Manganese deposition in the globus pallidus in patients with biliary atresia

BACKGROUND: Chronic liver diseases may alter trace element contents in the brain. Among these trace elements, manganese is a ubiquitous transition metal excreted by the liver into the bile. Blood concentrations of manganese are elevated in patients with biliary atresia who have undergone hepatic portoenterostomy. The present study investigated the effects of liver transplantation on manganese deposition in the brain in such patients. METHODS: The signal intensity of the globus pallidus was calculated as an index defined as the percentile ratio of signal intensity in the globus pallidus to the subcortical frontal white-matter in sagittal T1-weighted magnetic resonance imaging planes. RESULTS: Brain magnetic resonance imaging revealed hyperintense signals in the globus pallidus due to manganese deposition in biliary atresia patients. Few neurologic symptoms related to manganese intoxication were observed. However, one 23-year-old female with biliary atresia had depressive symptoms and dyskinesia; she improved after oral administration of the dopamine precursor, L-DOPA. Manganese deposition disappeared in two patients after living-related reduced-size hepatic transplantation. CONCLUSIONS: Manganese accumulates in the brain during cholestasis associated with biliary atresia and disappears after hepatic transplantation. Manganese deposition is likely to be subclinical and reversible but may be associated with some age-related neurologic symptoms.     

Manganese deposits in patients with biliary atresia after hepatic porto-enterostomy

PURPOSE: The aim of this study was to determine if there is latent manganese toxicity in patients with biliary atresia. METHODS: Fifteen children with biliary atresia were examined postoperatively with regard to whole-blood manganese levels using brain magnetic resonance imaging (MRI) and I-123 iodoamphetamine (IMP) per rectal portal scintigraphy. RESULTS: Nine (60%) of the 15 had high whole-blood manganese levels (mean, 4.1 microg/dL; range, 1.2 to 9.6; normal, 0.5 to 2.5), and these 9 had hyperintense globus pallidus on T1-weighted images, with no corresponding signal change in T2 sequences. I-123 IMP per rectal portal scintigraphy was done for 13 patients to evaluate portosystemic shunt flow. 12 (92%) of these patients had an increased flow. Mean shunt ratio was estimated to be 41% (range, 0.6 to 98; normal, <5%). Encephalopathy was evident in only 1 patient. CONCLUSIONS: Some patients with biliary atresia in the postoperative period have manganese deposits in globus pallidus on T1-weighted images and high whole-blood manganese levels, possibly caused by increased portsystemic shunt, and a latent or subclinical encephalopathy is also present.     

Dermoscopy of scalp tumours: a multi‐centre study conducted by the international dermoscopy society

Background Little is known about the dermoscopic features of scalp tumours. Objective To determine the dermoscopic features of scalp tumours. Methods Retrospective analysis of dermoscopic images of histopathologically diagnosed scalp tumours from International Dermoscopy Society members. Results A total of 323 tumours of the scalp from 315 patients (mean age: 52 years; range 3–88 years) were analysed. Scalp nevi were significantly associated with young age (<30 years) and exhibited a globular or network pattern with central or perifollicular hypopigmentation. Melanoma and non‐melanoma skin cancer were associated with male gender, androgenetic alopecia, age >65 years and sun damage. Atypical network and regression were predictive for thin (≤1 mm) melanomas, whereas advanced melanomas (tumour thickness > 1 mm) revealed blue white veil, unspecific patterns and irregular black blotches or dots. Conclusions The data collected provide a new knowledge regarding the clinical and dermoscopy features of pigmented scalp tumours.     

Predictive Factors for the Development or Regression of Fatty Liver in Japanese Adults

Fatty liver is commonly associated with alcohol or metabolic syndrome. We aimed to examine the longitudinal aspects of fatty liver, and clarify the independent predictors for the development or regression of fatty liver. In the present study, the clinical features of 1578 Japanese adults (1208 men and 370 women; 35 to 69 years of age) who visited our center both in 2000 and 2007–2008 were recorded and compared, including liver status diagnosed by ultrasonography. Of the 1578 participants, 217 (13.8%) showed fatty liver development, and 74 (4.7%) showed fatty liver regression. Logistic regression analysis revealed that body mass index and percentage body fat were strongly associated with the development or regression of fatty liver. Metabolic syndrome-related disorders such as serum levels of total cholesterol, triglyceride, uric acid, and fasting blood glucose were also associated with clinical course to some degree. However, the history of alcohol intake, the presence of metabolic syndrome, blood pressure, and habitual physical exercise were not independent predictors for the development or regression of fatty liver. Our present data suggest that control of body weight in men and the percentage body fat in women are particularly important for the prevention or treatment of fatty liver.     

