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1.
Journal of Korean Medical Science ; : e90-2022.
Artigo em Inglês | WPRIM | ID: wpr-925932

RESUMO

Background@#The study aimed to compare the growth responses to 3 years of growth hormone (GH) treatment in children and adolescents with GH deficiency (GHD) according to idiopathic, organic, isolated (IGHD), and multiple pituitary hormone deficiency (MPHD). @*Methods@#Total 163 patients aged 2–18 years (100 males and 63 females; 131 idiopathic and 32 organic GHD; 129 IGHD and 34 MPHD) were included from data obtained from the LG Growth Study. Parameters of growth responses and biochemical results were compared during the 3-year GH treatment. @*Results@#The baseline age, bone age (BA), height (Ht) standard deviation score (SDS), weight SDS, mid-parental Ht SDS, predicted adult Ht (PAH) SDS, and insulin like growth factor-1 (IGF-1) SDS were significantly higher in the organic GHD patients than in the idiopathic GHD patients, but peak GH on the GH-stimulation test, baseline GH dose, and mean 3-year-GH dosage were higher in the idiopathic GHD patients than in the organic GHD patients. The prevalence of MPHD was higher in the organic GHD patients than in the idiopathic GHD patients. Idiopathic MPHD subgroup showed the largest increase for the ΔHt SDS and ΔPAH SDS during GH treatment, and organic MPHD subgroup had the smallest mean increase after GH treatment, depending on ΔIGF-1 SDS and ΔIGF binding protein-3 (IGFBP-3) SDS.The growth velocity and the parental-adjusted Ht gain were greater in the idiopathic GHD patients than the organic GHD patients during the 3-year GH treatment, which may have been related to the different GH dose, ΔIGF-1 SDS, and ΔIGFBP-3 SDS between two groups.Multiple linear regression analysis revealed that baseline IGF-1 SDS, BA, and MPH SDS in idiopathic group and baseline HT SDS in organic group are the most predictable parameters for favorable 3-year-GH treatment. @*Conclusion@#The 3-year-GH treatment was effective in both idiopathic and organic GHD patients regardless of the presence of MPHD or underlying causes, but their growth outcomes were not constant with each other. Close monitoring along with appropriate dosage of GH and annual growth responses, not specific at baseline, are more important in children and adolescents with GHD for long-term treatment.

2.
International Journal of Thyroidology ; : 54-59, 2022.
Artigo em Inglês | WPRIM | ID: wpr-925091

RESUMO

Dual oxidase maturation factor 2 (DUOXA2) is necessary for the enzymatic activity of dual oxidase 2 (DUOX2) to generate hydrogen peroxide production during thyroid hormone synthesis. We describe two Korean children, who were initially suspected to have transient congenital hypothyroidism (CH), but later confirmed to have permanent CH caused by DUOXA2 mutation. Treatment with levothyroxine was discontinued after confirming thyroid-stimulating hormone (TSH) level to be below 10 μU/mL and normal thyroid scan at the first or second trial-off therapy. However, after therapy cessation, TSH elevated to more than 10 μU/mL, and goiter developed in case 2. As a result, levothyroxine was resumed. Next-generation sequencing showed compound heterozygous mutations of DUOXA2 at Y138X and Y246X in case 1 and homozygous mutations of DUOXA2 at Y246X in case 2. In this report, a longer follow-up is recommended even after treatment termination in transient CH, and genetic studies might help assess the permanence of hypothyroidism in cases of mildly elevated TSH after trial-off therapy.

3.
Journal of Korean Medical Science ; : e17-2021.
Artigo em Inglês | WPRIM | ID: wpr-874753

RESUMO

In April 2020, a pediatric report of an unusual inflammatory illness associated with coronavirus disease 2019 (COVID-19) led to similar cases in Europe and North America, which was referred to as multisystem inflammatory syndrome in children (MIS-C). Herein, we describe the case of a 12-year-old boy who had a history of polymerase chain reactionconfirmed COVID-19 and developed MIS-C approximately three weeks after an initial diagnosis of COVID-19. High fever with abdominal pain mimicking appendicitis was the initial manifestation of MIS-C, which could have been easily missed if the patient's history of COVID-19 was ignored. Intravenous immunoglobulin was administered twice, 24 hours apart, five days after the onset of MIS-C, and the patient fully recovered without any obvious sequelae. Early recognition by disease awareness and prompt management are the keys to saving the lives of children affected by MIS-C.

