• Title/Summary/Keyword: pediatric diseases

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Primary Immunodeficiencies in Children Initially Admitted with Gastrointestinal/Liver Manifestations

  • Murat Cakir ;Nalan Yakici ;Elif Sag ;Gulay Kaya ;Aysenur Bahadir;Alper Han Cebi ;Fazil Orhan
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.26 no.4
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    • pp.201-212
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    • 2023
  • Purpose: The gastrointestinal system is the most commonly affected organ, followed by the lungs, in patients with primary immunodeficiency disease (PID). Hence, it is common for children with PIDs to present with gastrointestinal symptoms. We aimed to analyze the clinical and histopathological findings of patients who were initially admitted to pediatric gastroenterology/hepatology clinics and subsequently diagnosed with PIDs to identify the clinical clues for PIDs. Methods: The demographic, laboratory, and histopathological findings, treatment modality, and outcomes of patients initially admitted to the pediatric gastroenterology/hepatology unit and subsequently diagnosed with PIDs were recorded. Results: The study included 24 patients (58.3% male; median age [range]: 29 [0.5-204] months). Common clinical presentations included chronic diarrhea (n=8), colitis (n=6), acute hepatitis (n=4), and acute liver failure (n=2). The association of autoimmune diseases, development of malignant diseases, and severe progression of viral diseases was observed in 20.8%, 8.3%, and 16.6% of the patients, respectively. Antibody deficiency was predominantly diagnosed in 29.2% of patients, combined immunodeficiency in 20.8%, immune dysregulation in 12.5%, defects in intrinsic and innate immunity in 4.2%, autoinflammatory disorders in 8.3%, and congenital defects of phagocytes in 4.2%. Five patients remained unclassified (20.8%). Conclusion: Patients with PIDs may initially experience gastrointestinal or liver problems. It is recommended that the association of autoimmune or malignant diseases or severe progression of viral diseases provide pediatric gastroenterologists some suspicion of PIDs. After screening using basic laboratory tests, genetic analysis is mandatory for a definitive diagnosis.

Analysis of reports on orphan lung diseases in Korean children

  • Jang, Sun-Jung;Seo, Hyun-Kyung;Yi, Sung-Jae;Kim, Kyong-Min;Jee, Hye-Mi;Han, Man-Yong
    • Clinical and Experimental Pediatrics
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    • v.53 no.6
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    • pp.711-717
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    • 2010
  • Purpose: Orphan lung diseases are defined as lung diseases with a prevalence of 1 or less in 2,000 individuals. Despite an increase in the numbers of patients with such diseases, few studies on Korean children have appeared. To obtain epidemiologic and demographic data on these diseases, we systematically reviewed reports on pediatric orphan lung diseases in Korea over the last 50 years. Methods: We reviewed 223 articles that have appeared since 1958 on orphan lung diseases in Korean children. These articles described a total of 519 patients aged between 0 and 18 years. We classified patients by year of publication, diagnosis, geographic region, and journal. Results: Of 519 patients, 401 had congenital cystic lung diseases and 66 had bronchiolitis obliterans. About 80% of patients were described in reports published in three journals, Pediatric Allergy and Respiratory Disease (Korea), the Korean Journal of Pediatrics, and the Korean Journal of Thoracic and Cardiovascular Surgery, in which papers on 157 (30.2%), 138 (26.6%), and 111 (21.4%) patients appeared, respectively. The frequency of publication of case reports has increased since 1990. Of the 519 patients, 401 (77.3%) were from Seoul/Gyeonggi-do and 72 (13.9%) from Busan/Gyeongsangnam-do. Conclusion: The prevalence of pediatric orphan lung disease has increased since 1990, and some provinces of Korea have a higher incidence of these diseases than do others. Studies exploring the incidence of pediatric orphan lung diseases in Korea are needed for effective disease management.

