Postinfectious bronchiolitis obliterans (PIBO) is an irreversible obstructive lung disease characterized by subepithelial inflammation and fibrotic narrowing of the bronchioles after lower respiratory tract infection during childhood, especially early childhood. Although diagnosis of PIBO should be confirmed by histopathology, it is generally based on history and clinical findings. Irreversible airway obstruction is demonstrated by decreased forced expiratory volume in 1 second with an absent bronchodilator response, and by mosaic perfusion, air trapping, and/or bronchiectasis on computed tomography images. However, lung function tests using spirometry are not feasible in young children, and most cases of PIBO develop during early childhood. Further studies focused on obtaining serial measurements of lung function in infants and toddlers with a risk of bronchiolitis obliterans (BO) after lower respiratory tract infection are therefore needed. Although an optimal treatment for PIBO has not been established, corticosteroids have been used to target the inflammatory component. Other treatment modalities for BO after lung transplantation or hematopoietic stem cell transplantation have been studied in clinical trials, and the results can be extrapolated for the treatment of PIBO. Lung transplantation remains the final option for children with PIBO who have progressed to end-stage lung disease.
Bronchiolitis obliterans; Post-infectious; Child; Inflammation; Fibrosis
This study attempted to examine parental satisfaction with pediatric emergency care (PEC) in Korea and investigate the features influencing overall satisfaction.
A nationwide, cross-sectional survey was conducted among parents who had taken their children to an Emergency Department (ED) in the three years prior to the study. A 21-item, structured questionnaire was administered to the parents through a web-based system. Participants' satisfaction levels and the strength of the association between PEC components and overall satisfaction were rated using a 7-point Likert scale.
In total, 1,000 parents participated in the survey, of which 402 (40.2%) stated that they were generally satisfied with the delivered PEC. Female participants, mothers, and parents with low-acuity patients were more likely to be dissatisfied with the delivered PEC. Although the ED environment was the lowest level of satisfaction, it did not significantly influence the respondents' overall levels of satisfaction. The most influential factors in terms of overall satisfaction were "nurses' professionalism" and "doctors' attitude and proper explanation".
Parents' overall level of satisfaction with PEC is relatively low and is closely related to factors associated with ED personnel.
Personal satisfaction; Hospital emergency service; Child
Adherence to treatment with inhaled corticosteroids (ICS) is a critical determinant of asthma control. The objective of this study was to assess factors that determine adherence to ICS therapy in children with asthma.
Fifty-eight children with asthma, aged 5 to 16 years, used ICS with or without a spacer for 3 months. Adherence rates as measured from questionnaires and canisters, asthma symptom scores, and inhalation technique scores were assessed every 30 days. The degree of supervision by caregivers was assessed at day 30.
Adherence rates measured using canisters were lower at day 60 than at day 30 (P=0.044) and did not change thereafter (74.4%±17.4% at day 30, 66.5%±18.4% at day 60, and 67.4%±22.2% at day 90). Adherence rates at days 60 and 90 and during the total study period were significantly different when measured by using questionnaires versus canisters (P<0.001, P=0.022, and P=0.001, respectively). In the comparison of adherence rates repeatedly measured at days 30, 60, and 90 and adherence rates during the total study period among the 3 groups, adherence rates in the high-degree supervision group were significantly higher than those in the low-degree supervision group (82.0±16.0 vs. 66.1±14.5, 75.4±14.4 vs. 56.2±18.4, 75.0±18.3 vs. 55.0±19.7 [P=0.027]; 77.9±12.2 vs. 59.1±11.4 [P=0.021]) after adjustment for sex and age.
The level of caregiver supervision is an important factor affecting adherence to ICS therapy in children with asthma. Therefore, a high degree of supervision may be required to increase adherence to ICS therapy in children with asthma.
Asthma; Adherence; Child; Inhaled corticosteroids; Supervision
Information on the clinical features of lung abscess, which is uncommon in children, at hospitalizationis helpful to anticipate the disease course and management. There is no report concerning lung abscess in Korean children. We aimed to identify the clinical characteristics of pediatric lung abscess and compare the difference between primary and secondary abscess groups.
