Mutations of the surfactant protein (SP)-C gene (SFTPC) have been associated with neonatal respiratory distress syndrome (RDS) and childhood interstitial lung disease (ILD). If accurate diagnosis and proper management are delayed, irreversible respiratory failure demanding lung transplantation may ensue. A girl was born at term but was intubated and given exogenous surfactant due to RDS. Cough and tachypnea persisted, and symptoms rapidly progressed at 16 months of age despite treatment with antibiotics, oral prednisolone, methylprednisolone pulse therapy, and intravenous immunoglobulin. At 20 months, she visited our hospital for a second opinion. A computed tomography scan showed a diffuse mosaic pattern with ground-glass opacity and subpleural cysts compatible with ILD. A video-assisted thoracoscopic lung biopsy revealed ILD with eosinophilic proteinaceous material and macrophages in the alveolar space. Bilateral lung transplant from a 30-month-old child was done, and she was discharged in room air without acute complications. Genetic analysis revealed a novel c.203T>A, p.Val68Asp mutation of SP-C, based on the same exon as a known pathogenic mutation, p.Glu66Lys.
Anti-Bacterial Agents ; Biopsy ; Child ; Child, Preschool ; Cough ; Diagnosis ; Eosinophils ; Exons ; Female ; Humans ; Immunoglobulins ; Lung Diseases, Interstitial* ; Lung Transplantation* ; Lung* ; Macrophages ; Methylprednisolone ; Prednisolone ; Referral and Consultation ; Respiratory Distress Syndrome, Newborn ; Respiratory Insufficiency ; Tachypnea

BACKGROUND: Many implants are being used for the reconstruction of orbital wall fractures. The effect of the choice of implant for the reconstruction of an orbital wall fracture on the surgical outcome is under debate. The purpose of this article is to compare the outcomes of orbital wall reconstruction of small orbital wall fractures on the basis of the implants used. METHODS: The authors conducted a retrospective study using electronic databases. Between March 2001 and December 2012, 461 patients with orbital wall fractures were included in this study. Among them, 431 patients in whom the fracture size was less than 300 mm2 were analyzed. The fracture size was calculated using computed tomography scans of the orbit in the sagittal and coronal images. Cases in which the fracture size was less than 300 mm2 were included in this study. RESULTS: One hundred and twenty-nine patients were treated with silastic sheets; 238 patients were treated with titanium meshes; and absorbable meshes were used in the case of 64 patients. Overall, 13 patients required revision, and the revision rate was 3.0%. The revision rate of the silastic sheet group was 5.4%. In the multivariable analysis, the revision rate of the group reconstructed with silastic sheets was highly statistically significant (P=0.043, odds ratio=3.65). However, other factors such as age, sex, fracture type, and fracture size were not significant. CONCLUSIONS: Reconstruction of orbital wall fractures with silastic sheets may cause more complications than that with other materials such as titanium meshes and absorbable meshes.
Humans ; Orbit* ; Orbital Fractures ; Retrospective Studies ; Silicones ; Titanium

PURPOSE: Teriparatide markedly increases bone formation and strength, while reducing the incidence of new-onset osteoporotic vertebral compression fractures (OVCFs). In some countries, expenses for teriparatide use are covered by medical insurance for up to 6 months; however, the national medical insurance of the authors' country does not cover these expenses. This retrospective cohort study compared the therapeutic effects of teriparatide on the initial onset of a new OVCF after treatment of osteoporosis and/or related OVCFs with regard to therapeutic durations of longer than 3 months (LT3M) or shorter than 3 months (ST3M). MATERIALS AND METHODS: From May 2007 to February 2012, 404 patients who were prescribed and administered teriparatide and who could be followed-up for longer than 12 months were enrolled. They were divided into two groups depending on teriparatide duration: LT3M (n=132) and ST3M (n=272). RESULTS: The group with the teriparatide duration of LT3M showed significantly less development of an initial OVCF within 1 year (p=0.004, chi-square). Duration of teriparatide use, body mass index, pre-teriparatide lowest spinal bone mineral density, and severity of osteoporosis significantly affected multiple regression analysis results (p<0.05). Survival analysis of first new-onset OVCFs demonstrated a significantly better survival rate for the LT3M group (log rank, p=0.005). Also, the ST3M group showed a higher odds ratio of 54.00 for development of an initial OVCF during follow-up than the LT3M group (Mantel-Haenzel common odds ratio, p=0.006). CONCLUSION: At least one cyclic teriparatide administration is recommended to provide a protective effect against the initial onset of a new OVCF for up to one year after therapy.
Aged ; Aged, 80 and over ; Bone Density/drug effects ; Bone Density Conservation Agents/*administration & dosage/pharmacology ; Cohort Studies ; Drug Administration Schedule ; Female ; Fractures, Compression/*drug therapy/etiology ; Humans ; Incidence ; Male ; Middle Aged ; Osteoporosis/complications ; Osteoporotic Fractures/*drug therapy/etiology ; Retrospective Studies ; Spinal Fractures/*drug therapy/etiology ; Teriparatide/*administration & dosage/pharmacology ; Time Factors ; Treatment Outcome

