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Purpose@#We aimed to characterize invasive group B streptococcal infection (IGBSI) in young infants hospitalized through the emergency department. @*Methods@#We analyzed medical records of infants younger than 90 days with IGBSI, defined as group B streptococcus bacteremia or meningitis, who visited the emergency department from January 2009 through December 2021. Their clinical features were compared as per 2 criteria, neonates and meningitis. For the comparisons, we defined 2 composite outcomes, “clinically important abnormality (any of the followings: temperature > 38.5℃, leukocytes 15,000/mm3, absolute neutrophils > 4,000/mm3 or C-reactive protein > 2.0 mg/dL)”and “poor outcomes (any of the followings: intensive care unit, transfer, seizure or complications).”Percentages are rounded to the first decimal places. @*Results@#A total of 35 infants, of whom 15 (43%) were neonates and 17 (49%) were boys, had IGBSIs, including 23 infants (66%) with isolated bacteremia, 11 (31%) with bacteremia with meningitis, and 1 (3%) with isolated meningitis. Ill appearances were noted in 27 infants (77%) who encompassed all with meningitis. The other 8 infants (23%) looking well had 1 or more components of the clinically important abnormality. The neonates showed a higher frequency of vaginal delivery (87% vs. 35%; P = 0.002) and a lower frequency of a temperature > 38.5℃, compared to their counterparts (47% vs. 80%; P = 0.040). The infants with meningitis had a longer median time required to visit (5.0 vs. 2.0 hours; P = 0.011), and higher frequencies of ill appearance (100% vs. 65%; P = 0.032), leukocytes < 5,000/mm3 (67% vs. 13%; P = 0.002), and the poor outcomes (75% vs. 0%; P < 0.001). @*Conclusion@#Young infants with ill-looking appearance or abnormality in any of the fever or inflammatory markers may have IGBSIs, particularly meningitis.
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Since October 2021, severe acute hepatitis of unknown etiology in pediatric patients has been observed in many countries around the world. Adenovirus (mainly enteric adenovirus) was detected in more than 50% of the cases. Nationwide surveillance on acute hepatitis of unknown etiology in pediatric patients was started in May 2022 in Korea. Taking into account the severity of the illness and the urgency of the epidemiological situation worldwide, we report a summary of changes in adenovirus epidemiology during the past five years and six months in Korea.
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An esophageal duplication cyst (EDC) is a congenital anomaly of the foregut. EDCs are often asymptomatic, so they are found incidentally on chest radiographs. In symptomatic patients, EDCs present with cough, dyspnea, vomiting, dysphagia, and chest pain. Potential complications of EDCs include perforation, obstruction, bleeding, and infection. The rarity of this condition may hinder timely diagnosis, which may lead to life-threatening complications. This article describes a case of esophagitis and mediastinitis developed in a 12-month-old infant with an EDC. We emphasize that although rare, EDCs should be considered in the differential diagnosis of non-specific respiratory signs such as cough, dyspnea, and stridor.
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Objective@#This case report was intended to report on a rare cause of spontaneous pneumomediastinum (SPM). @*Methods@#When a patient with Crohn's disease (CD) visited our hospital for pneumomediastinum, a disease activity evaluation was performed, and specific questions were asked to identify possible causes of SPM. @*Results@#The disease status of CD was confirmed as in active by evaluation. Specific questions about situations requiring the Valsalva maneuver, including vigorous exercise, lifting heavy objects, and difficult stool passage, were asked, but none were applicable. When asked an open question regarding incidents related to deep breathing, the patient answered that he had recently started smoking an electronic cigarette which required taking deep breaths. @*Conclusion@#Even when there is an underlying disease, specific history-taking is important if the possibility of complications related to the underlying disease is low. A new wave of previously unseen complications has emerged in parallel with increased electronic cigarette use.