4.
Endocrinology and Metabolism ; : 146-156, 2021.
Artigo em Inglês | WPRIM | ID: wpr-874539

RESUMO

Background@#The microencapsulation is an ideal solution to overcome immune rejection without immunosuppressive treatment. Poor biocompatibility and small molecular antigens secreted from encapsulated islets induce fibrosis infiltration. Therefore, the aims of this study were to improve the biocompatibility of microcapsules by dexamethasone coating and to verify its effect after xenogeneic transplantation in a streptozotocin-induced diabetes mice. @*Methods@#Dexamethasone 21-phosphate (Dexa) was dissolved in 1% chitosan and was cross-linked with the alginate microcapsule surface. Insulin secretion and viability assays were performed 14 days after microencapsulation. Dexa-containing chitosan-coated alginate (Dexa-chitosan) or alginate microencapsulated porcine islets were transplanted into diabetic mice. The fibrosis infiltration score was calculated from the harvested microcapsules. The harvested microcapsules were stained with trichrome and for insulin and macrophages. @*Results@#No significant differences in glucose-stimulated insulin secretion and islet viability were noted among naked, alginate, and Dexa-chitosan microencapsulated islets. After transplantation of microencapsulated porcine islets, nonfasting blood glucose were normalized in both the Dexa-chitosan and alginate groups until 231 days. The average glucose after transplantation were lower in the Dexa-chitosan group than the alginate group. Pericapsular fibrosis and inflammatory cell infiltration of microcapsules were significantly reduced in Dexa-chitosan compared with alginate microcapsules. Dithizone and insulin were positive in Dexa-chitosan capsules. Although fibrosis and macrophage infiltration was noted on the surface, some alginate microcapsules were stained with insulin. @*Conclusion@#Dexa coating on microcapsules significantly suppressed the fibrotic reaction on the capsule surface after transplantation of xenogenic islets containing microcapsules without any harmful effects on the function and survival of the islets.

5.
Annals of Pediatric Endocrinology & Metabolism ; : 14-20, 2018.
Artigo em Inglês | WPRIM | ID: wpr-713448

RESUMO

PURPOSE: Reported changes in body mass index (BMI) in central precocious puberty (CPP) during and after gonadotropin-releasing hormone analog (GnRHa) treatment are inconsistent. We, therefore, investigated auxological parameters in GnRHa-treated girls with idiopathic CPP (ICPP) until attainment of near final height (NFH). METHODS: From the medical records of 59 ICPP girls who attained NFH after GnRHa therapy, auxological changes were compared between overweight (BMI≥85th percentile) and normal-weight (BMI < 85th percentile) groups. BMIs were changed into standard deviation scores (BMISDSs) for subject chronologic age (BMISDS-CA) and bone age (BMISDS-BA). RESULTS: The incidence of overweight including obesity was high at the start of therapy (35.6%). The predicted adult height (PAH) at start of therapy was significantly shorter than the midparental height (MPH), whereas PAH at end of therapy approached MPH, and NFH was greater than MPH. Height velocity (HV) in the overweight group was higher during GnRHa therapy than that in the normal-weight group, but those in the two groups were not different after therapy until NFH. Both BMISDS-CA and BMISDS-BA increased significantly during therapy, but both BMISDSs decreased significantly after therapy until NFH. At NFH, neither BMISDS was different from that at baseline. In the normal-weight group, both BMISDSs increased during therapy and were maintained until NFH. In the overweight group, neither BMISDS changed during therapy, but there was a decrease after therapy until NFH. CONCLUSIONS: The different patterns of BMISDS change during and after GnRHa therapy until NFH between the 2 groups were related to the different HV during GnRHa therapy.


