Cases reported "Reflex, Abnormal"

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1/4. Hormonal and cardiovascular reflex assessment in a female patient with pure autonomic failure.

    We report the case of a 72-year-old female with pure autonomic failure, a rare entity, whose diagnosis of autonomic dysfunction was determined with a series of complementary tests. For approximately 2 years, the patient has been experiencing dizziness and a tendency to fall, a significant weight loss, generalized weakness, dysphagia, intestinal constipation, blurred vision, dry mouth, and changes in her voice. She underwent clinical assessment and laboratory tests (biochemical tests, chest X-ray, digestive endoscopy, colonoscopy, chest computed tomography, abdomen and pelvis computed tomography, abdominal ultrasound, and ambulatory blood pressure monitoring). Measurements of catecholamine and plasmatic renin activity were performed at rest and after physical exercise. Finally the patient underwent physiological and pharmacological autonomic tests that better diagnosed dysautonomia.
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2/4. vincristine-induced neuropathy as the initial presentation of charcot-marie-tooth disease in acute lymphoblastic leukemia: a Pediatric Oncology Group study.

    PURPOSE: After profound peripheral neurotoxicity during induction chemotherapy for acute lymphoblastic leukemia (ALL) in the index patient with Charcot-Marie-Tooth hereditary neuropathy (CMT), study coordinators of the Pediatric Oncology Group (POG) front-line ALL protocols reviewed patient registrations to identify any other patients with possible CMT. The goal was to provide preliminary information about patients with undiagnosed CMT who develop ALL. patients AND methods: Five children with ALL who were enrolled in POG B-precursor or T-cell ALL protocols from 1994 to 1999 subsequently were determined to have CMT hereditary neuropathy. Their clinical presentations and treatment records were reviewed in detail. Records of all patients entered on POG 9201 (lesser-risk ALL) were reviewed to identify all cases of significant vincristine toxicity noted in the first 6 months of treatment. RESULTS: The five identified patients all had substantial peripheral neurotoxicity that required alteration in treatment and/or orthopedic/physical therapy evaluation and follow-up. The POG 9201 review identified 25 of 686 patients (3.6%) with significant peripheral neuropathy. Three of 25 were diagnosed with CMT; the others have had no testing reported. CONCLUSIONS: A family history of CMT or other peripheral neuropathy should be sought at the time of diagnosis of ALL. Testing for CMT should be considered in any child with substantial vincristine-induced peripheral neurotoxicity. Treatment of such patients must be individualized. Testing of all patients with significant peripheral neuropathy would be necessary to determine the percentage of such neuropathy explained by underlying CMT.
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3/4. Toxic effects of epidural analgesia with ropivacaine 0.2% in a diabetic patient.

    A 51-year-old ASA physical status II, non-insulin-dependent diabetic male patient manifested lower limb nerve injury after receiving postoperative epidural analgesia with ropivacaine 0.2%. The case is presented, including a discussion of the relation between local anesthetic toxicity and diabetic neuropathy.
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4/4. Management of severe feeding dysfunction in children with fetal alcohol syndrome.

    In the past three years, we have seen three children who required prolonged inpatient admissions for severe feeding dysfunction resulting in nutritional and growth problems. The pregnancies were significant for severe maternal alcohol abuse during the entire pregnancy. All of the subjects had multiple physical findings and dysmorphic features seen in children with mild to severe fetal alcohol syndrome. Feeding evaluations showed significant delays in oral motor development. Specific oral motor dysfunction was not a commonality and did not appear to be a major determinant in delays in feeding development. Continued assessment revealed that the babies had limited suck patterns and were consistently noted to tire easily. Each required feeding by either gastrostomy and/or nasogastric feeding for prolonged periods of time. They started initial feeding by mouth at about 8 to 10 months. The times for complete oral feeding were 14, 17 and 18 months.
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