Internal medicine
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Birt-Hogg-Dubé syndrome (BHDS) is a rare autosomal dominant genodermatosis that presents as a clinical triad including follicular hamartomas, renal neoplasms and lung cysts associated with an increased risk of pneumothorax. FLCN gene defects have been identified as being responsible for BHDS. ⋯ In her family history, one of the patient's sons exhibited a similar phenotype, without renal neoplasms. Due to the relatively late age of onset of renal neoplasms among variable BHDS phenotypes, follow-up imaging is recommended for the son who has not yet developed renal neoplasms.
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A 69-year-old woman was admitted for further examinations and treatment of chest pain. Emergency cardiac catheterization showed no significant stenosis on coronary angiograms; however, diffuse wall hypokinesis was observed on a left ventriculogram. ⋯ A right ventricular endomyocardial biopsy revealed denaturation and fibrosis of the myocardium under the endocardium, thus suggesting the presence of myocardial ischemia. This case highlights coronary spasms as a cause of heart failure.
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Various unmeasured anions other than lactate appear in the blood of septic patients, including ketones. However, the occurrence of sepsis-induced ketoacidosis without diabetes mellitus has not been reported to date. We herein describe severe ketoacidosis in a patient with septic shock despite the absence of diabetes, alcohol and starvation. ⋯ Sepsis alone may lead to ketoacidosis in patients without diabetes under specific conditions. The accumulation of ketones and other strong anions can occur in cases involving a decreased metabolic function. There may be a pathological condition called septic ketoacidosis.
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A Japanese woman was treated with injectable methylprednisolone and oral prednisolone for dermatomyositis. On admission, her serum was positive for anti-hepatitis C virus (HCV) antibodies, although HCV RNA was undetectable on polymerase chain reaction. ⋯ The serum alanine aminotransferase and HCV RNA levels subsequently increased in a similar manner to that observed after the first course of therapy. Liver enzymes and the viral load should be monitored in anti-HCV-positive patients receiving immunosuppressives, even if serum HCV RNA is negative.
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A 47-year-old woman with idiopathic pulmonary arterial hypertension (IPAH) was referred to our hospital for treatment of an ovarian tumor. Although chest contrast-enhanced CT scans obtained on admission revealed pulmonary arterial thrombosis, she was diagnosed with IPAH with central pulmonary thrombosis based on a normal perfusion lung scan. ⋯ Gynecological surgery was performed under general anesthesia without any perioperative complications. Providing careful intensive management of patients with severe PAH can reduce the perioperative risks of non-cardiac and non-obstetric surgery.