The medical records of 11 lung abscess patients (7 males and 4 females) from March 1998 to August 2011 at two university hospitals were retrospectively reviewed. The clinical characteristics, symptoms, underlying disease, laboratory and radiologic findings, microbiological results, and treatments were examined.
Six patients had underlying structural-related problems (e.g., skeletal anomalies). No immunologic or hematologic problem was recorded. The mean ages of the primary and secondary groups were 2.4 and 5.3 years, respectively, but the difference was not statistically significant. The mean length of hospital stay was similar in both groups (22.8 days vs. 21.4 days). Immunologic studies were performed in 3 patients; the results were within the normal range. Most patients had prominent leukocytosis. Seven and 4 patients had right and left lung abscess, respectively. Staphylococcus aureus, Streptococcus pneumoniae, and antimycoplasma antibodies were detected in both groups. Two patients with primary lung abscess were administered antibiotics in the absence of other procedures, while 8 underwent interventional procedures, including 5 with secondary abscess.
The most common symptoms were fever and cough. All patients in the primary group were younger than 3 years. Structural problems were dominant. Most patients required interventional procedures and antibiotics.
Lung abscess; Child; Predisposing factor
To determine the efficacy and safety of oral propranolol as a first-line treatment for superficially located infantile hemangioma (IH) and propose an assessment tool to measure treatment response.
Patients with superficial IH under 1 year of age were prospectively recruited between May 2012 and December 2013 at the Department of Pediatrics of Chungbuk National University Hospital. Propranolol was administered to 12 infants (median age, 3.8 months) while monitoring cardiovascular and adverse metabolic effects. If a patient showed no adverse events, the dosage was gradually increased up to 3 mg/kg/day and maintained for 1 year. We used our own scoring system to assess treatment response using parameters like change in color, and longest diameter, and thickness of the IH.
Eleven out of 12 patients completed the protocol with consistent improvement of hemangiomas during therapy. Patients on propranolol showed a more than 50% involution in the first 3 months, with additional steady involution until 1 year. Patients with the highest scores at 1 month maintained their score and showed better responses until treatment termination. The patient with the lowest score at 1 month did not show any further regression and stopped propranolol treatment 4 months after initiation. In two children with recurrences after successful therapeutic regression, propranolol was effective after being reintroduced. Propranolol treatment was not interrupted in any patient due to adverse events.
Oral propranolol at 3 mg/kg/day showed a consistent, rapid, and therapeutic effect on superficial IHs without significant adverse events.
Hemangioma; Propranolol; Safety; Effect
To investigate the caustic ingestion in children among different continents according to demographic characteristics (core purpose), main symptoms, common caustic agents, signs and symptoms, management, treatment and complications.
This systematic review was performed by searching the databases Science Direct, ProQuest, Google Scholar, and PubMed, electronically and manually. We included studies that were published from 1980 to 2013, at University of Medical Sciences of Tabriz, Iran. A strategic search was performed with keywords including caustic, corrosive, ingestion and children, and was limited to articles in English and Persian. Statistical analysis was performed by SPSS ver. 18.
Of 63 selected articles of caustic ingestion with 9,888 samples, the proportion of Africa was 3 articles (4.8%) and 95 samples (1%), America 9 articles (14.3%) and 305 sample (3%), Asia 29 articles (46%) and 2,780 samples (28.1%), Europe 17 articles (27%) and 3,002 samples (30.4%), and Oceania 5 articles (7.9%) and 3,706 samples (37.5%). The average age was in the Africa 3.07±2.02 years, America 3.17±1.83 years, Asia 3.34±1.58 years, Europe 3.58±2.09 years and Oceania 3.52±2.02 years. Sex distribution was in Africa 76 males (0.91%) and 19 females (0.23%), America 49 males (0.58%) and 41 females (0.49%), Asia 1,575 males (18.76%) and 1,087 females (12.95%), Europe 1,018 males (12.13%) and 823 females (9.8%), and Oceania 1,918 males (22.85%) and 1,788 females (21.3%). Statistical analysis of the data indicated higher consumption in Europe and Oceania in the boys with higher average age of years.
