Epidemiologic and Clinical Outcomes of Pediatric Renal Tumors in Korea: A Retrospective Analysis of The Korean Pediatric Hematology and Oncology Group (KPHOG) Data / Journal of the Korean Cancer Association, 대한암학회지

Purpose@#Renal tumors account for approximately 7% of all childhood cancers. These include Wilms tumor (WT), clear cell sarcoma of the kidney (CCSK), malignant rhabdoid tumor of the kidney (MRTK), renal cell carcinoma (RCC), congenital mesoblastic nephroma (CMN) and other rare tumors. We investigated the epidemiology of pediatric renal tumors in Korea. @*Materials and Methods@#From January 2001 to December 2015, data of pediatric patients (0–18 years) newly-diagnosed with renal tumors at 26 hospitals were retrospectively analyzed. @*Results@#Among 439 patients (male, 240), the most common tumor was WT (n=342, 77.9%), followed by RCC (n=36, 8.2%), CCSK (n=24, 5.5%), MRTK (n=16, 3.6%), CMN (n=12, 2.7%), and others (n=9, 2.1%). Median age at diagnosis was 27.1 months (range 0-225.5) and median follow-up duration was 88.5 months (range 0-211.6). Overall, 32 patients died, of whom 17, 11, 1, and 3 died of relapse, progressive disease, second malignant neoplasm, and treatment-related mortality. Five-year overall survival and event free survival were 97.2% and 84.8% in WT, 90.6% and 82.1% in RCC, 81.1% and 63.6% in CCSK, 60.3% and 56.2% in MRTK, and 100% and 91.7% in CMN, respectively (p < 0.001). @*Conclusion@#The pediatric renal tumor types in Korea are similar to those previously reported in other countries. WT accounted for a large proportion and survival was excellent. Non-Wilms renal tumors included a variety of tumors and showed inferior outcome, especially MRTK. Further efforts are necessary to optimize the treatment and analyze the genetic characteristics of pediatric renal tumors in Korea.

Successful Treatment of Severe Hemolytic Disease of the Newborn Caused by Anti-Jk b / 임상소아혈액종양

Hemolytic disease of the newborn (HDN) is a condition in which maternal antibodies cross the placenta and cause hemolytic reactions. Anti-RhD was the most common cause, but with the introduction of immunoglobulin, the frequency has decreased sig nificantly, making hemolytic disease caused by other minor blood g roups more important. Kidd antigen is also known to cause hemolytic transfusion reactions. Only 13 cases have been reported so far, because Kidd antig en dose not usually cause HDN. Most cases have a good outcome, and only two fatal cases have been reported.A four-day-old male patient was hospitalized for jaundice, and hemolysis was confirmed by blood test. The mother’s blood was Jkb antibody positive. The patient did not improve with phototherapy, so an exchange transfusion was performed. Additional hemolysis occurred, so we undertook transfusion of red blood cells, resulting in cessation of hemolysis. We report HDN caused by Jkb antibody that responded to exchange blood transfusion.

Successful Treatment of Severe Hemolytic Disease of the Newborn Caused by Anti-Jk b / 임상소아혈액종양

Hemolytic disease of the newborn (HDN) is a condition in which maternal antibodies cross the placenta and cause hemolytic reactions. Anti-RhD was the most common cause, but with the introduction of immunoglobulin, the frequency has decreased sig nificantly, making hemolytic disease caused by other minor blood g roups more important. Kidd antigen is also known to cause hemolytic transfusion reactions. Only 13 cases have been reported so far, because Kidd antig en dose not usually cause HDN. Most cases have a good outcome, and only two fatal cases have been reported.A four-day-old male patient was hospitalized for jaundice, and hemolysis was confirmed by blood test. The mother’s blood was Jkb antibody positive. The patient did not improve with phototherapy, so an exchange transfusion was performed. Additional hemolysis occurred, so we undertook transfusion of red blood cells, resulting in cessation of hemolysis. We report HDN caused by Jkb antibody that responded to exchange blood transfusion.

