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PURPOSE: We aimed to determine the frequency of transient congenital hypothyroidism (TCH) in 17 participating centers in Türkiye, evaluate the etiological distribution in permanent congenital hypothyroidism (PCH) cases, and investigate the role of laboratory and clinical findings in predicting TCH. METHODS: This retrospective observational multicenter study included patients from 17 pediatric endocrinology centers identified by "National Newborn Screening Program" (NNSP) who were born in 2015 and followed for 6 years. Demographic, clinical, and laboratory information of the cases were compiled through the database http://cedd.saglik-network.org (CEDD-NET). RESULTS: Of the 239 cases initially treated for CH, 128 (53.6%) were determined as transient in whom a trial of levothyroxine (LT4) withdrawal was performed at a median age of 36 (34-38) months. Among the patients with PCH (n = 111), thyroid dysgenesis was diagnosed in 39.6% (n = 44). The predictive factors for TCH were: LT4 dose at the withdrawal of treatment, and initial newborn blood screening (NBS)-TSH level. Based on the receiver operating characteristic (ROC) curve analysis to predict optimal cut-offs for TCH predictors, LT4 dose < 2.0 µg/kg/day at treatment discontinuation was predictive for TCH and was associated with 94.5% specificity and 55.7% sensitivity, with an area under the curve (AUC) of 0.802. The initial NBS-TSH level value < 45 µIU/mL was predictive for TCH with 93.1% specificity and 45.5% sensitivity, with an AUC of 0.641. In patients with eutopic thyroid gland only LT4 dose < 1.1 µg/kg/day at withdrawal time was predictive for TCH with 84.7% sensitivity and 40.4% specificity, with an AUC of 0.750. CONCLUSION: According to our national follow-up data, the frequency of TCH was 53.6%. We determined the LT4 dose < 2.0 µg/kg/day at discontinuation of treatment and the initial NBS-TSH level < 45 µIU/mL as the best cut-off limits to predict TCH.
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The food insulin index (II) is a novel classification to rank foods based on their physiological insulin demand relative to an isoenergetic reference food and may be a valid predictor of postprandial insulin responses and appetite. The present study aimed to compare the postprandial metabolic responses and appetite sensations to two macronutrient- and glycaemic index-matched meals with either high or low II in obese adolescents with insulin resistance (IR). A randomised, single-blind and cross-over trial included fifteen obese adolescents aged 12-18 years with IR. All participants were provided with two different breakfasts: low glycaemic index, low insulin index (LGI-LII) and low glycaemic index, high insulin index (LGI-HII), with a 1-week washout period between meals. At time 0 (just before breakfast), 15, 30, 45, 60, 90, 120, 180 and 240 min after the meal, serum glucose, insulin and C-peptide levels and appetite scores were measured. At the end of 4 h, participants were served ad libitum lunch. Early (0-30 min), late (45-240 min) and total (0-240 min) postprandial insulin responses were lowered by 56·1, 34·6 and 35·6 % after the LGI-LII meal v. LGI-HII meal (P < 0·05). The feeling of hunger was also decreased by 25·8 and 27·5 % after the LGI-LII meal v. LGI-HII meal during the late and total responses (P < 0·05). The calculation II of meals or diets may be a useful dietary approach to reduce postprandial hyperinsulinaemia and the perceived hunger in obese adolescents with IR.
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Apetito/fisiología , Índice Glucémico/fisiología , Resistencia a la Insulina/fisiología , Insulina/sangre , Obesidad Infantil/sangre , Adolescente , Niño , Estudios Cruzados , Dieta/métodos , Femenino , Humanos , Masculino , Comidas , Obesidad Infantil/dietoterapia , Periodo Posprandial/fisiología , Método Simple CiegoRESUMEN
OBJECTIVE: Data regarding pregnancies in relation to pituitary tumors are limited. The effects of pregnancy on pituitary adenomas and the effects of adenoma itself (hormonal activity, mass effects and pituitary insufficiency) and/or treatment on the ongoing gestation and developing fetus were evaluated. METHODS: The study was a retrospective study. A questionnaire involving questions regarding medical history before index gestation, history of related pregnancy, result of index gestation and postpartum follow-up of the patients was filled by the investigator in one of the eight Referral Endocrinology Centers from Turkey. RESULTS: One hundred and thirteen (83 prolactinoma, 21 acromegaly, 8 NFPA and 1 plurihormonal pituitary adenoma) pregnancies of 87 (60 prolactinoma, 19 acromegaly, 7 NFPA and 1 plurihormonal pituitary adenoma) patients were reviewed. The clinically important pregnancy-related tumor growth of pituitary adenomas was found to be low in previously treated adenomas. Prolactinomas were more likely to increase in size during pregnancy especially if effective prior treatment was lacking. The risk of hypopituitarism is also minimal due to pituitary adenomas during pregnancy. The results of pregnancies did not differ in patients who were on medical treatment or not for prolactinomas and acromegaly during gestation. Neural tube defect and microcephaly associated with maternal cabergoline use; Down syndrome and corpus callosum agenesis associated with maternal bromocriptine use; unilateral congenital cataract, craniosynostosis and microcephaly associated with maternal acromegaly were detected for the first time. CONCLUSION: Medical treatment can be safely done stopped in patients with prolactinoma and acromegaly when pregnancy is confirmed and reinstituted when necessary. Prospective studies may help to determine the effects of medical treatment during gestation on the mother and fetus.
