• Childhood Kidney Diseases
• /
• v.13 no.1
• /
• pp.14-20
• /
• 2009

Growth retardation is a common consequenc of chronic kidney disease (CKD) in childhood. Many recent clinical and experimental data indicate that growth failure in CKD is mainly due to a relative GH insensitivity and functional IGF-I deficiency. Glucocorticoids also glucocorticoids interfere with the integrity of the somatotropic hormone axis at various levels. Over the past 10 years, recombinant growth hormone (rhGH) has been used to help short children with chronic kidney disease. A GH dosage of 0.35 mg/kg/week (28 IU/$m^2$/week) appears efficient and safe. Some clinical trial data show that final height will be within the normal target height range when GH treatment is continued for many years without remarkable adverse events.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• v.18 no.3
• /
• pp.168-174
• /
• 2015

Purpose: Recent studies have suggested that decreased serum potassium level may contribute to various metabolic disorders in adult patients including nonalcoholic fatty liver disease (NAFLD). We aimed to study the correlation between serum potassium levels and the histologic severity of NAFLD in children. Methods: Pediatric patients with biopsy-proven NAFLD were included in this study. Demographic, clinical, and histopathological data were obtained. Multivariable logistic regression analysis was used to assess whether potassium levels are associated with the presence of nonalcoholic steatohepatitis (NASH) or fibrosis after adjusting for possible confounders. A p-value <0.05 was considered statistically significant. Results: Among 125 biopsies, 49.6% (62) had evidence of NASH while 66.4% (83) had some degree of fibrosis (stage 1-3). Mean serum potassium was significantly lower in NASH group as compared to non-NASH group ($4.4{\pm}0.42mmoL/L$ vs. $4.8{\pm}0.21$, p<0.001). Higher potassium level had negative correlation with presence of steatosis, ballooning, lobular inflammation, fibrosis and NAFLD activity score (p<0.05). On multivariable analysis and after adjusting for the metabolic syndrome and insulin resistance, higher potassium level was significantly associated with lower likelihood of having a histological diagnosis of NASH on biopsy (odds ratio [OR], 0.12; 95% confidence interval [95% CI], 0.05-0.28; p<0.001). Similarly, the likelihood of having fibrosis decreases by 76% for every 0.5 mmoL/L increase in potassium (OR, 0.24; 95% CI, 0.11-0.54; p<0.001). Conclusion: Our study shows an inverse relationship between serum potassium levels and the presence of aggressive disease (NASH and fibrosis) in children with NAFLD.

• Childhood Kidney Diseases
• /
• v.21 no.2
• /
• pp.81-88
• /
• 2017

Purpose: The American Society for Apheresis provides clinical guidelines for therapeutic apheresis in adults, but there are no guidelines for children. This study aimed to analyze the effect of therapeutic plasma exchange (TPE) in pediatric patients with various kidney diseases in Korea. Methods: We retrospectively reviewed the data of 16 children (up to 18 years of age) who were admitted to Severance Children's Hospital with refractory kidney disease. All patients received TPE between 1994 and 2016. Clinical and laboratory characteristics such as age, weight, sex, change in blood urea nitrogen (BUN), and creatinine level before and after TPE, and complications after TPE were analyzed. Results: The mean age and weight of the 16 patients at the time of TPE was $11.3{\pm}4.0$ years and $34.6{\pm}17.5$ kg, respectively. The BUN level was 35.4 mg/dL before TPE and significantly decreased to 21.5 mg/dL (P=0.025) at 1 week and 20.5 mg/dL (P= 0.01) at 1 month after TPE. The creatinine level significantly decreased from 1.20 mg/dL before TPE to 0.90 mg/dL (P=0.02) at 1 week after TPE. Four complications (hypovolemia, anemia, hypocalcemia, and thrombocytopenia) were reported, but were not fatal. Conclusion: Our findings suggest that TPE is an effective therapeutic modality in children with refractory kidney disease and can be indicated for the treatment of various kidney diseases.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• v.20 no.4
• /
• pp.259-262
• /
• 2017

Mitochondria play essential role in eukaryotic cells including in the oxidative phosphorylation and generation of adenosine triphosphate via the electron-transport chain. Therefore, defects in mitochondrial DNA (mtDNA) can result in mitochondrial dysfunction which leads to various mitochondrial disorders that may present with various neurologic and non-neurologic manifestations. Mutations in the nuclear gene polymerase gamma (POLG) are associated with mtDNA depletions, and Alpers-Huttenlocher syndrome is one of the most severe manifestations of POLG mutation characterized by the clinical triad of intractable seizures, psychomotor regression, and liver failure. The hepatic manifestation usually occurs late in the disease's course, but in some references, hepatitis was reportedly the first manifestation. Liver transplantation was considered contraindicated in Alpers-Huttenlocher syndrome due to its poor prognosis. We acknowledged a patient with the first manifestation of the disease being hepatic failure who eventually underwent liver transplantation, and whose neurological outcome improved after cocktail therapy.

• Journal of Korean Neurosurgical Society
• /
• v.64 no.3
• /
• pp.380-385
• /
• 2021

Caudal agenesis refers to the congenital malformation with the essential feature of the agenesis of the sacrococcygeal bone. It is associated with various types of spinal cord anomaly as well as with complex anomalies of genitourinary or gastrointestinal system. The wide spectrum of the disease can be attributed to its pathoembryological origin, the secondary neurulation. This review presents the definition, etiology, classification, and clinical features of the disease.