Characteristics of pediatric rhabdomyolysis and the associated risk factors for acute kidney injury: a retrospective multicenter study in Korea

Background@#The clinical features of pediatric rhabdomyolysis differ from those of the adults with rhabdomyolysis; however, multicenter studies are lacking. This study aimed to investigate the characteristics of pediatric rhabdomyolysis and reveal the risk factors for acute kidney injury (AKI) in such cases. @*Methods@#This retrospective study analyzed the medical records of children and adolescents diagnosed with rhabdomyolysis at 23 hospitals in South Korea between January 2007 and December 2016. @*Results@#Among 880 patients, those aged 3 to 5 years old composed the largest subgroup (19.4%), and all age subgroups were predominantly male. The incidence of AKI was 11.3%. Neurological disorders (53%) and infection (44%) were the most common underlying disorder and cause of rhabdomyolysis, respectively. The median age at diagnosis in the AKI subgroup was older than that in the non-AKI subgroup (12.2 years vs. 8.0 years). There were no significant differences in body mass index, myalgia, dark-colored urine, or the number of causal factors between the two AKI-status subgroups. The multivariate logistic regression model indicated that the following factors were independently associated with AKI: multiorgan failure, presence of an underlying disorder, strong positive urine occult blood, increased aspartate aminotransferase and uric acid levels, and reduced calcium levels. @*Conclusions@#Our study revealed characteristic clinical and laboratory features of rhabdomyolysis in a Korean pediatric population and highlighted the risk factors for AKI in these cases. Our findings will contribute to a greater understanding of pediatric rhabdomyolysis and may enable early intervention against rhabdomyolysis-induced AKI.

Risk Factor Analysis for Secondary Malignancy in Dexrazoxane-Treated Pediatric Cancer Patients / Journal of the Korean Cancer Association, 대한암학회지

PURPOSE: Dexrazoxane has been used as an effective cardioprotector against anthracycline cardiotoxicity. This study intended to analyze cardioprotective efficacy and secondary malignancy development, and elucidate risk factors for secondary malignancies in dexrazoxane-treated pediatric patients. MATERIALS AND METHODS: Data was collected from 15 hospitals in Korea. Patients who received any anthracyclines, and completed treatment without stem cell transplantation were included. For efficacy evaluation, the incidence of cardiac events and cardiac event-free survival rates were compared. Data about risk factors of secondary malignancies were collected. RESULTS: Data of total 1,453 cases were analyzed; dexrazoxane with every anthracyclines group (D group, 1,035 patients) and no dexrazoxane group (non-D group, 418 patients). Incidence of the reported cardiac events was not statistically different between two groups; however, the cardiac event-free survival rate of patients with more than 400 mg/m2 of anthracyclines was significantly higher in D group (91.2% vs. 80.1%, p=0.04). The 6-year cumulative incidence of secondary malignancy was not different between both groups after considering follow-up duration difference (non-D, 0.52%±0.37%; D, 0.60%±0.28%; p=0.55). The most influential risk factor for secondary malignancy was the duration of anthracycline administration according to multivariate analysis. CONCLUSION: Dexrazoxane had an efficacy in lowering cardiac event-free survival rates in patients with higher cumulative anthracyclines. As a result of multivariate analysis for assessing risk factors of secondary malignancy, the occurrence of secondary malignancy was not related to dexrazoxane administration.

A Case of Diffuse Neonatal Hemangiomatosis Successfully Treated with Propranolol / 임상소아혈액종양

We present a case of a 41-day-old girl with diffuse hemangiomatosis on her chin, right thigh, occipital area, and liver. She also had hepatomegaly, heart failure, and hypothyroidism. Hemangiomatosis did not respond to 2 weeks of oral administration of prednisolone, but was successfully treated with propranolol. After 2 years of propranolol treatment, the lesions of hemangiomatosis nearly disappeared. Hypothyroidism was controlled by levothyroxine replacement. As hemangiomatosis regressed, thyroid function was normalized. Propranolol may have adverse effects including hypotension, hypoglycemia, bronchoconstriction, and restlessness, but she did not experience such adverse effects. Propranolol could be the first choice for diffuse neonatal hemangiomatosis (DNH). Our case also suggests that thyroid function test is needed in patient with DNH.