The comparison of caustic ingestion indicated that the cause substances of caustic ingestion in children are different among continents, therefore prevention strategy and different treatment guidelines among continents will be needed.
Caustic ingestion; Systematic review; Child; Continents
We present the case of a 5-year-old child with coronary complications due to Kawasaki disease; this patient unintentionally underwent both dual-source computed tomography (DSCT) coronary angiography and invasive coronary angiographic examination in 2 months. This case highlights the strong consistency of the results between DSCT coronary angiography and invasive coronary angiography. Compared to conventional invasive coronary angiography, DSCT coronary angiography offered additional advantages such as minimal invasiveness and less radiation exposure.
Coronary aneurysm; Mucocutaneous lymph node syndrome; Coronary angiography; Multidetector computed tomography
It is uncommon for pediatric patients with rhabdomyosarcoma to present with clinical and/or laboratory features of disseminated intravascular coagulation (DIC). We report a case of metastatic alveolar rhabdomyosarcoma with severe bleeding because of DIC in a 13-year-old boy. He experienced persistent oozing at the site of a previous operation, gross hematuria, and massive epistaxis. Two weeks after initiating combination chemotherapy consisting of vincristine, doxorubicin, and cyclophosphamide, the patients' laboratory indications of DIC began to resolve. During this period, the patient received massive blood transfusion of a total of 311 units (26 units of red blood cells, 26 units of fresh frozen plasma, 74 units of platelet concentrates, 17 units of single donor platelets, and 168 units of cryoprecipitate), antithrombin-III and a synthetic protease inhibitor. Despite chemotherapy and radiation therapy, he died 1 year later because of disease progression. In children with metastatic rhabdomyosarcoma and massive DIC, prompt chemotherapy and aggressive supportive care is important to decrease malignancy-triggered procoagulant activities.
Rhabdomyosarcoma; Disseminated intravascular coagulation; Chemotherapy
Chest pain is common in children and adolescents and is a reason for referral to pediatric cardiologists. Although most cases of chest pain in these age groups are benign and do not require treatment, timely diagnosis is important not to miss life-threatening diseases requiring prompt treatment. We investigated certain clinical characteristics that may be useful in the diagnosis of such critical diseases.
Patient medical records between July 2006 and September 2013 were retrospectively examined. We included 517 patients who presented with chest pain to the Department of Pediatrics at Kyung Hee University Hospital in Gangdong.
Most cases of chest pain were idiopathic in origin (73.6%), followed by cases with respiratory (9.3%), musculoskeletal (8.8%), cardiac (3.8%), gastrointestinal (2.9%), and psychiatric (1.4%) causes. In 6 patients (1.2%) with air-leak syndrome including pneumothorax or pneumomediastinum, the pain was abrupt, continuous, and lasted for a short period of 1-2 days after onset in the older adolescents. Of the patients with cardiac pain, 13 had cardiac arrhythmias (65.0%), 6 had congenital heart diseases (30%), and 1 had coronary aneurysms caused by Kawasaki disease (5.0%). One patient with atrial flutter had only symptoms of syncope and chest pain.
The abrupt, continuous chest pain of a short duration in the older children was characteristic of air-leak syndrome. In patients with pneumomediastinum, radiological diagnosis was difficult without careful examination. Combined syncope should not be neglected and further cardiac workup is essential in such patients.
Chest pain; Child; Adolescent; Musculoskeletal diseases
Compartment syndrome is a rare but devastating condition that can result in permanent neuromuscular or soft tissue injuries. Extravasation injuries, among the iatrogenic causes of compartment syndrome, occur under a wide variety of circumstances in the inpatient setting. Total parenteral nutrition via a peripheral route is an effective alternative for the management of critically ill children who do not obtain adequate nutrition via the oral route. However, there is an inherent risk of extravasation, which can cause compartment syndrome, especially when detected at a later stage. Herein, we report a rare case of compartment syndrome and skin necrosis due to extravasation, requiring emergency fasciotomy and skin graft in a 7-month-old boy who was treated with peripheral parenteral nutrition via a pressurized infusion pump. Although we cannot estimate the exact time at which extravasation occurred, the extent and degree of the wound suggest that the ischemic insult was prolonged, lasting for several hours. Pediatric clinicians and medical teams should carefully examine the site of insertion of the intravenous catheter, especially in patients receiving parenteral nutrition via a peripheral intravenous catheter with a pressurized infusion pump.
