Long-term follow-up on MURCS (Müllerian duct, renal, cervical somite dysplasia) association and a review of the literature

Müllerian duct aplasia-renal aplasia-cervicothoracic somite dysplasia (MURCS) association is a unique development disorder with four common types of malformations that include uterine aplasia or hypoplasia, renal ectopy or agenesis, vertebral anomalies, and short stature. The majority of MURCS patients are diagnosed with primary amenorrhea from late-adolescence. However, a few cases with MURCS association are not well diagnosed during childhood and long-term outcomes are not well reported. We report a case of an 8-year-old girl with MURCS association who presented with recurrent urinary tract infections and multiple congenital malformations, and who was followed for 10 years until adulthood. MURCS association should be considered as one of the differential diagnoses when evaluating prepubertal females with vertebral and renal malformations.

The Efficacy of Percutaneous Lateral Hemiepiphysiodesis on Angular Correction in Idiopathic Adolescent Genu Varum

BACKGROUND: Percutaneous lateral hemiepiphysiodesis of the lower extremity is a simple and excellent method to correct the angular and length problems cosmetically. However, the efficacy of percutaneous lateral hemiepiphysiodesis is not well established in the literature. The purpose of this study was to evaluate the efficacy of percutaneous lateral hemiepiphysiodesis for angular corrections in adolescent idiopathic genu varum patients with proximal tibia vara and identify the factors affecting the amount of deformity correction of the lower limb in the coronal plane. METHODS: We retrospectively reviewed 20 patients (40 lower limbs) who had percutaneous lateral hemiepiphysiodesis on the proximal lateral tibia between 1997 and 2010. Radiographic evaluations were made using (1) the hip-knee-ankle angle and (2) the length of the tibia. Furthermore, the intercondylar distance was evaluated at the level of the knee joint. Preoperative factors (gender, age, body mass index, intercondylar distance, preoperative hip-knee-ankle angle, remaining growth of tibia, and calculated correctable angle) were analyzed, as well as their correlation with the degree of the actual correction angle. RESULTS: The amount of coronal deformity of the lower limb was improved from its preoperative state. The median average of hip-knee-ankle angle improved from 8.0degrees (interquartile range [IQR], 7.0degrees to 10.0degrees) preoperatively to 3.0degrees (IQR, 2.5degrees to 4.0degrees) at the final follow-up (p < 0.001). The median percent ratio of the angular correction was 60% (IQR, 50% to 71.3%). The correlation coefficients were -0.537, 0.832, 0.791, and 0.685 for the bone age, preoperative hip-knee-ankle angle, the remaining growth of tibia, and calculated correctable angle, respectively. CONCLUSIONS: Despite the excellent cosmetic outcome of percutaneous lateral hemiepiphysiodesis on the proximal lateral tibia in adolescent idiopathic genu varum, the effect was limited in most cases. For optimum results, surgery a few months earlier is recommended, rather than at the calculated operation time.

Chondroblastoma in a Distal Phalanx of the Great Toe: A Case Report / 대한정형외과학회잡지

A chondroblastoma is a relatively rare benign bone tumor that is typically encountered in the epiphysis. The authors describe a case of a chondroblastoma arising in the great toe. An 82-year-old woman presented with pain over her left great toe with a 1 year duration. The radiographs showed an expansile osteolytic lesion with cortical thinning and coarse trabeculation that replaced the distal phalanx of the left great toe. An incisional biopsy and curettage were performed. Histologically, the tumor consisted of uniform, round to polygonal cells with clear to slightly eosinophilic cytoplasm and round to ovoid nuclei, mimicking chondroblasts, intermingled with osteoclast-like giant cells.

A Case of Triple Cancers of the Esophagus, Stomach and Duodenum / 대한소화기내시경학회지

Multiple primary cancer is defined as the multiple occurrence of malignant neoplasm in the same individual. The incidence of multiple primary cancer is 0.26~1.43% in Korea, but this malady is increasing due to development of diagnostic modalities, the treatment of cancer and the recording of accurate cancer statistics. The most common site of multiple primary cancer in Korea is the stomach, colon, breast, cervix, liver and lung. We report here on a case of triple cancer that was located at the middle esophagus, stomach and duodenum in 75 year-old man.