A Case of Diffuse Neonatal Hemangiomatosis Successfully Treated with Propranolol / 임상소아혈액종양

We present a case of a 41-day-old girl with diffuse hemangiomatosis on her chin, right thigh, occipital area, and liver. She also had hepatomegaly, heart failure, and hypothyroidism. Hemangiomatosis did not respond to 2 weeks of oral administration of prednisolone, but was successfully treated with propranolol. After 2 years of propranolol treatment, the lesions of hemangiomatosis nearly disappeared. Hypothyroidism was controlled by levothyroxine replacement. As hemangiomatosis regressed, thyroid function was normalized. Propranolol may have adverse effects including hypotension, hypoglycemia, bronchoconstriction, and restlessness, but she did not experience such adverse effects. Propranolol could be the first choice for diffuse neonatal hemangiomatosis (DNH). Our case also suggests that thyroid function test is needed in patient with DNH.

Overview of CNS Gliomas in Childhood / 임상소아혈액종양

Brain tumor is the second most common tumor in childhood. The overall survival (OS) has improved significantly during the past 30 years due to developments in surgical technique, radiotherapy, chemotherapy, and high-dose chemotherapy and autologous peripheral stem cell rescue. At present, the 5 year OS of pediatric brain tumor is about 75% which, however, is still lower than that of other malignancies in childhood. Various clinical studies using radiotherapy combined with chemotherapy, multidrug chemotherapy, and monoclonal antibody have been conducted in order to cure patients with brain tumor. The Korean Society of Pediatric Neuro-Oncology is trying to develop standard therapy for the various types of pediatric brain tumor. Of special note, the standard treatment for pediatric glioma has not yet been established. Glioma is the most common histologic type of brain tumor in children. They are classified into 3 groups based on the WHO grade and location of tumor; low-grade glioma (LGG), high-grade glioma (HGG), and brainstem glioma (BSG). The prognosis of LGG is usually good if a complete surgical resection is feasible. Incomplete resected or progressive LGG are troublesome disease for pediatric oncologists. The survival rate of HGG and BSG is very low in spite of combined treatments with surgery, radiation, and chemotherapy. The aim of this review is to provide an overview of treatments for pediatric gliomas.

Overview of CNS Gliomas in Childhood / 임상소아혈액종양

Brain tumor is the second most common tumor in childhood. The overall survival (OS) has improved significantly during the past 30 years due to developments in surgical technique, radiotherapy, chemotherapy, and high-dose chemotherapy and autologous peripheral stem cell rescue. At present, the 5 year OS of pediatric brain tumor is about 75% which, however, is still lower than that of other malignancies in childhood. Various clinical studies using radiotherapy combined with chemotherapy, multidrug chemotherapy, and monoclonal antibody have been conducted in order to cure patients with brain tumor. The Korean Society of Pediatric Neuro-Oncology is trying to develop standard therapy for the various types of pediatric brain tumor. Of special note, the standard treatment for pediatric glioma has not yet been established. Glioma is the most common histologic type of brain tumor in children. They are classified into 3 groups based on the WHO grade and location of tumor; low-grade glioma (LGG), high-grade glioma (HGG), and brainstem glioma (BSG). The prognosis of LGG is usually good if a complete surgical resection is feasible. Incomplete resected or progressive LGG are troublesome disease for pediatric oncologists. The survival rate of HGG and BSG is very low in spite of combined treatments with surgery, radiation, and chemotherapy. The aim of this review is to provide an overview of treatments for pediatric gliomas.

