• Childhood Kidney Diseases
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• v.23 no.1
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• pp.43-47
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• 2019

Atypical hemolytic uremic syndrome (aHUS), a rare form of thrombotic microangiopathy, is distinguished from the typical form by the absence of a preceding verotoxin-producing Escherichia coli infection. Notably, aHUS occurs in association with genetic or acquired disorders causing dysregulation of the alternative complement pathway. Patients with aHUS may show the presence of anti-complement factor H (CFH) autoantibodies. This acquired form of aHUS (antiCFH-aHUS) primarily affects children aged 9-13 years. We report a case of a 13-year-old Lao girl with clinical features of aHUS (most likely anti-CFH-aHUS). The initial presentation of the patient met the classical clinical triad of thrombotic microangiopathy (microangiopathic hemolytic anemia, thrombocytopenia, and acute kidney injury) without preceding diarrheal illness. Low serum levels of complement 3 and normal levels of complement 4 indicated abnormal activation of the alternative complement pathway. Plasma infusion and high-dose corticosteroid therapy resulted in improvement of the renal function and hematological profile, although the patient subsequently died of infectious complications. This is the first case report that describes aHUS (possibly anti-CFH-aHUS) in Laos.

• Clinical and Experimental Pediatrics
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• v.62 no.9
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• pp.325-333
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• 2019

Allergic diseases, including allergic rhinitis, asthma, and atopic dermatitis, are common heterogeneous diseases that encompass diverse phenotypes and different pathogeneses. Phenotype studies of allergic diseases can facilitate the identification of risk factors and their underlying pathophysiology, resulting in the application of more effective treatment, selection of better treatment responses, and prediction of prognosis for each phenotype. In the early phase of phenotype studies in allergic diseases, artificial classifications were usually performed based on clinical features, such as triggering factors or the presence of atopy, which can result in the biased classification of phenotypes and limit the characterization of heterogeneous allergic diseases. Subsequent phenotype studies have suggested more diverse phenotypes for each allergic disease using relatively unbiased statistical methods, such as cluster analysis or latent class analysis. The classifications of phenotypes in allergic diseases may overlap or be unstable over time due to their complex interactions with genetic and encountered environmental factors during the illness, which may affect the disease course and pathophysiology. In this review, diverse phenotype classifications of allergic diseases, including atopic dermatitis, asthma, and wheezing in children, allergic rhinitis, and atopy, are described. The review also discusses the applications of the results obtained from phenotype studies performed in other countries to Korean children. Consideration of changes in the characteristics of each phenotype over time in an individual's lifespan is needed in future studies.

• Pediatric Infection and Vaccine
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• v.18 no.1
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• pp.40-47
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• 2011

Purpose : Norovirus infection, a common cause of community-acquired gastroenteritis, can also lead to severe illness in immunocompromised patients. We investigated clinical manifestations of norovirus infection in pediatric cancer patients. Methods : Stool specimens were collected from pediatric patients with gastrointestinal symptoms between November 2008 and September 2009 at Samsung Medical Center, Seoul, Korea. Norovirus infection was identified by reverse-transcription polymerase chain reaction (RT-PCR). A retrospective chart review was performed in pediatric cancer patients who were diagnosed with norovirus infection. Results : Ten patients were diagnosed with norovirus infection by RT-PCR in stool samples. The median age was 0.83 years (range 0.25-5.5 years) and the male to female ratio was 1.5:1 (6 males and 4 females). Underlying diseases were hematologic malignancies (4/10, 40%), neuroblastoma (4/10, 40%), and brain tumors (2/10, 20%). Three patients were infected before hematopoietic cell transplantation (HCT) and four patients after HCT. All patients had diarrhea (10/10, 100%), with a median frequency of diarrhea of 8.5 times/day (range 4-22 times/day). Median virus shedding duration was 72.5 days (range 19-299 days). Four patients with pneumatosis intestinalis were conservatively treated with bowel rest and total parenteral nutrition. One patient with severe diarrhea and bloody stool had concomitant chronic gut graft-versus-host disease (GVHD). Norovirus infection-related mortality was not observed. Conclusion : Norovirus infection can cause significant clinical manifestations with prolonged viral shedding in immunocompromised patients. Norovirus should be considered in pediatric cancer patients with severe gastrointestinal symptoms.