Assuntos
Adulto , Feminino , Humanos , Índice de Massa Corporal , Seguimentos , Hormônio Liberador de Gonadotropina , Incidência , Prontuários Médicos , Obesidade , Sobrepeso , Puberdade Precoce
6.
Journal of Korean Medical Science ; : e54-2018.
Artigo em Inglês | WPRIM | ID: wpr-764911

RESUMO

A congenital bladder diverticulum (CBD) is caused by inherent muscular weakness instead of obstruction of the bladder outlet. The major clinical conditions are recurrent urinary tract infection (UTI) and voiding dysfunction. This report describes a 15-year-old male adolescent who developed sudden visual disturbance resulting from hypertensive retinopathy. The cause of hypertension was bilateral obstructive uropathy caused by enlarged paraureteral bladder diverticula. After the non-functioning right kidney and ureter and the bilateral diverticula were removed, the left ureter was reimplanted in the bladder. Pathologic findings showed chronic pyelonephritis and partial loss of the bladder musculature in the diverticular wall. This observation indicates that dilated CBD can cause latent UTI, ureteral obstruction, hydronephrosis, and secondary hypertension.


Assuntos
Adolescente , Humanos , Divertículo , Hidronefrose , Hipertensão , Hipertensão Renal , Retinopatia Hipertensiva , Rim , Debilidade Muscular , Pielonefrite , Ureter , Obstrução Ureteral , Bexiga Urinária , Infecções Urinárias
7.
Annals of Pediatric Endocrinology & Metabolism ; : 119-124, 2017.
Artigo em Inglês | WPRIM | ID: wpr-49250

RESUMO

PURPOSE: There are inconsistencies in the results reported in a small number of previous studies into growth hormone (GH) treatment in Korean children with idiopathic short stature (ISS) and idiopathic growth hormone deficiency (IGHD). Thus, the authors retrospectively compared the effects of GH in ISS and IGHD. METHODS: From the medical records of 26 ISS and 30 IGHD children, auxological and biochemical changes including chronologic age (CA), bone age (BA), height standard deviation score (HT-SDS), predicted adult height (PAH), midparental height (MPH), insulin-like growth factor-1 (IGF-1), and insulin-like growth factor binding protein-3 (IGFBP-3) were compared. RESULTS: Before treatment, IGHD group had younger BA, lower BA/CA ratio, and lower IGF-1 level than those in the ISS group. During GH treatment, the levels of IGF-1 and IGFBP-3 were not different. Although annual BA increment was higher in IGHD group, and annual PAH-SDS increment was higher in ISS group, annual HT-SDS increments were not different. Both HT-SDS and PAH-SDS in the ISS group increased significantly until the end of the second year, and then those were not significantly different from MPH-SDS. In the IGHD group, the HT-SDS showed a significant increase till the end of the second year, and the PAH-SDS was not significantly changed at each year, but both HT-SDS and PAH-SDS were not significantly different from MPH-SDS at the end of the third year. CONCLUSION: During GH treatment, both HT-SDS and PAH-SDS approached the genetic target range of MPH-SDS after 2 years in ISS children and 3 years in IGHD children.


Assuntos
Adulto , Criança , Humanos , Hormônio do Crescimento , Proteína 3 de Ligação a Fator de Crescimento Semelhante à Insulina , Fator de Crescimento Insulin-Like I , Prontuários Médicos , Estudos Retrospectivos
8.
Neurology Asia ; : 367-374, 2016.
Artigo em Inglês | WPRIM | ID: wpr-625555

RESUMO

Central nervous system impairment is common in diabetic patients, even in the early stages of the disease, and could be associated with peripheral neuropathy. The aims of this study were to prospectively investigate central nerve conduction in young adults with type 1 diabetes using pattern-reversal visual evoked potentials (PRVEP) and to determine how those results were related to clinical risk factors and the parameters of the peripheral nerve conduction study (NCS). A total of 36 type 1 diabetic patients (15 males) 5-24 years of age (mean 14.5 ± 4.7) underwent PRVEP and NCS annually for five years. For comparison, 39 healthy age and sex matched individuals (mean 14.8 ± 5.0) were evaluated as the control group. The P100 latencies of the PRVEP were prolonged at the study entry in the patients compared with the controls (p< 0.001). Significant correlations were not found between any of the parameters of PRVEP and the glycosylated hemoglobin levels; however, the changes in the parameters of the peripheral NCS were well correlated with metabolic control. The latencies and amplitudes of the P100 were not related to the majority of the parameters of the NCS. A prolonged PRVEP latency may be a sign of optic pathway dysfunction, which begins before apparent diabetic retinopathy. Poor glycemic control proved to be an important risk factor over the 5 years in terms of its relation to the development of peripheral neural pathway abnormalities. However, once central conduction was delayed, its changes were poorly related to diabetic control and the attributes of the peripheral nerve conduction study over the 5-year follow-up.


