· We suggest offering long-term macrolides to children with noncystic fibrosis bronchiectasis with frequent exacerbations (conditional recommendation, moderate quality of evidence). · We do not recommend the routine use of mucolytic agents, inhaled corticosteroids, or nonsteroidal anti-inflammatory drugs to prevent exacerbation of bronchiectasis in children (inconclusive, very low quality of evidence). · We recommend the use of nebulized hypertonic saline to prevent exacerbations and improve the lung function of children with noncystic fibrosis bronchiectasis (weak recommendation, moderate quality of evidence). |
Question: Is polycystic ovary syndrome (PCOS) a risk factor for nonalcoholic fatty liver disease (NAFLD) in adolescents? Finding: The frequency of NAFLD did not increase in adolescents with PCOS. However, hyperandrogenemia was a risk factor for NAFLD. Meaning: Adolescents with PCOS and hyperandrogenemia should be closely monitored for hepatic steatosis. |
Question: Have polycystic ovary syndrome (PCOS) increased risk of having an offspring with attention-deficit/hyperactivity disorder (ADHD)? Finding: Six articles (3 cohort and 3 case-control studies; 401,413 total ADHD cases) met the study criteria. Maternal PCOS was associated with an increased risk of ADHD in the offspring based on odds ratio (OR) and relative ratio (RR) (OR, 1.42; 95% confidence interval [CI], 1.27–1.57) and (RR, 1.43; 95% CI, 1.35–1.51), respectively. Meaning: Our study showed that maternal PCOS is a risk factor for ADHD. |
Background: β-Thalassemias are characterized by the presence of mutations in the globin gene that result in the absence or reduced synthesis of β-globin chains of the hemoglobin tetramer. Several studies have reported increased oxidative stress in β-thalassemia major (β-TM) patients. N-acetylcysteine (NAC), a derivative of L-cysteine amino acid, is commonly used as a mucolytic drug. Numerous studies have reported efficient... |
We report a unique case of intestinal duplication detected on posterior reversible encephalopathy syndrome (PRES) in a 13-year-old girl. She was admitted to the pediatric Emergency Department because of generalized seizures. Radiological assessment revealed a large, well-defined, thick-walled cystic lesion in the mid abdomen, suggestive of duplication cyst associated to a PRES. Exploration confirmed the diagnosis of ileal duplication cyst,... |
Congenital pulmonary airway malformation (CPAM), previously known as congenital cystic adenomatoid malformation, is a rare developmental lung abnormality associated with rhabdomyosarcoma, pleuropulmonary blastoma, and mucinous adenocarcinoma of the lung. We report an unusual case of a 10-day-old male newborn with a left lower lobe pulmonary cyst who underwent lobectomy, which revealed type II CPAM complicated by multifocal mucinous adenocarcinoma. KRAS... |
We identified pediatric patients younger than 19 years with microbiologically confirmed |
Choledochal cyst is a dilation that encloses the intrahepatic or both extra- and intrahepatic portions of the biliary ducts. Postnatally, ultrasonography is the initial diagnostic modality of choice, allowing for precise measurements of intra- or extrahepatic duct dilatation and identification of stones and sludge. Symptoms depend on the age at presentation. Common bile duct malformations should be considered as a... |
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... |
Hemorrhagic cystitis is a common stem cell transplantation-related complication. The incidence of early-onset hemorrhagic cystitis, which is related to the pretransplant conditioning regimen, has decreased with the concomitant use of mesna and hyperhydration. However, late-onset hemorrhagic cystitis, which is usually caused by the BK virus, continues to develop. Although the BK virus is the most common pathogenic microorganism of poststem... |
Cystic fibrosis (CF) is a genetic disease with autosomal recessive inheritance and is common in Caucasian people. The prevalence of this disease is between 1/2,000 and 1/3,500 live births, and the incidence varies between populations. Although the CF transmembrane conductance regulator gene is expressed in the kidneys, renal involvement is rare. With advances in the treatment of CF, life expectancy... |
Hyperhomocysteinemia is known as a risk factor for atherosclerosis. Preclinical arteriosclerosis is noted and premature atherosclerosis is known to be accelerated in Kawasaki disease (KD) patients. Genetic polymorphisms in the 5,10-methylenetetrahydrofolate reductase ( |
This study evaluated the clinical manifestations of and risk factors for pituitary insufficiency in children and adolescents with Rathke's cleft cysts. Forty-four patients with Rathke's cleft cysts younger than 19 years who visited Seoul National University Children's Hospital between January 1995 and September 2009 were enrolled. Rathke's cleft cysts were confirmed histologically through an operation in 15 patients and by brain... |
Orphan lung diseases are defined as lung diseases with a prevalence of 1 or less in 2,000 individuals. Despite an increase in the numbers of patients with such diseases, few studies on Korean children have appeared. To obtain epidemiologic and demographic data on these diseases, we systematically reviewed reports on pediatric orphan lung diseases in Korea over the last 50... |
Purpose : The neonatal screening test for homocystinuria primarily measures methionine by using a dried blood specimen. We investigated the incidence and clinical manifestations of homocystinuria, isolated hypermethioninemia, and transient hypermethioninemia among patients with hypermethioninemia on a neonatal screening test. Methods : We performed a retrospective study of 58 patients transferred to Shoonchunhyang Hospital because of hypermethioninemia on a neonatal... |
Lymphangioma is a rare benign congenital tumor of the lymphatic system, which is commonly diagnosed before 2 years of age. In the natronal report, cystic lymphangioma was usually reported as a huge translucent mass located in the head and neck area. It's occurrence in retropharyngeal space with respiratory obstruction and swallowing difficulty in neonate is extremely rare and postoperative nasopharyngeal... |