Compartment syndromes; Extravasation; Infusion pumps; Parenteral nutrition; Skin transplantation
Early-onset epileptic encephalopathies are one of the most severe early onset epilepsies that can lead to progressive psychomotor impairment. These syndromes result from identifiable primary causes, such as structural, neurodegenerative, metabolic, or genetic defects, and an increasing number of novel genetic causes continue to be uncovered. A typical diagnostic approach includes documentation of anamnesis, determination of seizure semiology, electroencephalography, and neuroimaging. If primary biochemical investigations exclude precipitating conditions, a trial with the administration of a vitaminic compound (pyridoxine, pyridoxal-5-phosphate, or folinic acid) can then be initiated regardless of presumptive seizure causes. Patients with unclear etiologies should be considered for a further workup, which should include an evaluation for inherited metabolic defects and genetic analyses. Targeted next-generation sequencing panels showed a high diagnostic yield in patients with epileptic encephalopathy. Mutations associated with the emergence of epileptic encephalopathies can be identified in a targeted fashion by sequencing the most likely candidate genes. Next-generation sequencing technologies offer hope to a large number of patients with cryptogenic encephalopathies and will eventually lead to new therapeutic strategies and more favorable long-term outcomes.
Epileptic encephalopathy; Early infantile; Genetics; Mutation; High throughput nucleotide sequencing
Bronchopulmonary dysplasia (BPD) is characterized by inflammation with proteolytic damage to the lung extracellular matrix. The results from previous studies are inconsistent regarding the role of proteinases and antiproteinases in the development of BPD. The aim of the present study was to investigate whether matrix metalloproteinase (MMP)-8, MMP-9, tissue inhibitor of metalloproteinase (TIMP)-2, and TIMP-1 levels in the serum of preterm infants at birth are related to the development of BPD.
Serum was collected from 62 preterm infants at birth and analyzed for MMP-8, MMP-9, TIMP-2, and TIMP-1 by using enzyme-linked immunosorbent assay. MMPs and TIMPs were compared in BPD (n=24) and no BPD groups (n=38). Clinical predictors of BPD (sex, birth weight, gestational age, etc.) were assessed for both groups. The association between predictors and outcome, BPD, was assessed by using multivariate logistic regression.
Sex, birth weight, and mean gestational age were similar between the groups. BPD preterm infants had significantly lower TIMP-2 levels at birth compared with no BPD preterm infants (138.1±23.0 ng/mL vs. 171.8±44.1 ng/mL, P=0.027). No significant difference was observed in MMP-8, MMP-9, and TIMP-1 levels between the two groups. Multivariate logistic regression analysis indicated that the TIMP-2 levels were predictive of BPD after adjusting for sex, birth weight, gestational age, proteinuric preeclampsia, and intraventricular hemorrhage (β=-0.063, P=0.041).
Low TIMP-2 serum levels at birth may be associated with the subsequent development of BPD in preterm infants.
Bronchopulmonary dysplasia; Matrix metalloproteinases; Premature infants; Tissue inhibitor of metalloproteinase
The vancomycin dosage regimen is regularly modified according to the patient's glomerular filtration rate (GFR). In the present study, we aimed to assess the usefulness of serum cystatin C (Cys-C) concentration, compared with serum creatinine (SCr) concentration, for predicting vancomycin clearance (CLvcm) in neonates.
We retrospectively analyzed the laboratory data of 50 term neonates who were admitted to the neonatal intensive care unit and received intravenous vancomycin, and assessed the pharmacokinetic profiles. Creatinine clearance (CLcr) and GFR based on Cys-C (GFRcys-c) were estimated using the Schwartz and Larsson formulas, respectively.