Assuntos
Diabetes Mellitus
9.
Annals of Pediatric Endocrinology & Metabolism ; : 143-148, 2016.
Artigo em Inglês | WPRIM | ID: wpr-139038

RESUMO

PURPOSE: The prevalence rates of metabolic syndrome (MetS) and percentile distribution of insulin resistance (IR) among Korean children and adolescents were investigated. The cutoff values of IR were calculated to identify high-risk MetS groups. METHODS: Data from 3,313 Korean subjects (1,756 boys and 1,557 girls, aged 10–18 years) were included from the Korean National Health and Nutrition Examination Survey conducted during 2007–2010. Three different sets of criteria for MetS were used. Indirect measures of IR were homeostasis model assessment (HOMA-IR) and triglyceride and glucose (TyG) index. The cutoff values of the HOMA-IR and TyG index were obtained from the receiver operation characteristic curves. RESULTS: According to the MetS criteria of de Ferranti el al., Cook et al., and the International Diabetes Federation, the prevalence rates in males and females were 13.9% and 12.3%, 4.6% and 3.6%, and 1.4% and 1.8%, respectively. Uses these 3 criteria, the cutoff values of the HOMA-IR and TyG index were 2.94 and 8.41, 3.29 and 8.38, and 3.54 and 8.66, respectively. The cutoff values using each of the 3 criteria approximately corresponds to the 50th–75th, 75th, and 75th–90th percentiles of normal HOMA-IR and TyG index levels. CONCLUSION: This study describes the prevalence rates of MetS in Korean children and adolescents, an index of IR, and the cutoff values for MetS with the aim of detecting high-risk groups. The usefulness of these criteria needs to be verified by further evaluation.


Assuntos
Adolescente , Criança , Feminino , Humanos , Masculino , Glucose , Homeostase , Resistência à Insulina , Insulina , Coreia (Geográfico) , Inquéritos Nutricionais , Prevalência , Triglicerídeos
10.
Annals of Pediatric Endocrinology & Metabolism ; : 143-148, 2016.
Artigo em Inglês | WPRIM | ID: wpr-139035

RESUMO

PURPOSE: The prevalence rates of metabolic syndrome (MetS) and percentile distribution of insulin resistance (IR) among Korean children and adolescents were investigated. The cutoff values of IR were calculated to identify high-risk MetS groups. METHODS: Data from 3,313 Korean subjects (1,756 boys and 1,557 girls, aged 10–18 years) were included from the Korean National Health and Nutrition Examination Survey conducted during 2007–2010. Three different sets of criteria for MetS were used. Indirect measures of IR were homeostasis model assessment (HOMA-IR) and triglyceride and glucose (TyG) index. The cutoff values of the HOMA-IR and TyG index were obtained from the receiver operation characteristic curves. RESULTS: According to the MetS criteria of de Ferranti el al., Cook et al., and the International Diabetes Federation, the prevalence rates in males and females were 13.9% and 12.3%, 4.6% and 3.6%, and 1.4% and 1.8%, respectively. Uses these 3 criteria, the cutoff values of the HOMA-IR and TyG index were 2.94 and 8.41, 3.29 and 8.38, and 3.54 and 8.66, respectively. The cutoff values using each of the 3 criteria approximately corresponds to the 50th–75th, 75th, and 75th–90th percentiles of normal HOMA-IR and TyG index levels. CONCLUSION: This study describes the prevalence rates of MetS in Korean children and adolescents, an index of IR, and the cutoff values for MetS with the aim of detecting high-risk groups. The usefulness of these criteria needs to be verified by further evaluation.