• Pediatric Infection and Vaccine
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• v.15 no.2
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• pp.162-166
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• 2008

Purpose : It has been previously reported that for patients with Mycoplasma pneumoniae pneumonia was previously recognized that overt illness is unusual under the age of three and the peak incidence of illness occurs in school-aged children. However, a higher incidence of this illness in younger children has been recently noted. Thus we investigated the incidence of M. pneumoniae pneumonia. Methods : The study subjects were 414 children who were diagnosed with M. pneumoniae pneumonia from January 2004 to December 2006 at Myong Ji Hospital were enrolled. The diagnostic criteria consisted of an anti-mycoplasma antibody (AMA) titer greater than 1: 320 or a four-fold rise in the titer at follow up. Results : The age distribution was as follows: before 2 years of age: 58 patients (14%), 2-4 years of age 157 patients (37.9%) and 5-15 years of age 199 patients (48.1%). The yearly incidence for the children before 5 years of age was 52 (44%), 49 (44.6%) and 114 (61.3%), respectively. The distribution according to the antibody titer was as follows; 1: 320 in 130 patients, 1:640 in 63 patients and greater than 1:1,280 in 221 patients. The hospital stay according to the antibody titer was not significant according to either age or the AMA titers. Conclusion : M. pneumoniae pneumonia showed a peak incidence in preschool children with a higher prevalence in children under the age of three than was previously recognized. The emergence of M. pneumoniae pneumonia as a cause of community acquired pneumonia in younger children calls for an epidemiologic study to investigate the changes of the pathogens in this age group and to recommend the proper treatment.

• Child Health Nursing Research
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• v.4 no.2
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• pp.274-285
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• 1998

Improvements in therapy have resulted in increasing numbers of children being successfully treated for cancer. However the agrressiveness of therapy & uncertainty about prognosis are associated with many adverse effects, psychological as well as physical for both the child & family. The purpose of this study were to measure the degree of perceived uncertainty, self-efficacy & coping, and then to examine the relationship between the perceived uncertainty, self-efficacy & coping in parents of children cancer patients. The subjects of this study consist of 140 parents with pediatric cancer, registered at pediatric cancer ward & Out Patient Department. Data was collected from July 1st to August 15th 1998. The tools used in this study were Mishel's the Parents' Perception of Uncertainty Scale (28 item, 4 likert scale), Shere's Self-Efficacy Scale (17 item, 5 likert scale) & Folkman & Lazarus Ways of Coping Checklist(34 items, 4 likert scale). Data was analyzed by t-test, Anova, Pearson Correlation coefficient. Results of this study are summerized as follows 1. Parents perceived their uncertainty to be slightly high(Mn 2.41). The degree of perceived uncertainty by the four components were followed as lack of clarity (2.60), unpredictability(2.59), ambiguity(2.51) & lack of information(1.90). The degree of perceived uncertainty of parents with pediatric cancer revealed to be influenced significantly by the family outcome, reliability about health care provider & perceived severity of illness. 2. The range of parents' self-efficacy was measured iron 35 to 85 point, so revealed slightly high. The degree of self-efficacy related to be Influenced signiicantly by the sequency of child birth, family religion & degree of perceived support. 3. The degree of parents' coping was measured slightly high (Mn 2.78). The degree of coping related to be influnced significantly by the sequency of child birth, number of sible & degree of perceived support. 4. parents' uncertainty was related inversely to the parents' self-efficacy(r=-.38, p<.001) & coping(r=-.26, p<.001). And also parents' self-efficacy was positively related to coping(r=.56, p<.001) From the above results, it can be concluded that predicting & controlling parents' uncertainty with children cancer are necessary to improve positive coping strategies. This information may be used as a foundation for developing nursing interventions to decrease perceived uncertainty & to foster self-efficacy & coping for parents with children cancer.

