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1.
Children (Basel) ; 10(2)2023 Feb 07.
Artigo em Inglês | MEDLINE | ID: mdl-36832443

RESUMO

This prospective case-cohort study examines the developmental pathway choices of 79 young people (13.25-23.75 years old; 33 biological males and 46 biological females) referred to a tertiary care hospital's Department of Psychological Medicine (December 2013-November 2018, at ages 8.42-15.92 years) for diagnostic assessment for gender dysphoria (GD) and for potential gender-affirming medical interventions. All of the young people had attended a screening medical assessment (including puberty staging) by paediatricians. The Psychological Medicine assessment (individual and family) yielded a formal DSM-5 diagnosis of GD in 66 of the young people. Of the 13 not meeting DSM-5 criteria, two obtained a GD diagnosis at a later time. This yielded 68 young people (68/79; 86.1%) with formal diagnoses of GD who were potentially eligible for gender-affirming medical interventions and 11 young people (11/79; 13.9%) who were not. Follow-up took place between November 2022 and January 2023. Within the GD subgroup (n = 68) (with two lost to follow-up), six had desisted (desistance rate of 9.1%; 6/66), and 60 had persisted on a GD (transgender) pathway (persistence rate of 90.9%; 60/66). Within the cohort as a whole (with two lost to follow-up), the overall persistence rate was 77.9% (60/77), and overall desistance rate for gender-related distress was 22.1% (17/77). Ongoing mental health concerns were reported by 44/50 (88.0%), and educational/occupational outcomes varied widely. The study highlights the importance of careful screening, comprehensive biopsychosocial (including family) assessment, and holistic therapeutic support. Even in highly screened samples of children and adolescents seeking a GD diagnosis and gender-affirming medical care, outcome pathways follow a diverse range of possibilities.

2.
Med J Aust ; 213(1): 30-43, 2020 07.
Artigo em Inglês | MEDLINE | ID: mdl-32578226

RESUMO

INTRODUCTION: The incidence of type 2 diabetes mellitus has increased in children and adolescents due largely to the obesity epidemic, particularly in high risk ethnic groups. ß-Cell function declines faster and diabetes complications develop earlier in paediatric type 2 diabetes compared with adult-onset type 2 diabetes. There are no consensus guidelines in Australasia for assessment and management of type 2 diabetes in paediatric populations and health professionals have had to refer to adult guidelines. Recent international paediatric guidelines did not address adaptations to care for patients from Indigenous backgrounds. MAIN RECOMMENDATIONS: This guideline provides advice on paediatric type 2 diabetes in relation to screening, diagnosis, diabetes education, monitoring including targets, multicomponent healthy lifestyle, pharmacotherapy, assessment and management of complications and comorbidities, and transition. There is also a dedicated section on considerations of care for children and adolescents from Indigenous background in Australia and New Zealand. CHANGES IN MANAGEMENT AS A RESULT OF THE GUIDELINES: Published international guidelines currently exist, but the challenges and specifics to care for children and adolescents with type 2 diabetes which should apply to Australasia have not been addressed to date. These include: recommendations regarding care of children and adolescents from Indigenous backgrounds in Australia and New Zealand including screening and management; tighter diabetes targets (glycated haemoglobin, ≤ 48 mmol/mol [≤ 6.5%]) for all children and adolescents; considering the use of newer medications approved for adults with type 2 diabetes under the guidance of a paediatric endocrinologist; and the need to transition adolescents with type 2 diabetes to a diabetes multidisciplinary care team including an adult endocrinologist for their ongoing care.


