Mental retardation (MR) is classically deficits in adaptive behavior and manifest during the developmental period. The causes of mental retardation were not understood in many cases. This study was undertaken to identify the etiologies of mentally retarded children enrolled in a special educational institution under the hypothesis that clarifying causes of MR can not only provide basic epidemiological data on MR in Korea, but also imply possibly preventable measures to avoid MR in some cases. In this study, complete medical history was taken in addition to a thorough individual physical examination with collection of urine specimens for metabolic screening tests including ferric chloride, DNPH, nitrosonaphthol, nitroprusside, CTAB, and reducing substance tests in 259 mentally retarded children aged between 3 through 18 year old. The cytogenetic, molecular genetic, and endocrine studies wire performed in 14 children with high clinical suspicion of chromosomal abnormalities and congenital hypothyroidism. Dysmorphism syndromes were delineated using computer software software program. Definite or presumptive etiological diagnosis has been made in 122 (47%) mentally retarded children, Among three major identifiable causes, perinatal brain damage resulted from difficulties in labor, prematurity, kernicterus, and neonatal sepsis, was most commonly found in 57 children (22%), followed by chromosomal abnormalities including Down's syndrome and fragile-X syndrome in 35 children (13.5%), and dysmorphism syndrome in 10 children (3.9%) in order. Other identifiable causes for mentally retarded children were listed as autism (2.3%), endocrine & hereditary metabolic disease (1.9%), central nervous system malformations, neurophakomatoses (1.9%), and postnatal accidents, poisoning, infection (1.5%). In conclusion, aforementioned data suggested that one fourth of MR can be avoided or treated by making efforts to improve peri, postnatal care and early detection. Routine urinary metabolic screening tests for inborn errors cannot be justified though CTAB test showed high specificity for the diagnosis of mucopolysaccharidosis. Some mentally retarded children's families need genetic counselling since Mendelian inherited disorders are culpable for causing MR in some children.
Adaptation, Psychological ; Adolescent ; Autistic Disorder ; Brain ; Central Nervous System ; Child* ; Chromosome Aberrations ; Classification* ; Congenital Hypothyroidism ; Cytogenetics ; Diagnosis ; Down Syndrome ; Humans ; Intellectual Disability ; Kernicterus ; Korea ; Mass Screening ; Mentally Disabled Persons* ; Metabolic Diseases ; Molecular Biology ; Mucopolysaccharidoses ; Nitroprusside ; Physical Examination ; Poisoning ; Postnatal Care ; Sensitivity and Specificity ; Sepsis

The National Forensic Hospital is the only forensic psychiatric hospital in Korea. As of January 2016, the average number of patients assigned to each psychiatrist is more than 120. In this situation, the role of psychopharmacologic treatment becomes very important. To investigate the prescription patterns of major psychotropic medications (i.e., antipsychotics, mood stabilizers, and antidepressants), we reviewed the medical records of the patients diagnosed with schizophrenia and schizoaffective disorder who were admitted in January 2016. The data from 418 patients (403 with schizophrenia and 15 with schizoaffective disorder) were retrospectively collected and analyzed. The average number of major psychotropic medications prescribed for each patient during hospitalization was as follows: antipsychotics, 3.5±1.8; mood stabilizers, 0.5±0.7; and antidepressants, 0.5±0.8. The three most frequently prescribed antipsychotics were risperidone (21.7%), olanzapine (15.5%), and quetiapine (14.4%). More than half of the patients (233, 55.9%) were prescribed an antipsychotic polypharmacy regimen. This study found that patients with schizophrenia and schizoaffective disorder in forensic psychiatric hospital tend to be prescribed many psychotropic medications.
Antidepressive Agents ; Antipsychotic Agents ; Forensic Psychiatry ; Hospitalization ; Hospitals, Psychiatric ; Humans ; Inpatients* ; Korea ; Medical Records ; Polypharmacy ; Prescriptions ; Psychiatry ; Psychopharmacology ; Psychotic Disorders* ; Quetiapine Fumarate ; Retrospective Studies ; Risperidone ; Schizophrenia*

Cardiac arrest associated with hyperkalemia during red blood cell transfusion is a rare but fatal complication. Herein, we report a case of transfusion-associated cardiac arrest following the initiation of extracorporeal membrane oxygenation support in a 9-month old infant. Her serum potassium level was increased to 9.0 mEq/L, soon after the newly primed circuit with pre-stored red blood cell (RBC) was started and followed by sudden cardiac arrest. Eventually, circulation was restored and the potassium level decreased to 5.1 mEq/L after 5 min. Extracorporeal membrane oxygenation (ECMO) priming is a relatively massive transfusion into a pediatric patient. Thus, to prevent cardiac arrest during blood-primed ECMO in neonates and infants, freshly irradiated and washed RBCs should be used when priming the ECMO circuit, to minimize the potassium concentration. Also, physicians should be aware of all possible complications associated with transfusions during ECMO.
Blood Transfusion ; Death, Sudden, Cardiac ; Erythrocyte Transfusion ; Erythrocytes ; Extracorporeal Membrane Oxygenation* ; Heart Arrest* ; Humans ; Hyperkalemia* ; Infant* ; Infant, Newborn ; Potassium

