Parry-Romberg syndrome(Progressive hemifacial atrophy), described in the last century by Parry(1825) and Romberg(1846), is a very rare disorder characterized by a slowly progressive and self-limited unilateral(rarely bilateral) atrophy of the faces affecting variably the skin, subcutaneous fat tissues, musculature, connective tissue, cartilage and bones. And this disorder is usually accompanied by contralateral Jacksonian epilepsy, trigerminal neuralgia, and changes in the eyes and hair. The onset is slow and progressive, starting at 5-15 years of age and lasting from 2-10 years, ending with the face being "burned out". There are a few cases of this disease which presented during the neonatal period. This disorder seems to affect females more than males, and its etiology and incidence has yet to be determined. Trauma, infection with a slow virus, sympathetic dysfunction, immunological abnormality and cranial vascular malformation are proposed causes. No typical or consistent neuropathologic findings occur. No specific treatment for the syndrome exists; however, various reconstructive surgical procedures can have in reasonably good cosmetic effects, as well as antiinflammatory or immunosuppressive treatment. We report a case of Parry-Romberg syndrome, which was presented at 1 month of age, and has progressd to contralateral hemiparesis.
Atrophy ; Cartilage ; Connective Tissue ; Epilepsy ; Facial Hemiatrophy* ; Female ; Hair ; Humans ; Incidence ; Infant, Newborn* ; Male ; Neuralgia ; Paresis ; Reconstructive Surgical Procedures ; Skin ; Subcutaneous Fat ; Vascular Malformations

Multiple symmetric lipomatosis (MSL) is an extremely uncommon disorder. In the medical literatures about 200 cases have been reported. MSL is not associated with other generalized lipomatous disorders, nor are these patient to be necessarily obese. The cause of MSL is unknown. The disorder usually occurs in middle-aged males and there is frequently a history of alcoholism. Some instances of familial occurrence have been reported, but the majority of cases are sporadic. Two cases of MSL are presented.
Humans ; Lipomatosis ; Lipomatosis, Multiple Symmetrical/*diagnosis/pathology ; Male ; Middle Aged

PURPOSE: Enterobacter is one of the important organisms in neonatal intensive care unit. We reviewed the clinical characteristics, underlying diseases, invasive procedures during admission, mortality and antibiotic sensitivity of Enterobacter infection in NICU. METHODS: We retrospectively reviewed 21 neonatal patients whose blood cultures yielded Enterobacter between June 1994 and June 1997 at Dongguk University Hospital. RESULTS: Blood cultures were positive in 62 from 2,025 neonates and 21 was Enterobacter. The clinical spectrums were diverse such as sepsis (85%), pneumonia, disseminated intravascular coagulopathy and necrotizing enterocolitis. The underlying conditions upon admission were composed of prematurity (38%), hyaline membrane disease (38%), jaundice, sepsis and pneumonia. The procedures used during admission were endotracheal intubation (57%), mechanical ventilation (57%), umbilical vessel catheterization, gastric tube inserition, total parenteral nutrition and exchange transfusion. The antibiotic sensitivity was as follows : ampicillin (0%), ceftriaxone (0%), amikacin (55%), gentamicin (85%), ciprofloxacin (100%), imipenem (100%). Overall mortality was 26.5%. Mortality was significantly high in cases of leukopenia (P< or = 0.01), thrombocytopenia (P< or = 0.01) and use of inappropriate antibiotics (P< or = 0.01). CONCLUSION: Enterobacter is an important organism in the cause of nosocomial infection in NICU and has a high rate of mortality. Enterobacter infection was associated with prolonged hospitalization, invasive procedures and preceding antibiotics. Commonly used antibiotics such as penicillin and cephalosporin would be inappropriate for the treatment of Enterobacter infection. We consider the use of gentamicin or imipenem to be far more effective in the initial therapy of Enterobacter infection.
Amikacin ; Ampicillin ; Anti-Bacterial Agents ; Bacteremia* ; Catheterization ; Catheters ; Ceftriaxone ; Ciprofloxacin ; Cross Infection ; Enterobacter* ; Enterocolitis, Necrotizing ; Gentamicins ; Hospitalization ; Humans ; Hyaline Membrane Disease ; Imipenem ; Infant, Newborn ; Intensive Care, Neonatal* ; Intubation, Intratracheal ; Jaundice ; Leukopenia ; Mortality ; Parenteral Nutrition, Total ; Penicillins ; Pneumonia ; Respiration, Artificial ; Retrospective Studies* ; Sepsis ; Thrombocytopenia

