• The Journal of Korea Assosiation for Disability and Oral Health
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• v.14 no.1
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• pp.22-25
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• 2018

Lennox-Gastaut syndrome (LGS) is one of the most severe epileptic encephalopathies of childhood onset. It is characterized by multiple types of generalized seizures, slowness of intellectual growth, and specific electroencephalogram pattern. It is one of the most difficult epilepsy syndromes to treat and is frequently resistant to treatment. In this case report, we report a case of dental treatment under general anesthesia in a child with LGS. A 4-year-6-month old boy with LGS visited Seoul National University Dental Hospital for dental treatment. Dental treatment was planned under general anesthesia because of his medical condition. Treatment was successfully done without any postoperative complications. If a LGS patient requires dental treatment, a treatment plan should be established to take into account the degree of seizure, the medication being used, and the cooperation of the patient. If the degree of seizure is severe and the patient's cooperation is poor, dental treatment under general anesthesia would be advantageous.

• Pediatric Infection and Vaccine
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• v.8 no.1
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• pp.123-128
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• 2001

Clonorchiasis, known as the parasitic fluke in the intrahepatic bile ducts, occurs throughout Korea, Japan, Taiwan, and Vietnam with high frequency. Most of the infections follow the consumptions of raw or undercooked freshwater fish, which explains its rare occurrence to childhood even in those areas mentioned earlier above. The authors have experienced it with an 8 year old boy. Abdominal ultrasonogram revealed multiple low echogenic nodules in the right lobe of liver and lymph nodes proliferation around the porta hepatis area. And abdominal C.T. scan also found multiple low attenuated nodules with delayed enhancement in liver parenchyma with hepatomegaly and multiple conglomerated lymph nodes in porta hepatis. Both abdominal ultrasonogram and C.T. scan didn't show any dilatation of peripheral intrahepatic bile ducts. In repeated stool examination, Clonorchis sinensis eggs were found in feces and the result of the skin test for Clonorchis sinensis was positive. The patient had a history of having eaten raw pond smelt back in Cheongju a month before the symptom onset. On the basis of the patient's history and the fact that Clonorchis eggs were found in feces 3 weeks after eosinophilia and symptoms were noted, the authors estimated that the findings of the patient's abdominal sonogram and C.T. scan were the radiologic findings of the acute phase of clonorchiasis, of which no report had ever been made before. So the authors report a case of clonorchiasis with atypical radiologic findings in an 8 year old boy.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.18 no.1
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• pp.30-34
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• 2018

Niemann Pick type C disease (NPC) is an inherited progressive neurodegenerative disorder, due to defects of intracellular lipid trafficking and storage. Hepatosplenomegaly may prevail, while progressive neurodegenerative symptoms such as cerebellar involvement, dystonia, vertical supranuclear ophthalmoplegia, cataplexy, and eventually seizures starting at juvenile or late infantile period may accompany after normal early development. Here we describe a 3-year-old Korean boy with NPC who presented with splenomegaly at age 3. Liver biopsy showed characteristic foamy cell stained by periodic acid-schiff, and molecular analysis for NPC1 identified the compound heterozygous mutations, novel mutation of c.1631G>A (p.Trp544Ter) and c.2662C>T (p. Pro888Ser) as a known mutation. Filipin was strongly stained with unesterified cellular cholesterol in the patient's skin fibroblasts. The patient has received migulstat since age 3 years and his long-term outcome is needed to be observed.

• Journal of the Korean Academy of Child and Adolescent Psychiatry
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• v.14 no.1
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• pp.3-11
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• 2003

Attention-deficit/Hyperactivity disorder(ADHD) is the most common psychiatric disorder of childhood and among the most prevalent chronic health conditions affecting school-aged children. Children with ADHD experience significant functional problems, such as school difficulties, academic underachievement, troublesome interpersonal relationships with family members and peers, and low self-esteem. The most widely used pharmacological treatments for ADHD are psychostimulants, such as methylphenidate and amphetamine salts. These medications provide clinical efficacy by increasing the availability of catecholamines, primarily dopamine, in the frontal lobe of the brain. immediate-release(IR) formulations of sychostimulants were among the most effective psychotrophic medications in the psychopharmacological treatment. However, there are some limitations of IR formulations：the short half-life and duration of efficacy, which result in the need for multiple daily dosing and the poor compliance. These limitations have led to the development of once-daily, extended-release(ER) formulations of methylphenidate and amphetamine salts. However, these ER formulations may not be as immediately helpful to ADHD children due to delayed onset of action and the acute tolerance which is the failure to sustain the efficacy with the same concentration of drug as the initial stage of medication. OROS-methylphenidate(Concerta$^{\circledR}$) given once a day produces an ascending-pattern plasma drug level generated by the osmotically released, timed drug-delivery system. These new formulations of the psychostimulants have been shown to be a useful alternative to old stimulant medications through the evidence by the clinical trials.

