No abstract available.
Malignant Atrophic Papulosis*

BACKGROUND: Healthcare-associated infections (HAIs) are among the most important threats to patient safety. When hospitalized children face these threats, there is morbidity, mortality, prolonged hospitalization, and increased healthcare costs. Research on local healthcare epidemiology is necessary to enhance collective knowledge and evidence formanaging this problem. METHODS: We performed a retrospective analysis of databases of patients who were diagnosed with HAIs at Chung-Ang University Hospital (CAUH) from 2007 through 2011. Cases were selected from the microbiology registry databases. The data on prevalence of HAIs in various wards and its annual trends were compared to previously reported nationwide data. Moreover, we analyzed the patterns of antibiotic susceptibility results for HAI pathogens. RESULTS: A total of 181 HAIs were identified in 122 patients. The HAI rate among pediatric patients at CAUH was 2.4/1,000 person-hospital days. Urinary tract infections (UTIs) (53 episodes, 29.3%) were the most common, followed by pneumonia (33 episodes, 18.2%). Staphylococcus aureus was found to be the most common gram-positive organism, whereas Escherichia coli was the most common gram-negative organism. Methicillin-resistant S. aureus (MRSA) comprised 84% of the S. aureus infections. Imipenem resistance was detected in 58.8% and 55.0% of Acinetobacter baumannii and Pseudomonas aeruginosa isolates, respectively. CONCLUSION: Between 2007 and 2011, UTIs were the most common type of HAIs, and MRSA was the most common pediatric HAI pathogen, both in the general ward and intensive care unit at the CAUH. Further research on the epidemiology and pathogenesis of HAIs is necessary and prevention measures should be implemented to prevent HAIs in children.
Acinetobacter baumannii ; Child ; Child, Hospitalized ; Delivery of Health Care ; Escherichia coli ; Health Care Costs ; Hospitalization ; Humans ; Imipenem ; Intensive Care Units ; Methicillin Resistance ; Methicillin-Resistant Staphylococcus aureus ; Microbial Sensitivity Tests ; Patient Safety ; Patients' Rooms ; Pneumonia ; Prevalence ; Pseudomonas aeruginosa ; Retrospective Studies ; Staphylococcus aureus ; Urinary Tract Infections

The musculocutaneous nerve has a short course and is deeply placed before piercing the coracobrachialis muscle at the point where it is relatively fixed by branches along its course between biceps brachii and brachialis muscles. Therefore, an isolated palsy of the musculocutaneous nerve is extremely rare and usually caused by stab, cut or bullet injuries. An isolated musculocutaneous nerve palsy, sparing the coracobrachialis muscle, can occur after heavy exercise of the arm musculature and has a good prognosis. We report a patient with an isolated musculocutaneous nerve palsy caused by motorcycle accident.
Arm ; Humans ; Motorcycles ; Muscles ; Musculocutaneous Nerve* ; Paralysis* ; Prognosis

Central diabetes insipidus is a rare disorder that can result as a consequence of diverse etiologies, including malformations, autoimmune, infiltrative(e.g. neoplastic or histiocytosis) or traumatic processes, as well as mutations in the gene encoding arginine vasopressin. Idiopathic central diabetes insipidus is a diagnosis of exclusion, one that has been made less frequently through the decades. Idiopathic central diabetes insipidus in children and adolescent requires a frequent follow-up regimen using serial brain MRI and CSF examinations especially if an isolated pituitary stalk thickening or loss of a hyperintense signal in the posterior lobe is observed. Also, so-called "idiopathic" central diabetes insipidus warrants close follow-up to determine the etiology, especially if anterior pituitary hormone deficiencies are detected. We report a case of idiopathic central diabetes insipidus with growth hormone deficiency and loss of a hyperintense signal in the posterior lobe of pituitary in the brain MRI. We followed up with serial contrast enhanced brain MRI and CSF evaluation for the early detection of an evolving occult hypothalamic-stalk lesion and finally detected a newly developed teratocarcinoma in the suprasellar region.
Adolescent ; Arginine Vasopressin ; Brain ; Child ; Diabetes Insipidus, Neurogenic* ; Diagnosis ; Follow-Up Studies ; Growth Hormone ; Humans ; Magnetic Resonance Imaging ; Pituitary Gland ; Pituitary Gland, Posterior ; Teratocarcinoma*

