Internal medicine
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Case Reports
An Autopsy Case of Mixed Connective Tissue Disease Complicated by Thrombotic Thrombocytopenic Purpura.
We herein report a patient with mixed connective tissue disease (MCTD) who had been stable for years but suddenly developed thrombotic thrombocytopenic purpura (TTP). The patient showed a clinical pentad of signs of TTP, low activity of ADAMTS13, and positivity of anti-ADAMTS13 antibodies. ⋯ An autopsy revealed microthrombi in the brain, heart, kidney, adrenal glands, esophageal submucosa, and bone marrow as well as diffuse alveolar hemorrhaging. Physicians should bear in mind that TTP can occur in MCTD patients regardless of disease activity.
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Case Reports
Successful Introduction of Peritoneal Dialysis in an End-stage Renal Failure Patient with Idiopathic Aplastic Anemia.
A 45-year-old man with idiopathic aplastic anemia required renal replacement therapy (RRT) due to end-stage renal disease (ESRD). We succeeded in inserting the peritoneal dialysis (PD) catheter under cover of frequent red blood cell and platelet infusions because of severe pancytopenia. During the one-year period after starting PD using an ultraviolet-ray sterilization device, he developed severe leukopenia but no PD-related peritonitis or exit site/tunnel infection until he died of pneumonia. This case suggests that PD might be a suitable choice as RRT in ESRD patients with aplastic anemia, even in those with severe pancytopenia.
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Objective Pneumonia develops in bedridden patients, even in those receiving oral care, and malnutrition is associated with the development of pneumonia. We examined the effects of nutritional treatment on the prevention of pneumonia. Patients and Methods We retrospectively examined the effects of nutritional treatment on the prevention of pneumonia by analyzing the records of bedridden patients (n=68; mean age: 68.0 years) who stayed in a hospital for 2 years or longer. ⋯ The proportions of patients with hypoalbuminemia (<3.5 g/dL) and hypoproteinemia (<6.5 g/dL) at one year after admission were lower than those at admission. The increases in the proportions of patients presenting a reduced frequency of pneumonia were correlated with increases in the proportions of patients presenting increased levels of albumin and/or TP. Conclusion Nutritional treatment may reduce the frequency of pneumonia by improving malnutrition in bedridden patients receiving oral care.
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An 82-year-old woman was admitted to our hospital with heart failure. Coronary angiography revealed one anatomically normal right coronary artery and three left coronary arteries (LCA-1, LSA-2, and LSA-3) separately originating from the right sinus of Valsalva, comprising multiple atherosclerotic lesions. LCA-1 became the obtuse marginal branch after branching off into the septal branches. ⋯ LCA-3 corresponded to the distal part of the anterior descending branch. The patient died 14 days after hospitalization. We describe the rarity of quadriostial origin, the unusual course, and the unusual branching of the coronary arteries.
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Case Reports
A Healthy Family of Familial Hypobetalipoproteinemia Caused by a Protein-truncating Variant in the PCSK9 Gene.
We present the first case of a Japanese patient with familial hypobetalipoproteinemia (FHBL) caused by a protein-truncating variant in the proprotein convertase subtilisin/kexin type 9 (PCSK9) gene. A 34-year-old woman was referred to our hospital due to her low low-density lipoprotein (LDL)-cholesterolemia (34 mg/dL). She did not have any secondary causes of hypobetalipoproteinemia. ⋯ We identified a protein-truncating variant in the PCSK9 gene (c.1090_1091del/p. Pro364ArgfsTer62) among them. None of them exhibited atherosclerotic cardiovascular diseases nor any other complications associated with low LDL cholesterol, including fatty liver, neurocognitive disorders, or cerebral hemorrhaging.