BACKGROUND: Sleep-related breathing disorders(SRBD) and periodic leg movements disorder(PLMD) are both common, and are considered as separate sleep disorders. However, both disorders show high comorbidity. SRBD and PLMD can result in excessive daytime sleepiness and insomnia due to frequent sleep fragmentation So, it is very important to consider the presence of PLMD, when we are dealing with the diagnosis and management of SRBD. The objectives of this study were to determine the incidence of PLMD in patients with SRBD, and 13 describe any differences between patients with and without PLMD. METHOD: The authors reviewed the sleep recordings of 106 Patients with a final diagnosis of SRBD(obstruclive sleep apnea or upper airway resistance syndrome), who underwent full nocturnal polysomnography, including the monitoring of the anterior tibialis electromyogram. All sleep records were recorded and scored using the standard criteria. The data was analyzed by the student t-test. RESULTS: 106 patients(M=76, F=30) were included in the analysis. Data revealed a mean age of 49.5 +/- 13.6 years, a respiratory disturbance index(RDI) of 22.3 +/- 25.4/hour sleep, a lowest oxygen saturation of 84.9 +/- 11.3%, a mammal esophageal pressure of -41.0 +/- 19.1cm H2O, and PLM index(PLMI) index(PLMI) 13.1 +/- 22.4 movements/ hour sleep. Forty four percent(47 of 106 patients) had a PLMI of greater than 5 on this study. The mean age of the patients with PLMD was significantly higher than that of the patients without PLMD(p < 0.005). Fe-male patients with SRBD accompanied more PLMD(p < 0.05). The apnea index of the patients with PLMD was significantly lower than that of the patients without PLMD(p < 0.01). The percentage of stage 1 sleep in the patients with PLMD was significantly lower than that of the patients without PLMD(p < 0.05). CONCLUSION: The prevalence of PLMD in the patients with SRBD was high at 44.3%. The patients with PLMD were older and had more high RDI in comparison to the patients without PLMD, which was consistent with previous findings. The authors recommend that more careful consideration of PLMD is required when diagnosing and treating SRBD.
Airway Resistance ; Apnea ; Comorbidity* ; Diagnosis ; Extremities* ; Humans ; Incidence ; Leg ; Mammals ; Oxygen ; Polysomnography ; Prevalence ; Respiration* ; Sleep Apnea Syndromes ; Sleep Deprivation ; Sleep Wake Disorders ; Sleep Initiation and Maintenance Disorders

Diffuse panbronchiolitis (DPB) is a progressive inflammatory respiratory disease of unknown cause mainly occurring in East Asian people. Studies on causes of the disease point to a genetic predisposition unique to Asians, but the cause remains unknown. If untreated, DPB progresses to bronchiectasis, respiratory failure, and death. The age of patients at onset of the disease varies from young to elderly people with a peak at 40-60 years. A few cases of DPB have been reported in Korean adults since 1992; however, the case of DPB in children is uncommon. Herein, we describe a 16-year-old girl with DPB who presented with chronic cough and sputum.
Adolescent* ; Adult ; Aged ; Asian Continental Ancestry Group ; Bronchiectasis ; Child ; Cough ; Female* ; Genetic Predisposition to Disease ; Humans ; Macrolides ; Respiratory Insufficiency ; Sputum

An 8-year-old male was admitted because of dysphagia and substernal pain suffered while eating followed by postprandial vomiting for 2 years. He was always hungry due to postprandial vomiting and willing to eat again just after vomiting. After this meals, he used to jump up and down to shake off the substernal discomfort. A narrowing of the gastroesophageal junction was noted by esophagogram. Manometry revealed high Lower esophageal sphincter (LES) pressure (51.6mmHg), incomplete LES relaxation during swallowing, loss of esophageal peristalsis and a positive pressure of the esophageal body compared to intragastric pressure. After the 1st balloon dilatation, symptoms were much improved even though LES pressure still remained high (37.2mmHg). About 2 months after the 1st balloon dilatation, symptoms relapsed and we managed him with a 2nd balloon dilatation. Symptoms were more improved than after the 1st dilatation and LES pressure normalized as well. Since the 2nd dilatation, symptoms have not recurred for 3 years. We present an 8-year-old boy with achalasia successfully managed by the use balloon dilatation.
Child ; Deglutition ; Deglutition Disorders ; Dilatation* ; Eating ; Esophageal Achalasia* ; Esophageal Sphincter, Lower ; Esophagogastric Junction ; Humans ; Male ; Manometry ; Meals ; Peristalsis ; Relaxation ; Vomiting

