Minimal dose of hematopoietic stem cell transplantation without myelosuppressive conditioning for T-B+NK- severe combined immunodeficiency.

We reviewed the medical records of five patients with T-B+NK- severe combined immunodeficiency (SCID) who received minimal dose allogeneic hematopoietic cell transplantation (HCT) (total nucleated cell count (TNC) lower than 1.0 × 10/kg). Patients were administered a median of 5.

Needs for Increased Awareness of Gastrointestinal Manifestations in Patients With Human Inborn Errors of Immunity.

The gastrointestinal (GI) tract is frequently affected by inborn errors of immunity (IEI), and GI manifestations can be present in IEI patients before a diagnosis is confirmed. We aimed to investigate clinical features, endoscopic and histopathologic findings in IEI patients. This was a retrospective cohort study conducted from 1995 to 2020.

Effect of prenatal antioxidant intake on infants' respiratory infection is modified by a CD14 polymorphism.

Background: Prenatal maternal diet may influence disease susceptibility in offspring with specific genetic backgrounds. We hypothesized that interactions between prenatal antioxidant intake and polymorphisms in immunity genes influence respiratory tract infection (RTI) susceptibility in infants at 12 months of age.

Methods: This study included 550 infants.

The effect of perinatal anxiety on bronchiolitis is influenced by polymorphisms in ROS-related genes.

Background: Exposure to perinatal anxiety affects disease susceptibility in offspring but studies on the association between perinatal anxiety and gene polymorphisms are lacking. This study aimed to elucidate the interaction between perinatal anxiety and polymorphisms in antioxidant defense and innate immunity genes on the development of respiratory tract infections (RTIs) during early infancy.

Methods: Trait anxiety levels in 440 women were assessed by the State-Trait Anxiety Inventory during late gestation.

Additive effect between IL-13 polymorphism and cesarean section delivery/prenatal antibiotics use on atopic dermatitis: a birth cohort study (COCOA).

Background: Although cesarean delivery and prenatal exposure to antibiotics are likely to affect the gut microbiome in infancy, their effect on the development of atopic dermatitis (AD) in infancy is unclear. The influence of individual genotypes on these relationships is also unclear. To evaluate with a prospective birth cohort study whether cesarean section, prenatal exposure to antibiotics, and susceptible genotypes act additively to promote the development of AD in infancy.

Allergenicity of an enzymatic hydrolysate of soybean 2S protein.

Background: This study was performed to examine how the characteristics of soybean 2S protein influence allergenicity after enzymatic hydrolysis. Soybean 2S protein was extracted and enzymatic hydrolysis was performed using pepsin and chymotrypsin. Allergenicity was observed using soybean-sensitive patients' sera.

Prenatal maternal depression is associated with low birth weight through shorter gestational age in term infants in Korea.

Background: Maternal prenatal depression is associated with lower offspring birth weight, yet the impact of gestational age on this association remains inadequately understood.

Aims: We aimed to investigate the effect of prenatal depression on low birth weight, gestational age, and weight for gestational age at term.

Study Design: Prospective cohort study.

Association between maternal characteristics and neonatal birth weight in a Korean population living in the Seoul metropolitan area, Korea: a birth cohort study (COCOA).

Previous studies suggest that maternal characteristics may be associated with neonatal outcomes. However, the influence of maternal characteristics on birth weight (BW) has not been adequately determined in Korean populations. We investigated associations between maternal characteristics and BW in a sample of 813 Korean women living in the Seoul metropolitan area, Korea recruited using data from the prospective hospital-based COhort for Childhood Origin of Asthma and allergic diseases (COCOA) between 2007 and 2011.

Retropharyngeal abscess coinfected with Staphylococcus aureus and Mycobacterium tuberculosis after rhinoviral infection in a 1-month-old infant.

A retropharyngeal abscess is a rare disease entity in young infants but can develop after nasopharyngeal viral infection. Group B Streptococcus and Staphylococcus aureus are the most common pathogens in young infants, however, Mycobacterium tuberculosis is very rare. We report the case of retropharyngeal abscess and coinfection with S.

Cord blood cellular proliferative response as a predictive factor for atopic dermatitis at 12 months.

Since the risk of developing allergic disease increases in individuals exposed to allergens previously, even during the neonatal period, the immunologic status of a fetus may be important in the subsequent development of allergy. We evaluated the fetal factors to predict atopic dermatitis (AD) at 12 months in 412 infants of a COhort for Childhood Origin of Asthma and Allergic Diseases (COCOA) in the general Korean population. Cord blood mononuclear cells (CBMCs) were stimulated with ovalbumin and phytohemagglutinin and cellular proliferative response and concentrations of interleukin-13 and interferon-γ, were measured.

The standard range of peak expiratory flow rates of Korean children.

Background: The importance in asthma management of monitoring of peak expiratory flow rate (PEFR) has been emphasized.

Objective: For effective asthma management in Korean children, we established reference ranges for the PEFR in children 4-18 years of age.

Methods: The Mini Wright Peak Flow Meter (MWPFM) and spirometry were used in this study.

Heterogeneous spectrum of CFTR gene mutations in Korean patients with cystic fibrosis.