Purpose:Glomerular filtration rate (GFR) is a fundamental parameter in assessing renal function and predicting the progression of chronic renal disease. Because the use of serum creatinine has several disadvantages, many studies have investigated the use of cystatin C for estimating GFR. We compared creatinine clearance and GFR with formulas using serum creatinine and cystatin C. Methods:We retrospectively analyzed 211 patients... |
Purpose : Cysteinyl leukotrienes are important proinflammatory mediators in asthma. Recently, it was suggested that a promoter polymorphism in the genes encoding for leukotriene C4 synthase (LTC4S), a key enzyme in the leukotriene synthetic pathway, and cysteinyl leukotriene receptor 1 (CysLTR1) might be associated with aspirin-intolerant asthma. We investigated whether polymorphisms in LTC4S and CysLTR1 genes or their interactions were... |
Duplication cysts are rare congenital malformations, that may be detected anywhere along the alimentary tract, and they may communicate with the intestinal tract. Cystic duplication of the cecum is especially rare. About 80% of these cases are detected in the first 2 years of life as a result of an acute intestinal obstruction, which manifests as vomiting, recurrent abdominal pain,... |
Megalencephalic leukoencephalopathy with subcortical cysts (MLC) is a rare white matter disorder, first described in the early 1990s. The brain in patients with MLC appears swollen on MRI, with diffuse white matter abnormalities; in addition, there is an invariable presence of subcortical cysts, primarily in the anterior temporal region sparing the deep white matter, basal ganglia, thalami, and cerebellum. Patients... |
Vallecular cyst is an uncommon but potentially dangerous condition causing stridor and has been associated with sudden airway obstruction resulting in death due to its anatomical location in neonates. It may also present with various degrees of feeding problems resulting in failure to thrive. When a vallecular cyst is suspected clinically, endoscopic laryngoscopy is necessary to visualize it. Other conditions... |
Meconium ileus (MI) is the earliest clinical manifestation of cystic fibrosis (CF) in infants. It arises from the intraluminal accumulation of highly viscid, protein-rich meconium, typically present in the terminal ileum as a neonatal intestinal obstruction. Therefore, the clinical symptoms include abdominal distension, bilious vomiting and delayed passage of meconium. CF is caused by mutations in the transmembrane conductance regulator... |
Basal cell nevus syndrome (BCNS), also known as Gorlin syndrome, is a rare autosomal dominant disorder characterized by variable manifestations, including multiple basal cell carcinomas, odontogenic keratocysts of the jaw, skeletal anomalies including scoliosis and bifid ribs, palmar and plantar pits, calcification of the falx cerebri, and biparietal frontal bossing. We report a case of a 9-year-old boy with the... |
Purpose : Homocysteine is a strong and independent risk factor for cardiovascular disease. The deleterious effects of homocysteine included endothelial dysfunction, arterial intimal-medial thickening, wall stiffness and procoagulant activity. However, the precise mechanism responsible for homocysteine release in children with coronary artery disease is still unknown. The purpose of this study was to investigate serum homocysteine and tumor necrosis factor(TNF)-α... |
A rare case is described of an intrathyroidal branchial cleft-like cyst in neonate. The patient was a newborn girl with a mass in the left lateral neck. The ultrasonography and computed tomography revealed a cystic lesion in the left thyroid. The lesion was enucleated surgically from the thyroid. Histologically, the cyst was lined by squamous or columnar epithelium and contained... |
Multicystic dysplastic kidney and congenital cystic adenomatoid malformation of the lung are independent disorders, but both result from abnormal morphogenesis during embryogenesis. Congenital cystic adenomatoid malformation of the lung is associated with renal anomalies as well as other extrapulmonary anomalies and almost all cases with these anomalies are stillborn. We report a case of a 21-month-old male who was... |
Purpose : Because voiding cystourethrography(VCUG) is an invasive method, we studied whether VCUG could be postponed through evaluation of alternative non-invasive tests including renal ultrasonography and 99mTc-DMSA renal scan. Methods : We reviewed the medical records of 175 patients initially diagnosed with febrile urinary tract infection during the one year period of 1999, and compared 3-tests : renal ultrasongraphy, 99mTc-DMSA renal... |
Purpose : As the prevalence of childhood obesity is increasing, hypertension, hyperlipidemia, insulin resistance and diabetes mellitus have become problems. High homocysteine levels and low vitamin B12 supplementation are acknowledged to have a role in coronary artery disease, but there are few studies on homocysteine, insulin and vitamin B12 levels in obese children. We aimed to study whether homocysteine, insulin,... |
Purpose : Cystatin C has been proposed for the assessment of glomerular filtration rate, being more accurate than creatinine determination. We measured serum cystatin C concentrations in the pediatric population, and analysed the correlation between cystatin C and glomerular filtration rate. Methods : Cystatin C and creatinine were measured by the particle enhanced nephelometric immunoassay and Jaff method, respectively, in... |
Mesenteric cysts are one of the least common intraabdominal lesions, which are very rare in neonates. Among mesenteric cysts, a chylous cyst is the rarest one of all. This entity can be found anywhere in the gastrointestinal tract from the duodenum to the rectum. Mesenteric cysts may be asymptomatic and found on routine examinations, or can present with symptoms such... |