Irritable bowel syndrome (IBS) is one of the most common functional gastrointestinal disorders. The prevalence rate is 10-20% and women have a higher prevalence. IBS adversely affects quality of life and is associated with health care use and costs. IBS comprises a group of functional bowel disorders in which abdominal discomfort or pain is associated with defecation or a change in bowel habit, and with features of disordered defecation. The consensus definition and criteria for IBS have been formalized in the “Rome II criteria”. Food, psychiatric disorders, and gastroenteritis are risk factors for developing IBS. The mechanism in IBS involves biopsychosocial disorders; psychosocial factors, altered motility, and heightened sensory function. Brain-gut interaction is the most important in understanding the pathophysiology of IBS. Effective management requires an effective physician-patient relationship. Dietary treatment, lifestyle therapy, behavioral therapy, and pharmacologic therapy play a major role in treating IBS. Calcium polycarbophil can benefit IBS patients with constipation or alternating diarrhea and constipation.
Since the first clinical use of implantable defibrillator in human, the technology and the function of implantable cardioverter-defibrillator (ICD) have been much improved and now, ICD can be implanted within the chest wall. ICD is the most reliable therapy to prevent sudden cardiac death (SCD) in patients with documented VT/VF and the efficacy is most clear in patients with depressed heart function. It is now extended as a tool of the primary prevention of SCD in high risk patients after myocardial infarction. However, such beneficial effect is not applicable to DCM though patients might have depressed heart function. ICD is not free from procedure- or device-related problems which need to be resolved. From unknown causes, VT/VF might recur in an incessant form and an emergency admission is needed. Therefore, even during ICD therapy, patients often require antiarrhythmic drugs or catheter ablation.
Coregulators are a group of proteins, which modulate the nuclear receptor transactivation function. In this study, a new “coregulator disease” concept was proposed from observations of a case of androgen insensitivity syndrome (AIS) and cases involving Rubinstein-Taybi syndrome and X-linked dementia and hypothyroidism syndrome. In addition, coregulators are thought to be closely associated with the pathogenesis of several diseases such as hormone-dependent cancers and leukemia. Based on these observations, the clinical disorders associated with some coregulator abnormalities were reviewed.
Objective We investigated the relationship between the right cardiac system and increased pulmonary artery systolic pressure (PASP) in the elderly. Patients and Methods Echocardiography stable state data were available for 163 of 200 consecutive autopsied patients. Of these, PASP could be estimated by extrapolation from the maximum pressure gradient in tricuspid valve regurgitation from echocardiograms in 73 cases; however, 22 cases with secondary changes attributable to left cardiac insufficiency had to be excluded. We studied the remaining 51 patients in detail (16 men, 35 women, age 68-103 years; mean, 87.7±8.1). We investigated the following: echocardiographic and pathologic variables, age, sex, body mass index, the survival time (from echocardiography to autopsy), and the presence or absence of chronic pulmonary disease. Results The average PASP was 39.8±10.3 mmHg, elevated compared with young persons. Linear regression analysis showed a close correlation of PASP with age (r=0.35, p=0.011), thickness of the right ventricle (RV) outflow tract wall as an index of RV hypertrophy (r=0.35, p=0.013) and the survival time (r=−0.36, p=0.0083). By multiple regression analysis, PASP was correlated with the thickness of RV outflow tract (p=0.0037) even after adjustment for other factors including chronic pulmonary disease. Conclusions PASP is elevated in the elderly and it is correlated with the thickness of the RV outflow tract wall as an index of RV hypertrophy.
Objective The aim of this study was to investigate the relation of QT dispersion to left ventricular (LV) systolic and diastolic function in patients undergoing anthracycline therapy. Methods We used echocardiography to evaluate LV systolic and diastolic function and electrocardiography to evaluate QT dispersion and corrected QT dispersion (QTcD) in patients with hematological diseases, who received anthracycline therapy. Patients Seventy-two patients with hematological diseases who were receiving anthracycline treatment were enrolled in the present study. Results LV end-diastolic diameter or LV end-systolic diameter had a significant positive correlation to QTcD (r=0.35, p<0.01, r=0.43, p<0.01). Also left ventricular ejection fraction of (LVEF) or fractional shortening had a significant negative correlation to QTcD (r=−0.46, p<0.001, r=−0.27, p=0.02). The highest QTcD group had a significantly larger LV end-diastolic diameter or LV end-systolic diameter than the lowest QTcD [48.5±5.7 vs. 44.4±4.5 (mm), p<0.001, 34.1±6.4 vs. 28.8±4.3 (mm), p<0.001] and the highest QTcD group had a significantly lower LVEF than the lowest QTcD [57.5±8.0 vs. 65.5±6.4 (%), p<0.001]. On the other hand, none of the diastolic function markers were significantly correlated with QTcD. Conclusion We concluded that increased QTcD is correlated with LV dilation and systolic dysfunction induced by anthracycline therapy, and does not reflect a dispersion of ventricular repolarization or asynchronous motion.