Assuntos
Diabetes Mellitus Tipo 2/diagnóstico , Diabetes Mellitus Tipo 2/terapia , Adolescente , Australásia/epidemiologia , Criança , Comorbidade , Complicações do Diabetes/diagnóstico , Complicações do Diabetes/epidemiologia , Complicações do Diabetes/terapia , Diabetes Mellitus Tipo 2/epidemiologia , Feminino , Humanos , Hipoglicemiantes/uso terapêutico , Estilo de Vida , Masculino , Programas de Rastreamento/normas , Educação de Pacientes como Assunto/normas , Transição para Assistência do Adulto/normas
3.
Clin Endocrinol (Oxf) ; 93(2): 97-103, 2020 08.
Artigo em Inglês | MEDLINE | ID: mdl-32301148

RESUMO

BACKGROUND: Adrenal crises (AC) are acute episodes of adrenal insufficiency (AI). Manifestations include hypotension and electrolyte disturbances. Glucocorticoid stress dosing (SD) can prevent AC progression, but its effect on physiological parameters has not been assessed in a 'real world setting'. AIMS: To assess the effect of prior self-managed glucocorticoid dose escalation on physiological markers in children with congenital adrenal hyperplasia (CAH) presenting to hospital for an acute illness. METHODS: An audit of records of all children with CAH presenting to paediatric referral hospital between 2000 and 2015. Potassium, sodium and glucose levels, and hypotension were compared between children who had and had not used SD. RESULTS: There were 321 attendances by patients with CAH and an acute illness during the study period. Any form of SD was used by 64.2% (n = 206); intramuscular (IM) hydrocortisone was used by 22.1% (n = 71) and oral only by 41.7% (n = 134). Use of SD (oral and/or IM) was associated with a significantly lower mean potassium level (4.02 ± 0.71 vs. 4.27 ± 0.79 mmol/l, P < .05). Linear regression analysis showed that age (beta: -0.04 years (95% CI -0.06, -0.02)), diarrhoea (beta: -0.41 (95% CI -0.06, -0.02)) and any form of stress dosing (oral, IM or both) (beta: -0.29 (95% CI -0.55, -0.04)) were each independently and significantly associated with potassium levels. SD was not significantly associated with sodium or glucose concentrations or with estimates of hypotension. CONCLUSION: Patient-initiated SD resulted in a significant reduction in hyperkalaemia and lowered mean potassium levels in paediatric patients with CAH but did not alter significantly sodium and glucose concentrations or incidences of hypotension.


Assuntos
Hiperplasia Suprarrenal Congênita , Insuficiência Adrenal , Doença Aguda , Insuficiência Adrenal/tratamento farmacológico , Pressão Sanguínea , Criança , Eletrólitos , Humanos , Hidrocortisona , Recém-Nascido
4.
Front Psychol ; 11: 582688, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-33510668

RESUMO

The current study examines patterns of attachment/self-protective strategies and rates of unresolved loss/trauma in children and adolescents presenting to a multidisciplinary gender service. Fifty-seven children and adolescents (8.42-15.92 years; 24 birth-assigned males and 33 birth-assigned females) presenting with gender dysphoria participated in structured attachment interviews coded using dynamic-maturational model (DMM) discourse analysis. The children with gender dysphoria were compared to age- and sex-matched children from the community (non-clinical group) and a group of school-age children with mixed psychiatric disorders (mixed psychiatric group). Information about adverse childhood experiences (ACEs), mental health diagnoses, and global level of functioning was also collected. In contrast to children in the non-clinical group, who were classified primarily into the normative attachment patterns (A1-2, B1-5, and C1-2) and who had low rates of unresolved loss/trauma, children with gender dysphoria were mostly classified into the high-risk attachment patterns (A3-4, A5-6, C3-4, C5-6, and A/C) (χ2 = 52.66; p < 0.001) and had a high rate of unresolved loss/trauma (χ2 = 18.64; p < 0.001). Comorbid psychiatric diagnoses (n = 50; 87.7%) and a history of self-harm, suicidal ideation, or symptoms of distress were also common. Global level of functioning was impaired (range 25-95/100; mean = 54.88; SD = 15.40; median = 55.00). There were no differences between children with gender dysphoria and children with mixed psychiatric disorders on attachment patterns (χ2 = 2.43; p = 0.30) and rates of unresolved loss and trauma (χ2 = 0.70; p = 0.40). Post hoc analyses showed that lower SES, family constellation (a non-traditional family unit), ACEs-including maltreatment (physical abuse, sexual abuse, emotional abuse, neglect, and exposure to domestic violence)-increased the likelihood of the child being classified into a high risk attachment pattern. Akin to children with other forms of psychological distress, children with gender dysphoria present in the context of multiple interacting risk factors that include at-risk attachment, unresolved loss/trauma, family conflict and loss of family cohesion, and exposure to multiple ACEs.

