Cyclopia is rare congenital craniofacial anomaly, in which the eyes are fused together and located in a single orbit. It is consistently associated with severe holoprosencephaly, which is the failure of cleavage of the prosencephalon with a deficit in the midline facial development. chromosomal study revealed 47, X( ), +13 (Patau syndrome).
Holoprosencephaly ; Orbit ; Prosencephalon ; Trisomy*

No abstract available.
Dandy-Walker Syndrome*

A variety files made of stainless steel (S-S) or nickel-titanium (Ni-Ti) are used during endodontic treatment. The purpose of this study was to evaluate the corrosion susceptibility of S-S and Ni-Ti endodontic files. Three brands of files were used for this study: K-flex(R) S-S files (Maillefer, USA), Profile(R) Ni-Ti files (Maillefer, USA), K-3(R) Ni-Ti files (SybronEndo, USA). 120 files of each brands (21mm, ISO size #20) were divided into 12 groups according to 1) sterilization methods using Autoclave or Ethylene Oxide (E-O) gas, 2) Irrigation solutions using 5.25 % NaOCl or Saline, 3) the number of sterilization (1, 5, 10 times). After above procedures, each of the files was inspected by three examiners with a light microscope and camera at X25. Each file was judged and ranked according to the following criteria: 0; no corrosion, 1; mild corrosion, 2; moderate corrosion, and 3; severe corrosion. The files of high score were examined under the Scanning Electron Microscope. Data were statistically analyzed with the Kruskal-Wallis test (p < 0.05). Most of the ten time-autoclaved files had showed mild to moderate corrosion. But, one or five time-autoclaved files did not show corrosive surface. NaOCl treatment and E-O gas sterilization did not influence on corrosion. There was a significant difference in corrosion susceptibility between sterilization methods and the number of autoclaving. However, there was no significant difference between brands and file materials.
Corrosion* ; Ethylene Oxide ; Stainless Steel ; Sterilization*

Perivascular epithelioid cell tumors (PEComas) are unusual mesenchymal neoplasms composed of histologically and immunohistochemically distinct perivascular epithelioid cells (PECs). Although PEComas have the potential to behave in a malignant fashion, malignant PEComas arising from the retroperitoneum are extremely rare. A 68-year-old woman presented with a painful palpable mass in her left upper abdomen. Computed tomography of the abdomen showed a 9 cm sized heterogeneous mass in left para-aortic space and multiple hypervascular nodules in the liver. 18F-fludeoxyglucose-PET/CT showed multifocal hypermetabolic lesions in retroperitoneum, liver, and skeletal bones. Percutaneous needle biopsies were done on the retroperitoneal and hepatic mass. Both specimens were positive for human melanoma black-45 (HMB-45) on histological and immunohistochemical staining which was compatible with PEComas. Herein, we report a rare case of retroperitoneal PEComa with multiple metastases involving liver and bone at initial diagnosis that exhibited aggressive behavior and resulted in a devastating prognosis.
Aged ; Bone Neoplasms/pathology/radiography/secondary ; Female ; Fluorodeoxyglucose F18 ; Humans ; Liver Neoplasms/pathology/radiography/secondary ; Perivascular Epithelioid Cell Neoplasms/*diagnosis/pathology ; Positron-Emission Tomography ; Tomography, X-Ray Computed

Small cell carcinomas are the most aggressive, highly malignant neuroendocrine tumors; among these, gastric small cell carcinoma (GSCC) is extremely rare. Here we report a case of a patient with primary GSCC, presenting as linitis plastic, who was diagnosed using endoscopic ultrasound (EUS)-guided biopsy. With undiagnosed linitis plastica, an 80-year-old woman was referred to our institution. Abdominal computed tomography revealed irregular wall thickening extending from the gastric body to the antrum. Endoscopy suspected to have Borrmann type IV advanced gastric cancer. EUS of the stomach showed diffuse submucosal thickening of the gastric wall, mainly the antrum. EUS-guided bite-on-bite biopsy confirmed the diagnosis of GSCC. In general, GSCC is difficult to diagnose and careful examination is necessary to determine the therapeutic strategy; however, EUS is particularly helpful in the differential diagnosis of a lesion presenting as linitis plastica.
Aged, 80 and over ; Biopsy ; Carcinoma, Small Cell ; Diagnosis ; Diagnosis, Differential ; Endoscopy ; Endosonography ; Female ; Humans ; Linitis Plastica ; Neuroendocrine Tumors ; Plastics ; Stomach ; Stomach Neoplasms ; Ultrasonography

Background/Aims: The effect of hyperammonemia on the mortality in patients with liver cirrhosis is well documented. However, little is known about the impact of hyperammonemia on mortality among intensive care unit patients without hepatic disease. We aimed to investigate factors associated with non-hepatic hyperammonemia among intensive care unit patients and to evaluate the factors related to the 7- and 90-day mortality. Methods: Between February 2016 and February 2020, 948 patients without hepatic disease who had 972 episodes of admission to the intensive care unit were retrospectively enrolled and classified as hyperammonemia grades 0 (≤ 80 µg/dL; 585 [60.2%]), 1 (≤ 160 µg/dL; 291 [29.9%]), 2 (≤ 240 µg/dL; 55 [5.7%]), and 3 (> 240 µg/dL; 41 [4.2%]). Factors associated with hyperammonemia and the 7- and 90-day mortality were evaluated by multivariate logistic regression analysis and Cox regression analysis, respectively. Kaplan-Meier survival curves for the 7- and 90-day mortality were constructed. Results: The independent risk factors for hyperammonemia were male sex (odds ratio, 1.517), age (0.984/year), acute brain failure (2.467), acute kidney injury (1.437), prothrombin time-international normalized ratio (2.272/unit), and albumin (0.694/g/dL). The 90-day mortality rate in the entire cohort was 24.3% and gradually increased with increasing hyperammonemia grade at admission (17.9%, 28.2%, 43.6%, and 61.0% in patients with grades 0, 1, 2, and 3, respectively). Additionally, non-hepatic hyperammonemia was an independent predictor of the 90- day mortality in intensive care unit patients. Conclusions Non-hepatic hyperammonemia is common (39.8%) and associated with the 90-day mortality among intensive care unit patients.

