• Pediatric Infection and Vaccine
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• v.24 no.2
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• pp.117-122
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• 2017

Blackwater fever is a serious clinical syndrome manifested by acute intravascular hemolysis, fever, and the passage of black or red urine, which is classically associated with falciparum malaria and irregular administration of quinine. In Korea, Plasmodium vivax is the only endemic malaria circulating; a number of imported cases of falciparum malaria have been reported in patients following return from international travel to a malaria endemic area. Therefore, it is important for health care professionals including pediatricians to be aware of the falciparum malaria and its clinical courses. Herein, we report a case of a 14-year-old girl with severe falciparum malaria that was complicated by blackwater fever.

• Clinical and Experimental Pediatrics
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• v.64 no.7
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• pp.364-369
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• 2021

Background: As the coronavirus disease 2019 (COVID-19) outbreak continues to evolve, it is crucially important for pediatricians to be aware of the differences in demographic and clinical features between COVID-19 and influenza A and B infections. Purpose: This study analyzed and compared the clinical features and laboratory findings of COVID-19 and influenza A and B infections in children. Methods: This retrospective study evaluated the medical data of 206 pediatric COVID-19 and 411 pediatric seasonal influenza A or B patients. Results: COVID-19 patients were older than seasonal influenza patients (median [interquartile range], 7.75 [2-14] years vs. 4 [2-6] years). The frequency of fever and cough in COVID-19 patients was lower than that of seasonal influenza patients (80.6% vs. 94.4%, P<0.001 and 22.8 % vs. 71.5%, P<0.001, respectively). Ageusia (4.9%) and anosmia (3.4%) were present in only COVID-19 patients. Leukopenia, lymphopenia, and thrombocytopenia were encountered more frequently in influenza patients than in COVID-19 patients (22.1% vs. 8.5%, P=0.029; 17.6% vs. 5.6%, P=0.013; and 13.2% vs. 5.6%, P=0.048, respectively). Both groups showed significantly elevated monocyte levels in the complete blood count (70.4% vs. 69.9%, P=0.511). Major chest x-ray findings in COVID-19 patients included mild diffuse ground-glass opacity and right lower lobe infiltrates. There were no statistically significant intergroup differences in hospitalization or mortality rates; however, the intensive care unit admission rate was higher among COVID-19 patients (2.4% vs. 0.5%, P=0.045). Conclusion: In this study, pediatric COVID-19 patients showed a wide range of clinical presentations ranging from asymptomatic/mild to severe illness. We found no intergroup differences in hospitalization rates, oxygen requirements, or hospital length of stay; however, the intensive care unit admission rate was higher among COVID-19 patients.

• Clinical and Experimental Pediatrics
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• v.64 no.7
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• pp.313-321
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• 2021

Here we describe the neurodevelopmental outcomes of very low birth weight (VLBW) infants (birth weight ≤1,500 g) at 3 years of age in the Neonatal Research Network of Japan (NRNJ) database in the past decade and review the methodological issues identified in follow-up studies. The follow-up protocol for children at 3 years of chronological age in the NRNJ consists of physical and comprehensive neurodevelopmental assessments in each participating center. Neurodevelopmental impairment (NDI)-moderate to severe neurological disability-is defined as cerebral palsy (CP) with a Gross Motor Function Classification System score ≥2, visual impairment such as uni- or bilateral blindness, hearing impairment requiring hearing amplification, or cognitive impairment with a developmental quotient (DQ) of Kyoto Scale of Psychological Development score <70 or judgment as delayed by pediatricians. We used death or NDI as an unfavorable outcome in all study subjects and NDI in survivors using number of assessed infants as the denominator. Follow-up data were collected from 49% of survivors in the database. Infants with follow-up data had lower birth weights and were of younger gestational age than those without follow-up data. Mortality rates of 40,728 VLBW infants born between 2003 and 2012 were 8.2% before discharge and 0.7% after discharge. The impairment rates in the assessed infants were 7.1% for CP, 1.8% for blindness, 0.9% for hearing impairment, 15.9% for a DQ <70, and 19.1% for NDI. The mortality or NDI rate in all study subjects, including infants without follow-up data, was 17.4%, while that in the subjects with outcome data was 32.5%. The NRNJ follow-up study results suggested that children born with a VLBW remained at high risk of NDI in early childhood. It is important to establish a network follow-up protocol and complete assessments with fewer dropouts to enable clarification of the outcomes of registered infants.