5.
J Clin Endocrinol Metab ; 105(3)2020 03 01.
Artigo em Inglês | MEDLINE | ID: mdl-31825489

RESUMO

CONTEXT: P450 oxidoreductase deficiency (PORD) is a rare genetic disorder that is associated with significant morbidity. However there has been limited analysis of reported PORD cases. OBJECTIVE: To determine, based on the cohort of reported PORD cases, genotype-phenotype relationships for skeletal malformations, maternal virilisation in pregnancy, adrenal insufficiency, and disorders of sexual development (DSD). DATA SOURCES: PubMed and Web of Science from January 2004 to February 2018. STUDY SELECTION: Published case reports/series of patients with PORD. Eligible patients were unique, had biallelic mutations, and their clinical features were reported. DATA EXTRACTION: Patient data were manually extracted from the text of case reports/series. A malformation score, representing the severity of skeletal malformations, was calculated for each patient. DATA SYNTHESIS: Of the 211 patients published in the literature, 90 were eligible for inclusion. More than 60 unique mutations were identified in this cohort. Four groups of mutations were identified, through regression modeling, as having significantly different skeletal malformation scores. Maternal virilization in pregnancy, reported for 21% of patients, was most common for R457H mutations. Adrenal insufficiency occurred for the majority of patients (78%) and was typically mild, with homozygous R457H mutations being the least deficient. DSD affected most patients (72%), but were less common for males (46XY) with homozygous R457H mutations. CONCLUSIONS: PORD is a complex disorder with many possible mutations affecting a large number of enzymes. By analyzing the cohort of reported PORD cases, this study identified clear relationships between genotype and several important phenotypic features.


Assuntos
Insuficiência Adrenal/genética , Fenótipo de Síndrome de Antley-Bixler/genética , Anormalidades Musculoesqueléticas/genética , Insuficiência Adrenal/patologia , Fenótipo de Síndrome de Antley-Bixler/patologia , Feminino , Genótipo , Humanos , Masculino , Anormalidades Musculoesqueléticas/patologia , Mutação , Fenótipo
6.
Complement Ther Med ; 44: 196-203, 2019 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-31126556

RESUMO

PURPOSE: Childhood cancer survivors are at risk of treatment late-effects. Physical activity represents a necessary complementary therapy and modifiable risk-factor across all ages for many cardio-metabolic late-effects. This study assessed perceived physical activity in Australian and New Zealander childhood cancer survivors. METHODS: We recruited parents of survivors aged <16 years, and adult survivors of childhood cancer aged ≥16 years, ≥5 years since diagnosis, with age-matched controls for comparison. We compared perceived moderate-vigorous physical activity between survivors and controls, using regression to identify associations with physical activity. RESULTS: We recruited 914 participants (570 childhood cancer survivors and 344 age-matched controls). Parents of survivors perceived more moderate-vigorous physical activity than child controls (248 ± 218, 95% Confidence Interval (CI) = 218-280 vs 185 ± 214 min/week, 95% CI = 144-225, p = 0.036), with no perceived difference between adult survivors and controls (125 ± 152, 95% CI = 108-140 vs 160 ± 201 min/week, 95% CI = 132-187, p = 0.477). Twenty-seven percent of child survivors (vs. 14.5% controls) and 30% of adult survivors (vs. 39.4% controls) met recommendations. Adult survivors who received radiotherapy (OR = 0.585, 95% CI = 0.343-0.995, p = 0.048) or not completed university (OR = 1.808, 95% CI = 1.071-3.053, p = 0.027) were less likely to meet recommendations. CONCLUSIONS: Over two-thirds of Australian and New Zealander childhood cancer survivors across all ages are perceived to not meet physical activity recommendations. Adult survivors who had radiotherapy or did not complete university appeared at-risk for low physical activity. PRACTICAL IMPLICATIONS: Physical activity is important for everyone, but critical among childhood cancer survivors due to increased late cardio-metabolic risks. Monitoring survivors' perceived but also objectively measured physical activity as complementary to routine care is warranted, to provide education and motivate survivors to take control of their health.