• Child Health Nursing Research
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• v.20 no.2
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• pp.113-122
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• 2014

Purpose: Purposes of this study were to identify the level of parental fever phobia and to investigate the relationship between level of parental concern about fever and related variables. Methods: Participants were 151 parents of children who visited a pediatric outpatient clinic. A selfreported structured questionnaire was used for data collection and data were analyzed using descriptive statistics and ${\chi}^2$-test. Results: Almost half of participants defined a minimum temperature for fever as $37.8^{\circ}C$ and a minimum temperature for high fever as $38.9^{\circ}C$. About 75% of participants identified harmful effects of fever as seizure and brain damage, were 'very worried' about fever, measured their child's temperature every hour or less, provided tepid massage and woke children to give antipyretics during febrile illness. There were significant relationships between level of parental concerns about fever and prior experience of febrile seizures, and/or being parents of a single child. Conclusion: Results indicate that fever phobia is prevalent among parents. Further studies are needed to develop and evaluate childhood fever management educational programs for parents. Considering health care providers as a primary information resource about fever management, health care providers should play a vital role to reduce parental unrealistic concerns about fever.

• Journal of Yeungnam Medical Science
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• v.9 no.1
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• pp.44-53
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• 1992

We compared the efficacy of each modality of treatment group in reducing the frequency of coronary artery abnormalities and changes of clinical courses in children with kawasaki disease in the children of 81 cases who were admitted in pediatric department of Yeungnam University Hospital from September 1985 to August 1990, Group A(37 cases)-aspirin alone, Group B(44 cases)-intravenous gammaglobulin(400mmg/kg/day) for 5 consecutive days, plus aspirin. We studied the frequency of echocardiographic abnormalities, the duration of fever, and changes in the total white blood cell counts, platelet counts, ESR and CRP value at 1, 2 and 3 weeks of the illness and compared the results between the two groups. The results were as follows, 1) There as no significant intergroup difference in age and sex ratio. 2) The duration of the febrile period after the initiation of the therapy was significant shorter in group B($2.5{\pm}1.2$ days) than in group A:($5.2{\pm}3.5$ days)(p<0.01). 3) No significant difference was noticed in the WBC and platelet counts in two groups as measured at admission day, 1 and 2 weeks of the illness. However, at 3weeks of illness significant difference was noted. 4) The CRP values measured at 1, 2 and 3 weeks after treatment were significantly lower in group B($2.42{\pm}1.8$, $2.00{\pm}1.2$, $1.16{\pm}1.0$) than in group A($7.22{\pm}5.3$, $5.25{\pm}3.9$, $1.85{\pm}1.2$) respectively(p<0.01). 5) In 2D- Echocardiogram, coronary artery dilataton was more frequent in Group A than in Group B at 6month of illness(p<0.01). In conclusion, intravenous gammaglobulin therapy was effective in the shortening of the duration of fever and in the antiinflammatory action and somewhat effective in prevention of coronary artery aneurysm.

• Clinical and Experimental Pediatrics
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• v.46 no.10
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• pp.1019-1023
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• 2003

Purpose : To determine the differences in clinical characteristics, blood chemistry and coronary artery complications between patients with Kawasaki disease who received intravenous immunoglobulin (IVIG) within the fourth day of illness and after the fifth day of illness. Methods : A retrospective chart review was conducted of all children with Kawasaki disease who were admitted to Ewha Mokdong Hospital between January 2001 and June 2002. The early treatment group received IVIG within the fourth day of illness(n=34) and the control group received IVIG after the fifth day of illness(n=53). Clinical manifestations, fever duration, hospitalization days, CBC, blood chemistry and coronary artery complications were compared between two groups. Results : No demographic differences were noted between the two groups(P>0.05). Total duration of fever was significantly shorter in the early treatment group than the control group($4.8{\pm}2.5days$ vs $7.4{\pm}3.0days$, P<0.05), but there were no differences in fever duration after IVIG treatment and hospitalization days between two groups(P>0.05). No significant differences were noted in the level of hemoglobin, WBC, ESR, CRP, AST, ALT and albumin between two groups(P>0.05). No significant differences in the incidence of IVIG retreatment were noted between the two groups(11.8% vs 5.7%, P>0.05). No significant differences in the incidence of coronary artery complications were noted between the two groups(11.7% vs 18.9%, P>0.05). No significant differences in the recurrence rate were noted between the two groups(3% vs 2%, P>0.05). Conclusion : Early IVIG treatment in patients with Kawasaki disease reduces the total fever duration. Coronary artery complications were not increased in patients with early IVIG treatment.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.17 no.1
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• pp.1-10
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• 2017

