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1.
BMC Endocr Disord ; 24(1): 63, 2024 May 09.
Artigo em Inglês | MEDLINE | ID: mdl-38724988

RESUMO

BACKGROUND: A chronic autoimmune disease with an increasing incidence rate, type 1 diabetes mellitus (T1DM) is typified by the degeneration of the pancreatic beta cells. Diabetes management is significantly impacted by nutrition. Although it has been demonstrated that following the Mediterranean diet (MD) improves metabolic control with type 2 diabetes in children and adults, its effects on children with T1DM have not received much attention. OBJECTIVE: Therefore, the purpose of this study was to assess whether adherence to Mediterranean diet is associated with better metabolic control and body composition in youths with Type 1 Diabetes Mellitus. The study recruited T1DM patients aged 6-18 years at Istanbul University Cerrahpasa Medical Faculty Hospital's Pediatric Endocrinology and Diabetes Outpatient Clinic for follow-up. METHODS: In addition to demographic variables, some anthropometric measurements, body composition and biochemical parameters such as: Trygliceride(TG), Total cholesterol (TC), High density lipoprotein cholesterol (HDL-C), Low density lipoprotein cholesterol (LDL-C), (Aspartate aminotransferase) AST, Alanine transaminase (ALT) and glycated hemoglobin (HbA1c) was analyzed. The time in range (TIR) is a value obtained from continuous glucose monitoring. KIDMED was used to assess the participants' adherence with the MD. RESULTS: Good adherence to the MD resulted in much larger height SDS than poor adherence. Poor adherence to MD resulted in higher body fat than moderate and good adherence. There is positivite correlation between TIR and KIDMED score. Adherence to MD is negatively associated with HbA1c. The regression anaylsis showed that a one-point rise in the KIDMED score would result in a 0.314-unit reduction in the HbA1c value (p < 0.01). CONCLUSIONS: In conclusion, this study found that adhering to MD led to improved anthropometric measurements, biochemistry, and diabetes outcomes. Awareness among children, adolescents with T1DM, and their parents about the benefits of MD compliance for glycemic and metabolic control should be raised.


Assuntos
Composição Corporal , Diabetes Mellitus Tipo 1 , Dieta Mediterrânea , Humanos , Diabetes Mellitus Tipo 1/metabolismo , Diabetes Mellitus Tipo 1/dietoterapia , Adolescente , Masculino , Feminino , Criança , Seguimentos , Glicemia/metabolismo , Glicemia/análise , Hemoglobinas Glicadas/análise , Hemoglobinas Glicadas/metabolismo , Cooperação do Paciente
2.
Clin Endocrinol (Oxf) ; 99(2): 174-181, 2023 08.
Artigo em Inglês | MEDLINE | ID: mdl-36971427

RESUMO

OBJECTIVE: 12,13-Dihydroxy-9Z-octadecenoic acid (12,13-diHOME) is a lipokine secreted from brown adipose tissue, and it has positive effects on dyslipidemia. Acute exercise has been shown to lead to an increase in its secretion. In this study, it was aimed to investigate the relationship of 12,13-diHOME with obesity, exercise and dyslipidaemia for the first time in the adolescent age group. DESIGN: Prospective study. PATIENTS: Twenty-eight male adolescents with obesity and the same number of age-matched healthy normal-weight male controls. MEASUREMENTS: Fasting serum glucose, insulin, lipid and 12,13-diHOME levels were measured. Cardiopulmonary exercise testing was performed in all subjects using a stress test treadmill. Peak oxygen consumption (peak VO2) and anaerobic threshold heart rate (ATHR) were measured. RESULTS: Adolescents with obesity had lower 12,13-diHOME levels than normal-weight adolescents both before and after acute exercise (p = .025 and p = .019, respectively), and after acute exercise, 12,13-diHOME levels significantly increased in both groups (p = .001 for both). 12,13-diHOME levels negatively correlated with triglyceride, total cholesterol, LDL-C, and positively correlated with HDL-C. Also, peak VO2 and ATHR levels showed positive correlation with 12,13-diHOME levels. CONCLUSION: 12,13-diHOME levels were found to be lower in adolescents with obesity than normal-weight adolescents and increased with acute exercise. Also, the close relationship of this molecule with dyslipidaemia in addition to that with obesity suggests that it has an important role in the pathophysiology of these disorders. Further molecular studies will further elucidate the role of 12,13-diHOME in obesity and dyslipidaemia.


