Internal medicine
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We present a case of bilateral cheiro-oral syndrome with paresthesia in the right toes following pontine infarction. The causative lesions were situated in the bilateral paramedian pontine tegmentum (PPM). ⋯ The responsible lesion of complete bilateral cheiro-oral syndrome showing paresthesia in the hand and mouth region on both sides is in the bilateral PPM, while that of incomplete bilateral cheiro-oral syndrome showing paresthesia around both sides of the mouth and in the unilateral hand is in the PPM contralateral to the hand with paresthesia. Bilateral cheiro-oral sensory impairment can occur only in the brainstem.
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Case Reports Comparative Study
Pulmonary manifestations in cerebrotendinous xanthomatosis.
We investigated five cases with cerebrotendinous xanthomatosis (CTX) with particular reference to biochemical and pathological pulmonary disorders. To date, few reports discuss the pathophysiology of pulmonary disorders of CTX patients. This study is the first investigation of such pulmonary disorders. ⋯ Biochemically, cholestanol was found to be increased in the BAL fluid as well as in serum. Pathological findings of transbronchial lung biopsy (TBLB) samples disclosed foamy macrophages and small granulomas in alveolar septa. In conclusion, the lung was apparently involved in CTX, and the lesions were characterized with the accumulation of foamy and giant cells with a high concentration of cholestanol, which likely results in the formation of foreign body granulomas.
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Spontaneous pneumomediastinum (SPM) is an uncommon disorder. The mechanism of spontaneous pneumomediastinum has been speculated as follows: when air pressure in the alveoli rapidly increases, free air leaks from ruptured alveoli and accumulates along the bronchovascular tissue sheath, and then free air moves toward the mediastinum. In this case report of a 19-year-old man of SPM, a chest computed tomography (CT) revealed air in a tubular shape along the pulmonary artery in the apico-posterior segment of the left lung field. This is the first case in which the process of SPM could be identified by chest CT.
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Comparative Study
Influence of erythropoietin treatment on hemoglobin A1c levels in patients with chronic renal failure on hemodialysis.
We studied the influence of erythropoietin (EPO) treatment on hemoglobin A1c (HbA1c) levels under conditions which eliminate the effect of changes in the blood glucose concentration. HbA1c levels, blood glucose, hematocrit (Hct) and reticulocyte counts were serially measured every two weeks after starting or stopping EPO administration in 15 non-diabetic hemodialysis patients. ⋯ The changes in HbA1c during the 2-week period correlated inversely with both the changes in Hct during the same 2 weeks and the reticulocyte counts at that time. We concluded that the change in Hct should be kept in mind when the HbA1c level is evaluated in EPO-treated patients and a formula should be proposed to correct HbA1c levels based on the change in Hct or the reticulocyte count.
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Case Reports
Isolated partial growth hormone deficient short stature with syringomyelia not associated with birth injury.
A 59-year-old female with 20-year history of slowly progressing muscle atrophy and sensory disturbance of upper extremities showed short stature, scoliosis, hunger type of sensory dissociation of the upper extremities and pyramidal tract sign of the lower extremities. Magnetic resonance imaging (MRI) clarified hypoplasia of the anterior pituitary lobe, Arnold-Chiari malformation and cervical syringomyelia. Insulin and arginine stimulating tests revealed partial type of isolated growth hormone (GH) deficiency but GH gene analysis detected no defects of GH genes. It was considered to be a rare case of non-hereditary hypopituitarism with Chiari malformation and syringomyelia not associated with perinatal injury, namely a midline anomaly syndrome.