There are surprisingly limited data regarding normal counts or distribution of eosinophils in the gastrointestinal tract, despite the increasing incidence of eosinophilic gastrointestinal tract diseases. Moreover, there are no published reports on the eosinophil number throughout the gastrointestinal tract of adults or Asian populations, or those investigating the effect of race on eosinophil count. First, in our study, the number of eosinophils from each portion of the gastrointestinal mucosa was quantified on biopsy slides from a Japanese adult population (132 samples). Next, the surgical resections from Japanese (110 samples), Japanese Americans (64), and Caucasians (57) were used to investigate the racial and environmental effects. Our results with the Japanese biopsy samples showed a significant increase in the number of eosinophils from the esophagus to the right colon (mean±SD/mm: 0.07±0.43 for the esophagus, 12.18±11.39 for the stomach, and 36.59±15.50 for the right colon), compared with a decrease in the left colon (8.53±7.83). Investigation using surgical samples showed that the distribution patterns in the gastrointestinal tract were very similar among the 3 ethnic groups, and there were no significant differences in the number of eosinophils among these groups, except in the esophageal epithelium. This study is the first report on the normal numbers and distribution of eosinophils throughout the gastrointestinal tract not only of an Asian population but also of adults. Our data suggest that a cutoff value for eosinophil counts, when rendering a diagnosis of eosinophilic gastrointestinal tract disease, should be individualized to the different biopsy sites. Interestingly, race and environmental factors did not seem to have a significant effect on eosinophil densities and distributions.

The presence of celiac disease in a non-clinical Japanese population was low at 0.05% and was rarely found in patients with unexplained chronic abdominal symptoms.

We successfully treated a 22-year-old woman with eosinophilic gastroenteritis (EGE) using a multiple food-elimination diet. The patient was diagnosed with EGE based on histopathological findings and initially treated with oral prednisolone. The symptoms immediately improved, although they recurred when prednisolone was tapered to a low dose. We then treated her with a multiple food-elimination diet, including milk, soy, eggs, wheat, nuts, seafood, and rice. During dietary treatment, we identified dairy products and eggs as causative foods of the symptoms, and we ceased prednisolone. Similar to eosinophilic esophagitis, an elimination diet may be effective for adult patients with EGE.

Adrenocortical carcinomas are relatively rare, but they are considered to be highly aggressive malignant tumors. Sarcomatoid carcinomas represent an even more aggressive type. Bilateral malignant adrenal tumors are extraordinary rare, except for those that represent metastatic spread from a primary neoplasm. Here we report a case of a 69-year-old woman who presented symptoms that raised strong suspicions of adrenal insufficiency. Bilateral adrenal masses, identified in the imaging study, were responsible for the clinical manifestation and surgically resected. Surgical specimens of the bilateral adrenal tumors shared histological features compatible with sarcomatoid carcinoma. It was very difficult to confirm that the sarcomatoid carcinomas were derived from the cortex of the adrenal glands, but careful morphological observation and the panel of antibodies used for immunohistochemistry made the diagnosis possible. This is the first report of sarcomatoid carcinomas involving both adrenal glands. It should be emphasized that sarcomatoid carcinoma can arise bilaterally from even functionally impaired adrenal glands.

We report a case of symptomatic carrier of Duchenne muscular dystrophy (DMD) in a 14-year-old girl with no prior family history of DMD. She presented with chest pain, enlarged calf muscles, an elevated serum creatine kinase (CK), and decreased left ventricular function. Histological examination of skeletal muscle revealed myopathic changes and immunostaining with anti-dystrophin antiserum demonstrated a mosaic pattern which are compatible with the observations in carriers of DMD. Southern blots using the dystrophin cDNA revealed no evidence of a deletion within the DMD gene in the patient or in her mother. We found this observation interesting for two reasons: 1. Cardiomyopathy is rare in female DMD patients. 2. Immunostaining of a muscle biopsy with anti-dystrophin serum proved to be valuable in the diagnosed for symptomatic carriers of the dystrophin gene mutation.