Early Spontaneous Recanalization of Sigmoid Sinus Thrombosis Following a Closed Head Injury in a Pediatric Patient : A Case Report and Review of Literature

Cerebral venous sinus thrombosis (CVST) following a closed head injury in pediatric patients is a rare condition, and an early spontaneous recanalization of this condition is extremely rare. A 10-year-old boy was admitted with a mild, intermittent headache and nausea five days after a bicycle accident. The brain computed tomography showed an epidural hematoma at the right occipital area with pneumocephalus due to a fracture of the occipital skull bone. The brain magnetic resonance imaging and the magnetic resonance venography demonstrated a flow signal loss from the right sigmoid sinus to the right jugular vein. The diagnosis was sigmoid sinus thrombosis, so close observations were selected as a treatment for the patient because of his gradually improving symptoms; however, he complained of vomiting 14 days the after conservative treatment. The patient was readmitted for a further examination of his symptoms. The laboratory and the gastroenterological examinations were normal. Due to concern regarding the worsening of the sigmoid sinus thrombosis, the brain magnetic resonance venography was rechecked and it revealed the recanalization of the venous flow in the sigmoid sinus and in the jugular vein.

Analysis of Information Security Management Systems at 5 Domestic Hospitals with More than 500 Beds / 대한의료정보학회지

OBJECTIVES: The information security management systems (ISMS) of 5 hospitals with more than 500 beds were evaluated with regards to the level of information security, management, and physical and technical aspects so that we might make recommendations on information security and security countermeasures which meet both international standards and the needs of individual hospitals. METHODS: The ISMS check-list derived from international/domestic standards was distributed to each hospital to complete and the staff of each hospital was interviewed. Information Security Indicator and Information Security Values were used to estimate the present security levels and evaluate the application of each hospital's current system. RESULTS: With regard to the moderate clause of the ISMS, the hospitals were determined to be in compliance. The most vulnerable clause was asset management, in particular, information asset classification guidelines. The clauses of information security incident management and business continuity management were deemed necessary for the establishment of successful ISMS. CONCLUSIONS: The level of current ISMS in the hospitals evaluated was determined to be insufficient. Establishment of adequate ISMS is necessary to ensure patient privacy and the safe use of medical records for various purposes. Implementation of ISMS which meet international standards with a long-term and comprehensive perspective is of prime importance. To reflect the requirements of the varied interests of medical staff, consumers, and institutions, the establishment of political support is essential to create suitable hospital ISMS.

Congenital Zinc Deficiency from Mutations of the SLC39A4 Gene as the Genetic Background of Acrodermatitis Enteropathica

Acrodermatitis enteropathica (AE) is an autosomal recessive disorder with the clinical triad of acral dermatitis, diarrhea and alopecia. AE is known to be caused by mutations of the SLC39A4 gene on the chromosome band 8q24.3, encoding the zinc transporter in human. An 8-month-old Korean boy presented with eczematous changes on the inguinal area and knees and was diagnosed with AE. Blood tests revealed a markedly decreased level of plasma zinc, and his symptoms improved on oral zinc replacement. To confirm the diagnosis of AE from congenital zinc deficiency, direct sequencing analysis of SLC39A4 was performed and revealed that he was compound heterozygous for a known missense mutation (Arg95Cys) and a novel splicing mutation in the donor site of intron 7 (c.1287+2T>C). Family study showed that his parents were heterozygous carriers of the mutations. To the best of our knowledge, this is the first report of genetically confirmed AE in Korea.

