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Deficiency of antidiuretic hormone: a rare cause of massive polyuria after kidney transplantation.

Kyung Mi JANG ; Young Soo SOHN ; Young Ju HWANG ; Bong Seok CHOI ; Min Hyun CHO

Korean Journal of Pediatrics.2016;59(4):202-204. doi:10.3345/kjp.2016.59.4.202

A 15-year-old boy, who was diagnosed with Alport syndrome and end-stage renal disease, received a renal transplant from a living-related donor. On postoperative day 1, his daily urine output was 10,000 mL despite normal graft function. His laboratory findings including urine, serum osmolality, and antidiuretic hormone levels showed signs similar to central diabetes insipidus, so he was administered desmopressin acetate nasal spray. After administering the desmopressin, urine specific gravity and osmolality increased abruptly, and daily urine output declined to the normal range. The desmopressin acetate was tapered gradually and discontinued 3 months later. Graft function was good, and urine output was maintained within the normal range without desmopressin 20 months after the transplantation. We present a case of a massive polyuria due to transient deficiency of antidiuretic hormone with the necessity of desmopressin therapy immediately after kidney transplantation in a pediatric patient.
Adolescent ; Deamino Arginine Vasopressin ; Diabetes Insipidus, Neurogenic ; Humans ; Kidney Failure, Chronic ; Kidney Transplantation* ; Kidney* ; Male ; Nephritis, Hereditary ; Osmolar Concentration ; Polyuria* ; Reference Values ; Specific Gravity ; Tissue Donors ; Transplants

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Mycoplasma pneumoniae associated stroke in a 3-year-old girl.

Gun Ha KIM ; Won Hee SEO ; Bo Kyung JE ; So Hee EUN

Korean Journal of Pediatrics.2013;56(9):411-415. doi:10.3345/kjp.2013.56.9.411

Infectious diseases precede a significant proportion of acute ischemic strokes in children. Here, we report a case of acute ischemic stroke in a 3-year-old girl with a Mycoplasma pneumonia-associated respiratory tract infection. She developed an acquired prothrombotic state of protein S deficiency and had increased fibrinogen and fibrinogen degradation product levels and increased titer of antinuclear antibodies. However, these conditions were completely alleviated at the 1-month follow-up examination. Infection with M. pneumoniae may cause a transient prothrombotic state that can potentially cause a thrombus.
Antibodies, Antinuclear ; Child ; Communicable Diseases ; Fibrinogen ; Follow-Up Studies ; Humans ; Mycoplasma ; Mycoplasma pneumoniae ; Pneumonia ; Pneumonia, Mycoplasma ; Preschool Child ; Protein S Deficiency ; Respiratory Tract Infections ; Stroke ; Thrombosis

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Klebsiella pneumoniae liver abscess in an immunocompetent child.

Jang Mi KWON ; Hye Lim JUNG ; Jae Won SHIM ; Deok Soo KIM ; Jung Yeon SHIM ; Moon Soo PARK

Korean Journal of Pediatrics.2013;56(9):407-410. doi:10.3345/kjp.2013.56.9.407

Klebsiella pneumoniae has emerged as a leading pathogen that causes pyogenic liver abscesses (PLAs) in Korea. K. pneumoniae liver abscess (KLA) is potentially life threatening, and the diagnosis is difficult. In developed countries, PLA is rarely observed in children and is frequently associated with disorders of granulocyte function and previous abdominal infection. We observed a case of KLA in a healthy 12-year-old boy. To our knowledge, this is the first reported case of KLA in an immunocompetent child without an underlying disease in Korea. The patient was treated with percutaneous catheter drainage and antibiotics. The catheter was placed in the intrahepatic abscess for 3 weeks and parenteral antibiotics (ceftriaxone and amikacin) were administered for 4 weeks, followed by oral antibiotics (cefixime) for 2 weeks. We reported this case to raise awareness of KLA in immunocompetent children among physicians, and to review the diagnosis, risk factors, potential complications, and appropriate treatment of KLA.
Abscess ; Anti-Bacterial Agents ; Catheters ; Child ; Developed Countries ; Drainage ; Granulocytes ; Humans ; Klebsiella ; Klebsiella pneumoniae ; Korea ; Liver ; Liver Abscess ; Liver Abscess, Pyogenic ; Pneumonia ; Risk Factors

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High-dose chemotherapy and autologous peripheral blood stem cell transplantation in the treatment of children and adolescents with Ewing sarcoma family of tumors.

