Biliary hamartoma and congenital hepatic fibrosis belong to fibrocystic disorders originating from ductal plate malformation. A 66-year-old man who had incidentally been diagnosed with biliary hamartoma two years ago presented to us with recurrent acute cholangitis. In the first episode, he had presented with septic shock and was treated with endoscopic retrograde cholangiopancreatography (ERCP) and cholecystectomy under the diagnosis of acute cholecystitis and cholangitis. However, during a two-month follow-up period, the patient experienced four episodes of acute cholangitis. Because he showed normal ERCP, and biliary hamartoma is usually asymptomatic, a liver biopsy was performed. Pathology revealed combined features of biliary hamartoma and congenital hepatic fibrosis, characterized as periportal fibrosis and intrahepatic ductular dysplasia. During follow-up for the last six months, he had experienced two episodes of acute cholangitis and was treated with antibiotics. A follow-up abdominal CT scan revealed aggravated hepatosplenomegaly compared to that of two years ago. We report a case of combined congenital hepatic fibrosis and biliary hamartoma and a literature review.
Aged ; Anti-Bacterial Agents ; Biopsy ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangitis* ; Cholecystectomy ; Cholecystitis, Acute ; Diagnosis ; Fibrosis* ; Follow-Up Studies ; Hamartoma* ; Humans ; Liver ; Pathology ; Shock, Septic ; Tomography, X-Ray Computed

A 56-year-old female was referred to our hospital due to a mass measuring 5 cm in size in the left pelvic cavity, which was found incidentally during a health examination by ultrasonography. Exploratory laparotomy was performed and the mass was located at the left retroperitoneal parametrium without invasion of the uterus and ovary. The pathology report confirmed squamous cell carcinoma. Even after further studies, we did not find any other primary lesion. Human papillomavirus (HPV) DNA chip test (HPV 9G DNA Membrane Kit, Biometrixtechnology Inc.) showed that the surgical specimen was positive for HPV 18. She received adjuvant chemotherapy and would receive radiation therapy for the possibility of occult gynecologic cancer. Retroperitoneal squamous cell carcinoma of unknown primary is extremely rare and little is known about it. It is reported that HPV may be associated with the disease. Hence, the result of HPV test could have an impact on finding a suspicious primary lesion and treatment modality in this case.
Carcinoma, Squamous Cell* ; Chemotherapy, Adjuvant ; DNA ; Female ; Human papillomavirus 18 ; Humans ; Laparotomy ; Membranes ; Middle Aged ; Oligonucleotide Array Sequence Analysis ; Ovary ; Pathology ; Retroperitoneal Neoplasms ; Ultrasonography ; Uterus

BACKGROUND: Autologous fat graft has become a useful technique for correction of acquired contour deformity in reconstructed breasts. However, there remains controversial regarding the efficacy and safety of the practice for reconstructive breast surgery. METHODS: A retrospective review was performed on 102 patients who had secondary fat grafting after breast reconstruction. Fat harvest, refinement and injection were done by Coleman's technique. All patients were followed up postoperatively within 1 month and after 6 months including physical examination and ultrasonography. In 38 patients, the reabsorption rate was calculated by serial changes of thickness between skin and pectoral fascia in the ultrasonic finding. Locoregional recurrence rate was compared with control group of 449 patients who had breast reconstruction without fat graft in the same time period. RESULTS: Average 49.3 mL fat was injected into each breast. The most common location of fat graft was upper pole, followed by axilla, lower and medial breasts. During 28.7 months of average follow-up period, 2.9% of total patients had symptoms of palpable mass on fat graft side and ultrasonography identified fat necrosis and cyst formation in 17.6% of the patients. Calculated fat reabsorption rate was 32.9%. Locoregional recurrence was occurred in 1 patient (0.9%) and the rate was not different significantly with control group (2%). CONCLUSIONS: Although further studies are required to provide surgeons with definitive guidelines for the implementation of fat grafting, we propose autologous fat graft is an efficient and safe technique for secondary breast reconstruction.
Absorption* ; Axilla ; Breast* ; Congenital Abnormalities ; Fascia ; Fat Necrosis ; Female ; Follow-Up Studies ; Humans ; Mammaplasty ; Physical Examination ; Recurrence ; Retrospective Studies ; Skin ; Transplants* ; Ultrasonics ; Ultrasonography*

