Localized Immunoglobulin Light-Chain Amyloidosis of the Ulnar Nerve

Amyloidosis is a disorder caused by extracellular tissue deposition of insoluble fibrils. Amyloidosis can be divided into systemic or localized disease. Primary systemic amyloidosis is a multisystem disease caused by the deposition of amyloid in various tissues. Localized amyloidosis has different characteristics than those of systemic amyloidosis. In this paper, we present the case of a middle-aged woman who presented with worsening ulnar nerve palsy. Electrophysiological examination and MRI indicated a tumor surrounding the ulnar nerve in the forearm. However, the operative findings revealed that ulnar nerve fascicles were replaced with a yellow tissue, which was diagnosed as amyloid light-chain λ amyloidosis, based on histopathological examination. Systemic amyloidosis was ruled out after the screening examinations. This paper is the first report of the ulnar nerve as the sole site of localized immunoglobulin light-chain amyloidosis manifestation

High Efficacy of Preoperative Low-Dose Radiotherapy with Sanazole (AK-2123) for Extraskeletal Ewing's Sarcoma: A Case Report

Extraskeletal Ewing's sarcoma is a rare soft tissue tumor that is morphologically indistinguishable from Ewing's sarcoma of bone. We report a case of extraskeletal Ewing's sarcoma with several systemic problems. A 69-year-old man presented with a 5-month history of a rapidly enlarging mass in the right thigh. Because preoperative radiotherapy with sanazole (AK-2123) contributed the tumor mass reduction down to 40% in size, the tumor was successfully resected with clear surgical margins and repaired with a musculocutaneous flap. The high efficacy of pre-operative low-dose radiotherapy with sanazole was histologically confirmed that the resected tumor specimen involved no viable tumor cells and showed 100% necrosis. Based on clinical outcomes in this case, the combined modality of pre-operative low-dose radiotherapy with hypoxic cell radiosensitizer and adequate surgical resection might provide for the useful clinical application of extraskeletal Ewing's sarcoma treatment

Idiopathic benign retroperitoneal cyst: a case report

<p>Abstract</p> <p>Introduction</p> <p>Retroperitoneal cysts are uncommon, with an estimated incidence of 1/5750 to 1/250,000.</p> <p>Case presentation</p> <p>A male patient was admitted with an abdominal pain, jaundice and fever. Clinical examination and investigations confirmed an idiopathic benign retroperitoneal cyst. He underwent surgery and was discharged after making good recovery.</p> <p>Conclusion</p> <p>Retroperitoneal cysts are very rare, and most of the time they are discovered incidentally. Patients may be asymptomatic or present with abdominal pain, referred pain to the legs or weight loss. Imaging may help diagnose these lesions, but surgery is the keystone in confirming the diagnosis. This case is very rare and very educational as it highlights an unusual presentation of a benign retroperitoneal cyst. In our patient, the course of the disease was unique as the patient presented with jaundice.</p

A Dipeptidyl Peptidase-4 Inhibitor, Des-Fluoro-Sitagliptin, Improves Endothelial Function and Reduces Atherosclerotic Lesion Formation in Apolipoprotein E–Deficient Mice

ObjectivesThe aim of this study was to investigate the antiatherogenic effects of the dipeptidyl peptidase-4 inhibitor, des-fluoro-sitagliptin (DFS).BackgroundThe new class of anti–type 2 diabetes drugs, dipeptidyl peptidase-4 inhibitors, improves glucose metabolism by increasing levels of active glucagon-like peptide (GLP)-1.MethodsEndothelial function was examined by acetylcholine-induced endothelium-dependent vasorelaxation using aortic rings and atherosclerotic lesion development in the entire aorta in apolipoprotein E–deficient mice fed a high-fat diet with or without DFS, and the antiatherogenic effects of DFS were investigated in cultured human macrophages and endothelial cells. Plasma levels of active GLP-1 were measured in patients with or without coronary artery disease.ResultsDFS significantly improved endothelial dysfunction (89.9 ± 3.9% vs. 79.2 ± 4.3% relaxation at 10−4 mol/l acetylcholine, p < 0.05) associated with increased endothelial nitric oxide synthase phosphorylation and reduced atherosclerotic lesion area (17.7% [15.6% to 25.8%] vs. 24.6% [19.3% to 34.6%], p < 0.01) compared with vehicle treatment. In cultured human macrophages, DFS significantly increased GLP-1-induced cytosolic levels of cyclic adenosine monophosphate compared with GLP-1 alone, resulted in inhibiting phosphorylation of c-jun N-terminal kinase and extracellular signal-regulated kinase 1/2 and nuclear factor-kappa B p65 nuclear translocation through the cyclic adenosine monophosphate/protein kinase A pathway, and suppressed proinflammatory cytokines (i.e., interleukin-1-beta, interleukin-6, and tumor necrosis factor-alpha) and monocyte chemoattractant protein-1 production in response to lipopolysaccharide. DFS-enhanced GLP-1 activity sustained endothelial nitric oxide synthase phosphorylation and decreased endothelial senescence and apoptosis compared with GLP-1 alone. In the human study, fasting levels of active GLP-1 were significantly lower in patients with coronary artery disease than those without (3.10 pmol/l [2.40 to 3.62 pmol/l] vs. 4.00 pmol/l [3.10 to 5.90 pmol/l], p < 0.001).ConclusionsA DPP-4 inhibitor, DFS, exhibited antiatherogenic effects through augmenting GLP-1 activity in macrophages and endothelium

