RESUMO
Evaluation of the child with abnormal pubertal development can be challenging for the primary care provider. Understanding the factors associated with timing of pubertal onset and the normal sequence of pubertal changes is useful in evaluation of children with puberty disorders. A thorough workup includes assessment of growth rate, Tanner staging, and rate of pubertal progression, in addition to an extensive history and physical examination to identify signs and symptoms of disorders associated with abnormal pubertal timing. Initial diagnostic studies will most often include a bone age, levels of gonadotropins, and levels of estradiol (for girls) or testosterone (for boys).
Assuntos
Transtornos Gonadais/diagnóstico , Transtornos Gonadais/terapia , Atenção Primária à Saúde/organização & administração , Criança , Feminino , Transtornos Gonadais/psicologia , Hormônios Esteroides Gonadais/fisiologia , Humanos , Hipogonadismo/diagnóstico , Hipogonadismo/terapia , Masculino , Puberdade Tardia/diagnóstico , Puberdade Tardia/terapia , Puberdade Precoce/diagnóstico , Puberdade Precoce/terapiaRESUMO
OBJECTIVES: This study describes the hormone profiles for gonadal late effects after alkylator-based hematopoietic stem cell transplant (HSCT) regimens used for sickle-cell disease (SCD). METHODS: This is a retrospective chart review of subjects followed in the post-HSCT clinic for sickle-cell disease. Patient demographics, pubertal development, characteristics of pre-HSCT disease severity, treatment before HSCT, conditioning regimens, presence of graft versus host disease and follicle-stimulating hormone, anti-Müllerian hormone (AMH), luteinizing hormone and testosterone were abstracted from the medical record. RESULTS: Forty subjects (24 female individuals) with SCD were 9 (±4.3) years old at HSCT and 7.9 years (±5.6) from HSCT. At the time of transplant, 8% of female individuals and no male individuals were pubertal and 58% of female individuals and 38% of male individuals had been treated with hydroxyurea. Post-HSCT, all of the female individuals had diminished ovarian reserve on the basis of low AMH values and 10 of the pubertal female individuals (71%) had premature ovarian insufficiency defined as follicle-stimulating hormone >40 mIU/mL ×2. There was no ovarian recovery and AMH remained very low or undetectable up to 13 years post-HSCT. In male individuals, luteinizing hormone and testosterone levels were normal for age. CONCLUSIONS: Post-HSCT for SCD, all female individuals had diminished ovarian reserve and most female individuals had POI, whereas male individuals had normal testosterone hormone production.
Assuntos
Anemia Falciforme/terapia , Transplante de Células-Tronco Hematopoéticas/métodos , Hipogonadismo/epidemiologia , Hipogonadismo/etiologia , Condicionamento Pré-Transplante/efeitos adversos , Alquilantes/efeitos adversos , Hormônio Antimülleriano/sangue , Criança , Feminino , Humanos , Estudos Longitudinais , Hormônio Luteinizante/sangue , Masculino , Reserva Ovariana/efeitos dos fármacos , Insuficiência Ovariana Primária/induzido quimicamente , Estudos Retrospectivos , Testosterona/sangue , Condicionamento Pré-Transplante/métodosRESUMO
STUDY OBJECTIVE: To obtain anti-Müllerian hormone (AMH) levels in female childhood cancer survivors and determine the association of therapeutic exposures with diminished ovarian reserve (DOR). DESIGN: Cross-sectional study. SETTING: Academic medical center. PARTICIPANTS: Forty-nine survivors (mean age = 14.9 years, SD = 3.3 years; mean time without therapy = 7.5 years, SD = 3.6 years) who received alkylator/heavy metal chemotherapy, and/or radiation exposure to the ovaries with 2 or more years without therapy were recruited. INTERVENTIONS: None. MAIN OUTCOME MEASURES: AMH, follicle stimulating hormone (FSH) levels (random), and therapeutic characteristics such as cyclophosphamide equivalent dose (CED), heavy metal exposure, and bilateral ovarian radiation exposure were determined for each subject. DOR was defined as a low AMH (less than the fifth percentile for age-matched controls), and premature ovarian insufficiency as an FSH greater than 40 IU/L with AMH less than the fifth percentile. RESULTS: Fourteen subjects (28.6%) had DOR, and 5 (10.2%) had premature ovarian insufficiency. Those with a low AMH were more likely exposed to a higher CED (P = .001) and/or bilateral ovarian radiation exposure (P = .048). In the multivariate model of DOR adjusted for age at diagnosis, DOR was associated with bilateral radiation (odds ratio = 39.9; 95% confidence interval 2.1-759.7; P = .04). There was a nonsignificant trend with increasing odds of low AMH with increased CED. CONCLUSION: DOR, defined by an AMH less than the fifth percentile, was observed in more than one-quarter of pediatric cancer survivors exposed to gonadotoxic cancer therapy and was significantly associated with bilateral ovarian irradiation. Identifying risk factors for low AMH prompts AMH and FSH surveillance in the early years after cancer therapy and, if needed, early referral to a reproductive specialist.
