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

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

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: The purposes of this study were to evaluate the plain radiologic findings of the childhood intussusception and to evaluate the role of plain abdominal films in predicting the success of air or barium reduction. SUBJECTS AND METHODS: We retrospectively reviewed 140 cases with the diagnosis of intussusception in children. The radiological signs that included soft tissue mass, dilatation of small bowel suggesting obstruction, crescent sign, and target sign were evaluated in terms of frequency. The relationship between radiological findings and outcome of reduction was analyzed. The site of soft tissue mass or crescent sign seen on plain radiographs was correlated with the position of the apex of the intussusceptum seen at the beginning of barium enema. The degree of dilated small bowel was evaluated by calculating the proportion of air-filled small bowel occupying peritoneal cavity and measuring the maximal diameter of dilated bowel lumen. The radiological finding for small bowel obstruction is determined by observation of the degree of small bowel dilatation and/or air-fulid levels. RESULTS: Ninety-two cases out of 140 showed one or more radiographic signs. Two most common signs were soft tissue mass and small bowel obstruction. The success rate of air or barium reduction was significantly lower in patients with most severe degree of dilatation of small bowel and/or more than 7 air-fulid levels on erect view. The suspected location of intussusception on plain radiographs correlated well with the true location of intussusception seen in the first few seconds of barium reduction. CONCLUSION: Plain abdominal radiography is useful in the diagnosis of intussusception and provides helpful informations for the reduction procedure as well as for the exclusion of the contraindications such as bowel perforation.
Barium ; Child ; Diagnosis ; Dilatation ; Enema ; Humans ; Intussusception* ; Peritoneal Cavity ; Radiography, Abdominal ; Retrospective Studies

PURPOSE: CTAP(CT during arterial portography) has been shown to be the most sensitive technique available for the detection of hepatic mass. But because of many false positive lesions, it is important to interpretate the findings correctly. So we tried to analyse the false positive findings in CTAP. MATERIALS AND METHODS: Materials were 47 patients that had (;TAP for the detection of hepatic lesions. After performing superior roesenteric artery (SMA) arterial portography for the detection of vascular variation, CT^P was done. 100 to 150 cc of 50% diluted nonionic contrast media was injected at 2--3ml/sec rate. Spiral CT was used with 8mm slice thickness and 8mm/sec table speed. We compared the findings of CTAP with CTHA(CT during hepatic arteriography), enhanced (;T, anglogram, US, Lipiodol CT and biopsy. RESULTS: Twenty nine false positive lesions were found. There were 11 peritumoral defects, 7 straight line signs, 5 perihilar defects, 2 periligamentous defects, 3 subcapsular defects and 1 defect by cirrhosis. CONCLUSION: When CTAP is used for the evaluation of hepatic lesion(esp, preoperative evaluation), understanding of the false positive findings and the characterization of the lesion through multi-modality approaches are needed for the correct diagnosis and the proper treatment.
Arteries ; Biopsy ; Contrast Media ; Diagnosis ; Ethiodized Oil ; Fibrosis ; Humans ; Portography ; Tomography, Spiral Computed*