Prevalence and Risk Factors of Gallbladder Polyp in Adults Living in Daegu and Gyeongbuk Provinces / 대한소화기학회지

BACKGROUND/AIMS: Although there are various reports on the prevalence of gallbladder (GB) polyp according to the race or area, there is no report on Daegu and Gyeongbuk provinces. Therefore, we investigated the prevalence and related risk factors of GB polyp in health screening population of Daegu and Gyeongbuk provinces. METHODS: Of 28,911 adult health screening examinees from January 1997 to May 2003, age and gender-matched 8,008 (3,896 men, 4,112 women) were randomly selected to evaluate the prevalence. Independent risk factors were analyzed by comparing various variables between the GB polyp positive and negative groups. RESULTS: The prevalence of GB polyp was 2.2% (175/8,008). Those were 2.7% (104/3,896) among men and 1.7% (71/4,112) among women. Independent risk factors related with GB polyp were male gender (OR 1.434, p<0.001), obesity (OR 1.237, p=0.024), and hepatitis B carrier (OR 1.888, p<0.001) CONCLUSIONS: Prevalence of GB polyp in Daegu and Gyeongbuk provinces is 2.2%. Male, obesity, and hepatitis B carrier may be the risk factors related with GB polyp in this region.

Factors Associated or Related to with Pathological Severity of Nonalcoholic Fatty Liver Disease

BACKGROUND: Nonalcoholic fatty liver disease (NAFLD) has been more and more often regarded as a serious disorder, because nonalcoholic steatohepatitis (NASH), a part of NAFLD, may progress to the end stage of liver disease. Though an advanced age, obesity, diabetes mellitus (DM) etc. being not infrequent conditions in Korea, are known to exacerbate the severity of this disease, there are only a few Korean reports on this subject. The purpose of this study is to identify possible factors that might add up to the pathological severity of this disorder in Korean patients. METHODS: Of 60 patients with steatosis found at liver biopsy, 43 NAFLD patients were reviewed retrospectively after exclusion of other liver diseases. RESULTS: The cases of steatosis were mild, moderate, and severe in 9, 10, and 24 patients, respectively. The degree of necroinflammatory activity was mild, moderate, and severe in 33, 9, and 1 patients, respectively. There were no established factors directly related to these classes. As to fibrosis, the cases were classified as none, mild, moderate, severe, and cirrhotic in 9, 11, 16, 7, and 0 patients, respectively. The stage of fibrosis correlated with the age (p< 0.001), BMI (body mass index) (p=0.032), and the platelet count (p=0.009), but the presence of NASH was associated only with BMI (p=0.002) and obesity (p=0.001). CONCLUSION: It seems that there are no factors that are directly related to the degree of steatosis or necroinflammatory activity. BMI seems to be a unique factor directly related to both the severity of fibrosis and the presence of NASH. The age and the platelet count are factors that are directly related to the degree of fibrosis but not to the presence of NASH.

A Case of Duodenal Perforation during Endoscopic Hemostasis in EST Site Bleeding / 대한소화기내시경학회지

The endoscopic retrograde cholangiopancreatography (ERCP) has become a commonly performed endoscopic procedure in biliary pathology. ERCP is a relatively safe procedure. however, there are chance of potentially severe complications; pancreatitis, hemorrhage, infection, and perforation. Duodenal perforation, uncommon but severe complication of ERCP, occurred in less than 1% of most series. According to the related mechanism, anatomical location, and the severity of injury, three to four distinct types of perforations have been described. We experienced the barotrauma associated duodenal perforation during endoscopic hemostasis in patient with EST site bleeding. This duodenal perforation was related with excessive air inflation to maintain the patency of a lumen. Endoscopists performing ERCP should bear in mind that continued air inflation may lead to duodenal perforation.