Primary aortoenteric fistula (PAEF) is a rare but catastrophic cause of gastrointestinal bleeding. The diagnosis of PAEF is difficult to make. The classic triad of symptoms, i.e. gastrointestinal bleeding, abdominal pain, and a pulsating abdominal mass is overemphasized, as it occurs in less than 11~25% of the patients. For two thirds of the patients, the diagnosis is made in the operating room. Endoscopic and radiographic studies are very helpful, but the absence of abnormalities can not exclude the diagnosis. PAEF is a clinical and surgical challenge associated with high mortality. A high index of suspicion, early diagnosis and prompt and appropriate surgical intervention are essential to patient survival. Two primary aortoenteric fistulas (aortoduodenal and aortoesophageal) cases are presented and the related literature are reviewed.
Abdominal Pain ; Aorta, Abdominal ; Aortic Aneurysm, Abdominal ; Early Diagnosis ; Fistula ; Gastrointestinal Hemorrhage ; Hemorrhage ; Humans ; Intestinal Fistula ; Operating Rooms ; Vascular Fistula

BACKGROUND: The prevalence and prognostic value of overt disseminated intravascular coagulation (DIC) in patients with septic shock presenting to emergency departments (EDs) is poorly understood, particularly following the release of a new definition of septic shock. The purpose of this study was to investigate the prevalence and prognostic value of DIC in septic shock. METHODS: We performed retrospective review of 391 consecutive patients with septic shock admitting to the ED of tertiary care, university-affiliated hospital during a 16-month. Septic shock was defined as fluid-unresponsive hypotension requiring vasopressor to maintain a mean arterial pressure of 65 mmHg or greater, and serum lactate level ≥ 2 mmol/L. Overt DIC was defined as an International Society on Thrombosis and Hemostasis (ISTH) score ≥ 5 points. The primary endpoint was 28-day mortality. RESULTS: Of 391 patients with septic shock, 290 were included in the present study. The mean age was 65.6 years, the 28-day mortality rate was 26.9%, and the prevalence of overt DIC was 17.6% (n = 51) according to the ISTH score. The median DIC score was higher in non-survivors than in survivors (5.0 vs. 2.0, p = 0.001). Significant higher risk of mortality was observed in overt DIC patients compared to those without (28.2% vs. 13.7%, p = 0.005). Multivariable logistic regression analysis identified DIC to be independently associated with 28-day mortality (odds ratio, 2.689 [95% confidence interval, 1.390-5.201]). CONCLUSIONS: Using the ISTH criteria of DIC, overt DIC in septic shock was found to be common among patients admitting to the ED and to be associated with higher mortality when it is accompanied with septic shock. Efforts are required to identify presence of overt DIC during the initial treatment of septic shock in patients presenting the the ED.
Arterial Pressure ; Consensus* ; Dacarbazine ; Disseminated Intravascular Coagulation* ; Emergencies* ; Emergency Service, Hospital* ; Hemostasis ; Humans ; Hypotension ; Lactic Acid ; Logistic Models ; Mortality ; Prevalence* ; Retrospective Studies ; Shock, Septic* ; Survivors ; Tertiary Healthcare ; Thrombosis