An Awareness Survey on the Protection and Use of Personal Health Information Focused on Applicants for Copies of the Medical Record / 대한의료정보학회지

OBJECTIVE: To supplement a previous study on the management of medical records with a view to preparing a system capable of ensuring basic patient rights regarding the protection of confidential medical information. The study objectives are to provide detailed guidelines to regulate the access and protection of medical information by analyzing patients' understanding and views regarding the dissemination of medical records. METHODS: A self-administered questionnaire was administered to 781 patients who visited five University hospitals located in Seoul, Busan, Gyeonggi, Chungnam and Jeonnam from July to September, 2008 and asked for copies of medical records to be issued. Data were analyzed by using the statistical program SPSS 13.0. RESULTS: More than 70% of respondents wanted to access their medical records after confirming the required documents. The highest distribution in the range of persons able to receive copies of medical records without the individual patient's consent or required documents was 'only personally' in each variable. Copies of medical records were issued mostly within 1 hour, while the appropriate time of issuing the copies was within one day. Half of respondents approved of a subscription system that did not require a doctor consultation. The results regarding changes in the ways to request/issue copies of medical records to improve the convenience for applicants differed significantly according to age. CONCLUSION: Considering the sensitivity of personal health information, medical records should only be issued with the patient's consent and by confirming the identity of the designated person with required documents. Furthermore, people should be aware of the importance of protecting personal health information, and medical institutes should inform the requirements for bringing the relevant documents. Medical institutes play an important role in protecting personal records, which necessitates generalized guidelines.

Iron Supplementation in a Girl with Attention-Deficit Hyperactivity Disorder / 대한정신약물학회지

New treatment strategies to attention deficit hyperactivity disorder (ADHD) have been introduced recently. We report and review the effect of oral ferrous sulfate administration in a 14 year old girl with ADHD, who has decreased serum iron, ferritin levels. We administered oral ferrous sulfate 120 mg to her for 8 weeks. There were improvements of scores in Attention Diagnostic System (ADS), Clinical Global Improvement (CGI), Conner's ADHD Rating scale and Dupaul ADHD Rating scale

Iron Supplementation in a Girl with Attention-Deficit Hyperactivity Disorder / 대한정신약물학회지

New treatment strategies to attention deficit hyperactivity disorder (ADHD) have been introduced recently. We report and review the effect of oral ferrous sulfate administration in a 14 year old girl with ADHD, who has decreased serum iron, ferritin levels. We administered oral ferrous sulfate 120 mg to her for 8 weeks. There were improvements of scores in Attention Diagnostic System (ADS), Clinical Global Improvement (CGI), Conner's ADHD Rating scale and Dupaul ADHD Rating scale

Prevalence and Clinical Characteristics of Pertussis in Children, Cheonan, Korea / 소아감염

PURPOSE: A number of countries have experienced an increase in pertussis during the past decade. In particular, there has been an increase in the incidence rate among adolescents and adults. To learn more about the current epidemiology of pertussis, we studied the prevalence and clinical characteristics of pertussis in children in Cheonan, South Korea. METHODS: We collected nasopharyngeal aspirates of 118 patients who were treated for respiratory symptoms at Dankook Univeristy Hospital between March 2008 and September 2009. We performed multiplex PCR for detection of Bordetella pertussis in those aspirates. RESULTS: Of the 118 patients, 10 (8%) were positive by PCR for B. pertussis. Six episodes occurred during the period July to September 2009. Nine of the 10 patients were less than 3 months old. Seven of them had not received DTaP vaccine. The mean duration of coughing before diagnosis was 10.9+/-5.2 days. Ten patients (100%) had paroxysmal cough and 8 (80%) had post-tussive vomiting. Only one patient had fever. One who had complications that include pneumonia, atelectasis and pneumomediastinum developed an absolute increase in leukocyte count (84,400/mm3). There was a statistically significant relation between vaccine being received and development of complications (P=0.033). CONCLUSION: We suspect that there was an epidemic of pertussis between July and September 2009. Further investigation by a pediatric or nationwide surveillance system is needed to monitor the changing epidemiology for pertussis.

