We report herein two cases of pachyonychia congenita in 19-month-old and 7-year-old girls. In addition to severely deformed nails, both had white plaques on the tongue and keratotic papules on the elbows and knees. In the 19-month-old girl, her father showed deformity of the nails and severe hyperkeratosis of the palms and soles, The family history was normal in other case.
Child ; Congenital Abnormalities ; Elbow ; Fathers ; Female ; Humans ; Infant ; Knee ; Nails, Malformed* ; Pachyonychia Congenita* ; Tongue

The author observed clinical study and response of -interferon treatment of 43 cases of chronic hepatitis who were admitted to Department of Pediatrics Pusan National University College of Medicine during the period of 4 year 6 months from Jenuary, 1988 to Jun, 1992. The results were as follows: 1) Of 43 patients with chronic hepatitis, CPH was 17 cases (39.5%) and CAH was 26 cases (60.5%). 2) Of 43 patients, chronic hepatitis caused by HBV was 37 cases (86.0%) 2 cases were by Wilson's disease, 1 case was by glycogen storage disease and in the 3 cases, the cause was undetermined and CPH by HBV and CAH were 14 cases (82.4%), 23 cases (88.5%) respectively. 3) A peak frequency (46.5%) of chronic hepatitis occurred between 6 to age and male predominated in a propotion of 3.5:1. 4) On the clinical manifestation, jaundice and hepatomegaly was higher frequency on CAH than on CPH. 5) On the laboratory findings, CAH had higher serum ALT and direct bilirubin level than CPH. 6) On effect of -interferon treatment for patients of CAH, serum ALT and AST levels normalized in 81.8% of patients, negative conversion of HBeAg was 38.5%, Anti-HBe serocoversion was 7.7%, negative conversion of HBsAg was 7.7% and the clearnce of HBV-DNA from seum showed 60.0%. 7) The HBeAg seroconversion (28.6%) on children of HBsAg(+) mother showed lower reponse than HBeAg seroconversion (50.0%) of HBsAg(-) mother after -interferon treatment for patients of CAH.
Bilirubin ; Busan ; Child* ; Glycogen Storage Disease ; Hepatitis B e Antigens ; Hepatitis B Surface Antigens ; Hepatitis B, Chronic* ; Hepatitis, Chronic* ; Hepatolenticular Degeneration ; Hepatomegaly ; Humans ; Jaundice ; Male ; Mothers ; Pediatrics

BACKGROUND: Chloral hydrate (CH) is the primary agent most commonly used for pediatric sedation prior to diagnostic, therapeutic procedures. In the management of pediatric facial laceration, the initial dose of CH has to balance the need for adequate sedation against the need to minimize sedative complications. METHODS: A retrospective review of medical records of 834 children who visited our emergency room for facial lacerations from August 2010 to September 2012 was conducted. They were divided into six groups on the basis of the initial dose of CH administered. Further, each group was compared with the standard group (70 to < or =80 mg/kg) with respect to sedation success, augmentation dose, failed sedation, time to procedure, and time of stay. RESULTS: With respect to the complication rate, only group 1 (range, 40 to < or =50 mg/kg) showed a significantly lower complication rate. In the case of all the other variables considered, there were no significant differences among any of the groups. CONCLUSIONS: An initial CH dose of 48+/-2 mg/kg does not negatively affect the success rate of sedation or the need for additional sedative during the primary closure of facial lacerations in pediatric patients. Further, lower doses reduce the incidences of adverse effects and do not delay procedure readiness. Therefore, 48+/-2 mg/kg of CH can be considered the optimal initial dose for pediatric sedation.
Child ; Chloral Hydrate* ; Conscious Sedation ; Emergency Service, Hospital ; Humans ; Incidence ; Infant ; Lacerations* ; Medical Records ; Retrospective Studies ; Sutures

