• Clinical and Experimental Pediatrics
• /
• v.61 no.2
• /
• pp.37-42
• /
• 2018

Hemolytic uremic syndrome (HUS) is often encountered in children with acute kidney injury. Besides the well-known shiga toxin-producing Escherichia coli-associated HUS, atypical HUS (aHUS) caused by genetic complement dysregulation has been studied recently. aHUS is a rare, chronic, and devastating disorder that progressively damages systemic organs, resulting in stroke, end-stage renal disease, and death. The traditional treatment for aHUS is mainly plasmapheresis or plasma infusion; however, many children with aHUS will progress to chronic kidney disease despite plasma therapy. Eculizumab is a newly developed biologic that blocks the terminal complement pathway and has been successfully used in the treatment of aHUS. Currently, several guidelines for aHUS, including the Korean guideline, recommend eculizumab as the first-line therapy in children with aHUS. Moreover, life-long eculizumab therapy is generally recommended. Further studies on discontinuation of eculizumab are needed.

• Proceedings of the Ginseng society Conference
• /
• 1984.09a
• /
• pp.89-95
• /
• 1984

The effect of hydrocortisone or Panax ginseng, and/or a combination of hydrocortisone and Panax ginseng on phytohaemagglutinin (PHA-P)-induced transformation of peripheral blood lymphocytes were studied in 4 normal healthy adult volunteers. Increasing concentrations of Panax ginseng 0.16-1.60${\mu}g$/ml caused a doserelated inhibition of PHA-P transformation of lymphocytes. A combination of 500${\mu}g$/ml hydrocortisone and 0.80${\mu}g$/ml Panax ginseng produced a greater suppression of PHA-P stimulation than either drug used alone. This suggests that Panax ginseng has a steroid-like effect in vitro, and may have a potentiating effect with hydrocortisone on T-cell mediated immunity. The in vivo effects of Panax ginseng were further studied in 6 healthy adult volunteers. PHA-P transformation studies were performed before and after Panax ginseng capsules were taken for 2 weeks and at a higher dosage at 4 weeks. Our results showed that in 3 subjects, there was significant inhibition of PHA-P transformation at 4 weeks.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
• /
• v.23 no.3
• /
• pp.103-108
• /
• 2012

Objectives : The aim of this study is to investigate the effectiveness of treatment with osmotic-release oral system methylphenidate (OROS-MPH) on quality of life (QOL) in children with attention-deficit hyperactivity disorder (ADHD). Another aim is to assess the relationship between change in QOL and other factors including children's symptoms and academic performance or parents' depression and parenting stress. Methods : A total of 111 medication-naive children with ADHD in a multicenter, open-label, 12-week trial of OROS-MPH completed an evaluation using diverse rating scales at two time points; at baseline and after 12 weeks of treatment. Scales for investigation of children included the Parent Report Form-Children's Health and Illness Profile-Children's Edition (PRF-CHIP-CE) on QOL, the ADHD Rating Scale-IV on symptoms, and the Academic Performance Rating Scale (APRS). The Beck Depression Inventory and Parenting Stress Index were used for assessment of their parents. Results : Total scores for mean PRF-CHIP-CE increased from $207.9{\pm}26.7$ at baseline to $226.3{\pm}25.9$ after 12 weeks of treatment (p<.001). The change of APRS showed the strongest correlation with the increment of PRF-CHIP-CE scores (Pearson coefficient=0.561, p<.001), even after controlling for other factors (partial correlation coefficient=0.420, p<.001). Conclusion : Treatment with MPH-OROS results in improvement of QOL in children with ADHD in Korea. The advance in academic performance plays a key role in this change of QOL.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
• /
• v.28 no.4
• /
• pp.213-219
• /
• 2017

