• Journal of Chest Surgery
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• v.31 no.11
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• pp.1102-1105
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• 1998

Chronic granulomatous disease in childhood is a rare inheritable disorder of phagocytic cells in which defective production of the reactive intermediates of oxygen predisposes the patient to severe recuring pyogenic infections. The lung is the most common site of infection and pulmonary disease is the primary cause of death for greater than 50% of children with chronic granulomatous disease. Although the role of surgery in management of this disease remains undefined, rapid diagnosis of the underlying pulmonary problem is crucial to determine the most appropriate antimicrobial therapy and surgical techniques such as lobectomy of involved areas lead to more rapid recovery and thus allow the antibiotics to be more efficacious in these cases. We have treated a one month old male baby who had the chronic granulomatous disease with pulmonary infection. Wide surgical resection of the affected lobe and use of antibiotics and antifungals were carried out with good clinical results. He was well after the operation.

• Asian Pacific Journal of Cancer Prevention
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• v.15 no.6
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• pp.2847-2850
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• 2014

Background: Retinoblastoma is a rare malignant intraocular neoplasm. About 90% of cases feature a germline mutation in the RB1 gene and these will develop retinoblastoma during their early childhood. An association between mutations in germline cells and aging has been demonstrated. This suggests a higher incidence of childhood cancer including retinoblastoma among children of older parents. Materials and Methods: In the present study we aimed to determine the association of paternal and maternal age with an increased risk of retinoblastoma in a case-control study in Iranian population. The study was carried out on 240 persons who were born during 1984-2012 in Mahak and Mofid hospitals in Tehran, Iran. The statistical analysis included studying the mean age of parents and in order to know whether parental age of patients is different from parental age of control group, (t-test) compare averages test is used perfectly. By binary logistic regression, odds ratios (ORs) and 95% confidence intervals (CIs) were calculated. Results: The results of statistical analysis including the study of mean parental age by the use of (t-test) compare averages test showed a significant difference between parental ages of patients and controls. Logistic regression showed that coefficients were significant for maternal but not paternal age. Conclusions: Our findings indicate that advanced maternal age can increase the risk of retinoblastoma in offspring, but the paternal age has no significant effect.

• Advances in pediatric surgery
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• v.7 no.1
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• pp.31-36
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• 2001

Mesenchymal hamartoma of the liver is a rare benign tumor, usually presenting in early childhood, Five children with mesenchymal hamartoma of the liver pathologically verified at Seoul National University Children's Hospital between 1978 and 2000 were analyzed retrospectively. There were two girls and three boys, and their mean age at the operation was 16.0months (range, 4-32 months). Three patients presented with abdominal distension. A patient was detected incidentally, and another was detected by prenatal ultrasongraphic examination. Tumor size ranged from $10{\times}8.5cm$ to $34{\times}29cm$. Three tumors were located in the right lobe and two in the left lobe. Four cases underwent complete surgical resection, and the other one underwent incomplete surgical resection and marsupialization. Recurrence or malignant transformation was not noted. Five patients survived without evidence of disease for 35, 36, 38, 142 and 228 months. In conclusion, although mesenchymal hamartoma of the liver is benign lesion. it may be confused. and mixed with embryonal sarcoma. A recent report showed recurrence or malignant transformation after partial excision of the tumor. Therefore. complete excision of the tumor with surrounding normal liver tissue is recommended.

