No abstract available.
Child ; Child Health* ; Child* ; Humans

Photoastigmatic refractive keratectomy (PARK) using VISX 20/20 B excimer laser with version 4.01 software was performed in 205 consecutive eyes of compound myopic astigmatism to assess the efficacy, predictability, and safety of excimer laser treatment of compound myopic astigmatism between September 1994 and June 1996. The mean preoperative manifest spherical equivalent refraction was -6.95+/-2.52D (range -1.13 to -15D) and the mean preoperative manifest cylindrical refraction was -1.25+/-0.76D (range -0.5 to -4.50D). The patients were followed from 6 months to 1 year (mean 9.19 months). At 1 year, 82(75.2%) of all 109 eyes receiving PARK were within 1D of plano refraction, and 88(80.7%) of all 109 eyes achieved uncorrected visual acuity of 20/30 or better. Postoperative refractions were stable after 3 months without significant early overcorrection. Mean postoperative astigmatism was -0.29+/-0.40D at 6 months and -0.24+/-0.42D at 12 months. Overall improvement of astigmatism was 75% at 6 months and 82% at 12 months by vector analysis using Alpins` method. In conclusion, PARK using VISX 20/20 B VisionKeyTM excimer laser with version 4.01 software appears to be effective in the treatment of compound myopic astigmatism with a relatively high degree of accuracy and safety. The predictability and stability of the postoperative refraction during the first 12 months seem to be quite reliable.
Astigmatism ; Humans ; Lasers, Excimer* ; Visual Acuity

In one case of cancer-associated Lambert-Eaton myasthenic syndrome (LEMS), treatment with cytotoxic chemotherapy and radiotherapy resulted in remission of both cancer and neuromuscular disorder. Serial repetitive nerve stimulation (RNS) findings showed that the typical RNS features of LEMS returned to normal in the orders of dramatic postexercise facilitation, an incremental response in high rate stimulation, and a decremental response in low rate stimulation with clinical improvement.
Drug Therapy* ; Lambert-Eaton Myasthenic Syndrome* ; Radiotherapy* ; Small Cell Lung Carcinoma*

PURPOSE: Maturity-onset diabetes of the young(MODY) is a subtype of type 2 diabetes defined by autosomal dominant mode of inheritance, onset of diabetes usually before the age of 25 yrs, and a primary defect in the function of the beta cells of the pancreas. MODY3 is known as the most common form and is caused by mutations in hepatocyte nuclear factor(HNF)-1alpha. We examined the prevalence of MODY3 in children with type 2 diabetes mellitus(DM). METHODS: Children with type 2 DM(N=17) and their family members with type 2 DM(N=5) were enrolled. Inclusion criteria for the children were fasting C-peptide and postprandial C-peptide more than 1.0 ng/mL and 1.5 ng/mL respectively, familial type 2 DM in at least two generations, and body mass index(BMI)(kg/m(2)) less than 95th percentile. Genomic DNA was extracted from blood samples. We analyzed HNF-1alpha for mutation by DNA microarray method and direct sequencing. RESULTS: We found one case with a mutation of the promoter region of HNF-1alpha(5'-ctaGGCTAGTGGGGTTTTGCGGGGGCAGTGGGTGCAAGG-3') in one child's family member among 22 children and adult subjects with type 2 DM. CONCLUSION: Although we found a mutation of HNF-1alpha in an adult family member with type 2 DM, we did not find this mutation in a child with type 2 DM. The further investigation of MODY in children, including other types, is required.
Adult ; C-Peptide ; Child* ; Diabetes Mellitus, Type 2* ; DNA ; Family Characteristics ; Fasting ; Hepatocyte Nuclear Factor 1-alpha ; Hepatocytes ; Humans ; Oligonucleotide Array Sequence Analysis ; Pancreas ; Prevalence* ; Promoter Regions, Genetic ; Wills

A discription is given of 24 patients with the benigen focal amyotrophy; Nineteen had upper-limb and five had lower-limb involvement. The characteristic clinca! Features were insidious onset in the second and third decades, male predominance. Sporadic occurance, wasting and weakness confined to one limb, relatively good prognosis and absence of involvement of the cranial nerves, cerebrum, brainstem, and sensory syetem. The nerve conduction studies, electromyographic pattems, somatosensory evoked potential studies, and CTMM or MR findings suggested anterior horn cell disorders. In nine cases we were able to demonstrate focal atrophy in the lower cervical cord limited to the ipsilateral anterior horn region, and two cases segmental cord atrophy in the lower cervical cord in MR or CTMM studies. The benign focal amyotrophy, for which no causes been described, is benign and self-limiting, unlike most motor neuron diseases.
Animals ; Anterior Horn Cells ; Atrophy ; Brain Stem ; Cerebrum ; Cranial Nerves ; Evoked Potentials, Somatosensory ; Extremities ; Horns ; Humans ; Male ; Motor Neuron Disease ; Neural Conduction ; Prognosis