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Purpose@#Infants in the neonatal intensive care unit (NICU) often show cardiorespiratory deterioration during gavage feeding. We aimed to determine whether slow infusion intermittent feeding (SIIF) can reduce respiratory deterioration during gavage feeding in preterm infants in the NICU. @*Methods@#Data on preterm infants whose gavage feeding method was changed to SIIF (1-hour infusion with an infusion pump and 2-hour rest within a 3-hour interval) from bolus gravity feeding (2- or 3-hour interval) due to feeding-associated cardiorespiratory deterioration were retrospectively reviewed. A significant cardiorespiratory event was defined as a saturation level below 80% or heart rate below 80 bpm. We compared the frequency of cardiorespiratory events and the level of respiratory support 24 hours before and after the application of SIIF. @*Results@#A total of 34 infants were enrolled and analyzed. The total frequency of desaturation or bradycardia significantly decreased after SIIF application (8.94 vs. 5.03, P=0.001). The frequency of feeding-related bradycardia and desaturation also significantly decreased (4.15 vs. 1.68, P=0.008). Out of 34 patients, 11 (32.4%) had a decreased level of ventilator support within 1 day after SIIF. The respiratory severity scores of the 10 patients who received invasive ventilator support decreased significantly after SIIF (5.24 vs. 4.59, P=0.032). @*Conclusion@#SIIF significantly decreased gavage feeding-associated cardiorespiratory events and reduced respiratory support in approximately one-third of subjects. Therefore, SIIF may be a therapeutic option for gavage feed-associated respiratory deterioration in preterm infants in the NICU.
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Pyoderma gangrenosum is one of the dermatological extra-intestinal manifestations of ulcerative colitis (UC). We report a case of a 26-year-old male patient suffering from relapsed UC with a newly developed pyoderma gangrenosum. His skin and intestinal symptoms were intractable to treatment with steroids, immunosuppressants, or a single biological agent such as infliximab, golimumab, or vedolizumab. For the first time in Korea, we report a successful treatment experience of pyoderma gangrenosum in UC using dual biological agents, vedolizumab and infliximab. We strategically targeted each of the intestinal and skin symptoms, with a specific biological agent based on the drug’s mechanism of action.
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Chronic recurrent multifocal osteomyelitis (CRMO) is an inflammatory bone disorder presenting with sterile osteomyelitis, most often presenting in childhood. Although the etiology is understood incompletely, its association with other auto-inflammatory diseases including inflammatory bowel disease (IBD); psoriasis; Wegener’s disease; arthritis; and synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome suggests that dysregulated innate immunity may play an important role in the pathogenesis. We report a case of a 13-year-old boy with CRMO associated with Crohn’s disease (CD) successfully treated with infliximab after failure of non-steroidal anti-inflammatory drug (NSAID) treatment. He initially was diagnosed with CRMO based on symmetric and aseptic bone lesions with no fever, lack of response to antibiotic treatment, vertebral involvement, and normal blood cell counts. Despite five months of NSAID treatment, his musculoskeletal symptoms were aggravated, and he developed gastrointestinal symptoms. Finally, he was diagnosed with CRMO associated with CD. Due to the severity of symptoms, infliximab was initiated and produced symptom improvement. This case supports infliximab as another choice for treatment of bowel symptoms in addition to the bone and joint symptoms of CRMO when other first-line treatments are ineffective.
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Purpose@#To determine the prevalence, clinical manifestations, and outcomes of renal involvements in pediatric Alagille syndrome (ALGS). @*Methods@#A total of 21 patients diagnosed with ALGS at age under 18 years who visited Samsung Medical Center from March 1999 to March 2022 were enrolled. ALGS was diagnosed either by clinical manifestations, targeted JAG1 sequencing, and/or liver biopsy. Medical records including sex, age, renal manifestations, urinalysis, serum creatinine, JAG1 sequencing, and ultrasonography were retrospectively reviewed. @*Results@#The male to female ratio was 9:12. The mean age of patients at confirmative diagnosis of ALGS was 18.4 months. Sanger sequencing was performed for 17 patients. Sixteen of 21 patients (76.1%) showed JAG1 mutations. Renal involvement was found in 10 patients (47.6%). The most common type of anomaly was renal dysplasia (40%). One patient having renal dysplasia was pathologically confirmed with glomerular lipid deposition. Two patients (20%) manifested nephrocalcinosisephrolithiasis. Among eight renal-involved patients who survived, four (50%) progressed to chronic kidney disease stage 3. Two of these chronic kidney disease patients were diagnosed with hepatorenal syndrome. The other four patients had renal functions preserved, including two without any interventions and two who underwent urological interventions. @*Conclusions@#The current study revealed a high prevalence of renal involvement in Korean pediatric ALGS with diverse phenotypes.