Total 87 patients were divided into 4 different dosage and try to observe what was affected to cardiovascular system depends on the increasing the plasma ionized cakium concentration (3% calcium chloride; 4 mg/kg, as its double 8 mg/kg and 10% calcium gluconate; 14 mg/kg, as its double 28 mg/kg). The results were as follows;, 1) Though using double dosage of 3% calcium chloride and 10% calcium gluconate, could not get to increase as double of plasma ionized calcium concentration. 2) Plasma ionized calcium concentration was increased to the highest level on 1 minute after intravenous administration with each dosage and after increasing to highest level was show to be decreased gradually during 30 minutes after injection. 3) Heart rate was decreased until 30 minute after injection with each dosage of 10% calcium gluconate with statistically significancy (p<0.05) but when 3% calcium chloride were used, the change of heart rate following increase of calcium concentration have not any statistically signi- ficancy. 4) When 3% calcium chloride and 10% calcium gluconate were used, mean arterial pressure (MAP) and increasing of plasma ionized calcium concentration have not any statistically signi- ficancy. 5) Changing of cardiac index (CI) following plasma ionized calcium concentration, have only statistically significancy after each dosage of 10% calcium gluconate was injected but each dosage of 3% calcium chloride have not any statistically significancy respectively. With the above results, equivalent dosage of 3% calcium chloride and 10% calcium gluconate were increased as similar change of plasma ionized calcium concentration but 10% calcium gluconate 14 mg/kg, 28 mg/kg are only have statistically significany between the change of PR, CI and increasing plasma ionized calcium concentration (p<0.05).
Administration, Intravenous ; Arterial Pressure ; Calcium Chloride* ; Calcium Gluconate* ; Calcium* ; Cardiovascular System* ; Heart Rate ; Humans ; Injections, Intravenous* ; Plasma*

Total 87 patients were divided into 4 different dosage and try to observe what was affected to cardiovascular system depends on the increasing the plasma ionized cakium concentration (3% calcium chloride; 4 mg/kg, as its double 8 mg/kg and 10% calcium gluconate; 14 mg/kg, as its double 28 mg/kg). The results were as follows;, 1) Though using double dosage of 3% calcium chloride and 10% calcium gluconate, could not get to increase as double of plasma ionized calcium concentration. 2) Plasma ionized calcium concentration was increased to the highest level on 1 minute after intravenous administration with each dosage and after increasing to highest level was show to be decreased gradually during 30 minutes after injection. 3) Heart rate was decreased until 30 minute after injection with each dosage of 10% calcium gluconate with statistically significancy (p<0.05) but when 3% calcium chloride were used, the change of heart rate following increase of calcium concentration have not any statistically signi- ficancy. 4) When 3% calcium chloride and 10% calcium gluconate were used, mean arterial pressure (MAP) and increasing of plasma ionized calcium concentration have not any statistically signi- ficancy. 5) Changing of cardiac index (CI) following plasma ionized calcium concentration, have only statistically significancy after each dosage of 10% calcium gluconate was injected but each dosage of 3% calcium chloride have not any statistically significancy respectively. With the above results, equivalent dosage of 3% calcium chloride and 10% calcium gluconate were increased as similar change of plasma ionized calcium concentration but 10% calcium gluconate 14 mg/kg, 28 mg/kg are only have statistically significany between the change of PR, CI and increasing plasma ionized calcium concentration (p<0.05).
Administration, Intravenous ; Arterial Pressure ; Calcium Chloride* ; Calcium Gluconate* ; Calcium* ; Cardiovascular System* ; Heart Rate ; Humans ; Injections, Intravenous* ; Plasma*

PURPOSE: Recent studies have suggested that specific single-nucleotide polymorphisms (SNPs) contribute to the clinical features of benign prostatic hyperplasia (BPH). In this study, we investigated the relationships of genetic polymorphisms of the epidermal growth factor (EGF) gene and the epidermal growth factor receptor (EGFR) gene with BPH. METHODS: A total of 218 patients with BPH were enrolled in this study. We evaluated the relationship between eight SNPs in the EGF and EGFR genes and prostate volume, prostate-specific antigen (PSA), and International Prostate Symptom Score of BPH patients. Each SNP was genotyped by direct sequencing. Statistical analysis applying codominant, dominant, recessive, and log-additive models was performed via logistic regression. RESULTS: The rs11568943 and rs11569017 SNPs in the EGF gene showed significant associations with prostate volume (rs11568943: P=0.038 in the log-additive model, P=0.024 in the allele distribution; rs11569017, P=0.031 in the dominant model, P=0.028 in the log-additive model, P=0.020 in the allele distribution). Additionally, the rs3756261, rs11568943, and rs11569017 SNPs of the EGF gene and the rs2293347 SNP of the EGFR gene were associated with PSA levels (P<0.05 in each model, respectively). CONCLUSIONS: These results suggest that the EGF gene may affect prostate volume. In addition, the EGF and EGFR genes may be associated with PSA levels in patients with BPH.
Alleles ; Epidermal Growth Factor* ; Genes, erbB-1 ; Humans ; Logistic Models ; Polymorphism, Genetic ; Polymorphism, Single Nucleotide ; Prostate ; Prostate-Specific Antigen ; Prostatic Hyperplasia* ; Receptor, Epidermal Growth Factor*