Assuntos
Exercício Físico/fisiologia , Neoplasias/fisiopatologia , Percepção/fisiologia , Adolescente , Adulto , Austrália , Sobreviventes de Câncer , Estudos de Casos e Controles , Criança , Feminino , Humanos , Masculino , Nova Zelândia , Pais , Grupos Raciais , Fatores de Risco , Sobrevivência , Adulto Jovem
7.
Clin Endocrinol (Oxf) ; 89(5): 577-585, 2018 11.
Artigo em Inglês | MEDLINE | ID: mdl-30086199

RESUMO

OBJECTIVE: Episodes of acute adrenal insufficiency (AI)/adrenal crises (AC) are a serious consequence of congenital adrenal hyperplasia (CAH). This study aimed to assess morbidity from acute illness in CAH and identify factors associated with use of IV hydrocortisone, admission and diagnosis of an AC. METHOD: An audit of acute illness presentations among children with CAH to paediatric hospitals in New South Wales, Australia, between 2000 and 2015. RESULTS: There were 321 acute presentations among 75 children with CAH. Two-thirds (66.7%, n = 214) of these resulted in admission and 49.2% (n = 158) of the patients received intravenous (IV) hydrocortisone. An AC was diagnosed in (9.0%). Prior to presentation, 64.2% (n = 206) had used oral stress dosing and 22.1% (n = 71) had been given intramuscular (IM) hydrocortisone. Vomiting was recorded in 61.1% (n = 196), 32.7% (n = 64) of whom had used IM hydrocortisone. Admission, AC diagnosis and use of stress dosing varied significantly between hospitals. IM use varied from 7.0% in one metropolitan hospital to 45.8% in the regional hospital. Children aged up to 12 months had the lowest levels of stress dosing and IV hydrocortisone administration. Higher numbers of prior hospital attendances for acute illness were associated with increased use of IM hydrocortisone. CONCLUSION: Prehospital and in-hospital management of children with CAH can vary between health services. Children under 12 months have lower levels of stress dosing prior to hospital than other age groups. Experience with acute episodes improves self-management of CAH in the context of acute illness in educated patient populations.


Assuntos
Hiperplasia Suprarrenal Congênita/diagnóstico , Hiperplasia Suprarrenal Congênita/tratamento farmacológico , Hospitais Pediátricos/estatística & dados numéricos , Hidrocortisona/uso terapêutico , Doença Aguda , Administração Intravenosa , Adolescente , Insuficiência Adrenal/diagnóstico , Insuficiência Adrenal/tratamento farmacológico , Criança , Pré-Escolar , Feminino , Hospitalização/estatística & dados numéricos , Humanos , Hidrocortisona/administração & dosagem , Lactente , Injeções Intramusculares , Masculino
8.
J Affect Disord ; 240: 105-112, 2018 11.
Artigo em Inglês | MEDLINE | ID: mdl-30059936

RESUMO

BACKGROUND: Symptoms of anxiety may arise from fear of cancer recurrence and memories of traumatic experiences during treatment. This study aimed to identify changes in mental health and cortisol, a biological marker of stress, associated with oncology surveillance clinic attendance. METHODS: Adolescent and young adult (AYA) survivors of childhood cancer (aged 12-30 years, N = 46) attending a survivorship clinic were recruited. The State-Trait Anxiety Inventory, an anxiety self-rating and open answer question, and salivary cortisol collections were completed two weeks before and one day before clinic, on clinic day and two weeks after. RESULTS: Trait anxiety scores were consistent with the normal population. State anxiety scores two weeks after clinic were significantly lower than baseline (p = 0.02). Cortisol diurnal slopes were flatter than baseline after clinic (p = 0.02). Evening cortisol levels were significantly higher than baseline two weeks post clinic (p = 0.02). LIMITATIONS: Combined results from biological and psychometric assessments can be difficult to interpret. Larger cohorts will further delineate cortisol pathway activity and distress in AYA cancer survivors. CONCLUSIONS: Psychometric evidence indicates that AYA survivors of childhood cancer perceive themselves to be less anxious after a survivorship clinic visit. Biological evidence, however, indicates a dysregulation of the hypothalamic-pituitary-adrenal axis which may be linked to clinic attendance. Weak correlations suggest that cortisol may not be a reliable indicator of self-perceived anxiety. This may be due to confounding lifestyle factors influencing the stress response or potential 'coping strategies' developed during past treatment experience which may, hypothetically, have masked self-perceived anxiety.