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Acute pancreatitis (AP) rarely occurs in association with food allergy (FA), and the mechanism is not clearly explored. We herein report a rare case of egg protein-induced AP. A 5-year-old girl was brought to the hospital because of tongue pruritus and an increased level of egg white-specific immunoglobulin E. During an oral food challenge, she developed hives, abdominal pain and vomiting after ingestion of a cumulative of 610 mg of boiled egg white (70 mg of protein). After management with intramuscular injection of epinephrine and orally administered prednisolone and levocetirizine, her skin symptoms improved. The next day, blood tests showed elevated levels of amylase and lipase, and computed tomography revealed a swollen pancreas with a low-attenuated necrotic portion. She was diagnosed with AP and treated with food restriction, intravenous methylprednisolone and gabexate mesilate. The serum levels of amylase and lipase were reduced to normal levels on day 5 and her symptoms completely resolved on day 10. A suspicion of food-induced AP is required for correct diagnosis and proper management in children with FA when they manifest severe abdominal pain.
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Purpose@#Extravasation of diagnostic and therapeutic materials might occur when the intravascular solution leaks into the surrounding tissues. Injury associated with extravasation depends on various factors. It may range from mild skin reaction to severe necrosis. However, the incidence rate for extravasation is largely unknown because of the limited reporting in Korea. Therefore, this study was conducted to identify the incidence of extravasation and nurses’ attitude and knowledge of extravasation for providing high-quality nursing care. @*Methods@#Three acute care hospitals were surveyed to estimate the occurrence of extravasation. Knowledge and attitude toward extravasation were investigated from 793 nurses working in six hospitals. @*Results@#The incidence rate of extravasation was 0.5%. Extravasation commonly occurred in elderly patients aged 66 or older (59.9%) and internal medicine (48.2%), and it happened 13.73±20.68 days after hospitalization on average.It mostly occurred in the forearm site (52.9%) and was mainly caused by parenteral nutrition (33.6%). The mean scores of nurses’ knowledge and attitude were 14.63±2.86 and 28.91±36.00, respectively. There was a significant negative correlation between the subjects' knowledge and attitude (r=-.11, p=.002). @*Conclusion@#It is necessary to have a reporting system that can accurately monitor the occurrence of extravasation for patient safety management. In addition, it is necessary to develop a protocol that can be applied to clinical practice and a nurse education program.
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Background/Aims@#Although mucosal healing (MH) is acknowledged as the treatment target in the treat-to-target era, there are limitations on repeated endoscopic examinations, especially in pediatric patients. We aimed to investigate whether fecal calprotectin (FC) could serve as a surrogate marker for the assessment of MH in pediatric patients with Crohn’s disease (CD) who have achieved sustained clinical remission (CR) while treated with anti-tumor necrosis factor (TNF) agents. @*Methods@#This multicenter retrospective cross-sectional study included pediatric CD patients who had sustained a CR for at least 6 months with anti-TNF agents and who simultaneously underwent ileocolonoscopy and FC tests during follow-up. MH was defined as the absence of any ulcer on ileocolonoscopy. @*Results@#A total of 131 patients were included in this study. MH was observed in 87 patients (66.7%). The FC level was significantly lower in patients with MH than in those without MH (median 49.0 mg/kg vs 599.0 mg/kg; p<0.001). According to the multivariate logistic regression analysis, FC was the only factor associated with MH (odds ratio, 0.62; 95% confidence interval [CI], 0.52 to 0.73; p<0.001). According to the receiver operating characteristic curve analysis, the optimal cutoff value for FC for the association with MH was <140 mg/kg (area under the curve 0.890, 95% CI 0.829 to 0.951, sensitivity 78.2%, specificity 88.6%, p<0.001). @*Conclusions@#FC was associated with MH in pediatric patients with CD who had achieved a sustained CR for at least 6 months with anti-TNF agents. In these patients, FC can be used to stratify patients and guide decisions regarding ileocolonoscopy in the treat-to-target era.
