Objective The aim of this study was to re-evaluate the upper limit of normal range (ULN) for serum alanine aminotransferase (ALT) in chronic hepatitis C (CH-C) patients who achieved sustained virological response (SVR) to interferon therapy. Methods Enrolled in this study were 136 consecutive patients, 84 males and 52 females, mean age 52.1±14.8 years, with CH-C who received interferon therapy during 1992 to 2008 and achieved SVR. AST and ALT levels (3 serial measurements) were measured every 3 to 4 months over one year after termination of interferon therapy and then the measurements were averaged for each patient. Results The distribution of AST and ALT showed normal distribution. Overall, AST levels were19.7±3 IU/L and ALT levels were 13.8±3.1 IU/L in all patients, AST levels were 19.8±3 IU/L and 12.9±2.9 IU/L and ALT levels were 14.4±3.2 IU/L and 9.9±3.5 IU/L in male and female patients, respectively. AST level was the highest in the 6th decade and ALT level was in the 5th decade. Conclusion In this study on CH-C patients with SVR to interferon therapy, ULN of serum ALT and AST were far lower than the current accepted value. We propose that a suitable ULN of serum AST is <25 IU/L and ALT is <20 IU/L in CH-C patients.
Objective Autoimmune pancreatitis (AIP) is frequently associated with various extrapancreatic lesions. The distribution and frequency of extrapancreatic lesions preceding or subsequent to AIP are unknown. The aim of this study was to investigate metachronous extrapancreatic lesions of AIP. Patients and Methods Extrapancreatic lesions were examined clinically, radiologically, and histologically in 56 AIP patients. Results Extrapancreatic lesions were associated in 25 (45%) of 56 AIP patients. Twenty-nine extrapancreatic lesions were detected synchronously with AIP in 18 patients, and 18 lesions were detected metachronously in 11 AIP patients. Fourteen patients had more than 2 extrapancreatic lesions. There was no significant difference in serum IgG4 levels between AIP patients with preceding extrapancreatic lesions and synchronous extrapancreatic lesions. Extrapancreatic lesions preceding AIP were sclerosing sialadenitis (n=8), cervical lymphadenopathy (n=4), swelling of the lacrimal glands (n=2), retroperitoneal fibrosis (n=1), and hilar lymphadenopathy (n=1). Retrospective histopathological examination confirmed that these lesions were compatible with IgG4-related sclerosing disease. Steroid therapy was not given for these initial lesions, and AIP occurred 3 to 48 months after these initial lesions. Swelling of the preceding extrapancreatic lesions persisted when AIP occurred. Extrapancreatic lesions subsequent to AIP were retroperitoneal fibrosis (n=1) and systemic lymphadenopathy (n=1), both of which occurred during follow-up of AIP without steroid therapy. All extrapancreatic lesions improved after steroid therapy. Conclusion Swelling of salivary or lacrimal glands, lymphadenopathy, and retroperitoneal fibrosis can precede AIP. Lymphadenopathy and retroperitoneal fibrosis can occur subsequent to AIP. Recognition of these findings will aid in the correct diagnosis of AIP.
Objective With conventional MRI and single-photon emission computed tomography (SPECT), accurate diagnosis and precise classification of cerebellar atrophy are often difficult. The objective was to verify the utility of MRI voxel-based morphometry (VBM) in combination with SPECT using easy Z-score imaging (eZIS) for diagnosing and classifying cerebellar atrophy. Patients and Methods We assessed gray matter atrophy using VBM and blood perfusion using SPECT with eZIS in fifteen patients with different types of cerebellar atrophy, such as the cerebellar variant of multiple system atrophy (MSA-C), spinocerebellar ataxia type 3 (SCA3), SCA6, and autoimmune cerebellar ataxia (AICA). Results In all five MSA-C patients, VBM imaging showed atrophy of the brainstem, the entire cerebellar vermis, and the cerebellar hemispheres, while SPECT using eZIS showed reduced perfusion in the same regions. Regarding SCA3, brainstem atrophy and reduced perfusion were recognized in two of the four patients, but none exhibited abnormal findings in the posterior lobe of the cerebellar vermis. SPECT showed that all four patients had obviously reduced perfusion in the anterior lobe of the vermis, but VBM demonstrated that there was no obvious atrophy of gray matter in any patient, meaning that the results of SPECT and VBM contradicted each other completely. All SCA6 and AICA patients exhibited atrophy and reduced perfusion in the cerebellar hemispheres but not in the brainstem. Only one AICA patient exhibited atrophy and reduced perfusion of the entire cerebellar vermis. Conclusion VBM clearly showed characteristic gray matter atrophy in the cerebellum and brainstem in different pathological conditions, thus indicating its high degree of utility in diagnosing and classifying cerebellar atrophy in combination with SPECT using eZIS.
