No abstract available.
Paralysis*

OBJECTIVES: The aims of this study are 1) to determine if GAST is a better indicator in predicting ovarian response to COH compared with patient's age or basal FSH level and 2) to evaluate its role in detecting abnormal ovarian response. DESIGN : Prospective study in 118 patients undergoing IVF-ET using GnRH-a short protocol during May-September 1995. MATERIALS AND METHODS: After blood sampling for basal FSH and estradiol (E2) on cycle day two, 0.5 ml (0.525 mg) GnRH agonist (Suprefact, Hoechst) was injected subcutaneously. Serum E2 was measured 24 hours later. Initial E2 difference (deltaE2) was defined as the change in E2 on day 3 over the baseline day 2 value. Sixteen patients with ovarian cyst or single ovary or incorrect blood collection time were excluded from the analysis. The patients were divided into three groups by deltaE2; group A (n=30):deltaE2<40 pg/ml, group B (n=52): 40 pg/ml< or =deltaE2<100 pg/ml, group C (n=20): deltaE2< or =100 pg/ml. COH was done by GnRH agonist/HMG/hCG and IVF-EF was followed. Ratio of E2 on day of hCG injection over the number of ampules of gonadotropins used (E2hCGday/Amp) was regarded as ovarian responsiveness. Poor ovarian response and overstimulation were defined as E2 hCGday less than 600 pg/ml and greater than 5000 pg/ml, respectively. RESULTS: Mean age (+/-SEM) in group A, B and C were 33.7+/-0.8*, 31.5 +/-0.6 and 30.6+/-0.5*, respectively (*: p<0.05). Mean basal FSH level of group A (11.1+/-1.1 mIU/ml) was significantly higher than those of B (7.4+/- 0.2 mIU/ml) and C (6.8+/-0.4 mIU/ml) 0<0.001). Mean E2hCGday of group A was significantly lower than those of group B or C, i.e., 1402.1+/-187.7 pg/ml, 3153.2+/- 240.0 pg/ml, 4078.8+/-306.4 pg/ml respectively (p<0.0001). The number of ampules of gonadotropins used in group A was significantly greater than those in group B or C: 38.6+/-2.3, 24.2+/-1.1 and 18.5+/-1.0 (p<0.0001). The number of oocytes retrieved in group A was significantly smaller than those in group B or C: 6.4+/-1.1, 15.5+/-1.1 and 18.6+/-1.6, respectively (p<0.0001). By stepwise multiple regression, only deltaE2 showed a significant correlation (r=0.68, p<0.0001) with E2HCGday/Amp, while age or basal FSH level were not significant. Likewise, only deltaE2 correlated significantly with the number of oocytes retrieved (r=0.57, p<0.001). All four patients whose COH was canceled due to poor ovarian response belonged to group A only (Fisher's exact test, p<0.01). Whereas none of 30 patients in group A (0%) had overstimulation, 14 patients among 72 patients (19.4%) in group B and C had overstimulation (Fisher's exact test, p<0.01). CONCLUSIONS: These data suggest that initial E2 difference after GAST may be a better prognostic indicator of ovarian response to COH than age or basal FSH level. Since initial E2 difference demonstrates significant association with abnormal ovarian response such as poor ovarian response necessitating cycle cancellation or overstimulation, GAST may be helpful in monitoring and consultation of patients during COH in IVF-ET cycle.
Estradiol ; Female ; Gonadotropin-Releasing Hormone* ; Gonadotropins ; Humans ; Oocytes ; Ovarian Cysts ; Ovary ; Ovulation Induction* ; Prospective Studies

Rhizomelic chondrodysplasia punctata(RCDP) is a rare autosomal recessive disorder clinically characterized by symmetrical shortening of the proximal limbs, contractures of joints, a typical dysmorphic face, cataracts, and itchyosis. Patients with RCDP can be subdivided into three subgroups based on biochemical analysis and complementation studies. RCDP type I results from mutations in the PEX7 gene encoding the peroxisomal targeting signal type II(PST2) receptors and presents with both a defect in plasmalogen biosynthesis and phytanic acid oxidation. RCDP type II is deficient in the activity of dihydroxyacetonephosphate acyltransferase(DHAP-AT). RCDP type III is deficient in alkyl-dihydroxyacetonephosphate synthase(alkyl-DHAP). We report a case of RCDP type I which was confirmed with biochemical study, fibroblast culture, and gene study.
Cataract ; Chondrodysplasia Punctata, Rhizomelic* ; Complement System Proteins ; Contracture ; Extremities ; Fibroblasts ; Humans ; Joints ; Phytanic Acid

