No abstract available.
Child* ; Guillain-Barre Syndrome* ; Humans

No abstract available.
Gonadal Dysgenesis, Mixed*

Pseudohypoaldosteronism (PHA) is rare herediary salt wasting syndrome due to peripheral resistance to aldostrone. PHA type I, subdivided into isolated renal insensitivity to aldosterone of autosomal dominant inheritance and multiple target organ defect of autosomal recessive inheritance, and PHA type II show similar clinical manifestations except hypertension which is limited to type II. PHA type I is charaterized by hyponatremia, hyperkalemia, high plasma aldosterone and renin activity. Variable degrees, completely asymptomatic to severe fatal, of salt loosing manifestationsdehydration, hypotension, failure to thrive, and renal tubular acidosis usually start during infancy. Sodium loss not only from kidney but also from sweat gland, salivary gland and colon may occur in some cases. Recently we experienced two cases of PHA, a 27 days of age male newborn infant with charateristic clinical symptoms and typical laboratory manifestations of PHA, confirm diagnosed as PHA type I of isolated renal defect by pilocarpin iontophoresis showing normal sodium concentration in sweat gland, and his asymptomatic 6 year-old brother with the history of salt loosing symptoms treated until second year of life diagnosed as PHA type I by markedly elevated plasma aldosterone and renin activity. Brief review and related literatures were also presented.
Acidosis, Renal Tubular ; Aldosterone ; Child ; Colon ; Failure to Thrive ; Humans ; Hyperkalemia ; Hypertension ; Hyponatremia ; Hypotension ; Infant, Newborn ; Iontophoresis ; Kidney ; Male* ; Plasma ; Pseudohypoaldosteronism* ; Renin ; Salivary Glands ; Siblings* ; Sodium ; Sweat Glands ; Vascular Resistance ; Wasting Syndrome ; Wills

The authors retrospectively analysed the ultrasonographic findings of 43 cases of surgically confirmed hypertrophic pyloric stenosis and their postoperative findings of sonograms taken at 1 month(n=40) or 3 months( n=5) after pyloromyotomy. In preoperative study, the thickened pyloric muscle was isoechoic or slight hypoechoic relative to liver on the midline longitudinal view and appeared as a "nonuniform acoustic ring" on the transverse view. The results of measurement in the all cases with hypertrophic pyloric stenosis were the pyloric thickness ≥3.8mm, the pyloric diameter ≥14mm, the pyloric channel length ≥16mm, the pyloric muscle volume ≥2.21Cm
Acoustics ; Follow-Up Studies ; Humans ; Liver ; Methods ; Pyloric Stenosis, Hypertrophic ; Reference Values ; Retrospective Studies ; Ultrasonography*

PURPOSE: An anorectal melanoma (AM) is a very rare tumor. However, sufficient data supporting effective surgical options for the disease do not exist. This retrospective review aimed to analyze treatment outcomes for an AM. METHODS: From June 1999 to December 2008, we retrospectively reviewed a prospectively collected consecutive series of 19 patients who had undergone a surgical resection for an AM at a single institute. Surgical method and clinicopathological factors were analyzed. RESULTS: The median age was 61.4 years (range, 46 to79 years). Main symptoms were an anal mass, hematochezia, perianal pain, tenesmus, fecal incontinence, and bowel habit change. The average duration of symptoms before diagnosis was 7.8 months (range, 1 to 36 months). S-100 and HMB-45 were positive in all patients, even in non-melanin pigmentation. There were 12 abdominoperineal resections (APRs) and 7 wide local excisions (WEs). The APR showed longer overall survival when compared with the WE (64.1 months vs. 10.9 months, P < 0.001). No patients who underwent a WE survived more than 13 months. CONCLUSION: A high index of suspicion is necessary to establish the diagnosis for an AM in patients with anal symptoms, and S-100 and HMB-45 can be useful markers for an AM. Even with the small number of cases and the short follow-up, our data suggest that an APR for an AM may provide longer survival than a WE.
Fecal Incontinence ; Follow-Up Studies ; Gastrointestinal Hemorrhage ; Humans ; Melanoma ; Pigmentation ; Prospective Studies ; Retrospective Studies

Gastric volvulus is a rare condition, and it is classified as the organoaxial or mesentericaxial type according to the axis of rotation. We experienced 1 case of pediatric recurrent mesenteroaxial gastric volvulus and we report here the ultrasonographic and CT findings.
Axis, Cervical Vertebra ; Stomach Volvulus*