Assuntos
Hormônio Antimülleriano/sangue , Protocolos de Quimioterapia Combinada Antineoplásica/efeitos adversos , Neoplasias/terapia , Insuficiência Ovariana Primária/sangue , Lesões por Radiação/sangue , Adolescente , Antineoplásicos Alquilantes/efeitos adversos , Estudos Transversais , Ciclofosfamida/efeitos adversos , Feminino , Hormônio Foliculoestimulante/sangue , Humanos , Metais Pesados/efeitos adversos , Ovário/efeitos dos fármacos , Ovário/efeitos da radiação , Insuficiência Ovariana Primária/etiologia , Lesões por Radiação/etiologia , SobreviventesRESUMO
Research has established that childhood cancer treatments can place survivors at risk for reproductive health problems, yet little is known about pediatric survivors' perceptions of their risk for infertility and worry about future family planning. The purpose of this study was to explore factors that affect awareness of risk for and worry about infertility among female pediatric cancer survivors aged 10 to 21 (N=48) and their parents (N=41) following exposure to treatments associated with reproductive late effects. The majority of female childhood cancer survivors (71%) and their parents (95%) reported worry about infertility following gonadotoxic therapy. Cross-sectional data indicated that survivors' awareness of risk for and worry about infertility increase during adolescence, whereas parents' awareness of risk and worry generally remain constant throughout their daughters' development. Survivor worry about infertility was predicted by a variety of factors, yet parent worry about infertility was only associated with increased gonadotoxic radiation exposure. Overall, these findings reinforce the necessity of developmentally appropriate education about reproductive health risks and fertility preservation options across the continuum of pediatric oncology care from diagnosis to survivorship.
Assuntos
Antineoplásicos/efeitos adversos , Conhecimentos, Atitudes e Prática em Saúde , Infertilidade Feminina/etiologia , Neoplasias/terapia , Radioterapia/efeitos adversos , Adolescente , Criança , Estudos Transversais , Feminino , Humanos , Percepção , Inquéritos e Questionários , Sobreviventes , Adulto JovemRESUMO
Gonadal hypofunction is described in male and female patients with sickle cell anemia (SCA) after bone marrow transplant (BMT) and in males treated with hydroxyurea (HU). Anti-Müllerian hormone (AMH) is a serum marker of ovarian reserve. This study describes AMH and follicle-stimulating hormone (FSH) levels in female SCA subjects treated with supportive care (SCA-SC), HU (SCA-HU) and BMT (SCA-BMT). SCA (SS/Sß(0)) subjects not on HU, on HU and status-post BMT, ages 10-21 years were recruited. SCA-HU subjects were treated with HU ≥ 20 mg/kg for ≥ 12 consecutive months. SCA-BMT subjects had received busulfan and cyclophosphamide. Serum AMH and random FSH levels were obtained. Diminished ovarian reserve (DOR) was defined as AMH level <5th percentile for age-matched controls. Subjects also with FSH >40 IU/L were classified as having premature ovarian insufficiency (POI). 14 SCA-SC (14.5 ± 2.7 years), 33 SCA-HU (14.4 ± 2.4 years) and 9 SCA-BMT (14.3 ± 2.7 years) females were included. AMH was undetectable in all SCA-BMT subjects and <5th percentile in 24% of SCA-HU subjects. FSH was menopausal (>40 IU/L) in 88.9% of SCA-BMT subjects. All SCA-BMT subjects and 24% of subjects on HU had DOR; 89% of SCA-BMT subjects had POI. AMH and FSH may be useful tools in assessing ovarian reserve and function.