Objective Paragonimiasis is a typical food-borne parasitic disease mainly endemic in Southeast Asia. In Japan, the disease has been re-emerging since the 1980s. In addition, recently we encountered an increasing number of immigrants with paragonimiasis in Japan. In this study we summarized the clinical features of immigrants. Patients and Methods Among a total of 152 paragonimiasis cases referred to and diagnosed in our laboratory during 1998 to 2002, 18 were immigrants. Their clinical features including laboratory data such as eosinophilia and total IgE level were gathered from the consultation sheets from attending physicians. Results Among a total of 18 immigrant cases, 16 were from China and 2 from Thailand. A majority of immigrants had eaten freshwater crabs. Most of the Chinese patients were infected as small groups of family and/or compatriots. Chest radiographic findings were variable and multiple lung lesions were seen in about one-half of the patients. About 80% of patients had peripheral blood eosinophilia and 65% had elevated serum IgE level. Conclusion The clinical features of paragonimiasis in immigrants in Japan were much more severe compared to those of Japanese patients.
A 47-year-old woman was admitted to our hospital because of upper abdominal and back pain. Abdominal ultrasonogram, computed tomogram, endoscopic retrograde cholangiopancreatography and arteriography examination did not reveal any abnormalities. As cholescintigraphy after lipid meal loading detected dysfunction of the gallbladder, we diagnosed dyskinesia of the gallbladder. And the output ratio of the gallbladder from scintigraphy was less than 1%. Cholecystectomy completely relived her from symptoms. Histological examination disclosed chronic cholecystitis and arteritis causing dysfunction of the gallbladder. This case suggested the usefulness of cholescintigraphy with lipid meal loading for gallbladder dysfunction in determining whether or not to do cholecystectomy.
Protein-losing gastroenteropathy (PLG) can occur as a manifestation of various diseases including autoimmune disorders, and optimal therapy of these underlying diseases may be the only effective remedy for PLG. In the present report, we describe a case of a 54-year-old woman with PLG associated with an autoimmune disease, presumably CREST syndrome. She failed to respond to steroid treatment. Subsequently, cyclosporine was initiated, which resulted in a rapid recovery. The patient was successfully treated with low-dose cyclosporine for five years. There has not been, to our knowledge, any report of PLG successfully treated with cyclosporine. Cyclosporine therapy may be effective not only in inducing but also in maintaining complete remission in patients with autoimmune-associated PLG, especially refractory or intolerable to steroids and/or immunosuppressive therapies.
A 19-year-old man with severe active ulcerative colitis was admitted to our hospital where he was prescribed 80 mg prednisolone and underwent leukocytapheresis (LCAP). Two weeks after initiating therapy, his symptoms had not recovered. We administered cyclosporin via continuous intravenous infusion for 12 days. Although his clinical symptoms improved, he complained of severe headache. Immediate plain computed tomography (CT) and magnetic resonance imaging angiography (MRA) revealed extensive thrombosis in the superior sagittal sinus and right transverse sinus. We treated this condition with the anticoagulant agent, heparin, which prevents and can treat venous thrombosis. We report an occurrence of cerebral sinus thrombosis accompanying ulcerative colitis, where active anticoagulant therapy was useful.
We report a case of self-limited colitis in cytomegalovirus (CMV) infection in an immunocompetent adult. A 22-year-old man developed a high fever and diarrhea. Laboratory data revealed an increased number of lymphocytes and liver damage. Enzyme immunoassays for anti-virus antibodies revealed that the patient was recently infected with CMV and rubella. Colonoscopy revealed severe erosive and edematous mucosa that resembled ulcerative colitis (UC). The symptoms, laboratory data and colonoscopic findings improved without any medical treatment. This case indicates that UC-like self-limited colitis can occur in an immunocompetent individual during the course of CMV infection.