Assuntos
Assistência Ambulatorial/psicologia , Ansiedade/metabolismo , Sobreviventes de Câncer/psicologia , Hidrocortisona/metabolismo , Saliva/metabolismo , Adolescente , Adulto , Ansiedade/psicologia , Criança , Ritmo Circadiano , Feminino , Humanos , Sistema Hipotálamo-Hipofisário/metabolismo , Masculino , Inventário de Personalidade , Sistema Hipófise-Suprarrenal/metabolismo , Adulto Jovem
9.
Support Care Cancer ; 26(8): 2743-2750, 2018 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-29500581

RESUMO

PURPOSE: Survivors of pediatric cancer are prone to late effects which require ongoing medical care. Young adult survivors often transition from specialist pediatric care to adult-oriented or community-based healthcare. This study aims to describe the attitudes and experiences of survivors and their parents towards transition barriers and enablers. METHODS: Long-term survivors and parents (of survivors < 16 years) were recruited from 11 hospitals in Australia and New Zealand to participate in a semi-structured telephone interview regarding their transition experiences. Transcribed interviews were coded and content analysis was used to number participants within themes. RESULTS: Thirty-three participants were interviewed, of which 18 were survivors (mean age 26 years, SD = 6.3; mean time since treatment completion 13.3 years, SD = 6.1) and 15 were parents (mean survivor age 15 years, SD = 1.9; mean time since treatment completion 8.4 years, SD = 2.8). Participants described their transition attitudes as positive (55%), neutral (15%), or negative (30%). Key barriers to transition included dependence on pediatric healthcare providers, less confidence in primary care physicians (PCPs), inadequate communication, and cognitive difficulty. Enablers included confidence in and proximity to physicians, good communication, information, independence, and age. CONCLUSIONS: Many survivors face barriers to their transition out of pediatric care. Early introduction to transition, greater collaboration between healthcare professionals, and better information provision to survivors may improve the transition process. Future research of survivors' experience of barriers/enablers to transition is needed. Development of interventions, such as those that address self-management skills, is required to facilitate transition and encourage long-term engagement.


Assuntos
Sobreviventes de Câncer/psicologia , Neoplasias/psicologia , Adolescente , Adulto , Atitude , Atenção à Saúde , Feminino , Humanos , Masculino , Neoplasias/mortalidade , Adulto Jovem
10.
Aust N Z J Obstet Gynaecol ; 57(3): 346-350, 2017 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-28299789

RESUMO

BACKGROUND: Requests for assistance in menstrual management and menstrual suppression are a common, emotive and sometimes controversial aspect of adolescent disability care. AIMS: To review the uptake and outcomes of menstrual suppression among adolescent patients with developmental delay. METHODS: A retrospective review of the medical records of adolescent females with intellectual disability referred for menstrual management to the Paediatric and Adolescent Gynaecology Clinic, Children's Hospital at Westmead, Sydney, for the three-year period between January 1, 2010 and January 1, 2013. RESULTS: Eighty adolescent patients with developmental delay were identified. A third (n = 28) of the patients were pre-menarcheal at first review with parent/caregivers seeking anticipatory advice. Of the post-menarcheal patients, the median age of menarche was 12 years (range 10-15 years). First and second line interventions were documented as were reasons for change where applicable. The combined oral contraceptive pill (COCP) was the most frequently used therapy (67%), and 19 patients in total had a levonorgestrel releasing intrauterine system (LNG-IUS) inserted (31%). Our study population differs from similar previously published groups in the marked absence of the use of depot medroxyprogesterone acetate or the subdermal etonogestrel releasing device. CONCLUSION: As a paediatrician, it is important to address menstrual management issues and allay caregiver concerns with appropriate advice. Our study supports the use of the COCP as sound first line management in achieving menstrual suppression. The LNG-IUS appears to be a favourable second line option. Further investigation into longer-term outcomes and potential complications of device insertion is recommended.