No abstract available.
Ecthyma ; Humans ; Infant

PURPOSE: To analyze localized RNFL defect cases that were identified in retinal nerve fiber layer (RNFL) fundus photographs but not in optical coherence tomography (OCT). METHODS: Analysis of OCT scans and images was performed for 14 eyes (17 locations) that showed localized RNFL defects in RNFL fundus photographs but not in RNFL thickness average analysis. RESULTS: With respect to the range of RNFL defects, 41.2% were less than 10degrees, 47.0% were 11 to 20degrees, and 11.8% were 21 to 30degrees. In 71.4% of the RNFL cases the defects were less than 10degrees and the decrease of RNFL thickness was not readily observable on the OCT scan images. In all cases of RNFL defects in the 11 to 30degrees range the decrease in RNFL thickness could be assessed on the OCT scan images. Nonetheless, the decrease of RNFL thickness could not be seen on the OCT analysis images in which the results of the RNFL thickness made through an automated computer algorithm were displayed. CONCLUSIONS: The range of localized RNFL defects that were difficult to detect with OCT consisted of those cases that were almost less than 20degrees. The limitations of the OCT scan itself in patients with RNFL with an angular width defect less than 10degrees and the problems of RNFL thickness analysis processing in patients with an angular width of 11 to 30degrees may decrease the sensitivity of OCT in diagnosing RNFL defects.
Eye ; Humans ; Nerve Fibers ; Retinaldehyde ; Tomography, Optical Coherence

Sarcoidosis is a multisystem granulomatous disorder commonly affecting young adults. Diagnosis is confirmed by evidence of non-caseating granuloma in more than one organ. A case is presented where the diagnosis was made on a bone marrow biopsy, the first case in Korea. A 54-year-housewife was admitted because of multiple variable sized, skin papules and macules, who showed pancytopenia and evidence of liver cirrhosis on ultrasound. We performed skin biopsy and bone marrow biopsy, which showed non-caseating granulomas. The chest CT, opthalmologic examination was performed and showed the involvement of sarcoidosis of mediastinal lymph nodes and uvea. Multiple joints of both hands and feet also seemed to be involved in view of simple X-ray. By this case, we emphasize the significance of bone marrow biopsy in the sarcoidosis with pancytopenia.
Biopsy ; Bone Marrow* ; Diagnosis ; Foot ; Granuloma ; Hand ; Humans ; Joints* ; Korea ; Liver Cirrhosis ; Liver* ; Lymph Nodes ; Pancytopenia ; Sarcoidosis* ; Skin* ; Tomography, X-Ray Computed ; Ultrasonography ; Uvea* ; Young Adult

Necrotizing sarcoid granulomatosis was first described by Liebow, who included it in the category of pulmonary angiitis and granulomatosis. Wegener's granulomatosis and limited Wegener's granulomatosis are also included in this category. Necrotizing sarcoid granulomatosis is characterized by histologically sarcoid-like granulomata, prominent and granulomatous vasculitis, and varying degrees of necrosis, radiographically pulmonary nodules but no enlarged hilar lymph nodes, clinically benign course. Whether this entity is a variant of necrotizig vasculitis with sarcoid reaction or sarcoidosis with prominent vascultic features and necrosis is not yet, clear, but many authors suggest the relationship with sarcoidosis from the observation of the similarity of histologic features and clinical course between the two entities. Patients are asymptomatic in about one fourth. Even symptomatic patients have vague symptoms such as cough, chest pain. Extrapulmonary involvement is rare. The prognosis is good even without therapy or with steroid alone, if necessary. We report a case that was diagnosed by open lung biopsy as necrotizing sarcoid granulomatosis, and it is assumed to be the first report of this entity in Korea.
Biopsy ; Chest Pain ; Cough ; Humans ; Korea ; Lung ; Lymph Nodes ; Necrosis ; Prognosis ; Sarcoidosis ; Vasculitis ; Wegener Granulomatosis