The mean CLvcm (±standard deviation) was 74.52±31.17 L/hr, the volume of distribution of vancomycin was 0.67±0.14 L, and vancomycin half-life was 9.16±17.42 hours. The SCr was 0.46±0.25 mg/dL and serum Cys-C was 1.43±0.34 mg/L. The peak and trough concentrations of vancomycin were 24.65±14.84 and 8.10±5.35 mcg/mL, respectively. The calculated GFR based on serum creatinine concentration (GFR-Cr) and GFRcys-c were 70.2±9.45 and 63.6±30.18 mL/min, respectively. The correlation constant for CLvcm and the reciprocal of Cys-C (0.479, P=0.001) was significantly higher than that for CLvcm and the reciprocal of SCr (0.286, P=0.044). GFRcys-c was strongly correlated with CLvcm (P=0.001), and the correlation constant was significantly higher than that for CLvcm and CLcr (0.496, P=0.001). Linear regression analysis showed that only GFRcys-c was independently and positively correlated with CLvcm (F=41.9, P<0.001).
The use of serum Cys-C as a marker of CLvcm could be beneficial for more reliable predictions of serum vancomycin concentrations, particularly in neonates.
Vancomycin; Cystatin C; Clearance
We investigated the vitamin D status of preterm infants to determine the incidence of vitamin D deficiency.
A total of 278 preterm infants delivered at Kyungpook National University Hospital between January 2013 and May 2015 were enrolled. The serum concentrations of calcium, phosphorous, alkaline phosphatase, and 25-hydroxyvitamin D (25-OHD) were measured at birth. We collected maternal and neonatal data such as maternal gestational diabetes, premature rupture of membranes, maternal preeclampsia, birth date, gestational age, and birth weight.
Mean gestational age was 33+5±2+2 weeks of gestation and mean 25-OHD concentrations were 10.7±6.4 ng/mL. The incidence of vitamin D deficiency was 91.7%, and 51.1% of preterm infants were classified as having severe vitamin D deficiency (25-OHD<10 ng/mL). The serum 25-OHD concentrations did not correlate with gestational age. There were no significant differences in serum 25-OHD concentrations or incidence of severe vitamin D deficiency among early, moderate, and late preterm infants. The risk of severe vitamin D deficiency in twin preterm infants was significantly higher than that in singletons (odds ratio, 1.993; 95% confidence interval [CI], 1.137-3.494, P=0.016). In the fall, the incidence of severe vitamin D deficiency decreased 0.46 times compared to that in winter (95% CI, 0.227-0.901; P=0.024).
Most of preterm infants (98.9%) had vitamin D insufficiency and half of them were severely vitamin D deficient. Younger gestational age did not increase the risk of vitamin D deficiency, but gestational number was associated with severe vitamin D deficiency.
Vitamin D; 25-hydroxyvitamin D; Premature infant
Kawasaki disease involves acute febrile systemic vasculitis that can cause a variety of symptoms by affecting various organs. Here, we aimed to evaluate the prevalence, causes, and prognosis of sensorineural hearing loss (SNHL) occurring in children with Kawasaki disease.
Patients who were diagnosed with Kawasaki disease and received inpatient treatment in the Pediatrics Department at one of three university hospitals in Daegu city from February 2012 to September 2012 were enrolled in the study. The clinical features, hematological results, echocardiography results, audiometry results, and aspirin and salicylic acid serum levels of the patients were evaluated.
Of the 59 children enrolled in the study, three showed mild bilateral SNHL on audiometry tests conducted after 48 hours of defervescence; these patients demonstrated normal patterns of recovery on follow-up tests 8 weeks later. Aspirin serum levels were significantly higher in the SNHL group after 48 hours of afebrile condition with high dose aspirin intake (P=0.034). However, no significant differences were found in other laboratory tests or for fever duration (P>0.05). Upon echocardiography, coronary artery abnormality was observed in 9 cases, but none of these patients showed hearing loss.