SARS-CoV-2 Antibodies in Children with Chronic Disease from a Pediatric Gastroenterology Outpatient Clinic

  • Kaya, Gulay;Issi, Fatma;Guven, Burcu;Ozkaya, Esra;Buruk, Celal Kurtulus;Cakir, Murat
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.25 no.5
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    • pp.422-431
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    • 2022
  • Purpose: At the beginning of the Coronavirus disease (COVID-19) epidemic, physicians paid close attention to children with chronic diseases to prevent transmission or a severe course of infection. We aimed to measure the severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) antibody levels in children with chronic gastrointestinal and liver diseases to analyze the risk factors for infection and its interaction with their primary disease. Methods: This cross-sectional study analyzed SARS-CoV-2 antibody levels in patients with gastrointestinal and liver diseases (n=141) and in healthy children (n=48) between January and February 2021. Results: During the pandemic, 10 patients (7%) and 1 child (2%) had confirmed COVID-19 infection (p=0.2). The SARS-CoV-2 antibody test was positive in 36 patients (25.5%) and 11 children (22.9%) (p=0.7). SARS-CoV-2 antibody positivity was found in 20.4%, 26.6%, 33.3%, and 33.3% of patients with chronic liver diseases, chronic gastrointestinal tract diseases, cystic fibrosis, and liver transplantation recipients, respectively (p>0.05, patients vs. healthy children). Risk factors for SARS-CoV-2 antibody positivity were COVID-19-related symptoms (47.2% vs. 14.2%, p=0.00004) and close contact with SARS-CoV-2 polymerase chain reaction-positive patients (69.4% vs. 9%, p<0.00001). The use, number, and type of immunosuppressants and primary diagnosis were not associated with SARS-CoV-2 antibody positivity. The frequency of disease activation/flare was not significant in patients with (8.3%) or without (14.2%) antibody positivity (p=0.35). Conclusion: SARS-CoV-2 antibodies in children with chronic gastrointestinal and liver diseases are similar to that in healthy children. Close follow-up is important to understand the long-term effects of past COVID-19 infection in these children.

A Clinical study on the pediatric patients who visited Emergency Room of Oriental Medical Hospital (한방의료기관내 응급실에 내원한 소아환자에 관한 임상적 고찰)

  • Chang, Gyu-Tae;Kim, Jang-Hyun;Kang, Mi-Sun
    • The Journal of Pediatrics of Korean Medicine
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    • v.17 no.1
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    • pp.117-129
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    • 2003
  • We made a clinical analysis of 135 pediatric patients who visited Emergency Room of Dongguk University Oriental Hospital, during 1 year from March 2002 to February 2003. The results were as follows ; 1. Whether they received medical examination and treatment or not : new patients were 95(70.4%) and pediatric patients who received medical examination and treatment were 40(29.6%). 2. Seasonal distribution: Spring(March, April, May) was 45(33.3%), Summer(June, July, August) was 35(25.9%), Autumn(September, October, November) was 32(23.7%) and Winter(December, January, February) was 23(17.0%). 3. The time interval between onset and arrival : within 6 hours were 68(50.4%), 6-12 hours were 14(10.4%), 12-24 hours were 26(9.3%), 24-48 hours were 17(12.6%), 48-72 hours were 6(4.4), over 72 hours were 4(3.0%). 4. Whether they went through other hospitals or not : pediatric patients who didn't go through other hospitals were 105(77.8%), pediatric patients who went through other hospitals were 30(22.2%). 5. Systemic distribution of diseases: Infectious diseases were 1(0.7%), Physique diseases were 4(3.0%), Digestive diseases were 73(54.1), Nervous also Mental diseases were 32(23.7%), Cardiovascular diseases were 2(1.5%), Skin disorders were 1(0.7%), Respiratory diseases were 22(16.3%). 6. Medical care : Acupuncture and moxibustion were 1(0.7%), moxibustion and medication were 1(0.7%), acupuncture, moxibustion and medication were 1(0.7%), venesection was 24(17.8% ), venesection and medication were 17(12.6%), consultation was 16(11.9%), medication was 44(32.6%), acupuncture was 3(2.2%), acupuncture and medication were 14(10.4%) and transferred out patients were 14(10.4%). 7. Revisit: revisit patients were 30(22.2%).

