• Clinical and Experimental Pediatrics
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• v.54 no.4
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• pp.157-162
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• 2011

Purpose: Exaggerated pro-inflammatory reactions during the acute phase of Kawasaki disease (KD) suggest the role of immune dysregulation in the pathogenesis of KD. We investigated the profiles of T regulatory cells and their correlation with the clinical course of KD. Methods: Peripheral blood mononuclear cells were collected from 17 KD patients during acute febrile and subacute afebrile phases. T cells expressing CD4, CD25, and Foxp3 were analyzed using flow cytometry, and the results were correlated with the clinical course of KD. Results: The percentage of circulating $CD4^+CD25^{high}Foxp3^+$ T cells among $CD4^+$ T cells was Significantly higher during the subacute afebrile phase than during the acute febrile phase ($1.10%{\pm}1.22%$ vs. $0.55%{\pm}0.53%$, P=0.049). Although levels of $CD4^+CD25^{low}Foxp3^+$ T cells and $CD4^+CD25^-Foxp3^+$ T cells were only slightly altered, the percentage of $CD4^+CD25^+Foxp3^-$ T cells among $CD4^+$ T cells was significantly lower during the subacute afebrile phase than during the acute febrile phase ($2.96%{\pm}1.95%$ vs. $5.64%{\pm}5.69%$, P=0.036). Consequently, the ratio of $CD25^{high}Foxp3^+$ T cells to $CD25^+Foxp3^-$ T cells was higher during the subacute afebrile phase than during the acute febrile phase ($0.45%{\pm}0.57%$ vs. $0.13%{\pm}0.13%$, P=0.038). Conclusion: Decreased $CD4^+CD25^{high}Foxp3^+$ T cells and/or an imbalanced ratio of $CD4^+CD25^{high}Foxp3^+$ T cells to $CD4^+CD25^+Foxp3^-$ T cells might playa role in KD development. Considering that all KD patients were treated with intravenous immunoglobulin (IVIG), recovery of $CD4^+CD25^{high}Foxp3^+$ T cells during the subacute afebrile phase could be a mechanism of IVIG.

• Parasites, Hosts and Diseases
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• v.52 no.2
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• pp.189-191
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• 2014

Visceral leishmaniasis or kala-azar is an endemic parasitic disease in some parts of the world which is characterized by fever, splenomegaly, and pancytopenia in most of the cases. Herein we report an 11 month-old male infant with diagnosis of kala-azar who presented with pallor, hepatosplenomegaly, failure to gain weight, and no history of fever. Surprisingly, fever started after beginning of meglumine antimoniate treatment in this patient. As far as we are aware of, this is a rare presentation of visceral leishmaniasis. Therefore, clinicians especially in endemic areas are highly recommended to include kala-azar among differential diagnosis of unexplained anemia without fever to prevent misdiagnosis of this potentially fatal, but treatable condition.

• Clinical and Experimental Pediatrics
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• v.57 no.7
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• pp.304-309
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• 2014

Benign convulsion with mild gastroenteritis (CwG) is a type of afebrile seizure that occurs in children. CwG is defined as a convulsion in a previously healthy child with no known central nervous system infection or encephalopathy, accompanying mild diarrhea without fever, electrolyte imbalance, or moderate to severe dehydration. Convulsions in CwG are characterized by multiple brief episodes of generalized or focal seizures. Although the etiology and pathophysiology have yet to be fully explained, many pathogenic mechanisms have been proposed including the possibility of direct invasion of the central nervous system by a gastrointestinal virus such as rotavirus or the possibility of indirect influence by the production and effects of certain mediators. The electroencephalogram findings are benign and long-term antiepileptic treatment is typically not required. Long-term prognosis has been favorable with normal psychomotor development. This review provides a general overview of CwG with the goal of allowing physicians practicing in the field of pediatrics to better recognize this unique entity and, ultimately, to minimize unnecessary evaluation and treatment.

• Advances in pediatric surgery
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• v.5 no.2
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• pp.126-129
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• 1999

A 6 and a half year-old girl developed recurrent cholangitis following hepatic portoenterostomy for biliary atresia. Computed tomogram showed an ovoid cyst ($4.5{\times}4.0$ cm in size) in the left hepatic lobe and another tubular dilatation ($2.0{\times}0.8$ cm in size) in the right hepatic lobe. Percutaneous transhepatic cholangiodrainage(PTCD) and cystogram showed an ovoid cyst in the left hepatic lobe (Tsuchida type A), measuring $6.6{\times}5.0$ cm in size. Following drainage and administration of parenteral antibiotics she became afebrile and anicteric. However she continued to drain 45-150 cc of bile per day via the tube over the next 2 weeks. The patient successfully underwent intrahepatic cystojejunostomy with intraoperative ultrasonographic guidance. This case illustrates relapsing cholangitis caused by Tsuchida type A intrahepatic cyst, successfully managed with PTCD followed by an internal drainage procedure.

