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1.
Yonsei Medical Journal ; : 542-546, 2020.
Article | WPRIM | ID: wpr-833355

ABSTRACT

Activated phosphoinositide 3-kinase δ syndrome (APDS)1 is caused by gain-of-function mutations in PIK3CD, which encodes the catalytic p110δ subunit of phosphoinositide 3 kinase. We describe three patients with APDS1, the first thereof in Korea. Therein, we investigated clinical manifestations of APDS1 and collected data on the efficacy and safety profile of sirolimus, a mammalian target of rapamycin inhibitor and pathway-specific targeted medicine. The same heterozygous PIK3CD mutation was detected in all three patients (E1021K). After genetic diagnosis, all patients received sirolimus and experienced an excellent response, including amelioration of lymphoproliferation and improvement of nodular mucosal lymphoid hyperplasia in the gastrointestinal tract. The median trough level of sirolimus was 5.5 ng/mL (range, 2.8–7.5) at a dose of 2.6–3.6 mg/m2. Two patients who needed highdose, short-interval, immunoglobulin-replacement treatment (IGRT) had a reduced requirement for IGRT after initiating sirolimus, and the dosing interval was extended from 2 and 3 weeks to 4 weeks. The IgG trough level after sirolimus treatment (median, 594 mg/dL; range, 332–799 mg/dL) was significantly higher than that before sirolimus treatment (median, 290 mg/dL; range, 163–346 mg/dL) (p<0.001). One episode of elevated serum creatinine with a surge of sirolimus (Patient 2) and episodes of neutropenia and oral stomatitis (Patient 1) were observed. We diagnosed the first three patients with APDS1 in Korea. Low-dose sirolimus may alleviate clinical manifestations thereof, including hypogammaglobulinemia.

2.
Article in English | WPRIM | ID: wpr-831569

ABSTRACT

Background@#Hodgkin's lymphoma (HL) constitutes 10%–20% of all malignant lymphomas and has a high cure rate (5-year survival, around 90%). Recently, interest has increased concerning preventing secondary complications (secondary cancer, endocrine disorders) in long-term survivors. We aimed to study the epidemiologic features and therapeutic outcomes of HL in children, adolescents, and young adults in Korea. @*Methods@#We performed a multicenter, retrospective study of 224 patients aged < 25 years diagnosed with HL at 22 participating institutes in Korea from January 2007 to August 2016. @*Results@#A higher percentage of males was diagnosed at a younger age. Nodular sclerosis histopathological HL subtype was most common, followed by mixed cellularity subtype.Eighty-one (36.2%), 101 (45.1%), and 42 (18.8%) patients were classified into low, intermediate, and high-risk groups, respectively. Doxorubicin, bleomycin, vinblastine, dacarbazine was the most common protocol (n = 102, 45.5%). Event-free survival rate was 86.0% ± 2.4%, while five-year overall survival (OS) rate was 96.1% ± 1.4%: 98.7% ± 1.3%, 97.7% ± 1.6%, and 86.5% ± 5.6% in the low, intermediate, and high-risk groups, respectively (P = 0.021). Five-year OS was worse in patients with B-symptoms, stage IV disease, highrisk, splenic involvement, extra-nodal lymphoma, and elevated lactate dehydrogenase level.In multivariate analysis, B-symptoms and extra-nodal involvement were prognostic factors for poor OS. Late complications of endocrine disorders and secondary malignancy were observed in 17 and 6 patients, respectively. @*Conclusion@#This is the first study on the epidemiology and treatment outcomes of HL in children, adolescents, and young adults in Korea. Future prospective studies are indicated to develop therapies that minimize treatment toxicity while maximizing cure rates in children, adolescents, and young adults with HL.

