DDS serum concentrations were determined using HPLC. After both routes of administration, linear pharmacokinetics were observed, the elimination half-life (t1/2) amounting between 5.8 and 10.2 h. After oral administration, AUC values between 56.1 and 99.2 mg X h X l-1 were found, while after intravenous administration AUC's were between 63.5 and 98.1 mg X h X l-1. The absolute oral bioavailability, corrected for differences in t1/2, averaged 107 +/- 9% (SD). A similar study was carried out in 2 female and 3 male healthy volunteers. The intravenous dose was reduced to 50 mg and given as an infusion. Pharmacokinetics were linear after both routes of administration. The t1/2 values amounted between 15.6 and 30.4 h. AUC's ranged from 24.0 to 75.4 mg X h X l-1 after oral administration and from 13.3 to 37.5 mg X h X l-1 after intravenous infusion of half of the oral dose. The absolute, t1/2-corrected oral bioavailability was calculated to be 86 to 104%. Complete bioavailability of DDS was demonstrated in dogs and healthy volunteers. The method used in this study might help to detect possible DDS malabsorption in leprosy and dermatitis herpetiformis patients.
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PMID:The absolute oral bioavailability of dapsone in dogs and humans. 362 44

Some aspects of humoral immunity and the status of the jejunal mucosa were investigated in 22 patients with classical dermatitis herpetiformis (DH). The mucosal status was characterized on the basis of immuno- and histopathological findings and functional analysis of malabsorption and disaccharidase activity. There was no significant abnormality in the serum immunoglobulins, whereas the IgA and C3 contents of the circulating immune complexes (established by polyethylene glycol precipitation and inverse radial immunodiffusion techniques) were significantly elevated. Polyorgan-specific autoantibodies of IgG type were present in the sera of 95% of the 22 patients. Functional analysis of the gastrointestinal tract revealed some abnormality in all of the 18 cases examined, while direct immunofluorescence studies demonstrated an increased number of subepithelial IgA lymphoid cells in all of the 15 cases examined. These findings did not correlate well with the jejunal mucosal morphology. This study supports the view that IgA deposited in the skin is formed in the gut.
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PMID:Dermatitis herpetiformis: relation between circulating immune complexes, small-intestinal mucosal status, and immunohistopathological findings. 363 36

Sixty-eight patients with dermatitis herpetiformis underwent jejunal suction biopsies and/or multiple endoscopic duodenal biopsies to evaluate the incidence of small bowel mucosal atrophy and to compare the diagnostic yield of the two methods. Small bowel function tests were also performed to evaluate the extent of functional impairment. Small bowel lesions were observed in 89.4% of jejunal suction biopsies and in 100% of endoscopic duodenal biopsies. Of the 10 patients who underwent both procedures, one had lesions only in the duodenum, one had more severe lesions in the duodenum than in the jejunum, while the remaining 8 patients showed identical lesions at both sites. The 1-h blood d-xylose test after a dose of 5 g proved more sensitive than xylosuria or serum folic acid assay in detecting subclinical malabsorption. Finally, histological features of gluten-sensitive enteropathy can be found in nearly 100% of patients with dermatitis herpetiformis. Upper gastrointestinal endoscopy with duodenal biopsies is at least as sensitive as jejunal suction biopsy in assessing small bowel involvement in dermatitis herpetiformis.
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PMID:Prevalence of duodenal and jejunal lesions in dermatitis herpetiformis. 367 97


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