Internal medicine
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A 72-year-old woman was referred for the examination of wall thickening of the bile duct and a polyp-like lesion on the gallbladder. The bile duct and gallbladder lesions were observed to be continuous on abdominal ultrasonography. ⋯ The pathological examination of the resected gallbladder also showed hyperplasia of the lymphoid follicles. We diagnosed the patient with lymphoid hyperplasia of the gallbladder extending to the bile duct.
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Case Reports
Schnitzler Syndrome Presenting as a Fever of Unknown Origin with Elevated Alkaline Phosphatase Levels.
Schnitzler syndrome (SchS) is a rare, acquired, autoinflammatory disease that is sometimes associated with a fever of unknown origin (FUO). Elevated alkaline phosphatase (ALP) stemming from abnormal bone remodeling is a characteristic laboratory finding of SchS and is included in the diagnostic criteria. However, its utility as a clue to the diagnosis of SchS has been under-emphasized. We herein report a case of SchS presenting with a FUO and highly elevated ALP concentration, which led to repeated, unnecessary liver biopsies.
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Case Reports
A Case of Demyelinating Neuropathy with Markedly Elevated Serum IgG4 Levels and Anti-Contactin 1 IgG4 Antibody.
We herein report a 77-year-old man with a 4-month history of progressive gait and sensory disturbances of the extremities. A nerve conduction study indicated demyelinating polyneuropathy. ⋯ Treatment with steroids resulted in an amelioration of functional status, improvement of nerve conduction parameters, decreased serum IgG4 levels, and negative conversion of anti-contactin 1 antibody. Further studies are needed to clarify the significance of IgG4-positive plasma cell infiltration in anti-contactin 1 antibody-positive neuropathies.
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Becker muscular dystrophy (BMD) is an X-linked neuromuscular disease characterized by progressive muscle weakness that currently has no cure. Immune-mediated necrotizing myopathy (IMNM) is a type of autoimmune inflammatory myopathy characterized by proximal muscle weakness that is treated with immunosuppressive therapy. ⋯ Oral glucocorticoid and methotrexate treatment partially improved the muscle weakness with decreased levels of serum creatine kinase. An accurate diagnosis is important for therapeutic decisions in these complicated cases.
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Objective Muscle atrophy is observed in a subset of patients with chronic inflammatory demyelinating polyneuropathy (CIDP). Its manifestation is associated with a poor functional prognosis and poor response to immunomodulatory therapies. We evaluated muscle atrophy in patients with CIDP using a bioelectrical impedance analysis (BIA). ⋯ A comparison of the outcomes from these two measures showed a good correlation between their muscle atrophy ratings (p<0.05). Conclusion We found that a BIA and muscle CT provided muscle atrophy assessments of equivalent accuracy. Therefore, a BIA can be a simple alternative to muscle CT that is suitable for regular use in daily clinical practice as a reliable tool for assessing muscle atrophy in patients with CIDP.