Background and study aims  Characteristic endoscopic findings, such as linear furrows, rings, and whitish exudates, indicate the presence of esophageal eosinophilia (EE), though no specific findings are known to distinguish eosinophilic esophagitis (EoE) from proton pump inhibitor-responsive esophageal eosinophilia (PPI-REE). Here, we present a novel endoscopic finding in some EE patients possessing a linear longitudinal arrangement of whitish nodules with the appearance of the back of an Ankylosaurus dinosaur, termed Ankylosaurus back sign (ABS), and evaluations of its significance in affected patients. Patients and methods  Fifty-five patients diagnosed with EE (≥ 15 eosinophils/high power field) who were treated at our hospital and shown to evaluate a PPI response were enrolled. Endoscopic findings at baseline and clinical parameters were retrospectively reviewed. Furthermore, the clinicopathological features of patients with ABS, as well as the relationship between its presence and PPI response were evaluated. Results  Fifty-five patients (47 males, 8 females) with EE (17 with EoE, 38 with PPI-REE) were evaluated, of whom 50 (90.9 %) had linear furrows, the most frequently found feature, while ABS was found in 9 (16.4 %). Inter-observer agreement was substantial for ABS (κ 0.77). Interestingly, all patients with ABS had PPI-REE. Our findings revealed that the presence of ABS was closely associated with reflux esophagitis (RE) in patients with PPI-REE. Conclusions  Although ABS was less frequent than typical endoscopic findings such as linear furrows in EE, this novel finding was closely associated with PPI-REE accompanied with RE. The clinical implications of ABS in patients with EE should be investigated further.

Lanthanum carbonate (LC) is a new type of phosphate adsorbent used to treat patients with hyperphosphatemia caused by chronic kidney diseases. Recent studies have pointed out that lanthanum deposition can be found in the cytoplasm of histiocytes in the gastroduodenal mucosa of these patients. On the other hand, it is well known that patients on long-term hemodialysis can develop deposition of β2-microglobulin-related amyloid (Aβ2M) mainly around joints. However, involvement of the gastrointestinal tract by hemodialysis-associated amyloidosis has been thought to be rare, and therefore only Aβ2M, if any, has been reported to accumulate in the vascular walls of the submucosa and muscularis propria. Thus, in contrast to AA amyloid, biopsy from gastrointestinal mucosa has long been considered to have little significance in detecting amyloid. We present unusual histologic findings on biopsy specimens taken from the gastroduodenal mucosa of 7 hemodialysis-dependent patients taking LC for more than a year. These findings were due to a combined deposition of lanthanum and β2-microglobulin-related amyloid in the cytoplasm of histiocytes. The deposition of amyloid was confirmed by conventional histochemistry, immunohistochemistry, and transmission electron microscopy, and that of lanthanum by transmission electron microscopy and scanning electron microscopy/energy dispersive X-ray spectrometry. This is the first report of such a peculiar combined deposition of lanthanum and amyloid in the gastroduodenal mucosa of hemodialysis patients. Although the exact mechanism of combination and pathogenesis is unclear, we believe that histologic examination of the gastrointestinal mucosa should be considered in the careful follow-up and observation of hemodialysis patients taking LC.

PurposePostmortem computed tomography (PMCT) has recently become important to clarify the cause of death in forensic medicine. It has also been proven to be useful for in-hospital deaths to a certain extent when interpreted by radiologists. However, accuracy of the interpretations of PMCT by non-radiologists remains to be elucidated. Nevertheless, they are often required to write death certificates based on the findings of PMCT in the absence of radiologists in Japan. We compared the interpretations of postmortem head CT (PMCT-H) by non-radiologists with the autopsy findings.MethodsThis study included 13 patients who underwent both brain dissection at autopsy and PMCT between June 2011 and December 2014. All cases were non-traumatic in-hospital deaths. Interpretation of PMCT was performed by the clinicians in charge of the patients, not by radiology experts.ResultsThe patients were first examined with PMCT and then autopsies were performed. Ten out of 13 cases were confirmed to have no lesions in the cranial cavity by both PMCT-H and autopsy. Two cases were diagnosed with intracranial hemorrhage (intracerebral and/or subarachnoid hemorrhage) and one with recurrent malignant lymphoma by both the clinicians and the pathologists. Intracranial hemorrhages were thought to be the direct causes of mortality of the two patients, and recurrent malignant lymphoma was considered to be one of the cardinal findings of the cancer death. There were no discrepancies between PMCT-H and autopsy findings.ConclusionsThe interpretations of PMCT-H by non-radiologists were completely the same as the autopsy findings regarding the non-traumatic in-hospital deaths in this study. It is premature to draw a definitive conclusion at present, but PMCT-H might be as effective as autopsy not only for those lesions described above but also for no remarkable changes in the brain. There has been no report on such a comparison. We believe further verification of the validity of interpretation of PMCT by non-radiologists is worthwhile in terms of death certificates made out in the absence of radiology experts and pathologists.