Internal medicine
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We herein report a 79-year-old woman with subacute progressive ataxic sensory neuropathy. The patient's symptoms began with numbness in the lower extremities, which rapidly deteriorated, resulting in gait disturbance and abnormal sensations in the extremities, reaching a peak over a period of approximately two months. Nerve conduction studies revealed pure axonal-type sensory polyneuropathy. ⋯ Nevertheless, after comprehensive evaluations, the conclusive diagnosis was confirmed as ataxic sensory neuropathy with Sjögren's syndrome. Intensive immunotherapy was administered; however, it was ineffective in halting disease progression. Consequently, this case underscores the significance of an early comprehensive diagnosis and prompt immunotherapy for ataxic sensory neuropathy associated with Sjögren's syndrome.
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We herein report a rare case of idiopathic portal hypertension (IPH)-like disease that developed after allogeneic hematopoietic stem cell transplantation (allo-HSCT). A 53-year-old woman who underwent allo-HSCT for acute myeloid leukemia showed portal hypertension with radiological and histopathological findings consistent with IPH, distinct from veno-occlusive disease (VOD) and graft-versus-host disease (GVHD) of the liver. This case highlights the importance of considering IPH-like disease as a potential cause of portal hypertension after allo-HSCT. Awareness of this complication can aid in the early diagnosis and appropriate management of patients post allo-HSCT.
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Cornelia de Lange syndrome (CdLS) is a congenital disorder occasionally associated with congenital portosystemic shunt (CPSS). We herein report a patient with CdLS and CPSS who developed hepatocellular adenomas (HCAs). ⋯ Imaging findings and pathological examination results indicated that the liver tumors were inflammatory HCAs that subsequently shrank following transcatheter arterial embolization (TAE). Patients with CdLS and CPSS are at risk of developing HCAs, and TAE may be an effective management strategy for HCA in these patients.
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A 42-year-old Japanese woman with end-stage renal failure due to hypertension presented with a systolic blood pressure of 160-200 mmHg despite treatment with 4 different antihypertensive agents. The plasma aldosterone concentration (PAC) and plasma renin activity (PRA) were elevated. ⋯ A pathological examination revealed zona glomerulosa hyperplasia but not microaldosteronoma. This report shows that bilateral nephrectomy, not unilateral adrenalectomy, is a potentially effective treatment option for resistant hypertension with an elevated renin-angiotensin-aldosterone system in hemodialysis patients.