A discription is given of 24 patients with the benigen focal amyotrophy; Nineteen had upper-limb and five had lower-limb involvement. The characteristic clinca! Features were insidious onset in the second and third decades, male predominance. Sporadic occurance, wasting and weakness confined to one limb, relatively good prognosis and absence of involvement of the cranial nerves, cerebrum, brainstem, and sensory syetem. The nerve conduction studies, electromyographic pattems, somatosensory evoked potential studies, and CTMM or MR findings suggested anterior horn cell disorders. In nine cases we were able to demonstrate focal atrophy in the lower cervical cord limited to the ipsilateral anterior horn region, and two cases segmental cord atrophy in the lower cervical cord in MR or CTMM studies. The benign focal amyotrophy, for which no causes been described, is benign and self-limiting, unlike most motor neuron diseases.
Animals ; Anterior Horn Cells ; Atrophy ; Brain Stem ; Cerebrum ; Cranial Nerves ; Evoked Potentials, Somatosensory ; Extremities ; Horns ; Humans ; Male ; Motor Neuron Disease ; Neural Conduction ; Prognosis

There have been few reports about extrusion of endodontic obturation materials into the maxillary sinus and inducing fungal sinusitis. Endodontic materials and fungus balls both are seen as high attenuation in a CT scan so may be overlooked. We report such a case in which the surgeon and radiologist had missed the foreign materials on preoperative CT scans, and recognized the filling defect on the alveolar bone and a foreign body in the nasal cavity postoperatively.
Foreign Bodies ; Fungi ; Gutta-Percha* ; Maxillary Sinus ; Nasal Cavity* ; Sinusitis* ; Tomography, X-Ray Computed

There have been few reports about extrusion of endodontic obturation materials into the maxillary sinus and inducing fungal sinusitis. Endodontic materials and fungus balls both are seen as high attenuation in a CT scan so may be overlooked. We report such a case in which the surgeon and radiologist had missed the foreign materials on preoperative CT scans, and recognized the filling defect on the alveolar bone and a foreign body in the nasal cavity postoperatively.
Foreign Bodies ; Fungi ; Gutta-Percha* ; Maxillary Sinus ; Nasal Cavity* ; Sinusitis* ; Tomography, X-Ray Computed

BACKGROUND: Charcot-Marie-Tooth (CMT) disease is a clinically and genetically heterogeneous disorder. Connexin32 (Cx32) gene mutations on Xq13.1 cause the X-linked form of CMT disease, and PMP22 gene duplication on 17p11.2-p12 causes CMT1A. The aim of the present study is to determine the clinical and electrophysiological characteristics between X-linked CMT patients with Cx32 missense mutations and CMT1A patients with PMP22 duplications. METHODS: We screened for 17p11.2-p12 duplication, and for point mutations in Cx32 genes of 48 Korean CMT families. Both neurological examination and nerve conduction studies were performed in all patients. RESULTS: Frequency of CMTX (6.3%) in our study was similar to Japanese, and was lower than those in European peoples. CMTX patients displayed no man-to-man transmission, and had cranial nerve involvement. CMTX patients showed more wide range of motor and sensory nerve conduction velocities than CMT1A patients. We found one family with axonal neuropathy and two families with demyelinating neuropathy in CMTX patients. CONCLUSIONS: Our findings suggest that mutations in Cx32 are probably less frequent in Asian CMT patients than European patients, and CMTX neuropathy is intermediary between CMT1 and CMT2. In addition, inheritance pattern and cranial nerve involvement are useful in differentiating CMTX from CMT1A with duplication.
Asian Continental Ancestry Group ; Axons ; Cranial Nerves ; Gene Duplication ; Humans ; Inheritance Patterns ; Mutation, Missense* ; Neural Conduction ; Neurologic Examination ; Point Mutation

Pheochromocytoma during pregnancy is very rarely discovered, and this condition is life threatening to both mother and fetus. Maternal and fetal survival depend a lot on an early diagnosis, a correct medical therapy and a correct timing of delivery and surgery. We describe a case of pheochromocytoma during pregnancy. A patient was transferred to our hospital during gestational week 24 with severe hypertension, generalized edema. Pheochromocytoma caused by a left adrenal mass was diagnosed. The patient was treated with titrated dose of phenoxybenzamine, propranolol, and intravenous nicardipine, but intermittent severe hypertension was developed. At 33 weeks gestation, fetal distress was developed, and emergent cesarean section was done. Two weeks after delivery, she underwent a successful left adrenalectomy. Two months after surgery, all antihypertensive medications were discontinued and her blood pressure have remained normal range.
Adrenalectomy ; Blood Pressure ; Cesarean Section ; Early Diagnosis ; Edema ; Female ; Fetal Distress ; Fetus ; Humans ; Hypertension ; Mothers ; Nicardipine ; Phenoxybenzamine ; Pheochromocytoma* ; Pregnancy* ; Propranolol ; Reference Values