Steroid 21-hydroxylase deficiency is the most prevalent form of congenital adrenal hyperplasia (CAH), accounting for approximately 95% of cases. With the advent of newborn screening and hormone replacement therapy, most children with CAH survive into adulthood. Adolescents and adults with CAH experience a number of complications, including short stature, obesity, infertility, tumor, osteoporosis, and reduced quality of life. Transition from pediatric to adult care and management of long-term complications are challenging for both patients and health-care providers. Psychosocial issues frequently affect adherence to glucocorticoid treatment. Therefore, the safe transition of adolescents to adult care requires regular follow-up of patients by a multidisciplinary team including pediatric and adult endocrinologists. The major goals for management of adults with 21-hydroxylase deficiency are to minimize the long-term complications of glucocorticoid therapy, reduce hyperandrogenism, prevent adrenal or testicular adrenal rest tumors, maintain fertility, and improve quality of life. Optimized medical or surgical treatment strategies should be developed through coordinated care, both during transition periods and throughout patients' lifetimes. This review will summarize current knowledge on the management of adults with CAH, and suggested appropriate approaches to the transition from pediatric to adult care.
Cell-cell adhesion managed by various adhesion molecules plays an important role in regulating functional activation of cells. This event mediates attachment of inflammatory cells to endothelial cells, interaction of antigen-presenting cells with T cells and metastatic adherence of cancer cells to epithelial tissue cells. Therefore, this cellular response is considered as one of therapeutic target to treat various cancers and inflammatory diseases. To develop proper model for evaluation of functional activation of adhesion molecules, the ability of U937 and Jurkat T cells responsive to various adhesion inducers such as phorbal-12-myristate-13-acetate (PMA), staurosporin and monoclonal antibodies to CD29, CD43 and CD98 was investigated using quantitative cell-cell adhesion assay. U937 cells made more cell-cell clusters by the treatment of antibodies to CD29 and CD43 than Jurkat T cells, while Jurkat T cells exhibited increased cell-cell adhesion ability in CD98 antibody treatment. In agreement, the surface levels of CD29 and CD98 were highly observed in U937 and Jurkat T cells, respectively. Therefore, our data suggest that Jurkat T and U937 cells can be used for model system to evaluate functional activation of adhesion molecules such as CD29 and CD98.
Paragangliomas are rare neuroendocrine tumors that are usually benign in nature. They may be either familial or sporadic in their occurrence. Numerous neuroendocrine tumors are collectively included under the umbrella of paragangliomas. Among them, carotid body tumors and glomus jugulare tumors are extremely rare. Thus, we present a rare case of 29-year-old male who was admitted with hearing difficulties and tinnitus in the left ear, with swelling on the left side of the neck. Based on clinical and radiological findings, a diagnosis of left-sided glomus jugulare with carotid body tumor was made. The patient underwent a two-stage surgery with an interval of approximately 2 months. Histopathology revealed a paraganglioma. Herein, we present the clinical features, imaging findings, management, and a brief review of literature on the classification, evaluation, and management of carotid body and glomus jugulare tumors. Paraganglioma is a slow-growing tumor. The synchronous occurrence of carotid body and glomus jugulare tumors is infrequent. Microsurgical resection remains the primary treatment modality. Therefore, our patient underwent two-stage surgery. The rarity of occurrence and the proximity and adherence to vital neurovascular structures have resulted in the treatment of paragangliomas remaining a challenge.
The prevalence of obesity in children and adolescents is increasing worldwide. Obesity in children and adolescents not only increases the risk of transitioning to obesity in adulthood but also increases the risk of cardiometabolic diseases such as high blood glucose, high blood pressure, dyslipidemia, fatty liver, and hyperinsulinemia during childhood. The goal of treating obesity in children and adolescents is not to focus on weight loss but to help children reach a healthy weight while maintaining normal growth appropriate for their age and sex. To achieve this goal, regular physical activity and exercise, dietary modification, improvement of obesity-prone environmental factors, and behavioral changes are required for a healthy lifestyle. If appropriate weight control is not achieved through lifestyle modifications, pharmacotherapy may be considered for adolescents with severe obesity aged 12 and above. Recent clinical trials have reported the efficacy and safety of pharmacotherapy in severely obese adolescents. Currently, two medications can be prescribed in Korea for patients with obesity aged 12 and above: Orlistat and Liraglutide. However, despite effective weight control through drug treatment, body weight may increase again after treatment discontinuation. Therefore, it is crucial to evaluate adherence to health behaviors during visits and continue to educate on lifestyle modifications, even during pharmacotherapy, to minimize weight regain.
Roder, David M.;Silva, Primali De;Zorbas, Helen N.;Webster, Fleur;Kollias, James;Pyke, Chris M.;Campbell, Ian D.