Purpose: This study aimed to investigate the perception and emotional experiences in rare and intractable diseases for caregivers of pediatric patients with mitochondrial diseases in order to provide therapeutic interventions for patients, caregivers, and families. Methods: A total of 83 caregivers of pediatric patients with mitochondrial diseases were recruited from the pediatric mitochondrial disease clinics of the Gangnam Severance Hospital in South Korea. Participants completed the survey about their perception of mitochondrial disease and emotional experiences after the diagnosis, and these clinical data were analyzed accordingly. Results: Surveys from a total of 83 caregivers of patients were analyzed, and the patients' age ranged from 6 to 12 years (33%), followed by ages 1 to 6 years (30%). Children with mitochondrial diseases were between 0 and 0.5 years of age at the time of first symptom onset (43%), and the duration of illness lasted more than 10 years in most cases (42%). Prior to diagnosis of mitochondrial diseases, the amount of awareness the caregivers had was 'Not at all' for both rare and intractable diseases and mitochondrial diseases in 44 cases and 68 cases, respectively. For the caregivers' emotional experiences, the most common initial responses were 'Discouraged/despair', 'Helpless/lethargic', and 'Disconcerted'. 'Anxious', 'Committed to treatment', and 'Responsibility as family members' were the most common emotional responses from the caregivers, followed by 'Disconcerted' and 'Helpless/lethargic'. Conclusion: It is important to consider the level of perception and emotional experiences of caregivers and patients with rare and intractable mitochondrial diseases for planning treatment programs.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.9 no.1
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• pp.39-47
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• 2006

Purpose: We proposed a new classification of pediatric intussusception based on clinical and radiologic findings. Methods: Data from 88 consecutive patients with intussusception were reviewed. We retrospectively analyzed six factors; patient age, sites of intussusception, symptoms, therapeutic methods, existence of enlarged mesenteric lymph nodes, and ultrasonographic (US) findings from clinical records. Results: 1) There was one neonatal case (1.1%), the others (98.9%) were infants and children. 2) These 87 infant and child cases consisted of 14 cases (16.1%) of small bowel intussusception (SBI) and 73 cases (83.9%) of ileo-colic intussusception (ICI). Of the 14 SBI cases, 12 cases were symptomatic and 2 cases were asymptomatic. The symptomatic group comprised 8 transient cases (66.7%), 3 operative cases (25.0%), and 1 enema-reduction case (8.3%). Two asymptomatic cases were incidentally captured by computed tomography. Of the 73 ICI cases, 19 cases (26.0%) required operation, and 54 (74.0%) enema-reduction. 3) When transient SBI cases were compared with operated SBI cases, enema-reduced and operated ICI cases, the age ($38.0{\pm}22.9$ months) of transient SBI cases were significantly higher than those of the others (p=0.003). Mean mass size ($20.8{\pm}2.7mm$) in transient SBI was significantly smaller than in the others (p=0.0001). 4) No correlation was found between the existence of enlarged mesenteric lymph nodes and therapeutic method or concomitant illness. 5) Most of the target types observed by US were in transient SBI cases, the remainder were in the enema-reduced ICI cases. In terms of the doughnuts type, all 8 cases (34.8%) with an external hypoechoic rim thickness of >8.9mm were treated surgically. Conclusion: Pediatric intussusception may be classified based on clinical and radiologic findings, which are likely to indicate appropriate therapies.