A 69-year-old woman was referred to our department for evaluation of hypokalemia, which had been treated by oral potassium for more than ten years. She complained of headache, knee joint pain, sleeplessness and paresthesia in extremities and, most prominently, depression. Laboratory data suggested Gitelman’s syndrome, which is caused by mutations in the gene encoding the thiazide-sensitive Na-Cl cotransporter. Direct sequencing of the gene in this patient revealed homozygous mutation R964Q in exon 25. Intravenous supplement of MgSO4 dramatically improved both the depression and the paresthesia, suggesting that hypomagnesemia played a role in the clinical manifestations.
We report a woman with ascites, hydrothorax, pancreatic tumor, left cystic ovarian tumor, and an elevated serum cancer antigen 125 level. Exploratory laparotomy was performed to determine peritoneal disseminated carcinoma of unknown origin. Immunohistochemical analysis demonstrated positive staining for carcinoembryonic antigen, trypsin, and progesterone receptor and nonspecific or negative reaction for calretinin, estrogen receptor, amylase, lipase, Wilms tumor gene 1 protein, and inhibin or chromogranin A. These results together with the morphology of tubular structure suggested the pathological diagnosis of adenocarcinoma with pancreatic characteristics and contradicted ovarian cancer or mesothelioma. Immunohistochemistry is an adjunct tool to differentiate the primary site of carcinomatous peritonitis.
We describe a patient wih subacute cor pulmonale caused by tumor emboli in the lungs. A 64-year-old female suffering from a subacute progressive cough and shortness of breathing died of severe pulmonary hypertension seven days after admission. Neither chest CT scans nor lung perfusion scintigraphy showed any abnormal findings. Microscopic examination after an autopsy revealed diffuse intravascular tumor emboli occluding not only the small pulmonary arteries and arterioles, but also the lymphatic vessels, which were suggested to be metastases of a breast carcinoma resected five years previously. Thus, pulmonary tumor embolism should be considered in the differential diagnosis of primary pulmonary hypertension, particularly in patients with a past history of cancers.
A 29-year-old man was admitted to our hospital because of fever elevation, dry cough, malaise, skin eruption, and dyspnea with hypoxemia. His serum levels of surfactant protein (SP) -A and SP-D were markedly high, but serum KL-6 was not. He was diagnosed as acute eosinophilic pneumonia (AEP) on the basis of CT imaging, bronchoalveolar lavage findings and the clinical course. He showed good response to steroid therapy and serum levels of SP-A and SP-D returned to almost normal levels. Our experience suggested that serum SP-A and SP-D might be helpful markers for monitoring the clinical course in AEP.
We present a case report of a 27-year-old man with MELAS, who presented with general convulsions and left flaccid hemiparesis. Anticonvulsant drugs failed to achieve complete control of his convulsions. A good response to oral administration of succinate has been maintained for more than 30 months, with no recurrence of any stroke-like episode. Succinate therapy may have potential for treatment of uncontrolled convulsive MELAS patients.
A 53-year-old Japanese woman presented with mild mental retardation, short stature, hypertelorism, saddle nose, vertebral fusion, and hydrocephalus, implying an underlying bone growth impairment mainly of the head and neck. A point mutation in fibroblast growth factor receptor 2 (FGFR2) was identified that had previously been seen only in sporadic cases of Crouzon syndrome. This patient did not exhibit any of the typical features of Crouzon syndrome primarily seen in affected infants, such as a severely deformed skull, an apical shaped skull, or severe mental retardation. The patient was diagnosed with a mild form of Crouzon syndrome. The patient’s symptoms very early in life may have been ameliorated and modified through growth and aging. The age-related phenotype modifications in Crouzon syndrome are discussed.
During the course of typhoid fever, the usual histologic finding of the liver is “nonspecific reactive hepatitis.” Hepatic granuloma (HG) is a rare complication of typhoid fever. We present two cases of typhoid fever with HG and review the relevant literature. Case 1 (a 53-year-old female) was found to have both hepatic and splenic granulomas. This is the first case of typhoid fever with splenic granulomas in the English language literature. Case 2 (a 66-year-old male) developed granulomas in the bone marrow in addition to HG. It should be considered that typhoid fever may lead to granulomas in several organs.
Patients with anorexia nervosa (AN) seldom present with infectious illness, despite malnutrition-induced immunodeficiency. We described two young women who had a long-standing history of severe emaciation and pulmonary or lymph node tuberculosis discovered during the treatment of AN. Both patients reported a positive history of BCG vaccination. Contact tracing failed to reveal sources of infection, although the tuberculosis was considered transferred. Since the decline of notification rates for tuberculosis have been stagnant and outbreaks in schools or hospitals have been increasing in Japan, special attention must be given to the possibility of opportunistic infections in AN patients.