Prospective Evaluation of Quality of Life in Patients with Zollinger–Ellison Syndrome

Background  

Zollinger–Ellison syndrome (ZES) is associated with complicated ulcer disease of the upper gastrointestinal tract. While management of ZES has dramatically improved with proton pump inhibitor therapy, quality of life in medically treated patients has not been evaluated.     

A case of Hughes–Stovin syndrome associated with hyperhomocysteinemia

We report a case of Hughes–Stovin syndrome (HSS) associated with hyperhomocysteinemia. A 24-year-old man who has no clinical features suggestive of Behcet’s disease was admitted for hemoptysis and dyspnea. Radiological and laboratory evaluation revealed multifocal pulmonary artery aneurysms involving bilateral segmental pulmonary artery, thrombi in right atrium and ventricle, and hyperhomocysteinemia. Accordingly, HSS associated with hyperhomocysteinemia was diagnosed, and the clinical and radiological improvement was achieved after treatment with prednisolone, warfarin, and folic acid.     

Sulfasalazine-induced hypersensitivity syndrome and hemophagocytic syndrome associated with reactivation of Epstein–Barr virus

A 58-year-old woman with rheumatoid arthritis (RA) developed fever, skin eruptions, leukocytopenia, and thrombocytopenia, 3 weeks after treatment with sulfasalazine. A skin biopsy showed hydropic degeneration of keratinocytes and lymphocytic infiltrate. A bone marrow aspiration demonstrated an increased number of macrophages with hemophagocytosis. Although serologic tests for Epstein–Barr virus (EBV) indicated a previous infection, EBV deoxyribonucleic acid was detected in her serum by polymerase chain reaction. Cessation of sulfasalazine and administration of steroids led to dramatic improvement. This case illustrates that the hemophagocytic syndrome associated with reactivation of EBV can occur as part of drug hypersensitivity reactions in RA patients taking sulfasalazine.     

Eosinophilic myocarditis due to Churg–Strauss syndrome mimicking reversible dilated cardiomyopathy

A 41-year-old woman with a history of asthma arrived at the emergency room of our hospital with dyspnea. The electrocardiogram showed no specific results. Echocardiography defects revealed an obvious decrease in the left ventricular systolic function and enlargement of the left chamber. We initially considered her condition to be dilated cardiomyopathy. However, she had eosinophilia in the peripheral blood and elevated cardiac enzymes. The coronary angiography showed normal coronary arteries. Single photon emission computed tomography (SPECT) showed infiltrative myocardial disease. She was then diagnosed with eosinophil infiltrations. Combined with peripheral nerve injury and lung involvement, she was diagnosed as having Churg–Strauss syndrome. After initiating prednisone treatment, her eosinophilia and rising cardiac enzymes recovered to normal, and both her echocardiographic abnormalities and symptoms noticeably improved.     

A case of Weber–Christian disease associated with myelodysplastic syndrome

Abstract

We report the case of a 73-year-old man with myelodysplastic syndrome (MDS) who developed Weber–Christian disease (WCD). Bone marrow aspirates showed refractory anemia with abnormal karyotypes such as trisomy 8, trisomy 8 and 14, and trisomy 8, 9, and 14. The patient had intermittent fever associated with multiple tender erythematous nodules on the skin. A biopsy sample taken from a nodule revealed focal subcutaneous infiltration of neutrophils and necrotizing fat tissue. We diagnosed the patient as having lobular panniculitis associated with myelodysplastic syndrome. The serum levels of soluble interleukin-2 (IL-2) receptor, interferon-Γ, IL-1-β, IL-6 and tumor necrosis factor-α were elevated in the active state but returned to normal after prednisolone therapy. This finding appears to implicate a T-cell immune response in the pathogenesis of Weber–Christian disease.     

A case of Weber–Christian disease associated with myelodysplastic syndrome

We report the case of a 73-year-old man with myelodysplastic syndrome (MDS) who developed Weber–Christian disease (WCD). Bone marrow aspirates showed refractory anemia with abnormal karyotypes such as trisomy 8, trisomy 8 and 14, and trisomy 8, 9, and 14. The patient had intermittent fever associated with multiple tender erythematous nodules on the skin. A biopsy sample taken from a nodule revealed focal subcutaneous infiltration of neutrophils and necrotizing fat tissue. We diagnosed the patient as having lobular panniculitis associated with myelodysplastic syndrome. The serum levels of soluble interleukin-2 (IL-2) receptor, interferon-, IL-1-, IL-6 and tumor necrosis factor- were elevated in the active state but returned to normal after prednisolone therapy. This finding appears to implicate a T-cell immune response in the pathogenesis of Weber–Christian disease.     