Assuntos
Adolescente , Criança , Feminino , Humanos , Masculino , Glucose , Homeostase , Resistência à Insulina , Insulina , Coreia (Geográfico) , Inquéritos Nutricionais , Prevalência , Triglicerídeos
11.
Journal of Korean Medical Science ; : 991-994, 2015.
Artigo em Inglês | WPRIM | ID: wpr-70181

RESUMO

Pancreatic islet transplantation is a physiologically advantageous and minimally invasive procedure for the treatment of type 1 diabetes mellitus. Here, we describe the first reported case of successful allogeneic islet transplantation alone, using single-donor, marginal-dose islets in a Korean patient. A 59-yr-old patient with type 1 diabetes mellitus, who suffered from recurrent severe hypoglycemia, received 4,163 islet equivalents/kg from a single brain-death donor. Isolated islets were infused intraportally without any complications. The immunosuppressive regimen was based on the Edmonton protocol, but the maintenance dosage was reduced because of mucositis and leukopenia. Although insulin independence was not achieved, the patient showed stabilized blood glucose concentration, reduced insulin dosage and reversal of hypoglycemic unawareness, even with marginal dose of islets and reduced immunosuppressant. Islet transplantation may successfully improve endogenous insulin production and glycemic stability in subjects with type 1 diabetes mellitus.


Assuntos
Feminino , Humanos , Pessoa de Meia-Idade , Glicemia/análise , Diabetes Mellitus Tipo 1/cirurgia , Hipoglicemia/cirurgia , Terapia de Imunossupressão/métodos , Imunossupressores/uso terapêutico , Ilhotas Pancreáticas/fisiologia , Transplante das Ilhotas Pancreáticas/métodos , República da Coreia , Doadores de Tecidos
12.
Annals of Pediatric Endocrinology & Metabolism ; : 98-101, 2015.
Artigo em Inglês | WPRIM | ID: wpr-163410

RESUMO

A 9-year-, 11-month-old girl was brought to the Emergency Department for sudden dyspnea caused by massive pericardial effusion. In addition to relative bradycardia despite impending cardiac tamponade, short stature, overweight, and hypercholesterolemia were clues for suspected hypothyroidism. During thyroxine supplementation, catch-up growth was incomplete by rapid skeletal maturation. The use of short-term growth hormone showed increased growth velocity. In conclusion, primary hypothyroidism should be included in the etiologic evaluation of pericardial effusion, especially when it is associated with relative bradycardia. Additional growth promoting therapy should be considered for incomplete catch-up growth in prolonged hypothyroidism during thyroxine supplementation.


Assuntos
Criança , Feminino , Humanos , Lactente , Bradicardia , Tamponamento Cardíaco , Dispneia , Serviço Hospitalar de Emergência , Hormônio do Crescimento , Hipercolesterolemia , Hipotireoidismo , Sobrepeso , Derrame Pericárdico , Tiroxina
13.
Annals of Pediatric Endocrinology & Metabolism ; : 226-229, 2015.
Artigo em Inglês | WPRIM | ID: wpr-96136

RESUMO

Turner syndrome is the most common chromosomal disorder in girls. Various phenotypic features show depending upon karyotype from normal female through ambiguous genitalia to male. Usually, Turner girls containing 45,X/46,XY mosaicism, or sex-determining region Y (SRY) gene may have mixed gonadal dysgenesis with various external sexual differentiation. We experienced a short statured 45,X Turner girl with normal external genitalia. Because SRY gene was positive, laparoscopic gonadectomy was performed. The dysgenetic gonads revealed bilateral ovotesticular tissues. The authors report a mixed gonadal dysgenesis case found in clinical 45,X Turner patient with positive SRY gene. Screening for SRY gene should be done even the karyotype is 45,X monosomy and external genitalia is normal.