• Childhood Kidney Diseases
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• v.23 no.1
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• pp.53-57
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• 2019

Renal cortical necrosis (RCN) is patchy or diffuse ischemic destruction of the renal cortex caused by significantly reduced renal arterial perfusion. It is a rare cause of acute kidney injury (AKI) and is associated with high mortality. Here, we review the case of RCN in a 15-year-old boy who developed AKI. A 15-year-old boy was referred to our hospital from a local hospital due to a sharp decrease in his renal function. He presented with acute flank pain, nausea with vomiting, and oliguria for the past two days. He had taken a single dose of antihistamine for nasal congestion. At our hospital, his peak blood pressure was 148/83 mmHg and he had a high body mass index of $32.9kg/m^2$. The laboratory data showed a blood urea nitrogen (BUN) of 28.4 mg/dL, a creatinine of 4.26 mg/dL, and a glomerular filtration rate estimated from the serum cystatin C of $20.2mL/min/1.73m^2$. Proteinuria (spot urine protein to creatinine ratio 1.66) with pyuria was observed. Kidney sonography showed parenchymal swelling and increased renal echogenicity. Due to rapidly progressing nephritis, steroid pulse therapy (750 mg/IV) was done on the second day of his admission and the patient showed complete recovery with normal renal function. However, the kidney biopsy findings revealed renal cortical hemorrhagic necrosis. Multifocal, relatively well-circumscribed, hemorrhagic necrotic areas (about 25%) were detected in the tubulointerstitium. Although RCN is an unusual cause of AKI, especially in children, pediatricians should consider the possibility of RCN when evaluating patients with rapidly decreasing renal function.

• Clinical and Experimental Pediatrics
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• v.63 no.11
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• pp.433-437
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• 2020

Background: Indonesia is one of the countries with the highest preterm birth rate. Preterm infants are more likely than term and normal weight infants to experience neonatal mortality and morbidity due to acute respiratory, gastrointestinal, immunologic, central nervous system, hearing, and vision problems. Kangaroo mother care (KMC) is a proven cost-effective intervention to help reduce mortality rates among preterm infants; however, it has not been fully implemented in hospitals. Purpose: Assess KMC knowledge and perceptions among health providers. Methods: This qualitative study was conducted from December 2015 to April 2016 and consisted of 21 in-depth interviews and 3 focus group discussions (FGDs). The 3 categories of health personnel in the study were clinical providers, hospital management representatives, and Indonesian Midwife Association members. Results: Most health providers know about the benefits of KMC including stabilizing temperatures, weight gain, and maternal-infant bonding and reducing human resources and labor costs. They were also aware of which newborns were eligible for KMC treatment. Their knowledge was mostly gained from observation or obtained from pediatricians and personal experience. They believed that a low birth weight infant in an incubator could not be treated with KMC and that it could only be practiced if a special gown was used when holding the baby. This perception could be caused by a lack of formal KMC training, leading to misunderstanding of its aspects. Conclusion: In conclusion, KMC knowledge of clinical providers in the 2 hospitals was sufficient, primarily due to their health-related educational background. Some perceptions could be potential barriers to or facilitate the implementation of KMC practice. These perceptions should be considered in future KMC training designs.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.24 no.4
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• pp.325-336
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• 2021

The occurrence of functional gastrointestinal disorders (FGIDs) is a formidable challenge for infants, parents, and healthcare professionals. Although data from the Middle East are scarce, experts consider FGIDs a prevalent condition in everyday clinical practice. The new Rome IV criteria revisited the definitions from a clinical perspective to provide a practical and consistent diagnostic protocol for FGIDs. However, the treatment practices for functional disorders vary considerably among Middle Eastern countries, often resulting in mismanagement with unnecessary investigations and treatments. In addition, the role of various treatment modalities, including probiotics such as Lactobacillus reuteri DSM 17938, in FGIDs requires further discussion and evaluation. During a consensus meeting, a locally relevant approach for treating common FGIDs such as infant regurgitation, infant colic, and functional constipation was discussed and approved by regional experts. The participants suggested a simplified treatment plan and protocol for general pediatricians and other primary care physicians managing FGIDs. This easy-to-follow standardized protocol will help streamline the initial management of this complex disorder in the Middle East region and even globally.

• Pediatric Infection and Vaccine
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• v.29 no.2
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• pp.110-117
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• 2022

Herpes zoster (HZ) has been reported in immunocompetent children who received the varicella vaccine. In vaccinated children, HZ can be caused by vaccine-strain or by wild-type varicella-zoster virus (VZV). Like wild-type VZV, varicella vaccine virus can establish latency and reactivate as HZ. We report two cases of HZ in otherwise healthy 16- and 14-month-old boys who received varicella vaccine at 12 months of age. They presented with a vesicular rash on their upper extremities three to four months after varicella vaccination. In one case, a swab was obtained by abrading skin vesicles and VZV was detected in skin specimens by polymerase chain reaction. The VZV open-reading frame 62 was sequenced and single nucleotide polymorphism analysis confirmed that the virus from skin specimen was vaccine-strain. This is the first HZ case following varicella vaccination confirmed to be caused by vaccine-strain VZV in the immunocompetent children in Korea. Pediatricians should be aware of the potential for varicella vaccine virus reactivation in vaccinated young children.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.25 no.3
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• pp.263-275
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• 2022