PURPOSE: The purpose of this article was to evaluate the efficiency of an automated prescription program serving pediatric patients who visited our emergency department. We expected that the program would reduce pediatric patient prescription errors and shorten the length of stay in the emergency department. METHODS: We developed the computerized physician order entry system to serve the pediatric patients visiting the emergency department and recommended that the emergency medicine residents use this program. We compared the rate of error as it affected prescription dosage and length of stay in the emergency department, before and after the program was deployed. We also performed a survey with 20 emergency medicine residents that have used the program. RESULTS: Of the total number of prescriptions filled, the number of prescription dosing errors observed was 40(4.5%) before and 22(2.8%) after the program was deployed. The results of the survey revealed high system usability and satisfaction. However, the length of stay in the emergency department was not affected. CONCLUSION: The computerized physician order entry system for pediatric patients visiting the emergency department was effective in reducing prescription related medical errors.
Emergencies ; Emergency Medicine ; Humans ; Length of Stay ; Medical Errors ; Medical Order Entry Systems ; Prescriptions

BACKGROUND: In this report, we present the annual data of the intensive care unit (ICU) module of the Korean Nosocomial Infections Surveillance System (KONIS) from July 2009 through June 2010. METHODS: We performed a prospective surveillance of nosocomial urinary tract infections (UTIs), bloodstream infections (BSIs), and pneumonia (PNEU) at 116 ICUs in 63 hospitals by using KONIS. Nosocomial infection (NI) rates were calculated as the number of infections per 1,000 patient-days or device-days. RESULTS: We identified 3,965 NIs during the study period: 2,156 cases of UTIs (2,119 were urinary catheter-associated), 1,110 cases of BSIs (948 were central line-associated), and 699 cases of PNEU (410 were ventilator-associated). The rate of urinary catheter-associated UTIs (CAUTIs) was 4.75 cases per 1,000 device-days (95% confidence interval, 4.55-4.95), and urinary catheter utilization ratio was 0.86 (range, 0.859-0.861). The rate of central line-associated BSIs was 3.28 (range, 3.07-3.49), and the utilization ratio was 0.56 (range, 0.559-0.561). The rate of ventilator-associated PNEUs (VAPs) was 1.95 (range, 1.77-2.15), and the utilization ratio was 0.41 (range, 0.409-0.411). Although ventilator utilization ratio was lower in the hospitals with 400-699 beds than in the hospitals with 700-899 beds and more than 900 beds, the rate of VAPs were higher in the hospitals with 400-699 beds than in hospitals with 700-899 beds and more than 900 beds. The incidence of infections due to imipenem-resistant Acinetobacter baumannii increased from 43.6% to 82.5% since July 2006. CONCLUSION: The risk of acquiring VAP and CAUTI is highest in the ICUs of hospitals with 400-699 beds than that in hospitals with more beds. Imipenem-resistant A. baumannii was identified as an emerging gram-negative pathogen of nosocomial infections.
Acinetobacter baumannii ; Cross Infection ; Incidence ; Critical Care ; Intensive Care Units ; Pneumonia ; Urinary Catheters ; Urinary Tract Infections ; Ventilators, Mechanical