PURPOSE: Diamond-Blackfan anemia (DBA) is a rare heterogeneous genetic disorder of infancy and early childhood. It is characterized by red cell aplasia, congenital anomalies, and a predisposition to cancer. Corticosteroids and red cell transfusions are the mainstays of therapy. We describe our experience of 6 cases of DBA that were encountered over a period of 16 years. METHODS: Medical records of 6 patients diagnosed to have DBA and admitted to the Chonnam National University Hospital between 1992 and 2008 were retrospectively reviewed. RESULTS: Three patients were males. The age at diagnosis ranged from 3 to 18 months (median, 5.5 months). Heart defects were observed in 4, polydactyly in 2, and strabismus in 1 patient. The median number of transfusions was 3 (range, 2 -8). All patients responded to initial treatment with steroids and had a hemoglobin level > or =9 g/dL with a median of 12.5 days (range, 7-22 days). Three patients are currently not receiving steroid therapy. A minimum dose of oral prednisolone (<5 mg per day) was required to maintain hemoglobin > or =9 mg/dL in 3 cases. Red cell transfusion was infrequently required in 1 patient. In the median follow-up of 14 years, there was no development of malignancy. No significant side effects of steroids were found, except for short stature in 2. CONCLUSION: The majority of DBA patients achieved complete response and under maintenance therapy with low dose of steroids. Close observation is needed to monitor steroid side effects, cardiac function, and development of malignancy. A nation-wide survey is necessary to further characterize this rare disease in Korean children.
Adrenal Cortex Hormones ; Anemia, Diamond-Blackfan ; Child ; Follow-Up Studies ; Heart ; Hemoglobins ; Humans ; Male ; Medical Records ; Organothiophosphorus Compounds ; Polydactyly ; Prednisolone ; Rare Diseases ; Retrospective Studies ; Steroids ; Strabismus

PURPOSE: Pneumomediastinum is rare in children and adolescents, and its causes have not yet been clearly determined. We aimed to identify the causes, clinical manifestations and prognosis of noniatrogenic pneumomediastinum in children. METHODS: From February 2007 to June 2014, we retrospectively investigated 121 patients with pneumomediastinum under 18 years of age in 2 hospitals. Eighteen patients with pneumomediastinum after thoracotomy and 35 patients with iatrogenic pneumomediastinum were excluded. RESULTS: Sixty-eight patients were divided into 4 age groups: those under 1 year of age (n=9, 13.2%), those 1 to 5 years of age (n=9, 13.2%), those 6 to 10 years of age (n=17, 25.0%) and those over 11 years of age (n=33, 48.5%). Chest pain (n=43, 63.2%) was the most common initial complaint and subcutaneous emphysema was identified in 18 patients (26.5%). Chest x-ray was diagnostic in all except 9 patients (13.2%). Predisposing causes of pneumomediastinum were idiopathic (n=26, 38.2%), respiratory tract infection (n=23, 33.8%), asthma exacerbation (n=4, 5.9%), trauma (n=4, 5.9%), endobronchial foreign body (n=2, 2.9%), interstitial lung disease (n=5, 7.4%), and neonatal respiratory disease (n=4, 5.9%). Chest pain (P<0.001) and idiopathic cause (P=0.001) were shown to linearly increase with age. On the contrary, tachypnea (P<0.001), dyspnea (P=0.016), and interstitial lung disease (P=0.008) were shown to have a decreasing linear association with age. The length of hospital stay was significantly increased in patients with interstitial lung disease (P=0.042), those with pneumothorax (P=0.044), and those without chest pain (P=0.013). CONCLUSION: According to age groups, there were significant differences in causes and clinical manifestations. In particular, pneumomediastinum that developed in younger patients with interstitial lung disease showed unfavorable outcomes, such as dyspnea, pneumothorax, and increased length of hospital stay. Therefore, precise evaluation of predisposing causes and careful management are needed for children with pneumomediastinum.
Adolescent ; Asthma ; Chest Pain ; Child ; Dyspnea ; Foreign Bodies ; Humans ; Length of Stay ; Lung Diseases, Interstitial ; Mediastinal Emphysema ; Pneumothorax ; Prognosis ; Respiratory Tract Infections ; Retrospective Studies ; Subcutaneous Emphysema ; Tachypnea ; Thoracotomy ; Thorax