3.
Article | WPRIM | ID: wpr-831549

ABSTRACT

Background@#Hereditary hemolytic anemia (HHA) is a rare disease characterized by premature red blood cell (RBC) destruction due to intrinsic RBC defects. The RBC Disorder Working Party of the Korean Society of Hematology established and updated the standard operating procedure for making an accurate diagnosis of HHA since 2007. The aim of this study was to investigate a nationwide epidemiology of Korean HHA. @*Methods@#We collected the data of a newly diagnosed pediatric HHA cohort (2007–2016) and compared this cohort's characteristics with those of a previously surveyed pediatric HHA cohort (1997–2006) in Korea. Each participant's information was retrospectively collected by a questionnaire survey. @*Results@#A total of 369 children with HHA from 38 hospitals distributed in 16 of 17 districts of Korea were investigated. RBC membranopathies, hemoglobinopathies, RBC enzymopathies, and unknown etiologies accounted for 263 (71.3%), 59 (16.0%), 23 (6.2%), and 24 (6.5%) of the cases, respectively. Compared to the cohort from the previous decade, the proportions of hemoglobinopathies and RBC enzymopathies significantly increased (P < 0.001 and P = 0.008, respectively). Twenty-three of the 59 hemoglobinopathy patients had immigrant mothers, mostly from South-East Asia. @*Conclusion@#In Korea, thalassemia traits have increased over the past 10 years, reflecting both increased awareness of this disease and increased international marriages. The enhanced recognition of RBC enzymopathies is due to advances in diagnostic technique; however, 6.5% of HHA patients still do not have a clear diagnosis. It is necessary to improve accessibility of diagnosing HHA.

4.
Article in English | WPRIM | ID: wpr-763511

ABSTRACT

A 14 year-old boy with acute lymphoblastic leukemia (ALL) on maintenance chemotherapy presented with vision-threatening cytomegalovirus (CMV) retinitis. Treatment with intavitreal ganciclovir injection (2 mg/0.1 mL) followed by oral ganciclovir resulted in successful resolution of CMV retinitis. Another 13 year-old boy with ALL on maintenance chemotherapy presented with prolonged fever with no response to antibiotics administration. CMV and real-time PCR revealed positive result and a titer of 2,618,700 copies/mL, respectively. Ganciclovir was used for more than the approved duration of treatment, but viral titer frequently recurred with elevated liver enzymes and fever. In these 2 cases of CMV infection, a high index of suspicion and prompt management is important in children receiving ALL chemotherapy.


Subject(s)
Anti-Bacterial Agents , Child , Cytomegalovirus Infections , Cytomegalovirus , Drug Therapy , Fever , Ganciclovir , Humans , Liver , Maintenance Chemotherapy , Male , Precursor Cell Lymphoblastic Leukemia-Lymphoma , Real-Time Polymerase Chain Reaction , Retinitis
5.
Article in English | WPRIM | ID: wpr-762440

ABSTRACT

BACKGROUND: von Willebrand disease (VWD), characterized by quantitative or qualitative defects of von Willebrand factor (VWF), is the most common inheritable bleeding disorder. Data regarding the genetic background of VWD in Korean patients is limited. To our knowledge, this is the first comprehensive molecular genetic investigation of Korean patients with VWD. METHODS: Twenty-two unrelated patients with VWD were recruited from August 2014 to December 2017 (age range 28 months–64 years; male:female ratio 1.2:1). Fifteen patients had type 1, six had type 2, and one had type 3 VWD. Blood samples were collected for coagulation analyses and molecular genetic analyses from each patient. Direct sequencing of all exons, flanking intronic sequences, and the promoter of VWF was performed. In patients without sequence variants, multiplex ligation-dependent probe amplification (MLPA) was performed to detect dosage variants. We adapted the American College of Medical Genetics and Genomics guidelines for variant interpretation and considered variants of uncertain significance, likely pathogenic variants, and pathogenic variants as putative disease-causing variants. RESULTS: VWF variants were identified in 15 patients (68%): 14 patients with a single heterozygous variant and one patient with two heterozygous variants. The variants consisted of 13 missense variants, one small insertion, and one splicing variant. Four variants were novel: p.S764Efs*16, p.C889R, p.C1130Y, and p.W2193C. MLPA analysis in seven patients without reportable variants revealed no dosage variants. CONCLUSIONS: This study revealed the spectrum of VWF variants, including novel ones, and limited diagnostic utility of MLPA analyses in Korean patients with VWD.