Bronchogenic cysts are rare congenital anomalies that arise early in gestation from abnormal budding of the developing respiratory system. Mediastinal bronchogenic cysts account for 10-15 percent of all primary mediastinal masses; 63.7 percent of patients are symptomatic. Common symptoms are fever, chest pain, cough, dyspnea, and dysphagia. Gastrointestinal symptoms except dysphagia are rare. It can be life threatening with compression, infection, hemorrhage, or rupture. Symptoms and signs of compression are more frequent in infants and children than in adults. It may be asymptomatic, or cough, infection, and hemoptysis may be observed. Complete excision is recommended. We report a case of bronchogenic cyst misdiagnosed as chronic gastritis with nausea and epigastric pain for a year.
Adult ; Bronchogenic Cyst* ; Chest Pain ; Child ; Cough ; Deglutition Disorders ; Dyspnea ; Fever ; Gastritis ; Hemoptysis ; Hemorrhage ; Humans ; Infant ; Nausea* ; Pregnancy ; Respiratory System ; Rupture

Pfeiffer syndrome is one of a rare form of craniosynostosis syndrome, showing variable degree of craniosynostosis, midface hypoplasia, broad thumbs and toes and syndactyly. This is transmitted in autosomal dominant pattern and known to be related to mutations in FGFR (Fibroblast Growth Factor Receptor) 1 or FGFR 2. We experience a case of newborn Pfeiffer syndrome type 3 who had multiple facial anomalies, thumbs and great toes anomalies, ankylosis of radius and ulnar and hydrocephalus.
Acrocephalosyndactylia* ; Ankylosis ; Craniosynostoses ; Humans ; Hydrocephalus* ; Infant, Newborn ; Radius ; Syndactyly ; Thumb ; Toes

BACKGROUND: We identified virulence genes in uropathogenic E. coli isolates and studied the association between virulence gene and clinical characteristics in order to predict the severity and recurrency. METHODS: 39 Escherichia coli strains from patients with urinary tract infection were clinically and genotypically characterized. The strains were examined genotypically by using the multiplex polymerase chain reaction for presence of 5 urovirulence genes : pyelonephritis-associated pili(pap), S. fimbriae(sfa), afimbrial adhesin(afa), cytotoxic necrotizing factor (cnf), and alpha-hemolysin(hly). The patient's clinical characteristics were determined retrospectively. RESULTS: 17 pap(+), 4 sfa(+), 7 afa(+), 6 cnf(+), and 8 hly(+) strains were identified. And there were 10 genotypes. Among them, genotype pap(+)sfa(-)afa(-)cnf(-) hly(-) was most dominant(36%). But no urovirulence gene was detected in 12 strains(31%). When the data was analyzed, it was apparent that an association among various urovirulence genes exists. sfa gene was frequently associated with cnf gene(p < 0.001). And afa gene was associated cnf and hly gene(p= 0.026, <0.001). An association between cnf gene and hly gene was observed(p=0.002). Positive rates of virulence genes were not different between male and female. In infancy, pap(-)sfa(-)afa(+)cnf(+)hly(+) genotype was dominant. In 2-15 years old age group, pap(-) sfa(-)afa(-)cnf(-)hly(-) genotype was dominant. And in 16- 40 years old age group, pap(+)sfa(-)afa(-)cnf(-)hly(-) was dominant. So, some virulence genotype might be associated with specific age group. Presence of virulence gene or specific genotype was not different among diseases(acute pyelonephritis, cystitis, asymptomatic bacteriuria). So, virulence genes were not associated with severity of urinary tract infection. Virulence genes were not associated with susceptibility of recurrent infection. In neurogenic bladder patients, there were significantly more sfa(+) strains (p=0.019). And all isolates of neurogenic bladder patients were genotype pap(+)sfa(+)afa(-)cnf(+)hly(-)(p < 0.001). CONCLUSION: In this study, We found which genotype is most dominant in uropathogenic Escherichia coli, and that virulence genes do not suggest severity or recurrency of urinary tract infection. In neurogenic bladder patients, some virulence genes were more prevalent.
Adult ; Cystitis ; Escherichia coli ; Female ; Genotype ; Humans ; Male ; Multiplex Polymerase Chain Reaction ; Polymerase Chain Reaction ; Pyelonephritis ; Retrospective Studies ; Urinary Bladder, Neurogenic ; Urinary Tract Infections ; Uropathogenic Escherichia coli* ; Virulence*

