Objective Growth hormone (GH) deficiency has recently been reported as a cause of nonalcoholic fatty liver disease (NAFLD), and GH supplementation has been shown to improve the histology of NAFLD. The aim of the present study was to clarify the relationship between the histological severity of NAFLD and production of the GH/insulin-like growth factor 1 (IGF-1) axis.
Methods A total of 222 Japanese patients with liver biopsy-confirmed NAFLD and 55 patients with hepatitis C virus (HCV)-related chronic liver disease (CLD) were enrolled in the present study. The serum levels of GH, IGF-1, and IGF-binding protein 3 (IGFBP-3) were measured and their relationships with the histological severity of liver disease were assessed. To exclude age- and sex-related differences, the IGF-1 standard deviation score (IGF-1:SDS) was determined for each patient.
Results With respect to the stage of fibrosis in patients with NAFLD, the serum GH levels were higher and the serum IGFBP-3 levels and IGF-1:SDSs were lower in patients with cirrhosis (grade F4 fibrosis) than in patients grade F1-F3 fibrosis; moreover, these differences were statistically significant (all p<0.01). The GH, IGF-1, and IGFBP-3 levels were not correlated with fibrosis in patients with HCV-related CLD. Furthermore, the GH levels were lower and the IGFBP-3 levels were significantly higher in patients with severe steatosis (S3) than in patients with mild to moderate steatosis (S1-S2) (p<0.05).
Conclusion Increased GH levels and decreased IGF-1 and IGFBP-3 levels might contribute to the progression of NAFLD. The GH/IGF-1 axis may be important in the development of NAFLD, but not in patients with HCV-related CLD.
Objective The National Kidney Foundation (NKF) Kidney Disease Outcome Quality Initiative (KDOQI) guidelines have recommended the use of arteriovenous fistula (AVF) at the initiation of dialysis. However, there are significant differences in the dialysis environments of Japan and the United States, and there are few people who receive hemodialysis via a central venous catheter (CVC) in Japan. The aim of the present study was to examine the association between the type of vascular access at the initiation of dialysis and the incidence of mortality in Japan.
Methods This study was a prospective, multicenter, cohort study. The data was collected by the Aichi Cohort study of Prognosis in Patients newly initiated into dialysis (AICOPP) in which 18 Japanese tertiary care centers participated. The present study enrolled 1,524 patients who were newly introduced to dialysis (the patients started maintenance dialysis between October 2011 and September 2013). After excluding 183 patients with missing data, 1,341 patients were enrolled. The Cox proportional hazards model was used to evaluate mortality based on the type of vascular access. The types of vascular access were divided into four categories: AVF, arteriovenous graft (AVG), CVC changed to AVF during the course (CAVF), CVC changed to AVG during the course (CAVG).
Results A multivariate analysis revealed that AVG, CAVF and CAVG were associated with a higher risk of mortality in comparison to AVF [hazard ratio (HR), 1.60; p=0.048; HR, 2.26; p=0.003; and HR, 2.45; p=0.001, respectively].
Conclusion The research proved that the survival rate among patients in whom hemodialysis was initiated with AVF was significantly higher than that in patients in whom hemodialysis was initiated with AVG or CVC.
Objective Legionnaires' disease (LD) is a common form of lobar pneumonia, but the optimum diagnostic modality has long been a subject of debate due to incomplete sensitivity and specificity. A delay in the initiation of specific therapy for LD is associated with increased mortality. The decision to treat a patient for Legionella must be made quickly. The purpose of this study was to evaluate the ability of the modified Winthrop-University Hospital WUH system to identify LD while discriminating against pneumococcal pneumonia at the time of hospitalization for community-acquired pneumonia.
Methods Five patients with LD and 13 patients with pneumococcal pneumonia were retrospectively analyzed.
Results The WUH system identified 4 of 5 patients with LD (sensitivity, 80%) while excluding legionellosis in 12 of 13 patients with pneumococcal pneumonia (specificity, 92%). The positive and negative likelihood ratios were 10.4 and 0.2. The area under the receiver operating characteristic curve was 0.969.
Conclusion The WUH system is useful for obtaining a rapid presumptive clinical diagnosis of LD. Further investigation with a larger number of patients is strongly recommended.
Objective Irritable bowel syndrome (IBS) is associated with a high prevalence of psychological disorders. Coping, which is used to manage stressful events, can have adaptive or maladaptive effects on a patient's health status. However, these coping behaviors have not been well studied in individuals with IBS. The association between the use of coping behaviors and the reported levels of depressive symptoms was examined in individuals with IBS.