Assuntos
Anticoncepcionais Orais Combinados/uso terapêutico , Deficiências do Desenvolvimento/psicologia , Dispositivos Intrauterinos Medicados , Levanogestrel/administração & dosagem , Menstruação/psicologia , Adolescente , Comportamento do Adolescente , Anti-Inflamatórios não Esteroides/uso terapêutico , Cuidadores/psicologia , Criança , Comportamento Infantil , Feminino , Humanos , Menarca , Menstruação/efeitos dos fármacos , Metrorragia/tratamento farmacológico , Pais/psicologia , Estudos Retrospectivos
11.
J Adolesc Young Adult Oncol ; 5(4): 344-350, 2016 12.
Artigo em Inglês | MEDLINE | ID: mdl-27195593

RESUMO

PURPOSE: Childhood cancer survivors (CCS) are at increased risk of primary gonadal insufficiency (PGI). This study evaluated the prevalence and clinical characteristics of PGI in CCS. METHODS: In this single-center, retrospective, observational, longitudinal study, we characterized CCS with PGI attending the oncology Long-Term Follow-Up (LTFU) Clinic at an Australian university hospital (January 2012-August 2014). From a cohort of 276 CCS, 54 (32 males) met criteria for PGI: elevated gonadotropins plus low estradiol/amenorrhoea (females) or low testosterone/small testicles for age (males). RESULTS: Median age at primary diagnosis was 4.8 years (inter-quartile range [IQR] 3.0-9.7 years) and at LTFU, it was 22.3 years (IQR 18.2-25.7 years). Fifty-three participants (98.1%) were treated with known highly gonadotoxic therapies: alkylating chemotherapy (96.3%), radiotherapy (70.3%), total body irradiation (29.6%), bone marrow transplantation (51.9%), or multimodal protocols (68.5%). At primary diagnosis, 86.7% participants were Tanner stage I and at LTFU, 89.1% participants were Tanner stage V. More females (95.5%; n = 21) than males (40.6%; n = 13) were treated with hormone development therapy (HDT) (p < 0.01). Of these, more than half (n = 18; 7 males) required pubertal induction. There was no significant difference in serum luteinizing hormone/follicle stimulating hormone (LH/FSH), testosterone/estradiol between those untreated and those treated with HDT. Among those on HDT, 60.7% had persistently elevated FSH±LH and 33.3% had low testosterone or estradiol. Six males had semen analysis (five azoospermic, one oligospermic). Psychological assessment was documented in 61.1% of participants, and two-thirds reported fertility concerns. CONCLUSION: PGI is an evolving phenotype that is common in CCS. Suboptimal treatment and non-adherence occur frequently. Ongoing assessment is essential to ensure prompt diagnosis, adequate intervention and to promote HDT adherence.


Assuntos
Sobreviventes de Câncer , Hipogonadismo/etiologia , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Seguimentos , Humanos , Hipogonadismo/diagnóstico , Hipogonadismo/fisiopatologia , Estudos Longitudinais , Masculino , Prevalência , Estudos Retrospectivos
12.
Int J Adolesc Med Health ; 30(1)2016 Apr 09.
Artigo em Inglês | MEDLINE | ID: mdl-27060739

RESUMO

AIM: We investigated the utility of enzyme immunoassay kits for measuring low levels of salivary estradiol and testosterone in adolescents and objectively assessed prevalence of blood contamination. METHODS: Endocrine patients provided plasma and saliva for estradiol (females) or testosterone (males) assay. Saliva samples were also tested with a blood contamination kit. RESULTS: Picomolar levels of salivary estradiol in females failed to show any significant correlation with plasma values (r=0.20, p=0.37). The nanomolar levels of salivary testosterone in males showed a strong correlation (r=0.78, p<0.001). A significant number of saliva samples had blood contamination. After exclusion, correlations remained non-significant for estradiol, but strengthened for testosterone (r=0.88, p<0.001). CONCLUSION: The salivary estradiol enzyme immunoassay is not clinically informative at low levels. Users should interpret clinical saliva with caution due to potential blood contamination. Our data supports the utility of the salivary testosterone enzyme immunoassay for monitoring adolescent boys on hormone developmental therapy.