• Clinical and Experimental Pediatrics
• /
• v.49 no.8
• /
• pp.830-832
• /
• 2006

Immune thrombocytopenic purpura(ITP) is an autoimmune disease characterized by increased peripheral platelet destruction due to antibody to platelet, which results in thrombocytopenia and cutaneous or mucosal bleeding. Bleeding generally occurs when platelet counts fall to less than $20,000/{\mu}L$. Children affected with ITP are usually healthy prior to the onset of the disease and typically present suddenly after a viral infection or insidiously with progressive petechiae, bruising, or purpura. In most cases the disease is self-limited; approximately 80% of children recover by 6 months after diagnosis, with or without treatment. Children with thrombocytopenia persisting for more than 6 months are defined as having chronic ITP. Clinical manifestations, diagnosis, laboratory findings, differential diagnosis and various treatment modalities are reviewed.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• v.24 no.2
• /
• pp.197-206
• /
• 2021

Purpose: Celiac disease (CD) is a common autoimmune disease with extra-intestinal manifestations, including neurological disorders. There are few reports to assess various factors in increasing the chances of developing neurological disorders in CD, so we designed this study. Methods: All patients with CD at any age who had been referred to the Celiac Clinic were evaluated for neurological problems. CD was defined as IgA anti-transglutaminase antibodies (anti-tTG) of 18 IU/mL or higher in serology and Marsh type I or more severe in histopathological evaluation. Logistic regression analysis was used to evaluate the impact of various independent variables on the neurological manifestations. Results: A total of 540 patients enrolled in this study. A 360 (66.7%) of patients were children. A 64.8% and 35.2% were female and male, respectively. Overall, 34.1% of patients had neurological manifestation, including headache, neuropathy, epilepsy, and ataxia. The odds of developing neurological manifestations in children were significantly lower than in adults (odds ratio [OR], 0.66; 95% confidence interval [CI], 0.45-0.96; p=0.03) and in patients with gastrointestinal (GI) symptoms significantly higher than in the group without GI manifestations (OR, 1.77; 95% CI, 1.18-2.63; p=0.005). Other variables, including Marsh classification (OR, 0.44; 95% CI, 0.18-1.11; p=0.08) and anti-tTG levels (OR, 1.00; 95% CI, 0.999-1.001; p=0.59) did not significantly increase the chances of developing neurological disorders. Conclusion: Our study showed that increasing age and the presence of GI symptoms, but not serological and histological findings, could increase the chances of developing neurological diseases in CD patients.

• Clinical and Experimental Pediatrics
• /
• v.46 no.2
• /
• pp.188-191
• /
• 2003

Tsutsugamushi fever is a serious febrile disease outwardly similar to malaria and various arboviral infections. The endemic area is Asia-Pacific. This disease are generally associated with disturbed habitats favoring large populations of vector larval trombiculid mites. Therapy with tetracycline, chloramphenicol or ciprofloxacin is currently recommanded. Unfortunately these drugs is suboptimal for children and pregnant women. Recently, azithromycin has been proven to be effective in therapy of scrub typhus. There is no evidence that azithromycin causes adverse reaction to developing fetus or children. We report four cases of tsutsugamushi fever in children successfully treated with azithromycin. This is the first report describing clinical application of azithromycin to tsutsugamushi fever in Korean children.

• Clinical and Experimental Pediatrics
• /
• v.50 no.5
• /
• pp.422-429
• /
• 2007

Measurement of lung function is an integral component of respiratory physiology and of clinical assessment of lung diseases in school age children and adults. Pulmonary function test of infants and children under the age of 2 years have now been standardised and are being used both in research and as an adjunct to clinical management. By contrast, until recegntly, children of preschool age, i.e. between 2-6 years represented a major challenge for pulmonary function test assessment, this particular period commonly being referred to as the 'dark ages' of Pediatric Pulmonology. Measurement of lung function in preschool-aged children is now feasible. However, much work remains to be done in standardizing how these tests are performed, and in understanding the most appropriate role for the various tests in the study of growth and development of the respiratory system and in the clinical management of children in this age group. As the field develops and the knowledge of respiratory physiology in this age group expands, investigation of different and more appropriate algorithm use in preschool children, together with development of more appropriate reference data, may result in improved disease discrimination.

• Pediatric Gastroenterology, Hepatology & Nutrition
• /
• v.24 no.5
• /
• pp.492-499
• /
• 2021

Purpose: Celiac disease (CD) is an autoimmune disorder of the small intestine caused by an abnormal immune response to gluten proteins and is often characterized by gastrointestinal symptoms. Food allergy (FA) is an adverse immune sensitivity to ingested food proteins leading to inflammation in various organs including the gastrointestinal tract. The relationship between CD and FA remains unclear. This study aimed to assess the prevalence and clinical relevance of immunoglobulin E (IgE)-mediated food sensitization in children with CD. Methods: Fifty-nine children diagnosed with CD were reviewed for clinical symptoms and evidence of IgE-sensitization to food and airborne allergens using the PolyCheck method. Results: IgE-mediated sensitization has been diagnosed in 20.3% of children with CD (CD/A). In the CD/A group, 58.3% of children were sensitized to food and 66.7% to airborne allergens. Further, 41.7% of patients with CD and allergy reported gastrointestinal tract symptoms associated with the ingestion of sensitizing foods. Analysis of the clinical status revealed that the incidence of other allergic disorders in the CD/A group was as follows: atopic dermatitis (33.3%), asthma (25.0%), and allergic rhinitis (16.7%). The percentage of eosinophils was significantly higher in the CD/A group than in the CD group (0.33±0.25 vs. 0.11±0.09; p=0.006). Conclusion: The diagnosis of CD does not exclude FA. The gastrointestinal symptoms in children with CD may be the result of both CD and FA; therefore, children with CD should be evaluated for the presence of FA regardless of age.