Methods Among 993 volunteers (382 men and 611 women) who participated in the Iwaki Health Promotion Project in 2013, we included 58 subjects who fulfilled the Rome III criteria for IBS in our analysis. Coping behaviors were assessed using the Brief Scale for Coping Profile (BSCP). The Center for Epidemiologic Studies Depression Scale (CES-D) was used to assess the four dimensions of depressive symptoms (depressed affect, somatic symptoms, interpersonal problems, and lack of positive affect) and the prevalence of probable depression using a cut-off score of 16.
Results Among the 58 subjects with IBS, 22 (36.1%) exhibited probable depression. After adjustment for confounders, "active solution" was found to be significantly associated with somatic symptoms. Under the same conditions, "avoidance and suppression" was significantly associated with the CES-D total score, depressed affect, somatic symptoms, and (lack of) positive affect. There were no other significant relationships between depressive symptoms and the BSCP sub-scale scores.
Conclusion These findings indicate that coping behaviors may influence the experience of depressive symptoms among individuals with IBS. Psychological therapy may reduce depressive symptoms as well as the severity of IBS symptoms. Additional studies are needed to examine the relationships between coping behaviors and depressive symptoms using a longitudinal study design.
We report the case of a 57-year-old man with a 1.0-cm grade 1 neuroendocrine tumor (G1 NET) of the ampulla of Vater (ampullary NET) who underwent endoscopic snare papillectomy. Pancreatitis occurred after endoscopic resection but was cured with conservative therapy. In two years of follow-up, no local recurrence or metastasis occurred. Endoscopic snare papillectomy for small G1 NET of the ampulla of Vater is relatively safe and less invasive than surgical treatment and should be considered as treatment option.
We herein report the case of a rapidly progressive sporadic mesenteric desmoid tumor (DT). A 62-year-old woman presented with a 4-cm-diameter palpable mass in the left supraumbilical area. The mass showed an ill-defined margin with heterogeneous delayed enhancement on computed tomography and heterogeneous high intensity on T2-weighted magnetic resonance imaging. Sixteen months after the initial observation, the mass had grown in size, reaching 13 cm in diameter. The resected mass was histologically confirmed as a DT of the mesentery. Since DT often has an unpredictable clinical course, clinicians should bear in mind the need for imaging follow-up.
Overlap syndrome between primary sclerosing cholangitis (PSC) and autoimmune hepatitis (AIH) is extremely rare in Japan. We herein report two adult patients with PSC-AIH overlap syndrome. They were diagnosed with PSC-AIH overlap syndrome based on the findings of endoscopic retrograde cholangiography and liver biopsy, and using the International Autoimmune Hepatitis Group scoring system. In both cases, PSC preceded AIH, and combination therapy with steroid and ursodeoxycholic acid was effective. Because there are few reported cases in Japan, it is important to study more cases to shed light on the clinical and pathological features of PSC-AIH overlap syndrome.
A 53-year-old man presented with diarrhoea and hypokalaemia and was diagnosed with a neuroendocrine tumour of unknown origin with multiple liver metastases. Somatostatin analogues led to a reduction in the size of the tumours and improvement of his symptoms. However, after several years, the tumours grew in size, and the patient's clinical symptoms recurred. The patient underwent transcatheter arterial embolization (TAE) of the hepatic artery to treat the liver metastases. Immediately after embolization, the symptoms disappeared. Although the patient had an unresectable vasoactive intestinal polypeptide-producing neuroendocrine tumour, the endocrine symptoms were able to be controlled with chemotherapy and TAE, resulting in a long-term survival.
We experienced a man in his 20s with inappropriate sinus tachycardia (IST) initially diagnosed and treated as depression who was steadily treated with radiofrequency catheter ablation (RFCA) using an EnSite™ system. The patient has remained well without any symptoms or medications, including antidepressants, for two years since the RFCA. To avoid missing IST and treating it as an emotional problem and/or mental illness such as depression, physicians - including cardiologists - should be aware of these conditions when examining patients with multiple and incapacitating complaints including palpitations and general fatigue and/or tachycardia, especially characterized by an elevated resting heart rate or a disproportionate increase in the heart rate with minimal exertion.
We herein report a 31-year-old Japanese woman with evolving hypopituitarism due to pituitary stalk transection syndrome. She had a history of short stature treated with growth hormone (GH) in childhood and had hypothyroidism and primary amenorrhea at 20 years old. Levothyroxine replacement and recombinant follicle stimulating hormone-human chorionic gonadotropin (FSH-hCG) therapy for ovulation induction were started. GH replacement therapy (GHRT) was resumed when she was 26 years old. She developed mild adrenocortical insufficiency at 31 years old. She succeeded in becoming pregnant and delivered twice. GHRT was partially continued during pregnancy and stopped at the end of the second trimester without any complications.