Subject(s)
Exons , Genetic Background , Genetics, Medical , Genomics , Hemorrhage , Humans , Introns , Korea , Molecular Biology , Multiplex Polymerase Chain Reaction , von Willebrand Disease, Type 3 , von Willebrand Diseases , von Willebrand Factor
6.
Article in English | WPRIM | ID: wpr-764849

ABSTRACT

BACKGROUND: New B-domain deleted third generation recombinant factor VIII (FVIII; GreenGene F™, beroctocog alfa) was launched in 2010. We determined safety and efficacy of GreenGene F™ during routine clinical practice in patients with hemophilia A over a period of 12 months. METHODS: From July 2010 to July 2014, a total of 136 hemophilia A patients were enrolled in a post-marketing surveillance (PMS) study. Among them, 134 patients were assessed for drug safety and 114 patients were analyzed for drug efficacy. Patients with differing hemophilia A severities and medical histories were monitored during 12 months of prophylactic and/or on-demand therapy. RESULTS: Among 134 patients evaluated, 85 (63.4%) had severe hemophilia. Ninety-two received a total of 1,266,077 units for prophylaxis, and 42 received 516,491 units for bleeding episodes. Three patients developed inhibitors. In 112 previously treated patients, one patient (0.9%) developed inhibitor after intensive FVIII treatment for surgery. Among 22 previously untreated patients, inhibitors were observed in 2 infants (9.1%). Overall, there were a total of 47 adverse events (other than inhibitors) of all types in 30 patients (22.4%), 11 in 10 patients (7.5%) of which were considered showing serious adverse events (SAEs); most of which were hemorrhages at different sites. None of the SAEs were judged as product related. An excellent/good efficacy rate of 91.3% for hemostasis and 89.4% for hemorrhage prevention was recorded. CONCLUSION: The results of this PMS study support the use of GreenGene F™ as safe and efficacious in hemorrhage prevention and treatment of hemophilia A. These results are consistent with the findings from previously published GreenGene F™ studies.


Subject(s)
Factor VIII , Hemophilia A , Hemorrhage , Hemostasis , Humans , Infant
7.
Article in English | WPRIM | ID: wpr-716849

ABSTRACT

PURPOSE: In recent years, there has been an increasing focus on thyroid function in pediatric obese patients, but no nationwide study evaluating the relationship between thyroid function and obesity has yet been conducted in Korea. We aimed to evaluate thyroid dysfunction in obese Korean children. METHODS: We analyzed the associations between obesity and thyroid hormone levels among 975 Korean boys and girls aged 10–18 years and who participated in the Korean National Health and Nutrition Examination Survey VI (2013–2015). RESULTS: Average serum thyrotropin (TSH) and serum free thyroxine (fT4) levels in the nonobese group were 2.7±0.1 μIU/mL and 1.3±0.0 ng/dL, respectively, and those in the overweight group were 3.1±0.2 μIU/mL and 1.2±0.0 ng/dL. Serum TSH level was significantly higher in the abdominal obesity group than in the normal group (P=0.023). fT4 level was significantly lower in both the overweight and abdominal obesity groups than in the normal group (P < 0.001, P=0.014). Serum TSH level was associated positively with abdominal obesity and levels of high-density lipoprotein cholesterol and triglyceride. Serum fT4 level was negatively correlated with abdominal obesity (P=0.014). CONCLUSIONS: Korean children with abdominal obesity showed increased TSH and decreased fT4 levels compared to normal children.


Subject(s)
Adolescent , Child , Cholesterol , Female , Humans , Korea , Lipoproteins , Nutrition Surveys , Obesity , Obesity, Abdominal , Overweight , Thyroid Gland , Thyrotropin , Thyroxine , Triglycerides
8.
Article in English | WPRIM | ID: wpr-714203

ABSTRACT

Over the past several decades, hemophilia treatment in Korea has progressed dramatically. It has become possible to prevent hemophilia complications by maintenance treatment as well as on-demand treatment with the help of the National Health Insurance program. Treatment and prevention of hemorrhage, prevention of joint complications, treatment and prevention of infectious complications have greatly improved the quality of life and life expectancy of hemophilia patients. However, the development of inhibitor is the most serious and challenging complication of clotting factor replacement therapy, although immune tolerance regimens and bypassing agents have shown some efficacy in countering this complication. The development of novel methods of therapy, including the use of extended half-life factors and gene therapy, will further improve the outcome of hemophilia patients. Administering the right drug to the right patients with the right dose at the right time will be necessary for treating the patient. Achievement of optimal therapeutic goals will require continued cooperation between patients and medical staff.