Few literatures have elaborated on the clinical characteristics of children with thalassemia from low-prevalence areas. A retrospective analysis was conducted on children genetically confirmed with thalassemia at Seoul National University Children's Hospital in Korea. Nine children (1alpha thalassemia trait, 6beta thalassemia minor, 2beta thalassemia intermedia) were diagnosed with thalassemia at median age of 4.3 yr old with median hemoglobin of 9.7 g/dL. Seven (78%) children were incidentally found to be anemic and only 2 with beta thalassemia intermedia had presenting symptoms. Five children (56%) were initially misdiagnosed with iron deficiency anemia. Despite the comorbidities due to alpha thalassemia mental retardation syndrome, the child with alpha thalassemia trait had mild hematologic profile. Children with beta thalassemia intermedia had the worst phenotypes due to dominantly inherited mutations. None of the children was transfusion dependent and most of them had no complications associated with thalassemia. Only 1 child (11%) with codon 60 (T-->A) mutation of the HBB gene needed red blood cell transfusions. He also had splenomegaly, cholelithiasis, and calvarial vault thickening. Pediatricians in Korea must acknowledge thalassemia as a possible diagnosis in children with microcytic hypochromic hemolytic anemia. High level of suspicion will allow timely diagnosis and managements.
Blood Transfusion ; Child ; Child, Preschool ; Female ; Genotype ; Hemoglobin A, Glycosylated/genetics ; Hemoglobin A2/genetics ; Humans ; Male ; Medical Records/statistics & numerical data ; Prevalence ; Republic of Korea/epidemiology ; Retrospective Studies ; alpha-Globins/*genetics ; alpha-Thalassemia/diagnosis/epidemiology/*genetics ; beta-Globins/*genetics ; beta-Thalassemia/diagnosis/epidemiology/*genetics

PURPOSE: The aims of this pilot study were to introduce implant loading devices designed for animal study and to evaluate the validity of the load transmission ability of the loading devices. MATERIALS AND METHODS: Implant loading devices were specially designed and fabricated with two implant abutments and cast metal bars, and orthodontic expansion screw. In six Beagles, all premolars were extracted and two implants were placed in each side of the mandibles. The loading device was inserted two weeks after the implant placement. According to the loading protocol, the load was applied to the implants with different time and method,simulating early, progressive, and delayed loading. The implants were clinically evaluated and the loading devices were removed and replaced to the master cast, followed by stress-strain analysis. Descriptive statistics of remained strain (microepsilon) was evaluated after repeating three cycles of the loading device activation. Statistic analysis was performed using nonparametric, independent t-test with 5% significance level and Friedman's test was also used for verification. RESULTS: The loading devices were in good action. However, four implants in three Beagles showed loss of osseointegration. In stress-strain analysis, loading devices showed similar amount of increase in the remained strain after applying 1-unit load for three times. CONCLUSION: Specialized design of the implant loading device was introduced. The loading device applied similar amount of loads near the implant after each 1-unit loading. However, the direction of the loads was not parallel to the long axis of the implants as predicted before the study.
Animal Experimentation ; Animals ; Axis, Cervical Vertebra ; Bicuspid ; Dental Implants ; Dental Stress Analysis ; Immediate Dental Implant Loading ; Mandible ; Osseointegration ; Pilot Projects ; Sprains and Strains

No abstract available.
Antiviral Agents/therapeutic use ; Ascites/diagnosis/prevention & control/therapy ; Cholagogues and Choleretics/therapeutic use ; Fatty Liver/diagnosis/diet therapy ; Fatty Liver, Alcoholic/diagnosis/drug therapy ; Hemorrhage/prevention & control/therapy ; Hepatic Encephalopathy/diagnosis/prevention & control/therapy ; Hepatitis B, Chronic/diagnosis/drug therapy ; Hepatitis C, Chronic/diagnosis/drug therapy ; Humans ; Liver Cirrhosis/*diagnosis/drug therapy/pathology/*therapy ; Liver Cirrhosis, Biliary/drug therapy ; Vasodilator Agents/therapeutic use

