PURPOSE: The purpose of this study was to identify the causes, symptoms, and complications of hypoproteinemia to prevent hypoproteinemia and provide appropriate treatment to children with atopic dermatitis. METHODS: Children diagnosed with atopic dermatitis with hypoproteinemia and/or hypoalbuminemia were retrospectively reviewed. The patients’ medical records, including family history, weight, symptoms, treatment, complications, and laboratory test results for allergies and skin cultures, were examined. RESULTS: Twenty-six patients (24 boys) were enrolled. Seven cases had growth retardation; 7, keratoconjunctivitis; 6, aural discharges; 5, eczema herpeticum; 4, gastrointestinal tract symptoms; and 2, developmental delays. In 21 cases, topical steroids were not used. According to the blood test results, the median values of each parameter were elevated: total IgE, 1,864 U/mL; egg white-specific IgE, 76.5 kU(A)/L; milk IgE, 20.5 kU(A)/L; peanut IgE, 30 kU(A)/L; eosinophil count, 5,810/μL; eosinophil cationic protein, 93.45 μg/L; and platelet count, 666.5×10³/μL. Serum albumin and total protein levels decreased to 2.7 g/dL and 4.25 g/dL, respectively. Regarding electrolyte abnormality, 10 patients had hyponatremia, and 12, hyperkalemia. Systemic antibiotics were used to treat all cases, and an antiviral agent was used in 12 patients. Electrolyte correction was performed in 8 patients. CONCLUSION: Hypoproteinemia accompanying atopic dermatitis is common in infants younger than 1 year and may occur because of topical steroid treatment continuously being declined or because of eczema herpeticum. It may be accompanied by growth retardation, keratoconjunctivitis, aural discharge, and eczema herpeticum and can be managed through skin care and topical steroid application without intravenous albumin infusion.
Anti-Bacterial Agents ; Arachis ; Child ; Dermatitis, Atopic* ; Eosinophil Cationic Protein ; Eosinophils ; Gastrointestinal Tract ; Hematologic Tests ; Humans ; Hyperkalemia ; Hypersensitivity ; Hypoalbuminemia ; Hyponatremia ; Hypoproteinemia* ; Immunoglobulin E ; Infant ; Kaposi Varicelliform Eruption ; Keratoconjunctivitis ; Medical Records ; Milk ; Ovum ; Platelet Count ; Retrospective Studies ; Serum Albumin ; Skin ; Skin Care ; Steroids

Approximately 13–20% of infants with milk allergies concurrently exhibit beef allergies. Here, we report a 24-month-old infant who exhibited both pork and beef allergies, concurrently with a milk allergy. The infant's laboratory test results were: 3.73 ISU-E (ISAC standardized unit for IgE) for cow milk β-lactoglobulin, 23.8 ISU-E for casein, 12.8 ISU-E for cow milk Bos d 6 of serum albumin, and 4.85 ISU-E for cat Fel d 2. This case report summarizes an infant patient diagnosed with a meat allergy that was associated with cow's milk allergy, using ImmunoCAP ISAC®. Not only ImmunoCAP ISAC® but also immunocap can be used to diagnose milk allergy and meat allergy at the same time, immunocap testing for component antigen is rare. ImmunoCAP ISAC® is used to diagnose these allergies in our case study, as it has advantage that only 1ml of blood is needed to run various component antigen tests.
Anesthesia, General ; Animals ; Atrioventricular Block ; Bradycardia ; Caseins ; Cats ; Child, Preschool ; Humans ; Hypersensitivity ; Infant ; Meat ; Milk Hypersensitivity ; Milk ; Red Meat ; Serum Albumin

Mycoplasma pneumoniae is the most common bacterial strain causing atypical pneumonia in children and adolencents. Although it is known to cause mild symptoms, it can also cause severe pulmonary or extrapulmonary complications in rare cases. Necrotizing pneumonia (NP) is often reported as a complication of Streptococcus pneumoniae and is very rarely caused by M. pneumoniae. We report a case in which a 5-year-old boy was diagnosed with lobar pneumonia with symptoms that aggravated even with macrolide antibiotic treatment. Anti-mycoplasma pneumoniae Ig-M test yielded high values, and direct polymerase chain reaction results were also positive. NP caused by M. pneumoniae was confirmed on computed tomography. After treatment involving tosufloxacin and systemic steroid, the lesion decreased in size and improved gradually when followed-up for more than 1 year. The patient did not have any predisposing or risk factors for NP.
Child ; Child, Preschool ; Humans ; Male ; Mycoplasma pneumoniae ; Mycoplasma ; Pneumonia ; Pneumonia, Mycoplasma ; Polymerase Chain Reaction ; Risk Factors ; Streptococcus pneumoniae

