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)

A 62 year old man with chronic lymphocytic leukaemia presented with malabsorption, the cause of which could not be found during life. Necropsy examination showed aspergillosis, limited to the stomach, where tumour-like masses were seen, the oesophagus, and lungs. This case illustrates the problems of diagnosing fungal infections in life and the importance of clinicopathological correlation at necropsy.
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PMID:Gastrointestinal tract aspergilloma: possible cause of malabsorption. 813 33

The systematic fungal infections are very serious diseases, the crude mortality in invasive aspergillosis reaches 75 to 100 per cent. Systemic mycosis tends to develop in immunocompromised patients with subsequent physiopathological state, causing a risk of impairment in their digestive absorption potential; in addition these patients are polymedicated and the emergence of multiple drug interactions is frequent. Itraconazole is a very potent antifungal drug with large safety margin and the drug monitoring to maintain a satisfactory plasma level. Immunocompromised patients with suspected malabsorption and treated with oral capsule itraconazole were monitored during two years. In such difficult patients (approximately equal to 500 cases), the result has shown good trough steady-state plasma levels in 72 per cent and insufficient in 28 per cent of the observed patients. However concomitant treatments with antacids produce, in a lot of cases (approximately equal to 48 per cent), a noticeable decrease of the itraconazole availability for these patients. A training program was established to perform the analytical determination of itraconazole and hydroxyitraconazole in biological samples by HPLC method. Analytical validation procedures were associated to this training program which included 115 scientific, technical staff (pharmacists, biochemists ...) from 56 hospitals and institutes.
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PMID:[Example of active therapeutic follow-up: itraconazole]. 913 84

Antimicrobial prophylaxis in neutropenic patients has been practised in one form or another for several decades but the goal is no longer clear. From being initially solely an attempt at decontamination, drugs such as co-trimoxazole and later the fluoroquinolones were preferred to non-absorbable regimens because they achieve reliable protection against bacteraemia due to Gram-negative bacilli. Nevertheless, fever still invariably occurs during neutropenia leading to the initiation of traditional empirical therapy. Not only is this approach illogical but it also ignores the flexibility afforded the oral and parenteral formulations of the fluoroquinolones. Instead, it might be as effective and less costly if these agents were given orally until the end of neutropenia unless there was evidence of malabsorption or poor oral intake, in which case treatment would be continued parenterally. Should patients develop fever, an attempt would be made to complement treatment with another anti-microbial agent for microbiologically or clinically defined infection. This would be carried out at diagnosis, before any changes in the prophylactic regimen could be made. Otherwise, treatment with the prophylactic regimen would continue without modification. There is a less compelling need for prophylaxis against candidosis, herpes simplex and cytomegalovirus disease as these would be better managed pre-emptively when there is evidence of yeast carriage or re-activation of viral infection. Similarly, prophylaxis of aspergillosis is a forlorn hope and again a pre-emptive approach might serve us better once there is a screening test available and a safe and effective drug.
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PMID:Is there a rationale for the use of antimicrobial prophylaxis in neutropenic patients? 935 Jan 87

Cystic fibrosis is a recessive disease that causes changes in mucus secretions, affecting different systems: respiratory, digestive, pancreatic, hepatic; resulting in obstructions and secondary infections. Transplantation may be used for the most severe forms and is then complicated by the pediatric context, the existence of malabsorption and secondary infections and the type of transplantation (pulmonary and/or hepatic). The follow-up is characterized by pulmonary infections and pulmonary chronic rejection. In our experience, the initiation of the immunosuppressive treatment must avoid corticoids in the early post-transplantation days and have recourse to intravenous cyclosporin (CyA) 2 mg/kg/day, given on average for one month in an oral form. In case of persistent acute rejection, tacrolimus (FK 506) is instituted. Oral CyA (10-12 mg/kg/day) seems more sensitive to malabsorption syndrome than FK 506 (0.2 mg/kg/day). In both cases, the development of an inhibitory metabolic interaction in the presence of itraconazole must be taken into account: used against aspergillosis, itraconazole is metabolized as CyA and FK 506 by Cyt P450 3A4. The intensity of the interaction is twofold for CyA versus fivefold for FK 506. The strategy for the use of other recently available immunosuppressives such as mycophenolate is under evaluation.
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PMID:[Immunosuppression: a case of mucoviscidosis]. 943 88

Dyskeratosis congenita (DC) is a rare inherited disorder often associated with aplastic anaemia. We report the cases of five boys transplanted with an HLA-identical related donor for severe aplastic anaemia (SAA) associated to DC; in all cases successful engraftment was observed. Three patients died 2-8 years after bone marrow transplantation (BMT) with signs of endothelial cell damage syndrome (kidney microangiopathy and liver veno-occlusive disease). Another boy died 1 year after BMT from Evans syndrome and invasive aspergillosis. One boy currently presents anaemia, polyarthritis of unknown origin, pulmonary fibrosis and gut malabsorption 7.5 years after BMT. SAA associated with DC can be successfully treated by allogeneic BMT. However, these early and late complications observed are very unusual after BMT and probably reflect the association of transplanted-related factors, evolution of the underlying disease, and increased sensitivity of endothelial cells. Modified conditioning approaches, advances in supportive care and surveillance of these unusual complications offer the possibility of improved outcome for these patients.
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PMID:Unusual complications after bone marrow transplantation for dyskeratosis congenita. 979 16

Cystic fibrosis (CF) is a common autosomal-recessive inherited disease, which often results in premature death. Due to treatment advances, life expectancy has however continuously improved in recent years. Currently about half of all patients are adults. There are also "atypical" variants of CF with symptoms occurring in late adulthood. CF is caused by a mutation in the gene coding for a chloride ion channel, known as the cystic fibrosis transmembrane conductance regulator (CFTR). This mutation results in abnormally viscous mucosal secretions, leading to multi-organ disease with particular emphasis in the respiratory and digestive tracts. Impaired mucociliary clearance results in bacterial colonization of the airways (e. g. Pseudomonas aeruginosa) and consequently in chronic pulmonary inflammation, inevitably leading to progressive bronchiectasis and combined ventilatory disorders. Typical acute complications are infective exacerbations - the most frequent cause of death in cystic fibrosis - along with allergic bronchopulmonary aspergillosis, haemoptyses and pneumothoraces. Involvement of the gastrointestinal tract generally manifests as exo- and later endocrine pancreatic insufficiency with diabetes mellitus, malabsorption and sometimes biliary liver cirrhosis. Typical acute complications are pancreatitis and ileus. The article describes epidemiology and pathophysiology of CF and focuses on the signs and symptoms, as well as the diagnostic and multi-modal therapeutic strategies used in adult patients.
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PMID:[Cystic fibrosis in adults]. 2012 4