Asian Pacific Journal of Cancer Prevention
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v.13
no.4
/
pp.1675-1682
/
2012
Aim: The study aim was to determine the frequency with which women decline clinicians' treatment recommendations and variations in this frequency by age, cancer and service descriptors. Design: The study included 36,775 women diagnosed with early invasive breast cancer in 1998-2005 and attending Australian and New Zealand breast surgeons. Rate ratios for declining treatment were examined by descriptor, using bilateral and multiple logistic regression analyses. Proportional hazards regression was used in exploratory analyses of associations with breast cancer death. Results: 3.4% of women declined a recommended treatment of some type, ranging from 2.6% for women under 40 years to 5.8% for those aged 80 years or more, and with parallel increases by age presenting for declining radiotherapy (p<0.001) and axillary surgery (p=0.006). Multiple regression confirmed that common predictors of declining various treatments included low surgeon case load, treatment outside major city centres, and older age. Histological features suggesting a favourable prognosis were often predictive of declining various treatments, although reverse findings also applied with women with positive nodal status being more likely to decline a mastectomy and those with larger tumours more likely to decline chemotherapy. While survival analyses lacked statistical power due to small numbers, higher risks of breast cancer death were suggested, after adjusting for age and conventional clinical risk factors, (1) for women not receiving breast surgery for unstated reasons (RR=2.29; p<0.001); and (2) although not approaching statistical significance $p{\geq}0.200$), for women declining radiotherapy (RR=1.22), a systemic therapy (RR1.11), and more specifically, chemotherapy (RR=1.41). Conclusions: Women have the right to choose their treatments but reasons for declining recommendations require further study to ensure that choices are well informed and clinical outcomes are optimized.
Njuguna, F;Burgt, RHM van der;Seijffert, A;Musimbi, J;Langat, S;Skiles, J;Sitaresmi, MN;Ven, PM van de;Kaspers, GJL;Mostert, S
Asian Pacific Journal of Cancer Prevention
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v.17
no.9
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pp.4445-4450
/
2016
Background: This study explored perspectives of health-care providers on childhood cancer treatment in Kenya. Materials and Methods: A self-administered questionnaire was completed by 104 health-care providers in January and February 2013. Results: Seventy six percent of the health-care providers believed cancer to be curable. More doctors than other health-care providers had this positive opinion (p=0.037). The majority of health-care providers (92%) believed that most children with cancer will not be able to finish their treatment due to financial difficulties. They considered that prosperous highly-educated parents adhere better with treatment (88%) and that doctors adhere better with treatment for prosperous highly-educated parents (79%). According to 74% of health-care providers, quality of care is better for prosperous highly-educated parents (74%). Most health-care providers reported giving more explanation (71%), work with greater accuracy (70%) and use less difficult vocabulary (55%) to prosperous more educated families. Only 34% of health-care providers reported they feel more empathy towards patients from prosperous families. Reasons for non-adherence with the protocol according to health-care providers are: family refuses drugs (85%), inadequate supply of drugs at pharmacy (79%), child looks ill (75%), and financial difficulties of parents (69%). Conclusions: Health-care providers' health beliefs and attitudes differ for patients with families having high versus low socio-economic backgrounds.
Kim, Sun-Jin;Lee, Jae-Kwan;Chang, Beom-Seok;Lee, Si-Young;Um, Heung-Sik
Journal of Dental Rehabilitation and Applied Science
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v.32
no.1
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pp.16-23
/
2016
Purpose: The aim of this study was to evaluate whether fluorides at various pH cause changes in the surface roughness of titanium implants that alter the adherence of bacterial biofilms. Materials and Methods: The titanium disks were assigned randomly to the following seven groups according to the fluoride agents and application time (1 minute or 30 minute) used: control (no treatment); group 1 (1.23% acidulated phosphate fluoride [APF] at pH 3.5 for 1 minute); group 2 (1.23% APF at pH 3.5 for 30 minute); group 3 (1.23% APF at pH 4.0 for 1 minute); group 4 (1.23% APF at pH 4.0 for 30 minute); group 5 (2% NaF gel at pH 7.0 for 1 minute); group 6 (2% NaF gel at pH 7.0 for 30 minute). The surface roughness of the titanium disks and the amount of adherent bacteria were measured. Results: Group 2 showed a significantly greater surface roughness than the control group (P < 0.0001). No significant differences in the amount of surface bacteria were observed between the treated samples and the controls. In addition, there were no significant differences in bacterial adherence relative to the incubation period between the treated samples and the controls. Conclusion: The surface roughness of the titanium disks was significantly greater after treatment with APF at pH 3.5 for 30 min compared with that of the controls. In addition, we found that the amount of Porphyromonas gingivalis, Fusobacterium nucleatum, and Aggregatibactor actinomycetemcomitans was similar among all groups
Presented are guidelines for the prevention, diagnosis, and treatment of cow's milk protein allergy (CMPA) which is the most common food allergy in infants. It manifests through a variety of symptoms that place a burden on both the infant and their caregivers. The guidelines were formulated by evaluation of existing evidence-based guidelines, literature evidence and expert clinical experience. The guidelines set out practical recommendations and include algorithms for the prevention and treatment of CMPA. For infants at risk of allergy, appropriate prevention diets are suggested. Breastfeeding is the best method for prevention; however, a partially hydrolyzed formula should be used in infants unable to be breastfed. In infants with suspected CMPA, guidelines are presented for the appropriate diagnostic workup and subsequent appropriate elimination diet for treatment. Exclusive breastfeeding and maternal dietary allergen avoidance are the best treatment. In infants not exclusively breastfed, an extensively hydrolyzed formula should be used with amino acid formula recommended if the symptoms are life-threatening or do not resolve after extensively hydrolyzed formula. Adherence to these guidelines should assist healthcare practitioners in optimizing their approach to the management of CMPA and decrease the burden on infants and their caregivers.