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Background/aims@#The efficacy of biologics for the treatment of Crohn's disease (CD) is affected by the drug concentrations. We aimed to evaluate the importance of albumin and globulin which are known to be associated with drug concentrations as prognostic biomarkers in CD. @*Methods@#In total, 121 pediatric patients with CD who had received anti-tumor necrosis factor (TNF)-α therapy were retrospectively examined between January 2010 and February 2019. @*Results@#Relapse was observed in 48.8% of patients (59/121). The level of calprotectin (odds ratio, 2.13; p=0.03) and the albumin-to-globulin ratio (AGR) at 1 year after anti-TNF-α therapy (odds ratio, 0.0002; p=0.003) were associated with relapse. The AGR at 1 year after anti-TNF-α therapy was the only factor associated with the time-to-relapse (hazard ratio, 0.02; p<0.001). The optimal AGR cutoff value for the prediction of relapse was 1.47 (area under the curve, 0.916; p<0.001). The median infliximab trough level (TL) was lower in patients with AGRs <1.47 than in those with AGRs ≥1.47. Anti-drug antibody (ADA) concentrations were negatively correlated with the AGR at 1 year of anti-TNF-α therapy (r=-0.413, p=0.032). @*Conclusions@#AGR can be used to predict relapse. Patients with AGRs <1.47 at 1 year after anti-TNF-α therapy are more likely to have low drug TLs and develop ADAs, which increase the possibility of relapse than those with AGRs ≥1.47. Therefore, if the AGR at 1 year after anti-TNF-α therapy is less than 1.47, clinicians should monitor disease activity, assess the TLs of the anti-TNF-α agents, test for ADAs and determine the appropriate therapeutic strategies.
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Background@#Chronic exposure to low-dose persistent organic pollutants (POPs) can induce mitochondrial dysfunction. This study evaluated the association between serum POP concentrations and oxygen consumption rate (OCR) as a marker of mitochondrial function in humans and in vitro cells. @*Methods@#Serum concentrations of organochlorine pesticides (OCPs) and polychlorinated biphenyls (PCBs) were measured in 323 adults. The OCRs of platelets and peripheral blood mononuclear cells (PBMCs) were assessed in 20 mL of fresh blood using a Seahorse XF analyzer. Additionally, the in vitro effects of Arochlor-1254, β-hexachlorocyclohexane, and p,p´-dichlorodiphenyltrichloroethane at concentrations of 0.1 pM to 100 nM were evaluated in human platelets, human PBMCs, and Jurkat T-cells. @*Results@#The association between serum POP concentrations and OCR differed depending on the cell type. As serum OCP concentrations increased, basal platelet OCR levels decreased significantly; according to the OCP quintiles of summary measure, they were 8.6, 9.6, 8.2, 8.0, and 7.1 pmol/min/μg (P trend=0.005). Notably, the basal PBMC OCR levels decreased remarkably as the serum PCB concentration increased. PBMC OCR levels were 46.5, 34.3, 29.1, 16.5, and 13.1 pmol/min/μg according to the PCB quintiles of summary measure (P trend <0.001), and this inverse association was consistently observed in all subgroups stratified by age, sex, obesity, type 2 diabetes mellitus, and hypertension, respectively. In vitro experimental studies have also demonstrated that chronic exposure to low-dose POPs could decrease OCR levels. @*Conclusion@#The findings from human and in vitro studies suggest that chronic exposure to low-dose POPs can induce mitochondrial dysfunction by impairing oxidative phosphorylation.
