Internal medicine
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Objective This study was conducted to clarify the prevalence of short segment Barrett's esophagus (SSBE) using endoscopic observations with linked color imaging (LCI). In addition, the relationship between the presence of Barrett's epithelium (BE) and the status of H. pylori infection was investigated. Methods The study subjects were 3,353 individuals (2,186 men, 1,167 women; mean age 55.2±9.4 years old) whose status of H. pylori infection had been determined. ⋯ The degree of gastric mucosal atrophy was higher in cases with BE (p<0.001), although negativity for H. pylori infection and mild gastric mucosal atrophy were significant factors for the development of longer BE. Conclusion A high prevalence of SSBE was noted when LCI was used to determine the area of BE, as the distal end of the palisade vessels was easily visualized. Negativity for H. pylori infection and mild gastric mucosal atrophy were not correlated with SSBE prevalence.
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In triple A (Allgrove) syndrome, motor neuron disease is a co-morbid condition. We herein report a 38-year-old Japanese man with triple A (Allgrove) syndrome and novel tandem mutations: a novel c.881delT deletion mutation and c.835C>T localized to the AAAS gene. A nerve conduction study revealed marked axonal damage in several motor nerves. Tandem mutations in the AAAS gene may be involved in co-morbid motor neuron disease and aberrant electrophysiological findings.
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Hermansky-Pudlak syndrome (HPS) is an autosomal recessive hereditary disease that may be complicated by progressive and potentially fatal interstitial pneumonia. We herein report a 64-year-old woman with interstitial pneumonia associated with HPS type 4 whom we treated with nintedanib after pirfenidone proved ineffective. To our knowledge, there have been no previous reports of nintedanib being used to treat a patient with HPS type 4. There is a need for clinical trials of antifibrotic agents, including nintedanib, pirfenidone, and new therapeutic agents with different mechanisms of action in these patients.