Backgrounds and propose Intravenous thrombolysis using tissue plasminogen activator (tPA) can improve patient outcomes in acute stroke if administered within 3 hours of onset. However, patients with aortic dissection should avoid tPA therapy due to the possibility of tPA administration inducing rupture of the aortic dissection. We studied the frequency and clinical characteristics of stroke patients presenting with aortic dissection within 3 hours of onset. Methods Among stroke patients admitted to our hospital within 3 hours of onset, we examined the frequency of patients presenting with aortic dissection. Next, we examined the clinical characteristics of such patients, including cases published on PubMed. Results Among 208 stroke patients presenting within 3 hours of onset, 2 patients (1%) displayed aortic dissection. Carotid duplex ultrasonography could exclude them from tPA therapy. For 19 patients, including 17 published cases from PubMed, median age was 61.0 years, 47% were women, right pulse weakness was seen in 70%, chest pain in 22%, and lone left hemiparesis in 72%. In 6 patients, carotid ultrasonography showed arterial dissection due to aortic dissection. Conclusion Aortic dissection may not be a rare complication in acute stroke within 3 hours of onset. Right pulse weakness and left hemiparesis are often present. Carotid ultrasonography may be useful in diagnosing aortic dissection before tPA infusion.
Background Several lines of evidence from studies involving both general and non-diabetic populations have shown that a family history of diabetes was associated with an increased risk for cardiovascular diseases and metabolic alterations. However, little is known about the relationship of a family history of diabetes to glycemic control and metabolic risks among people with diabetes. Methods We conducted a cross-section study of 946 diabetic adults from the National Health and Nutrition Examination Survey between 1999 and 2004. Familial risk of diabetes was classified as average, moderate, or high. Logistic regression analyses were conducted to determine the association between familial risk of diabetes and poor glycemic control, as defined by A1C ≥8%. According to stratified levels of familial risk of diabetes, adjusted means of various metabolic risks, including A1C, BMI, lipid profiles, and C-reactive protein, were obtained by using multiple linear regression. Results Independent of basic demographics, health-related behaviors, use of anti-diabetic medications, diabetes duration, cardiovascular co-morbidities, and various metabolic risks, the odds ratio of poor glycemic control comparing participants with a high familial risk of diabetes to those with an average risk was 1.91 (95% confidence interval 1.02-3.58). In the multivariate analysis, the adjusted means of A1C in participants with high, moderate, and averaged familial risk of diabetes were 7.75%, 7.45%, and 7.25%, respectively (p for trend 0.036). Participants with a high familial risk of diabetes also had higher triglycerides and body mass index (p for trend 0.042 and 0.02, respectively). Conclusion Diabetic adults with a higher familial risk of diabetes have a worse glycemic control, higher BMI, and higher triglycerides. Obtaining family history of the disease is crucial in identifying and targeting high risk diabetic patients who may require more stringent lifestyle changes as well as pharmaceutical intervention.
A 59-year-old man who had undergone left nephrectomy for renal cell carcinoma (RCC) 14 years previously was admitted for the treatment of obstructive jaundice. Imaging studies showed head-to-tail dilation of the main pancreatic duct (MPD) and a few ring-shaped enhanced nodules. Main duct-type intraductal papillary mucinous neoplasm was suspected and total pancreatectomy was performed. Pathologically, the entire length of MPD was filled with tumor. It consisted mainly of necrotic material, but included some clear cell carcinoma; the final diagnosis was metastatic RCC of the pancreas. This is an extremely rare case of pancreatic metastasis from RCC, with marked extension into MPD.
Congenital diseases causing an obstruction of the right ventricular outflow are difficult to precisely diagnose, especially in elderly patients. Here, we describe a 76-year-old woman who presented to our hospital with shortness of breath on exertion and was finally diagnosed as infundibular pulmonary stenosis by multiple modalities. Surgery was successfully performed and the symptom was relieved. This case underscored the usefulness of multiple modalities for the precise assessment of the right ventricular outflow obstruction and the effectiveness of surgery even in elderly patients with infundibular pulmonary stenosis.