The ‘32-year-old sailor’ patient was a native Korean male, infected with HIV through heterosexual contacts in the high risk areas of foreign country, mostly during. 1984 to 1986. During the following 6-months until his death, the skin nodules suggesting Kaposi's sarcoma, and penile ulcers like chancroid were gradually disappeared. Several skin diseases such as condyloma accuminatum, generalized xeiosis of the skin, acquired icthyosis, seborfheic dermatitis, diffuse hair loss and alopecia developed. His health gradually deteriorated with general symptom such as weakness, weight loss, remAtmt mild fever cough with sputum, and dyspnea with frequent pneumonia signs. The tongue became coated white and thick in which candida albicans was detected, and sore throat and dysphagia accompanied. The patients expired suddenly with acute respiratory distress syndrome, which was may be caused by a opportunistic infection of Pneumocystis carinii. In post-mortem autopsy studies, most of the lung tissue showed the findings of severe interstitial pneumonia and hyaline membrane formation, and pneumocystis cadnii and candida albicans were found in histopathological or micorbiological examination. As other infections, add fast bacilli suggesting Mycobacterium avium-intracellulare were found out in the lymph node, spleen, liver. Cytomegalovirus infection was found on the adrenal gland by histopathological examination. And various lymphoid tissue of the lymph node, spleen, thymus, respiratory tract, gastomtestinal tracts showed severe lymphoid depletion with fibrosis. The other findings had multiple glial nodules in the cerebral white matter and focal segmental glomerulosderosm There wasr t any evidence of Kaposi's sarcoma.
Adrenal Glands ; Alopecia ; Autopsy* ; Candida albicans ; Chancroid ; Cough ; Cytomegalovirus ; Cytomegalovirus Infections ; Deglutition Disorders ; Dermatitis ; Dyspnea ; Fever ; Fibrosis ; Hair ; Heterosexuality ; HIV ; Humans ; Hyalin ; Liver ; Lung ; Lung Diseases, Interstitial ; Lymph Nodes ; Lymphoid Tissue ; Male ; Membranes ; Mycobacterium avium Complex ; Opportunistic Infections ; Pharyngitis ; Pneumocystis ; Pneumocystis carinii ; Pneumonia ; Respiratory Distress Syndrome, Adult ; Respiratory System ; Sarcoma, Kaposi ; Skin ; Skin Diseases ; Spleen ; Sputum ; Thymus Gland ; Tongue ; Ulcer ; Weight Loss ; White Matter

PURPOSE: Recent studies have reported the association of obesity and advanced stage prostate cancer. We researched the association between body mass index(BMI), which is widely used to diagnose obesity, and the prostate-specific antigen(PSA) screening test for prostate cancer. METERIALS AND METHODS: The authors examined the association between BMI and PSA for 26,193 men who visited the health promotion center at our medical center from July 1998 to June 2004. The BMI(weight in kg/height in m2) was calculated from the measured height and weight and this was categorized as follows: BMI<18.5(underweight), BMI 18.5-23.0(normal weight), BMI 23.0-27.5(overweight), BMI>27.5(obesity). We measured the mean PSA value of each BMI category. After adjust for age, we evaluated the PSA according to BMI by using error bars and 95% confidence intervals. RESULTS: The patients' age distribution was from 20 to 90 years old and their mean age was 46.1. The mean PSA value increased in a linear fashion with an increase in the age category(p<0.001), while it decreased in a linear fashion with an increase in the BMI category(p<0.001). After adjusting for age, the mean PSA value also decreased in a linear fashion with an increase in the BMI category(p<0.001). CONCLUSIONS: Men with an elevated BMI tend to have a lower value of PSA and to be misdiagnosed with prostate cancer. The PSA value should considered in relation to age and the BMI for the early detection of prostate cancer.
Age Distribution ; Aged, 80 and over ; Body Mass Index* ; Health Promotion ; Humans ; Male ; Mass Screening ; Obesity ; Prostate-Specific Antigen* ; Prostatic Neoplasms