A case of rapid development of arc welder's lung during the course of a year]

A 57-year-old man who had worked as a welder for about 40 years was admitted to our hospital. Every year he had had medical examinations in his workplace. On March 17, 1999, there was no finding of pneumoconiosis, but he had worked in a tunnel on a special arc welding project from December 20, 1999 to January 10, 2000. On admission, chest radiography revealed reticular shadows in the middle and lower lung fields. CT scans showed ground-glass opacities in both lungs. Serum iron was high (231 micrograms/dl) and serum ferritin was extremely high (2,309 ng/ml). Many iron particles were detected in the alveoli of the transbronchial biopsy specimen, and also in the sputum. A pathological diagnosis of siderosis was therefore made, and, considering the patient's occupation, the condition was termed arc welder's lung. This rapidly developing case was characterized by reticular shadows on chest radiography.     

Pica during pregnancy among Mexican‐born women: a formative study

Although pica, the craving and purposive consumption of non‐food substances, is common among many populations, especially during pregnancy, the health consequences are not well understood. Further, very little is known about pica among Mexican populations in the United States and Mexico. Therefore, we conducted formative research to understand pica in this understudied population. Our objectives were to identify the frequency and types of pica behaviours, to understand perceived aetiologies and consequences of pica and to ascertain if the behaviour was common enough to warrant a larger study. We held nine focus group discussions (three in the Salinas Valley, California; six in Xoxocotla, Morelos, Mexico) with 76 Mexican‐born women who were currently pregnant or had delivered within the past 2 years. Earth, adobe, bean stones and ice were the most commonly reported pica substances. Twenty‐eight of the 76 participants (37%) reported ever engaging in pica; 22 participants (29%) reported doing so during pregnancy. The proportion of women reporting pica in the United States and Mexico was 43% and 34%, respectively. Women attributed pica to the overwhelming organoleptic appeal of pica substances (especially smell and texture) and to micronutrient deficiencies. Perceived consequences of unfulfilled pica cravings were birthmarks or fetal loss; fulfilled pica cravings were also thought to be generally harmful to the mother or child, with several women specifying toxic lead, pesticides or ‘worms’. In sum, pica among Mexican women is common enough to warrant a larger epidemiologic study of its sociodemographic correlates and physiological consequences.     

Surgical Correction of Patent Ductus Venosus in Three BroThers

We report the presence of a patent ductusvenosus in three brothers who underwent surgicalcorrection. Patent ductus venosus was demonstrated byultrasonography. Portosystemic venous shunt ratios asevaluated by [¹²³I]iodoamphetamine per rectalportal scintigraphy were 67%, 50%, and 77%,respectively. Histologic examination of liver biopsyspecimens revealed fatty degeneration in all cases.Portal vein pressure before and after temporarily occluding thepatent ductus venosus was estimated by an Anthron P-Ucatheter introduced into the portal vein via theligament teres hepatis. Portal venous pressure increased from 10 to 17 cm H₂O, 16 to 23 cmH₂O, and 14 to 27 cm H₂O,respectively. Therefore, banding of the ductus venosuswith Teflon tape was attempted to prevent portalhypertension following complete ligation. As a result, portal venous pressures afterstricture of the ductus venosus were 12, 21, and 20 cmH2), respectively. Bile acid and liver enzymes decreasedand returned to normal within 14 days after surgery. Interestingly, serum concentrations ofhepatocyte growth factor (HGF) increased significantlyafter restoration of the portal blood flow and thengradually decreased, but remained persistently elevated for at least two weeks in two cases measuredafter surgical correction. One month after correction,liver function returned to normal as assessed byserological and histological parameters in all cases. These results suggest that it is important todetermine whether stricture or complete ligation isindicated for a patent ductus venosus during surgicalcorrection, based on the portal venous pressure after temporal test occlusion of the duct. Inaddition, HGF may be a useful marker for normalizationof hepatic microcirculation after surgery.     

Patient-specific radiotherapy quality assurance for estimating actual treatment dose

This study aimed to evaluate the optimal method for planning computed tomography (CT) for prostate cancer radiotherapy to avoid a dose difference of ≥3% between the actual and planned treatments using multiple acquisition planning CT (MPCT). We calculated the 3-dimensional (3D) displacement error between the pelvic bone and matching fiducial marker on MPCT and cone-beam CT scans of 25 patients who underwent prostate volumetric-modulated arc therapy for prostate cancer. The correlation of the 3D displacement error and the dose difference between planned and actual treatments was calculated using least squares second-order polynomial model. The 3D displacement error showed a moderate correlation with differences between planned and accumulated treatment doses (r = 0.587, p < 0.0001). Moreover, the improvement rate of the minimum 3D displacement error showed a strong correlation with the relative error between each MPCT image (r = 0.793, p < 0.0001). Significant differences were observed between planned and actual treatment doses (p < 0.0001) in the relative 3D displacement errors of <1 mm, 1 to 3 mm, and >3 mm. The 3D displacement error on MPCT (as the selection estimation index for optimal planning CT) is useful for monitoring patient-specific intensity-modulated radiation therapy quality assurance. This new method allows to estimate dose differences from the planned dose before commencing treatment, thereby ensuring high-quality therapy. As radiotherapy quality is critical for patient outcome, these findings may contribute to better management of prostate cancer.