Hepatic osteodystrophy is frequent complication in patients with chronic liver disease, particularly with chronic cholestasis. We report a male infant with congenital hepatoblastoma, who had osteodystrophy complicated by multiple bone fractures despite adequate supplementation of fat-soluble vitamins including vitamin D. He was born by Caesarean section because of a 7 cm–sized abdominal mass detected by prenatal ultrasonography. The pathologic diagnosis was hepatoblastoma, PRETEXT staging III or IV. Whole body bone scan at the time of diagnosis showed no abnormal uptake. Oral vitamin D3 of 2,000 IU/day was administered with other fat-soluble vitamins. Serum direct bilirubin level gradually increased up to 28.9 mg/dL at postnatal 6 days and was above 5 mg/dL until 110 days of age. Bony changes consistent with rickets became apparent in left proximal humerus since 48 days of age, and multiple bone fractures developed thereafter. With resolving cholestasis by chemotherapy, his bony lesions improved gradually after add-on treatment of bisphosphonate and parenteral administration of vitamin D with calcium. High level of suspicion and prevention of osteodystrophy is needed in patients with hepatoblastoma, especially when cholestasis persists.
Bilirubin ; Calcium ; Cesarean Section ; Cholecalciferol ; Cholestasis ; Diagnosis ; Drug Therapy ; Female ; Fractures, Bone ; Hepatoblastoma ; Humans ; Humerus ; Infant ; Liver Diseases ; Male ; Pregnancy ; Rickets ; Ultrasonography, Prenatal ; Vitamin D ; Vitamins

The vertebrate neuromuscular junction (NMJ) is considered as a “tripartite synapse” consisting of a motor axon terminal, a muscle endplate, and terminal Schwann cells that envelope the motor axon terminal. The neuregulin 1 (NRG1)-ErbB2 signaling pathway plays an important role in the development of the NMJ. We previously showed that Grb2-associated binder 1 (Gab1), a scaffolding mediator of receptor tyrosine kinase signaling, is required for NRG1-induced peripheral nerve myelination. Here, we determined the role of Gab1 in the development of the NMJ using muscle-specific conditional Gab1 knockout mice. The mutant mice showed delayed postnatal maturation of the NMJ. Furthermore, the selective loss of the gab1 gene in terminal Schwann cells produced delayed synaptic elimination with abnormal morphology of the motor endplate, suggesting that Gab1 in both muscles and terminal Schwann cells is required for proper NMJ development. Gab1 in terminal Schwann cells appeared to regulate the number and process elongation of terminal Schwann cells during synaptic elimination. However, Gab2 knockout mice did not show any defects in the development of the NMJ. Considering the role of Gab1 in postnatal peripheral nerve myelination, our findings suggest that Gab1 is a pleiotropic and important component of NRG1 signals during postnatal development of the peripheral neuromuscular system.
Animals ; Mice ; Mice, Knockout ; Motor Endplate ; Muscle, Skeletal* ; Muscles ; Myelin Sheath ; Neuregulin-1 ; Neuromuscular Junction ; Peripheral Nerves ; Presynaptic Terminals ; Protein-Tyrosine Kinases ; Schwann Cells* ; Synapses* ; Vertebrates

BACKGROUND: Burden of disease can be used to prioritize the healthcare budget allocation. We analyzed the research and development (R&D) budget of the Ministry of Health and Welfare (MOHW) in 2018 and compared the results with those of the 2015 Korean National Burden of Disease (KNBD) study. METHODS: The 2018 MOHW R&D Project integrated implementation plan was used to analyze the R&D budget of the MOHW. The budget was allocated according to the KNBD disease group and according to the budget lines. The allocated budget was compared with the economic burden and the disability adjusted life years (DALYs) in 2015. Also, for budget targets for risk factors, DALYs of attributable risk factors were compared with corresponding budgets. RESULTS: In 2018, the MOHW major R&D budget of USD 435.1 million accounted for 3% of the total government budget. Within the disease specific R&D budget, 35.9% was allocated to communicable disease groups, 64.1% to non-communicable diseases, and 0% to injury and violence. Among level 2 disease groups, neoplasm was ranked first. Among risk factors, climate change and behavioral risk were targeted for R&D. CONCLUSIONS It would be difficult to say that current R&D allocations focus to minimize the burden of disease. A mismatch was observed between the R&D budget and the burden of disease in terms of economic burden and DALYs. There was a similar finding for risk factors R&D. A novel approach for allocating government R&D funding that is based on the goal of minimizing the disease burden in the Korean population should be considered.