The World Health Organization (WHO) declared the outbreak of H1N1 pandemic in 2009. South Korea also had outbreaks of H1N1 virus and used oseltamivir in large volume with increased reports of adverse drug reaction(ADR). The present study was aimed to investigate the ADR frequency, the factors related to ADR, and characteristics of oseltamivir's ADR. Participants for the study were patients randomly drawn from those who were prescribed oseltamivir for treatment from CHA Bundang Medical Center during October 1 and October 30. The information examined as factors related to ADR were collected by a subsequent cross-sectional telephone survey. The factors are the following; a) age; b) gender; c) patient medical history; d) diagnosis of H1N1 virus; e) adherence; f) whether taking other medication with oseltamivir or not; and g) the number of combined medications. We also asked ADR after taking oseltamivir. Total subjects were 86 patients. The average age is $22.6{\pm}18.48$ years old. The gender was 45.3% women and 54.7% men. Half (50%) of all respondents showed one or more ADR, 67.4% were positively diagnosed for H1N1 virus, and 54.7% were completed the full course of oseltamivir (i.e. twice daily x 5days). The most frequently reported ADR symptoms were: dizziness (15.1%), nausea (11.6%), lethargy (10.4%), diarrhea (10.4%), abdominal pain (8.1%), headache and vomiting (6.9%). ADR classifications by categories are gastro intestinal (44.2%), neuropsychiatric events (22.1%), systemic symptom (20.9%), skin events (5.8%), eye events (4.7%), and other cases (2.3%). The onset of ADR 'after taking 1~3 doses' was 69.7%. No increase in neuropsychiatric events was detected in children and adolescents. No factors examined for the study do have significant influence on the presence of ADR. This study showed that ADR of oseltamivir have occurred in half of the patients. The use of oseltamivir is essential for treatment and prophylaxis of influenza A(H1N1). But mass treatment should be properly monitored for ADR.
Alisha Wehdnesday Bernardo Reyes;Heejin Kim;Tran Xuan Ngoc Huy;Trang Thi Nguyen;Wongi Min;Hu Jang Lee;Jin Hur;John Hwa Lee;Suk Kim
Journal of Microbiology and Biotechnology
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v.33
no.4
/
pp.441-448
/
2023
Brucellosis is a contagious zoonotic disease that infects millions of people annually with hundreds of millions more being exposed. It is caused by Brucella, a highly infectious bacterial species capable of infecting humans with an estimated dose of 10-100 organisms. Sirtuin 1 (SIRT1) has been reported to contribute to prevention of viral diseases as well as a chronic infection caused by Mycobacterium bovis. Here, we investigated the role of SIRT1 in the establishment of Brucella abortus infection in both in vitro and in vivo systems using the reported SIRT1 activators resveratrol (RES), piceatannol (PIC), and ginsenoside Rg3 (Rg3). In RAW264.7 cells, SIRT1 activators did not alter the adherence of Brucella or Salmonella Typhimurium. However, reduced uptake of Brucella was observed in cells treated with PIC and Rg3, and survival of Brucella within the cells was only observed to decrease in cells that were treated with Rg3, while PIC treatment reduced the intracellular survival of Salmonella. SIRT1 treatment in mice via oral route resulted in augmented Brucella resistance for PIC and Rg3, but not RES. PIC treatment favors Th2 immune response despite reduced serum pro-inflammatory cytokine production, while Rg3-treated mice displayed high IL-12 and IFN-γ serum production. Overall, our findings encourage further investigation into the complete mechanisms of action of the different SIRT1 activators used as well as their potential benefit as an effective alternative approach against intracellular and extracellular pathogens.
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