• Archives of Plastic Surgery
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• v.34 no.1
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• pp.77-80
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• 2007

Purpose: Purpura fulminans is a rare but rapidly progressive, serious, often life-threatening disorder in childhood, which is complicated with septic shock or disseminated intravascular coagulopathy during acute infection. It occurs first as acute-onset petechial rash, and spreads rapidly into full thickness skin and soft tissue necrosis. In the past, it had high mortality rate, up to 80%, but recently, survival rate has increased due to early diagnosis, and rapid advancement of critical care and antibiotics. From our experiences of PF management, we would like to review the pathophysiology and suggest the surgical treatment guideline about meningococcal induced purpura fulminans. Methods: Two cases of purpura fulminans over the last 3 years were reviewed retrospectively about reconstructive management. After they were treated resuscitative management initially by the critical intensive care, reconstructive surgery was performed by plastic surgeon as soon as the patients were vitally and mentally stable. Results: There were 6 procedures in case 1, and 3 procedures in case 2. The mean delayed period from admission with sepsis to the first surgical debridement was 24 days and 42 days, respectively. Total hospitalization period was 103 days and 69 days, respectively. All of them were treated with debridement and split thickness skin graft, but delayed debridement was superior to early one in the point of preserving much more tissues. Conclusion: From our experience, we suggest that conservative therapy to the wounds appears to be the best tool in the initial vitally unstable period in order to preserve as much tissues and functions as possible if no active inflammation and compartment syndrome are detective.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.5 no.1
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• pp.62-67
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• 2002

Purpose: The aim of this study was to investigate the age of onset and the prevalence of lactose malabsorption in early childhood in Korea. Methods: We conducted a study of lactose malabsorption by breath hydrogen test in healthy children aged between 25~96 months old. Standard lactose loading (2 g lactose/kg, maximum 40g) test was done in 129 children and cow's milk (10 mL/kg) loading in 126 children followed by breath sampling of 60 and 120 minutes after the loading. An increase above baseline of 20 ppm or more was used as a criteria for positive responses. Results: The prevalence of lactose malabsorption was 7% in 25~36 months old, 19% in 37~48 months old, 35% in 49~60 months old, 55% in 61~72 months old, 82% in 72~84 months old, 80% in 85~96 months old children. Only 1% of the children showed positive result in breath hydrogen test after the cow's milk challenge. Conclusion: The prevalence of lactose malabsorption was increased between 37 months and 60 months of age, reached to adult level of prevalence after 72 months of age. When physiological dose of lactose was used as the challenge, the number of lactose malabsorbers become clinically insignificant.

• Sleep Medicine and Psychophysiology
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• v.15 no.2
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• pp.94-99
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• 2008

The most common cause of obstructive sleep apnea syndrome (OSAS) in childhood is adenotonsillar hypertrophy. Adenotonsillectomy improves the symptoms quite well in most cases. However, some patients could experience the OSAS again after adenotonsillectomy, who might have several risk factors such as incomplete operation, misdiagnosis, combined anatomical malformation, sinusitis or chronic allergic rhinitis, obesity, initial severe OSAS, and early onset OSAS. We report a case of 11-year-old obese boy who presented with snoring for several years. He was obese with body mass index (BMI) of $26.3kg/m^2$ and also found to have fatty liver by ultrasonogram. Initial polysomnography (PSG) showed that he met the criteria of severe OSAS with the apnea-hypopnea index (AHI) of 70.5. He underwent adenotonsillectomy and symptoms improved immediately. Four months later symptoms were relieved with AHI of 0, but 1 year after the adenotonsillectomy he started to complain snoring again and the subsequent PSG results showed that OSAS has relapsed with AHI of 43. Paranasal sinus X-ray and physical examination showed sinusitis and re-growth of adenoid. Obesity was proved not to be a contributing factor because his BMI decreased to normal range ($23.1kg/m^2$) after diet control and regular exercise. Also, liver transaminase was normalized and fatty liver was disappeared on follow-up abdominal ultrasonogram. After treatment of sinusitis, symptoms were relieved with decreased AHI (8.5). This case suggests that simple adenotonsillectomy might not be the end of OSAS treatment in childhood. Patients who had adenotonsillectomy should be followed by subsequent PSG if symptoms recur. It is also important to be aware of risk factors in the recurrent OSAS for the proper intervention according to the cause.