BACKGROUND/AIMS: Fabry disease is an X-linked recessive and progressive disease caused by alpha-galactosidase A (alpha-GaL A) deficiency. We sought to assess the prevalence of unrecognized Fabry disease in dialysis-dependent patients and the efficacy of serum globotriaosylceramide (GL3) screening. METHODS: A total of 480 patients of 1,230 patients among 17 clinics were enrolled. Serum GL3 levels were measured by tandem mass spectrometry. Additionally, we studied the association between increased GL3 levels and cardiovascular disease, cerebrovascular disease, or left ventricular hypertrophy. RESULTS: Twenty-nine patients had elevated serum GL3 levels. The alpha-GaL A activity was determined for the 26 patients with high GL3 levels. The mean alpha-GaL A activity was 64.6 nmol/hr/mg (reference range, 45 to 85), and no patient was identified with decreased alpha-GaL A activity. Among the group with high GL3 levels, 15 women had a alpha-GaL A genetics analysis. No point mutations were discovered among the women with high GL3 levels. No correlation was observed between serum GL3 levels and alpha-GaL A activity; the Pearson correlation coefficient was 0.01352 (p = 0.9478). No significant correlation was observed between increased GL3 levels and the frequency of cardiovascular disease or cerebrovascular disease. CONCLUSIONS: Fabry disease is very rare disease in patients with end-stage renal disease. Serum GL3 measurements as a screening method for Fabry disease showed a high false-positive rate. Thus, serum GL3 levels determined by tandem mass spectrometry may not be useful as a screening method for Fabry disease in patients with end stage renal disease.
Adult ; Aged ; Fabry Disease/blood/*diagnosis ; Female ; Humans ; Kidney Failure, Chronic/blood/*therapy ; Male ; Middle Aged ; *Renal Dialysis ; Trihexosylceramides/*blood ; alpha-Galactosidase/genetics/metabolism

BACKGROUND: A nationwide prospective multicenter study was performed in Korea to determine the incidence and risk factors for surgical site infections (SSI) after craniotomies (CRAN), ventricular shunt operations (VS), gastric operations (GAST), colon operations (COLO), rectal operations (RECT), hip joint replacements (HJR), and knee joint replacements (KJR). METHODS: We collected data regarding demographics, clinical and operative risk factors for SSI, and antibiotics administered to the patients who underwent CRAN in 18 hospitals, VS in 19 hospitals, GAST in 19 hospitals, COLO in 19 hospitals, RECT in 19 hospitals, HJR in 24 hospitals, and KJR in 23 hospitals between January and December 2009. All the data were collected using a real-time web-based reporting system. RESULTS: The SSI rate of CRAN, VS, GAST, COLO, RECT, HJR, and KJR was 3.68 (22/1,169), 5.96 (14/235), 4.25 (75/1,763), 3.37 (22/653), 5.83 (27/463), 1.93 (23/1,190), and 2.63 (30/1,139), respectively, per 100 operations. The only significant risk factor for SSI after CRAN was postoperative cerebrospinal fluid leakage. The independent risk factors for SSI after GAST were multiple procedure, reoperation, infection of other sites, and transfusion. In HJR, the duration of preoperative hospital stay and operation time were longer, and the need for general anesthesia, transfusion, and steroid use and the incidence of contaminated/dirty wound, obesity, and infection of other sites were significantly increased in the infected group. In KJR, the duration of preoperative hospital stay was longer and the need for reoperation was significantly higher in the infected group, and in addition, the incidence of SSI was higher among males. CONCLUSION: The maintenance of surveillance on SSI is very important because surveillance provides valuable information to the surgeon and infection control personnel, which in turn helps decrease the incidence of SSI.
Anesthesia, General ; Anti-Bacterial Agents ; Colon ; Craniotomy ; Demography ; Hip Joint ; Humans ; Incidence ; Infection Control ; Knee Joint ; Korea ; Length of Stay ; Male ; Obesity ; Reoperation ; Risk Factors

BACKGROUND: In this report, we present the annual data of the intensive care unit (ICU) module of the Korean Nosocomial Infections Surveillance System (KONIS) from July 2008 through June 2009. METHODS: We performed a prospective surveillance of nosocomial urinary tract infections (UTI), bloodstream infections (BSI), and pneumonia (PNEU) at 101 ICUs in 57 hospitals using KONIS. Nosocomial infection (NI) rates were calculated as the numbers of infections per 1,000 patient-days or device-days. RESULTS: We identified 3,287 NIs during the study period: 1,787 UTIs (1,772 cases were urinary catheter-associated), 917 BSIs (797 were central line-associated), and 583 PNEUs (335 were ventilator-associated). The rate of urinary catheter-associated UTIs was 4.80 cases per 1,000 device-days (95% confidence interval, 4.58-5.03) and urinary catheter utilization ratio was 0.85 (0.849-0.851). Although the urinary catheter utilization ratios were lower in the hospitals with 400-699 beds than in the hospitals with 700-899 beds and more than 900 beds, the rates of urinary catheter-associated UTIs were higher in hospitals with 400-699 beds than in the larger ones. The rate of central line-associated BSIs was 3.27 (3.05-3.51) and the utilization ratio was 0.56 (0.559-0.561). The rate of ventilator-associated PNEUs was 1.86 (1.67-2.07) and the utilization ratio was 0.41 (0.409-0.411). The rate of ventilator-associated PNEUs was lower in July 2008-June 2009 than in July 2007-June 2008 and July 2006-December 2006. CONCLUSION: It appears that the KONIS influences the reduction in the rate of device-associated infections, especially ventilator-associated PNEU; therefore, ongoing targeted surveillance and infection control strategies are needed to control device-associated infections.
Cross Infection ; Gossypol ; Infection Control ; Critical Care ; Intensive Care Units ; Pneumonia ; Urinary Catheters ; Urinary Tract Infections