PURPOSE: Intravenous gamma globulin(IVGG) treatment has reduced symptoms and complications in Kawasaki disease(KD). However, fever persisted in 20-30% of the patients, and there are no reliable data on the indication and dosage of IVGG re-treatment. Therefore, we tried to reveal the effectiveness of IVGG re-treatment and to find risk factors in predicting the re-treatment. METHODS: Among 57 patients with typical KD, 47(82.5%) patients were put into group A, which improved after the treatment with standard 2g/kg of IVIG. 10(17.5%) patients were put into group B, which retreated with 1-2g/kg of IVIG due to persistent fever for at least 3 days after standard IVIG therapy. We compared clinical symptoms, laboratory findings and echocardiograms between group A and B, retrospectively. RESULTS: All patients in group B responded IVGG re-treatment and no considerable side effects. The total duration of the fever was significantly longer(P<0.001) and the initial and peak levels of CRP and the peak levels of ESR were significantly higher(P<0.01) in group B compared to group A. Even though leukocytosis, cervical lymphadenitis and coronary artery aneurysm were more frequent, and the levels of serum lipids at admission were lower in group B, without significance. CONCLUSION: IVGG re-treatment appeared to be effective in the treatment of refractory KD, but could not reduce the incidence of coronary artery aneurysm. We concluded it was difficult to predict risk factors for IVGG re-treatment from these data. Further studies are needed to determine the indication and appropriate dosage of IVGG re-treatment.
Aneurysm ; Coronary Vessels ; Fever ; gamma-Globulins* ; Humans ; Immunoglobulins, Intravenous ; Incidence ; Leukocytosis ; Lymphadenitis ; Mucocutaneous Lymph Node Syndrome* ; Retrospective Studies ; Risk Factors

PURPOSE: Forecasting the demand for Emergency Physicians (EP) is a long, complex problem involving many variables. Although Korean Emergency Medicine Law has guidelines for the minimal numbers of EPs, it doesn't take into account the number of patients or the severity of their symptoms. Previous research involved rough estimates of the number of physicians for Emergency Departments (ED), without any consideration for workload. Our study objective was to determine whether there are an adequate number of EPs by estimating the number of EPs while considering their workload and working hours, according to the 2010 Korean Emergency Physician Survey (KEPS). METHODS: Data on the workload and working hours of Korean EPs were analyzed. The annual increase in the number of emergency patients and the annual application for residency posts were used to calculate the EP demand. Ideally, all patients must be treated by EPs; one full time equivalent (FTE) was calculated as serving 36 hours per week with two patients per hour (PPH) as the minimum. RESULTS: There were 841 EPs working in EDs and 463 EDs across Korea in 2010. A total of 635(75.5%) EPs were clinically active. EPs spent 65.6% of their working hours on clinical duty. Ideally, there should have been 2,871 FTEs and 5,797 EPs needed in 2010. CONCLUSION: There is an absolute shortage of EPs and it will get worse. The excessive hours and workloads of EPs threaten the safety of patients. Therefore, EP demand and forecasting should be focused on increasing patient coverage by EPs.
Emergencies ; Emergency Medicine ; Forecasting ; Humans ; Internship and Residency ; Jurisprudence ; Korea

BACKGROUND: Many laboratories use 4 delta check methods: delta difference, delta percent change, rate difference, and rate percent change. However, guidelines regarding decision criteria for selecting delta check methods have not yet been provided. We present new decision criteria for selecting delta check methods for each clinical chemistry test item. METHODS: We collected 811,920 and 669,750 paired (present and previous) test results for 27 clinical chemistry test items from inpatients and outpatients, respectively. We devised new decision criteria for the selection of delta check methods based on the ratio of the delta difference to the width of the reference range (DD/RR). Delta check methods based on these criteria were compared with those based on the CV% of the absolute delta difference (ADD) as well as those reported in 2 previous studies. RESULTS: The delta check methods suggested by new decision criteria based on the DD/RR ratio corresponded well with those based on the CV% of the ADD except for only 2 items each in inpatients and outpatients. Delta check methods based on the DD/RR ratio also corresponded with those suggested in the 2 previous studies, except for 1 and 7 items in inpatients and outpatients, respectively. CONCLUSIONS: The DD/RR method appears to yield more feasible and intuitive selection criteria and can easily explain changes in the results by reflecting both the biological variation of the test item and the clinical characteristics of patients in each laboratory. We suggest this as a measure to determine delta check methods.
Alanine Transaminase/blood ; Alkaline Phosphatase/blood ; Aspartate Aminotransferases/blood ; Bilirubin/blood ; Blood Urea Nitrogen ; Chemoembolization, Therapeutic ; Clinical Chemistry Tests/methods/*standards ; Creatine/blood ; *Decision Trees ; Humans ; Reference Values ; Renal Dialysis ; Uric Acid/blood