• Childhood Kidney Diseases
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• v.6 no.2
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• pp.259-265
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• 2002

Bartter syndrome is a rare disorder characterized by the association of hypokalemic hypochloremic metabolic alkalosis, hyperreninemia, hyperaldosteronemia, short stature and nephrocalcinosis. This disorder presents with hyperplasia of juxtaglomerular apparatus on renal biopsy. We experienced a case of late-onset Bartter syndrome with nephrocalcinosis in a 9-year-old boy, whose chief pictures were muscle weakness, short stature, persistent sterile pyuria and microscopic hematuria. We report this case with a brief review of related literatures.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.23 no.1
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• pp.3-7
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• 2012

Objectives : The early onset of mood symptoms in bipolar disorder has been associated with poor outcomes in many studies. However, aspects of the clinical course of bipolar disorder in children and adolescents are controversial. The goal of this article is to review the clinical characteristics and longitudinal course of children and adolescents with bipolar disorders. Methods : Searches were conducted in MedLine, PsycINFO, KISS, and RISS using the terms phenomenology, clinical course, outcome, BPD, pediatric, children and adolescents. Twenty-one reports were selected : either original articles reporting symptoms and clinical characteristics of subjects (ages 5-18 years), or published articles in reviewed journals about bipolar disorder in children and adolescents. Results : Approximately 70% of subjects with bipolar disorder recovered from their index episode, and 50% had at least 1 syndromal recurrence, particularly depressive episodes. For 60% of the follow-up time, subjects had syndromal or subsyndromal symptoms with numerous changes in symptoms and shifts of polarity. Approximately 20% of BP-II subjects converted BP-I. Conclusion : Bipolar disorders in children and adolescents are characterized by episodic illness with subsyndromal and syndromal episodes with mainly depressive and mixed symptoms and rapid mood changes. Extensive follow-up time is needed to evaluate the continuity of bipolar disorder symptoms from childhood to adulthood.

• Journal of mucopolysaccharidosis and rare diseases
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• v.2 no.1
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• pp.5-7
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• 2016

Mucolipidoses II and III alpha/beta (ML II and ML III) are lysosomal disorders in which the essential mannose-6-phosphate recognition marker is not synthesized onto lysosomal hydrolases and other glycoproteins. The disorders are caused by mutations in GNPTAB, which encodes two of three subunits of the heterohexameric enzyme, N-acetylglucosamine-1-phosphotransferase ML II, recognizable at birth, often causes intrauterine growth impairment and sometimes the prenatal "Pacman" dysplasia. The main postnatal manifestations of ML II include gradual coarsening of neonatally evident craniofacial features, early cessation of statural growth and neuromotor development, dysostosis multiplex and major morbidity by hardening of soft connective tissue about the joints and in the cardiac valves. Fatal outcome occurs often before or in early childhood. ML III with clinical onset rarely detectable before three years of age, progresses slowly with gradual coarsening of the facial features, growth deficiency, dysostosis multiplex, restriction of movement in all joints before or from adolescence, painful gait impairment by prominent hip disease. Cognitive handicap remains minor or absent even in the adult, often wheelchair-bound patient with variable though significantly reduced life expectancy. As yet, there is no cure for individuals affected by these diseases. So, clinical manifestations and conservative treatment is important. This review aimed to highlight the extra-skeletal clinical problems in ML II and III.

• The Korean Journal of Pain
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• v.28 no.3
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• pp.193-197
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• 2015

Background: Herpes Zoster is a disease that occurs after the virus is reactivated due to infection of the varicella virus in childhood. Risk factors are advanced age, malignant neoplasm, organ transplantation, immunosuppressive agents taking are known. The purpose of this study was to investigate the relationship between the seasonal effect and other risk factors on the incidence of herpes zoster. Methods: The medical records of 1,105 patients admitted to the outpatient diagnosed with herpes zoster were retrospectively examined. The patients' sex, age, dermatome, onset, underlying disease, residential areas were collected. Results: The incidence of women outnumbered men and increased for those above the age of 50. The number of occurrences of herpes zoster patients was higher in the spring and summer than in winter. Unlike men, women had the most frequent outbreaks in March. The most common occurrence of dermatome is in the thoracic region. The number of occurrence was similar on the left as the right. Conclusions: In this study, herpes zoster occurs more often in women than in men and more frequently occurs in women in the spring and summer.

• Clinical and Experimental Pediatrics
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• v.57 no.9
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• pp.384-395
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• 2014

Febrile seizure (FS) is the most common seizure disorder of childhood, and occurs in an age-related manner. FS are classified into simple and complex. FS has a multifactorial inheritance, suggesting that both genetic and environmental factors are causative. Various animal models have elucidated the pathophysiological mechanisms of FS. Risk factors for a first FS are a family history of the disorder and a developmental delay. Risk factors for recurrent FS are a family history, age below 18 months at seizure onset, maximum temperature, and duration of fever. Risk factors for subsequent development of epilepsy are neurodevelopmental abnormality and complex FS. Clinicians evaluating children after a simple FS should concentrate on identifying the cause of the child's fever. Meningitis should be considered in the differential diagnosis for any febrile child. A simple FS does not usually require further evaluation such as ordering electroencephalography, neuroimaging, or other studies. Treatment is acute rescue therapy for prolonged FS. Antipyretics are not proven to reduce the recurrence risk for FS. Some evidence shows that both intermittent therapy with oral/rectal diazepam and continuous prophylaxis with oral phenobarbital or valproate are effective in reducing the risk of recurrence, but there is no evidence that these medications reduce the risk of subsequent epilepsy. Vaccine-induced FS is a rare event that does not lead to deleterious outcomes, but could affect patient and physician attitudes toward the safety of vaccination.