Assuntos
Anemia Falciforme/terapia , Hormônio Antimülleriano/sangue , Antidrepanocíticos/uso terapêutico , Transplante de Medula Óssea , Hidroxiureia/uso terapêutico , Insuficiência Ovariana Primária/terapia , Adolescente , Anemia Falciforme/sangue , Anemia Falciforme/complicações , Anemia Falciforme/diagnóstico , Biomarcadores/sangue , Bussulfano/uso terapêutico , Estudos de Casos e Controles , Criança , Ciclofosfamida/uso terapêutico , Feminino , Hormônio Foliculoestimulante/sangue , Hemoglobina Falciforme/metabolismo , Heterozigoto , Homozigoto , Humanos , Menarca/fisiologia , Agonistas Mieloablativos/uso terapêutico , Reserva Ovariana/efeitos dos fármacos , Insuficiência Ovariana Primária/sangue , Insuficiência Ovariana Primária/complicações , Insuficiência Ovariana Primária/diagnóstico , Adulto JovemRESUMO
BACKGROUND: Anti-Mullerian Hormone (AMH), a proposed indicator of ovarian follicle reserve in adults, has not been characterized in pediatric and adolescent females by race and/or ethnicity. OBJECTIVES: To describe AMH levels in healthy American girls and determine the influence of age and race/ethnicity on AMH. SUBJECTS: SUBJECTS aged 10-21 years were recruited from primary care settings and emergency departments. Race/ethnicity was characterized as White, Black or Hispanic. METHODS: Serum for AMH levels (ng/mL) was measured using an enzyme-linked immunosorbent assay. RESULTS: Thirty-one White, 60 Black and 24 Hispanic subjects were recruited. Mean AMH levels were 3.19 ng/mL (22.8 pmol/L) (SD 2.12) for Whites, 3.25 ng/mL (23.2 pmol/L) (SD 2.23) for Blacks and 2.97 ng/mL (21.2 pmol/L) (SD 1.75) for Hispanics. ANCOVA showed no difference in AMH levels among race/ethnicities, controlling for age (p=0.91). Age was significantly associated with AMH (p<0.001, R²=0.12). CONCLUSION: AMH levels do not vary by race/ethnicity, and AMH levels increase with age.
Assuntos
Hormônio Antimülleriano/sangue , Etnicidade/estatística & dados numéricos , Reserva Ovariana/fisiologia , Grupos Raciais/estatística & dados numéricos , Adolescente , Adulto , Fatores Etários , Criança , Feminino , Humanos , Estados Unidos/epidemiologia , Adulto JovemRESUMO
BACKGROUND: Abnormalities in BMI are well documented in childhood cancer survivors. Perceptions of BMI status in cancer survivors have been understudied. This study determines the accuracy of parent/survivor and provider reporting of BMI status in a cancer survivor program. PROCEDURE: This is a retrospective study. Parent/survivor assessment of BMI status was obtained from a baseline questionnaire of subjects enrolled in Children's Healthcare of Atlanta-Childhood, Adolescent, and Young Adult Cancer Survivor Study (CHOA-CAYACSS). Provider reporting of BMI was obtained from a clinic visit close in date to completion of the survey. Perceptions of BMI were compared to actual BMI status calculated from clinic visits and categorized based on the Centers for Disease Control and Prevention (CDC) BMI guidelines. RESULTS: Perceptions of BMI were collected from 290 survivors of pediatric cancer or their parents (range, 4.3-22.9 years). Nearly 5% of survivors were underweight, 19.7% overweight and 16.2% obese. High BMI was the BMI state least likely to be correctly identified by parents, survivors, and providers. Among survivors with high BMI, parents, survivors, and providers failed to identify the problem 49.4%, 66.7%, and 26.9% of the time, respectively. Providers were less likely to correctly identify overweight compared to obese status (P < 0.0001). Accuracy of BMI recognition was independent of gender of survivor, ethnicity, or primary cancer diagnosis. CONCLUSION: Abnormal BMI states, especially overweight, are frequently not correctly perceived by parents/survivors or providers. Assessment of BMI status and discussion about steps to normalize BMI is needed to prevent weight related morbidities in this population.