1/12. Case report: portal vein thrombosis associated with hereditary protein c deficiency: a report of two cases.protein c deficiency is one of the causes of curable or preventable portal vein thrombosis. We report two patients of portal vein thrombosis associated with hereditary protein c deficiency. The first patient presented with continuous right upper quadrant pain and high fever. The abdominal sonography revealed normal liver parenchyma but portal vein and superior mesenteric vein thrombosis. Based on a 55% (normal 70-140%) plasma protein C level, he was diagnosed as having protein c deficiency. A trace of his family history showed that his elder brother also had protein c deficiency with a 50% plasma C level. Both patients received anticoagulant therapy. The younger brother showed good response. Unfortunately, the elder one suffered from recurrent episodes of variceal bleeding and received a life-saving splenectomy and devascularization. We herein remind clinicians that early screening and therapy are helpful in preventing late complications of protein c deficiency with portal vein thrombosis.- - - - - - - - - - ranking = 1keywords = deficiency (Clic here for more details about this article) |
2/12. Recurrent haemoperitoneum in a mild von Willebrand's disease combined with a storage pool deficit.Haemoperitoneum secondary to haemorrhagic corpus luteum has been described in severe bleeding disorders such as afibrinogenaemia, type 3 von Willebrand's disease and patients under oral anticoagulation. We have studied one patient who presented three episodes of severe bleeding at ovulation, requiring surgery twice, with the diagnosis of mild von Willebrand's disease and mild storage pool deficiency. Mild von Willebrand's disease (associated with other thrombopathies or coagulopathies) should be considered in this pathology, although physicians would prefer to find a severe haemorrhagic disorder as the underlying condition in these cases.- - - - - - - - - - ranking = 0.11111111111111keywords = deficiency (Clic here for more details about this article) |
3/12. Antiretroviral-induced hepatic steatosis and lactic acidosis: case report and review of the literature.As the prevalence of human immunodeficiency virus (hiv) infection continues to rise the clinician is encountered with a diagnostic challenge. Nonsurgical diseases such as acute colitis or enteritis can appear similar to such true surgical emergencies as abscess, perforation, or mesenteric ischemia. We report a case of fulminant hepatic failure associated with didanosine and masquerading as a surgical abdomen and compare the clinical, biologic, histologic, and ultrastructural findings with reports described previously. This entity should be kept in mind when evaluating the acute abdomen in the hiv-positive patient.- - - - - - - - - - ranking = 0.11111111111111keywords = deficiency (Clic here for more details about this article) |
4/12. Changes in splenoportal axis calibre and flow in a patient affected by hereditary angioedema.The authors describe a case of hereditary angioedema characterised by abdominal pain accompanied by ascites. Ultrasound (US) examination performed after acute abdominal attack implied the presence of increased splenoportal axis calibre and reduced blood flow. According to the authors, this may confirm the pathogenic role of C1-inhibitor deficiency induced oedema that is capable of creating major haemodynamic involvement also of abdominal vessels. US findings of transient appearance, especially related to the specific treatment, may help physicians make early diagnosis and avoid dangerous invasive procedures resulting from incorrect diagnosis of acute abdomen.- - - - - - - - - - ranking = 0.11111111111111keywords = deficiency (Clic here for more details about this article) |
5/12. Acute abdominal attack of hereditary angioneurotic oedema associated with ultrasound abnormalities suggestive of acute hepatitis.Hereditary angioneurotic oedema (HANO) is an autosomal dominant disorder caused by a deficiency of the inhibitor protein Cl-esterase. Recurrent subcutaneous and/or submucosal oedema formation is a hallmark of this disease. HANO is a rare, but potentially life-threatening disorder with a mortality around 20-30%. Acute oedematous abdominal attacks of HANO can mimic a surgical emergency; this is exemplified by the case of a 14-y-old male patient with HANO admitted for such clinical manifestations. Conclusion: Diagnostic clues include ascites and abnormalities of hepatic structure visible with ultrasound during the oedematous attack. The importance of appropriate treatment is emphasized.- - - - - - - - - - ranking = 0.11111111111111keywords = deficiency (Clic here for more details about this article) |
6/12. Severe acute necrotizing pancreatitis associated with lipoprotein lipase deficiency in childhood.An 11-year-old girl with lipoprotein lipase deficiency experienced recurring episodes of abdominal pain. She initially underwent appendectomy for suspected appendicitis; however, the appendix was normal. pancreatitis was subsequently identified as the cause of her pain.- - - - - - - - - - ranking = 0.55555555555556keywords = deficiency (Clic here for more details about this article) |