Videofluoroscopic Findings in Infants with Aspiration Symptom

OBJECTIVE: To describe the findings of videofluoroscopic study (VFSS) and investigate the usefulness of VFSS as a predictor of aspiration pneumonia in infants with aspiration symptoms. METHOD: Thirty-eight infants with aspiration symptoms were divided into two groups: those who showed aspiration symptom but had no pneumonia (Aspiration group); those with episode of aspiration pneumonia (Pneumonia group). The subjects' medical records and the results of VFSS were reviewed. RESULTS: In aspiration group, oral phase was normal and the abnormalities were found only in pharygeal phase in a small number of subjects. On the other hand, pneumonia groups showed poor lip closure and bolus formation in oral phase. Abnormalities of pharyngeal phase were reduced laryngeal elevation, coating of pharyngeal wall, increased residue of valleculae, and delay of pharyngeal transit time. Penetration and aspiration were more frequently observed (p <0.05). Aspiration pneumonia occurred more frequently among infants who had supraglottic penetration (p <0.05). CONCLUSION: Mild abnormalities were found only in pharyngeal phase in aspiration group, whereas VFSS abnormalities were observed in both oral and pharyngeal phase in infants with pneumonia. Our results suggest that VFSS would be useful in predicting the development of pneumonia in infants with aspiration symptoms.

Intracranial Fibroxanthoma in an Infant: a Case Report

A primary fibroxanthoma of the central nervous system is very rare. We present a case of an infantile fibroxanthoma that arose from the cranial dura mater in a six-month-old girl with US, MRI and PET/CT features that mimicked a meningioma. The tumor appeared as a large, well-circumscribed echogenic mass in the right parieto-occipital area on US. The tumor was seen as isoattenuated to slightly hypoattenuated on pre-contrast CT scan and as hypometabolic on PET/CT. As seen on T2-weighted image, the mass was heterogeneously hyperintense to the gray matter. The mass was isointense on T1-weighted image and homogeneously strongly enhanced on contrast enhanced T1-weighted image.

A Case of Fanconi Anemia Diagnosed by a Chromosome Breakage Test with Skin Fibroblasts

Fanconi anemia is an autosomal recessive disease that's characterized by congenital anomalies, defective hematopoiesis and a high risk of developing acute myeloid leukemia and certain solid tumors. The clinical phenotype is extremely variable; therefore, the diagnosis is frequently delayed until the pancytopenia appears. Chromosomal instability, especially on exposure to an alkylating agent, may be seen in affected patients and it is the basis for a diagnostic test. This cellular phenotype can be demonstrated in cultured T cells, B cells, fibroblasts and fetal cells cultured from both amniotic fluid and chorionic villi. But somatic mosaicism may make the diagnosis of Fanconi anemia difficult because of inconclusive chromosome breakage studies. If the test is negative in lymphocytes and yet the clinical setting is highly suspicious, then the skin fibroblasts must be assessed. Because skin fibroblasts are somatic cells, a definitive test can be performed on primary skin fibroblasts. In this report we describe a case of Fanconi anemia that was diagnosed with the use of cultured skin fibroblasts, and this was despite the normal breakage studies in the peripheral blood.

Primary leiomyosarcoma of the chest wall in a child: a case report / 소아과

Leiomyosarcoma is an uncommon soft tissue sarcoma of mesenchymal cell origin, which shows smooth muscle differentiation. Leiomyosarcoma is seldom found in the pediatric population, and accounts for fewer than 2% of all soft tissue sarcomas. Leiomyosarcoma of the chest wall is extremely rare in children. We report here a case of an 8-year-old boy with a primary leiomyosarcoma that was incidentally found as a rib mass. The patient underwent a complete resection for a suspected osteochondroma diagnosed by a three-dimensional chest computed tomography examination. Pathological findings of the mass revealed intersecting fascicles of spindle cells showing cigar-shaped nuclei, inconspicuous nuclear pleomorphism and occasional mitotic figures in the background of a suspected osteochondroma of the rib. This report documents the first description of a leiomyosarcoma possibly arising in an osteochondroma of the rib in a child.