Objectives: Early intensive interventions are very important for children with autism spectrum disorder. We examined the actual conditions of hospital-based early intensive interventions for autism spectrum disorder in Seoul, in order to help develop and implement an evidence-based early intensive intervention model for use in Korea. Methods: Nine hospital-based institutes running an early intensive intervention program for children with autism spectrum disorder responded to a questionnaire in September 2014. They provided a brief introduction to their program, explained its theoretical bases, and reported the number of children, their age, intervention time, duration and so on. Results: In the majority of the institutions, the intervention was provided for over 20 hours every week, and the theoretical bases included various applied behavioral analysis (ABA) methods and other therapies (language and occupational therapy). The therapist-child ratio ranged from 1:1 to 5:3. Various types of therapists were involved, including behavioral analysts, special education teachers and (or) language pathologists. There was only one clinic where the behavioral analyst was the main therapist. Usually, the intervention was terminated just before the child entered elementary school. The main merit of the hospital-based intervention in our survey was the effectiveness of the multi-disciplinary intervention plan and its other merits were the accuracy of the diagnosis, its ability to be combined with medicine, and so on. Conclusion: The current hospital-based early intensive intervention programs provide interventions for over 20 hours per week and employ multidisciplinary approaches. However, there are very few institutes for children with autism and very few intervention specialists and specialist education courses in the country. We need more educational programs for intervention therapists and have to try to develop policies which encourage the implementation of an evidence-based early intensive intervention program nationwide.

• Journal of Acupuncture Research
• /
• v.19 no.4
• /
• pp.89-100
• /
• 2002

Objective : We want to have a correct understanding and the distance about the Oriental Medical therapy on the CP(cerebral palsy) children. Methods : We made up a question of parents of CP children and analyzed it. Conclusion : 19 patients were taken physical treatment and 12 patients were taken occupational treatment in company with physical treatment(In our OPD CP patient) Parents complained about poor surroundings of our hospital for children and deficiency of physical treatment. The most satisfactory thing is acupuncture treatment, then the next thing is aroma therapy. On the satisfaction degree about the Oriental Medical therapy, very good is 27.3%, good 45.5%, ordinary 27.3% and unsatisfaction is none. The place Oriental medical therapy before our hospital was taken is local Oriental medical clinic, 9 cases out of 22.

• Parasites, Hosts and Diseases
• /
• v.52 no.5
• /
• pp.545-549
• /
• 2014

Sparganosis is an infection with a parasitic tapeworm larva that occurs by eating infected foods or drinking contaminated water. The larvae can migrate to a tissue or muscle in the chest, abdominal wall, extremities, eyes, brain, urinary tract, pleura, pericardium, spinal canal, or scrotum. Herein, we report a 5-month old infant with scrotal sparganosis who was initially suspected to have a scrotal inflammatory mass with a history of applying raw frog meat into the umbilicus. Preoperative ultrasound examinations and computed tomography (CT) scanning misdiagnosed the mass as a scrotal teratoma. The scrotal mass was surgically removed, and the histopathology proved it to be scrotal sparganosis. This case displays the youngest patient ever reported with scrotal sparganosis, and the first description of CT characteristics of scrotal sparganosis. A detailed medical history is necessary for patients with scrotal masses suspected of sparganosis. In addition, ultrasound and CT examinations are helpful to rule out other causes of a scrotal mass.

• Pediatric Infection and Vaccine
• /
• v.30 no.1
• /
• pp.20-32
• /
• 2023

Purpose: This study aimed to identify the etiology and risk factors of community-acquired pneumonia (CAP) requiring hospitalization in Korean children during the coronavirus disease 2019 (COVID-19) pandemic. Methods: Clinical information of children admitted with CAP to Seoul National University Children's Hospital (SNUCH) between January 1, 2021, and February 28, 2022, was retrospectively collected and analyzed. In addition, the etiologic diagnosis and demographic data of children with CAP who were discharged at the other seven hospitals between January and February 2022 were collected. Pneumonia was diagnosed using strict criteria comprising clinical symptoms, physical examination findings, and chest radiographic findings. Results: Among 91 children hospitalized with CAP at SNUCH during the 14-month period, 68.4% were aged <5 years and 79.1% had underlying diseases. Among the 95 CAP cases, respiratory assistance was required in 70.5%, and the use of a ventilator was required in 20.0%. A total of five patients expired, all of whom were either immunocompromised or had underlying neurological diseases. Neurological diseases and immunosuppression were significantly correlated with respiratory assistance (P=0.003) and death (P=0.014). A total of 55% of the detected respiratory pathogens were viruses, the most common of which was rhinovirus at 35.9%. Among the 169 children hospitalized for CAP at the eight institutions, ≥1 respiratory virus was detected in 92.3%, among which respiratory syncytial virus (79.8%) was the most prevalent. Conclusions: Even during the COVID-19 pandemic, Korean children were hospitalized with CAP caused by seasonal respiratory viral pathogens. Although atypical and pyogenic bacteria were not detected, continuous clinical monitoring and further prospective studies should be conducted.