Assuntos
Obesidade Infantil , Humanos , Masculino , Adolescente , Estudos Prospectivos , Exercício Físico , Índice de Massa Corporal
3.
Eat Weight Disord ; 28(1): 36, 2023 Apr 08.
Artigo em Inglês | MEDLINE | ID: mdl-37029851

RESUMO

PURPOSE: It was aimed to investigate the frequency of the risk of diabetes-specific eating disorder (DSED) in adolescents with type 1 diabetes mellitus (T1DM) and to reveal the accompanying psychopathologies. METHODS: Adolescents with T1DM aged 12-18 who applied to the pediatric diabetes outpatient clinic between July 2021 and March 2022 were included. Diabetes Eating Problem Survey-Revised (DEPS-R) was applied to all patients to determine the risk of DSED. In order to detect accompanying psychopathologies, Eating Disorder Examination Questionnaire (EDE-Q), Child Anxiety and Depression Scale-Child version (RCADS) and Parenting Style Scale were applied. After completing the scales, semi-structured interviews were conducted with all patients by a child and adolescent psychiatrist. RESULTS: Ninety-two adolescents (45 boys, 47 girls) were included. DSED risk was found in 23.9% of the cases. A positive correlation was found between DEPS-R and EDE-Q scores (p = 0.001, rho = 0.370). RCADS mean scores were significantly higher in the group with DSED risk (p < 0.001). When the Parenting Style Scale was evaluated, psychological autonomy scores were significantly lower in the group with DSED risk (p = 0.029). As a result of the psychiatric interviews, 30 (32.6%) patients had at least 1 psychiatric disorder. Of these, 2 patients were diagnosed with eating disorder. CONCLUSION: Almost one-fourth of adolescents with T1DM were found to be at risk of DSED. Routine screening of adolescents with T1DM with the DEPS-R scale may provide early detection of DSED, and referral of those at risk to child psychiatry enables early diagnosis and intervention for both eating disorders and accompanying psychopathologies. LEVEL OF EVIDENCE: Level III: Evidence obtained from cohort or case-control analytic studies.


Assuntos
Diabetes Mellitus Tipo 1 , Transtornos da Alimentação e da Ingestão de Alimentos , Masculino , Feminino , Humanos , Adolescente , Criança , Diabetes Mellitus Tipo 1/complicações , Diabetes Mellitus Tipo 1/psicologia , Inquéritos e Questionários , Transtornos da Alimentação e da Ingestão de Alimentos/complicações , Transtornos da Alimentação e da Ingestão de Alimentos/diagnóstico , Comportamento Alimentar , Psicometria
4.
BMC Pediatr ; 21(1): 344, 2021 08 14.
Artigo em Inglês | MEDLINE | ID: mdl-34388993

RESUMO

BACKGROUND: Hypothyroxinemia is defined by low levels of thyroxine (T4) despite low or normal levels of thyroid-stimulating hormone (TSH). This study aimed to evaluate the factors associated with transient hypothyroxinemia of prematurity (THOP) in newborns admitted to the neonatal intensive care unit (NICU). METHOD: This is a single center, retrospective, case-control study. Premature newborns, between 24 and 34 weeks of gestation, hospitalised between January 2014-December 2019 in Istanbul University-Cerrahpasa Faculty of Medicine NICU were analyzed through their medical records. Thyroid function tests were routinely performed between the 10th and 20th days of postnatal life and were evaluated according to the gestational age references. Thirty six possible associated factors (prenatal and postnatal parameters, medical treatments, clinical diagnoses and applications in NICU) were searched in the patient group with THOP (n = 71) and the control group with euthyroid prematures (n = 73). The factors for THOP were identified by univariate analysis, followed by multivariate analysis. RESULTS: Mean gestational ages of the study and the control groups were 29.7 ± 2.48 and 30.5 ± 2.30 weeks, respectively (p = 0.606). The birth weight, small for gestational age (SGA), intraventricular hemorrhage (IVH), congenital heart disease (CHD) were found to be the possible associated factors for THOP in the univariate analysis and CHD (p = 0.007, odds ratio [OR]:4.9, 95% confidence interval [CI]: 1.5-15.8), BW (p = 0.004, OR:0.999, 95% CI: 0.9-1.0) and SGA (p = 0.010, OR:4.6, 95% CI: 1.4-14.7) were found to be factors associated with THOP determined by univariate logistic regression analysis. CONCLUSiONS: Although some treatment practices might have had direct effects on pituitary-thyroid axis, related with the severity of the newborn clinical conditions, non of them was found to be a associated factor for THOP. However, CHD and SGA may be considered as associated factors with THOP detected in preterm infants.


