Gene/Protein Disease Symptom Drug Enzyme Compound
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This brief review of abdominal emergencies is by no means encyclopedic. Indeed, it simply reflects the multiplicity of problems that can occur and suggests the need for a high index of suspicion and an optimistic attitude toward their solution. In addition, the surgeon must keep in mind the fact that cancer patients may also suffer acute abdominal distress from extra-abdominal causes such as pneumonia, myocardial infarction, diabetes mellitus, and hematologic abnormalities such as porphyria or sickle cell anemia. Inflammatory bowel disease, pelvic inflammatory disease, acute hepatitis or other similar problems more commonly seen in general hospital populations may also develop. Consultations for an acute condition of the abdomen in patients receiving marrow-suppressing chemotherapy are challenging problems and repeated examination every few hours is required to detect subtle changes. Hypovolemia, sepsis, confusion and unexplained metabolic acidosis may be the only criteria for surgical exploration. An unnecessary operation in a leukopenic and thrombocytopenic patient is indeed risky, but failure to drain an occult abscess or resect a perforated segment of bowel is always lethal. An additional consideration is the likelihood of response to further treatment of the underlying disease. Unless further effective therapy is unavailable, pessimism is unwarranted.
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PMID:Abdominal emergencies. 31 58

Rheumatoid arthritis is being increasingly treated with sulphasalazine. We report the first case of a patient being treated for rheumatoid arthritis who developed severe dyspnoea with chest radiograph shadowing, reversible on discontinuation of sulphasalazine and subsequent steroid therapy. An histological diagnosis of fibrosing alveolitis was made. Thirteen cases of similar reactions to sulphasalazine, all in patients treated for inflammatory bowel disease, can be found in the literature. We identify two types of pulmonary reaction, an eosinophilic pneumonitis and a fibrosing alveolitis. Adequate histological investigation is needed to differentiate between the two and management may be different. The diagnosis of pulmonary reactions are important because they can be easily confused with complications of the underlying disease but the prognosis is much better.
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PMID:Fibrosing alveolitis due to sulphasalazine in a patient with rheumatoid arthritis. 197 34

A 29-yr-old white woman presented with chronic pneumonia in the left lower lobe and with left pleural effusion. She was known to have inflammatory bowel disease, but she was asymptomatic under maintenance treatment with 5-ASA. She received numerous antibiotic regimens according to susceptibility testing of microorganisms cultured from sputum and bronchial lavage and on an empiric basis was also given antituberculosis treatment, but there was no clinical improvement or change in the chest radiographic findings. Sputum was repeatedly examined and yielded, among other organisms, Clostridium inocuum, Enterobacter, Klebsiella pneumoniae, Proteus mirabilis, and Staphylococcus aureus. On one microscopic examination of sputum, the presence of feculent material was suspected. A subsequent gastrografin enema revealed a cologastric and colobronchial fistula between the splenic flexture of the colon and the greater curve of the stomach and the bronchial system. Segmental resection of the colon and resection of the lower lobe of the left lung were performed. Histologic findings of the resected colon were consistent with Crohn's disease. After a long period of postoperative recovery, the patient returned to good general health and well-being. To our knowledge, a colobronchial fistula caused by Crohn's colitis has not been previously reported.
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PMID:Colobronchial fistula: a rare complication of Crohn's colitis. 224 Aug 49

Pneumonitis, bilateral pleural effusions, echocardiographic evidence of cardiac tamponade, and positive autoantibodies developed in a 43-year-old man, who was receiving long-term sulfasalazine therapy for chronic ulcerative colitis. After cessation of the sulfasalazine and completion of a six-week course of corticosteroids, these problems resolved over a period of four to six months. It is suggested that the patient had sulfasalazine-induced lupus, which manifested with serositis and pulmonary parenchymal involvement in the absence of joint symptoms. Physicians who use sulfasalazine to treat patients with inflammatory bowel disease should be aware of the signs of sulfasalazine-induced lupus syndrome.
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PMID:Sulfasalazine-induced lupus erythematosus. 289 66