OBJECTIVE: C2 laminar screw fixation is considered as an excellent alternative to Magerl's transfacetal approach or Harms construct for the atlantoaxial stabilization. However, to our knowledge, there is no report on the feasibility of the new approach to Korean population. We investigated morphometric parameters of the dorsal arch of the C2 to provide the quantitative data for the feasibility of laminar screw fixation. METHODS: One-hundred-and-two patients' cervical computed tomography had been reconstructed and investigated on the anatomical parameters related with C2 laminar screw placement. Sixty patients were male and forty-two patients were female. Measurements included the laminar thickness and slope, spino-laminar angle, and maximal screw length. RESULTS: Ages ranged from 20 to 81 and the mean age was 48.4. Mean laminar thickness was 5.7 mm (+/-1.0) (5.8 mm in male and 5.4 mm in female). Fifty-one patients (50%) had a laminar thickness smaller than 5.5 mm at least unilaterally, therefore the patients were considered as inappropriate candidates for the laminar screw fixation in the smaller side of the laminae. Mean value of maximal length of screw was 33.3 mm (34.3 mm in male and 31.9 mm in female). Mean spino-laminar angle was 43.2degrees and mean slope angle was 32.9degrees. CONCLUSION: Half of patients had inappropriate laminar profiles to accommodate a 3.5 mm screw in at least one side of the axis. The three-dimensional computed tomography reconstruction is mandatory for the preoperative assessment for the feasibility of the C2 lamina.
Axis, Cervical Vertebra ; Female ; Humans ; Male

BACKGROUND: This is the first annual data on the surveillance of intensive care unit (ICU) module by the Korean Nosocomial Infections Surveillance System (KONIS) from July 2007 through June 2008. METHODS: The KONIS performed a prospective surveillance for nosocomial urinary tract infections (UTI), bloodstream infections (BSI), and pneumonia (PNEU) at 96 ICUs in 56 hospitals. Nosocomial infection (NI) rates were calculated as the numbers of infections per 1,000 patient-days or device-days. RESULTS: A total of 2,637 NIs were found during the study period; 1,391 UTIs (1,365 cases were urinary catheter-associated), 667 BSIs (563 were central line-associated), and 579 PNEUs (357 were ventilator-associated). The rate of urinary catheter-associated UTIs was 4.43 cases per 1,000 device-days (95% confidence interval, 4.20-4.67) and urinary catheter utilization ratio was 0.84 (0.839-0.841). The rate of central line-associated BSIs was 2.83 (2.61-3.07) and the utilization ratio was 0.54 (0.538-0.542). The rate of ventilator-associated PNEUs was 2.49 (2.25-2.76) and the utilization ratio was 0.39 (0.388-0.392). Although the ventilator utilization ratios were lower in the hospitals with less than 900 beds than in the hospitals with more than 900 beds, the rates of ventilator-associated PNEUs were higher in the smaller hospitals than in the larger ones. CONCLUSION: This result suggests that ongoing targeted surveillance and implementation of proven infection control strategies are needed.
Cross Infection ; Infection Control ; Critical Care ; Intensive Care Units ; Pneumonia ; Prospective Studies ; Urinary Catheters ; Urinary Tract Infections ; Ventilators, Mechanical