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A Study of the Chief Complaints of Pediatric Outpatients (소아과(小兒科) 외래(外來) 환자(患者)의 주소증(主訴證)에 관한 고찰(考察))

  • Song, In-Sun;Shin, Ji-Na;Shin, Youn-Guo
    • The Journal of Pediatrics of Korean Medicine
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    • v.16 no.2
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    • pp.69-81
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    • 2002
  • The Purpose of this study was to collect statistics of pediatric problems, and so to investigate the new effective oriental medicine treatments in pediatric disease and its clinical applicability. The Study was composed of 698 new patient who had been treated at the pediatric unit in th Dong Sea Oriental Medical Hospital for 1 year, from 1 September 2001 to 31 October 2002, and aged between 0 and 18 years. The Chief complaints and their care-givers. Result: 1. In the study, male children are 403 and female children are 295. So the sex ratio between male and female is 1.37 : 1. 2. In age distribution, 0-3 years : 317(45.4%), 4-6 years : 148(21.2%). 7-9 years 98(14.0%), 10-12 years: 66(9.5%), 13-15 years : 40(5.7%), 16-18 years 29(4.2%). 3. The chief complaint according to systemic division was Respiratory diseases covers 30.1%, Digestive diseases covers 20.8%, Physique diseases covers 10.6%. 4. The Respiratory diseases tended to increase in autumn and appeared mostly in the age group between 0 and 9 years. The Digestive diseases increase in summer and appeared mostly in the age group between 10 and 12 years. The physique diseases increase in winter and appeared mostly in he age group between 13 and 18 years. 5. Most of patient came to the hospital from diseases such as common cold, weakness, dyspepsia, atopic dermatitis, rhinitis, take a easy cold, short stature, sweating, Bell's palsy, abdominal pain, etc. Conclusion : 1. The chief complaint In pediatric diseases that needed an oriental medical treatment was mainly the disease that tends to take long time and the weakness, and appeared frequency in respiratory and digestive disease. 2. The oriental medical treatment was still preferred as a way to improve the weakness by patients, rather than a way to overcome their disease. in particular, the study shows that the oriental medical treatment should be emphasized in terms of preventing the disease. 3. The new disease, which were developed with change of human life and environment, should be investigated as a new fie of oriental medical treatment.

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Short Bowel Syndrome as the Leading Cause of Intestinal Failure in Early Life: Some Insights into the Management

  • Goulet, Olivier;Nader, Elie Abi;Pigneur, Benedicte;Lambe, Cecile
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.22 no.4
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    • pp.303-329
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    • 2019
  • Intestinal failure (IF) is the critical reduction of the gut mass or its function below the minimum needed to absorb nutrients and fluids required for adequate growth in children. Severe IF requires parenteral nutrition (PN). Pediatric IF is most commonly due to congenital or neonatal intestinal diseases or malformations divided into 3 groups: 1) reduced intestinal length and consequently reduced absorptive surface, such as in short bowel syndrome (SBS) or extensive aganglionosis; 2) abnormal development of the intestinal mucosa such as congenital diseases of enterocyte development; 3) extensive motility dysfunction such as chronic intestinal pseudo-obstruction syndromes. The leading cause of IF in childhood is the SBS. In clinical practice the degree of IF may be indirectly measured by the level of PN required for normal or catch up growth. Other indicators such as serum citrulline have not proven to be highly reliable prognostic factors in children. The last decades have allowed the development of highly sophisticated nutrient solutions consisting of optimal combinations of macronutrients and micronutrients as well as guidelines, promoting PN as a safe and efficient feeding technique. However, IF that requires long-term PN may be associated with various complications including infections, growth failure, metabolic disorders, and bone disease. IF Associated Liver Disease may be a limiting factor. However, changes in the global management of IF pediatric patients, especially since the setup of intestinal rehabilitation centres did change the prognosis thus limiting "nutritional failure" which is considered as a major indication for intestinal transplantation (ITx) or combined liver-ITx.