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Primary intestinal lymphangiectasia (IL) can cause leakage of lymphatic fluids into the gastrointestinal tract, eventually leading to protein-losing enteropathy. A 15-year-old male patient, whose disease began at the age of 8 years, recently felt worsening general weakness. After diagnosing abnormal lymphatic lesions in the duodenum through endoscopy with biopsy and contrast-enhanced magnetic resonance lymphangiography, glue embolization of the leaking duodenal lymphatic channel was successfully performed. This procedure is typically reserved for adult patients, although as shown in this case, it can be properly performed in children. His serum albumin level was initially 1.5 g/dL, but elevated to 5.0 g/dL after two sessions of lymphatic embolization. Accordingly, we suggest that embolization could potentially be considered a first-line treatment for focal lesions of primary intestinal IL.
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Purpose@#We aimed to investigate clinical outcomes between top-down (TD) and conventional step-up (SU) therapies in pediatric patients with moderate to severe ulcerative colitis (UC). @*Materials and Methods@#All patients underwent clinical and endoscopic evaluation at diagnosis and 4 months and 1 year after treatment. Patients who started treatment with corticosteroid were grouped in the SU group, while those that initiated early infliximab (IFX) were grouped in the TD group. Among the SU group, patients who eventually changed to IFX treatment due to steroid resistance or dependency were included in the SU(R) group. @*Results@#In total, 44 children with moderate to severe UC were included for analysis. Twenty-one patients were included in the SU group, 23 were included in the TD group, and 10 were enrolled in the SU(R) group. Relapse rates were 47.6% (10/21) in the SU group and 17.4% (4/23) in the TD group (p=0.033). Among relapsed patients, the durations from remission to relapse were 17.3 months (0.9–46.9) in the SU group and 24.3 months (1.8–44.9) in the TD group. There was no statistically significant difference in the sustained durations of remission after IFX administration between the SU(R) and TD groups [3.9 (1.4–6.3) and 2.3 (0.3–5.2) years, respectively (p>0.05)]. @*Conclusion@#According to our study, early use of IFX without corticosteroid treatment for children with moderate to severe UC helps to lower relapse rates. We also found that IFX was a very effective treatment for pediatric UC, with a sustained duration of remission similar between TD and SU(R) groups.
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With the increasing incidence of pediatric inflammatory bowel disease (IBD) in children and its impact throughout life, transition care has become an important issue. In Korea, no guidelines have been proposed to support the transition from the pediatric clinic to the adult IBD clinic. This paper reviews the current issues related to IBD patient care during the transition from pediatrics to adults to identify the barriers and critical elements for a successful transition. Thus far, a multi-disciplinary pediatric/adult clinic or alternate visits between pediatric and adult health care providers is the best model for pediatric to adult IBD clinics. Self-reliance and independence of patients with pediatric IBD are also essential for a successful transition. In addition, the timing of the transition from a pediatric clinic to an adult IBD clinic should not be determined because the issue is not the chronological age but rather the individual maturity.
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Intestinal lymphangiectasia is a rare disease which is causing protein-losing enteropathy.Treatment of intestinal lymphangiectasia can be a challenge for clinicians because of the lack of specific guidelines regarding pharmacological indications. We sought to introduce a diagnostic approach and suggest guidelines for treatment. After exclusion of secondary intestinal lymphangiectasia, magnetic resonance lymphangiography is a promising tool for the assessment of abnormal lymphatic lesions in primary intestinal lymphangiectasia.Determining the extent of the lesion provides direction for treatment options. Focal shortsegment intestinal lymphangiectasia can be treated via intestinal resection or radiologic embolization after dietary therapy failure. Diffuse intestinal lymphangiectasia and extensive lymphangiectasia should be treated with several drugs with a full understanding of their mechanisms.