OBJECTIVE: The aim of this study was to analyze a relation between the recurrence of cervical intraepithelial neoplasia (CIN) and the detection of high-risk human papillomavirus (HPV) DNA after loop electrosurgical excision procedure (LEEP), and to evaluate the clinical efficacy of HPV DNA test by Hybrid Capture Assay as a predictor of the recurrence of CIN after LEEP. METHODS: From January 2000 through March 2003, the charts of 238 women diagnosed as CIN and treated with LEEP were reviewed retrospectively. HPV DNA test (Hybrid Capture System I) for high-risk HPV was performed in all patients before LEEP. They were followed up with Papanicolaou smears and HPV DNA tests at the interval of 3 months during the first 6 months and then at the interval of 6 months. The Chi-square test was used for the statistical analysis. RESULTS: The rate of detection of high-risk HPV declined chronologically after LEEP. A higher recurrence rate was noted in the HPV DNA positive group after LEEP, comparing with the negative group, at 3 months (54.5% in positive vs. 6.5% in negative; p<0.05), at 6 months (61.1% in positive vs. 5.4% in negative; p<0.05), at 12 months (50.0% in positive vs. 14.0% in negative; p<0.05), and at 18 months (50.0% in positive vs. 15.2% in negative; p<0.05). The negative predictive value was high at 3 months (93.5%), at 6 months (94.6%), at 12 months (86.0%), and at 18 months (84.6%). There were significant associations between the recurrence of CIN and positive resection margin except for glandular involvements. CONCLUSION: The detection of high-risk HPV is a risk factor for the presence of CIN after LEEP. HPV DNA test by Hybrid Capture Assay after LEEP may be useful marker for the negative prediction of recurrence of CIN after LEEP.
Cervical Intraepithelial Neoplasia* ; DNA* ; Female ; Human Papillomavirus DNA Tests ; Humans* ; Papanicolaou Test ; Recurrence ; Retrospective Studies ; Risk Factors

BACKGROUND: Patients with obstructive sleep apnea syndrome (OSAS) have some deficits in attention and frontal functions. The pathophysiology of the cognitive dysfunction is still controversial. We investigated the cognitive performances of OSAS patients, and the relationship of cognitive functioning and nocturnal respiratory findings and daytime sleepiness. METHODS: Neuropsychological testing, the Beck Depression Inventory and the Epworth sleepiness scale (ESS) were administered to 35 patients with OSAS and to 31 normal controls. RESULTS: Compared to the controls, OSAS patients had significant impairment on the Korean mini mental state examination (K-MMSE), semantic and phonemic fluency, the time and number of correct on the Korean Stroop color word test, the Digit cancellation test and delayed response on the Seoul verbal learning test (SVLT). ESS was significantly correlated with K-MMSE, trail making A, the time and number of correct on the Korean Stroop color word test, delayed recall of SVLT and Rey figure test and number of fulfilled categories on the Wisconsin card sorting test in OSAS patients. The Apnea-hypopnea index (AHI) was significantly correlated with the copy of the Rey figure test. Arousal index, total time slept with oxygen saturation below 90% and the lowest oxygen saturation were not correlated with any neuropsychological parameters. CONCLUSIONS: Patients with OSAS have cognitive impairment concerning attention, executive function and retrieval of memory. The cognitive impairment may be attributed not to nocturnal hypoxemia, but to subjective daytime sleepiness.
Anoxia* ; Arousal ; Depression ; Executive Function ; Humans ; Memory ; Neuropsychological Tests ; Oxygen ; Polysomnography ; Semantics ; Seoul ; Sleep Apnea Syndromes ; Sleep Apnea, Obstructive* ; Verbal Learning ; Wisconsin