• Clinical and Experimental Pediatrics
• /
• v.53 no.6
• /
• pp.705-710
• /
• 2010

Purpose: To determine the clinical manifestations and outcomes of patients with tracheoesophageal fistula (TEF) and esophageal atresia (EA) born at a single neonatal intensive care unit. Methods: A retrospective analysis was conducted for 97 patients with confirmed TEF and EA who were admitted to the neonatal intensive care unit between 1990 and 2007. Results: The rate of prenatal diagnosis was 12%. The average gestational age and birth weight were $37^{+2}$ weeks and $2.5{\pm}0.7kg$, respectively. Thirty-one infants were born prematurely (32%). Type C was the most common. The mean gap between the proximal and distal esophagus was 2 cm. Esophago-esophagostomy was performed in 72 patients at a mean age of 4 days after birth; gastrostomy or duodenostomy were performed in 8 patients. Forty patients exhibited vertebral, anorectal, cardiac, tracheoesophageal, renal, limb (VACTERL) association with at least 2 combined anomalies, and cardiac anomaly was the most common. The most common post-operative complications were esophageal stricture followed by gastroesophageal reflux. Balloon dilatation was performed for 1.3 times in 26 patients at a mean age of 3 months. The mortality and morbidity rates were 24% and 67%, respectively, and the most common cause of death was sepsis. The weight of approximately 40% patients was below the 10th percentile at 2 years of age. Conclusion: Mortality and morbidity rates of patients with TEF and EA are high as compared to those of infants with other neonatal surgical diseases. Further efforts must be taken to reduce mortality and morbidity and improve growth retardation.

• Clinical and Experimental Pediatrics
• /
• v.53 no.1
• /
• pp.106-110
• /
• 2010

Hippocampal sclerosis (HS) is one of the most common features of intractable temporal lobe epilepsy. Generally it can be identified through brain magnetic resonance imaging (MRI) with high degree of sensitivity and specificity. Typical brain MRI findings of HS are hippocampal atrophy with hyperintense signal confined to the lesion. On the other hand cortical dysplasia exhibits blurring of the gray-white matter junction and abnormal white matter signal intensity. We present a case where preoperative brain MRI strongly suggested the presence of diffuse cortical dysplasia in the left temporal lobe but postoperative pathology revealed the temporal lesion to be unremarkable except for hippocampal sclerosis.

• Journal of Genetic Medicine
• /
• v.14 no.2
• /
• pp.56-61
• /
• 2017

Purpose: Neurofibromatosis type 2 (NF2) is characterized by multiple tumors, including vestibular schwannoma (VS) and others affecting cranial and peripheral nerves. NF2 is caused by mutation of the NF2 gene. The mutation spectrum of NF2 has not been characterized in Korean patients. In the current study, the clinical and genetic characteristics of Korean NF2 patients were analyzed. Materials and Methods: Twenty-five unrelated Korean families were enrolled according to the Manchester criteria. Genetic analysis was performed by direct sequencing and multiplex ligation-dependent probe amplification methods using genomic DNA from peripheral lymphocytes or tumor tissues. Results: All patients had bilateral/unilateral VS and/or other cranial and peripheral nerve tumors. Two patients were familial cases and the other 24 patients were sporadic. Germline NF2 mutations were detected in peripheral lymphocytes from both familial cases, but only in 26.1% of the 23 sporadic families. Somatic mutations were also found in tumor tissues from two of the sporadic families. These somatic mutations were not found in peripheral lymphocytes. A total of 10 different mutations including 2 novel mutations were found in 40.0% of studied families. Five mutations (50.0%) were located in exon 6 of NF2, the FERM domain coding region. Conclusion: Family history was an important factor in identifying germline NF2 mutations. Further study is required to investigate whether exon 6 is a mutation hotspot in Korean NF2 patients and its correlation to phenotypic severity.