Subject(s)
Factor VIII , Genetic Therapy , Half-Life , Hemophilia A , Hemorrhage , Humans , Immune Tolerance , Joints , Korea , Life Expectancy , Medical Staff , National Health Programs , Quality of Life
9.
Article in English | WPRIM | ID: wpr-714200

ABSTRACT

BACKGROUND: A patient's adherence to prophylactic treatment is one of the most significant factors to achieve desired outcomes, in regards to the quality of life and treatment cost-effectiveness. The aim of this study is to evaluate the effectiveness of educational interventions in enhancing adherence to prophylactic treatment in Korean hemophilia patients. METHODS: The Validated Hemophilia Regimen Treatment Adherence Scale-Prophylaxis (VERITAS-Pro) was used to measure adherence. The study design consisted of two groups. One group was not educated with the education card which presented the morning administration of clotting factor concentrates and self-infusion skill. The other group was educated with the card. The scores of each subscale in the two groups and scores focused on the ‘Timing’ subscale were compared. RESULTS: Participants were recruited from five hemophilia treatment centers in Korea with 95 eligible patients forming the uneducated group and 123 patients in the educated group. The mean total score was 40.4 and 39.9, respectively. The subscale mean scores of the uneducated group and educated group were 8.43 and 7.90 (Timing), 6.12 and 5.88 (Dosing), 6.43 and 6.33 (Planning), 5.99 and 6.35 (Remembering), 6.22 and 6.25 (Skipping), and 7.23 and 7.24 (Communicating), respectively. CONCLUSION: Results showed that education programs on prophylactic treatment for hemophilia patients need to be well-designed with precise and subject-appropriate contents. Although the mean total scores of VERITAS-Pro could not be improved, the appropriate timing of injection with the educational intervention was administered. Results suggest that this whole process can enhance Korean hemophilia patients' adherence to prophylactic treatment.


Subject(s)
Education , Hemophilia A , Humans , Korea , Quality of Life
10.
Article in English | WPRIM | ID: wpr-217620

ABSTRACT

Calcinosis cutis-calcification in soft tissue-is a rare benign disease that is separated into the following subtypes: dystrophic, iatrogenic, metastatic, calciphylaxis, and idiopathic. One of common site of calcinosis cutis is the scrotum. The nodules slowly grow for years or decades. The characteristic of calcinosis cutis of the scrotum is generally asymptomatic, yellowish marble-like, hard, polypoidal, solitary, or multiple. However, the pathogenesis of this nodule remains ambiguous and controversial. Thus, we reviewed possible causes and therapeutic consideration of calcinosis cutis of the scrotum.


Subject(s)
Calcinosis , Calciphylaxis , Scrotum , Skin
11.
Article in Korean | WPRIM | ID: wpr-788614

ABSTRACT

BACKGROUND: von Willebrand disease (VWD) is the most common inherited bleeding disorder with a prevalence of up to 1%. However in Korea, only 126 VWD patients were registered in Korea Hemophilia Foundation (KHF). The aim of this study was to determine the status of VWD patients in Korea. We analyzed VWD patients by age, gender, blood group, family history and bleeding history.METHODS: One hundred twenty-six VWD patients registered in the KHF by December 2016, and 74 patients diagnosed at six university hospitals were enrolled in this study. We evaluated the medical records from the KHF and the questionnaires from six university hospitals retrospectively.RESULTS: Seventeen patients misdiagnosed and ten patients duplicated were excluded. One hundred nine patients registered in the KHF and 64 patients diagnosed at six university hospitals met the criteria for VWD. The blood type O accounts for 72 (51.8%). VWF mutation was detected in 30 patients (17.3%). Median age at diagnosis was 10.5 yr. The bleeding score of adults was higher than that of children (P < 0.001). The most common bleeding symptom was epistaxis (48.5%). The distribution of VWD types was: 67% of type 1, 30.1% of type 2, and 2.9% of type 3.CONCLUSION: Even though only six hospitals responded to the survey, 64 patients not registered in the KHF were diagnosed with VWD. Our results suggest the prevalence of Korean VWD might be higher than previously reported. A nationwide registration system is warranted in order to accurately identify the national prevalence of VWD.