BACKGROUND: Montelukast is a type 1 cysteinyl-leukotrienes receptor antagonist that has been widely used in allergic disease. However, the effect of combination of leukotriene receptor antagonist and antihistamine is controversial. The aim of this study was to compare the effect of combination treatment of montelukast and antihistamine, fexofenadine, over antihistamine alone in patients with allergic rhinitis (AR). SUBJECTS AND METHODS: Retrospective chart review of 60 patients with AR was undertaken. Patients were classified into combination group (montelukast and fexofenadine, n=28) and antihistamine only group (fexofenadine, n=32) according to treatment modalities. Questionnaire survey was performed and allergic symptoms (VAS scale, 5pointscale), and SNOT (sinonasal outcome test)-20 score were obtained before and after the treatment. RESULTS: Mean follow-up duration was 6.7+/-4.6weeks. There was no significant difference in demographic data between two groups. Allergic symptoms and SNOT-20 score(nasal, QOL domain) were improved significantly in both groups after the treatment (all p < 0.001). Combination treatment group showed better improvement in nasal obstruction than antihistamine treatment group(p = 0.03). However, there were no significant differences in other allergic symptoms (rhinorrhea and sneezing) and SNOT-20 between two groups (all p > 0.05, respectively). CONCLUSION: A combination treatment of montelukast and fexofenadine showed more efficacies in nasal obstruction than single fexofenadine treatment in patients with AR. Therefore, montelukast could be used effectively with antihistamine in patients with AR complaining nasal congestion.
Acetates ; Estrogens, Conjugated (USP) ; Follow-Up Studies ; Humans ; Nasal Obstruction ; Quinolines ; Receptors, Leukotriene ; Retrospective Studies ; Rhinitis* ; Rhinitis, Allergic, Perennial ; Terfenadine ; Surveys and Questionnaires

PURPOSE: To evaluate the correlation between colon transit time (CTT) test value and initial maintenance dose of polyethylene glycol (PEG) 4000 or lactulose. METHODS: Of 415 children with chronic functional constipation, 190 were enrolled based on exclusion criteria using the CTT test, defecation diary, and clinical chart. The CTT test was performed with prior disimpaction. The laxative dose for maintenance was determined on the basis of the defecation diary and clinical chart. The Shapiro-Wilk test and Pearson's and Spearman's correlations were used for statistical analysis. RESULTS: The overall group median value and interquartile range of the CTT test was 43.8 (31.8) hours. The average PEG 4000 dose for maintenance in the overall group was 0.68±0.18 g/kg/d; according to age, the dose was 0.73±0.16 g/kg/d (<8 years), 0.53±0.12 g/kg/d (8 to <12 years), and 0.36±0.05 g/kg/d (12 to 15 years). The dose of lactulose was 1.99±0.43 mL/kg/d (<8 years) or 1.26±0.25 mL/kg/d (8 to <12 years). There was no significant correlation between CTT test value and initial dose of laxative, irrespective of the subgroup (encopresis, abnormal CTT test subtype) for either laxative. Even in the largest group (overall, n=109, younger than 8 years and on PEG 4000), the correlation was weak (Pearson's correlation coefficient [R]=0.268, p=0.005). Within the abnormal transit group, subgroup (n=73, younger than 8 years and on PEG 4000) correlation was weak (R=0.267, p=0.022). CONCLUSION: CTT test value cannot predict the initial maintenance dose of PEG 4000 or lactulose with linear correlation.
Child* ; Colon* ; Constipation* ; Defecation ; Humans ; Lactulose ; Laxatives ; Polyethylene Glycols

It is thought that horizontal canal benign paroxysmal positional vertigo (BPPV) is the most common cause of apogeotropic direction-changing positional nystagmus (DCPN). But there are many reports about cerebellar or brainstem lesions as the cause of apogeotropic DCPN. We also report a 72-year-old male patient who showed apogeotropic DCPN, but was proven to have a pontine infarction. The patients complained of disequilibrium which has lasted for 3-4 years and aggravated recently. The symptom was present only when he stood up, and was absent as soon as he sat down. He was not able to successfully perform the Romberg test and tandem gait on physical examination. Vestibular function test revealed apogeotropic DCPN without spontaneous nystagmus. Rotation chair test and caloric test results were all within normal limit. On the brain magnetic resonance imaging, newly detected infarction in the left basal ganglia, pons and right parietal lobe was found. Although horizontal canal BPPV is the most common cause of apogeotropic DCPN, we should be aware that there can be patients with central origin DCPN. In this report, we present the detailed history of this patient and tried to point out the clues to suspect central lesion in patients with apogeotropic DCPN.
Aged ; Basal Ganglia ; Brain ; Brain Stem ; Caloric Tests ; Gait ; Humans ; Infarction ; Magnetic Resonance Imaging ; Male ; Nystagmus, Physiologic ; Parietal Lobe ; Physical Examination ; Pons ; Vertigo ; Vestibular Function Tests

