Objective Aspartate aminotransferase-to-platelet ratio index (APRI), aspartate aminotransferase-to-alanine aminotransferase (AST/ALT) ratio, platelet count, AST, albumin, bilirubin and alkaline phosphatase were retrospectively evaluated for the prediction of advanced liver fibrosis and cirrhosis in patients with resectable hepatocellular carcinoma in this study. Patients In total, the 97 selected patients consisted of 9 (9.3%) patients with non-B, non-C chronic hepatitis, 48 (49.5%) patients with chronic hepatitis B (CHB) and 40 (41.2%) patients with chronic hepatitis C (CHC). Results The APRI, but not AST/ALT or other serum markers, showed a significant correlation with advanced liver fibrosis and cirrhosis (p<0.05). The area under receiver operating characteristic curves (AUROC) for predicting advanced fibrosis was 0.69 in CHB patients and 0.87 in CHC patients, whereas AUROC for predicting cirrhosis was 0.75 in CHB patients and 0.84 in CHC patients. In addition, the sensitivity and specificity of APRI were greater than 80% for predicting advanced fibrosis and cirrhosis in the CHC patients. Conclusion APRI is a simple and non-invasive biochemical marker of liver fibrosis and cirrhosis, particularly in CHC patients. APRI potentially could be used to decrease the number of liver biopsies.
Objective Isolated left ventricular noncompaction (ILVNC) is a rare congenital cardiomyopathy characterized by numerous excessive trabeculations and deep intertrabecular recesses. To date, the clinical features and genetic causes of ILVNC remain unclear. Here, we report the clinical presentation and genetic analysis of a five generation Chinese family with ILVNC. Methods For this study, 21 living family members were recruited. Each individual underwent a detailed clinical examination for ILVNC. Peripheral blood samples were collected for direct gene sequencing to determine any mutations in the known disease-causing genes of ILVNC, which include the genes TAZ, DTNA, LDB3, LMNA and FKBP12. Results Classic echocardiographic presentation of ILVNC was identified in the proband who had his first onset of heart failure at age 52. His 28-year-old son and 26-year-old daughter showed similar heart anomalies as their father. Although they had no symptoms to date, depressed ventricular systolic function was noted in both of them. Pedigree analysis suggested an autosomal domain mode of inheritance. DNA sequencing found no mutation in the known disease-causing genes of ILVNC. Interestingly, two other members of the family, the proband's wife (also his first cousin) and her sister had classic echocardiographic presentation of hypertrophic cardiomyopathy (HCM). Conclusion A single Chinese family with ILVNC associated with HCM is reported; no mutations in TAZ, DTNA, LDB3, LMNA and FKBP12 was found.
Objective Low-dose, long-term macrolide therapy has been shown to be effective for the treatment of diffuse panbronchiolitis (DPB) and similar disorders in terms of the presence of airway mucus hypersecretion such as bronchiectasis, chronic bronchitis and sinobronchial syndrome. However, there are some patients, especially advanced cases, whose volume of sputum does not decrease sufficiently with macrolide therapy. These patients suffer from copious expectoration. There is currently no effective treatment, and an effective therapy is therefore urgently required. The aim of this study was to clarify whether or not the inhalation of tiotropium improves the symptoms in these cases. Methods Tiotropium (18 μg/day) was administered to patients with DPB and similar disorders with airway mucus hypersecretion who did not respond to macrolide. The symptoms were evaluated by a visual analog scale (VAS) prior to and at 1 and 3 months after tiotropium administration. Radiological and pulmonary function tests were also performed to evaluate the effects of tiotropium. Results Thirteen patients (DPB 5, sinobronchial syndrome 5, bronchiectasis 3) were enrolled. The VAS scores were dramatically improved after the introduction of tiotropium. FEV1 was significantly improved after 3 months of treatment with tiotropium. In contrast, the radiological findings remained unchanged. Conclusion Tiotropium improved the symptoms of cough, sputum and breathlessness in the macrolide-resistant cases of DPB or similar disorders. These beneficial effects might be due to the suppression of airway secretion through the anticholinergic effect of tiotropium on the submucosal gland, however, the long-term efficiency of this treatment still needs to be further assessed.