A twenty six year old homosexual male with the diseases Hepatitis B, Lues, Gonorrhoea, ascending myelitis, lymphadenitis got additionally a chronic-inflammatory bowel disease, which first was discussed as Crohn's disease. Two "attacks" were "successfully" treated with Methylprednisolone. The patient died twelve month later in the follow of a pneumocystitis carinii pneumonia. The cause of the symptoms of the chronic-inflammatory bowel disease was a cytomegalovirus infection with acquired immune deficiency-syndrome.
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PMID:[AIDS--Crohn disease as a diagnostic error. Case report]. 301 28

Three bacterial strains of Bifidobacterium and Clostridium sp. from patients with inflammatory bowel disease (I.B.D.) and Streptococcus pneumoniae from a patient with pneumonia were identified to produce extracellular proteases cleaving IgA into Fab and Fc fragments. Although the proteases from the Bifidobacterium and the Streptococcus pneumoniae showed the characteristics of typical IgA1 proteases, cleaving the IgA of only the IgA1 subclass, the protease from Clostridium sp. revealed a dual substrate specificity, in that it cleaved both IgA1 and IgA2 of the A2m(1) allotype. The latter protease, however, did not show any activity with respect to the IgA2 of the A2m(2) allotype. Fc fragments isolated from the IgA1 and the IgA2 A2m(1) by digestion with the Clostridium sp. protease were identified to have an identical amino terminal residue of valine. The site of cleavage in both the alpha 1 and the alpha 2 of A2m(1) by the protease was assumed to be an identical peptide bond at Pro(221)-Val(222), which is a common one present just before the hinge of both the alpha 1 and the alpha 2 of the A2m(1) but not of the alpha 2 of the A2m(2). The protease was sensitive to ethylene-diamino tetraacetic acid, a chelating agent, similar to other already reported IgA1 proteases.
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PMID:A novel IgA protease from Clostridium sp. capable of cleaving IgA1 and IgA2 A2m(1) but not IgA2 A2m(2) allotype paraproteins. 388 May 75

Four of five patients survived perforation of the colon incidental to barium-enema examination, while the other survived without sequela of infection until hemiplegia and pneumonia supervened two and 36 days, respectively, postoperatively. It is postulated that adequate intravenous fluids, early operation, with complete cleansing by antibiotic irrigation and mechanical debridement of the abdominal cavity, along with excision of the perforated segment of bowel with establishment of an end colostomy or ileostomy and distal mucous fistula, if possible, are keys to survival in these patients. Triple antibiotics, using ampicillin, gentamicin, and clindamycin, or metronidazole, should be administered in all patients as soon as the diagnosis is made, and continued postoperatively as indicated until there is no evidence of infection. An intraluminal intestinal tube, for stichless plication of the small bowel, may be helpful in some patients. A stomacone should be used for a barium enema through a colostomy stoma. Care should be exercised in performing barium contrast studies in patients with inflammatory bowel disease.
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PMID:Survival after colonic perforation during barium-enema examination. Modified radical surgical debridement. 682 70

We report on a 6-year-old boy with mosaic trisomy 9. The patient was born at 42 weeks of gestation to a 27-year-old G1 white woman. Birth weight was 2,820 g, length 52 cm, and Apgar scores were 4 and 6 at 1 and 5 min, respectively. The infant presented with apparently low-set ears, overfolded helices, epicanthal folds, prominent nasal bridge, high-arched palate, micrognathia, bilateral dislocated hips, left genu recurvatum, and cryptorchidism. Chromosome analysis showed an unusual karyotype: 47,XY,+inv(9qh+)/47,XY,+mar. The marker chromosome was thought to be a remnant of the inv(9qh+) chromosome. The mother's karyotype was 46,XX,inv(9qh+), while the father's was 46,XY. At age 5 months, the patient developed seizures and gastroesophageal reflux. Crohn disease was diagnosed at age 2 years, although symptoms began at age 1 year. Recurrent bouts of pneumonia have occurred since the patient's birth. Severe psychomotor retardation was also noted. Trisomy 9 syndrome was first reported in 1973. Over 30 cases have been reported since then. Of these cases reports, only 5 patients were older than 1 year. Inflammatory bowel disease has been reported in association with other chromosome abnormalities, but to our knowledge, has not been reported in trisomy 9 syndrome.
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PMID:Trisomy 9 syndrome: report of a case with Crohn disease and review of the literature. 777 85