Subject(s)
Adult , Child , Diagnosis , Epistaxis , Hemophilia A , Hemorrhage , Hospitals, University , Humans , Korea , Medical Records , Prevalence , Retrospective Studies , von Willebrand Diseases
12.
Article in Korean | WPRIM | ID: wpr-788613

ABSTRACT

BACKGROUND: Although the overall survival of childhood acute lymphoblastic leukemia (ALL) approaches 85-90%, the prognosis of relapsed or refractory (R/R) ALL is grave. This study aimed to identify the treatment pattern, treatment response, and overall survival of these patients.METHODS: We reviewed data of 64 patients with R/R ALL whose initial diagnosis of ALL had been made between 1 and 21 years of age. Patients who received clofarabine as part of an induction regimen were excluded. Relapsed patients were limited to those who relapsed after ≥2 prior induction regimens. Treatment patterns, response rates, and overall survival were analyzed.RESULTS: Patients' median age was 15.0 years (range, 6.0-25.0) at the diagnosis of R/R ALL. The most frequently used agents other than steroid were vincristine (54.0%), cytarabine (44.6%), and idarubicin (36.5%), while L-asparaginase was used in only one patient. The complete remission (CR) and overall response (OR) rates were 38.1 and 42.9%, respectively. Sixteen patients (25.4%) underwent allogeneic hematopoietic stem cell transplantation (HSCT). The 5-year overall survival was 6.7%. The survival of patients with HSCT was significantly higher compared with those without HSCT (35.2% vs 0%, P=0.0097). Among 14 patients who achieved CR or CR without platelet recovery (CRp) before HSCT, the 3-year survival was 46.9%.CONCLUSION: The survival of Korean patients with R/R childhood ALL was dismal despite a reasonable CR rate, whereas that of those who received HSCT after CR or CRp was excellent. More treatment options are needed to improve the overall outcome of R/R childhood ALL.


Subject(s)
Blood Platelets , Cytarabine , Diagnosis , Hematopoietic Stem Cell Transplantation , Humans , Idarubicin , Precursor Cell Lymphoblastic Leukemia-Lymphoma , Prognosis , Retrospective Studies , Vincristine
13.
Article in Korean | WPRIM | ID: wpr-23112

ABSTRACT

BACKGROUND: von Willebrand disease (VWD) is the most common inherited bleeding disorder with a prevalence of up to 1%. However in Korea, only 126 VWD patients were registered in Korea Hemophilia Foundation (KHF). The aim of this study was to determine the status of VWD patients in Korea. We analyzed VWD patients by age, gender, blood group, family history and bleeding history. METHODS: One hundred twenty-six VWD patients registered in the KHF by December 2016, and 74 patients diagnosed at six university hospitals were enrolled in this study. We evaluated the medical records from the KHF and the questionnaires from six university hospitals retrospectively. RESULTS: Seventeen patients misdiagnosed and ten patients duplicated were excluded. One hundred nine patients registered in the KHF and 64 patients diagnosed at six university hospitals met the criteria for VWD. The blood type O accounts for 72 (51.8%). VWF mutation was detected in 30 patients (17.3%). Median age at diagnosis was 10.5 yr. The bleeding score of adults was higher than that of children (P < 0.001). The most common bleeding symptom was epistaxis (48.5%). The distribution of VWD types was: 67% of type 1, 30.1% of type 2, and 2.9% of type 3. CONCLUSION: Even though only six hospitals responded to the survey, 64 patients not registered in the KHF were diagnosed with VWD. Our results suggest the prevalence of Korean VWD might be higher than previously reported. A nationwide registration system is warranted in order to accurately identify the national prevalence of VWD.