Assuntos
Recém-Nascido Prematuro , Tiroxina , Estudos de Casos e Controles , Feminino , Idade Gestacional , Humanos , Lactente , Recém-Nascido , Gravidez , Estudos Retrospectivos , Tireotropina
5.
Pediatr Nephrol ; 35(7): 1267-1276, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32211992

RESUMO

BACKGROUND: The aim of the study is to identify the effect of salt intake and diabetes itself on blood pressure (BP) profile and microalbuminuria in children with type one diabetes mellitus (T1DM). Our hypothesis is that higher amount of salt consumption and/or hyperglycemia may impair blood pressure pattern in children with T1DM. METHODS: This cross-sectional study included 84 children and adolescents with T1DM (62% females, age 13.9 ± 3.2 years, disease duration 7.3 ± 3.1 years, 43% poorly controlled diabetes) and 54 aged- and sex-matched healthy children with an adequately collected 24-h urine samples. Urine sodium, creatinine, and microalbumin were measured and salt intake was assessed on the basis of sodium excretion in 24-h urine. Blood pressure profile of the children with T1DM was evaluated with 24-h ambulatory blood pressure monitoring. RESULTS: Compared to the children with well-controlled diabetes, children with poorly controlled diabetes had significantly higher standard deviation scores (SDS) of nighttime systolic BP (0.22 ± 1.28 vs - 0.87 ± 0.76, p = 0.003) and lower dipping in diastole (13.4 ± 5.9 vs 18.4 ± 8.1, p = 0.046). Among T1DM group, children with the highest quartile of salt intake had higher nighttime systolic and diastolic BP-SDS (0.53 ± 1.25 vs - 0.55 ± 0.73, p = 0.002 and 0.89 ± 1.19 vs 0.25 ± 0.63, p = 0.038, respectively) and lower dipping in systole compared to their counterparts (7.7 ± 5.0 vs 11.5 ± 6.1, p = 0.040). High averaged HbA1c was independently associated with higher both daytime and nighttime systolic BP-SDS (p = 0.010, p < 0.001) and nighttime diastolic BP-SDS (p = 0.001), and lower diastolic dipping (p = 0.001). High salt intake was independently associated with higher nighttime systolic BP-SDS (p = 0.002) and lower systolic dipping (p = 0.019). A 24-h MAP-SDS was the only independent risk factor for microalbuminuria (p = 0.035). CONCLUSION: Beside poor diabetic control, high salt consumption appears to be an important modifiable risk factor for impaired BP pattern, which contributes to the development of diabetic kidney disease in children with T1DM.


Assuntos
Pressão Sanguínea , Diabetes Mellitus Tipo 1/complicações , Hipertensão/etiologia , Cloreto de Sódio na Dieta/efeitos adversos , Adolescente , Albuminúria/urina , Monitorização Ambulatorial da Pressão Arterial , Estudos de Casos e Controles , Criança , Creatinina/urina , Estudos Transversais , Diabetes Mellitus Tipo 1/urina , Feminino , Hemoglobinas Glicadas/análise , Humanos , Masculino , Fatores de Risco , Cloreto de Sódio na Dieta/urina , Açúcares
9.
Artigo em Inglês | MEDLINE | ID: mdl-38374795

RESUMO

Objectives: We report a patient with papillary thyroid carcinoma (PTC) who developed acute kidney injury (AKI) and elevated creatine kinase (CK) after thyroid hormone withdrawal (THW) prior to radioiodine therapy. Case presentation: A 12-year-old female patient who had undergone total thyroidectomy for PTC one year ago presented with leg pain for the past 2 days. Following THW 3 weeks ago, the case had received 70 mCI radioiodine treatment 6 days ago. Serum creatinine (1.53 mg/dL, normal range [NR]: 0.3-1.1), aspartate aminotransferase (102 IU/L, NR: 0-40) and CK (3451 IU/L, NR: 26-174) levels were elevated. Thyrotropin level was elevated (>100 µIU/ml, NR: 0.51-4.3), and free T4 level was decreased (0.05 ng/dL, NR: 0.98-1.63). Serum creatinine and CK levels decreased after intravenous hydration and levothyroxine treatment. Conclusion: In PTC cases with thyroidectomy, kidney function and CK elevation should be assessed after THW and dehydration should be prevented.