Juhee SEO ; Dong Ho KIM ; Jung Sub LIM ; Jae Soo KOH ; Ji Young YOO ; Chang Bae KONG ; Won Seok SONG ; Wan Hyeong CHO ; Dae Geun JEON ; Soo Yong LEE ; Jun Ah LEE

Korean Journal of Pediatrics.2013;56(9):401-406. doi:10.3345/kjp.2013.56.9.401

PURPOSE: We performed a pilot study to determine the benefit of high-dose chemotherapy and autologous peripheral blood stem cell transplantation (HDCT/autoPBSCT) for patients with Ewing sarcoma family of tumors. METHODS: We retrospectively analyzed the data of patients who received HDCT/autoPBSCT at Korea Cancer Center Hospital. Patients with relapsed, metastatic, or centrally located tumors were eligible for the study. RESULTS: A total of 9 patients (3 male, 6 female), with a median age at HDCT/autoPBSCT of 13.4 years (range, 7.1 to 28.2 years), were included in this study. Patients underwent conventional chemotherapy and local control either by surgery or radiation therapy, and had achieved complete response (CR, n=7), partial response (n=1), or stable disease (n=1) prior to HDCT/autoPBSCT. There was no transplant-related mortality. However, the median duration of overall survival and event-free survival after HDCT/autoPBSCT were 13.3 months (range, 5.3 to 44.5 months) and 6.2 months (range, 2.1 to 44.5 months), respectively. At present, 4 patients are alive and 5 patients who experienced adverse events (2 metastasis, 2 local recur, and 1 progressive disease) survived for a median time of 2.8 months (range, 0.1 to 10.7 months). The 2-year survival after HDCT/autoPBSCT was 44.4%+/-16.6% and disease status at the time of HDCT/autoPBSCT tended to influence survival (57.1%+/-18.7% of cases with CR vs. 0% of cases with non-CR, P=0.07). CONCLUSION: Disease status at HDCT/autoPBSCT tended to influence survival. Further studies are necessary to define the role of HDCT/autoPBSCT and to identify subgroup of patients who might benefit from this investigational treatment.
Adolescent ; Child ; Disease-Free Survival ; Humans ; Korea ; Male ; Neoplasm Metastasis ; Peripheral Blood Stem Cell Transplantation ; Pilot Projects ; Retrospective Studies ; Sarcoma, Ewing ; Stem Cell Transplantation ; Therapies, Investigational

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Transcatheter closure of small ductus arteriosus with amplatzer vascular plug.

Eun Hyun CHO ; Jinyoung SONG ; I Seok KANG ; June HUH ; Sang Yoon LEE ; Eun Young CHOI ; Soo Jin KIM

Korean Journal of Pediatrics.2013;56(9):396-400. doi:10.3345/kjp.2013.56.9.396

PURPOSE: The purpose of this study was to share our experience of transcatheter closure of small patent ductus arteriosus (PDA) by using an Amplatzer vascular plug (AVP). METHODS: We reviewed the medical records of 20 patients who underwent transcatheter closure at Samsung Medical Center and Sejong General Hospital from January 2008 to August 2012. The size and shape of the PDAs were evaluated by performing angiograms, and the PDA size and the AVP devices size were compared. RESULTS: The mean age of the patients was 54.9+/-45.7 months old. The PDAs were of type C (n=5), type D (n=12), and type E (n=3). The mean pulmonary end diameter of the PDA was 1.7+/-0.6 mm, and the aortic end diameter was 3.6+/-1.4 mm. The mean length was 7.3+/-1.8 mm. We used 3 types of AVP devices: AVP I (n=5), AVP II (n=7), and AVP IV (n=8). The ratio of AVP size to the pulmonary end diameter was 3.37+/-1.64, and AVP size/aortic end ratio was 1.72+/-0.97. The aortic end diameter was significantly larger in those cases repaired with AVP II than in the others (P=0.002). The AVP size did not significantly correlate with the PDA size, but did correlate with smaller ratio of AVP size to aortic end diameter (1.10+/-0.31, P=0.032). CONCLUSION: Transcatheter closure of small PDA with AVP devices yielded satisfactory outcome. AVP II was equally effective with smaller size of device, compared to others.
Cardiac Catheterization ; Ductus Arteriosus ; Ductus Arteriosus, Patent ; Hospitals, General ; Humans ; Medical Records ; Vascular Access Devices

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Changes of clinical characteristics and outcomes in patients with Kawasaki disease over the past 7 years in a single center study.

Hye Jin KANG ; Gee Na KIM ; Hong Ryang KIL

Korean Journal of Pediatrics.2013;56(9):389-395. doi:10.3345/kjp.2013.56.9.389

PURPOSE: This single-center study was conducted to assess the changes in epidemiological and clinical characteristics and outcomes of patients with Kawasaki disease (KD) over the past 7 years. METHODS: This retrospective study included 135 children with KD, admitted to Chungnam National University Hospital, Daejeon, between 2004 and 2005 (group A, n=53) and between 2011 and 2012 (group B, n=82). Medical records were reviewed to obtain information regarding the presenting signs and symptoms, demographic characteristics, and laboratory and echocardiographic findings associated with KD. RESULTS: The hospital admission date after onset was significantly earlier in group B than in group A (P=0.008). The proportion of patients with incomplete KD was 45.3% and 65.9% in group A and B, respectively (P=0.018). The number of pretreatment coronary artery lesions (CALs) were significantly lesser in group B than in group A. (10/53 vs. 5/82, P=0.021). No significant differences was observed in the incidence of CALs at discharge, febrile phase duration, hospital stay duration, incidence of retreatment, and intravenous immunoglobulin dose between 2 groups. The total febrile phase was shorter in patients with incomplete KD than in those with complete KD in both groups. CONCLUSION: The proportion of incomplete KD has become higher. Furthermore, early admission and management of patients with KD may be related to increased incomplete KD and decreased CALs. Therefore, we believe that a diagnostic strategy for incomplete KD should be established regardless of the presence of coronary lesions.
Child ; Coronary Vessels ; Humans ; Immunoglobulins ; Incidence ; Length of Stay ; Medical Records ; Mucocutaneous Lymph Node Syndrome ; Retreatment ; Retrospective Studies

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ERRATUM: A childhood case of spinal tuberculosis misdiagnosed as muscular dystrophy.

