Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0024312 (lymphopenia)
 4,859 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The clinical and laboratory features of 18 adult pellagrins are reviewed. Only four patients (22%) had the full trial of dermatitis, diarrhea and dementia. Dermatitis alone occurred in six(33%), dementia in five(28%) and dermatitis and diarrhea in three(17%). In one patient, dermentia was the initial sign of a relapse. Steatorrhea was found in six patients and was usually associated with marked alopecia. Edema without evidence of cardiac failure was present in seven patients. A diffuse increase in slow wave activity on the electroencephalogram was characteristic in patients with dementia. Fever occurred in 14 patients, and an infection was documented in 10 of these. Common laboratory abnormalities included a normochromic, normocytic anemia, lymphopenia, eosinopenia hyperuricemia, and low serum levels of albumin, urea, cholesterol, carotene, potassium, calcium, and magnesium. Adrenal and thyroid function were normal, but a low serum T4, high serum free T4, and an elevated T3 resin uptake were frequently observed. These abnormalities were corrected with treatment of the underlying nutritional disorder. In two patients initially treated with thiamine alone, and in one who received inadequate amounts of niacin and protein, there was marked deterioration of mental function, which responded to administration of niacin and proper diet.
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PMID:Pellagra: an analysis of 18 patients and a review of the literature. 86 2

Two Chinese infants with asymmetrical lymphoedema and steatorrhoea were investigated. The laboratory investigations showed that they had hypoproteinaemia without proteinuria. One of them showed peripheral lymphopenia and hypogammaglobulinaemia. Radiologically, they had evidence of extensive lymphangiectasia of the small intestine. This was confirmed histologically. Endoscopy of one of them showed diffuse milky nodular duodenal mucosa. The appearance of the mucosa was rather typical of intestinal lymphangiectasia.
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PMID:Intestinal lymphangiectasia--a report in two Chinese children. 646 32

A 16-year-old boy with primary intestinal lymphangiectasia presented with peripheral edema of 6 weeks duration. Laboratory and radiological studies included absolute lymphopenia, hypoalbuminemia, steatorrhea, abnormal stool 51Cr-albumin value, edema of small intestinal folds, dilated mesenteric lymphatics, as well as bilateral filling defects in external and common iliac lymph nodes. Abdominal CT scan revealed a possible periaortic mass. Small intestinal biopsies demonstrated normal villous architecture with dilated lacteals, and electron microscopy revealed enterocytes with normal as well as blunted microville, enlarged, dilated lacteals and intercellular vacuoles. An exploratory laparatomy was performed and revealed no masses but dilated serosal lymphatic vessels. Medical treatment, including marked restriction of long chain triglyceride intake, resulted in loss of peripheral edema, weight stabilization, and normal activity.
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PMID:Primary intestinal lymphangiectasia. Case report with radiological and ultrastructural study. 722 69

Intestinal lymphangiectasia in the adult may be characterized as a disorder with dilated intestinal lacteals causing loss of lymph into the lumen of the small intestine and resultant hypoproteinemia, hypogammaglobulinemia, hypoalbuminemia and reduced number of circulating lymphocytes or lymphopenia. Most often, intestinal lymphangiectasia has been recorded in children, often in neonates, usually with other congenital abnormalities but initial definition in adults including the elderly has become increasingly more common. Shared clinical features with the pediatric population such as bilateral lower limb edema, sometimes with lymphedema, pleural effusion and chylous ascites may occur but these reflect the severe end of the clinical spectrum. In some, diarrhea occurs with steatorrhea along with increased fecal loss of protein, reflected in increased fecal alpha-1-antitrypsin levels, while others may present with iron deficiency anemia, sometimes associated with occult small intestinal bleeding. Most lymphangiectasia in adults detected in recent years, however, appears to have few or no clinical features of malabsorption. Diagnosis remains dependent on endoscopic changes confirmed by small bowel biopsy showing histological evidence of intestinal lymphangiectasia. In some, video capsule endoscopy and enteroscopy have revealed more extensive changes along the length of the small intestine. A critical diagnostic element in adults with lymphangiectasia is the exclusion of entities (e.g. malignancies including lymphoma) that might lead to obstruction of the lymphatic system and "secondary" changes in the small bowel biopsy. In addition, occult infectious (e.g. Whipple's disease from Tropheryma whipplei) or inflammatory disorders (e.g. Crohn's disease) may also present with profound changes in intestinal permeability and protein-losing enteropathy that also require exclusion. Conversely, rare B-cell type lymphomas have also been described even decades following initial diagnosis of intestinal lymphangiectasia. Treatment has been historically defined to include a low fat diet with medium-chain triglyceride supplementation that leads to portal venous rather than lacteal uptake. A number of other pharmacological measures have been reported or proposed but these are largely anecdotal. Finally, rare reports of localized surgical resection of involved areas of small intestine have been described but follow-up in these cases is often limited.
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PMID:Intestinal lymphangiectasia in adults. 2136 42