• Childhood Kidney Diseases
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• v.7 no.1
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• pp.103-105
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• 2003

Scrotal swelling may be acute or chronic, painful or painless. Common causes of scrotal swelling in newborns are hydrocele, inguinal hernia, testicular torsion, testicular tumor, scrotal hematoma, meconium peritonitis and epididymitis. Abrupt onset of a painful scrotal swelling necessitates prompt evaluation. Testicular torsion and incarcerated inguinal hernia require urgent surgical management. We report a case of scrotal swelling caused by a tunica vaginalis abscess in a 20-days-old boy. He was admitted to the hospital due to fever, irritability and left scrotal swelling with local heat, tenderness and redness. Exploratory laparotomy was performed to rule out testicular torsion. On the operative field, congestive erythematous inflammation on the left tunica vaginalis was noted and it was filled with a pus like discharge. The cultured organism was Streptococcus agalactiae(group B). He recovered quickly after debridement and administration of empirical antibiotics.

• Childhood Kidney Diseases
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• v.8 no.1
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• pp.63-67
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• 2004

A 5-year-old girl was admitted because of an acute onset of weakness in her extremities. She had experienced a similar episode before but had recovered spontaneously. She had previously been diagnosed with distal renal tubular acidosis(RTA) at the age of 2 months. During the period of acute paralysis, her serum potassium level was 1.8 mmol/L and the muscle enzymes were markedly raised suggesting massive rhabdomyolysis. Although hypokalemia is common in renal tubular acidosis, acute paralytic presentation is uncommon and is rarely described in children. We report a case of distal RTA complicated with hypokalemic paralysis with a brief review of related literatures.

• Journal of the Korean Dietetic Association
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• v.9 no.2
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• pp.106-113
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• 2003

The purpose of this study was to compare the growth development between age-matched 51 postmenarcheal girls (mean age 153.1$\pm$5.8 month) who were rapidly maturing and 51 premenarcheal girls (mean age 153.1$\pm$5.7 month) who were slowly maturing. Anthropometirc measurements were taken for height, body weight, body fat(%), waist and hip circumferences of subjects. These measurements of menarcheal girls were significantly higher than those of nonmenarcheal girls. There was no significant difference in BMI distribution between two groups. However, 43.2% and 20.0% respectively in the menarcheal and nonmenarcheal girls had body fat levels of 30% or above. There were significant differences in the anthropometric measurements during past 4 years from 3rd grade elementary school to present. The greatest difference between the two groups were the amount and the rate of increased height and body weight from age 9 to 10. Among menarcheal girls, height, body weight, BMI, and Röhrer index were positively related to the onset of menarche. Distinctively, there was a stronger relationship between age at menarche and anthropometric measurements when the girls were 5th grade elementary school children. These findings support that during childhood and puberty, obese girls grow faster and have earlier menarche. Furthermore, the importance of prevention of obesity was recognized in order to accelerate growth of height among the girls by delaying the age of menarche.

• Korean Journal of Psychosomatic Medicine
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• v.18 no.1
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• pp.3-10
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• 2010

Gilles de la Tourette syndrome is a chronic motor and vocal tic disorder of childhood onset. Abnornmalities in basal ganglia-thalamo-cortical circuits may play an important role in the pathophysiology underlying the involuntary tics. It is often complicated by comorbid attention-deficit/hyperactivity disorder or obsessive-compulsive disorder. Transcranial magnetic stimulation(TMS) is a neurophysiologic technique with research ap-plication. As there is good evidence that this technique can modify cortical activity, repetitive TMS is also used for treatment to change the cortical excitability and therefore affect underlying interconnected cortical-sub-cortical loop. We reviewed the neurophysiologic parameters and the clinical applicability of TMS and rTMS.

• Annals of Clinical Neurophysiology
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• v.9 no.2
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• pp.63-68
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• 2007

The EEG plays an important diagnostic role in epilepsy and provides supporting evidence of a seizure disorder as well as assisting with classification of seizures and epilepsy syndromes. There are a variety of electroclinical syndromes that are really defined by the EEG such as Lennox-Gastaut syndrome, benign rolandic epilepsy, childhood absence epilepsy, juvenile myoclonic epilepsy and also for localization purposes, it is vitally important especially for temporal lobe epilepsy. The sensitivity of first routine EEG in diagnosis of epilepsy has been known about 20-50%, but this proportion rises to 80-90% if sleep EEG and repetitive recording should be added. Convincing evidences suggest that the EEG may also provide useful prognostic information regarding seizure recurrence after a single unprovoked attack and following antiepileptic drug (AED) withdrawal. Moreover, patterns in the EEG make it possible to disclose an ictal feature of nonconvulsive status epilepticus, separate epileptic from other non-epileptic episodes and clarify the clues predictive of the cause of the encephalopathy (i.e., triphasic waves in metabolic encephalopathy). Therefore, regardless of its low sensitivity and other pitfalls, EEG should be considered not only in the situation of new onset episode such as a newly developed, unprovoked seizure or a condition manifesting decreased mentality from obscure origin, but also as a barometer of the long-term outcome following AED withdrawal.