• Journal of Korean Neurosurgical Society
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• v.29 no.12
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• pp.1668-1672
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• 2000

Nontraumatic, incracranial giant aneurysm has rarely been reported as the cause of the spontaneous subarachnoid hemorrhage in childhood. Multiple, dissecting giant aneurysms on the left middle cerebral artery with sudden onset of headache in a 14-year-old girl were successfully clipped and followed by complete relief of symptoms. The rarity and characteristics of such lesion in childhood and its successful surgical treatment are discussed briefly.

• Asian Pacific Journal of Cancer Prevention
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• v.15 no.22
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• pp.9707-9711
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• 2014

Background: Acute lymphoblastic leukemia (ALL) is a complex disease caused by interactions between hazardous exogenous or/and endogenous agents and many mild effect inherited susceptibility mutations. Some of them are known, but their functional roles still requireinvestigation. Age is a recognized risk factor; children with disease onset after the age of ten have worse prognosis, presumably also triggered by inherited factors. Materials and Methods: The MDR1 gene polymorphisms rs1045642, rs2032582 and MTHFR gene polymorphisms rs1801131 and rs1801133 were genotyped in 68 ALL patients in remission and 102 age and gender matched controls; parental DNA samples were also available for 42 probands. Results: No case control association was found between analyzed polymorphisms and a risk of childhood ALL development. Linkage disequilibrium was not observed in a family-based association study either. Only marginal association was observed between genetic marker rs2032582A and later disease onset (p=0.04). Conclusions: Our data suggest that late age of ALL onset could be triggered by mild effect common alleles.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.14 no.1
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• pp.1-25
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• 2011

Inflammatory bowel disease (IBD) develops during childhood or adolescence in approximately 25% of patients with IBD. Recent studies on pediatric IBD have revealed that early-onset IBD has distinct phenotype differences compared to adult onset IBD. Pediatric early-onset IBD differs in many aspects including disease type, location of the lesions, disease behavior, gender preponderance and genetically attributable risks. This review examines the currently published data on the clinical, epidemiological and genetic differences between early-onset and adult-onset IBD. And finally, therapeutic considerations in the management of pediatric-onset IBD are also discussed.

• Clinical and Experimental Pediatrics
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• v.53 no.4
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• pp.477-480
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• 2010

Advances in research and medical and supportive care have contributed to a growing population of adults formerly treated for childhood cancer. History of cancer and its therapy can have significant life-long health implications. Late effects of cancer therapy can be insidious on onset, occur outside the pediatric age, and contribute to premature morbidity and mortality. In this review, I have focused on the key long-term effects of pediatric cancer therapy, particularly on the metabolic syndrome, including cardiopulmonary complications, infertility, and secondary neoplasm.

• Childhood Kidney Diseases
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• v.10 no.2
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• pp.132-141
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• 2006

Purpose : This study was aimed to determine the predictive risk factors for the treatment response and relapse rate in children diagnosed with idiopathic nephrotic syndrome. Methods : We analyzed the medical records of children who were diagnosed and treated for childhood idiopathic nephrotic syndrome from November 1991 to May 2005. Variables selected in this study were age at onset, sex, laboratory data, concomitant bacterial infections, days to remission, and interval to first relapse. Results : There were 46 males and 11 females, giving a male:female ratio of 4.2:1. The age($mean{\pm}SD$) of patients was $5.8{\pm}4.1$ years old. Of all patients who were initially given corticosteroids, complete remission(CR) was observed in 54(94.7%). Of the 54 patients who showed CR with initial treatment, 40(70.2%) showed CR within 2 weeks and 14(24.6%) showed CR after 2 weeks. The levels of serum IgG were lower in the latter group who showed CR after 2 weeks(P=0.036). Of the 54 patients who showed CR with initial treatment, 47(82.5%) relapsed. Of these patients, 35.1% were frequent relapsers and 43.9% were infrequent relapsers. There was no significant correlation between the frequency of relapse and the following variables : sex, days to remission, and laboratory data. However, age at onset and interval to first relapse had a negative correlation with the frequency of relapse(Pearson's coefficient=-0.337, -0.433, P<0.012, P<0.01). Conclusion : The age at onset and the interval to first relapse were found to be predictive clinical parameters for the relapse rate, while the levels of serum IgG at initial presentation were a predictive laboratory factor for treatment response in childhood idiopathic nephrotic syndrome.