10.
Clin Rheumatol ; 43(1): 399-406, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-37646858

RESUMO

BACKGROUND/OBJECTIVES: The aim of the study is to assess the effect of juvenile idiopathic arthritis (JIA) and biologic disease-modifying anti-rheumatic drugs (bDMARDs) on ovarian reserve in children. MATERIALS AND METHODS: A cross-sectional study was performed from March 2021 to March 2022 and included 81 patients with JIA and 49 healthy children. Serum anti-Mullerian hormone (AMH), follicle-stimulating hormone (FSH), luteinizing hormone (LH), and estradiol levels were analyzed using electrochemiluminescence methods. RESULTS: The mean of current age (13.5 ± 3.2 vs. 14.4 ± 2.4 years), height standard deviation score (SDS) (- 0.35 ± 1.18 vs. - 0.44 ± 0.94), body mass index SDS (0.12 ± 1.33 vs. 0.25 ± 1.28), and the median weight SDS (- 0.13 (- 2.27-3.23) vs. - 0.52 (- 3.4-3.3)) were similar in JIA patients and controls (p > 0.05). Patients with JIA were divided into two groups according to their treatment regimens: treated with methotrexate (MTX) (biologic naive) (n = 32) and treated with MTX plus bDMARDs (n = 49). No significant differences were detected between the 3 groups regarding menarche age, menstrual cycle length, and flow duration (for all p > 0.05). The median serum concentration of AMH was 2.94 (1.12-7.88) ng/ml in the control group, 3.02 (0.36-8.54) ng/ml in the biologic naïve group, and 3.01 (0.99-8.26) ng/ml in the MTX plus bDMARD group. There were no significant differences between 3 groups according to serum AMH, FSH, LH, and estradiol levels (p > 0.05). CONCLUSION: Biologic DMARDs are reassuring in terms of ovarian reserve in girls with JIA and demonstrate that AMH is unaffected by treatment. Prospective studies with larger sample sizes are needed to confirm our findings and to evaluate the impact on the future fertility of patients. Key Points • Although biologic disease-modifying anti-rheumatic drugs (bDMARDs) are being game-changing treatment options in juvenile idiopathic arthritis, their effect on fertility and ovarian reserve is one of the most discussed issues. • In addition to treatment used, autoimmune diseases might also have a negative effect on fertility. • In this cross-sectional study, we found that anti-Mullerian hormone level of patients who were on bDMARDs, patients who were on methotrexate, and healthy controls were similar. • Our results suggest that bDMARDs are reassuring in terms of ovarian reserve in girls with JIA and demonstrate that AMH is unaffected by treatment.


Assuntos
Antirreumáticos , Artrite Juvenil , Produtos Biológicos , Reserva Ovariana , Feminino , Criança , Humanos , Artrite Juvenil/tratamento farmacológico , Metotrexato/farmacologia , Estudos Transversais , Hormônio Antimülleriano , Estudos Prospectivos , Hormônio Luteinizante , Hormônio Foliculoestimulante , Antirreumáticos/uso terapêutico , Antirreumáticos/farmacologia , Estradiol/farmacologia
11.
J Pharm Biomed Anal ; 243: 116072, 2024 Jun 15.
Artigo em Inglês | MEDLINE | ID: mdl-38437786

RESUMO

AIM: Type 1 diabetes (T1D) and its complications are known to be associated with oxidative stress. Pteridine derivatives and indoleamine 2,3-dioxygenase (IDO) activity can be used as biomarkers in the evaluation of oxidative stress. In this study, our aim is to compare the concentrations of serum and urinary pteridine derivatives, as well as serum IDO activity, in children and adolescents diagnosed with T1D and those in a healthy control group. METHOD: A cross-sectional study was performed and included 93 patients with T1D and 71 healthy children. Serum and urine biopterin, neopterin, monapterin, pterin, isoxanthopterin, and pterin-6-carboxylic acid (6PTC) and serum tryptophan and kynurenine levels were analyzed and compared with healthy controls. High-performance liquid chromatography was used for the analysis of pteridine derivatives, tryptophan, and kynurenine. Xanthine oxidase (XO) activity, a marker of oxidative stress, was defined by measurement of serum and urine isoxanthopterin. As an indicator of indolamine 2,3-dioxygenase (IDO) activity, the ratio of serum kynurenine/tryptophan was used. RESULTS: Serum isoxanthopterin and tryptophan concentrations were increased, and serum 6PTC concentration was decreased in children with T1D (p=0.01, p=0.021, p<0.001, respectively). In children with T1D, IDO activity was not different from healthy controls (p>0.05). Serum neopterin level and duration of diabetes were weakly correlated (p=0.045, r=0.209); urine neopterin/creatinine and isoxanthopterin/creatinine levels were weakly correlated with HbA1c levels (p=0.005, r=0.305; p=0.021, r=0.249, respectively). Urine pterin/creatinine level negatively correlated with body mass index-SDS. (p=0.015, r=-0.208). CONCLUSION: We found for the first time that isoxanthopterin levels increased and 6PTC levels decreased in children and adolescents with T1D. Elevated isoxanthopterin levels suggest that the XO activity is increased in TID. Increased XO activity may be an indicator of vascular complications reflecting T1D-related endothelial dysfunction.