The Importance of Esophageal and Gastric Diseases as Causes of Chest Pain

  • Kim, Yong Joo;Shin, Eun Jung;Kim, Nam Su;Lee, Young Ho;Nam, Eun Woo
    • Pediatric Gastroenterology, Hepatology & Nutrition
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    • v.18 no.4
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    • pp.261-267
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    • 2015
  • Purpose: Pediatric chest pain is considered to be idiopathic or caused by benign diseases. This study was to find out how much upper gastrointestinal (UGI) diseases are major causes of chest pain in pediatric patients. Methods: The records of 75 children (42 boys and 33 girls, aged 3-17 years old) who have presented with mainly chest pain from January 1995 to March 2015 were retrospectively reviewed. Chest X-ray and electrocardiography (ECG) were performed in all aptients. Further cardiologic and gastrointestinal (GI) evaluations were performed in indicated patients. Results: Chest pain was most common in the children of 6 and 9 to 14 years old. Esopha-gogastric diseases were unexpectedly the most common direct causes of the chest pain, the next are idiopathic, cardiac diseases, chest trauma, respiratory disease, and psychosomatic disease. Even though 21 showed abnormal ECG findings and 7 showed abnormalities on echocardiography, cardiac diseases were determined to be the direct causes only in 9. UGI endoscopy was performed in 57 cases, and esophago-gastric diseases which thereafter were thought to be causative diseases were 48 cases. The mean age of the children with esophago-gastric diseases were different with marginal significance from that of the other children with chest pain not related with esophago-gastric diseases. All the 48 children diagnosed with treated with GI medicines based on the diagnosis, and 37 cases (77.1%) subsequently showed clinical improvement. Conclusion: Diagnostic approaches to find out esophageal and gastric diseases in children with chest pain are important as well as cardiac and respiratory investigations.

The prevalence of pediatric endocrine and metabolic diseases in Korea (한국 소아 내분비 및 대사질환의 역학)

  • Lee, Dong Hwan
    • Clinical and Experimental Pediatrics
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    • v.51 no.6
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    • pp.559-563
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    • 2008
  • The government neonatal screening program has a 17-years history. Therefore, it can now provide information on the prevalence of endocrine and metabolic diseases which included in neonatal screening. Knowledge of the prevalence of metabolic diseases is very important with these results, the most effective screening methods can be determined and diseases can be identified that should be added to neonatal screening. For these purposes regular follow-up of patients with metabolic diseases and quality assuarance are also needed.

The role of fecal calprotectin in pediatric disease

  • Jeong, Su Jin
    • Clinical and Experimental Pediatrics
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    • v.62 no.8
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    • pp.287-291
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    • 2019
  • Fecal calprotectin (FC) is a calcium- and zinc-binding protein of the S100 family, mainly expressed by neutrophils and released during inflammation. FC became an increasingly useful tool both for gastroenterologists and for general practitioners for distinguishing inflammatory bowel disease (IBD) from irritable bowel syndrome. Increasing evidences support the use of this biomarker for diagnosis, follow-up and evaluation of response to therapy of several pediatric gastrointestinal diseases, ranging from IBD to nonspecific colitis and necrotizing enterocolitis. This article summarizes the current literature on the use of FC in clinical practice.

Knowledge and Attitudes about Human Papillomaviruses and Immunization among Turkish Pediatricians

  • Ozsurekci, Yasemin;Oncel, Eda Karadag;Bayhan, Cihangul;Celik, Melda;Ozkaya-Parlakay, Aslinur;Arvas, Mehmet;Ceyhan, Mehmet
    • Asian Pacific Journal of Cancer Prevention
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    • v.14 no.12
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    • pp.7325-7329
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    • 2013
  • Background: Human papillomavirus (HPV) is one of the most common sexually transmitted infectious agents, and the effectiveness of vaccine delivery programs will depend largely upon whether providers recommend vaccines. The objectives of this study were to examine pediatrician characteristics, knowledge, and attitudes associated with HPV and HPV immunization. Materials and Methods: Attendees of the national pediatric meeting in 2011, were asked to complete a questionnaire that, aside from demographic information, elicited level of agreement with statements regarding HPV, its related diseases, and HPV vaccination. It also documented attitudes and beliefs about HPV vaccination. Results: Of the 480 attendees, 226 (47%) filled in the questionnaire. The level of pediatrician HPV-related knowledge varied. The majority (78%) were aware that HPV infection is the most common sexually transmitted infection, while 51% were unaware that a condom is ineffective protection against HPV infection. Between 60-80% of respondents were aware of the effectiveness of HPV vaccination for women. On the other hand, only 10% were aware of reasons why men should be vaccinated against HPV. The majority (75%) of Turkish pediatricians were likely to recommend HPV vaccination to their daughter, if they had one. Seventy percent of pediatricians agreed that the HPV vaccination should be added to the National Immunization Program (NIP) in Turkey. However, the respondents documented concerns about the cost of the vaccination. Conclusions: Increasing pediatricians' knowledge and awareness of HPV and HPV vaccination may assist with the implementation of an effective NIP.