Doo Il SONG ; Su Ye SOHN ; Yun Kyung KIM ; So Hee EUN ; Young Jun RHIE ; Gi Young JANG ; Chan Wook WOO ; Byung Min CHOI ; Jung Hwa LEE ; Bo Kyung JE

Korean Journal of Pediatrics.2010;53(6):727-727. doi:10.3345/kjp.2010.53.6.727

No abstract available.

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A case report of chronic granulomatous disease presenting with aspergillus pneumonia in a 2-month old girl.

Eun LEE ; Seak Hee OH ; Ji Won KWON ; Byoung Ju KIM ; Jinho YU ; Chan Jeoung PARK ; Soo Jong HONG

Korean Journal of Pediatrics.2010;53(6):722-726. doi:10.3345/kjp.2010.53.6.722

Chronic granulomatous disease (CGD) is an uncommon inherited disorder caused by mutations in any of the genes encoding subunits of the superoxide-generating phagocyte NADPH oxidase system, which is essential for killing catalase producing bacteria and fungi, such as Aspergillus species, Staphylococcus aureus, Serratia marcescens, Nocardia species and Burkholderia cepacia. In case of a history of recurrent or persistent infections, immune deficiency should be investigated. Particularly, in the case of uncommon infections such as aspergillosis in early life, CGD should be considered. We describe here a case of CGD that presented with invasive pulmonary aspergillosis in a 2-month-old girl. We confirmed pulmonary aspergillosis noninvasively through a positive result from the culture of bronchial alveolar lavage fluid, positive serological test for Aspergillus antigen and radiology results. She was successfully treated with Amphotericin B and recombinant IFN-gamma initially. Six weeks later after discharge, she was readmitted for pneumonia. Since there were infiltrates on the right lower lung, which were considered as residual lesions, voriconazole therapy was initiated. She showed a favorable response to the treatment and follow-up CT showed regression of the pulmonary infiltrates.
Amphotericin B ; Aspergillosis ; Aspergillus ; Bacteria ; Bronchoalveolar Lavage Fluid ; Burkholderia cepacia ; Catalase ; Follow-Up Studies ; Fungi ; Granulomatous Disease, Chronic ; Homicide ; Humans ; Infant ; Invasive Pulmonary Aspergillosis ; Lung ; NADPH Oxidase ; Nocardia ; Phagocytes ; Pneumonia ; Pulmonary Aspergillosis ; Pyrimidines ; Serologic Tests ; Serratia marcescens ; Staphylococcus aureus ; Triazoles

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A case of Rubinstein-Taybi Syndrome with a CREBbinding protein gene mutation.

Se Hee KIM ; Byung Chan LIM ; Jong Hee CHAE ; Ki Joong KIM ; Yong Seung HWANG

Korean Journal of Pediatrics.2010;53(6):718-721. doi:10.3345/kjp.2010.53.6.718

Rubinstein-Taybi syndrome (RTS) is a congenital disorder characterized by typical facial features, broad thumbs and toes, with mental retardation. Additionally, tumors, keloids and various congenital anomalies including congenital heart defects have been reported in RTS patients. In about 50% of the patients, mutations in the CREB binding protein (CREBBP) have been found, which are understood to be associated with cell growth and proliferation. Here, we describe a typical RTS patient with Arnold-Chiari malformation. A mutation in the CREBBP gene, c.4944_4945insC, was identified by mutational analysis.
Arnold-Chiari Malformation ; Congenital, Hereditary, and Neonatal Diseases and Abnormalities ; CREB-Binding Protein ; Heart Defects, Congenital ; Humans ; Intellectual Disability ; Keloid ; Rubinstein-Taybi Syndrome ; Thumb ; Toes

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Analysis of reports on orphan lung diseases in Korean children.

Sun Jung JANG ; Hyun Kyung SEO ; Sung Jae YI ; Kyong Min KIM ; Hye Mi JEE ; Man Yong HAN

Korean Journal of Pediatrics.2010;53(6):711-717. doi:10.3345/kjp.2010.53.6.711

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.
Aged ; Bronchiolitis Obliterans ; Child ; Child, Orphaned ; Disease Management ; Humans ; Hypersensitivity ; Incidence ; Korea ; Lung ; Lung Diseases ; Pediatrics ; Prevalence ; Publications

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