Assuntos
Diabetes Mellitus Tipo 1 , Triptofano , Xantopterina , Criança , Adolescente , Humanos , Cinurenina/metabolismo , Neopterina , Creatinina , Estudos Transversais , Pteridinas
12.
Artigo em Inglês | MEDLINE | ID: mdl-38488049

RESUMO

Objective: Treatment adherence is crucial for the success of growth hormone (GH) therapy. Reported nonadherence rates in GH treatment have varied widely. Several factors may have an impact on adherence. Apart from these factors, the global impact of the COVID-19 pandemic, including problems with hospital admission and routine follow-up of patients using GH treatment, may have additionally affected the adherence rate. The primary objective of this study was to investigate adherence to treatment in patients receiving GH. In addition, potential problems with GH treatment during the pandemic were investigated. Materials and Methods: This was a multicenter survey study that was sent to pediatric endocrinologists in pandemic period (June 2021-December 2021). Patient data, diagnosis, history of pituitary surgery, current GH doses, duration of GH therapy, the person administering therapy (either parent/patient), duration of missed doses, reasons for missed doses, as well as problems associated with GH therapy, and missed dose data and the causes in the recent year (after the onset of the pandemic) were queried. Treatment adherence was categorized based on missed dose rates over the past month (0 to 5%, full adherence; 5.1 to 10% moderate adherence; >10% nonadherence). Results: The study cohort consisted of 427 cases (56.2% male) from thirteen centers. Median age of diagnosis was 8.13 (0.13-16) years. Treatment indications were isolated GH deficiency (61.4%), multiple pituitary hormone deficiency (14%), Turner syndrome (7.5%), idiopathic GH deficiency (7.5%), small for gestational age (2.8%), and "others" (6.8%). GH therapy was administered by parents in 70% and by patients in 30%. Mean daily dose was 32.3 mcg/kg, the annual growth rate was 1.15 SDS (min -2.74, max 9.3). Overall GH adherence rate was good in 70.3%, moderate in 14.7%, and poor in 15% of the patients. The reasons for nonadherence were mainly due to forgetfulness, being tired, inability to access medication, and/or pen problems. It was noteworthy that there was a negative effect on adherence during the COVID-19 pandemic reported by 22% of patients and the main reasons given were problems obtaining an appointment, taking the medication, and anxiety about going to hospital. There was no difference between genders in the adherence rate. Nonadherence to GH treatment decreased significantly when the patient: administered the treatment; was older; had longer duration of treatment; and during the pandemic. There was a non-significant decrease in annual growth rate as nonadherence rate increased. Conclusion: During the COVID-19 pandemic, the poor adherence rate was 15%, and duration of GH therapy and older age were important factors. There was a negative effect on adherence during the pandemic period.

13.
Artigo em Inglês | MEDLINE | ID: mdl-38665000

RESUMO

Objective: Maturity-onset diabetes of the young (MODY) occurs due to mutations in genes involved in pancreatic beta cell function and insulin secretion, has heterogeneous clinical and laboratory features, and account for 1-5% of all diabetes cases. The prevalence and distribution of MODY subtypes vary between countries. The aim of this study was to evaluate the clinical and laboratory characteristics, mutation distribution, and phenotype-genotype relationship in a large case series of pediatric Turkish patients genetically diagnosed with MODY. Methods: MODY cases from 14 different pediatric endocrinology departments were included. Diagnosis, treatment, follow-up data, and results of genetic analysis were evaluated. Results: A total of 224 patients were included, of whom 101 (45%) were female, and the mean age at diagnosis was 9.4±4.1 years. Gene variant distribution was: 146 (65%) GCK; 43 (19%) HNF1A; 8 (3.6%) HNF4A, 8 (3.6%) KLF11 and 7 (3.1%) HNF1B. The remaining 12 variants were: PDX (n=1), NEUROD1 (n=3), CEL (n=1), INS (n=3), ABCC8 (n=3) and KJNC11 (n=1). Of the cases, 197 (87.9%) were diagnosed with incidental hyperglycemia, 16 with ketosis (7%) and 7 (3%) with diabetic ketoacidosis (DKA), while 30% presented with classical symptoms of diabetes. Two-hundred (89%) had a family history of diabetes. Anti-GAD antibody was detected in 13 cases, anti-islet antibody in eight and anti-insulin antibody in four. Obesity was present in 16. Distribution of therapy was: 158 (71%) diet only; 23 (11%) intensive insulin treatment; 17 (7.6%) sulfonylureas; 10 (4.5%) metformin; and 6 (2.7%) insulin and oral antidiabetic treatment. Conclusion: This was the largest genetically diagnosed series from Turkey. The most common gene variants were GCK and HNF1A with much lower proportions for other MODY types. Hyperglycemia was the most common presenting symptom while 11% of patients had diabetes-associated autoantibodies and 7% were obese. The majority of patients received dietary management only.

