Hereditary anhidrotic ectodermal Dysplasia is a congenital disease displaying characteristics of anhidrosis, hypotrichosis and dental defect which are caused by developmental anomaly of ectodermal epidermis and its appendages. We experienced two cases of hereditary anhidrotic ectodermal dysplasia in two-year and four-month old twin brothers. These patients suffered from intermittent high fever early in life which brought them to our clinical attention. However the diagnosis of anhidrotic ectodermal dysplasia was not suspected by means physicians who cared the patients previously. The diagnosis was made on the basis of clinical features, and confirmed by starch iodine sweat test and skin biopsy on the palm and axilla. We report the two cases in a twin brothers with brief review of related literatures.
Axilla ; Biopsy ; Diagnosis ; Ectoderm ; Ectodermal Dysplasia* ; Epidermis ; Fever ; Humans ; Hypohidrosis ; Hypotrichosis ; Iodine ; Siblings ; Skin ; Starch ; Sweat ; Twins*

No abstract available.
Mycobacterium tuberculosis* ; Mycobacterium*

Pena-Shokeir I syndrome is a multiple malformation syndrome displaying characteristics of camptodactyly, multiple ankylosis, severe muscle weakness, facial anomalies (low set ears, hypertelorism, depressed tip of nose), polyhydramnios, fetal growth retardation & pulmonary hypoplasia which are inherited by autosomal recessive trait. We experienced 1 case of Pena-Shokeir I syndrome in a neonate (41 weeks, 2.08Kg). This patient suffered from dyspnea. Respiratory destress was not relieved after ventilatory care. He died aged 10 days. We report this case with brief review of literature.
Ankylosis ; Dyspnea ; Ear ; Fetal Growth Retardation ; Humans ; Hypertelorism ; Infant* ; Infant, Newborn ; Muscle Weakness ; Polyhydramnios

Objective: To compare preventable trauma death rates (PTDRs) in patients with traumatic brain injury before and after the establishment of a regional trauma center (RTC) at a single center. Methods: Our institution established an RTC in 2014. A total of 709 patients were enrolled from January 2011 to December 2013 (before RTC) and 672 from January 2019 to December 2021 (after RTC). The revised trauma score, injury severity score, and trauma and injury severity score (TRISS) were evaluated. Definitive preventable (DP), possibly preventable (PP), and non-preventable deaths were defined as TRISS >0.5, TRISS 0.25–0.5, and TRISS <0.25, respectively. PTDR was the proportion of deaths from DP+PP out of all deaths, and the preventable major trauma death rate (PMTDR) was the proportion of deaths from DP+PP out of all DP+PP. Results: The overall mortality rates before and after the establishment of RTC were 20.3 and 13.1%, respectively. PTDR was lower after the establishment of RTC than before (90.3% vs.79.5%). The PMTDR was also lower after the establishment of RTC than before (18.8% vs.9.7%). The ratio of direct hospital visits was higher in patients before the establishment of RTC than in those after (74.9% vs. 61.3%, p<0.001). Conclusion Establishing the RTC reduced PTDRs. Additional studies on factors associated with PTDR reduction are required.

BACKGROUND AND OBJECTIVES : Ageotrophic nystagmus in the horizontal canal BPPV has been explained as a result of cupulolithiasis theory, and has been reported to have the less therapeutic response to conservative rehabilitations than the other type BPPV . Though methods to detach the debris with vibrator have been introduced, the effect has been questioned and it's not physiologic. MATERIALS AND METHOD : We introduce a new head shaking-forced prolonged position method as a more convenient method, and report typical 2 cases of ageotrophic horizontal canal BPPV managed with it and analyzed results of 25 cases all told. RESULTS AND CONCLUSION : The average number of rehabilitation was less than two, and loss of direction changing positional nystagmus could be observed immediately after rehabilitation.
Head ; Nystagmus, Physiologic ; Rehabilitation ; Vertigo*

PURPOSE: The aim of this study was to evaluate the effectiveness and indication of radiofrequency ablation(RFA) using renal VX2 tumors by implantation of VX2 tumor cells under the renal capsule in rabbits. MATERIALS AND METHODS: Ten rabbits were injected with 30-40microliter VX2 tumor cells(1.2x10(7) viable cells/ml) under the renal capsule of the right kidney by right subcostal incision. On the 14th day after the tumor cells were implanted, we checked for the development of renal tumors, and the sizes and shapes(exophytic or central) of the tumors by the use of computed tomography. We performed RFA in the renal VX2 tumors with a 17G StarBurst electrode through kidney exposure. After the first and third day following RFA, renal function was checked. On the third day, we performed CT and harvested the kidneys for gross and microscopic evaluation. RESULTS: We confirmed the development of renal VX2 tumors in nine cases. Tumor shapes were exophytic in seven cases and central in two cases; the mean size of the tumors was 2.1 cm(range, 1.1-3.8cm). In all tumors, RFA was performed. From the use of enhanced CT after RFA on the third day, all of the lesions treated with RFA showed no enhancement. From the pathological findings, coagulative necroses were seen on all of the lesions treated with RFA. The necrotized tumor size after RFA was not different statistically as measured by CT and a pathological examination (p=0.833) CONCLUSIONS:: In centrally located renal tumors, we experienced thermal injury in pelvocalyceal systems. RFA is an effective method for nephron sparing surgery as the tumor cells completely disappear and there is preserved renal function and the procedure is easy to apply. We suggest that the RFA method for exophytic renal tumors is more effective than other procedures.
Rabbits ; Animals

No abstract available.
Erythromycin* ; Gastric Emptying* ; Gastroparesis*

No abstract available.
Liver Cirrhosis* ; Liver*

In published article by Li et al., an author's name was misspelled.

BACKGROUND AND OBJECTIVES: The clinical outcome of patient with an acute myocardial infarction (AMI) undergoing percutaneous coronary intervention (PCI), with or without achievement of target low density lipoprotein-cholesterol (LDL-C), has little known information. This study investigated if target LDL-C level (below 70 mg/dL) achievements in patients with AMI showed better clinical outcomes or not. SUBJECTS AND METHODS: Between May 2008 and September 2012, this study enrolled 13473 AMI patients in a large-scale, prospective, multicenter Korean Myocardial Infarction (KorMI) registry. 12720 patients survived and 6746 patients completed a 1-year clinical follow up. Among them 3315 patients received serial lipid profile follow-ups. Propensity score matching was applied to adjust for differences in clinical baseline and angiographic characteristics, producing a total of 1292 patients (646 target LDL-C achievers vs. 646 non-achievers). The primary end point was the composite of a 1-year major adverse cardiac event (MACE) including cardiac death, recurrent myocardial infarction (MI), target lesion revascularization (TLR) and coronary artery bypass grafting. RESULTS: After propensity score matching, baseline clinical and angiographic characteristics were similar between the two groups. Clinical outcomes of the propensity score matched patients who showed no significant differences in cardiac death (0.5% vs. 0.5%, p=1.000), recurrent MI (1.1% vs. 0.8%, p=0.562), TLR (5.0% vs. 4.5%, p=0.649), MACEs (6.5% vs. 5.9%, p=0.644) and stent thrombosis (2.5% vs. 1.9%, p=0.560). CONCLUSION: In this propensity-matched comparison, AMI patients undergoing PCI with a target LDL-C (below 70 mg/dL) achievement did not show better clinical outcomes.
Coronary Artery Bypass ; Death ; Follow-Up Studies ; Humans ; Myocardial Infarction* ; Percutaneous Coronary Intervention ; Propensity Score ; Prospective Studies ; Stents ; Thrombosis ; Treatment Outcome