Subject(s)
Adult , Child , Diagnosis , Epistaxis , Hemophilia A , Hemorrhage , Hospitals, University , Humans , Korea , Medical Records , Prevalence , Retrospective Studies , von Willebrand Diseases
14.
Article in Korean | WPRIM | ID: wpr-23111

ABSTRACT

BACKGROUND: Although the overall survival of childhood acute lymphoblastic leukemia (ALL) approaches 85-90%, the prognosis of relapsed or refractory (R/R) ALL is grave. This study aimed to identify the treatment pattern, treatment response, and overall survival of these patients. METHODS: We reviewed data of 64 patients with R/R ALL whose initial diagnosis of ALL had been made between 1 and 21 years of age. Patients who received clofarabine as part of an induction regimen were excluded. Relapsed patients were limited to those who relapsed after ≥2 prior induction regimens. Treatment patterns, response rates, and overall survival were analyzed. RESULTS: Patients' median age was 15.0 years (range, 6.0-25.0) at the diagnosis of R/R ALL. The most frequently used agents other than steroid were vincristine (54.0%), cytarabine (44.6%), and idarubicin (36.5%), while L-asparaginase was used in only one patient. The complete remission (CR) and overall response (OR) rates were 38.1 and 42.9%, respectively. Sixteen patients (25.4%) underwent allogeneic hematopoietic stem cell transplantation (HSCT). The 5-year overall survival was 6.7%. The survival of patients with HSCT was significantly higher compared with those without HSCT (35.2% vs 0%, P=0.0097). Among 14 patients who achieved CR or CR without platelet recovery (CRp) before HSCT, the 3-year survival was 46.9%. CONCLUSION: The survival of Korean patients with R/R childhood ALL was dismal despite a reasonable CR rate, whereas that of those who received HSCT after CR or CRp was excellent. More treatment options are needed to improve the overall outcome of R/R childhood ALL.


Subject(s)
Blood Platelets , Cytarabine , Diagnosis , Hematopoietic Stem Cell Transplantation , Humans , Idarubicin , Precursor Cell Lymphoblastic Leukemia-Lymphoma , Prognosis , Retrospective Studies , Vincristine
15.
Article in English | WPRIM | ID: wpr-16104

ABSTRACT

PURPOSE: Quadriplegic children with cerebral palsy are more susceptible to osteoporosis because of various risk factors that interfere with bone metabolism. Pamidronate is effective for pediatric osteoporosis, but there are no guidelines for optimal dosage or duration of treatment in quadriplegic children with osteoporosis. We aimed to evaluate the efficacy of low-dose pamidronate treatment in these patients. METHODS: Ten quadriplegic patients on antiepileptic drugs (6 male, 4 female patients; mean age, 10.9±5.76 years), with osteoporosis and gross motor function classification system level V, were treated with pamidronate (0.5–1.0 mg/kg/day, 2 consecutive days) every 3–4 months in a single institution. The patients received oral supplements of calcium and vitamin D before and during treatment. The lumbar spine bone mineral density (BMD) z score and biochemical markers of bone metabolism were measured regularly during treatment. RESULTS: The main underlying disorder was perinatal hypoxic brain damage (40%, 4 of 10). The mean cumulative dose of pamidronate was 4.49±2.22 mg/kg/yr, and the mean treatment period was 10.8±3.32 months. The BMD z score of the lumbar spine showed a significant increase from −4.22±1.24 before treatment to −2.61±1.69 during treatment (P=0.008). Alkaline phosphatase decreased during treatmentn (P=0.037). Significant adverse drug reactions and new fractures were not reported. CONCLUSION: Low-dose pamidronate treatment for quadriplegic children with cerebral palsy increased lumbar BMD and reduced the incidence of fracture.