This case report describes a 20-year-old immunocompetent man with an episode of chest pain radiating into both arms, an increase in the level of myocardial enzymes, electrocardiogram abnormalities (widespread ST-segment elevation and q waves in leads V4-V6) and serological evidence for acute Epstein-Barr Virus infection preceding typical signs and symptoms of infectious mononucleosis.
Endometriosis is a common disease, but ureteral involvement is rare. The symptoms and signs of ureteral endometriosis mimic those of ureteral malignancy. This case report describes a woman who presented with chronic back pain for 5 years. Imaging studies showed a right small contracted kidney with hydronephrosis and a bladder tumor. Endometriosis of the right lower ureter was ultimately diagnosed. The patient was healthy without recurrence during follow-up. It is difficult to differentiate between ureteral endometriosis and malignancy; in fact, renal loss may occur before diagnosis. Ureteral endometriosis should be considered for women with ureteral obstruction manifesting as chronic backache.
Anti-glomerular basement membrane (GBM) antibody disease may progress to end-stage renal failure, requiring either dialysis or renal transplantation. In patients with end-stage renal disease (ESRD) due to anti-GBM-ab disease, the recurrence rate after transplantation is as high as 50% (2) and delaying renal transplantation until circulating anti-GBM antibody levels have been undetectable for at least 12 months reduces the recurrence rate to 5-15%. We report a case of ESRD due to renal limited anti-GBM disease with circulating anti-GBM-ab resistant to standard treatment approach who achieved undetectable anti-GBM-ab with mycophenolic acid.
Primary hyperparathyroidism (PHPT) and multiple myeloma (MM) are frequently observed in the adult population and can each independently lead to hypercalcemia. Despite the frequency of hypercalcemia secondary to PHPT and MM, these two conditions only rarely concurrently present in patients. We describe the management of PHPT in the setting of poorly differentiated MM in a patient presenting with hypercalcemia and pancytopenia. The patient was deemed at increased risk for surgical removal of the parathyroid gland and refused surgical intervention, so we chronically managed her PHPT and hypercalcemia with Cinacalcet and bisphosphonates. All of the similar cases in the literature are reviewed in this report along with medical management of PHPT. We believe that we describe the first medically managed PHPT in the setting of MM.
The patient was a 38-year-old woman diagnosed with nephrotic syndrome. Steroid pulse therapy and mizoribine was started in late October 2002 and continued for about 10 months, but no apparent therapeutic effect was obtained. During this period, the patient was diagnosed with nasopharyngeal carcinoma. As nephrotic syndrome did not improve, renal biopsy was performed and membranous nephropathy (MN) was diagnosed. After resection of nasopharyngeal carcinoma was performed, the urinary protein level decreased rapidly. Since MN caused by nasopharyngeal carcinoma is very rare, this represents an interesting case of malignancy-associated MN.
Subcutaneous sarcoidosis (previously referred to as Darier-Roussy sarcoidosis) is an unusual and atypical form of sarcoidosis. It is characterized by formation of the noncaseating granulomas in the subcutaneous tissue with no other systemic manifestations present. Sometimes subcutaneous granulomas are the first sign of systemic sarcoidosis. We report a case of a 51-year-old woman with an isolated form of subcutaneous sarcoidosis with no apparent sign of any other systemic manifestations. The final diagnosis should be made by excluding all other more common diseases. As spontaneous remissions have been reported, regular follow-ups for the first three to six months are advisable. If the remission does not take place within the given period of time or the symptoms progress rapidly, glucocorticoids are the drugs of choice. After improvement is seen, slow tapering of glucocorticoids is advisable.
Cardiac failure is the leading cause of mortality in patients with thyroid storm. But the underlying cardiac pathology is unclear. Here, we report a 46-year-old woman who presented with hyperpyrexia and sinus tachycardia subsequent to accidental neck contusion. Her hyperthyroidism was verified by abnormal biochemical changes. Despite vigorous antithyroid treatment including a beta-blocker, glucocorticoid and potassium iodide, the patient eventually succumbed to refractory congestive heart failure in 4 days. Autopsy revealed lymphocytic myocarditis. We propose that lymphocytic myocarditis played a prominent role in her demise.