PURPOSE: To evaluate the hemodynamic change by portal tumor thrombus in hepatocellular carcinoma(HCC). MATERIALS AND METHODS: We reviewed 35 cases of combined spiral CTHA and CTAP in 25 HCC patients with portal tumorthrombus from April 1993 to October 1994, regarding to portal tumor thrombus, the involved area of arterioportal(AP) shunt and the development of cavernous transformation of portal vein, in comparison with hepaticand superior mesenteric arteriography. RESULTS: Spiral CTHA showed hyperattenuating tumor, portal tumor thrombus and hyperattenuating peritumoral parenchymal area. Spiral CTAP showed perfusion defect area including tumor, portal tumor thrombus and peritumoral area distal to portal vein obstruction. In 15 cases, portal tumor thrombus showed intraluminal and marginal hyperattenuating linear structures on CTHA due to transvasal AP shunt and tumor feeding arteries, which were corresponding to thread and streaks sign on hepatic arteriography. Cavernous transformation of portal vein was demonstrated in 15 cases as irregular periportal hyperattenuating collateral vessels on spiral CTAP. In 32 cases, portal vein was visualized on CTHA due to AP shunt. And according to shunt amount, we classified AP shunt into 4 grades. Grade I means only the presence of portal tumor thrombus without AP shunt, grade II with segmental AP shunt, grade III with one lobar AP shunt, and grade IV with both lobar AP shuntor the presence of cavernous transformation of portal vein. Grade I was seen in 3, grade II in 4, grade III in 13 and grade IV in 15 cases. CONCLUSION: Variable CTHA and CTAP findings were shown in HCC patients with portal tumor thrombus according to the amount of AP shunt and the presence of cavernous transformation of portal vein. Combined CTHA and CTAP are useful to differentiate the tumor thrombus from simple thrombus and are very sensitive method for detecting AP shunt. Understanding these findings related with portal tumor thrombus is important topredict patient}s prognosis and to decide treatment method.
Angiography* ; Arteries ; Carcinoma, Hepatocellular* ; Hemodynamics* ; Humans ; Perfusion ; Portal Vein ; Portography* ; Prognosis ; Thrombosis*

PURPOSE: To investigate the safety and usefulness of Lipiodol-percutaneous transhepatic ethanolinjection(L-PEI) and to determine the appropriate concentration of Lipiodol during L-PEI. This was achieved byevalvating CT findings and histopathologic changes according to the concentration of Lipiodol, amount of ethanol,and the time interval after injection into normal rabbit liver. MATERIALS AND METHODS: This experimental studyinvolved 18 New Zealand rabbits under US guidance. They were divided into five groups according to injectedmaterials; two rabbits with 0.4cc of normal saline(group I), six with 0.4cc of ethanol in the left hepaticlobe(group II), and 0.4cc of Lipiodol in the right hepatic lobe(group III), five rabbits with 5%Lipiodol-ethanol(5% vol. of Lipiodol+95% vol. of ethanol), 0.2cc in the right hepatic lobe, and 0.4cc in theleft(group IV); and five rabbits with 10% Lipiodol-ethanol as per group IV(group V). CT was performed immediately,one week, two weeks, and three-four weeks after injection, and pathologic specimens were obtained on the thirdday(acute phase) and during the third or fourth week(chronic phase) after injection. RESULTS: On CT, intrahepaticlocalization of the L-PEI injection site was well demonstrated as a focal high attenuated area which graduallydecreased in attenuation on follow up CT. The opacification of the inferior vena cava by Lipiodol, the lineardistribution of Lipiodol along portal veins or fissures, and peritoneal leakage were clearly demonstrated ingroups III-V, though the effects gradually disappeared during follow-up CT. There was no remarkable difference ingross CT attenuation between group IV and group V. The main pathologic findings during the acute phase of group IIwere coagulation necrosis surrounded by macrophage, inflammatory reaction, and early periportal and subcapsularfibrosis. The findings in group IV and V were similar to those in group II and additional fat vacuoleaccumulations in the necrotic area were also seen. During the chronic phase of group II, areas of necrosis wereabsent or smaller and were surrounded or replaced by more organized fibrosis, macrophage or multinucleated giantcell infiltration. Periportal, subcapsular fibrosis was also found. In group IV and V, the findings were similarto those of group II, though additional fat vacuoles in fibrotic or necrotic areas, foreign body reaction to fatvacuole, regenerating nodule and calcification were also observed. CONCLUSION: L-PEI is more useful for thedetection by CT of an injection site than PEI alone, and with regard to CT and histopathologic findings, there wasno significant difference between the 5% and 10% Lipiodol-ethanol groups. Compared to PEI, L-PEI provoked nosighificant additional hepatic injury; only fatty change and foreign body reaction were noted. Thus, L-PEI is moreuseful than PEI for the management of HCC.
Animals ; Contrast Media ; Ethanol* ; Ethiodized Oil* ; Fibrosis ; Follow-Up Studies ; Foreign-Body Reaction ; Liver* ; Macrophages ; Necrosis ; Portal Vein ; Rabbits ; Vacuoles ; Vena Cava, Inferior