Background: Cystic fibrosis (CF) is one of the most common hereditary disorders among Caucasians. The most common mutations of the cystic fibrosis transmembrane conductance regulator (CFTR) gene have been well established among Caucasian populations. In Koreans, however, there are very few cases of genetically confirmed CF thus far, and the spectrum of mutations seems quite different from that observed in Caucasians.

Oral food challenges in children.

Many patients assume that allergic reactions against foods are responsible for triggering or worsening their allergic symptoms. Therefore, it is important to identify patients who would benefit from an elimination diet, while avoiding unnecessary dietary restrictions. The diagnosis of food allergy depends on the thorough review of the patients's medical history, results of supplemented trials of dietary elimination, and in vivo and in vitro tests for measuring specific IgE levels.

Effect of probiotic mix (Bifidobacterium bifidum, Bifidobacterium lactis, Lactobacillus acidophilus) in the primary prevention of eczema: a double-blind, randomized, placebo-controlled trial.

Controversy exists regarding the preventive effect of probiotics on the development of eczema or atopic dermatitis. We investigated whether supplementation of probiotics prevents the development of eczema in infants at high risk. In a randomized, double-blind, placebo-controlled trial, 112 pregnant women with a family history of allergic diseases received a once-daily supplement, either a mixture of Bifidobacterium bifidum BGN4, B.

[Standardized sweat chloride analysis for the diagnosis of cystic fibrosis in Korea].

Background: Cystic fibrosis is a chronic progressive autosomal recessive disorder caused by the CFTR gene mutations. It is quite common in Caucasians, but very rare in Asians. Sweat chloride test is known to be a screening test for the cystic fibrosis due to the fact that electrolyte levels in sweat are elevated in patients.

Treatment of congenital cystic adenomatoid malformation: should lobectomy always be performed?

Background: Although parenchyma-saving resection makes it possible to preserve the lung parenchyma, most surgeons are reluctant to perform it for congenital cystic adenomatoid malformation (CCAM) because it could also result in recurrent pulmonary infection or residual lesion. This study compared the early and late postoperative outcomes according to the extent of resection in CCAM patients to determine if the extent of resection would influence the short- and long-term results.

Methods: Between 1995 and 2006, 45 patients underwent surgical resection for CCAM.

Characterization of Staphylococcus aureus nasal carriage from children attending an outpatient clinic in Seoul, Korea.

Nasal swabs were collected to isolate S. aureus in 296 children, who visited the pediatrics department with a variety of symptoms. Staphylococcus aureus was isolated from 95 children (32.

Pulmonary recovery after rigid bronchoscopic retrieval of airway foreign body.

Objective: The aim of this study was to determine how long postoperative pulmonary care is needed in patients after rigid bronchoscopic retrieval of foreign body from the airway and to identify the factors affecting pulmonary recovery.

Materials And Method: A retrospective review of 98 pediatric patients who had foreign body removed from the airway by rigid bronchoscopic procedure was conducted. After the removal of the foreign body, the time required for complete clinical and radiologic pulmonary recovery was reviewed for each subject.

High body mass index and dietary pattern are associated with childhood asthma.

The increasing prevalence of asthma has coincided with an increase of body mass index (BMI) in both children and adults. We investigated the relationship between BMI and the symptom prevalence of asthma and the possible influences of dietary pattern. This was a community-based, cross-sectional study of 24,260 school children aged 6-12 years.

X-linked Opitz G/BBB syndrome: identification of a novel mutation and prenatal diagnosis in a Korean family.

X-linked Opitz G/BBB syndrome (XLOS; MIM 300000) is a rare multiple congenital anomaly disorder that is characterized by facial anomalies, laryngeal/tracheal/esophageal defects and genitourinary abnormalities. XLOS is caused by mutations in the MID1 gene which encodes a microtubule-associated RING-Bbox-Coiled-coil (RBCC) protein. We recently found a four-year Korean male patient who was suspected of having XLOS.

Complications of tracheotomy in patients with mucopolysaccharidoses type II (Hunter syndrome).

Objective: To investigate the complication rate of tracheotomy in patients with mucopolysacchridoses (MPS) type II (Hunter syndrome).

Materials And Methods: From 2004 to 2005, seven tracheotomy procedures were performed for the airway management in three patients with MPS type II. The complications for each procedure were analyzed, which included the stomal narrowing, granulation formation, infrastomal tracheal stenosis, and wound infection.

Delayed bronchostenosis after blunt chest trauma in children: CT and pathologic findings.

Tracheobronchial disruption is an uncommon injury associated with blunt chest trauma. We report CT features and pathologic findings of two pediatric cases in which a bronchial injury was unnoticed initially but was diagnosed later by appearance of delayed bronchostenosis with distal atelectasis after blunt chest trauma in recent motor vehicle accidents. Pathologically, obliteration of the bronchial lumen was caused by dense fibrous overgrowth and granulation tissue.

Complete surgical resection of inflammatory myofibroblastic tumor with carinal reconstruction in a 4-year-old boy.

Inflammatory myofibroblastic tumor of the carina and the main bronchus is a rare tumor. The authors report here on a case of a 4-year-old boy with an inflammatory myofibroblastic tumor at the carina and extending to the left main bronchus. He presented with fever and a cough of 2 months' duration.