Purpose: The present international survey among healthcare providers aimed to collect data on theoretical knowledge and clinical practices in the diagnosis and management of cow's milk protein allergy (CMPA) and lactose intolerance (LI) in infants. Methods: A global survey was conducted in several countries with diverse health care settings. The survey consisted of multiple-choice questions in 3 main domains: (1) understanding and clinical practices around CMPA and LI; (2) case scenarios; and (3) disease-specific knowledge and potential educational needs. Results: Responses were available from 1,663 participants. About 62% of respondents were general practitioners or general pediatricians, and the remainder were pediatric allergists/gastroenterologists (18%) or other health practitioners (20%). The survey identified knowledge gaps regarding the types of CMPA (IgE-mediated vs. non-IgE-mediated) and the clinical overlap with LI. The survey suggested diverse clinical practices regarding the use of hypoallergenic formulas, as well as misconceptions about the prebiotic benefits of lactose in extensively hydrolyzed formulas in non-breastfed infants with CMPA. Responses to the two case scenarios highlighted varying levels of awareness of the relevant clinical practice guidelines. While respondents generally felt confident in managing infants with CMPA and LI, about 80% expressed an interest for further training in this area. Conclusion: The current survey identified some knowledge gaps and regional differences in the management of infants with CMPA or LI. Local educational activities among general and pediatric healthcare providers may increase the awareness of clinical practice guidelines for the diagnosis and treatment of both conditions and help improve clinical outcomes.

• Journal of Genetic Medicine
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• v.19 no.2
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• pp.76-84
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• 2022

Purpose: Whole-exome sequencing (WES) has been a useful tool for novel gene discovery of various disease categories, further increasing the diagnostic yield. This study aimed to investigate the clinical utility of WES prospectively in undiagnosed genetic diseases. Materials and Methods: WES tests were performed on 110 patients (age range, 0-28 years) with suspected rare genetic diseases. WES tests were performed at a single reference laboratory and the variants reported were reviewed by clinical geneticists, pediatricians, neurologists, and laboratory physicians. Results: The patients' symptoms varied with abnormalities in the head or neck, including facial dysmorphism, being the most common, identified in 85.4% of patients, followed by abnormalities in the nervous system (83.6%). The average number of systems manifesting phenotypic abnormalities per patient was 3.9±1.7. The age at presentation was 2.1±2.7 years old (range, 0-15 years), and the age at WES testing was 6.7±5.3 years (range, 0-28 years). In total, WES test reported 100 pathogenic/likely pathogenic variants or variants of uncertain significance for 79 out of 110 probands (71.8%). Of the 79 patients with positive or inconclusive calls, 55 (50.0%) patients were determined to have good genotype-phenotype correlations after careful review. Further clinical reassessment and family member testing determined 45 (40.9%) patients to have been identified with a molecular diagnosis. Conclusion: This study showed a 40.9% diagnostic yield for WES test for a heterogeneous patient cohort with suspected rare genetic diseases. WES could be the feasible genetic test modality to overcome the diversity and complexity of rare disease diagnostics.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.26 no.5
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• pp.277-283
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• 2023

Purpose: Cow's milk protein allergy (CMPA) is a common condition in infants, but little is known about healthcare providers' clinical experience treating infants with CMPA. To address this gap, we analyzed prospectively collected data from healthcare providers (HCPs) who treated infants under six months old with suspected CMPA using hypoallergenic formulas. The study focused on a commercial extensively hydrolyzed formula containing Lactobacillus rhamnosus GG (ATCC53103) (eHF-LGG) or a commercial amino acid formula (AAF). Methods: In this secondary analysis of prospectively collected survey data, 52 HCPs treated 329 infants under six months old with suspected CMPA using hypoallergenic formulas. A series of two de-identified surveys per patient were collected by HCPs to assess short-term symptom relief in the patients and HCP's satisfaction with the management strategies. The initial survey was completed at the initiation of treatment of CMPA, and the second survey was completed at a follow-up visit. Results: The majority of HCPs (87%) in the study were general pediatricians, and most saw 2 to 10 CMPA patients weekly. Results showed that clinicians reported satisfaction with treatment in 95% of patients in the EHF cohort and 97% of patients in the AAF cohort and achieved expected clinical results in 93% and 97% of patients using eHF and AAF, respectively. Furthermore, few patients were switched from the hypoallergenic formula once initiated. Conclusion: The study provides new insights into HCP perspectives on treating infants with CMPA and supports using hypoallergenic formulas to manage this condition. However, additional prospective controlled studies are needed to confirm these initial findings.