The results indicate that SNHL in children with Kawasaki disease might occur during treatment of the acute phase; this SNHL usually involves mild bilateral hearing loss and recovers naturally. However, this study suggests that determination of the causes and clinical implications of hearing loss in Kawasaki disease requires long-term follow-up studies with more cases.
Kawasaki disease; Sensorineural hearing loss; Aspirin
Meningitis is among the most common infections affecting the central nervous system. It can be difficult to determine the exact pathogen responsible for the infection and patients are often treated with empiric antibiotics. This study was conducted to identify the most common clinical characteristics of enteroviral meningitis in children and evaluate the diagnostic efficacy of reverse transcriptase-polymerase chain reaction (RT-PCR) for early detection of an enterovirus.
We analyzed the medical records of children admitted to Korea University Medical Center and diagnosed with meningitis on the basis of cerebrospinal fluid (CSF) analysis and RT-PCR from CSF and other samples from January 2010 to August 2013.
A total of 333 patients were enrolled and classified into four groups based on diagnosis: enteroviral meningitis (n=110), bacterial meningitis (n=23), other viral meningitis (n=36), and unknown etiology (n=164). Patients with bacterial meningitis were younger than those in the other groups (P<0.001). Pleocytosis in CSF was similar across all groups. Of patients in the enteroviral meningitis group, 92.7% were diagnosed based on RT-PCR findings. Mean length of hospital stay for patients with enteroviral meningitis was 6.08 days, which was significantly shorter than that for patients with meningitis of bacterial etiology (19.73 days, P<0.001).
Diagnosis of enteroviral meningitis before viral culture results are available is possible using RT-PCR. Accurate diagnosis reduces the length of hospital stay and helps to avoid unnecessary empiric antibiotic treatment.
Meningitis; Enterovirus; Reverse transcriptase polymerase chain reaction; Cerebrospinal fluid
Diphyllobothrium latum infection in humans is not common in Republic of Korea. We report a case of fish tapeworm infection in a 10-year-old boy after ingestion of raw perch about 8 months ago. The patient complained of recurrent abdominal pain and watery diarrhea. A tapeworm, 85 cm in length, without scolex and neck, was spontaneously discharged in the feces of the patient. The patient was treated with 15-mg/kg single dose praziquantel, and follow-up stool examination was negative after one month. There was no evidence of relapse during the next six months.
Diphyllobothrium latum; Abdominal pain; Diarrhea
Incomplete Kawasaki disease (KD) is frequently associated with delayed diagnosis and treatment. Delayed diagnosis leads to increasing risk of coronary artery aneurysm. Anterior uveitis is an important ocular sign of KD. The purpose of this study was to assess differences in laboratory findings, including echocardiographic measurements, clinical characteristics such as fever duration and treatment responses between KD patients with and those without uveitis.
We conducted a prospective study with 110 KD patients from January 2008 to June 2013. The study group (n=32, KD with uveitis) was compared with the control group (n=78, KD without uveitis). Laboratory data were obtained from each patient including complete blood count (CBC), erythrocyte sedimentation rate (ESR), platelet count, and level of alanine aminotransferase, aspartate aminotransferase, serum total protein, albumin, C-reactive protein (CRP), and N-terminal probrain natriuretic peptide (NT-pro BNP). Echocardiographic measurements and intravenous immunoglobulin responses were compared between the two groups.
The incidence of uveitis was 29.0%. Neutrophil counts and patient age were higher in the uveitis group than in the control group. ESR and CRP level were slightly increased in the uveitis group compared with the control group, but the difference between the two groups was not significant. No significant differences in coronary arterial complication and treatment responses were observed between the two groups.
Uveitis is an important ocular sign in the diagnosis of incomplete KD. It is significantly associated with patient age and neutrophil count.
Kawasaki disease; Uveitis; Brain natriuretic peptide
To evaluate the outcomes and prognostic factors in children with extracranial germ cell tumors (GCTs) treated at a single institution.