Telepathology systems will be common systems in hospitals. The two systems were designed and implemented in web environments for test. One was implemented with the Common Object Request Broker Architecture (CORBA) technique. The other system was implemented in the form of ActiveX. The histopathological materials were stained by Hematoxylin and Eosin. By the Donpisha CCD camera attached to an Olympus BX-51 optical microscope 180 color images come to be acquired. For evaluation of the systems, transmission times and telediagnosis concordance rates were measured. Image processing ability was tested using two telepathology systems. For the local area test, system I using CORBA had measured image transmission times of 0.1 s, 0.2 s, and 0.4 s at the file sizes of 100 K byte, 900 K byte and 3.6 M byte respectively. Transmission times for system II using Component Object Model (COM) were slightly slower, ranging from 0.02 s to 0.05 s. In the long distance area test, system II transmission times were 0.5 s, 0.8 s, and 2.0 s. The overall concordance rate of telediagnosis for the 180 images was 78.3%. In this study, we compared our systems about image transmission, and processing for the further development of system configurations.
Eosine Yellowish-(YS) ; Hematoxylin ; Internet* ; Telepathology*

PURPOSE: Hepatic veno-occlusive disease (VOD) is a life-threatening complication occurring early after stem cell transplantation (SCT). Early diagnosis and effective treatment has not been established in severe VOD. Because there are few reports on VOD in Korean children, we evaluated the clinical characteristics of VOD following SCT in children. METHODS: We retrospectively reviewed the chart of all patients (n=116) receiving SCTs in CNUH Pediatric BMT center between May, 1991 and June, 2004. RESULTS: VOD developed in 11 patients (9.5%) (median age, 9.8 years; range, 2 to 13.9). Underlying diagnoses were ALL (n=3), severe aplastic anemia (n=3), AML (n=2), acute biphenotypic leukemia (n=1), neuroblastoma (n=1), and myelodysplastic syndrome (n=1). The median day of onset of VOD was D+9 (range, D-3 to D+19). VOD was classified as moderate in 5 and severe in 6 cases. Maximum level of serum total bilirubin was 2.9 mg/dL (range, 2.1 to 9.2) in moderate VOD and 7.3 mg/dL in severe VOD (range, 2.0 to 24.2) at D+18 (range, D-5 to D+59). We successfully treated VOD with various combinations including tPA and heparin (2/5, 40%), ursodeoxycholic acid (2/5, 40%), N-acetylcysteine (3/5, 60%), and defibrotide (1/2, 50%). All of 5 patients with moderate VOD survived at D+100 (range, 5.5+ to 66.6+ months). Five of 6 (83%) patients with severe VOD died within first 19 day from complications of VOD. CONCLUSION: This retrospective study showed that the incidence of VOD was 9.5%, and the mortality of severe VOD was still high which would necessitate early diagnosis, effective prevention and treatment.
Acetylcysteine ; Anemia, Aplastic ; Bilirubin ; Child ; Diagnosis ; Early Diagnosis ; Hematopoietic Stem Cell Transplantation* ; Hematopoietic Stem Cells* ; Heparin ; Hepatic Veno-Occlusive Disease* ; Humans ; Incidence ; Leukemia, Biphenotypic, Acute ; Mortality ; Myelodysplastic Syndromes ; Neuroblastoma ; Retrospective Studies* ; Stem Cell Transplantation ; Ursodeoxycholic Acid