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Adenoma/patología , Neoplasias Hipofisarias/patología , Complicaciones Neoplásicas del Embarazo/patología , Prolactinoma/patología , Adenoma/sangre , Adulto , Femenino , Hormona de Crecimiento Humana/sangre , Humanos , Factor I del Crecimiento Similar a la Insulina/metabolismo , Neoplasias Hipofisarias/sangre , Embarazo , Complicaciones Neoplásicas del Embarazo/sangre , Resultado del Embarazo , Prolactina/sangre , Prolactinoma/sangre , Estudios Retrospectivos , TurquíaRESUMEN
INTRODUCTION: Growth hormone resistance defines several genetic (primary) and acquired (secondary) pathologies that result in completely or partially interrupted activity of growth hormone. An archetypal disease of this group is the Laron-type dwarfism caused by mutations in growth hormone receptors. The diagnosis is based on high basal levels of growth hormone, low insulin like growth factor-I (IGF-1) level, unresponsiveness to IGF generation test and genetic testing. Recombinant IGF-1 preparations are used in the treatment CONCLUSION: In this article, clinical characteristics, diagnosis and therapeutic approaches of the genetic and other diseases leading to growth hormone insensitivity are reviewed.
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Resistencia a Medicamentos , Trastornos del Crecimiento/diagnóstico , Trastornos del Crecimiento/terapia , Hormona del Crecimiento/uso terapéutico , Síndrome de Laron/diagnóstico , Síndrome de Laron/terapia , Resistencia a Medicamentos/genética , Trastornos del Crecimiento/tratamiento farmacológico , Hormona de Crecimiento Humana/genética , Hormona de Crecimiento Humana/metabolismo , Humanos , Proteína 3 de Unión a Factor de Crecimiento Similar a la Insulina/genética , Proteína 3 de Unión a Factor de Crecimiento Similar a la Insulina/metabolismo , Factor I del Crecimiento Similar a la Insulina/genética , Factor I del Crecimiento Similar a la Insulina/metabolismo , Receptores de Somatotropina/genéticaRESUMEN
OBJECTIVE: The aim of the present study was to evaluate the levels of serum androgens and prostate-specific antigen (PSA) levels in patients with endometriosis. MATERIALS AND METHODS: Patients with Stage III/IV (advanced stage) endometriosis were compared to controls with respect to basal serum levels of total testosterone (T), free testosterone (fT), androstenedion (A), dehydroepiandrosterone (DHEA), dehydroepiandrosterone-sulphate (DHEA-S), and PSA in the early follicular phase of menstrual cycle for this prospective case control study. RESULTS: Level of T, fT, A, DHEA, and DHEA-S were higher in patients with endometriosis when compared to control subjects, but the difference was not statistically significant. The mean PSA level was 0.0074 +/- 0.0120 ng/ml in patients with endometriosis and 0.0059 +/- 0.0056 ng/ml in control group and there was no statistically significant difference between groups (p = 0.58). CONCLUSION: Serum basal androgens and PSA levels are higher in endometriosis group with respect to control but the differences are not statistically significant.
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Androstenodiona/sangre , Deshidroepiandrosterona/sangre , Endometriosis/sangre , Antígeno Prostático Específico/sangre , Testosterona/sangre , Adulto , Estudios de Casos y Controles , Endometriosis/fisiopatología , Femenino , Fase Folicular/fisiología , Humanos , Estudios Prospectivos , Adulto JovenRESUMEN
BACKGROUND AND OBJECTIVES: Organophosphate insecticides (OPIs) are widely used in agriculture and horticulture for controlling insects in crops, ornamentals, lawns, fruits, and vegetables. But, there have not yet any study about effects of sulforophane (SFN) and curcumin (CUR) on the oxidative stress created by acute toxic effects of malathion (MAL) as an OPI often causing human and animal poisoning. AIM: The aim of this study was to investigate the effects of SFN and CUR on the oxidative stress created in the lung, liver, and kidney tissues of rats by acute MAL toxicity. MATERIALS AND METHODS: Thirty-six mature Sprague Dawley rats weighing 200-250 g were used. The rats were randomly divided into six groups: unmedicated control, SFN, CUR, MAL control, MAL + SFN, and MAL + CUR. Tissue samples were analyzed for glutathione (GSH), malondialdehyde (MDA), and nitric oxide (NO) levels in the lung, liver, and kidney tissues. Biochemical parameters were measured colorimetrically by using a spectrophotometer. RESULTS: No statistically significantly difference was found when comparing the unmedicated control, SFN, and CUR groups. MAL significantly increased MDA levels in the liver and kidney tissues, but SFN and CUR these levels. MAL did significantly reduce the GSH levels, but SFN and CUR increased these levels by blocking the MAL effect in the liver tissues. Also, MAL significantly increased the NO levels, depending on the severity of the tissue damage, and SFN and CUR attenuated to NO levels and remained under the effect of MAL. CONCLUSIONS: SFN and CUR, which showed similar effects, could be used to protect against the oxidative stress caused by acute malathion intoxication.