• Journal of Genetic Medicine
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• v.1 no.1
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• pp.33-37
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• 1997

Spinal muscular atrophy (SMA) is the second most common fatal disease of childhood with autosomal dominant mode of inheritance, and in its less severe form the third most common neuromuscular disease of childhood after Duchenne muscular dystrophy. The genetic defect was found to be on the long arm of chromosome 5 (5q11.2-q13.3) where many genes and microsatellite markers were missing. One of the most important genes is the Survival Motor Neuron (SMN) gene which is homozygously missing in 90% of SMA patients. Another important gene, the Neuronal Apoptosis Inhibitory Protein (NAIP) gene was found to be defective in 67% of SMA type I patients. Studies so far suggest SMA occurs when the genes on the long arm of chromosome 5 are mutated or deleted. Recently our hospital encountered 2 SMA patients of type I and II respectively. These patients both had homozygously defective SMN genes but intact NAIP genes. We are reporting these cases with bibliographic review and discussion. Korean SMA patients presumably have defects in SMN genes similar to those found in European patients, although the significance of NAIP genes remains to be established. SMN gene defects can be easily diagnosed using PCR and restriction enzymes, and this method could be applied towards convenient prenatal diagnosis and towards screening for family members at risk.

• Asian Pacific Journal of Cancer Prevention
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• v.16 no.13
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• pp.5205-5210
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• 2015

Background: Acute leukaemia is characterized by fast growth of abnormal clones of haemopoietic precursor cells inside bone marrow leading to undue accumulation in the bone marrow. Acute lymphoblastic leukemia (ALL) is the most common form of childhood cancer. Materials and Methods: The study concerned 50 children diagnosed with ALL (mean age, $8.55{\pm}2.54$) compared to 40 healthy controls (mean age, $8.00{\pm}1.85$). The Hb, serum copper, ceruloplasmin oxidase, advanced oxidation protein products (AOPPs), total antioxidant activity (TAA) and protein were measured in all groups.One proteinous component was isolated by gel filtration chromatography from the precipitate produced by polyethylene glycol. Results: Significantly higher levels of AOPP, copper and decrease in total antioxidant activity were noted in the cases. Statistical analysis also showed a significant increase (p<0.01) in the activity of serum ceruloplasmin oxidase in patients with ALL compared to normal subjects .The maximum velocity (Vmax) and Michaelis constant had values of 104.2 U/L and 11.7 mM, respectively. The ${\Delta}H^*$ values for ceruloplasmin oxidase in ALL patients were positive, confirming the reaction to be endothermic. Conclusions: The results from this study showed a significant increase in AOPP, ceruloplasmine oxidase and decrease in total antioxidant activity .These parameters may play a role in development of DNA damage in childhood patients with acute lymphoblastic leukemia (ALL).The ${\Delta}S^*$ and ${\Delta}G^*$ values were negative, these refer that the reaction of ES formation is spontaneous, but needs energy in a so-called endergonic reaction. Also the negative ${\Delta}S^*$ value of ceruloplasmin oxidase indicates that the complex [$ES^*$] is further modulated through increasing structure arrangement.

• Asian Pacific Journal of Cancer Prevention
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• v.15 no.20
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• pp.8855-8859
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• 2014

Purpose: To investigate the impact of hyperglycemia during inductive treatment on the prognosis of acute lymphocytic leukemia (ALL) in children. Materials and Methods: Clinical data of 159 ALL childhood cases were reviewed. The patients were divided into the hyperglycemia group (fasting $glucose{\geq}126mg/dl$ and/or random blood $glucose{\geq}200mg/dl$) and the euglycemia group according to the blood glucose values. The X2 test was performed to compare the complete remission rates of the two groups, and Kaplan-Meier and log-rank tests were performed to compare the 5-year overall and relapse-free survival. Results: The incidence of hyperglycemia in the $age{\geq}10-year-old$ group was higher than the younger-age group (P=0.009). Values in the interim- and high-risk groups were higher than the standard-risk group (P=0.028), while there was no significant difference between genders (P=0.056). The complete remission rates of the 2 groups demonstrated no significant difference (P=0.134), while the 5-year OS of the hyperglycemia group was lower than in the euglycemia group ($83.8{\pm}6.0%$ vs $94.9{\pm}2.4%$, P=0.014). The 5 -year RFS was significantly lower than the euglycemia group ($62.9{\pm}8.7%$) vs $80.2{\pm}9.1%$, P<0.001). Conclusions: Children with $age{\geq}10year$ old, and in the middle- and high-risk groups appear prone to complicating hyperglycemia during inductive chemotherapy, associated with lower 5-year OS and RFS.