BACKGROUND AND OBJECTIVES: Meniere's disease (MD) is a clinical cluster of common symptoms by various causes rather than a single disease entity. Many causes such as autoimmune, allergy, vascular insufficiency have been thought to be related with Meniere's disease. We assumed that different pathologic mechanisms have contribution in each gender. With this premise, we compared clinical characteristics between male and female patients to determine if there is any difference indicating heterogeneous underlying pathology. MATERIALS AND METHODS: We reviewed medical records of 61 patients (43 female, 18 male) who were diagnosed as unilateral definite MD and underwent vestibular function test and audiologic evaluation (more than two times of pure tone audiometry during the follow-up period) from October 2005 to December 2011. RESULTS: The average duration of dizziness in females was longer than in males. In the worst ipsilateral pure tone audiometry, low frequency thresholds were lower in females than in males. Female had lesser hearing difference at all frequencies between the sides and showed more hearing fluctuation than male. There was no significant difference between male and female in the vestibular function test. CONCLUSION: These results are insufficient to suggest that the pathogenesis of MD differs between the genders. However, some differences between the genders prompt a need for future studies involving more patients.
Audiometry ; Dizziness ; Female ; Follow-Up Studies ; Hearing ; Humans ; Hypersensitivity ; Male ; Medical Records ; Meniere Disease ; Vestibular Function Tests

BACKGROUND AND OBJECTIVES: Rectified vestibular evoked myogenic potential (rVEMP) is a relatively new method that simultaneously measures the muscle contraction power during VEMP recording and corrects the difference of contraction power afterwards. Several studies showed rVEMP is more reliable than non-rectified VEMP (nVEMP). However, those studies evaluated usefulness of rVEMP in patients with normal vestibular function. Thus, we evaluate the effect of rectification to predict lesion side in unilateral vestibulopathy patients. MATERIALS AND METHODS: One-hundred nine acute unilateral vestibulopathy patients whom VEMP were performed in were included retrospectively. We regarded hearing loss side as lesion side in sudden hearing loss (n=33), meniere's disease (n=29) and in vestibular neuritis (n=45), the side of positive head thrust test with canal paresis >30% was regarded as a lesion side. We excluded bilateral vestibulopathy. The inter-aural amplitude difference (IAD) ratio was calculated by the nVEMP and rVEMP. RESULTS: Mismatch rate between nVEMP and rVEMP was 36.61%, match rate was 49.54%, opposition rate was 13.76%. rVEMP predicted lesion side on 15 patients of mismatch group correctly, while nVEMP predicted lesion side on 25 patients of mismatch group. There was no significant difference in IAD ratio between nVEMP and rVEMP in patients who showed lesion side weakness on both nVEMP and rVEMP. But, the younger the patient was, the more chance of mismatch was significantly (p=0.03). CONCLUSION: There was no more corrective role in determining lesion side by rectification in unilateral vestibulopathy. Thus rVEMP might not be helpful for predicting lesion side in unilateral vestibulopathy.
Head ; Hearing Loss ; Hearing Loss, Sudden ; Humans ; Meniere Disease ; Methods ; Muscle Contraction ; Paresis ; Retrospective Studies ; Vestibular Neuronitis

BACKGROUND AND OBJECTIVES: Determination of the lesion side based on the direction of the nystagmus could result in confusions to the clinicians due to mismatch between the vestibular function tests and also between vestibular and audiologic features. To minimize these mistakes, we elucidated the clinical manifestation and vestibular function test results in cases with recovery spontaneous nystagmus (rSN). SUBJECTS AND METHODS: Patients who visited ENT clinic of tertiary referral hospital for acute onset continuous vertigo from January 2008 to December 2011 were enrolled. In these patients, we assessed onset time of vertigo, time point of paralytic spontaneous nystagmus (SN) and time point of rSN. At each time point of SN, vestibular function tests and hearing function tests were performed. RESULTS: We confirmed the rSN among patients with unilateral vestibulopathy and demonstrated that high gain of the rotatory chair test (slow harmonic acceleration) and/or mismatch of the SN direction and contralateral caloric weakness could indicate the recovery state of patients and nystagmus observed in this stage is recovery phase nystagmus. CONCLUSIONS: In acute vestibulopathy patients, recovery phase nystagmus was observed and on this stage of disease vestibular function tests shows several features that could predict recovery state.
Hearing ; Humans ; Tertiary Care Centers ; Vertigo ; Vestibular Function Tests

Menkes disease (also known as kinky hair disease) is an X-linked recessive neurodegenerative disorder caused by diverse mutations in a copper-transport gene, ATP7A. Affected patients are characterized by kinky hair, hypotonia, and generalized myoclonic seizures. Here, we report a case of Menkes disease in which the patient presented with progressive hypotonia and intractable seizures. A 4-month-old male infant visited our pediatric clinic for focal seizures with blinking eyes. He was generally hypotonic and suffered from malnutrition. The focal seizures became more frequent, and the patient became intractable to anti-seizure medications. An electroencephalogram (EEG) indicated diffuse cerebral dysfunction with focal seizure, and a brain magnetic resonance imaging (MRI) showed tortuous and ectatic intracranial arteries, as well as several ischemic lesions. A genetic analysis was performed, and a c.2473_2474del (p.Leu825fsX1) of the ATP7A gene was detected.
Arteries ; Blinking ; Brain ; Electroencephalography ; Epilepsy* ; Hair ; Humans ; Infant ; Magnetic Resonance Imaging ; Male ; Malnutrition ; Menkes Kinky Hair Syndrome* ; Muscle Hypotonia ; Neurodegenerative Diseases ; Seizures