• Clinical and Experimental Pediatrics
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• v.59 no.sup1
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• pp.157-160
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• 2016

Coexistence of paroxysmal kinesigenic dyskinesia (PKD) with benign infantile convulsion (BIC) and centrotemporal spikes (CTS) is very rare. A 10-year-old girl presented with a 3-year history of frequent attacks of staggering while laughing and of suddenly collapsing while walking. Interictal electroencephalogram (EEG) revealed bilateral CTS, but no changes in EEG were observed during movement. The patient's medical history showed afebrile seizures 6 months after birth, while the family history showed that the patient's mother and relatives on the mother's side had similar dyskinesia. Genetic testing demonstrated that the patient had a heterozygous mutation, c.649_650insC, in the PRRT2 gene. To our knowledge, this constitutes only the second report of a patient with PKD, BIC, CTS, and a PRRT2 mutation.

• Clinical and Experimental Pediatrics
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• v.48 no.7
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• pp.753-759
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• 2005

Purpose : This study was performed to characterize clinical features of benign convulsions with gastroenteritis(CwG) in infants. Methods : We reviewed clinical features of 67 episodes in 64 patients with afebrile seizure accompanied gastroenteritis admitted to Dept. of Pediatrics Bundang CHA hospital from January 2001 to June 2004. Patients with meningitis, encephalitis/encephalopathy or apparent history of epilepsy were excluded. Results : There were 32 boys and 35 girls. The age of onset ranged from 1 to 42 months($18.5{\pm}6.1$ months). The number of children admitted to the hospital with acute gastroenteritis was 2,887 in the same period. The percentage of patients with CwG was 2.3. Seizure type was exclusively generalized tonic or tonic-clonic seizure. The average number of seizures during a single episode was 3.1 (range, 1-13). Two or more seizures occurred in 53(79.1%) of the 67 episodes. Antiepileptic drugs were administered for 42 episodes. Seizure did not cease after the administration of one kind of antiepileptic drug in 23 episodes(54.7%). The seizures were rather refractory to initial antiepileptic treatment. There were no abnormalities in serum biochemistry test including glucose and electrolytes. Cerebrospinal fluid was normal in all 54 episodes. Stool cultures were negative in 49 episodes. Rotavirus was positive in stools in 51(82.3%) of 62 episodes. Norovirus was positive in stools in 2 episodes and astrovirus in 1 of 18 episodes. CT and/or MRI were performed in 15 cases and demonstrated no neuroradiologic abnormalities. Of 73 Interictal EEG, initial 24 cases showed occasional spike or sharp wave discharges from the mid-line area during stage I-II sleep, which were apparently differentiated from vertex sharp transient or K-complexes. The mean follow-up period was 5.7 months(1-36 months). Three patients experienced a recurrence of CwG, but all patients exhibited normal psychomotor development at the last follow-up. Conclusion : Afebrile infantile convulsions with gastroenteritis are brief generalized seizure in cluster with normal laboratory findings and good prognosis. Therefore CwG is likely to be categorized as situation-related seizure of special syndrome. Recognition of this entity should lead to assurance of the parents and long-term anticonvulsant therapy is not usually warranted.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.12 no.2
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• pp.183-193
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• 2009

