Internal medicine
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Case Reports
Pulmonary Hypertension and Hereditary Hemorrhagic Telangiectasia Related to an ACVRL1 Mutation.
Pulmonary hypertension and hereditary hemorrhagic telangiectasia (HHT) have an association mediated by activin A receptor type II-like 1 (ACVRL1) gene pathogenic variants. A 30-year-old woman was previously admitted to a hospital due to lung hemorrhage, and was diagnosed with pulmonary hypertension, but stopped follow-up visits. ⋯ After the initiation of pulmonary vasodilator treatment, the patient's mean pulmonary artery pressure started to decrease from 43 mmHg, declining to 37 mmHg when she was 58 years of age. This is the first report describing the 28-year follow-up of an HHT and pulmonary hypertension patient with an ACVRL1 mutation.
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Case Reports
New-onset Refractory Status Epilepticus Involving the Limbic System, Spinal Cord, and Peripheral Nerves.
A healthy 28-year-old woman presented suddenly with intractable status epilepticus: a focal seizure evolved into a generalized seizure preceded by a high fever. Brain magnetic resonance imaging indicated bilateral hyperintensities in the hippocampus on T2-weighted imaging. Electroencephalograms continuously demonstrated diffuse sharp waves and poly-spikes. ⋯ We therefore diagnosed her with cryptogenic limbic encephalitis and new-onset refractory status epilepticus (NORSE). We detected positive anti-ganglioside antibodies, IgG-GQ1b, GD1a, and GT1b, which were negative at six months after the onset. We emphasize the heterogeneous pathogenesis and intractable conditions of NORSE.
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Non-typhoidal Salmonella (NTS) infection is a major pathogen causing gastroenteritis among immunocompetent adults. NTS infection is mainly transmitted by contaminated food and water, but some cases are transmitted by animal contact. Salmonella enterica subsp. enterica serovar Poona (S. ⋯ Poona, although he did not recall any exposure to reptiles. He was treated successfully with intravenous ceftriaxone without any subsequent complications. This case implies that NTS bacteremia can occur in immunocompetent adults, and the diagnosis may be challenging since there may be no clear exposure or focal physical signs.
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Objective The revised Standards of Medical Care in Diabetes-2018 recommend a less-intensive HbA1c target for elderly individuals than for younger ones. This study aimed to investigate the development and progression of chronic kidney disease (CKD) according to HbA1c levels separately for elderly and middle-aged individuals in a general Japanese population. Methods This was a retrospective cohort study using health checkup data in Iki City, Japan. ⋯ No significant differences in the effects of HbA1c level on the development or progression of CKD were observed between elderly and middle-aged individuals (p>0.3 for interaction). Conclusion The risks of the development and progression of CKD increased from HbA1c levels of 7% in a general Japanese population. Similar associations were observed for both elderly and middle-aged individuals.
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Objective Acute abdominal pain (AAP) of diverse etiology is a common chief complaint of patients who present to the emergency department (ED). AAP may pose a diagnostic challenge to physicians in training. We aimed to evaluate whether or not resident trainee doctors examine patients presenting with AAP in a Japanese acute-care hospital following Kendall's diagnostic algorithm. ⋯ Abdominal ultrasonography is one of the recommended examinations for patients with signs of peritoneal irritation, shock or toxic appearance, right lower-quadrant pain, and left upper- or lower-quadrant pain, but the rates were relatively low at 25.0%, 34.4%, 31.8%, and 26.7%, respectively. Conclusion Abdominal ultrasonography required by Kendall's diagnostic algorithm was not performed appropriately in patients with symptoms and signs of peritonitis, shock or toxic appearance, right lower-quadrant pain, and left upper- or lower-quadrant pain or in female patients by resident trainees. Our findings underscore the importance of providing resident doctors with focused training concerning ultrasonography by attending physicians.