Assuntos
Feminino , Humanos , Masculino , Transtornos Cromossômicos , Transtornos do Desenvolvimento Sexual , Genes sry , Genitália , Disgenesia Gonadal Mista , Gônadas , Cariótipo , Programas de Rastreamento , Monossomia , Mosaicismo , Diferenciação Sexual , Síndrome de Turner
14.
Korean Journal of Clinical Neurophysiology ; : 8-14, 2014.
Artigo em Coreano | WPRIM | ID: wpr-156823

RESUMO

BACKGROUND: Early detection of neuropathy may prevent further progression of this complication in the diabetic patients. The purpose of this study was to evaluate the prevalence of early neuropathic complication in patients with newly diagnosed type 1 and type 2 diabetes. METHODS: Nerve conduction studies (median, ulnar, posterior tibial, peroneal, and sural nerves) were performed for 49 type 1 (27 males, mean 14.1+/-7.5 years) and 40 type 2 (27 males, 42.0+/-14.1 years) diabetic patients at onset of diabetes. Children with age at onset under 4 years and adults over 55 years were excluded to eliminate the aging effect and the influence of obstructive arteriosclerosis. Neuropathy was defined as abnormal nerve conduction findings in two or more nerves including the sural nerve. RESULTS: Mean HbA1c level was 12.6+/-3.3% for type 1 and 10.5+/-2.9% for type 2 diabetes. The prevalence of neuropathy was 12.2% for type 1, and 35.0% for type 2 diabetes, respectively. There were significant trends in the prevalence of neuropathy with increasing age (p<0.05). The effect of the mean level of glycosylated hemoglobin on the prevalence of polyneuropathy at onset of diabetes was borderline (p=0.0532). Neither sex of the patients nor the type of diabetes affected the neurophysiologic abnormalities at the diagnosis. CONCLUSIONS: Even in a population with diabetes at the diagnosis, the prevalence of subclinical neuropathy was not low. Neuropathy has been significantly associated with increasing age indicating the possibility of longer duration of undetected diabetes among them, especially in type 2 diabetes.


Assuntos
Adulto , Criança , Humanos , Masculino , Envelhecimento , Arteriosclerose , Diabetes Mellitus , Neuropatias Diabéticas , Diagnóstico , Hemoglobinas Glicadas , Condução Nervosa , Nervos Periféricos , Polineuropatias , Prevalência , Nervo Sural
15.
Experimental & Molecular Medicine ; : e19-2013.
Artigo em Inglês | WPRIM | ID: wpr-147327

RESUMO

New colchicine analogs have been synthesized with the aim of developing stronger potential anticancer activities. Among the analogs, CT20126 has been previously reported to show immunosuppressive activities. Here, we report that CT20126 also shows potential anticancer effects via an unusual mechanism: the modulation of microtubule integrity and cell cycle arrest at the G2/M phase before apoptosis. When we treated COS-7 cells with CT20126 (5 muM), the normal thread-like microtubules were disrupted into tubulin dimers within 10 min and thereafter repolymerized into short, thick filaments. In contrast, cells treated with the same concentration of colchicine exhibited microtubule depolymerization after 20 min and never underwent repolymerization. Furthermore, optical density (OD) analysis (350 nm) with purified tubulin showed that CT20126 had a higher repolymerizing activity than that of Taxol, a potent microtubule-polymerizing agent. These results suggest that the effects of CT20126 on microtubule integrity differ from those of colchicine: the analog first destabilizes microtubules and then stabilizes the disrupted tubulins into short, thick polymers. Furthermore, CT20126 induced a greater level of apoptotic activity in Jurkat T cells than colchicine (assessed by G2/M arrest, caspase-3 activation and cell sorting). At 20 nM, CT20126 induced 47% apoptosis among Jurkat T cells, whereas colchicine induced only 33% apoptosis. Our results suggest that the colchicine analog CT20126 can potently induce apoptosis by disrupting microtubule integrity in a manner that differs from that of colchicine or Taxol.