Although most women with type 1 diabetes experience the normal transition to menopause, there is little information about the impact of hormone replacement therapy on their glycemic profiles. A 54-year-old postmenopausal woman with fulminant type 1 diabetes was admitted to our hospital due to diabetic ketoacidosis. She was treated with fluid replacement and a continuous insulin infusion. Thereafter, her glycemic profile was well maintained by daily multiple insulin injections. However, her glycemic profiles immediately deteriorated following the administration of progesterone in hormone replacement therapy. This transient deterioration implies that external progesterone can lead to the deterioration of glycemic profiles in postmenopausal women with type 1 diabetes.
A 77-year-old man was suspected of having tumor-induced osteomalacia (TIO) because of hypophosphatemia (1.9 mg/dL) and elevated serum fibroblast growth factor 23 (FGF23) level (186.9 pg/mL). We detected a tumor in his left parotid gland, and the FGF23 level in the left external jugular vein indicated that the tumor overproduced FGF23. After the removal of the tumor, the serum FGF23 level rapidly decreased, and the serum phosphate normalized. This is the first case of TIO caused by a tumor in a parotid gland. This case indicates that the responsible tumors for TIO can be quite diverse.
Henoch Schönlein purpura (HSP), also known as IgA vasculitis (IgAV), is a systemic small-vessel vasculitis that predominantly affects adolescents and is rare in adults. In many cases, the onset of HSP has been causally linked to an infectious disease. We encountered a case of HSP with severe renal involvement diagnosed by renal biopsy following bacillus Calmette-Guerin (BCG) therapy for bladder cancer. This is of clinical relevance, as intravesical BCG administration is becoming an established therapy for superficial bladder cancer and is supposed to be safe. It is important for all clinicians to recognize that BCG therapy has this rare but potentially serious systemic complication.
Although patients with anorexia nervosa (anorexia) are known to show tubulointerstitial nephritis (TIN), the pathophysiology of its progression is not fully understood. We herein report a 31-year-old woman with anorexia who showed acute exacerbation of chronic kidney disease. Renal biopsy showed non-uniform chronic TIN; some areas were obsolete lesions and other areas were active lesions. In addition, many calcium-containing crystals were widely deposited in the distal tubules. The results suggest that chronic TIN in the setting of anorexia does not uniformly progress and that not only TIN but also widespread calcification of distal tubules might aggravate the renal function of anorexia patients.
We herein report the case of a 44-year-old man who was diagnosed with pneumocystis pneumonia (PCP) concomitant with ectopic adrenocorticotropic hormone (ACTH) syndrome, which had been caused by a large cell neuroendocrine carcinoma of the thymus. Chest computed tomography revealed ground-glass opacities in the lungs. PCP was diagnosed by a polymerase chain reaction with bronchoalveolar lavage. The levels of cortisol were slowly corrected with an adrenal enzyme inhibitor, and the exacerbation of PCP was successfully avoided. Our case indicates that in addition to prophylaxis, the early diagnosis of PCP and the slow correction of hypercortisolemia should be considered in order to prevent an exacerbation due to the reconstitution of the immune function in patients with ectopic ACTH syndrome.
An 85-year-old woman underwent emergent splenectomy due to left abdominal pain and active bleeding in a massively enlarged spleen. The histological diagnosis was splenic marginal zone lymphoma (SMZL). A prolonged activated partial thromboplastin time (APTT) was noted, and additional tests led to the diagnosis of type 2A-like acquired von Willebrand syndrome (AVWS). An APTT cross mixing test ruled out the presence of inhibitors. She received eight courses of rituximab monotherapy. The coagulation data showed no improvement, possibly because the lymphoma showed a poor response to the treatment. AVWS rarely causes bleeding in solid organs. This is the first case of SMZL with AVWS diagnosed via splenic bleeding.
Body lateropulsion is known to be caused commonly by lateral medullary lesions but rarely by pontine lesions. It is also known to be associated with lesions of the dorsal spinothalamic tract or ascending graviceptive pathways. We herein report the case of a 75-year-old woman presenting with contralateral lateropulsion and cerebellar tremor caused by pons infarction. To our knowledge, this is the first case report of pontine infarction causing both lateropulsion and cerebellar tremor. Our case may be helpful in anatomical studies of ascending graviceptive pathways.
Merkel cell carcinoma (MCC) is a rare neuroendocrine carcinoma of the skin with an aggressive clinical course. Although anthracycline- and platinum-based regimens are empirically used as first-line treatments for metastatic or unresectable cases, no salvage therapy has been established. A 73-year-old man with platinum-refractory recurrent MCC was treated with amrubicin. The symptoms improved soon, and a partial response was achieved. A total of nine cycles of amrubicin were administered in nine months with manageable adverse events until disease progression was finally observed. The present findings suggest the potential of amrubicin monotherapy as a second-line therapy for patients with advanced/recurrent MCC.