Objective Chronic kidney disease (CKD) is a major public health problem, but there is controversy over whether or not CKD is an independent risk factor for peripheral arterial stiffness in community residents. Patients and Methods We randomly recruited 107 men, aged 68±9 (mean ± standard deviation) years, and 203 women, aged 67±7 years during their annual health examination in a single community. Study subjects did not have a clinical history of cerebrovascular disease or current neurological abnormalities. Peripheral arterial stiffness was evaluated by mean pulse wave velocity (PWV) determined at three points: from heart to the carotid artery, to the brachial artery, and to the ankle, and CKD was evaluated by estimated glomerular filtration rate (eGFR) using the Modification of Diet in Renal Disease Study Group equation. Results Estimated GFR was significantly correlated with mean PWV (r=-0.317, p<0.001). Stepwise multiple linear regression analysis using mean PWV as an objective variable, adjusted by explanatory variables, showed that eGFR (β,-0.171; p<0.001) significantly contributed to mean PWV, along with age, body mass index, systolic blood pressure, diastolic blood pressure, and antihypertensive drug use, and improved multiple coefficient of determination in the model. Conclusion Decreased eGFR is associated with an increased risk of arterial stiffness in community residents.
Benign recurrent intrahepatic cholestasis (BRIC) is an autosomal recessive disorder characterized by bouts of cholestasis that resolve spontaneously without leaving considerable liver damage. Most of BRIC patients have mutations in ATP8B1 gene coding FIC1 protein. It has been suggested that an imbalance between the gut absorption of bile acids and the liver excretion possibly causes the development of cholestasis. We encountered a Japanese woman patient with familial intrahepatic cholestasis type 1 (FIC1) deficiency manifesting BRIC, in whom a rapid and gross elevation of serum total bile acid (TBA) level preceded that of serum total bilirubin level. Interestingly, the early administration of colestimide prevented the development of hyperbilirubinemia along with the additional elevation of serum TBA level. This case suggests that FIC1 deficiency causes an imbalance between the gut absorption of bile acids and the liver excretion leading to cholestasis, and raised the possibility that colestimide may be used as an optional treatment for BRIC.
Autoimmune pancreatitis (AIP) is a new category of pancreatic diseases. AIP associated with pseudocysts is rare; only 8 cases have been reported in the literature. A 63-year-old man was admitted to our department because of upper left abdominal pain and back pain. Various imaging studies demonstrated swelling of the tail of the pancreas with hemorrhagic pseudocysts. The patient underwent a surgical operation. A pancreatogram of the specimen revealed total occlusion of the main pancreatic duct in the tail of the pancreas. Histopathological examination revealed that it was AIP with hemorrhagic pseudocysts.
While ventricular premature contractions have been noted during colonoscopy (CS), ventricular fibrillation (VF) is rare. We recently had a patient who developed VF during CS and recovered without any sequelae despite severe complications after cardiopulmonary resuscitation (CPR). If electrocardiogram (ECG) monitoring had been done during CS, a direct current shock defibrillation could have been accomplished and prevented complications. CS in high-risk patients should be done with ECG monitoring.
A 55-year-old hemiplegic woman with percutaneous endoscopic gastrostomy (PEG) was referred to our clinic for upper gastrointestinal system bleeding and for a high level of cholestatic enzymes. She had a medical history of cerebra vascular accident three years previously and cholecystectomy one year previously. We performed gastroscopy and saw a retained surgical sponge in the bulbus. After removal of the gossypiboma, endoscopic sclerotherapy was performed for the bleeding area at the bulbus. After the procedure, the upper gastrointestinal bleeding stopped and the high level of cholestatic enzymes returned to normal.
Primary small bowel bezoars are rare and may cause acute abdomen due to small bowel obstruction (SBO). A 70-year-old Japanese woman presented to the emergency room with abdominal pain, nausea and vomiting. The patient reported that she had eaten a large amount of highly-concentrated, agar dissolved in boiling water two days prior to presentation. Double balloon enteroscopy (DBE) revealed that white-colored, hard bezoars were clogged in the jejunum. At surgery, many bezoars were found impacted in the distal jejunum, and enterotomy was performed. The bezoars were elastic hard, crystallized objects. These bezoars were considered to have formed from highly-concentrated, dissolvable agar.