• Asian Pacific Journal of Cancer Prevention
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• v.14 no.2
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• pp.1127-1130
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• 2013

Background: The standard national protocol for treatment of acute lymphoblastic leukemia (ALL) in children was implemented in 2006. A systematic evaluation of the treatment outcome is needed. This study examined the relapse-free survival among childhood ALL cases treated with this protocol and related factors. Materials and Methods: A descriptive study was conducted in children aged between 0-15 years, newly diagnosed with ALL between March 2006 and March 2011 at Srinagarind Hospital, Department of Pediatrics, Faculty of Medicine, Khon Kaen University. The patients were treated on the basis of stratified risk as per the Thai national protocol. Data were compiled from the hospital records. The Kaplan-Meier method was used to describe relapse-free survival and the Cox proportional hazard model to investigate the associated factors. Results: Of the 103 children recruited, 86 (83.5%) achieved complete remission. The total follow-up time was 3132.5 person-months. Eighteen (20.9%) relapsed. The incidence density was 0.6 per 100 person-months (95%CI: 0.4, 0.9). The respective relapse-free rates at 1, 3 and 5 years were 93.0% (95%CI: 85.1, 96.8), 84.5% (95%CI: 74.0, 90.9) and 64.1% (95%CI: 45.6, 77.8). A factor associated with the relapse-free rate was age under 1 year (HR=6.0; 95%CI: 1.1, 33.8). Conclusions: The rate of being relapse-free in ALL children treated under the Thai national protocol at Srinagarind Hospital was better than with former protocols; however, it is still not as good as in developed countries. Further review of the treatment approach of ALL is needed.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.100-108
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• 2001

Purpose : We investigated the incidence and predisposing factors of pulmonary embolism in minimal change nephrotic syndrome(MCNS). Methods : Lung perfusion scan using 99mTC-MAA were done on 14 patients who were diagnosed to minimal change nephrotic syndrome. Group h: Five patients who had perfusion defects on scan, Group B; Nine patients who had no perfusion defect on scan. Between the two groups, the differences of platelet number, hematocrits, albumin, cholesterol, triglyceride, proteinuria were evaluated. Results : Five patients were found to have perfusion defect consistent with pulmonary embolism($35.7\%$). However, there were minimal or no respiratory symptoms and signs. In our laboratory studies, the mean proteinuria on admissions was $676{\pm}31\;mg/m2/hr$ in the group with pulmonary embolism, and $313{\pm}28\;mg/m2/hr$ in the group without pulmonary embolism. There were more severe proteinuria in group with pulmonary embolism(P<0.05). The mean platelet count at early stage of remission after steroid treatment was $746,600{\pm}280,000/mm3$ in the group with pulmonary embolism, $511,890{\pm}90,000/mm3$ in the group without pulmonary embolism. There were significant difference of platelet count between the two groups(P<0.01). In patients with pulmonary embolism, there were more higher and sustained increasement of platelet count. All cases of pulmonary embolism were treated with dipyridamole(5 mg/kg). In 4 cases the perfusion defects were improved in two weeks, however, one case showed persistent perfusion defect after 1 month. Conclusion : Our study suggested that pulmonry embolism might be one of tile major complications in childhood MCNS The occurrence rate was correlated with severity of proteinuria before treatment and sustained increasement of platelet counts in early remission state after steroid treatment. Therefore, the scintigraphic pulmonary perfusion study is mandatory in childhood MCNS, especially in the high risk patients, such as the patients with severe proteinuria and sustained increasement of platelet count. (J Korean Soc Pediatr Nephrol 2001;5 : 100-8)

• Clinical and Experimental Pediatrics
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• v.45 no.4
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• pp.512-518
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• 2002