Purpose: Rotaviruses, noroviruses, astroviruses, and enteric adenoviruses cause acute gastroenteritis (AGE) in children. Some children with AGE have afebrile convulsions associated with viral gastroenteritis. The purpose of this study was to detect and genotype viruses from children with AGE or benign infantile seizures associated with mild gastroenteritis (BIS-MG). Methods: Between August 2004 and June 2005, 311 children with AGE were included. Four viral agents, including rotavirus, norovirus, astrovirus, and adenovirus, were analyzed from stool specimens of each patient using the latex agglutination method, enzyme immunoassay, and reverse transcriptase polymerase chain reaction. Genotyping of each virus was performed in 217 of the 311 children. Results: Among 217 children (male, 121; female, 96; mean age, 20.6${\pm}$15.4 months), rotavirus was detected in 109 (50.2%), norovirus in 28 (12.9%), adenovirus in 13 (6.0%), and astrovirus in 2 children (0.9%). Genotyping of rotavirus revealed positive results in 97 children; P[8]G3 in 36, P[4]G2 in 21, P[6]G4 in 10, P[4]G4 in 9, P[8]G9 in 6, P[8]G1 in 6, P[4]G3 in 4, P[4]G9 in 3, and P[6]G2 in 2. Genotyping of norovirus showed GII-4 in 27 of 28 children and GII-6 in 1 child. Sixteen children were diagnosed with BIS-MG. Rotavirus was detected in 13 of 16 children with BIS-MG, and norovirus in 2 children. Genotyping of rotavirus detected in children with BIS-MG revealed P[8]G3 in 6 children, P[4]G2 in 2 children, and P[4]G9 in 1 child. Conclusion: Analysis of viruses from stool specimens indicates that both rotavirus and norovirus are the main viruses related to BIS-MG in children.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.5 no.1
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• pp.1-8
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• 2005

Glutaric acidemia (GA) type II is a very rare inherited disorder that have no accruate figure on its icidende. People with Glutaric acidemia type II have an enzyme that does not work properly. Two specific enzymes are associated with Glutaric acidemia type II:1. Electron transfer flavoprotein (ETF), 2. ETF-ubiquinone oxidoreductase (ETF-QO). Both of these enzymes have similar functions in the body, and children with Glutaric acidemia type II may lack one or the other of these enzymes. They play an important role in breaking down fats and proteins, and help the body to produce energy. GA II clinically manifested as (1) neonatal onset with congenital anomalies (2) neonatal onset without anomalies, and (3) mild and/or later onset. The first two groups are sometimes said to have multiple acyl CoA dehydrogenation deficiency-severe and the third to have multiple acyl CoA dehydrogenation deficiency-mild. The course and age at presentation of later-onset glutaric acidemia type II is extremely variable, therefore it is difficult to diagnosis. We experienced one case of late onset form glutaric acidemia type II with afebrile status epilepticus-like convulsion.

• Clinical and Experimental Pediatrics
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• v.49 no.9
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• pp.987-990
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• 2006

Intravenous immunoglobulin (IVIG) infusion is an effective therapy for acute Kawasaki disease (KD). Nonetheless, approximately 10 percent to 20 percent of patients have persistent or recrudescent fever despite IVIG treatment, leading to a higher risk for coronary artery aneurysms (CAA). This unresponsiveness may pose a challenge to the clinicians. Tumor necrosis $factor-{\alpha}$ levels are elevated in the acute phase of the disease, especially in patients who develop CAA. We report a 10-month-old male with KD who failed to respond to multiple doses of IVIG and methylprednisolone and who then was treated with infliximab (5 mg/kg single dose). After infliximab treatment, he became afebrile with normalization of inflammatory markers and no further progression of CAA.

• Clinical and Experimental Pediatrics
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• v.56 no.12
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• pp.534-539
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• 2013

Purpose: Electroencephalography (EEG) is frequently ordered for patients with febrile seizures despite its unclear diagnostic value. We evaluated the prevalence of abnormal EEGs, the association between clinical findings and abnormal EEGs, and the predictive value of EEG for the recurrence of febrile seizures Methods: Data were collected on 230 children who were treated for febrile seizures at Kyung Hee University Medical Center from 2005 to 2009. EEGs were recorded after 1-2 days of hospitalization when children became afebrile. EEG patterns were categorized as normal, epileptiform, or nonspecific relative to abnormalities. The patients' medical records were reviewed, and telephone interviews with the families of the children were conducted to inquire about seizure recurrence. The relationships between clinical variables, including seizure recurrence, and EEG abnormalities were evaluated. Results: Of the 131 children included, 103 had simple and 28 had complex febrile seizures. EEG abnormalities were found in 41 children (31%). EEG abnormalities were more common in children with complex than simple febrile seizures (43% vs. 28%), but the difference was not statistically significant. Logistical regression analysis showed that having multiple seizures in a 24-hour period was significantly predictive of abnormal EEG (odds ratio, 2.98; 95% confidence interval, 1.0 to 88; P =0.048). The frequency of recurrence did not differ significantly in the normal (31%) and abnormal (23%) EEG groups. Conclusion: Multiple seizures within 24 hours were predictive of abnormal EEG in children with febrile seizures. Abnormal EEG was not predictive of febrile seizure recurrence.