• Childhood Kidney Diseases
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• v.8 no.2
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• pp.176-185
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• 2004

Purpose: Rapidly progressive glomerulonephritis (RPGN) is a clinicopathologic entity characterized by extensive crescent formation and rapid deterioration of renal function within few months. For better understanding of its clinical course and designing better treatment strategies, a clinicopathological study of childhood RPGN was performed. Methods: The clinical manifestations and pathological findings were reviewed retrospectively in 12 children who were diagnosed as having RPGN by clinical manifestations and renal biopsy during a period from 1991 to 2003. Several clinicopathological parameters were analyzed as prognostic factors. Results: Among a total of 12 patients, 4 were male and 8 were female. The median onset age was 11.5 years(range 5.5-14.6 years), and the median period of follow-up was 25 months(range 7 months-6.6 years). According to the pathological classification, 10 patients (83%) were type II RPGN(immune-complex mediated glomerulonephritis), 2 patients were type III RPGN(pauci-immune glomerulonephritis), and none was type I RPGN(anti-glomerular basement membrane nephritis). All patients were treated with oral steroid in various combinations with methylprednisolone pulse therapy(10 patients, 83%), cyclophosphamide(8 patients, 67%), or plasmapheresis(4 patients, 33%). Clinical outcomes of 12 patients were complete remission in 1(8%), end-stage renal disease in 2(17%), chronic renal insufficiency with persistent proteinuria in 2(17%), and normal renal function with persistent proteinuria in 7(58%) at the last follow-up. Poor prognosis is associated with increased serum creatinine level, severe anemia and younger age at the time of diagnosis. Conclusion: Immune-complex mediated glomerulonephritis is the major cause RPGN in children and most cases showed improvement of renal function with aggressive management. For better understanding of this rare disease, a prospective multicenter study should be done.

• Childhood Kidney Diseases
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• v.9 no.2
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• pp.137-142
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• 2005

Purpose : Acute poststreptococcal glomerulonephritis(APSGN) follows infection of group A$\beta$-hemolytic streptococci. The prognosis of APSGN has been reported as favorable. However, several studies have reported that some patients progress to chronic renal failure. In an attempt to clarify this, we analyzed the clinical course of patients with APSGN. Methods : Between January 2000 and December 2004, a total of 48 children who were diagnosed with APSGN according to the presence of hematuria, transient hypocomplementemia and evidence of group A $\beta$-hemolytic streptococcal infection were evaluated. Results : Six(12.5$\%$) patients showed elevation of serum creatinine level but there was no patient with Persistent renal dysfunction. Blood pressure was controlled with ease in all patients and there was no case of persistent hypertension. Renal biopsy was done in 5 patients who showed heavy proteinuria or renal insufficiency and the outcomes showed findings consistent with ordinary APSGN except one with findings of rapidly progressive glomerulonephritis(RPGN). Serum complement levels normalized within 8 weeks(92.9$\%$). Hematuria disappeared within 6 months(79$\%$) and proteinuria within 6 months(100$\%$) from the disease onset. Conclusion : Prolonged renal dysfunction or heavy proteinuria found in five patients(10.4$\%$) led to renal biopsy. All these problems resolved within 6 months. Our data support that the prognosis of childhood APSGN is favorable without any serious sequoia. (J Korean Soc Pediatr Nephrol 2005;9:137-142)

• Tuberculosis and Respiratory Diseases
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• v.42 no.2
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• pp.175-183
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• 1995

Background: The incidence of mycoplasmal pneumonia is predominantly at childhood and early adulthood, but in adults, its incidence is low and its symptoms and physical findings are nonspecific. The definite diagnosis of M. pneumoniae pneumonia can be made by sputum culture, but requires several weeks for positive results, and the early diagnosis must initially be based on the serologic tests and appropriate clinical findings. Thus, we evaluated the clinical aspects of M. pneumoniae pneumonia in the adults patients. Method: Among the admitted patients due to pneumonia, the definite diagnosis is anti-M. pneumoniae antibody titer of $\geq$ 1:40 and a single cold agglutinin titer of $\geq$ 1:64. The presumptive diagnosis is anti-M. pneumoniae antibody titer of $\geq$ 1:40 or a single cold agglutinin titer of $\geq$ 1:64 and the clinical characteristics or chest X-ray findings are compatible with M. pneumoniae pneumonia. We studied the age and sex distribution, seasonal distribution, clinical symptoms, physical findings, serologic test, chest X-ray findings, treatment and its progression. Results: 1) The age distribution was even and the ratio of male to female was 1:1. 2) The monthly distribution was most common in January(16.7%) and the seasonal distribution in autumn and winter(autumn: 30%, winter: 33.3%). 3) The cold agglutinin titers were higher than 1:64 in 12 cases(40%), and reached the peak level around 2 weeks from onset and antimycoplasma antibody titers were higher than 1:160 in 5 cases(16.7%). 4) On the chest X-ray, pulmonary infiltration was noted in 28 cases(93.3%) among 30 cases and right lower lobe involvement was the most common(33.3%) and both lower lobe involvement was noted in 7 cases(23.3%). 5) The mean treatment duration was most common(33.3%) in 1 week to 2 weeks after admission and 26 cases(86.7%) were improved within 4 weeks. 6) On admission, there was fever(${\geq}38.9^{\circ}C$) in 17 cases(56.7%), and the fever subsided in 12 cases(70%) within 3 days after treatment using erythromycin. Conclusion: The mycoplasmal pneumonia in adults shows milder clinical patterns than that in childhood and can be completely recovered without complication by early diagnosis and treatment.