The Effect of Combined Growth Hormone and a Gonadotropin-Releasing Hormone Agonist Therapy on Height in Korean 3-M Syndrome Siblings

3-M syndrome is a rare autosomal recessive growth disorder characterized by severe growth retardation, low birth weight, characteristic facial features, and skeletal anomalies, for which three causative genes (CUL7, OBSL1, and CCDC8) have been identified. We herein report two Korean siblings with 3-M syndrome caused by two novelOBSL1 mutations, and describe the effect of a combined treatment with growth hormone (GH) and a gonadotropin-releasing hormone (GnRH) agonist. A 7-year-old girl with short stature (-3.37 standard deviation score, SDS) and breast budding presented with subtle dysmorphic features, including macrocephaly, frontal bossing, a triangular face, prominent philtrum, full lips, a short neck, and fifth-finger clinodactyly. GnRH stimulation test revealed a pubertal pattern and advanced bone age of 8 years and 10 months. Her older sister, aged 10 years and 9 months, had experienced an early menarche, and had an advanced bone age (13.5 years) and predicted adult height of 142 cm (-4.04 SDS). Targeted exome sequencing identified that the siblings had two heteroallelic mutations in OBSL1. Both siblings underwent a combination therapy with GH and a GnRH agonist. A height gain was noted in both siblings even after short-term treatment. To fully elucidate the effects of the combined therapy, a larger cohort should be analyzed following a longer treatment period. However, such an analysis would be challenging due to the rarity of this disease.

Bacterial Contamination and Disinfection Status of Laryngoscopes Stored in Emergency Crash Carts / 예방의학회지

OBJECTIVES: To identify bacterial contamination rates of laryngoscope blades and handles stored in emergency crash carts by hospital and area according to the frequency of intubation attempts. METHODS: One hundred forty-eight handles and 71 blades deemed ready for patient use from two tertiary hospitals were sampled with sterile swabs using a standardized rolling technique. Samples were considered negative (not contaminated) if no colonies were present on the blood agar plate after an 18-hour incubation period. Samples were stratified by hospital and according to the frequency of intubation attempts (10 attempts per year) using the χ2-test and Fisher exact test. RESULTS: One or more species of bacteria were isolated from 4 (5.6%) handle tops, 20 (28.2%) handles with knurled surfaces, and 27 (18.2%) blades. No significant differences were found in microbial contamination levels on the handle tops and blades between the two hospitals and two areas according to the frequency of intubation attempts. However, significant differences were found between the two hospitals and two areas in the level of microbial contamination on the handles with knurled surfaces (p<0.05). CONCLUSIONS: Protocols and policies must be reviewed to standardize procedures to clean and disinfect laryngoscope blades and handles; handles should be re-designed to eliminate points of contact with the blade; and single-use, one-piece laryngoscopes should be introduced.

Various autogenous fresh demineralized tooth forms for alveolar socket preservation in anterior tooth extraction sites: a series of 4 cases / 대한악안면성형재건외과학회지

The aim of this study was to evaluate the clinical relevance of autogenous fresh demineralized tooth (Auto-FDT) prepared at chairside immediately after extraction for socket preservation. Teeth were processed to graft materials in block, chip, or powder types immediately after extraction. Extraction sockets were filled with these materials and dental implants were installed immediately or after a delay. A panoramic radiograph and a conebeam CT were taken. In two cases, tissue samples were taken for histologic examination. Vertical and horizontal maintenance of alveolar sockets showed some variance depending on the Auto-FDT and barrier membrane types used. Radiographs showed good bony healing. Histologic sections showed that it guided good new bone formation and resorption pattern of the Auto-FDT. This case series shows that Auto-FDT prepared at chairside could be a good material for the preservation of extraction sockets. This study will suggest the possibility of recycling autogenous tooth after immediate extraction.

A case of Ophthalmic Zoster Presenting as Multiple Cranial Nerve Palsies, Ultimately Leading to Death

No abstract available.

Successful Brain Dead Donor Management with CRRT: A Case Report / 대한구급학회지

Brain death results in adverse pathophysiologic effects in many brain-dead donors with cardiovascular instability. We experienced a brain-dead donor with continuous renal replacement therapy (CRRT) who was in a severe metabolic, electrolyte derangement and poor pulmonary function. The thirty-nine-year-old male patient with subarachnoid hemorrhage and intraventricular hemorrhage was admitted into the intensive care unit (ICU). After sudden cardiac arrest, he went into a coma state and was referred to as a potential organ donor. When he was transferred, his vital sign was unstable even under the high dose of inotropics and vasopressors. Even with aggressive treatment, the level of blood sugar was 454 mg/dl, serum K+ 7.1 mEq/L, lactate 5.33 mmol/L and PaO2/FiO2 60.3. We decided to start CRRT with the mode of continuous venovenous hemodiafiltration (CVVHDF). After 12 hours of CRRT, vital sign was maintained well without vasopressors, and blood sugar, serum potassium and lactate levels returned to 195 of PaO2/FiO2. Therefore, he was able to donate his two kidneys and his liver.

Observation of the change of the dental implant stability and bone density evaluation methods / 대한치주과학회지

PURPOSE: The aim of this study was to examine the correlation of the subjective and the objective evaluation of edentulous ridge bone quality, and to evaluate the change of the dental implant stability in each bone density group for early healing period after implant installation. METHODS:Sixty-seven implants(Osstem implant(R), Seoul, Korea) were included in this study. We evaluated the bone density by 2 methods. The one was the subjective method which was determined by practitioner's tactile sense, the other was the objective bone type was based on Hounsfield units. The implant stability in each bone type group was assessed by resonance frequency analyzer(Osstell mentor(R)). Data were analyzed for the change of the implant stability, and they were compared to verify the difference of groups at the time of installation, 2, 6, 10, 14 weeks postoperatively. Spearman's correlation was used to demonstrate the correlation between the subjective and the objective evaluation of the bone density, and analysis of variance(ANOVA) was used to analyze the differences of implant stability at each time point. RESULTS: There was no close relation between the subjective and the objective evaluation of the bone density(r=0.57). In the subjective groups, there was statistically significant difference between the type 1 and 3 at 10 weeks and between the type 2 and 3 at 14 weeks. In the objective groups, there was no statistically significant difference between the D 1, 2, 3, 4, and 5 group with regard to RFA from baseline to 14 weeks(p>0.1). CONCLUSIONS: The implant stability increased over time during the study, and it was improved with bone density proportionally after 2weeks postoperatively. It is recommended that the decision of bone density is base on Hounsfield unit for implant loading time.