A case of acute hepatitis E associated with multidrug hypersensitivity and cytomegalovirus reactivation

A 65-year-old Japanese man was hospitalized because of acute hepatitis and severe cholestasis due to hepatitis E virus (HEV) infection combined with a drug reaction to a cold preparation. He died of disseminated intravascular coagulation and severe intestinal bleeding due to systemic cytomegalovirus reactivation following the development of severe eruptions with marked eosinophilia due to drug hypersensitivity to taurine and ursodeoxycholate preparations. The close interaction between viral infection or reactivation and drug hypersensitivity was considered as a pathophysiology in this case, which emphasizes the need for further study of the immunological mechanism of the interaction.     

Epidemiological considerations following long-term surveillance of invasive group A streptococcal disease in The Netherlands, 1992–2003

A nationwide laboratory-based surveillance system for invasive group A streptococcal (GAS) infections was conducted in The Netherlands from March 1992 until December 2003. Until 1996, all isolates submitted were evaluated clinically and demographically. During this period there was a transition from passive to active surveillance for some of the participating laboratories, corresponding to a national coverage of 50%. During active surveillance, participating laboratories submitted twice as many isolates from invasive GAS disease, whereas the relative submission of isolates representing very severe manifestations (toxic shock-like syndrome, fatality) did not increase. From 1997 onwards, invasiveness was defined solely on the basis of source of isolation (without clinical evaluation). During the period of microbiological and clinical evaluation, microbiological evaluation alone was found to be specific (> 99%), but had limited sensitivity (66%). Estimation of the true rate of invasive GAS disease should be based on an active surveillance system with inclusion of both microbiological and clinical data.     

Complete response, as determined by prostate-specific antigen level, to chlormadinone acetate withdrawal persisting longer than 2 years in patients with advanced prostate cancer: Two case reports

Antiandrogen withdrawal syndrome (AWS) is a well-established phenomenon in prostate cancer. However, responses to AWS are usually of limited duration, and a complete response (CR) is extremely rare. We present two patients who exhibited a chemical CR for more than 2 years after the discontinuation of steroidal antiandrogen chlormadinone acetate use. Whether patients who respond to antiandrogen withdrawal include a group of patients with a better prognosis remains uncertain. However, considering that the usual survival period of patients with hormone-resistant prostate cancer is approximately 12 months, both of the patients reported here, who are present in excellent physical condition, exhibiting an improved quality of life, and attending their hospital as outpatients, obviously acquired a prolonged survival because of AWS.     

腕管综合征96例分析

自1993年1月-2000年12月,诊治腕管综合征96例,其中手术治疗31例,现将有关资料进行分析。1临床资料1.1一般资料本组96例,男25例,女71例;年龄为26～63岁,平均43岁。16例为双侧性,病程最短为1周,最长为10年。病因:腕部外伤史17例,手部关节酸痛史23例,18例女性患者认为发病与妊娠或     

A novel mutation in the last exon of ATRX in a patient with α-thalassemia myelodysplastic syndrome

Abstract:  We describe a patient with acquired alpha-thalassemia myelodysplastic syndrome (ATMDS). A previously healthy 66-year-old man presented with hemoglobin of 9.3 g/dL, mean corpuscular volume 59 fL, and a bone marrow aspirate with increased erythroid precursors and hypolobulated megakaryocytes. Hemoglobin H inclusions were seen in most red cells after 1% brilliant cresyl blue supravital stain of the peripheral blood. At the molecular level, we identified of a novel mutation in the most 3' exon of the ATRX gene ( C GA→ T GA substitution in codon 2407) resulting in a premature termination codon (p.R2407X). This case provides further evidence for a link between ATRX mutations and ATMDS, and suggests a possible role for the conserved Q-box element in ATRX function.     

NASOGALLBLADDER DRAINAGE FOR MIRIZZI’S SYNDROME

The authors experienced a case of Mirizzi’s syndrome successfully treated with endoscopic nasogallbladder drainage (ENGBD). The patient was a 63‐year‐old man. He was admitted with abdominal pain and jaundice. Laboratory data indicated leukocytosis and elevation of serum bilirubin level. Abdominal ultrasound showed marked swelling of gallbladder and debris in the gallbladder, therefore, the authors strongly suspected Mirizzi’s syndrome. He had past history of acute myocardial infarction and treated with anticoagulation therapy. Then, the authors couldn’t perform surgical removal or percutaneous transhepatic drainage, and tried endoscopic transpapillary drainage. Endoscopic retrograde cholangiopancreatography revealed smooth stricture in the superior portion of common bile duct and occlusion of the cystic duct, and ENGBD was then performed. After ENGBD, his complaints, laboratory data, swelling of gallbladder and stricture of common bile duct were all remarkably improved.     

