Successful endoscopic ultrasound-guided overstenting biliary drainage through a pre-existing proximal migrated metal biliary stent

Biliary endoscopic drainage using metallic self-expanded stents has become a well-established method for palliative treatment of malignant biliary obstruction. However, its occlusion, mainly by tumor overgrowth, is still the main complication without a standard treatment. We here describe a new method of treatment for biliary metallic stent occlusion, through the echo guided biliary drainage. We present a 68-year-old patient with metastatic pancreatic cancer previously treated for jaundice with ERCP and self-expandable metallic stent insertion. Four weeks later, the patient developed jaundice and symptoms of gastric outlet obstruction. A new ERCP confirmed obstruction of the second portion of the duodenum, due to diffuse tumor growth. EUS was performed, and the previous metal biliary stent was seen occluded at the distal portion in the common bile duct. A EUS-guided choledocododenostomy was performed and then, an overlapping self-expanding metal enteral stent was placed through the malignant obstruction. There were no early complications and the procedure was also clinically effective in relieving jaundice and gastric outlet obstruction symptoms. If ERCP fails in the management of occluded biliary metallic stents, EUS biliary drain can provide effective biliary decompression and should be considered an alternative to other endoscopic techniques.     

Is Transforming Stem Cells to Pancreatic Beta Cells Still the Holy Grail for Type 2 Diabetes?

Diabetes is a progressive disease affecting millions of people worldwide. There are several medications and treatment options to improve the life quality of people with diabetes. One of the strategies for the treatment of diabetes could be the use of human pluripotent stem cells or induced pluripotent stem cells. The recent advances in differentiation of stem cells into insulin-secreting beta-like cells in vitro make the transplantation of the stem cell-derived beta-like cells an attractive approach for treatment of type 1 and type 2 diabetes. While stem cell-derived beta-like cells provide an unlimited cell source for beta cell replacement therapies, these cells can also be used as a platform for drug screening or modeling diseases.     

Case of toxic epidermal necrolysis occurring after bone marrow transplantation accompanied by engraftment failure

Toxic epidermal necrolysis (TEN) is a rare condition, causing life‐threatening adverse cutaneous reactions. TEN occurrence after bone marrow transplantation (BMT) is a well‐known phenomenon; however, to date, only a few cases have been reported in the published work. Here, we describe the case of a 53‐year‐old woman who experienced TEN after undergoing allogenic BMT for malignant lymphoma. Skin erosion spread across a maximum of 70% of the body surface area and severe mucosal lesions developed. Steroid pulse therapy, plasma apheresis and immunoglobulin therapy were administrated, which resulted in the complete resolution of TEN. However, she developed hemophagocytic lymphohistiocytosis and died 38 days after BMT, owing to rupture of the lower digestive tract complicated by multi‐organ failure. In our case, engraftment failure occurred, and the peripheral white blood cell count was less than 100/μL during the TEN course, suggesting that the presence of only a few immune cells could cause TEN. Our findings showed that high mortality rates and widespread skin erosion could be regarded as the most important characteristics of TEN occurring after BMT.     

Cortical Distribution of Fragile Periventricular Anastomotic Collateral Vessels in Moyamoya Disease: An Exploratory Cross-Sectional Study of Japanese Patients with Moyamoya Disease

BACKGROUND AND PURPOSE:Collateral vessels in Moyamoya disease represent potential sources of bleeding. To test whether these cortical distributions vary among subtypes, we investigated cortical terminations using both standardized MR imaging and MRA.MATERIALS AND METHODS:Patients with Moyamoya disease who underwent MR imaging with MRA in our institution were enrolled in this study. MRA was spatially normalized to the Montreal Neurological Institute space; then, collateral vessels were measured on MRA and classified into 3 types of anastomosis according to the parent artery: lenticulostriate, thalamic, and choroidal. We also obtained the coordinates of collateral vessel outflow to the cortex. Differences in cortical terminations were compared among the 3 types of anastomosis.RESULTS:We investigated 219 patients with Moyamoya disease, and a total of 190 collateral vessels (lenticulostriate anastomosis, n = 72; thalamic anastomosis, n = 21; choroidal anastomosis, n = 97) in 46 patients met the inclusion criteria. We classified the distribution patterns of collateral anastomosis as follows: lenticulostriate collaterals outflowing anteriorly (P < .001; 95% CI, 67.0–87.0) and medially (P < .001; 95% CI, 11.0–24.0) more frequently than choroidal collaterals; lenticulostriate collaterals outflowing anteriorly more frequently than thalamic collaterals (P < .001; 95% CI, 34.0–68.0); and choroidal collaterals outflowing posteriorly more frequently than thalamic collaterals (P < .001; 95% CI, 14.0–34.0). Lenticulostriate anastomoses outflowed to the superior or inferior frontal sulcus and interhemispheric fissure. Thalamic anastomoses outflowed to the insular cortex and cortex around the central sulcus. Choroidal anastomoses outflowed to the cortex posterior to the central sulcus and the insular cortex.CONCLUSIONS:Cortical distribution patterns appear to differ markedly among the 3 types of collaterals.