A 52-year-old man was admitted to our hospital in June 2008 presenting abnormal tumor lesions along the left pleura on chest X-ray. The needle-biopsied specimen of the left pleura proved the biphasic type of malignant mesothelioma. However, he complained of acute abdominal pain 7 days after the diagnosis. Chest X-ray revealed free air below the right diaphragm. Emergency surgery revealed a 4-cm perforating jejunal tumor with peritonitis. Histopathology of the resected jejunum demonstrated a metastatic tumor of malignant pleural mesothelioma. This is the first reported case of malignant pleural mesothelioma presenting as an acute surgical abdomen due to jejunal metastasis with perforation.
Genetic alterations of echinoderm microtubule-associated protein-like 4 (EML4)-anaplastic lymphoma kinase (ALK) inversion were recently found in lung cancer. A 39-year-old woman with multiple brain metastases and bulky mediastinal lymph node metastases was admitted. Biopsy from her supraclavicular lymph nodes was performed to differentiate the diagnosis between lymphoma and lung cancer. Pathologically, the lymph nodes had a feature of adenocarcinoma. On the other hand, the commercially available chromosomal fluorescent in situ hybridization (FISH) analysis showed split signals of ALK, which was confirmed to be the EML4-ALK inversion. The commercial-based ALK FISH is useful for screening pulmonary ALKoma.
Although the incidence of Hodgkin lymphoma (HL) - a lymphoid tissue malignity - increases in the presence of several viruses, particularly EBV, as well as with autoimmune diseases and following transplantation, although to date, the exact etiopathogenesis is not known. The higher frequency of HL among family members suggests involvement of genetic factors in its etiology. Studies aiming to elucidate the etiopathogenesis of patients with familial HL (FHL) have reported that human leukocyte antigen (HLA) haplotypes might be involved. In this case presentation, the associations between HLs diagnosed in a father of consanguineous marriage and his two children and HLAs in other family members were investigated and the findings are discussed in view of the published literature; no direct association was found between HLA alleles and the development of the disease in the present case with familial HL.
The association of myasthenia gravis (MG) and premature ovarian failure (POF) has rarely been recognized, and the influence of hormone replacement therapy on MG in patients with POF has not been reported. We describe a patient diagnosed with MG and POF, whose myasthenic symptoms were precipitated by estrogen treatment. Such combined clinical symptoms of MG and POF may reflect potentially common autoimmune disease mechanisms, although the precise pathogenesis remains to be defined.
Central pontine myelinolysis (CPM) and extrapontine myelinolysis (EPM) are osmotic demyelination syndrome. A 45-year-old man with a history of alcoholism visited the ER with dysarthria and dysphagia for 2 days. These symptoms occurred 3 days after he had stopped drinking alcohol. The neurological symptoms progressed to anarthria, pseudobulbar palsy and gait disturbance. During admission, the electrolyte studies were within the normal range. Diffusion-weighted images revealed high signal intensities in the pons, thalamus and basal ganglia. Apparent diffusion coefficient image showed low signal intensities in the pontine lesion, but isosignal intensities in the extrapontine lesion. The symptoms gradually improved after 1 month with only conservative treatment. The 1 month-follow-up MRI showed significant reduction of the previous extrapontine lesions. These findings suggest that cytotoxic edema is central to the pathogenesis of CPM, but vasogenic edema plays an important role in the pathogenesis of EPM occuring during alcohol withdrawal.
Familial Mediterranean fever (FMF) is an autoinflammatory disorder characterized by recurrent febrile polyserositis and arthritis attacks. Accompanying seronegative spondyloarthropathy has been reported in FMF in addition to its own joint involvement. However, the coexistence of FMF with juvenile idiopathic arthritis (JIA) is very rare, only three cases with severe joint involvement and mortal outcome have been reported in the literature. Here, we present another case with FMF and JIA with osteoporosis, successfully treated with etanercept with a four-year follow-up.
Community-acquired methicillin-resistant Staphylococcus aureus (CA-MRSA) continues to emerge as a cause of serious infections. We present the first case of abdominal mycotic aneurysm caused by Panton-Valentine leukocidin-positive CA-MRSA. This case adds another presentation to the clinical spectrum of CA-MRSA infections, and highlights the problem encountered in the choice of the therapy of serious community-acquired infections in an era of increasing CA-MRSA infections.