Kawasaki diseases occur frequently in children beyond the age of 4 years, but classic Kawasaki disease in newborns are rare and have not been reported in Korea yet. We report a case of classic Kawasaki disease in neonate who is 21 days old girl and has no response to gamma-globulin although early diagnosis and treatment. Her clinical features met typical Kawasaki disease, but unusually she had a non suppurative labia major erythema and severe edema instead of cervical lymphadenitis. She had persistent fever and progressive carditis with coronary dilatation in spite of 3 doses of gamma globulin. Her fever was subsided after 3 doses of intravenous methyl prednisolone. In the subacute stage her other symptoms subsided and appeared typical desquamation of fingers and toe. Finally she had mild mitral regurgitation and diffuse coronary dilatation when she was discharged. Since one year follow up, with continuous aspirin medication, now she is very healthy, and her coronary dilatation was normalized and not any more valvular regurgitation.
Aspirin ; Child ; Dilatation ; Early Diagnosis ; Edema* ; Erythema* ; Female ; Fever ; Fingers ; Follow-Up Studies ; gamma-Globulins ; Humans ; Infant, Newborn* ; Korea ; Lymphadenitis ; Mitral Valve Insufficiency ; Mucocutaneous Lymph Node Syndrome* ; Myocarditis ; Prednisolone ; Toes

OBJECTIVE: Low birth weight (LBW) infants are known to be at the risk of increased morbidity and mortality, and also of developing adult or metabolic diseases later in their life. Recently, the number of LBW infants has increased in Korea. Therefore, we examined the changing patterns of LBW infants and associated risk factors. METHODS: Data from the Korean National Statistical Office during 13 years (1995-2007) were used. We analyzed the changing mean gestational age, mean birth weight, mean maternal age, and the changing incidence of premature infant, LBW infant, multiple births, the proportion of first baby, and sex of the baby. Also, we analyzed the incidence of unmarried mother, old aged mother (>35 years), young aged mother (<20 years), high school graduated mother. In addition, we analyzed the relationship between LBW infants and risk factors. RESULTS: The incidence of LBW infants was 2.99% in 1995, and 4.66% in 2007. The proportion of premature infant, multiple birth, first baby, unmarried mother, old aged mother, female baby increased. The increase in premature infant, multiple birth, old aged mother, unmarried mother, and female infant were found to be the important factors regarding increase in LBW infants in Korea. CONCLUSION: In Korea, an increase in the prevalence of LBW infants and prematurity from 1995 to 2007 was observed. The increase in premature infants, multiple birth, old aged mother, unmarried mother, and female infant are associated with the increase in LBW infants.
Adult ; Aged ; Birth Weight ; Female ; Gestational Age ; Humans ; Illegitimacy ; Incidence ; Infant ; Infant, Low Birth Weight ; Infant, Newborn ; Infant, Premature ; Korea ; Maternal Age ; Metabolic Diseases ; Mothers ; Multiple Birth Offspring ; Prevalence ; Risk Factors

Mutation of the ATRX gene leads to X-linked alpha-thalassemia/mental retardation (ATR-X) syndrome and several other X-linked mental retardation syndromes. We report the first case of ATR-X syndrome documented here in Korea. A 32-month-old boy came in with irritability and fever. He showed dysmorphic features, mental retardation and epilepsy, so ATR-X syndrome was considered. Hemoglobin H inclusions in red blood cells supported the diagnosis and genetic studies confirmed it. Mutation analysis for our patient showed a point mutation of thymine to cytosine on the 9th exon in the ATRX gene, indicating that Trp(C), the 220th amino acid, was replaced by Ser(R). Furthermore, we investigated the same mutation in family members, and his mother and two sisters were found to be carriers.
Amino Acid Substitution ; Body Dysmorphic Disorders/complications ; Child, Preschool ; DNA Mutational Analysis ; Epilepsy/complications ; Exons ; Hemoglobin H/*genetics ; Humans ; Male ; Mental Retardation/complications ; Mental Retardation, X-Linked/complications/diagnosis/genetics ; Point Mutation ; Republic of Korea ; alpha-Thalassemia/complications/diagnosis/genetics