Sixty-six children diagnosed with extracranial GCTs between 1996 and 2012 were included in the study. Primary treatment was surgical excision, followed by six cycles of cisplatin-based chemotherapy. The survival rates were compared according to the International Germ Cell Cancer Cooperative Group classification used for GCTs in adults to validate the classification guidelines for GCTs in children.
The median patient age was 4.4 years. In 34 patients (51.5%), the primary tumor site was the gonad. Extragonadal GCTs were detected in 32 patients. The 5-year overall survival and event-free survival (EFS) were 92.0%±3.5% and 90.4%±3.7%, respectively. In univariate analysis, tumor histology, metastasis, and elevated alpha-fetoprotein were not prognostic factors in children with extracranial GCTs. However, EFS was poorer in patients with mediastinal disease (n=12, 66.7%±13.6 %) than in those with nonmediastinal disease (n=54, 96.0%±2.8%) (P=0.001). The 5-year EFS was lower in patients older than 10 years, (n=21, 80.0%±8.9%) compared with those younger than 10 years (n=45, 95.2%±3.3%) (P=0.04). Multivariate analysis identified the mediastinal tumor site as the only independent prognostic factor.
The prognosis of children with extracranial GCTs was favorable. However, nongerminomatous mediastinal tumors were associated with poor survival in children. Further research is needed to improve the prognosis of children with malignant mediastinal GCTs.
Germ cell and embryonal neoplasms; Child; Prognosis; Mediastinum; Survival
IgA nephropathy usually presents as asymptomatic microscopic hematuria or proteinuria or episodic gross hematuria after upper respiratory infection. It is an uncommon cause of end-stage renal failure in childhood. Pulmonary hemorrhage associated with IgA nephropathy is an unusual life-threatening manifestation in pediatric patients and is usually treated with aggressive immunosuppression. Pulmonary hemorrhage and renal failure usually occur concurrently, and the pulmonary manifestation is believed to be caused by the same immune process. We present the case of a 14-year-old patient with IgA nephropathy who had already progressed to end-stage renal failure in spite of immunosuppression and presented with pulmonary hemorrhage during oral prednisone treatment. His lung disease was comparable to diffuse alveolar hemorrhage and was successfully treated with plasmapheresis followed by oral prednisone. This case suggests that pulmonary hemorrhage may develop independently of renal manifestation, and that plasmapheresis should be considered as adjunctive therapy to immunosuppressive medication for treating IgA nephropathy with pulmonary hemorrhage.
Plasmapheresis; Pulmonary hemorrhage; IgA nephropathy
Inflammatory bowel disease (IBD) is a chronic inflammatory disease mainly affecting the gastrointestinal tract. The incidence of the disease is rapidly increasing worldwide, and a number of patients are diagnosed during their childhood or adolescence. Aside from controlling the gastrointestinal symptoms, nutritional aspects such as growth, bone mineral density, anemia, micronutrient deficiency, hair loss, and diet should also be closely monitored and managed by the pediatric IBD team especially since the patients are in the development phase.
Inflammatory bowel disease; Nutrition status; Child
In 2004, the American Heart Association (AHA) had published an algorithm for the diagnosis of incomplete Kawasaki disease (KD). The aim of the present study was to investigate characteristics of supplemental laboratory criteria in this algorithm.
We retrospectively examined the medical records of 355 patients with KD who were treated with intravenous immunoglobulin (IVIG) during the acute phase of the disease. Laboratory data were obtained before the initial IVIG administration and up to 10 days after fever onset. In 106 patients, laboratory testing was performed more than twice.
The AHA supplemental laboratory criteria were fulfilled in 90 patients (25.4%), and the frequency of laboratory examination (odds ratio [OR], 1.981; 95% confidence interval [CI], 1.391-2.821; P<0.001) was a significant predictor of it. The fulfillment of AHA supplemental laboratory criteria was significantly associated with refractoriness to the initial IVIG administration (OR, 2.388; 95% CI, 1.182-4.826; P=0.013) and dilatation of coronary arteries (OR, 2.776; 95% CI, 1.519-5.074; P=0.001).
Repeated laboratory testing increased the rate of fulfillment of the AHA supplemental laboratory criteria in children with KD.