PURPOSE: Given the predictability of dental implant procedure from the studies of successful osseointegration, implant dentistry is often the treatment of choice to replace missing teeth in edentulous patient instead of the fixed prosthesis or removable denture. The Renova(R) dental implant has a RBM(Resorbable Blast Media) surface, internal hex prosthetic connection and a tapered design. At this study gives the analysis of the implant and the short term survival rate of the implant. MATERIAL AND METHODS: In this study, a multilateral analysis was performed on the subjects undergoing placement with Renova(R) implant between August 2006 and February 2008 in Yonsei University dental hospital. 96 implants were placed in 56 patients and they were surveyed for cumulative survival rate. Among them 78 implants in 44 patients were surveyed for the rest analyses. RESULT: 1. The cumulative survival rate was 96.88% of 96 implants in 56 patients. 2. The mean marginal bone loss was 0.803mm and the marginal bone loss in augmentation group has higher value than the marginal bone loss in non augmentation group. 3. The health scale for the implants were 87% in success group, 9% in satisfactory survival group, 1% in compromised survival group, and 3% in failure group. 4. Two implants placed in poor bone posterior area by 2-stage failed during prosthetic procedure. CONCLUSION: Renova(R) dental implant showed high cumulative survival rate in installation on partial edentulous ridge and could be a predictable implant system.
Dental Implants ; Dentistry ; Dentures ; Humans ; Osseointegration ; Prostheses and Implants ; Survival Rate ; Tooth

Idiopathic hypertrophic pachymeningitis is a rare inflammatory disorder of the dura mater. Spinal involvement is extremely rare and there are few case reports. We present a 36 year-old female of idiopathic hypertrophic spinal pachymeningitis compressing thoracic spinal cord which showed rapid recurrence.
Dura Mater ; Female ; Humans ; Meningitis ; Recurrence ; Spinal Cord ; Spine

Henoch-Schonlein purpura (H-S purpura) is a systemic small-vessel vasculitis involving skin, joint, gastrointestinal tract, and kidney. It is characterized by the classic tetrad of abdominal pain, arthralgia, typical rash, and renal involvement. All of these clinical findings can occur in any order and at any time over several days to weeks. Gastrointestinal manifestations such as abdominal pain, melena, or hematochezia occur in 45-85% and preceed skin lesions upto 40% in H-S purpura. However, endoscopically proven gastrointestinal lesion is rare because majority of involved sites are small intestine. We report a case of Henoch-Schonlein purpura with terminal ileal ulcer, healed after treatment with high dose steroid, proven by colonoscopy.
Adult ; Anti-Inflammatory Agents/*therapeutic use ; Colonoscopy ; Humans ; Ileal Diseases/*drug therapy/etiology/pathology ; Leg/pathology ; Male ; Prednisolone/*therapeutic use ; Purpura, Schoenlein-Henoch/complications/*diagnosis/drug therapy ; Ulcer/*drug therapy/etiology/pathology

Primary hepatic neuroendocrine cell carcinoma is a very rare tumor. We experienced a 75-year-old woman with primary hepatic neuroendocrine carcinoma presenting with pyogenic liver abscess. Abdominal CT scan revealed a multiseptated liver abscess and an enlarged lymph node in portocaval portion. We performed percutaneous drainage of the liver abscess, but the amount of drained pus did not decrease after 20 days. The follow-up abdominal CT scan showed that the cystic portion of liver abscess had been replaced by the solid tumor. Microscopic examination of the tumor tissue showed nests of epithelial cells with uniform round hyperchromatic nuclei and high nuclear to cytoplasmic ratio. Immunohistochemical staining was strongly positive for synaptophysin and chromogranin A.
Adenine/analogs & derivatives/therapeutic use ; Antiviral Agents/*adverse effects/therapeutic use ; Drug Eruptions/diagnosis/*pathology ; Female ; Hepatitis B, Chronic/*drug therapy ; Humans ; Ichthyosis/chemically induced/pathology ; Lamivudine/*adverse effects/therapeutic use ; Middle Aged ; Phosphonic Acids/therapeutic use