14.
Turk Arch Pediatr ; 58(4): 401-406, 2023 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-37317576

RESUMO

OBJECTIVE: The risk of malignancy in pediatric thyroid nodules is higher compared to the risk in adults. Our aim was to investigate the clinical, radiological, and histopathological characteristics of pediatric thyroid nodules. MATERIALS AND METHODS: The data of 132 children and adolescents who had thyroid nodules were collected retrospectively from medical records. RESULTS: The mean age of the patients was 12.07 ± 4.08 years and 67% were female. Fine-needle aspiration biopsy was performed in 86 patients (65%) and the results were as follows: benign in 53.4% (n = 46), atypia or follicular lesion of undetermined significance in 3.5% (n = 3), suspicious for follicular neoplasia in 2.3% (n = 2), and malignancy in 32.5% (n = 28). The overall malignancy rate was 22.7% (n = 30). Malignancy was detected after surgery in 2 thyroid nodules belonging to the atypia or follicular lesion of undetermined significance category. Malignancy was detected in 7 patients who had autoimmune thyroiditis and in 1 patient who had congenital dyshormonogenesis. The malignancy rate of the nodules in the patients, who had autoimmune thyroiditis, was found to be 13.4%. Mixed echogenicity, microcalcifications, nodules larger than 10 mm, abnormal lymph nodes, and irregular borders were more common in the malignant group. The nodule size, abnormal lymph nodes, and irregular borders were found to be significant in terms of predicting malignancy. CONCLUSION: We found malignancy in 22.7% of the thyroid nodules, and the malignancy rate of nodules in the patients, who had autoimmune thyroiditis, was 13.4%. The nodule size, abnormal lymph nodes, and irregular nodule borders emerged as the most significant risk factors for malignancy.

15.
Arch Endocrinol Metab ; 68: e220524, 2023 Nov 17.
Artigo em Inglês | MEDLINE | ID: mdl-37988665

RESUMO

Objective: This research aimed to evaluate retrospectively the effect of anastrozole on height gain and sex hormone levels in pubertal boys receiving growth hormone (GH). Materials and methods: Pubertal boys who received both GH and anastrozole (GH+A) were one-to-one matched with boys who received only GH (GH-Only) for chronological and bone age, pubertal stage and height before the GH initiation, treatment duration and midparental height. Anthropometric measurements throughout treatment and adult heights were compared between the groups. Sex hormone levels were evaluated longitudinally in the GH+A group. Results: Forty-eight cases (24 in each group) were included. There was no statistical difference in adult height between the GH+A and GH-Only (p = 0.071). However, when the analysis was limited to those receiving anastrozole for at least 2 years, mean adult height was higher in the GH+A than in the GH-Only group (173.1 ± 6.2/169.8 ± 5.6 cm, p = 0.044). Despite similar growth rates between the two groups, bone age advancement was slower in the GH+A than in the GH-Only in a mean anastrozole treatment period of 1.59 years (1.37 ± 0.80/1.81 ± 0.98 years, p = 0.001). The greatest increase for FSH, LH, total and free testosterone and decrease for estradiol levels were observed in the third month after anastrozole was started, albeit remaining within the normal ranges according to the actual pubertal stages. Conclusion: Using anastrozole with GH for at least 2 years decelerates the bone age advancement resulting in adult height gain with no abnormality in sex hormone levels. These results suggest anastrozole can be used as an additional treatment to GH for further height gain in pubertal boys.