Subject(s)
Alkaline Phosphatase , Anticonvulsants , Biomarkers , Bone Density , Calcium , Cerebral Palsy , Child , Classification , Drug-Related Side Effects and Adverse Reactions , Female , Humans , Hypoxia, Brain , Incidence , Male , Metabolism , Osteoporosis , Quadriplegia , Risk Factors , Spine , Vitamin D
16.
Blood Research ; : 268-273, 2016.
Article in English | WPRIM | ID: wpr-167168

ABSTRACT

BACKGROUND: In Korea, the prevalence of anemia and iron deficiency anemia (IDA) among older infants and young children remains high. To detect IDA early and to reduce its adverse impact, we assessed the characteristics of infants and young children who had IDA or were at risk of developing IDA, or who exhibited characteristics associated with severe anemia. METHODS: Among the 1,782 IDA-affected children aged 6 months to 18 years who visited the hospital, we retrospectively analyzed the medical records and laboratory data of 1,330 IDA-affected children aged 6–23 months who were diagnosed between 1996 and 2013. We excluded patients with a C-reactive protein level ≥5 mg/dL. RESULTS: IDA was predominant in boys (2.14:1) during infancy and early childhood. The peak IDA incidence was noted among infants aged 9–12 months. Only 7% patients exhibited symptoms of IDA, while 23.6% patients with severe IDA demonstrated classic symptoms/signs of IDA. Low birth weight (LBW) infants with IDA demonstrated low adherence to iron supplementation. In a multivariate analysis, prolonged breastfeeding without iron fortification (odds ratio [OR] 5.70), and a LBW (OR 6.49) were identified as risk factors of severe anemia. CONCLUSION: LBW infants need more attention in order to increase their adherence to iron supplementation. For the early detection of IDA, nutritional status of all infants, and iron batteries of high-risk infants (LBW infants, infants with prolonged breastfeeding, picky eaters, and/or infants with the presence of IDA symptoms) should be evaluated at their health screening visits.


Subject(s)
Anemia , Anemia, Iron-Deficiency , Breast Feeding , C-Reactive Protein , Child , Humans , Incidence , Infant , Infant, Low Birth Weight , Infant, Newborn , Iron , Korea , Mass Screening , Medical Records , Multivariate Analysis , Nutritional Status , Prevalence , Retrospective Studies , Risk Factors
17.
Article in Korean | WPRIM | ID: wpr-788590

ABSTRACT

BACKGROUND: Iron pots have long been used for cooking in several countries. Early studies have shown that the use of such iron pots can increase the iron content of food cooked in them and that this increased iron content has some effect on iron uptake. This study was designed to evaluate the iron content in rice cooked in a traditional iron pot and study the iron uptake by macrophages through heme oxygenase-1 (HO-1).METHODS: The iron pot used in this study was round-shaped and had no legs. The iron content of rice cooked in the iron pot was measured. Thereafter, the bioavailability of iron was measured using western blot analysis.RESULTS: A total of 35 samples were analyzed for iron concentrations, which were 10.94±18.08 mg/L (range: 0.18–56.53 mg/L). The biochemical activity in most of materials was 1.5–9 times that of the activity observed in the control group.CONCLUSION: The iron concentration of rice cooked in iron pots were found to be relatively high. The introduction of iron pots in routine cooking practices may be a promising way of increasing the supply of iron, especially for people with severe iron deficiency anemia. Further, increased activity of HO-1, induced by supplementation of iron from the cast iron, may help in maintaining iron homeostasis.


Subject(s)
Anemia, Iron-Deficiency , Biological Availability , Blotting, Western , Cooking , Heme Oxygenase-1 , Heme , Homeostasis , In Vitro Techniques , Iron , Leg , Macrophages
18.
Article in Korean | WPRIM | ID: wpr-87521