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Purpose@#The ginger rhizome (Zingiber officinale) is widely cultivated as a spice for its aromatic and pungent components. One of its constituents, 6-hydroxydopamine (6-OHDA) is usually thought to cross the cell membrane through dopamine uptake transporters, and induce inhibition of mitochondrial respiration and the generation of intracellular reactive oxygen species (ROS). This study examines the neuroprotective effect and acetylcholinesterase (AChE) inhibitory activity of fermented ginger extracts (FGEs) on 6-OHDA induced toxicity in SH-SY5Y human neuroblastoma cells. @*Methods@#Ginger was fermented using 2 species of Bacillus subtilis, with or without enzyme pretreatment. Each sample was extracted with 70% ethanol. Neurotoxicity was assessed by applying the EZ-Cytox cell viability assay and by measuring lactic dehydrogenase (LDH) release. Morphological changes of apoptotic cell nuclei were observed by Hoechst staining. Cell growth and apoptosis of SH-SY5Y cells were determined by Western blotting and enzyme activity analysis of caspase-3, and AChE enzymatic activity was determined by the assay. @*Results@#In terms of cell viability and LDH release, exposure to FGE showed neuroprotective activities against 6-OHDA stimulated stress in SH-SY5Y cells. Furthermore, FGE reduced the 6-OHDA-induced apoptosis, as determined by Hoechst staining. The occurrence of apoptosis in 6-OHDA treated cells was confirmed by determining the caspase-3 activity. Exposure to 6-OHDA resulted in increased caspase-3 activity of SH-SY5Y cells, as compared to the unexposed group. However, pre-treatment with FGE inhibited the activity of caspase-3. The neuroprotective effects of FGE were also found to be caspase-dependent, based on reduction of caspase-3 activity. Exposure to FGE also inhibited the activity of AChE induced by 6-OHDA, in a dose-dependent manner. @*Conclusion@#Taken together, our results show that FGE exhibits a neuroprotective effect in 6-OHDA treated SH-SY5Y cells, thereby making it a potential novel agent for the prevention or treatment of neurodegenerative disease.
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Background@#Despite advancements in heart transplantation for pediatric patients in Korea, the waiting list mortality has not been reported. Therefore, we investigated the waiting list mortality rate and factors associated with patient mortality. @*Methods@#We reviewed the medical records of pediatric patients who were registered for heart transplantation at three major hospitals in Korea from January 2000 to January 2020.All patients who died while waiting for heart transplantation were investigated, and we identified the waiting list mortality rate, causes of mortality and the median survival periods depending on the variable risk factors. @*Results@#A total of 145 patients received heart transplantations at the three institutions we surveyed, and the waiting list mortality rate was 26%. The most common underlying diseases were cardiomyopathy (66.7%) and congenital heart disease (30.3%). The leading causes that contributed to death were heart failure (36.3%), multi-organ failure (27.2%), and complications associated with extracorporeal membrane oxygenation (ECMO) (25.7%). The median survival period was 63 days. ECMO was applied in 30 patients. The different waiting list mortality percentages according to age, cardiac diagnosis, use of ECMO, and initial Korean Network of Organ Sharing (KONOS) level were determined using univariate analysis, but age was the only significant factor associated with waiting list mortality based on a multivariate analysis. @*Conclusion@#The waiting list mortality of pediatric heart transplantation candidates was confirmed to be considerably high, and age, underlying disease, the application of ECMO, and the initial KONOS level were the factors that influenced the survival period.
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Crohn’s disease (CD) presenting as gastric outlet obstruction is rare but serious clinical presentation of CD causing severe morbidity. However, there have been few case reports concerning this disorder in East Asian children and adolescents. The current case report describes 2 pediatric patients with CD who had had gastric outlet obstruction as an initial symptom of CD. Two pediatric patients developed postprandial vomiting, bloating, and unintentional weight loss. The upper endoscopy result indicated that there was pyloric obstruction with mucosal edema, inflammation and ulcers. The serologic test and colonoscopy results suggested CD. These patients were treated with infliximab, and endoscopic balloon dilation without surgery and showed remarkable improvement in obstructing symptoms with maintaining clinical and biochemical remission. This case report elucidates the benefits of early intervention using infliximab and endoscopic balloon dilation to improve gastric outlet obstruction and achieve baseline recovery in patients with upper gastrointestinal B2 phenotype of CD.