Myelosuppression is an important and potentially lethal complication of azathioprine treatment. The blood count has been reviewed in all patients treated with azathioprine for inflammatory bowel disease over 27 years in one hospital. Altogether 739 patients (422 with Crohn's disease, 284 with ulcerative colitis, and 33 with indeterminate colitis) were treated with 2 mg/kg/day azathioprine for a median of 12.5 months (range 0.5-132) between 1964 and 1991. Full blood counts were performed monthly for the duration of treatment. In 37 patients (5%) who developed bone marrow toxicity, the drug was withdrawn or the dose reduced. Thirty two of these patients were asymptomatic and five developed symptoms. Leucopenia (white blood count less than 3.0 x 10g/l) occurred in 28 (3.8%) patients, in nine of whom it was severe (white blood count < 2.0 x 10(9)/l). Of these nine patients, three were pancytopenic: two died from sepsis and the other had pneumonia but recovered. A further two patients with severe leucopenia developed a mild upper respiratory infection only. Thrombocytopenia (platelet count < 100,000 x 10(6)/l) in 15 patients was associated with leucopenia in six and developed in isolation in a further nine (total 2%). Isolated thrombocytopenia was never clinically severe. Myelotoxicity from azathioprine developed at any time during drug treatment (range 2 weeks-11 years after starting the drug) and occurred either suddenly or over several months. Bone marrow suppression as a result of azathioprine treatment is uncommon when a moderate dose is used, but is potentially severe. Leucopenia is the commonest and most important haematological complication. Regular monitoring of the full blood count is recommended during treatment.
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PMID:Bone marrow toxicity caused by azathioprine in inflammatory bowel disease: 27 years of experience. 817 58

Respiratory involvement in patients with inflammatory bowel disease (IBD) has been reported mainly since 1976. This form of involvement should clearly be separated from interstitial lung disease due to sulfasalazine or mesalamine, although the distinction may be difficult in some cases. We report the data of an ongoing Registry containing 33 cases (23 cases receiving no drug therapy) with ulcerative colitis or, less often, Crohn's disease, who developed varied bronchopulmonary problems. In several cases, the exact diagnosis and the relation of the bronchopulmonary disease to IBD had not been established for many years, thus delaying effective treatment with steroids. In most cases (28/33), respiratory involvement followed the onset of IBD (8 of these 28 cases were postcolectomy), and in the remainder, respiratory manifestations predated the IBD. Patterns of involvement included: 1) Airway inflammation, in the form of subglottic stenosis, chronic bronchitis, severe chronic bronchial suppuration, bronchiectasis, and chronic bronchiolitis. In cases with large airway involvement, endoscopy showed exuberant inflammatory tissue in the airways and narrowing of tracheal and/or bronchial lumen. Histologically, airways were heavily infiltrated by a dense aggregate of inflammatory cells, and there were mucosal ulcerations. 2) Varied patterns of interstitial lung disease, mainly bronchiolitis obliterans with organizing pneumonia, and pulmonary infiltrates and eosinophilia. (3) Miscellaneous other forms of involvement including striking neutrophilic necrotic parenchymal nodules (corresponding histologically to sterile aggregates of neutrophils), and serositis. Steroids were very effective in the majority of cases. Inhaled steroids were of durable benefit in patients with chronic bronchitis, but less often so in those with chronic bronchial suppuration, bronchiectasis, or chronic bronchiolitis. Steroids administered orally led to marked improvement in patients with interstitial lung disease and necrotic nodules, but lacked effectiveness in several patients with severe airway inflammation or chronic bronchiolitis. Intravenous steroids were required in the initial management of life-threatening complications such as asphyxiating subglottic stenosis or extensive interstitial lung disease. Bronchial lavages with methylprednisolone were effective in some patients with severe airway inflammation. Patients with IBD can develop varied inflammatory complications in the lung, and a sizable fraction of these complications is steroid-sensitive.
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PMID:The lung in inflammatory bowel disease. 850 68


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