PEUTZ‐JEGHERS SYNDROME ASSOCIATED WITH RENAL AND GASTRIC CANCER THAT DEMONSTRATED AN STK11 MISSENSE MUTATION

A 75‐year‐old male was admitted to the gastroenterology unit of Nagoya City University Hospital due to epigastralgia after surgical treatment for right renal cancer. Endoscopy revealed advanced type 1 gastric cancer in the corpus of the stomach and multiple polypoid lesions in the stomach and duodenum. X‐ray examination of the small intestine using barium showed multiple polyps in the upper jejunum. Faint pigmentation on the palm was also detected. Peutz‐Jeghers syndrome (PJS) was diagnosed, despite a lack of family history. Total gastrectomy, resection of part of the upper jejunum and intraoperative endoscopic polypectomy of duodenal polyps was performed. This is the second reported case of PJS associated with renal cancer. We also detected a missense mutation in the tumor suppressor gene STK11 that, when mutated, is causative for PJS.     

Clinical characteristics and imaging manifestations of AIDS complicated with disseminated Penicillium marneffei infection

Objective To investigate the clinical characteristics and imaging manifestations of AIDS complicated with disseminated Penicillium marneffei （PM） infection. Methods A total of 12 patients with AIDS complicated with disseminated PM infection were collected and the symptoms, signs, laboratory examination results and image manifestations of these patients were analyzed retrospectively. Results （1） The diagnosis of PM infection in all the 12 cases were confirmed by peripheral blood culture. All the 12 cases （100%） had irregular fever （38-41℃） and enlarged lymph nodes, 8 cases （66%） had skin rashes; 8 cases （66%） had hepatomegaly; 9 cases （75%） had splenomegaly while 8 cases （66%） had anemia. （2） Imaging manifestation： Five cases manifested bilateral pulmonary disseminated miliary nodular shadows or lattice signs; 1 case showed enlarged hilar lymph node and 2 cases showed patchy shadow with pleuritis. One case presented sub-pleural curve line shadow at the posterior part of the right lower lung, and adhesion between the intestinal wall and intestinal mesentery in mass form in the abdomen by CT examination. Conclusion Patients suffering from AIDS （CD4 T lymphocytes 〈50/μ L） with impaired immunity might be susceptible to complication of disseminated PM infection, which presents mainly damage of multiple organs and symptoms such as fever; enlargement of liver, spleen and lymph nodes, as well as specific skin maculopapular rashes. Imaging manifestations in the lungs were revealed as miliary nodular shadows and lattice-like shadows. Intensified abdominal CT might reveal presence of several enlarged postperitoneal lymph nodes and intestinal adhesion in shape of ＂cakes＂.     

Time to REST: epidemiology and burden

Restless legs syndrome (RLS) is a neurological disorder characterized by a compelling urge to move, accompanied by disturbing sensations within the legs. It is now recognized that RLS is a common condition that has a substantial impact on sleep, daily activities and quality of life. Recent data from the REST (RLS Epidemiology, Symptoms, and Treatment) general population study show that symptoms of RLS are present in approximately 7% of the general population, and that 2–3% experience moderate or severe symptoms at least twice a week. Amongst this RLS cohort, approximately 88% reported sensory disturbances and 76% reported sleep-related symptoms. These symptoms had a marked effect on everyday life, with up to 50% of the RLS cohort reporting disruption of everyday activities or personal relationships. Sleep disturbances in turn lead to impaired daytime functioning. RLS is associated with a significant impairment of quality of life, comparable with that seen in chronic medical conditions such as diabetes or depression. Given the significant burden of morbidity associated with RLS, there is a strong case for treatment in patients with troublesome symptoms.     

Boerhaave’s syndrome: Primary repair vs. esophageal resection—Case reports and meta-analysis of the literature

Boerhaave’s syndrome is a life-threatening disease with a high mortality. With regard to the heterogeneity of treatment strategies, no comparative studies exist and recommendations remain controversial. Seventeen cases of Boerhaave’s syndrome operated on between 1989 and 2000 at our hospital were reviewed retrospectively to compare the time period between perforation and diagnosis, and the morbidity and mortality among the different treatment options. In addition, we conducted a meta-analysis of the literature including all series containing five or more patients and compared the findings with our own data. Our patients with a perforation history of less than 12 hours showed significantly fewer signs of sepsis compared to patients with a history of more than 12 hours. In a comparison of patients with primary repair vs. patients treated with esophageal resection or an exclusion operation, no differences were found. In the literature, patients with a long period of perforation (more than 24 hours) were treated more often with an esophageal resection than patients with primary repair. In cases of Boerhaave’s syndrome, primary suturing of the esophageal perforation should be reserved only for those patients presenting within 12 hours after perforation. In all other cases, depending on the extent of the tissue damage, a two-stage esophageal resection with cervical esophagostomy and gastrostomy is recommended as the safest treatment.