Cases reported "Hyperbilirubinemia"

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1/12. Three episodes of delayed hemolytic transfusion reactions due to multiple red cell antibodies, anti-Di, anti-Jk and anti-E.

    There is no report in which three episodes of delayed hemolytic transfusion reaction (DHTR) occurred from multiple antibodies to red cells (RBCs) in the course of treatment of a patient. This paper describes episodes of anemia and hyperbilirubinemia in concert with the development of three alloantibodies in a multiple transfused patient. The patient was a 71-year-old male suffering from valvular heart disease and hemophilia b with a history of transfusions. Although he received compatible RBCs from 14 donors as judged by a crossmatch test using the albumin-antiglobulin method, three episodes of DHTR occurred after surgery. The first hemolytic episode on day 7 after surgery was due to anti-Di(a) because of clinical and laboratory evidence which included jaundice, sudden increases in total bilirubin (T-Bil) and lactate dehydrogenase (LD) levels, and a decrease (2.2 g/dl) in hemoglobin (Hb) level. The second hemolytic episode on day 16 resulted from newly producted anti-Jk(b). The patient experienced fever, fatigue, nausea and anorexia, and laboratory data showed a second increase in T-Bil, a second decrease (3 g/dl) in Hb, and moderate elevations of blood urea nitrogen (BUN) and creatinine (CRE) levels. The third hemolytic episode on day 39 was due to anti-E. The patient complained of fever and fatigue and had a third unexplained drop (1.5 g/dl) in Hb despite no bleeding. This is the first reported case in which three episodes of DHTR occurred from different red cell antibodies.
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2/12. Potential benefit of plasma exchange in treatment of severe icteric leptospirosis complicated by acute renal failure.

    leptospirosis is a common zoonosis seen worldwide, but it is rare in our locality (hong kong). Clinical manifestations of leptospirosis are variable and may range from subclinical infection to fever, jaundice, hemorrhagic tendency, and fulminant hepato-renal failure. Severe hyperbilirubinemia and acute renal failure have been associated with high mortality. We report our experience with a patient who developed severe Weil's syndrome with marked conjugated hyperbilirubinemia and oliguric acute renal failure. These complications persisted despite treatment with penicillin and hemodiafiltration. plasma exchange was instituted in view of the severe hyperbilirubinemia (970 micromol/liter). This was followed by prompt clinical improvement, with recovery of liver and renal function. The beneficial effects of plasma exchange could be attributed to amelioration of the toxic effects of hyperbilirubinemia on hepatocyte and renal tubular cell function. We conclude that plasma exchange should be considered as an adjunctive therapy for patients with severe icteric leptospirosis complicated by acute renal failure who have not shown rapid clinical response to conventional treatment.
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3/12. The evolution of lemierre syndrome: report of 2 cases and review of the literature.

    lemierre syndrome (postanginal septicemia) is caused by an acute oropharyngeal infection with secondary septic thrombophlebitis of the internal jugular vein and frequent metastatic infections. A high degree of clinical suspicion is necessary for diagnosis. fusobacterium necrophorum is the usual etiologic agent. The disease progresses in several steps. The first stage is the primary infection, which is usually a pharyngitis (87.1% of cases). This is followed by local invasion of the lateral pharyngeal space and IJV septic thrombophlebitis (documented in 71.5% of cases), and finally, the occurrence of metastatic complications (present in 90% of cases at the time of diagnosis). A sore throat is the most common symptom during the primary infection (82.5% of cases). During invasion of the lateral pharyngeal space and IJV septic thrombophlebitis, a swollen and/or tender neck is the most common finding (52.2% of patients) and should be considered a red flag in patients with current or recent pharyngitis. The most common site of metastatic infection is the lungs (79.8% of cases). In contrast to the preantibiotic era, cavitating pneumonia and septic arthritis are now uncommon. Most patients (82.5%) had fever at some stage during the course of the disease. Gastrointestinal complaints such as abdominal pain, nausea, and vomiting were common (49.5% of cases). An elevated white blood cell count occurred in 75.2% of cases. hyperbilirubinemia with slight elevation of liver enzyme levels occurred in one-third of patients, but frank jaundice was uncommon, in contrast to its high frequency reported in the preantibiotic era. We conclude that, most likely as a consequence of widespread antibiotic use for pharyngeal infections, the typical course of the disease has changed since Lemierre's original description. The typical triad in our series was: pharyngitis, a tender/swollen neck, and noncavitating pulmonary infiltrates. The previous classical description of severe sepsis with cavitating pneumonia and septic arthritis was not commonly seen in our review. mortality was low in our series (6.4%), but significant morbidity occurred, which was likely preventable by early diagnosis and treatment. The pathophysiology, natural history, diagnostic methods for internal jugular vein thrombosis, and management are discussed.
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4/12. The urgency of diagnosis and surgical treatment of acute suppurative cholangitis.

    Twenty patients with suppurative cholangitis were seen at the massachusetts General Hospital over a nine year period. Fifteen patients had acute obstructive suppurative cholangitis due to complete obstruction of the common duct, many with coma, hypotension, and positive blood cultures. Sixty per cent of patients were older than seventy years, and most had a history of biliary tract disease. Although most had jaundice, abdominal pain, and fever, clinical symptoms were variable. The diagnosis of cholangitis was made in only 30 per cent of patients before autopsy or surgery. Eighteen patients had calculi in the common duct, and two had primary fibrosis of the ampulla. patients explored less than 24 hours after admission or deterioration died less often than those operated on after some delay. Most patients underwent common duct exploration and four had a concomitant sphincterotomy. In one instance, cholecystostomy only was performed and this patient died because of ongoing sepsis. The overall mortality was 40 per cent; of those subjected to operation, 25 per cent died in the hospital. Recovery was dramatic among most survivors, and calculous disease did not recur, except for two patients with retained stones. Prophylactic cholecystectomy is recommended to prevent the occurrence of this subtle and highly dangerous syndrome.
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5/12. dapsone induced cholangitis as a part of dapsone syndrome: a case report.