• Clinical and Experimental Pediatrics
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• v.64 no.6
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• pp.269-279
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• 2021

Clustered regularly interspaced short palindromic repeats and CRISPR-associated protein 9 (CRISPR-Cas9) is an ancient prokaryotic defense system that precisely cuts foreign genomic DNA under the control of a small number of guide RNAs. The CRISPR-Cas9 system facilitates efficient double-stranded DNA cleavage that has been recently adopted for genome editing to create or correct inherited genetic mutations causing disease. Congenital heart disease (CHD) is generally caused by genetic mutations such as base substitutions, deletions, and insertions, which result in diverse developmental defects and remains a leading cause of birth defects. Pediatric CHD patients exhibit a spectrum of cardiac abnormalities such as septal defects, valvular defects, and abnormal chamber development. CHD onset occurs during the prenatal period and often results in early lethality during childhood. Because CRISPR-Cas9-based genome editing technology has gained considerable attention for its potential to prevent and treat diseases, we will review the CRISPR-Cas9 system as a genome editing tool and focus on its therapeutic application for CHD.

• Korean Journal of Family Medicine
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• v.39 no.6
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• pp.375-378
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• 2018

Benign acute childhood myositis (BACM) is a rare transient condition that occurs in children during the early convalescent phase of a viral upper respiratory infection. BACM is self-limiting and characterized by sudden-onset bilateral calf pain that leads to difficulty in walking. We report a case of a 5-year-old boy with BACM who presented with acute-onset bilateral calf pain after a resolved episode of viral pharyngitis and subsequently refused to walk. With conservative treatment, the patient recovered completely after approximately 1 week. Although perplexing and challenging for clinicians unfamiliar with BACM, awareness of this rare clinical condition is essential to preventing unnecessary investigations and reassuring the patient and parents of its excellent prognosis.

• Clinical and Experimental Pediatrics
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• v.50 no.8
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• pp.726-731
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• 2007

This review describes the wide spectrum of paroxysmal events during sleep in infancy and childhood. The differential diagnosis between sleep-related non-epileptic paroxysmal events and epileptic seizures is difficult in special occasions. The nocturnal frontal lobe seizure and of the more common nonepileptic paroxysmal events during sleep are described. The main differentiating features characterizing parasomnias are: onset in early childhood, rare episodes of long duration, relatively lower frequency per night, absence of stereotypy, gradual disappearance of older age. Video-polysomnography is the gold standard to diagnosing and differentiating parasomnias from nocturnal frontal lobe seizures.

• Clinical and Experimental Pediatrics
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• v.50 no.8
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• pp.718-725
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• 2007

Sleep disorders are very common among pediatric patients. Its prevalence is between 10% and 45% in preschool- and school-aged children. However parents commonly do not concern about their children's sleeping habits and for many pediatricians, there is not part of the routine office visit about a childs sleep. Sleep disorders were classified by International Classification of Sleep Disorder (ICSD) as dyssomnias, parasomnias, sleep disorders associated with mental, neurologic, or other medical disorders, and proposed sleep disorders. There are lots of differences in the causes, manifestations, and managements of sleep disorders between children and adult. The sleep disorders in childhood may manifest themselves as bedtime resistance, refusal to go to bed at a parentally described time, sleep-onset delay, inability to fall asleep within a reasonable time, prolonged nighttime awakening, and inability to return to sleep without assistance after waking during the night, and so have wide-ranging influences on children's behavior, mood, school performance, and family life. It's very important for pediatrician to concern about the sleep disturbances in childhood and so the problems of sleep in children should be early detected and managed.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.13 no.1
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• pp.20-29
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• 2013

Inborn error of metabolism usually presents with a constellation of clinical pictures involving multiorgan systems. Because of its rarity and clinical diversity, it is difficult to make diagnosis accurately and efficiently. Many inborn error of metabolism shows predominantly hepatic symptoms and signs. The onset of symptoms is also varying depending the disease. The onset might be even prenatal, either neonatal or infantile, and late childhood. The major manifestation patterns are jaundice or cholestasis, hepatomegaly with or without splenomegaly, hypoglycemia and acute or chronic hepatocellular dysfunction. Based on pronounced hepatic symptoms and onset of symptoms, differential diagnosis can be more easily made with subsequent further laboratory investigation. In this review paper, major inborn error of metabolism with hepatic symptoms are described from the perspective of mode of clinical presentations.