BACKGROUND: Heparin salts induce negative proportional bias according to anticoagulant concentration for analysis of ionized calcium (iCa) However, D-phenylalanyl -L-prolyl- L-arginine chloromethyl ketone (PPACK), a selective thrombin inhibitor, do not bind to ionized calcium. Therefore, we evaluated PPACK as an alternative anticoagulant to lithium heparin (Li-Hep) for analysis of ira, blood gases and electrolytes. METHODS: The concentration of iCa in whole blood anticoagulated with heparin was compared with that in serum of patients admitted to Chungbuk National University Hospital (n=27). The blood gases, electrolytes and iCa according to each anticoagulant concentration (Ll-Hep or PFACK) were analyzed. The concentrations of anticoagulated whole blood (Li-Hep; 50 kIU/L, PPACK ; 75 mumol/L) were compared with those of nonanticoagulated whole blood for blood gases, electrolytes and iCa (n=17), RESULTS: The results were as follows; whole blood anticoagulated with Li-Hep demonstrated -0.28+/-0.15 mmol/L (26.6%) bias for ira compared with serum. No bias according to each anticoagulated concentrations were observed in analysis of blood gases, potassium and chloride. Negative proportional bias for iCa and sodium in serum anticoagulated with Li-HeP was observed. In comparison, no bias for ira and sodium was observed with PPACK. No bias was observed in analysis of blood gas or electrolytes with each anticoagulated whole blood except for sodium and chloride that had clinically nonsignificant bias. Whole blood anticoagulated with Li-Hep demonstrated a consistent -0.08+/-0.02 mmol/L (6.3%) bias for ira compared with nonanticoagulated whole blood. In comparison, no bias was observed with PPACK-anticoagulated whole blood for iCa. CONCLUSIONS: We concluded that PPACK is an ideal anticoagulant without bias for analysis of iCa, blood gases and electrolytes.
Arginine ; Bias (Epidemiology) ; Calcium* ; Chungcheongbuk-do ; Electrolytes* ; Gases ; Heparin* ; Humans ; Lithium ; Potassium ; Salts* ; Sodium ; Thrombin

Purpose: To introduce a method of correcting lower eyelid epiblepharon with severe epicanthal fold combined with minimal incision of medial epicanthoplasty (Hiraga method) and to report its clinical outcomes. Methods: We retrospectively reviewed the medical records of patients who underwent surgery for lower eyelid epiblepharon and minimal incision of medial epicanthus from January 2015 and January 2019. Seventy-eight eyes of 39 patients were analyzed for surgical outcomes, subjective symptoms, recurrence, and complications for more than six months of the follow-up period. Results: A total of 39 patients (14 male, 25 female) were included in the analysis. The mean patient age was 8.4 ± 4.3 years, and the mean postoperative follow-up was 14 ± 5.2 months. Eyelid shape and function were well maintained in 39 patients (100%), with no recurrence during follow-up. Conclusions For patients with lower eyelid epiblepharon with severe medial epicanthal fold, lower eyelid epiblepharon with a minimal incision of medial epicanthoplasty (Hiraga method) is a simple surgical technique to prevent recurrence of medial cilia touch, showing successful results without complications.

PURPOSE: Organ transplant recipients are at high risk of developing malignancies due to immunosuppressive regimens. Unlike post-transplant lymphoproliferative diseases (PTLDs), where Epstein-Barr virus (EBV) plays an etiological role, there are conflicting data regarding the association of EBV with post-transplant epithelial malignancies. In order to clarify the role of EBV in carcinomas that develop after solid-organ transplantation, the presence of EBV infection in the carcinomas of post-kidney transplant patients was examined. MATERIALS AND METHODS: The presence of EBV infection in skin carcinoma (PTSC), gastric carcinoma (PTGC) and urothelial carcinoma (PTUC), which developed in the patients under an immune suppression regime following kidney transplantation, was examined. Tumors from the patients without organ transplantation were also used as a comparison in the study. The study group included five nasopharyngeal carcinomas (NPCs), one Hodgkin's disease (HD), one B-cell non-Hodgkin's malignant lymphoma (NHL) and one hypopharynx (HPC) tumor. RESULTS: Immunofluorescence assay and Western blot analysis, using sera from the same patients, confirmed that all of the tested patients were previously infected with EBV. From in situ hybridization, no EBER positive cells were detected in any of the tumor tissues obtained from the three kidney transplant recipients (PTSC, PTGC and PTUC) or in the NHL and HPC tissues. In contrast, all five of the NPC and HD tissues showed strong EBER positivity. CONCLUSION: These results suggest that there is a strong association of EBV with NPC and HD as previously reported, while no such strong association of EBV was found with epithelial malignancies that developed after kidney transplantation.
B-Lymphocytes ; Blotting, Western ; Epstein-Barr Virus Infections ; Fluorescent Antibody Technique ; Herpesvirus 4, Human* ; Hodgkin Disease ; Humans ; Hypopharynx ; In Situ Hybridization ; Kidney Transplantation* ; Kidney* ; Lymphoma ; Organ Transplantation ; Skin ; Transplantation ; Transplants