The clinical impact of macrophage polarity after Kasai portoenterostomy in biliary atresia

IntroductionBiliary atresia (BA) is a cholestatic hepatopathy caused by fibrosing destruction of intrahepatic and extrahepatic bile ducts, and its etiology has not been clearly revealed. In BA, liver fibrosis progression is often observed even after Kasai portoenterostomy (KPE), and more than half of cases require liver transplantation in their lifetime in Japan. Macrophages play an important role in liver fibrosis progression and are classically divided into proinflammatory (M1) and fibrotic macrophages (M2), whose phenotypic transformation is called “macrophage polarity.” The polarity has been reported to reflect the tissue microenvironment. In this study, we examined the relationship between macrophage polarity and the post-KPE clinical course.Materials and methodsThirty BA patients who underwent KPE in our institution from 2000 to 2020 were recruited. Multiple immunostainings for CD68, CD163, CK19, and α-SMA were carried out on liver biopsy specimens obtained at KPE. ROC curves were calculated based on each clinical event, and the correlation with the clinical data was analyzed.Results and discussionThe M2 ratio, defined as the proportion of M2 macrophages (CD163-positive cells), was correlated inversely with the occurrence of postoperative cholangitis (AUC: 0.7602). The patients were classified into M2 high (n = 19) and non-high (n = 11) groups based on an M2 ratio value obtained from the Youden index ( = 0.918). As a result, pathological evaluations (Metavir score, αSMA area fraction, and CK19 area fraction) were not significantly different between these groups. In mild liver fibrosis cases (Metavir score = 0–2), the M2 non-high group had a significantly lower native liver survival rate than the high group (p = 0.02). Moreover, 4 out of 8 cases in the M2 non-high group underwent early liver transplantation within 2 years after KPE.ConclusionsNon-M2 macrophages, including M1 macrophages, may be correlated with postoperative cholangitis, and the M2 non-high group in mild liver fibrosis cases had a significantly lower native liver survival rate than the high group, requiring early liver transplantation in this study. Preventing advanced liver fibrosis is a key factor in improving native liver survival for BA patients, and liver macrophages may play important roles in liver homeostasis and the promotion of inflammation and fibrosis

Accumulation of Pericardial Fat Correlates with Left Ventricular Diastolic Dysfunction in Patients with Normal Ejection Fraction

Background Left ventricular diastolic dysfunction (LVDD) plays an important role inheart failure with normal left ventricular ejection fraction (LVEF). Obesity is one ofthe major comorbid conditions of LVDD. Pericardial fat (PF) is an ectopic fat depotwith possible paracrine or mechanical effects on the coronary circulation and35 myocardial function.Methods We measured PF volume on 64 slice computed tomography and analyzedechocardiographic parameters to confirm LVDD in 229 consecutive patients suspectedof coronary artery disease with LVEF of more than 50% and no symptomatic heartfailure (59% men, 67±12 years). LVDD was defined as the ratio of transmitral40 Doppler early filling velocity to tissue Doppler early diastolic mitral annular velocity(E/e’) >10.Results PF volume correlated significantly with E/e’ (r=0.21, p<0.01), left ventricularmass index (r=0.23, p<0.001), and left atrial diameter (r=0.32, p<0.001). The mean PFvolume was significantly greater in patients with LVDD (184±61 cm3, n=141) than in45 those without LVDD (154±58, n=88, p<0.001). Multivariate logistic regressionanalysis indicated that PF volume correlated significantly with the presence of LVDD(odds ratio: 2.00 per 100 cm3 increase in PF volume, p=0.02) independent of age,gender, abdominal obesity, hypertension, and diabetes.Conclusions PF volumes are significantly associated with LVDD, independent of50 other factors such as hypertension or diabetes. PF may be implicated in the pathogenesis of LVDD in patients with normal LVEF