Small cell carcinoma of the bile duct system is extremely rare, and surgical procedures have been complicated by early hematogenous dissemination. In this study, we report a patient with small cell carcinoma of the bile duct system presenting with jaundice. The diagnosis was made early by endoscopic ultrasonography-guided fine needle aspiration biopsy (EUS-FNA). We performed radiation therapy of a total of 30 Gy, resulting in a marked decrease of serum neuron specific enolase levels and amelioration of jaundice, which had been resistant to drainage procedures. The patient was then treated with combined chemotherapy of cisplatin and CPT-11, which resulted in the disappearance of the tumor mass by image tests.
Acute viral infections can lead to heart inflammation, including acute myocarditis. We report the first case of myopericarditis in a young immunocompetent adult, in the context of recent Epstein-Barr virus infection. Clinical presentation was common acute pericarditis, but with risk biomarkers: high troponin I levels and multiple inflammation-compatible images on MRI. Diagnosis of myopericarditis was established, and then hospitalization was necessary. Clinical electrocardiographic settings and MRI are shown; EBV cardiac effects are discussed.
A 75-year-old woman had a low circulating level of aldosterone, despite the mineralocorticoid excess state. These abnormalities were improved by spironolactone administration. The distinct elevation of urinary cortisol/cortisone ratio revealed 11β-hydroxysteroid dehydrogenase type 2 (11β-HSD2) impairment. Moreover, slight but distinct elevation of the ratio was found in a 95-year-old woman with normotension and normopotassemia. The mineralocorticoid excess state with reduced aldosterone level appeared following with vomiting and diarrhea, exaggerating asymptomatic impairment of 11β-HSD2 to induce apparent mineralocorticoid excess (AME)-like condition.
A 25-year-old man with severe lumbago was referred to our department for further evaluation. Serum phosphate and TmP/GFR levels were decreased. Physical examination revealed an elastic tumor in the instep of the right foot, which the patient reported having since the age of 10 years. He had no symptoms of osteomalacia at that time. Within the recent years, the tumor had grown in size and the patient developed lumbago. To examine the existence of a fibroblast growth factor-23 (FGF-23)-producing tumor, venous blood was collected from four main veins. FGF-23 levels were significantly increased in the right femoral vein, compared with other veins. After the resection of the tumor, the histopathology was consistent with a phosphaturic mesenchymal tumor (mixed connective tissue variant). Taken together, these results indicated that the development of osteomalacia in this patient was associated with the production of FGF-23 in the subcutaneous tumor.
Fleet enema (sodium phosphate, C.B. Fleet Co., Inc., Lynchburg, Virginia) is widely used for bowel preparation or constipation relief in the hospital and over the counter. The potential risks, including hyperphosphatemia and hypocalcemic coma should be kept in mind of primary care physician. The patients with older age, bowel obstruction, small intestinal disorders, poor gut motility, and renal disease are contraindicated or should be administered with caution. We present a patient with old age and chronic renal failure who developed severe hyperphosphatemia and hypocalcemic tetany with coma after sodium phosphate enema. We recommend the use of alternative enema preparations, such as simple tap water or saline solution enemas, which can prevent fatal complications in high risk patients.
A 68-year-old woman, who had been diagnosed as idiopathic interstitial pneumonia, complained of progressive dyspnea on exertion for a week. Although her chest radiograph did not worsen, arterial blood gas findings were markedly worsened. Contrast-enhanced chest computed tomography showed filling defects of the right upper and middle lobe branches of the pulmonary artery. She was diagnosed as having acute pulmonary thromboembolism (APTE). Clinical symptoms and contrast-enhanced chest computed tomography findings were remarkably improved after the treatment with heparin and urokinase. APTE should be considered as a differential diagnosis in patients with interstitial pneumonia who have worsening of respiratory symptoms with unchanged chest radiograph.
To prevent fungal infections in patients undergoing treatment for hematological malignancies, we investigated the use of oral itraconazole solution as opposed to itraconazole or fluconazole capsules. Herein, we report five lymphoma patients with severe vincristine neurotoxicity in strong association with oral itraconazole solution. Four patients suffered from severe myalgia with or without arthralgia which clinically resembled polymyalgia rheumatica. Two patients suffered from constipation due to subileus and one patient had a severe paralytic ileus. Appropriate management of the above symptoms, which included discontinuation of oral itraconazole solution, resulted in rapid recovery from neurotoxicity. Given the more consistent plasma concentrations of oral itraconazole solution when compared to itraconazole capsules and the ability of itraconazole to interfere with hepatic vincristine metabolism, we strongly recommend avoiding the combined administration of oral itraconazole solution and vincristine.