Assuntos
Hormônio do Crescimento , Hormônio do Crescimento Humano , Masculino , Adulto , Humanos , Lactente , Anastrozol/farmacologia , Estudos Retrospectivos , Transtornos do Crescimento/tratamento farmacológico , Hormônio do Crescimento Humano/farmacologia , Testosterona , Estatura , Puberdade
16.
Mol Syndromol ; 14(1): 65-70, 2023 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-36777707

RESUMO

Introduction: Primary adrenal insufficiency associated with cardiomyopathy has been rarely reported in children. We report a case of left ventricular (LV) systolic dysfunction related to adrenal insufficiency with autoimmune polyendocrine syndrome type 1 (APS1). Case Presentation: A 7-year-old girl presented with a loss of consciousness. She had hyperpigmentation over joints and enamel hypoplasia. Laboratory tests showed hypoglycemia, hyponatremia, hypocalcemia, and hyperphosphatemia. Endocrine evaluations revealed low serum parathyroid hormone, low cortisol, and high ACTH. Echocardiography showed moderate to severe mitral regurgitation and LV systolic dysfunction. Serum pro-brain natriuretic peptide (pro-BNP) level was high (2,348 pg/mL). Adrenal insufficiency, hypoparathyroidism, and enamel dysplasia suggested APS1. A novel homozygous variant in the AIRE gene, NM_000383, p.Cys322Arg (c.964T>C) confirmed the diagnosis. Calcium, calcitriol, and hydrocortisone treatments were started. Serum pro-BNP level returned to normal, and LV systolic function improved. Conclusion: Here, we present a case of adrenal insufficiency and hypoparathyroidism associated with LV systolic dysfunction whose cardiac findings improved completely with hydrocortisone and calcitriol treatments. Our case is the second reported case of APS1 presenting with LV dysfunction.

17.
Metab Syndr Relat Disord ; 21(1): 35-40, 2023 02.
Artigo em Inglês | MEDLINE | ID: mdl-36576499

RESUMO

Aim: To evaluate the cardiometabolic risk in patients with CAH (21 (OH) enzyme deficiency) on the basis of the visceral adiposity index (VAI), which indicates dysfunction of the visceral adipose tissue (VAT). Materials and Methods: A total of 41 patients and 38 body mass index (BMI), age, and gender-matched healthy controls (HC) were included. The patients' and HCs' age, gender, waist circumference (WC), BMI information and total cholesterol (TC), high-density lipoprotein (HDL), triglyceride (TG) values, smoking, and medication history were obtained from medical charts. Weight, height, WC, and blood pressure levels were measured. Patients' and HCs' BMI, Framingham risk scores (FRS), VAI and Ferriman-Gallwey scores were calculated. The patients' and HCs' age, gender TC, HDL, and TG, androstenedione, dehydroepiandrosterone sulfate (DHEASO4), 17 hydroxyprogesterone (17(OH)P) values, smoking, and medication history were obtained from medical charts. Body fat and muscle mass levels were measured with Tanita T 6360. Results: Gender distribution, mean age, and BMI of patients with CAH were 34/7, 30 ± 8, 27 ± 5.4; HC subjects 30/8, 30 ± 6, 27 ± 3.8 (P = 0.9, 0.6, 0.9, respectively). The VAI values of patients with a diagnosis of CAH 3.7 (2.3-6.9) were found to be significantly higher than those of HC patients 2.5 (1.8-3.9; P = 0.02). The mean glucocorticoid doses of the patients were 17 ± 9 mg/day. The glucocorticoid dose level was determined as independent risk factor on the FRS (P = 0.03, ß = 0.04) and VAI (P = 0.018, ß = 0.17). Conclusion: Glucocorticoid dose optimization should be done more carefully to improve metabolic and cardiovascular outcomes in CAH patients.


Assuntos
Hiperplasia Suprarrenal Congênita , Doenças Cardiovasculares , Humanos , Adiposidade , Hiperplasia Suprarrenal Congênita/complicações , Hiperplasia Suprarrenal Congênita/metabolismo , Glucocorticoides/metabolismo , Índice de Massa Corporal , Obesidade Abdominal/complicações , Lipoproteínas HDL , Triglicerídeos , Doenças Cardiovasculares/diagnóstico , Doenças Cardiovasculares/epidemiologia , Doenças Cardiovasculares/etiologia , Gordura Intra-Abdominal/metabolismo
18.
Sisli Etfal Hastan Tip Bul ; 57(2): 224-231, 2023.
Artigo em Inglês | MEDLINE | ID: mdl-37899815