During administration of anesthesia, intraoperative blood pressure control is very important. Sometimes a hypertensive or anti-hypertensive agent is used in order to maintain blood pressure at an adequate level; alpha-agonist and beta-blocker are drugs frequently used. Alpha-agonists are used in various ways including their application together with local anesthetics during an operation for the purpose of vasoconstriction, to control systemic absorption of local anesthetics, to extend the duration of action, or to reduce bleeding from the surgical site. In addition, alpha-agonists are used in cardiopulmonary resuscitation. Beta-blockers are used widely as a therapeutic agent for hypertension, angina, and arrhythmia, and to lower portal pressure in liver cirrhosis. Here, we are reporting the case of acute pulmonary edema that occurred after the administration of phenylephrine, in order to maintain blood pressure in a 52-year-old female patient with liver cirrhosis. The patient was underwent emergent decompressive craniectomy for intracranial hemorrhage without acknowledging her long-term use of a beta-blocker medication.
Absorption ; Anesthesia ; Anesthetics, Local ; Arrhythmias, Cardiac ; Blood Pressure ; Cardiopulmonary Resuscitation ; Decompressive Craniectomy ; Female ; Hemorrhage ; Humans ; Hypertension ; Intracranial Hemorrhages ; Liver Cirrhosis ; Middle Aged ; Phenylephrine ; Portal Pressure ; Pulmonary Edema ; Vasoconstriction

Methemoglobinemia results from the oxidation of ferrous iron (Fe++) to ferric iron (Fe+++) within hemoglobin. It is caused by various etiologies, including the herbicide Propanil. Patients with low levels of methemoglobin (metHb) are asymptomatic but symptomatic patients and patients with high levels of metHb require treatment. Methylene blue is the first choice for the treatment of methemoblobinemia, but has some complications such as hemolytic anemia and rebound methemoglobinemia. We report the case of a 91-year-old woman who died of aniline herbicides poisoning. The level of metHb in her blood was initially 20.7% and her mental status was drowsy. She was intravenously treated with methylene blue within a therapeutic range for methemoglobinemia. After treatment with methylene blue, the level of metHb decreased but later increased above 20%. Methylene blue treatment was repeatedly attempted, but the patient suffered from hemolytic anemia and rebound methemoglobinemia. The patient finally died from renal failure and cardiopulmonary collapse. We must be careful because methemoglobinemia can occur even when treated pesticides such as propranil.
Anemia, Hemolytic ; Female ; Herbicides ; Humans ; Iron ; Methemoglobin ; Methemoglobinemia* ; Methylene Blue ; Pesticides ; Poisoning ; Propanil* ; Renal Insufficiency

No abstract available.
Mucocele*

Odontogenic keratocysts (OKCs) are one of the most aggressive cysts in the oral and maxillofacial area because of their high recurrence rate and infiltrative behavior. In growing patients with OKCs, a radical treatment approach might cause numerous complications, including the disturbance of jaw growth and loss of the involved tooth. This case report describes successful comprehensive orthodontic treatment combined with marsupialization of the cyst in a young girl who exhibited an OKC with impacted teeth. The 10-year-old girl presented with an OKC extending from the mandibular symphysis through the left mandibular body, with ectopic impaction of the mandibular left canine and first premolar, as well as congenitally missing bilateral mandibular second premolars. Interestingly, spontaneous improvement of the positions of the ectopic impacted teeth, along with a reduction in the size of the cyst, occurred during marsupialization. The sequential use of removable and fixed appliances enabled orthodontic traction of the impacted teeth. The treatment outcome was stable at 2.5 years after the end of the treatment. We speculate that comprehensive orthodontic treatment combined with marsupialization can be an effective treatment strategy for patients with OKCs, especially when they are encountered in young, growing patients with impacted teeth.