This study was conducted to evaluate the health status of elderly women who attended in a Health Promotion Program of the Seo-gu Health Center in Daegu. The study subjects were 158 elderly women (over 65 years) in an urban community. The subjects were investigated by means of individual interviews using a questionnaire, Blood tests for analyzing their biochemical status were carried out. The average age of the study subjects was 70.9 +/- 2.3 years. Of the subject group 79.1% ranged in age from 65 to 74 year and 20.9% were over 75 years. With respect to health related factors, 23.4% of subjects drank alcoholic beverages and 15.2% of sujects currently smoker. The prevalence with chronic diseases was 51.9%, and 26.6% of the subjects were healthy. The diseases most frequently reported as having been or being treated were arthritis (38.1%), hypertension (21.4%), and diabetes (17.9%). The average height of subjects was below the standard established in the Korean Recommended Dietary Allowances, while the average weight was close to the standard. The means of systolic and diastolic blood pressure were 130.2 mmHg and 71.9 mmHg, respectively. The mean serum albumin level was 4.5 mg/dl, and the value of hemoglobin and hematocrit was 12.5 mg/dl and 0.7%, respectively. The mean serum cholesterol level was 207.1 mg/dl and the mean triglyceride level was 187.7 mg/dl. The serum lipid levels were higher then in those reported in research. The serum cholesterol levels of 71.5%of subjects were within the normal range. The GOT and GTP levels were within the moderate range. In conclusion, the health status of the elderly who attended the Health Promotion Program in the Seo-gu Health Center were very average. However, it was necessary to prepare a health management program to deal with the serum lipids so as to establish and maintain good health. When we carry out the health promotion program in a community, individual program of adequate to health status should be developed more.
Aged* ; Alcoholic Beverages ; Arthritis ; Blood Pressure ; Cholesterol ; Chronic Disease ; Daegu ; Female ; Guanosine Triphosphate ; Health Promotion* ; Hematocrit ; Hematologic Tests ; Humans ; Hypertension ; Prevalence ; Recommended Dietary Allowances ; Reference Values ; Serum Albumin ; Triglycerides ; Surveys and Questionnaires

Galloway-Mowat syndrome (GMS) is a rare autosomal recessive disorder comprising of early-onset nephrotic syndrome and central nervous system involvement including microcephaly, seizure and developmental delay. Although hiatal hernia is no longer considered essential findings for diagnosis, clinical triad of GMS included nephrotic syndrome, neurological manifestations, and hiatal hernia in the original description. We experienced a case of newborn with GMS presenting these clinical triad in neonatal period. A male infant weighing 2,250 g was born at gestational week 39+3 by cesarean section. The patient revealed mild dysmorphic facial features and microcephaly. On day 7, Nissen fundoplication was done because of hiatal hernia with gastric volvulus. At the age of 2 weeks he developed nephrotic syndrome with proteinuria and hypoalubuminemia. This is the first case of GMS that three classic findings were present in neonatal period in Korea.
Central Nervous System ; Cesarean Section ; Diagnosis ; Female ; Fundoplication ; Hernia, Hiatal ; Humans ; Infant ; Infant, Newborn ; Korea ; Male ; Microcephaly ; Nephrotic Syndrome ; Neurologic Manifestations ; Pregnancy ; Proteinuria ; Seizures ; Stomach Volvulus

BACKGROUND: In mammals, the master circadian pacemaker is localized in an area of the ventral hypothalamus known as the suprachiasmatic nucleus (SCN). Previous studies have shown that pacemaker neurons in the SCN are highly coupled to one another, and this coupling is crucial for intrinsic self-sustainability of the SCN central clock, which is distinguished from peripheral oscillators. One plausible mechanism underlying the intercellular communication may involve direct electrical connections mediated by gap junctions. METHODS: We examined the effect of mefloquine, a neuronal gap junction blocker, on circadian Period 2 (Per2) gene oscillation in SCN slice cultures prepared from Per2::luciferase (PER2::LUC) knock-in mice using a real-time bioluminescence measurement system. RESULTS: Administration of mefloquine causes instability in the pulse period and a slight reduction of amplitude in cyclic PER2::LUC expression. Blockade of gap junctions uncouples PER2::LUC-expressing cells, in terms of phase transition, which weakens synchrony among individual cellular rhythms. CONCLUSION: These findings suggest that neuronal gap junctions play an important role in synchronizing the central pacemaker neurons and contribute to the distinct self-sustainability of the SCN master clock.
Animals ; Circadian Rhythm ; Electrical Synapses ; Gap Junctions* ; Hypothalamus ; Luminescent Measurements ; Mammals ; Mefloquine* ; Mice* ; Neurons ; Phase Transition ; Suprachiasmatic Nucleus*