7/12. Addison's disease presenting with an acute abdomen and complicated by cardiomyopathy.adrenal insufficiency typically presents with non-specific symptoms, followed by shock secondary to a salt-wasting crisis and/or the vascular effects of glucocorticoid deficiency if it progresses to acute adrenal insufficiency. rhabdomyolysis and cardiac dysfunction are rarely reported, particularly in children. We report a child with adrenal insufficiency, presenting with acute abdominal signs, probably secondary to rhabdomyolysis, and complicated by reversible cardiomyopathy. Acute adrenal insufficiency should be considered with this presentation. The possibility of impaired cardiac function during its treatment should also be considered.- - - - - - - - - - ranking = 0.11111111111111keywords = deficiency (Clic here for more details about this article) |
8/12. Intestinal invasion and disseminated disease associated with penicillium chrysogenum.BACKGROUND: Penicillium sp., other than P. marneffei, is an unusual cause of invasive disease. These organisms are often identified in immunosuppressed patients, either due to human immunodeficiency virus or from immunosuppressant medications post-transplantation. They are a rarely identified cause of infection in immunocompetent hosts. CASE PRESENTATION: A 51 year old African-American female presented with an acute abdomen and underwent an exploratory laparotomy which revealed an incarcerated peristomal hernia. Her postoperative course was complicated by severe sepsis syndrome with respiratory failure, hypotension, leukocytosis, and DIC. On postoperative day 9 she was found to have an anastamotic breakdown. pathology from the second surgery showed transmural ischemic necrosis with angioinvasion of a fungal organism. Fungal blood cultures were positive for penicillium chrysogenum and the patient completed a 6 week course of amphotericin b lipid complex, followed by an extended course oral intraconazole. She was discharged to a nursing home without evidence of recurrent infection. DISCUSSION: penicillium chrysogenum is a rare cause of infection in immunocompetent patients. Diagnosis can be difficult, but Penicillium sp. grows rapidly on routine fungal cultures. prognosis remains very poor, but aggressive treatment is essential, including surgical debridement and the removal of foci of infection along with the use of amphotericin b. The clinical utility of newer antifungal agents remains to be determined.- - - - - - - - - - ranking = 0.11111111111111keywords = deficiency (Clic here for more details about this article) |
9/12. Acute lead poisoning in inherited porphobilinogen synthase (delta-aminolevulinic acid dehydrase) deficiency.The second enzyme of the porphyrin and heme synthesis pathway, porphobilinogen synthase (EC 4.2.1.24), can be lowered due to a hereditary defect or toxic damage. Lead is the most common cause for a toxically lowered activity. We report on a 48-year-old patient with an acute abdominal syndrome, anemia and a grave toxic disorder of porphyrin metabolism which was diagnosed as acute lead intoxication. Although the hematologic findings and porphyrins in the blood, as well as porphyrin precursors and porphyrin excretion in the urine, returned to normal within five months after exacerbation of the acute symptoms the activity of porphobilinogen synthase showed only 50% physiologic activity of healthy subjects six years after the acute syndrome with complete normalization of prophyrin metabolites and lead levels in blood and urine. The influence of alcohol was excluded. enzyme activation by zinc and reactivation by dithiothreitol lies within the normal range and thus contradicts an increased body burden of lead. In connection with findings of a familiar porphobilinogen synthase defect we are most likely dealing with a hereditarily determined enzyme deficiency in this patient which sensitizes him to a lead intoxication.- - - - - - - - - - ranking = 0.55555555555556keywords = deficiency (Clic here for more details about this article) |
10/12. Surgical treatment of lung cancer in patients with human immunodeficiency virus.BACKGROUND. Since January 1986, more than 20 patients have been seen at the University of Miami/Jackson Memorial Medical Center and the Miami veterans Administration Medical Center with concurrent human immunodeficiency virus infection and bronchogenic carcinoma. Four of these patients were treated surgically with curative intent. methods. The histories, records, operative reports, and pathology reports of the 4 patients were reviewed. RESULTS. The 4 surgically treated patients had stage I T1 N0 M0 lung cancer. Three patients had T4 cell counts of less than 200/microL and were managed by lobectomy. These patients died 5, 3 1/2, and 5 months postoperatively. More recently, a fourth patient had a T4 cell count of 963/microL and was treated with wedge resection. He is currently alive 12 months postoperatively. CONCLUSIONS. It is concluded that surgically treated patients with lung cancer, human immunodeficiency virus infection, and T4 cell counts lower than 200/microL have high mortality and morbidity. Although it may be best to base surgical intervention on the stage of the patient's human immunodeficiency virus infection, further analysis is essential to determine which subgroup of human immunodeficiency virus-positive patients, if any, would benefit from surgical treatment of lung cancer.- - - - - - - - - - ranking = 0.88888888888889keywords = deficiency (Clic here for more details about this article) |
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