Collateral vessels in Moyamoya disease develop as the disease progresses.¹ Lenticulostriate arteries (LSAs), perforators from the posterior communicating artery (PcomA), and anterior and posterior choroidal arteries (choAs) are representative collateral vessels that supply the cortex.^2-4 Development of such collateral vessels represents a risk factor for intracerebral hemorrhage,^3,5-7 and these vessels have frequently been considered as the vessels responsible for bleeding in recent reports.^8-10 These collateral vessels connect with medullary arteries near the lateral ventricle and thus supply the cortex via the medullary arteries.^3,4 However, no reports have addressed the cortical distributions of these collateral vessels.Bypass surgery reduces the risk of rebleeding in patients with hemorrhagic onset of Moyamoya disease^7,11-13 and also shrinks collateral vessels in Moyamoya disease.^7,12,14,15 Augmentation of cerebral blood flow via bypass seems to decrease the necessity for development of collateral flow and shrinks existing collaterals.¹⁵ To shrink risky collateral vessels effectively and prevent hemorrhage, well-designed and planned bypass surgeries may be required.¹⁶ Comprehension of the nature and cortical distribution of collateral vessels may thus be clinically useful.MRA performed using a 3T scanner has proved useful for detecting the abnormally extended collateral vessels in Moyamoya disease.² We investigated the cortical distribution of collateral vessels using 3T MR imaging and MRA to clarify whether cortical distributions vary among anastomotic subtypes and to better understand collateral networks.     

Evaluation of avulsed primary incisor in 3-year-old girl with hypophosphatasia who received enzyme replacement therapy

Hypophosphatasia (HPP) is an inherited skeletal disease with features of hypomineralization of bone and early exfoliation of primary teeth due to disturbed cementum formation. Recent introduction of enzyme replacement therapy has reduced mortality in severe cases, though the effects of that treatment on dental manifestations remain unknown. We examined an avulsed primary incisor in a 3-year-old female diagnosed with perinatal HPP who underwent enzyme replacement therapy from 1 day after birth and report our findings.     

Successful application of molecular biological technique for evaluation of changes in periodontopathic bacteria in Japanese children with developmental disabilities

Developments in molecular biological techniques enables rapid and easy identification of periodontopathic bacterial species in clinical specimens. However, there are few reports regarding their application for community dentistry. The aim of this study was to show successful application of a molecular biological technique for evaluation of changes in periodontal bacterial species in children at daycare centers. We studied 187 children who received oral examinations in 2009 and 186 who received examinations in 2010, among whom 102 were examined in both years. Clinical parameters regarding periodontal conditions were evaluated and the distribution of 10 periodontopathic species in dental plaque specimens were determined by polymerase chain reaction. Periodontal pocket depth values in the 2010 group were significantly smaller than those in 2009. When the subjects were divided into those with (positive group) and without (negative group) Porphyromonas gingivalis, Treponema denticola, Tannerella forsythia, or Campylobacter rectus in 2009, the positive group had significantly smaller periodontal pocket values than the negative group. In addition, the rate of subjects with P. gingivalis, T. denticola, T. forsythia, or C. rectus in the positive group in 2010 was significantly reduced. Our findings demonstrate that molecular biological methods provide more information as compared to a standard clinical examination when evaluating changes of periodontal conditions in the field of community dentistry.     

Hypertrophic cardiomyopathy with progression from apical hypertrophy to asymmetrical septal hypertrophy: a case report

A 41-year-old man was referred to our hospital for further examination because of abnormal electrocardiography findings at a health-check examination. Transthoracic echocardiography showed left ventricular hypertrophy confined to the most distal portion of the left ventricle, which is a typical feature of apical hypertrophic cardiomyopathy. Ten years later, he was again admitted for the evaluation of chest pain. Echocardiography showed asymmetrical septal hypertrophy in addition to apical hypertrophy. These findings demonstrate morphologic evolution in hypertrophic cardiomyopathy from apical hypertrophy to asymmetrical septal hypertrophy.     

Prognostic and clinical significance of newly acquired complete right bundle branch block in Japan Airline pilots

OBJECTIVE: The purpose of this study was to evaluate the prognostic and clinical significance of newly acquired complete right bundle branch block (CRBBB) in airline pilots. PATIENTS: This study included pilots with acquired CRBBB, identified from a group of over 2,700 Japan Airline pilots. When the pilots applied for employment, a past medical history, physical examination, electrocardiogram, and chest radiograph were obtained. The pilots with ECG abnormality including CRBBB were not included in the study because of hiring requirements. RESULTS: Thirty-six pilots with CRBBB were identified between 1983 and 2002. All pilots with CRBBB were evaluated for the presence of ischemic heart disease by treadmill exercise testing, echocardiogram and exercise thallium scintigraphy. Twelve individuals underwent coronary angiography. The mean age of pilots was 44.4 +/- 5.8 years. The mean observation period was 10.9 +/- 5.7 years. For each of the 36 study subjects, Holter electrocardiogram and echocardiogram were obtained every 6 months after the CRBBB was detected. Exercise stress testing was performed every year. Exercise thallium scintigraphy was performed every 2 years to detect ischemic heart disease. During the observation period, two pilots stopped flying temporarily because of frequent ventricular premature beats and one pilot stopped flying permanentaly because of atrial fibrillation. During the follow-up period, no cardiovascular events were observed in pilots with CRBBB who had no underlying ischemic heart disease. CONCLUSION: Acquired CRBBB does not confer a poor prognosis, particularly in young men working as a pilot if there is no evidence of ischemia on exercise stress testing, echocardiography and exercise thallium scintigraphy.