    BACKGROUND: dapsone can rarely cause a hypersensitivity reaction called dapsone syndrome, consisting of fever, hepatitis, exfoliative dermatitis, lymphadenopathy and hemolytic anemia. dapsone syndrome is a manifestation of the DRESS (drug rash with eosinophilia and systemic symptoms) syndrome which is a serious condition that has been reported in association with various drugs. cholangitis in dapsone syndrome has not been reported so far in the world literature. CASE PRESENTATION: We report a patient who presented with fever, exfoliative dermatitis, jaundice and anemia within three weeks of starting of dapsone therapy. These features are typical of dapsone syndrome, which is due to dapsone hypersensitivity and is potentially fatal. Unlike previous reports of hepatitic or cholestatic injury in dapsone syndrome we report here a case that had cholangitic liver injury. It responded to corticosteroids. CONCLUSION: We conclude that cholangitis, though unusual, can also form a part of dapsone syndrome. physicians should be aware of this unusual picture of potentially fatal dapsone syndrome.
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6/12. Is hyperbilirubinemia a component or just a coincidence of familial mediterranean fever: a case report and review of the literature.

    familial mediterranean fever is a multisystem disorder, usually seen in subjects of Mediterranean and Middle Eastern origin, characterized by recurrent bouts of fever and pain due to inflammation of the peritoneum, synovia, or pleura. In this article we report a case of familial mediterranean fever with recurrent abdominal pain and hyperbilirubinemia, review the literature and discuss whether the hyperbilirubinemia is co-existant or a feature of the disease.
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keywords = mediterranean fever, familial mediterranean fever, fever
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7/12. Dubin-Johnson syndrome--a clinicopathologic study of twenty cases.

    Dubin-Johnson syndrome (DJS) is a rare benign chronic disorder of bilirubin metabolism, characterized by conjugated hyperbilirubinemia, darkly pigmented liver and presence of abnormal pigment in hepatic parenchymal cells. This is a retrospective study of twenty cases of DJS highlighting their major clinical and pathological findings. liver biopsies were available in all the cases, obtained during a fourteen-year period (January 1991 to March 2005). The patients' age ranged from 7-63 years (median 21 years). These twenty cases comprised 13 males and 7 females. Major clinical manifestations were recurrent or persistent jaundice, abdominal pain and fever. Duration of illness ranged from 9 months to 58 years (median 10 years). All of them had conjugated hyberbilirubinemia and total serum bilirubin levels ranged between 1.4-13 mg/dl (mean 4.4 mg/dl). liver biopsies revealed presence of coarse granular brown pigment in the cytoplasm of hepatocytes more concentrated in the pericanalicular region and more prominent in centrilobular hepatocytes. Associated findings were presence of hepatitis b virus related chronic hepatitis (1), history of tubercular lymphadenitis (1), chronic cholecystitis in (2), coronary heart disease (1) and exacerbation during pregnancy (1).
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8/12. Severe hyperbilirubinemia in glucose-6-phosphate dehydrogenase deficient patients during viral hepatitis.

    Nine G-6-PD subjects developed acute hemolysis and severe hyperbilirubinemia (up to 61.1 mg/dl) following viral hepatitis. All except one had fever at presentation. Neutrophilic leukocytosis was a common feature. Elevation of both alanine aminotransferase (SGPT) and extremely high level of aspartate aminotransferase (SGOT) were prominent. Three developed acute renal failure. All patients survived, one after peritoneal dialysis. Recognition of the clinical picture is essential to prevent serious complications and for successful management.
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9/12. Acute viral hepatitis, intravascular haemolysis, severe hyperbilirubinaemia and renal failure in glucose-6-phosphate dehydrogenase deficient patients.

    Five patients with acute viral hepatitis developed severe intrasvascular haemolysis and unusually high levels of serum bilirubin (427 to 1368 mumol/l). All 5 had high fever, marked anaemia, reticulocytosis and neutrophilic leucocytosis. Three of them developed acute renal failure, which was of non-oliguric type in 2. The clinical course was protracted, but complete recovery occurred in 4 patients between 4 to 10 weeks. One patient with hepatic coma and oliguric renal failure died. Deficiency of the enzyme G-6-PD was confirmed in 4 cases. Massive haemolysis in the patients was probably induced by the administration of chloroquine and other drugs. Intravascular haemolysis should be suspected in patients with acute viral hepatitis, if they show unexplained anaemia and very high serum bilirubin levels, and measures to prevent renal failure should be instituted in such cases.
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10/12. Infectious hepatitis and glucose-6-phosphate dehydrogenase deficiency.

    The occurrence of infectious hepatitis in patients with coexistent glucose-6-phosphate dehydrogenase (G6PD) deficiency may present a clinical picture similar to that of fulminant hepatitis. To determine the factors which enable a rapid diagnosis of this disease combination, the clinical and biochemical findings in 14 patients were compared with those in 50 patients with uncomplicated hepatitis and those in 14 patients with fulminant hepatitis. Similarities with the latter group included persistence of fever, tachycardia, leukocytosis and hyperbilirubinemia of greater than 340 mumol/liter (20 mg/dl). A rise in bilirubin values of more than 50 mumol/liter (3 mg/dl) per day in patients with viral hepatitis strongly suggested the presence of G6PD deficiency. Despite the severity of the illness, prothrombin activity was well maintained in G6PD-deficienct patients.
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