13.
Int J Endocrinol ; 2016: 5748264, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-26880914

RESUMO

Objective. To examine patterns of hospitalisation for acute medical conditions in children with congenital adrenal hyperplasia (CAH). Design. A retrospective study of hospitalisation using administrative data. Setting. All hospitals in NSW, Australia. Patients. All patients admitted with CAH and a random sample of admissions in patients aged 0 to 18 years without adrenal insufficiency (AI). Main Outcome Measures. Admissions and comorbidities by age and sex. Results. Of 573 admissions for medical problems in CAH children, 286 (49.9%) were in males, and 236 (41.2%) had a principal diagnosis of CAH or had an adrenal crisis (AC). 37 (6.5%) ACs were recorded. An infection was found in 43.5% (n = 249) of the CAH patient admissions and 51.7% (n = 1613) of the non-AI group, p < 0.001. Children aged up to one year had the highest number of admissions (n = 149) and six ACs (four in males). There were 21 ACs recorded for children aged 1-5 years. Older CAH children had fewer admissions and fewer ACs. No in-hospital deaths were recorded. Conclusions. Admission for medical problems in CAH children declines with age. An AC was recorded in 6.5% of the admissions, with the majority of ACs occurring in the 1 to 5 years age group and there were no deaths.

14.
J Adolesc Young Adult Oncol ; 5(1): 24-30, 2016 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-26812459

RESUMO

PURPOSE: Childhood cancer survivors (CCS) are at increased risk of metabolic dysfunction as a late effect of cancer treatment. However, pediatric metabolic syndrome (MetS) lacks a unified definition, limiting the diagnosis of MetS in CCS. This study evaluated individual metabolic health risk factors and potential areas for intervention in this at-risk population. METHODS: This single center, retrospective observational longitudinal study evaluated the metabolic health of all CCS attending an oncology long-term follow-up clinic at a university hospital in Sydney, Australia (January 2012-August 2014). Participants were 276 CCS (52.2% male; mean age 18.0 years; range 6.8-37.9 years), at least 5 years disease free with a broad spectrum of oncological diagnoses. Primary metabolic health risk factors included raised body mass index, hypertension, and hypertransaminasemia. Participants treated with cranial radiotherapy (n = 47; 17.0% of cohort) had additional biochemical variables analyzed: fasting glucose/insulin, HDL/LDL cholesterol, and triglycerides. RESULTS: Hypertension was common (19.0%), with male sex (p < 0.01) and being aged 18 years or above (p < 0.01) identified as risk factors. Cranial irradiation was a risk factor for overweight/obesity (47.8% in cranial radiotherapy-treated participants vs. 30.4%; p = 0.02). Hypertransaminasemia was more prevalent among participants treated with radiotherapy (15.6% vs. 7.3%; p = 0.03), and overweight/obese participants (17.6% vs. 8.2%; p = 0.04). CONCLUSION: Metabolic health risk factors comprising MetS are common in CCS, placing this population at risk of premature adverse cardiovascular consequences. Proactive surveillance and targeted interventions are required to minimize these metabolic complications, and a unified definition for pediatric MetS would improve identification and monitoring.


