1/67. Autoimmune enteropathy with distinct mucosal features in T-cell activation deficiency: the contribution of T cells to the mucosal lesion.BACKGROUND: Autoimmune enteropathy is normally characterised by crypt hyperplastic villous atrophy with enterocyte autoantibodies, activation of mucosal lymphocytes and increased epithelial HLA-DR. This case involved a severely affected Portuguese infant who was found to have lymphocyte activation deficiency and demonstrated correspondingly distinct mucosal features. methods: A female infant of nonconsanguineous parents was treated for vomiting and diarrhoea, first with milk exclusion and then with parenteral nutrition. lymphocyte subsets and immunoglobulin concentrations were normal, but in vitro testing showed no activation in response to phytohaemagglutinin, candida, or purified protein derivative, although the response to interleukin (IL)-2 was intact. interleukin-2 deficiency was excluded. Analysis of jejunal biopsy specimens revealed only mild villous blunting with absent goblet cells, normal epithelial proliferation, and no crypt hyperplasia. The dense infiltrate of CD8 and CD4 T lymphocytes showed normal CD2 and CD3 expression but no activation or proliferation markers. HLA-DR was not increased on epithelium or lymphocytes. Thus, in addition to in vitro evidence for lymphocyte activation deficiency, the mucosal specimens showed no evidence of in situ T-cell activation. RESULTS: After development of overwhelming septicaemia, the patient died at 18 months, just before a planned bone marrow transplant. CONCLUSIONS: These findings confirm significant heterogeneity within autoimmune enteropathy. Formal immune function testing should be performed in all affected infants to identify T-cell activation deficiencies. The distinct mucosal findings suggest that activated T cells usually induce the crypt hyperplastic villous atrophy characteristic of classic autoimmune enteropathy.- - - - - - - - - - ranking = 1keywords = bone (Clic here for more details about this article) |
2/67. adenocarcinoma of the colon with neuroendocrine features and secretory diarrhea.We report the case of a 63-yr-old man who had severe secretory diarrhea associated with colonic adenocarcinoma, with a prominent signet ring cell component and numerous endocrine cells as demonstrated by positive chromogranin-A staining. Improvement in the secretory diarrhea by the somatostatin analog Sandostatin suggested that the diarrhea was related to a functional neuroendocrine tumor within the colonic tumor, the first case to be reported in the literature.- - - - - - - - - - ranking = 491.87813232378keywords = endocrine (Clic here for more details about this article) |
3/67. Toxic epidermal necrolysis and graft vs. host disease: a clinical spectrum but a diagnostic dilemma.We describe a 53-year-old man who developed partial and full thickness skin loss associated with pyrexia, diarrhoea, liver, renal and bone marrow failure, during treatment for an aggressive B cell lymphoblastic lymphoma. The clinical features and histology were compatible with both toxic epidermal necrolysis and graft vs. host disease, causing a diagnostic and therapeutic dilemma. We discuss the possibility that methotrexate was the causative drug, with review of its cutaneous side-effects. Histologically our patient demonstrated the sparse dermal infiltrate with full thickness epidermal necrosis typical of toxic epidermal necrolysis and graft vs. host disease. We discuss this finding with respect to the pathogenesis of toxic epidermal necrolysis.- - - - - - - - - - ranking = 1keywords = bone (Clic here for more details about this article) |
4/67. Overview of chronic diarrhea caused by functional neuroendocrine neoplasms.Eight different neoplastic disorders can cause chronic diarrhea attributable to humoral-mediated diarrhea. These include pancreatic endocrine tumor (PET) syndromes (gastrinomas, VIPomas, glucagonomas, somatostatinomas, PET's releasing calcitonin), carcinoid syndrome, medullary thyroid cancer, and systemic mastocytosis. Because these disorders are an uncommon cause of all chronic diarrheas (<1%), they are not often considered in the differential diagnosis, leading to a delay in diagnosis. This is problematic not only because all are treatable, but also because the neoplasm is frequently malignant. In this article, the characteristics and pathogenesis of the diarrhea, important clinical and diagnostic laboratory features, and treatment of each disorder are briefly reviewed, with an emphasis on recent insights.- - - - - - - - - - ranking = 409.89844360315keywords = endocrine (Clic here for more details about this article) |
5/67. Thymic carcinoid and parathyroid hyperplasia detection with 99mTc-MIBI men type 1.We report a case of a 35-year-old male, with a history of diarrhea, renal lithiasis with frequent expulsions of calculus and hypercalcemia during the last 2 years. The patient was studied and diagnosed with a multiple endocrine neoplasia type I (men I), familiar (mother with men I). A scintigraphic study with 99mTc-MIBI was performed in order to localize hyperfunctioning parathyroid glands because of biochemical diagnosis of primary hyperparathyroidism. Double phase 99mTc-MIBI scan detected one hyperfunctioning parathyroid gland and a large anterior mediastinal mass. Subsequent, plain radiograph and CT of the chest showed a soft-tissue mass in that localization. Punch biopsy of the lesion guided by CT revealed malignant cells of neuroendocrine tumor. The tumor was removed and histologically confirmed as a carcinoid within a thymus in a men type I syndrome. men I patients can benefit from the examination with this agent which can potentially localize not only parathyroid endocrine pathology but also unknown associated tumors.- - - - - - - - - - ranking = 245.93906616189keywords = endocrine (Clic here for more details about this article) |