Assuntos
Animais , Bovinos , Humanos , Acetilação/efeitos dos fármacos , Apoptose/efeitos dos fármacos , Células COS , Caspase 3/metabolismo , Divisão Celular/efeitos dos fármacos , Chlorocebus aethiops , Colchicina/análogos & derivados , Ativação Enzimática/efeitos dos fármacos , Fase G2/efeitos dos fármacos , Células Jurkat , Microtúbulos/metabolismo , Poli(ADP-Ribose) Polimerases/metabolismo , Tubulina (Proteína)/metabolismo , Moduladores de Tubulina/química
16.
Annals of Pediatric Endocrinology & Metabolism ; : 152-155, 2013.
Artigo em Inglês | WPRIM | ID: wpr-178351

RESUMO

Type I (distal) renal tubular acidosis (RTA) is a disorder associated with the failure to excrete hydrogen ions from the distal renal tubule. It is characterized by hyperchloremic metabolic acidosis, an abnormal increase in urine pH, reduced urinary excretion of ammonium and bicarbonate ions, and mild deterioration in renal function. Hypercalciuria is common in distal RTA because of bone resorption, which increases as a buffer against metabolic acidosis. This can result in intractable rickets. We describe a case of distal RTA with nephrocalcinosis during follow-up of rickets in a patient who presented with clinical manifestations of short stature, failure to thrive, recurrent vomiting, dehydration, and irritability.


Assuntos
Humanos , Acidose , Acidose Tubular Renal , Bicarbonatos , Reabsorção Óssea , Desidratação , Insuficiência de Crescimento , Seguimentos , Concentração de Íons de Hidrogênio , Hipercalciúria , Túbulos Renais Distais , Nefrocalcinose , Prótons , Compostos de Amônio Quaternário , Raquitismo , Vômito
17.
Journal of Bacteriology and Virology ; : 210-216, 2013.
Artigo em Coreano | WPRIM | ID: wpr-68533

RESUMO

The structure of coxsackievirus and adenovirus receptor's CAR is similar to adhesion molecules. In the adult heart, the majority of CAR localizes at the intercalated disc. Germ line CAR deletion induces embryonic lethality at E11.5 with evidence of a cardiac abnormality. The CAR role as a viral receptor is well known; however, its precise function in the heart for enterovirus infection is not clear. To understand the role of CAR in the cardiac myocyte, we generated cardiac-specific CAR knockout mice using a CAR floxed allele and alpha-MHC-Mer CRE Mer mice. Western blot analysis and immunofluorescent stain of ventricles at 6 weeks after 2 weeks tamoxifen administration, CAR expression was significantly decreased in CAR(f/f) MCM mice but not in CAR(f/f) mice heart. Enterovirus was intraperitoneally infected into CAR(f/f) MCM and CAR(f/f) mice (n=10 each). CAR disruption was dramatically reduced virus infection and replication in the heart but not different in liver, spleen, and pancreas. Cardiac myocyte damage was significantly reduced in the CAR(f/f) MCM mutant mice by evans blue dye stain. In addition, the CAR(f/f) MCM mutant mice heart inflammation and fibrosis were decreased in H&E and trichrome stain compare to CAR(f/f) control mice. CAR expression was required for normal ventricular function, but it is the cause of enterovirus infection. In the adult mice heart, CAR deletion was significantly reduced viral infection, proliferation, and myocarditis. These results suggested that CAR deletion could be useful therapeutic strategy to prevent viral myocarditis.


Assuntos
Adulto , Animais , Humanos , Camundongos , Adenoviridae , Alelos , Compostos Azo , Western Blotting , Enterovirus , Infecções por Enterovirus , Amarelo de Eosina-(YS) , Azul Evans , Fibrose , Células Germinativas , Coração , Inflamação , Fígado , Verde de Metila , Camundongos Knockout , Miocardite , Miócitos Cardíacos , Pâncreas , Receptores Virais , Baço , Tamoxifeno , Função Ventricular , Vírus
18.
Annals of Pediatric Endocrinology & Metabolism ; : 19-25, 2013.
Artigo em Inglês | WPRIM | ID: wpr-45899