Pneumatosis intestinalis (PI) is a rare condition characterized by multiple pneumocysts in the submucosa or subserosa of the bowel. Here, we report a rare case of asymptomatic PI after chemotherapy induction in an 18-yr-old man with B lymphoblastic leukemia with recurrent genetic abnormalities. The patient was treated conservatively and recovered without complications. The possibility of PI should be considered as a complication during or after chemotherapy for hematologic malignancies. Conservative treatment should be considered unless there are complications, including peritonitis, bowel perforation, and severe sepsis.
Adolescent ; Hematologic Neoplasms ; Humans ; Peritonitis ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Sepsis

OBJECTIVE: Our purpose was to investigate lipid peroxide levels, total peroxyl radical-trapping antioxidative parameter (TRAP) values, and antioxidant vitamin levels in placental tissues of women with preeclampsia and to evaluate their roles in the pathophysiology of preeclampsia. STUDY DESIGN: Samples of placental tissue homogenates were obtained from 23 normal and 18 preeclamptic women between 33 and 40 weeks gestation. TNF-alpha was assayed by an enzyme-linked immunoassay. Lipid peroxide levels measured by thiobarbituric acid reaction. The TRAP values were measured by modified Wayners method. Ascorbic acid, uric acid, retinol, alpha-tocopherol, and gamma-tocopherol were measured by high performance liquid chromatography (HPLC) CoulArray detector. RESULTS: TNF-alpha levels in placental tissue homogenates of women with preeclampsia were significantly higher than that of women with preeclampsia (21.4+/-3.39 vs. 10.3+/-1.06 pg/ml, p<0.05). Lipid peroxide levels in placental tissue homogenates of women with preeclampsia were significantly higher than that of women with preeclampsia (6.65+/-0.57 vs. 4.98+/-0.37 nmol/mg protein, p<0.05). TRAP values in placental tissue homogenates of women with preeclampsia were significantly lower than that of women with normal pregnancy (0.24+/-0.02 vs. 0.31+/-0.03 mM, p<0.05). Ascorbic acid levels in placental tissue homogenates of women with preeclampsia were significantly lower than that of women with normal pregnancy (217.0+/-21.0 vs. 333.3+/-32.8 nmol/ml, p<0.05). CONCLUSION: The above results suggest that increased oxidative stress in placenta is involved in the pathophysiology of preeclampsia and ascorbic acid may act as an important preventive factor in the development of preeclampsia.
alpha-Tocopherol ; Ascorbic Acid ; Chromatography, Liquid ; Female ; gamma-Tocopherol ; Humans ; Immunoassay ; Oxidative Stress ; Placenta* ; Pre-Eclampsia* ; Pregnancy* ; Tumor Necrosis Factor-alpha ; Uric Acid ; Vitamin A ; Vitamins

BACKGROUND/AIMS: Fructose malabsorption (FM) mimics symptoms of irritable bowel syndrome (IBS), and its prevalence has increased. Diagnosing FM in IBS is challenging because of its overlap with small intestinal bacterial overgrowth (SIBO). We assessed the prevalence of FM by comparing patients with IBS with asymptomatic control individuals after excluding SIBO using the glucose hydrogen breath test (HBT). METHODS: Patients diagnosed with IBS and asymptomatic control individuals were enrolled prospectively. Dietary habits were assessed with the Food Frequency Questionnaire. After excluding SIBO, participants underwent HBTs with both 15 g and 25 g of fructose. RESULTS: Thirty-five patients with IBS and 35 age- and sex-matched asymptomatic control individuals were enrolled. The 15-g fructose HBT yielded positive results in 7 of the 35 (20.0%) patients with IBS and in 2 of 35 (5.7%) controls (P = 0.070). The 25-g fructose HBT was positive in 16 of the 35 (45.7%) patients with IBS and in 8 of the 35 (22.9%) controls (P = 0.040). Analysis of the Food Frequency Questionnaire responses showed no significant differences between the 2 groups in dietary intake, although patients with IBS showed a significantly higher mean fiber intake than controls (21.24 ± 11.35 g vs 15.87 ± 7.07 g, respectively, P = 0.040). CONCLUSIONS: The 25-g fructose HBT identified FM in a significantly higher percentage of SIBO-negative patients with IBS than in asymptomatic control individuals, suggesting that FM may correlate with IBS. Education regarding dietary control of foods containing fructose may be useful for the management of patients with IBS.
Breath Tests ; Education ; Food Habits ; Fructose ; Glucose ; Humans ; Hydrogen ; Irritable Bowel Syndrome ; Prevalence ; Prospective Studies

Primary cilia have critical roles in coordinating multiple cellular signaling pathways. Dysregulation of primary cilia is implicated in various ciliopathies. To identify specific regulators of autophagy, we screened chemical libraries and identified mefloquine, an anti-malaria medicine, as a potent regulator of primary cilia in human retinal pigmented epithelial (RPE) cells. Not only ciliated cells but also primary cilium length was increased in mefloquine-treated RPE cells. Treatment with mefloquine strongly induced the elongation of primary cilia by blocking disassembly of primary cilium. In addition, we found that autophagy was increased in mefloquine-treated cells by enhancing autophagic flux. Both chemical and genetic inhibition of autophagy suppressed ciliogenesis in mefloquine-treated RPE cells. Taken together, these results suggest that autophagy induced by mefloquine positively regulates the elongation of primary cilia in RPE cells.
Autophagy* ; Cilia* ; Humans ; Mefloquine ; Retinaldehyde ; Small Molecule Libraries