A 55-year-old man suddenly developed neck pain, tetraplegia and decreased sensation below the neck. He was diagnosed with SSEH. Surgical removal of the hematoma, and laminoplasty were performed. At 2 months after the onset of the disease, the patient regained the ability to walk. However, at 5 months after the onset of the disease, the patient remained in a state of urinary retention even though his neurological findings were normal, except for mildly brisk reflexes in the lower extremities and decreased superficial sensation below the level of T4 including the perineal area. A urodynamic study showed normal bladder sensation, despite an acontractile detrusor and an unrelaxing external sphincter upon voiding. It is postulated that the descending micturition pathways (just inside the pyramidal tracts) were selectively affected, while the ascending micturition pathways (the dorsal columns) were preserved in the present case.
A 65-year-old woman with a sore throat and cough suddenly collapsed. She regained spontaneous circulation following resuscitation, but hypoxic encephalopathy was identified. Her vocal cords and the results of chest radiography were normal and no obstructive mass was identified in the neck on computed tomography (CT), but she demonstrated signs of obstructive upper airway. Bronchoscopy revealed tracheal stenosis. Chest CT showed tracheal compression by an esophageal tumor. Investigation of the trachea and surrounding organs by bronchoscopy and CT is important even when a patient with suspected respiratory arrest displays normal findings on chest radiography.
A 30-year-old Japanese man developed dactylitis with sausage-like fingers in addition to balanitis and stomatitis. One year prior to these symptoms, acute chlamydial urethritis had been successfully treated by levofloxacin. On admission, Chlamydia trachomatis DNA was not detected in the urine sediment by PCR method, however, he was diagnosed to have reactive arthritis based on the clinical findings of asymmetric dactylitis, circinate balanitis, stomatitis and positivity for HLA B27 antigen. He was treated with methotrexate and his arthritis improved. The past chlamydial infection may have been involved in the pathogenesis of reactive arthritis in this patient.
A 68-year-old woman was admitted to a hospital with pulmonary hypertension (PH) in August 2006. Perfusion scintigraphy of the lung was normal and showed no interstitial change. Liver dysfunction was noted and antinuclear antibodies (×1,280) were positive. In November 2006, muscle pain and weakness gradually developed in the brachial muscle and a quadriceps. She was referred and admitted to our hospital for elevated CPK and liver dysfunction in March 2007. She was diagnosed with polymyositis (PM) on the basis of the histological findings of muscle biopsy and treated with prednisolone. In addition, because anti-centromere antibodies and anti-mitochondrial M2 antibodies were positive with high titers, she was also diagnosed with primary biliary cirrhosis (PBC). Although PBC is often associated with other autoimmune diseases, there have been no reports of PBC complicated by PM and PH.
A 54-year-old man had undergone transcatheter arterial embolization (TAE) three times to treat hepatitis B virus-related hepatocellular carcinoma (HCC), but recurrence was found in June 2005. A large tumor in the left lateral portion of the liver showed extrahepatic growth and was attached to the gastric wall. TAE was performed a forth time. In September 2005, the patient was admitted with worsening anemia. Computed tomography and upper gastrointestinal endoscopy revealed that the HCC had directly invaded the stomach and caused gastrointestinal hemorrhage. Endoscopic hemostasis was effective, but the patient died because of worsening hepatic failure.
A 59-year-old man with poor oral hygiene presented to our hospital because of fever and chills. Abdominal ultrasonography and enhanced computed tomography (CT) revealed a liver abscess. The patient had no history of immunodeficiency and we confirmed the patient had no immunologic abnormalities. Blood culture revealed Fusobacterium nucleatum, a bacterium commonly found in the oral cavity. Even if a patient is immunocompetent, poor oral hygiene might be an independent risk factor for a pyogenic liver abscess. Professional mechanical tooth cleaning (PMTC) and appropriate self-care are recommended as a prophylaxis against not only dental, but also systemic diseases.