Purpose : Cerebral palsies are the most common and severe motor disabilities in childhood. There is currently increased interest in their occurrence and patterns of likely cause for a variety of reasons. Therefore, a retrospective study was carried out to understand the clinical features of cerebral palsy. Methods : A retrospective chart review was conducted of all children with cerebral palsy who were diagnosed at St. Benedict Hospital between March 1999 and March 2001. Results : Cerebral palsy patients were classified into 6 major groups. Of six groups, spastic diplegia is the most common type of cerebral palsy(55.3%). The risk factors of cerebral palsy were placenta previa(1 case), placenta abruption(1 case), cytomegalovirus infection(1 case), prematurity (53 cases), neonatal asphyxia(12 cases), dystocia(2 cases), breech delivery(1 case), multiple birth(5 cases), head trauma(3 cases), meningitis(2 cases) and unknown(26 cases). Among the 59 in the preterm group, 37 patients showed MR or CT images of periventricular leukomalacia. Among the 44 in the term group, 15 patients showed MR or CT images of atrophy. Among 103 patients, 29 patients(28.2%) had a seizure disorder. Conclusion : It is very importent to understand the clinical features and risk factors of cerebral palsy for physicians to diagnose and manage cerebral palsy patient.

• Korean Journal of Head & Neck Oncology
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• v.13 no.1
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• pp.51-57
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• 1997

A mass appearing in the anterior or lateral side of neck often can be a diagnostic challenge. Differential diagnosis of the neck mass covers a broad spectrum of diseases and the proper evaluation and management of a neck mass requires an impressive amount of anatomic and pathologic information. Because improper diagnosis and management may convert a potentially curable malignant metastasis into incurable disease, a differential diagnosis must be considered in all patients who present with a neck mass. Authors reviewed 2,148 cases of neck mass who were diagnosed by surgical resection, biopsy or aspiration during the period between October 1982 to December 1993, excluding those with thyroid and parathyroid disease. The evaluated characteristics were age, sex, site of lesion, and pathologic diagnosis. The results were as follows: Of 2,148 cases of neck mass, the overall ratio of benign to malignant tumor was 3 : 1. In 1,603 cases of benign mass lesion, the most common disease was lymphadenitis(non-specific and tuberculosis) showing 53% incidence, the second was salivary gland tumor(13%), and the third was congenital lesion(12%). The minor problems such as lipoma and sebaceous cyst were 21 %. In the age distribution of benign lesion, tuberculous lymphadenitis showed peak incidence in second decade, non-specific lymphadenitis was main disease of childhood, salivary gland tumor was peak in fourth decade, and most of congenital lesions were diagnosed at the age below 15. In 545 malignant tumors, the most common lesion was metastatic cancer to cervical lymph nodes yielding 71 % incidence(head and neck primary 52%, infraclavicular primary 42%, unknown primary 5%), the second common disease was lymphoma(19%), and the third was salivary gland cancer(9%). In the age incidence of malignant tumor, 60% of them developed in the fifth and sixth decade, head and neck primary was more common in the fifth decade than sixth, however lymphoma showed higher incidence in sixth decade. In the analysis of mass location according to lymph node level grouping(I - V), lymphadenitis developed mostly in level V nodes, the next common occurring site was level IV in tuberculous lymphadenitis and level II in non-specific lymphadenitis. The majority of metastatic cancers were found in level IV and III, and common occurring site of lymphoma was in level II and IV. Pathologic diagnosis of neck masses were made by fine needle aspiration cytology 80 cases, incisional biopsy 533 cases, excisional surgery 1,399 cases, and neck dissection 116 cases. For the proper management of neck mass, a proper diagnostic modality should be selected from imaging techniques, cytology, biopsy or neck dissection, with the consideration of patient's age, history and clinical findings. The scapel biopsy could be used freely in the inflammatory disease or inoperable metastatic cancer, but it should be reserved in the curable metastatic cancer or clinically possible malignancy.