Internal biliary fistula due to cholelithiasis： A single-centre experience

AIM: To discuss about the perioperative problems encountered in patients with internal biliary fistula (IBF) caused by cholelithiasis. METHODS: In our hospital, 4 130 cholecystectomies were carried out for symptomatic cholelithiasis from January 2000 to March 2004 and only 12 patients were diagnosed with IBF. The perioperative data of these 12 IBF patients were analyzed retrospectively. RESULTS: The incidence of IBF due to cholelithiasis was nearly 0.3%. The mean age was 57 years. Most of the patients presented with non-specific complaints. Only two patients were considered to have IBF when gallstone ileus was observed during the investigations. Nine patients underwent emergency laparotomy with a pre-operative diagnosis of acute abdomen. In the remaining three patients, elective laparoscopic cholecystectomy was converted to open surgery after identification of IBF. Ten patients had cholecystoduodenal fistula and two patients had cholecystocholedochal fistula. The mean hospital stay was 13 d. Two wound infections, three bile leakages and three mortalities were observed. CONCLUSION: Cholecystectomy has to be performed in early stage in the patients who were diagnosed as cholelithiasis to prevent the complications like IBF which is seen rarely. Suspicion of IBF should be kept in mind, especially in the case of difficult dissection during cholecystectomy and attention should be paid in order to prevent iatrogenic injuries.     

Opportunistic infection due to unexplained CD4+ lymphocytopenia and associated Sjögren's syndrome

SIR, Patients with primary Sjögren's syndrome (SS) usuallypresent with glandular (sicca) symptoms or systemic manifestations[1]. Immune-mediated cytopenias may occur but are unusual aspresenting manifestations [2]. We report a patient with seriousopportunistic infection due to severe CD4+ T lymphocytopenia,immune activation markers and a concurrent diagnosis of SS. A 62-yr-old woman developed progressive headache, nausea andlow-grade fever over 4 weeks. On admission, obtundation andBrudzinski's sign were the only notable findings. Chest radiography,ECG, electroencephalogram, head CT and laboratory tests werenormal,     

Guillain-Barré syndrome associated with scrub typhus

We report a 42-y-old female with Guillain-Barré syndrome (GBS) who presented with scrub typhus for a duration of 2 weeks. Subsequently, ascending paralysis and facial diplegia developed. GBS was confirmed with nerve conduction studies and cerebrospinal fluid examinations. After administration of intravenous immunoglobulin, symptoms gradually disappeared.     

Relationship of Plasma IL-6 to the Metabolic Measures associated with Insulin Resistance due to Adiposity

Objectives To elucidate the relationship of plasma interleukin-6 (IL-6) to the metabolic measures associated with insulin resistance (IR) due to adiposity. Methods For a cross-sectional study, eighty normotensive men with and without obe-sity were enrolled consecutively in our health examination center. Fasting blood glucose (FBG), fasting plasma immunoreactive insulin (FIRI), HOMA-R (Homeostasis Model Assessment Insulin Resistance Index), plasma lipids (cholesterol, triglyceride, highdensity lipoprotein cholesterol), cortisol, dehydroepiandrosterone-sulfate (DHEA-S), interleukin-6 and C-reactive protein(CRP) were measured. Results Plasma levels of FIRI, triglyceride fiG), DHEA-S, CRP and HOMA-R were significantly higher inobese group with BMI over 25 than non-obese group, whereas HDL-C was significantly lower in obese group. BMI was positively correlated with FIRI, TG, hsCRP and HOMA-R, whereas negatively with HDL-C. BMI was positively correlated with plasma DHEA-S levels but not with cortisol. Plasma levels of IL-6 were positively correlated with FIRI, TG, CRP and HOMA-R but in a multiple regression analysis with IL-6, only HOMA-R and TG remained explainable variables. Conclusions Each of commonly used mea-sures of inflammatory reaction, CRP and IL-6, showed a significantly positive correlation with either FIRI or HOMA-R, suggesting associations between subclinical inflammation and obesity as the risk of type 2 diabetes mellitus.     

Diabetes ketoacidosis associated with Guillan-Barré syndrome

Guillain-Barré syndrome (GBS) is a disorder caused by exaggerated immune response to infectious process. Diabetes Melito (DM) is not recognized as one cause of this inflammatory polyradiculoneuropathy with just a few cases of this association been described in the literature so far. We report here the case of a 44 years-old female patient admitted with a history of polyuria, polydipsia, weight loss, asthenia, hyperglycemia (562 mg/dL) and ketoacidosis without any infectious focus. The patient progressed with poliradiculopathy, respiratory insufficiency and liquoric alteration completing the picture of Guillain-Barré syndrome. The patient fully recovered from the neurologic deficit and then stopped with insulin therapy.     

Churg–Strauss syndrome associated with leukotriene receptor antagonists (LTRA)

Churg–Strauss syndrome (CSS) is a rare vasculitic disorder that generally occurs in patients with bronchial asthma. CSS is being increasingly recognized in asthmatic patients treated with leukotriene receptor antagonists. However, the nature of this relationship remains to be elucidated. The present report describes three asthmatic patients who developed clinical manifestations highly suggestive of CSS, although one patient lacked the presence of eosinophilia. The patient, however, exhibited biopsy-proven cutaneous necrotizing vasculitis, which improved after withdrawal of montelukast. The second patient presented with systemic constitutional signs including fever, malaise, arthralgias, clinical jaundice, peripheral blood eosinophilia, and biopsy-proven eosinophilic hepatitis. The third patient also had circulating eosinophilia, scleritis, and arthritis. All patients improved after discontinuation of the leukotriene receptor antagonist (montelukast).