ABSTRACT

PURPOSE: Enterovirus (EV) infection in children can manifest various diseases from asymptomatic infection to nonspecific febrile illness, hand-foot-mouth disease, and aseptic meningitis. This study was aimed to investigate epidemiology and clinical significance of various genotypes of EV infections in pediatric inpatient. METHODS: We collected the stool samples from the admitted pediatric patients in Inha University Hospital from March 2014 to March 2015. EV detection and genotype identification were performed by real-time RT-PCR and semi-nested RT-PCR. Phylogenetic trees were constructed by neighbor joining method. RESULTS: A total of 400 samples were collected during study period and 112 patients (28%) were diagnosed with EV infections. The mean age of EV positive patients was 2.66 years (0.1-14) and sex ratio was 1.73:1. Genetic sequences of EVs were identified; coxsackievirus B5 (17, 15.2%), coxsackievirus A16 (13, 11.6%), enterovirus 71 (10, 8.9%), and coxsackievirus A2 (9, 8.0%). Nonspecific febrile illness (96, 86%) was the most common clinical manifestation and the duration of fever was 0-11 days (mean 3.1 days). Rash (44, 39%) and meningitis (43, 38%) were followed. Patients who were attending daycare center or had siblings accounted for 82.1%. Phylogenetic relationship tree revealed 6 distinct genogroups among 56 types of EVs. CONCLUSIONS: This study is the report of epidemiology, serotype distribution and clinical manifestations of children with EV infection in Incheon. This data will be helpful for further study about the epidemiology of EV infection in Korea.


Subject(s)
Asymptomatic Infections , Child , Enterovirus , Epidemiology , Exanthema , Fever , Genotype , Humans , Inpatients , Korea , Meningitis , Meningitis, Aseptic , Sex Ratio , Siblings , Trees
19.
Article in English | WPRIM | ID: wpr-39526

ABSTRACT

PURPOSE: Obesity is related to many diseases, including urological conditions. We investigated the prevalence, risk factors, and treatment of male obesity. MATERIALS AND METHODS: This study included 17,485 men older than 20 years of age who participated in the fourth, fifth, and sixth administrations of the Korean National Health and Nutrition Examination Survey. Two main cutoff points for obesity were defined: a body mass index (BMI) ≥25 kg/m2 and a BMI≥30 kg/m2. Additionally, we defined obesity requiring pharmacotherapy as the presence of a BMI≥30 kg/m2 or a BMI≥27 kg/m2 co-occurring with at least one associated comorbid medical condition, such as hypertension, dyslipidemia, or diabetes. RESULTS: The prevalence rates of a BMI≥25 kg/m2, a BMI≥30 kg/m2, and obesity requiring pharmacotherapy were 35.7%, 3.4%, and 10.5%, respectively. The prevalence of obesity increased over time for all definitions of obesity. The prevalence of obesity requiring pharmacotherapy was highest in Jeju (12.5%) and lowest in Gangwon-do (7.7%). Having a higher income, being a non-manual worker, and having completed a high level of education were significantly related to obesity requiring pharmacotherapy. More than 70% of patients with obesity requiring pharmacotherapy reported taking diet pills, eating functional foods, or consuming a one-food diet for weight reduction, but only 13.9% reported exercising for this purpose. CONCLUSIONS: Male obesity is a common condition, the prevalence of which is expected to continue to increase over time. A better strategy is required to manage male obesity in Korea.


Subject(s)
Body Mass Index , Diet , Drug Therapy , Dyslipidemias , Eating , Education , Functional Food , Humans , Hypertension , Korea , Male , Nutrition Surveys , Obesity , Prevalence , Risk Factors , Weight Loss
20.
Article in English | WPRIM | ID: wpr-34967

ABSTRACT

Klinefelter syndrome (KS) is one of the most common disease entities characterized by X-chromosomal aberration causing the primary hypogonadism in adult men. Patients with KS seem to be typically characterized by tall, slender bodies with delayed puberty and hypergonadotropic hypogonadism. However, it has been known that they have a broad spectrum of phenotype ranging from almost normal external appearances to typical phenotype. Only 25% KS Patients are ever diagnosed because KS remains unrecognized. Also, boys with KS have an onset of pubertal development within the normal range, not delayed onset of puberty. Adolescents with KS are generally diagnosed as having the lack of pubertal progress. Early detection of KS can be difficult without awareness. We report an unusual case of early onset of puberty in obese boy with KS who presented with a unilateral non-hormone secreting testicular teratoma.


Subject(s)
Adolescent , Adult , Child , Humans , Hypogonadism , Klinefelter Syndrome , Male , Phenotype , Puberty , Puberty, Delayed , Reference Values , Teratoma
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