Liver cirrhosis is commonly associated with bleeding complications due to portal hypertension or coagulopathy. Spontaneous muscle hematoma is a rare but potentially lethal complication of liver cirrhosis. Here we report three cases of spontaneous muscle hematoma diagnosed in patients with alcoholic liver cirrhosis. All three patients died due to recurrent bleeding and liver failure although they had undergone repeated transcatheter arterial embolization of the actively bleeding vessels. We reviewed 14 cases of spontaneous muscle hematoma that were associated with liver cirrhosis, including our cases, and found that the mortality rate was 86%, despite early diagnosis and treatment. Cirrhosis-associated spontaneous muscle hematoma occurred more frequently in patients with alcoholic liver cirrhosis, who accounted for -93% of cases. Thus, spontaneous muscle hematoma should be considered a life-threatening complication in patients with alcoholic liver cirrhosis, and abstinence from alcohol may help to prevent the occurrence of this deadly condition.
Alcoholics* ; Early Diagnosis ; Fibrosis ; Hematoma* ; Hemorrhage ; Humans ; Hypertension, Portal ; Liver Cirrhosis ; Liver Cirrhosis, Alcoholic* ; Liver Failure ; Mortality

BACKGROUND/AIMS: Fragmented care in nephrology can cause treatment delays. Nephrologists are qualified to perform vascular access-related procedures because they understand the pathophysiology of renal disease and perform physical examination for vascular access. We compared treatment delays associated with tunneled hemodialysis catheter (TDC) placement between interventional radiologists and nephrologists. METHODS: We collected data by radiologists from January 1, 2011 through December 31, 2011 and by nephrologists from since July 1, 2012 through June 30, 2013. We compared the duration from the hemodialysis decision to TDC placement (D-P duration) and hemodialysis initiation (D-H duration), catheter success and the complication rate, and the frequency and the usage time of non-tunneled hemodialysis catheters (NDCs) before TDC placement. RESULTS: The study analyzed 483 placed TDCs: 280 TDCs placed by radiologists and 203 by nephrologists. The D-P durations were 319 minutes (interquartile range [IQR], 180 to 1,057) in the radiologist group and 140 minutes (IQR, 0 to 792) in the nephrologist group. Additionally, the D-H durations were 415 minutes (IQR,260 to 1,091) and 275 minutes (IQR, 123 to 598), respectively. These differences were statistically significant (p = 0.00). The TDC success rate (95.3% vs. 94.5%, respectively; p = 0.32) and complication rate (16.2% vs. 11%, respectively; p = 0.11) did not differ between the groups. The frequency (24.5 vs. 26%, respectively; p = 0.72) and the usage time of NDC (8,451 vs. 8,416 minutes, respectively; p = 0.91) before TDC placement were not statistically significant. CONCLUSIONS: Trained interventional nephrologists could perform TDC placement safely, minimizing treatment delays.
Catheters* ; Nephrology ; Physical Examination ; Renal Dialysis* ; Vascular Access Devices

This study was performed to investigate the association between hair mineral levels and nutrient intakes, age, and BMI in female adults who visited a woman's clinic located in Seoul. Dietary intakes were assessed by food frequency questionnaire and mineral levels were measured in collected hairs, and the relationship between these was examined. The average daily nutrient intakes of subjects were compared to those of the KDRIs, and the energy intake status was fair. The average intake of calcium in women of 50 years and over was 91.35% of KDRIs and the potassium intake was greatly below the recommended levels in all age groups. In the average hair mineral contents in subjects, calcium and copper exceeded far more than the reference range while selenium was very low with 85.19% of subjects being lower than the reference value. In addition, the concentrations of sodium, potassium, iron, and manganese in the hair were below the reference ranges in over 15% of subjects. The concentrations of sodium, chromium, sulfur, and cadmium in the hair showed positive correlations (P < 0.05) with age, but the hair zinc level showed a negative correlation (P < 0.05) with age. The concentrations of sodium, potassium, chromium, and cadmium in the hair showed positive correlations (P < 0.05) with BMI. Some mineral levels in subjects of this study showed significant correlations with nutrient intakes, but it seems that the hair mineral content is not directly influenced by each mineral intake. As described above, some hair mineral levels in female adults deviated from the normal range, and it is considered that nutritional intervention to control the imbalance of mineral nutrition is required. Also, as some correlations were shown between hair mineral levels and age, BMI, and nutrient intakes, the possibility of utilizing hair mineral analysis for specific purposes in the future is suggested.
Adult ; Body Mass Index ; Cadmium ; Calcium ; Chromium ; Copper ; Energy Intake ; Female ; Hair ; Humans ; Iron ; Manganese ; Potassium ; Surveys and Questionnaires ; Reference Values ; Selenium ; Sodium ; Sulfur ; Zinc