Assuntos
Síndrome Metabólica/etiologia , Neoplasias/complicações , Sobreviventes/psicologia , Adolescente , Adulto , Índice de Massa Corporal , Criança , Feminino , Humanos , Hipertensão/etiologia , Estudos Longitudinais , Masculino , Síndrome Metabólica/diagnóstico , Neoplasias/psicologia , Neoplasias/terapia , Hepatopatia Gordurosa não Alcoólica/etiologia , Radioterapia/efeitos adversos , Estudos Retrospectivos , Fatores de Risco , Adulto Jovem
15.
J Pediatr Endocrinol Metab ; 26(11-12): 1047-52, 2013.
Artigo em Inglês | MEDLINE | ID: mdl-23843580

RESUMO

BACKGROUND: Central diabetes insipidus (DI) is a rare disorder in children caused by a deficiency of antidiuretic hormone arginine (vasopressin). Desmopressin is the first line agent in management of central DI. However, one of the side effects of desmopressin is water intoxication and hyponatraemia. This study reviews the patterns of desmopressin use and side effects in our institution. METHODS: Retrospective chart review of all patients with central DI followed up in one tertiary centre between 1 January 2008 and 31 December 2010. RESULTS: Forty-one patients (22 males and 19 females) were included. Twelve patients (29.3%) had congenital and 29 patients (70.7%) had acquired DI, mostly as a result of intracranial tumours. Thirty-six (87.8%) patients were on oral desmopressin and the remaining on nasal formulation. The median oral dose was 9.5 (4.2-17.0) µg/kg/day with median frequency of 2.5 (2-3). The median nasal dose was 0.7 (0.4-1.4) µg/kg/day with median frequency of 2.0 (2-3.5). Fourteen patients (34.1%) were switched from nasal to oral desmopressin with the median dose conversion factor of 20.1 (10.7-31.8). Forty percent of patients on nasal desmopressin experienced hypo/hypernatraemia compared to 18.1% on oral, however, there were no significance difference between standardized hypo/hypernatraemia episodes per treatment year. CONCLUSIONS: Oral desmopressin is used in the majority of our patients including infants and toddlers. There is wide inter-individual variation in dose requirement and dosing intervals. Management of central diabetes insipidus remains a challenge in adipsic patients and in young children during intercurrent illness regardless of the desmopressin formulation.


Assuntos
Antidiuréticos/administração & dosagem , Desamino Arginina Vasopressina/administração & dosagem , Diabetes Insípido/tratamento farmacológico , Adolescente , Antidiuréticos/uso terapêutico , Criança , Desamino Arginina Vasopressina/uso terapêutico , Feminino , Humanos , Masculino , Estudos Retrospectivos
18.
Clin Endocrinol (Oxf) ; 71(6): 834-9, 2009 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-19508604

RESUMO

OBJECTIVE: The cortisol response to surgical stress has been frequently studied, and recommendations developed for steroid replacement in adrenally insufficient patients. There are currently no guidelines, however, for adrenal hormone replacement during anaesthesia alone. The objective of this study was to characterize the normal cortisol response to general anaesthesia in the absence of a surgical procedure in children. DESIGN: Prospective observational study. PATIENTS: Thirty-seven children (aged 0.5-7 years) without known endocrine disease or cranial neoplasms undergoing outpatient magnetic resonance imaging, under general anaesthesia for investigation of nonacute problems in a tertiary referral paediatric hospital. MEASUREMENTS: Serum cortisol and salivary cortisol were measured before and after anaesthesia and during recovery. RESULTS: The mean cortisol level was 303 (± 117) nmol/l at induction, 396 (± 241) nmol/l at emergence from anaesthesia and 584 (± 218) nmol/l during recovery. A stress response (increase in serum cortisol >550 nmol/l) occurred in 23% of children at emergence and in 52% of children at recovery. Eight children (31%) actually demonstrated a decrease in cortisol levels during anaesthesia, without an increase in complications. Mean salivary cortisol levels were 6.5 ± 4.8 nmol/l before induction, 23.5 ± 13.8 nmol/l at emergence from anaesthesia and 26.9 ± 21.6 nmol/l during recovery. A stress response (an increase in salivary cortisol greater than seven-fold) occurred in 26% of children during the study. CONCLUSIONS: While some children demonstrated a rise in their cortisol levels in response to anaesthesia without surgery, the response was variable and often more pronounced during recovery. There was consistently no classic stress response.


Assuntos
Anestesia Geral/efeitos adversos , Hidrocortisona/sangue , Hidrocortisona/metabolismo , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Imageamento por Ressonância Magnética , Masculino , Estudos Prospectivos , Saliva/química
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