Kawasaki disease; Incomplete Kawasaki disease; Laboratory examinations; Diagnosis of Kawasaki disease
Croup is a common pediatric respiratory illness with symptoms of varying severity. Moreover, epiglottitis is a rare disease that can rapidly progress to life-threatening airway obstruction. Although the clinical course and treatments differ between croup and epiglottitis, they are difficult to differentiate on presentation. We aimed to compare the clinical characteristics of croup and epiglottitis in Emergency Department patients.
The 2012 National Emergency Department Information System database of 146 Korean Emergency Departments was used to investigate patients aged ≤18 years presenting with croup or epiglottitis.
We analyzed 19,374 croup patients and 236 epiglottitis patients. The male:female sex ratios were 1.9:1 and 2.3:1 and mean ages were 2.2±2.0 and 5.6±5.8 years, respectively. The peak incidence of croup was observed in July and that of epiglottitis was observed in May. The hospitalization rate was lower in croup than in epiglottitis patients, and the proportion of patients treated in the intensive care unit was lower among croup patients. The 3 most common chief complaints in both croup and epiglottitis patients were cough, fever, and dyspnea. Epiglottitis patients experienced dyspnea, sore throat, and vomiting more often than croup patients (P<0.05).
Both groups had similar sex ratios, arrival times, 3 most common chief complaints, and 5 most common comorbidities. Epiglottitis patients had a lower incidence rate, higher mean age of onset, and higher hospitalization rate and experienced dyspnea, sore throat, and vomiting more often than croup patients. Our results may help in the differential diagnosis of croup and epiglottitis.
Croup; Epiglottitis; Emergency Department
Alagille syndrome is a complex hereditary disorder that is associated with cardiac, hepatic, skeletal, ocular, and facial abnormalities. Mutations in the Notch signaling pathway, such as in JAG1 and NOTCH2, play a key role in embryonic development. A cardiac or hepatic presentation is a critical factor for determining the prognosis.
We conducted a retrospective study of 41 patients with Alagille syndrome or a JAG1 mutation between 1983 and 2013.
The first presentations were jaundice, murmur, cyanosis, and small bowel obstruction at a median age of 1.0 months (range, 0-24 months). The JAG1 mutation was found in 27 of the 28 genetically-tested patients. Cardiovascular anomalies were identified in 36 patients, chronic cholestasis was identified in 34, and liver transplantation was performed in 9. There was no significant correlation between the severity of the liver and cardiac diseases. The most common cardiovascular anomaly was peripheral pulmonary stenosis (83.3%), with 13 patients having significant hemodynamic derangement and 12 undergoing surgical repair. A total bilirubin level of >15 mg/dL with a complex surgical procedure increased the surgical mortality (P=0.022). Eight patients died after a median period of 2.67 years (range, 0.33-15 years). The groups with fetal presentation and with combined severe liver and heart disease had the poorest survival (P<0.001).
The group with combined severe liver and heart disease had the poorest survival, and a multidisciplinary approach is necessary to improve the outcome.
Alagille syndrome; Cardiovascular diseases; Pulmonary valve stenosis; Cholestasis
Tularemia is a potentially severe zoonotic disease caused by Francisella tularensis. A lack of awareness about tularemia can be embarrassing and could result in delayed treatment because of improper diagnosis. The diagnosis of tularemia is difficult, because the infections are rare and the clinical spectrum is broad. As only 1 adult case has been reported in Korea thus far, pediatricians in Korea may be unfamiliar with tularemia. We report our experience with a 14-year-old male adolescent with tularemia who presented with atypical pneumonia and possible infective endocarditis. Although the infectivity and mortality rates for tularemia are very high if left untreated, we did not suspect tularemia in this case until the incidental isolation of F. tularensis. The present case suggests that clinicians in Korea should be more aware of tularemia. This case also suggests that tularemia should be considered in undetermined cases of atypical pneumonia or acute febrile illness without local signs.
Francisella tularensis; Pneumonia; Endocarditis; Pediatrics; Korea