RESUMO

PURPOSE: The progressivity of central precocious puberty (CPP) seems to depend on the age at presentation. We evaluated the clinical courses of CPP girls according to their age at initiation of treatment. METHODS: One hundred thirty five girls with CPP diagnosed between Jan. 2003 and Dec. 2009 and regularly followed for more than one year were included. They were treated with gonadotropin-releasing hormone agonists (GnRHa) every four weeks. Subjects were divided into two groups based on whether they were treated before (Group I, N=20) or after seven years of age (Group II, N=115). We compared the anthropometric parameters, the predicted adult height (PAH), predicted treatment periods, and the laboratory findings of the two groups every six months. RESULTS: Out of 135 CPP patients, 123 were idiopathic and twelve had neurogenic problems. At the baseline, patients' average bone age (BA) was significantly older than chronologic age (CA) and PAH was significantly shorter than target height (TH). BA and CA were significantly older in group II, but the BA/CA ratio was significantly greater in group I. The average treatment period required to overcome the CA-BA difference was 4.64 yr (group I vs II; 7.98 yr vs 4.24 yr, P < 0.01), and the period needed to overcome PAH-TH difference was 2.49 yr (group I vs II; 4.37 yr vs 2.32 yr, P < 0.01). CONCLUSION: Among the girls with CPP, the younger age group had more advanced BA than CA, and needed significantly longer treatment periods to overcome the BA-CA gap and PAH-TH gaps.


Assuntos
Adulto , Humanos , Hormônio Liberador de Gonadotropina , Piperazinas , Puberdade Precoce
19.
Journal of Korean Medical Science ; : 325-328, 2012.
Artigo em Inglês | WPRIM | ID: wpr-226775

RESUMO

Pericarditis is a rare manifestation of tuberculosis (Tb) in children. A 14-yr-old Korean boy presented with cardiac tamponade during treatment of pulmonary tuberculosis. He developed worsening anemia and persistent fever in spite of anti-tuberculosis medications. Echocardiography found free floating multiple discoid masses in the pericardial effusion. The masses and exudates were removed by pericardiostomy. The masses were composed of pink, amorphous meshwork of threads admixed with degenerated red blood cells and leukocytes with numerous acid-fast bacilli, which were confirmed as Mycobacterium species by polymerase chain reaction. The persistent fever and anemia were controlled after pericardiostomy. This is the report of a unique manifestation of Tb pericarditis as free floating masses in the effusion with impending tamponade.


Assuntos
Adolescente , Humanos , Masculino , Tamponamento Cardíaco/etiologia , Ecocardiografia , Derrame Pericárdico/diagnóstico , Pericardiectomia , Pericardite Tuberculosa/complicações
20.
Annals of Pediatric Endocrinology & Metabolism ; : 33-38, 2012.
Artigo em Coreano | WPRIM | ID: wpr-89112

RESUMO

PURPOSE: The aim of this study was to observe clinical course including remission rate during antithyroid medication for Graves' disease in children and adolescents, and to evaluate factors related to remission. METHODS: This is a retrospective study of 42 patients (8 males), who were diagnosed at pediatric endocrine clinic in Chungbuk National University Hospital from January 1994 to December 2009. They were treated with antithyroid drugs only and were followed for at least 2 years. Their average age at diagnosis was 11.5 +/- 3.4 years, and average follow-up period was 4.5 +/- 2.2 years. RESULTS: At diagnosis, 64.3% of the subjects were in puberty, 87.8% showed goiter which was significantly prevalent in females (P < 0.05), and 38.9% manifested exophthalmos. Thyrotropin receptor antibody was positive in all the patients, whereas anti-microsomal antibody was positive in 88.1% and anti-thyroglobulin antibody in 81.0%. Twenty two (52.4%) patients remitted with 4.3 +/- 2.5 years' medication, and the others were continuously medicated at the last follow-up of 4.8 +/- 1.8 years. By quartile estimate for the remission time, 25 percentile was remitted at 3.7 years, 50 percentile at 7.1 years, and 75 percentile at 9.2 years. Among the factors for the remission, initial thyroid stimulating hormone level was significantly lower in non-remission group compared to remission group (P < 0.05). CONCLUSION: In pediatric Graves' disease treated only antithyroid drugs, the time required for remission is so long that it is necessary to establish fixed medication period and to choose definitive treatment modality as in adult Graves' disease through multicenter clinical study.


Assuntos
Adolescente , Adulto , Criança , Feminino , Humanos , Antitireóideos , Autoanticorpos , Exoftalmia , Seguimentos , Bócio , Doença de Graves , Puberdade , Receptores da Tireotropina , Estudos Retrospectivos , Fatores de Risco , Tireotropina
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