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Curcumina/farmacología , Malatión/toxicidad , Estrés Oxidativo/efectos de los fármacos , Tiocianatos/farmacología , Animales , Antioxidantes/farmacología , Colorimetría , Femenino , Insecticidas/toxicidad , Isotiocianatos , Riñón/efectos de los fármacos , Riñón/patología , Hígado/efectos de los fármacos , Hígado/patología , Pulmón/efectos de los fármacos , Pulmón/patología , Óxido Nítrico/metabolismo , Distribución Aleatoria , Ratas , Ratas Sprague-Dawley , Índice de Severidad de la Enfermedad , Espectrofotometría , SulfóxidosRESUMEN
A newborn male presented with intestinal malrotation, facial anomalies, hypertrichosis, hypertrophic, hyperpigmented nipples and enlarged genitals with a hyperpigmented scrotum. In addition, the patient displayed a marked lipodystrophy of trunk and limbs. His karyotype demonstrated a small supernumerary NOR-positive marker chromosome that was subsequently identified as del(22)(q12->qter). This extra structurally abnormal chromosome probably derives from a maternal balanced translocation, which was found by karyotype analysis of the mother. The patient's growth hormone (GH) serum levels were elevated, whereas serum insulin-like growth factor 1 (IGF-I) was almost undetectable. Molecular genetic analysis of the IGF-I and type 1 IGF receptor (IGF-IR) genes revealed a heterozygous mutation within exon 21 of the IGF-IR (Pro1257Ser). Findings in our patient correlate to a large extent with partial trisomy 22. Phenotypic variation from classical partial trisomy 22 syndrome may lie within the variability of this syndrome, originate from disturbances within the GH-IGF/IGF-IR axis or, alternatively, reflect the pathogenesis of a new syndrome due to the synergistical impact of the combination of the genetic aberrations. Additional studies are necessary to confirm or refute this hypothesis.
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Anomalías Múltiples/genética , Cromosomas Humanos Par 22 , Lipodistrofia/genética , Mutación , Receptor IGF Tipo 1/genética , Trisomía , Humanos , Recién Nacido , Masculino , SíndromeRESUMEN
OBJECTIVES: Although well-defined principles of rational antimicrobial use are available, inappropriate prescribing patterns are reported worldwide. Accurate information on the usage of antimicrobials, including factors associated with and influencing their use, is valuable for improving the quality of prescription practices. METHODS: In this cross-sectional point prevalence survey, data on patients hospitalized in 12 different children's hospitals were collected on a single day. Appropriateness of prescription was compared between the types of antimicrobials prescribed, indications, wards, and presence of/consultation with an infectious disease physician (IDP). RESULTS: A total 711 of 1302 (54.6%) patients evaluated were receiving one or more antimicrobial drugs. The antimicrobial prescription rate was highest in pediatric intensive care (75.7%) and lowest in the surgery wards (37.0%). Of the 711 patients receiving antimicrobials, 332 patients (46.7%) were found to be receiving at least one inappropriately prescribed drug. Inappropriate use was most frequent in surgery wards (80.2%), while it was less common in oncology wards (31.8%; p<0.001). Respiratory tract infection was the most common indication for antimicrobial use (29.4%). Inappropriate use was more common in deep-seated infections (54.7%) and respiratory infections (56.5%). Fluoroquinolones were used inappropriately more than any other drugs (81.8%, p=0.021). Consultation with an IDP appears to increase appropriate antimicrobial use (p=0.008). CONCLUSIONS: Inappropriate antimicrobial use remains a common problem in Turkish pediatric hospitals. Consultation with an IDP and prescribing antimicrobial drugs according to microbiological test results could decrease the inappropriate use of antimicrobials.
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Antiinfecciosos/administración & dosificación , Prescripciones de Medicamentos/normas , Hospitales Pediátricos , Preescolar , Estudios Transversales , Prescripciones de Medicamentos/estadística & datos numéricos , Revisión de la Utilización de Medicamentos , Humanos , Prevalencia , Turquía/epidemiologíaRESUMEN
Abstract Introduction Ammonia is an important chemical agent used in industry. Accidental inhalation of ammonia has resulted in upper airway and bronchoalveolar injury. If a large amount of ammonia is breathed, the agent can cause unconsciousness, shock and even death. Case In this article, we report a case of non-accidental poisoning in adolescence. The patient was a 14-year-old boy and working as an apprentice at a workshop of a jeweller. He had drunk some amount of diluted ammonia by the force of another, 18-year-old, male apprentice. This patient presented with ammonia intoxication symptoms culminating in coma. Conclusions We reported this case as an unusual ammonia poisoning seen in adolescence.