6/67. Sequential vidarabine infusion in the treatment of polyoma virus-associated acute haemorrhagic cystitis late after allogeneic bone marrow transplantation.Late onset haemorrhagic cystitis (HC) occurs in 20-30% of allogeneic bone marrow transplant patients. Human polyomavirus BK (BKV) (or less frequently adenovirus) may be involved in the pathogenesis of viral HC and can represent a serious post-transplant complication. diagnosis and treatment of viral HC can be difficult and has an uncertain outcome. We report the efficacy of sequential vidarabine in the treatment of a patient with severe BKV-associated HC, despite the delay in implementing therapy. bone marrow transplantation (2000) 25, 319-320.- - - - - - - - - - ranking = 5keywords = bone (Clic here for more details about this article) |
7/67. colchicine poisoning by accidental ingestion of meadow saffron (colchicum autumnale): pathological and medicolegal aspects.Although intoxications with colchicine, the alkaloid of colchicum autumnale (meadow saffron), are well known, in most cases the intoxications are evoked by oral or parenteral preparations traditionally used as medication against gout. The accidental ingestion of colchicum autumnale, on the other hand, is a rare event and has to our knowledge only twice been described in detail. We report a further case in which two persons confused this highly poisonous plant with wild garlic (allium ursinum), a popular spice in the Central European cuisine. While one person merely complained about a 3-day episode of nausea, vomiting and watery diarrhea, the second person died of multi-organ system derangements 48 h after the ingestion of the colchicum leaves. At autopsy hemorrhagic lung oedema, hypocellular bonemarrow, centrilobular fatty necrosis of the liver and necrosis of the proximal convoluted tubuli of the kidneys were observed. A colchicine concentration of 7.5 micrograms/ml was found in the bile whereas no substance was detected in the postmortem blood.- - - - - - - - - - ranking = 1keywords = bone (Clic here for more details about this article) |
8/67. Pseudomembranous gastritis: a novel complication of aspergillus infection in a patient with a bone marrow transplant and graft versus host disease.A 36-year-old Hispanic man who had undergone allogeneic bone marrow transplantation, complicated by graft versus host disease, was admitted with acute gastrointestinal symptoms, including severe diarrhea and diffuse abdominal pain. He also had a persistent cough with sputum production. blood cultures yielded escherichia coli, and sputum cultures grew Apergillus species. The patient was treated with antifungal agents and broad-spectrum antibiotics. Despite aggressive medical therapy, the patient died 10 days after admission. Postmortem examination disclosed severe, bilateral confluent bronchopneumonia, with numerous septated branching hyphae consistent with aspergillus species fungal organisms that involved the pulmonary parenchyma and tracheobronchial tree. Although the small and large bowels were only mildly congested, the entire gastric mucosa was covered with a 1.5-cm-thick pseudomembrane that contained numerous aspergillus organisms. Our report represents the first description, to our knowledge, of a diffuse inflammatory pseudomembrane in the stomach, a complication that to date has only been associated with small and large bowel involvement.- - - - - - - - - - ranking = 5keywords = bone (Clic here for more details about this article) |
9/67. Diffuse cutaneous mastocytosis with bone marrow infiltration in a child: a case report.mastocytosis encompasses a range of disorders characterized by overproliferation and accumulation of tissue mast cells. Mast cell disease is most commonly seen in the skin, but the skeleton, gastrointestinal tract, bone marrow, and central nervous system may also be involved. We present a 10-year-old boy with diffuse cutaneous mastocytosis characterized by disseminated papular, nodular, and infiltrated leathery lesions. The patient presented with chronic diarrhea and malnutrition. Laboratory studies were normal except for an elevated urinary 1-methylhistamine level. The bone marrow aspirate showed a dense mast cell infiltrate confirming systemic involvement.- - - - - - - - - - ranking = 6keywords = bone (Clic here for more details about this article) |
10/67. Continuous octreotide infusion for the treatment of secretory diarrhea caused by acute intestinal graft-versus-host disease in a child.This report describes the use of octreotide, a synthetic somatostatin analogue, for severe diarrhea caused by acute intestinal graft-versus-host disease (GVHD) after bone marrow transplantation. A 22-month-old boy suffered grade 4 intestinal GVHD, with profuse diarrhea, intestinal inflammation, and grossly bloody stools after matched, unrelated donor transplant for biphenotypic leukemia. He required intensive blood product support. In addition to aggressive anti-GVHD therapy, octreotide acetate was initiated at 30 microg (2 microg/kg) intravenously 3 times per day and escalated to continuous infusion at 15 microg/hr (1 microg/kg per hour). The diarrhea did not improve with anti-GVHD treatment. However, moderate dose octreotide therapy resulted in prompt control of the bloody diarrhea, which rebounded on cessation of octreotide therapy. Rebound diarrhea responded promptly when the dose of octreotide was escalated. octreotide was associated with an exacerbation of preexisting hypertension, but it appeared to be effective for control of severe, bloody diarrhea caused by acute GVHD in a child, with manageable side effects. Further studies of this application in infants and children are warranted.- - - - - - - - - - ranking = 1keywords = bone (Clic here for more details about this article) |
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