RESUMO

Objectives: This study aims to investigate the clinical and pathological features of patients with differentiated thyroid cancer (DTC) treated at our tertiary care institution. Methods: Thirty-two children and adolescents followed up with the diagnosis of DTC between 2001 and 2017 were enrolled. We classified patients with DTC into two groups as below and above 10 years of age, and compared their clinical and pathological features. Results: The mean age at presentation was 11.2±4 years. The female/male ratio was 7 (28:4). The diagnosis was papillary thyroid cancer (PTC) in 90.6% (n=29). The frequencies of lymph node and pulmonary metastases were 53.1% and 21.8%, respectively. The groups were comparable in terms of gender, initial clinical signs and tumor histopathology. The mean tumor size was greater in the younger age group (p=0.008). However, there was no difference between the two groups in terms of lymph node and pulmonary metastases. The pathological parameters associated with tumor aggressiveness were also similar between the groups, except lymphovascular invasion. Lymphovascular invasion was more frequent in the younger age group (p=0.01). Patients with lymph node and pulmonary metastases were more likely to have extrathyroidal extension and lymphovascular invasion. Conclusion: PTC was the most common type of DTC and presented with considerable rates of lymph node and pulmonary metastases. Tumor size was greater and lymphovascular invasion was more common in younger patients. Overall prognosis was favorable despite high rates of lymph node and pulmonary metastases.

19.
Turk Arch Pediatr ; 2023 Oct 11.
Artigo em Inglês | MEDLINE | ID: mdl-37818842

RESUMO

OBJECTIVE: Mercury poisoning is a condition with multiple-organ dysfunction that has effects on the central nervous system, gastrointestinal system, cardiovascular system, skin, lungs, and kidneys. It can be fatal or may result in sequelae such as neurological disturbances, if treated late or left untreated. The endocrinological effects of mercury exposure are not well-known. We aimed to evaluate patients with mercury poisoning. MATERIALS AND METHODS: A total of 6 cases of mercury poisoning from 3 families were included in the study. Clinical, laboratory, and follow-up data were recorded. RESULTS: Thyroid dysfunction was presented as high thyroid hormones and normal thyrotropin level (unsuppressed) in 5 cases (83.3%). On the other hand, pheochromocytoma-like syndrome was detected in 5 cases (83.3%) with hypertension. The 4 cases were the first to use methimazole for mercury poisoning due to tachycardia and hypertension despite antihypertensive treatment due to catecholamine excess and thyroid dysfunction. Hyponatremia was detected in 3 cases (50%). CONCLUSION: Mercury poisoning is difficult to diagnose because it is rare and presents with nonspecific physical and laboratory findings. Early diagnosis and providing appropriate treatment are essential in order to prevent sequelae. Mercury poisoning should be considered in patients with unexplained hypertension and tachycardia suggesting the involvement of thyroid hormones and catecholamines.

20.
Endocrinol Diabetes Nutr (Engl Ed) ; 69(3): 201-208, 2022 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-35396118

RESUMO

INTRODUCTION: Metabolic control in type 1 diabetes (T1D) depends on many factors such as eating habits, exercise and lifestyle. The objective of this study was to investigate how these factors were affected during the coronavirus disease 2019 (COVID-19) lockdown and impacted metabolic control in children with T1D. MATERIALS AND METHOD: One hundred children with T1D were enrolled in the study. Anthropometric measurements, snack and meal frequency, carbohydrate consumption, HbA1c levels, and exercise patterns were recorded and compared before and after the lockdown. Subjects were divided into two subgroups-patients with decreased and patients with increased HbA1c levels after the lockdown-and comparisons of the same parameters were also made between these two subgroups. RESULTS: In the overall group, the mean HbA1c level was significantly higher after the lockdown compared to before (p=0.035). Meal schedules changed due to delayed sleep and waking times, and total daily carbohydrate consumption increased in the subgroup with increased HbA1c while it decreased in the subgroup with decreased HbA1c (p<0.001 for both). CONCLUSION: Our study supports the notion that blood sugar management in children with T1D worsened during the COVID-19 pandemic. Although it is not possible to explain this with any one factor, some behavioral changes observed in our study, such as inactivity, irregular meal frequency and timing, and irregular sleep and waking patterns appeared to be associated with blood sugar management.


Assuntos
COVID-19 , Diabetes Mellitus Tipo 1 , Adolescente , Glicemia/metabolismo , Criança , Controle de Doenças Transmissíveis , Hemoglobinas Glicadas , Humanos , Pandemias/prevenção & controle , Quarentena , SARS-CoV-2
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