Earlier puberty in boys with type 1 diabetes mellitus compared to a simultaneously recruited group of control adolescents.

BACKGROUND: Recent studies have suggested that there is an earlier age of onset of puberty in healthy boys. However, no study has determined the age of pubertal development in boys with type 1 diabetes (T1D) and compared the results with a simultaneously recruited group of healthy children. OBJECTIVE: The aim of this study was to evaluate the age of pubertal events in boys with TD1 and determine whether the duration of diabetes, metabolic control or insulin dose are associated with the age of puberty in boys with T1D. METHODS: Boys aged 7 to 19 years with T1D (n = 148, age 12.9 ± 3.0 years) and healthy boys recruited from schools (n = 388 controls, age 12.8 ± 2.2 years) were studied. A pediatric endocrinologist evaluated pubertal development. RESULTS: Boys at genital Tanner stage 2 and the final stages of puberty (genital Tanner 4 and 5) were younger than the control group (P = 0.005, P = 0.003, and P = 0.015, respectively). Both groups of boys had a similar age of pubic Tanner stage development. There were no cases of pubertal delay observed in the T1D cohort. There was no association observed between metabolic control with pubertal timing. T1D adolescents had lower height-SDS than the C group at the final stages of puberty. CONCLUSIONS: Boys with T1D who are treated with modern insulin therapy appear to have an earlier age of onset and an earlier age of final pubertal events than a simultaneously studied group of healthy children. These data suggest that pubertal delay is not a frequent problem for male T1D patients.

[Evaluation of bone mineral density in children with vertical infection by HIV]. / Evaluación de la densidad mineral ósea en niños con infección vertical por VIH.

Changes in bone mineral density (BMD) are common in adults infected with human immunodeficiency virus (HIV). There are few studies evaluating bone involvement in children infected. OBJECTIVE: To evaluate BMD in vertically HIV-infected children. METHODS: We studied 53 infected children (8-18 years) from five hospitals. Disease status, nutritional assessment, vitamin D (25-OHD) levels and immunological status were recorded. BMD was measured by densitometry. Descriptive analysis, comparison of means and simple and multiple linear regression were used. RESULTS: 88.7% children were in stage B and C, 57% were eutrophic and 18.9% had short stature. 33.3% had 25-OHD levels < 20 ng / ml. 11%, 6% and 4% of the children had BMD <-2DE in hip, spine and whole body respectively. BMD was correlated with BMI, height, disease stage and years of treatment. Only protease inhibitors (PIs) maintained their significance when adjusted for other variables. CONCLUSION: children infected with HIV had lower BMD by age compared to NHANES III data. The severity of the disease, height, zBMI, years of treatment with antiretrovirals, mainly IP, are related to the reduction of bone mass.

Evaluación de la densidad mineral ósea en niños con infección vertical por VIH / Evaluation of bone mineral density in children with vertical infection by HIV

Resumen Los cambios en la densidad mineral ósea (DMO) son comunes en adultos infectados con virus de la inmunodeficiencia humana (VIH). Existen pocos estudios que evalúen el compromiso óseo en niños. Objetivo: Evaluar la DMO en niños infectados verticalmente por VIH. Métodos: Se estudiaron 53 niños infectados (8-18) de cinco hospitales. Se registró severidad de enfermedad, evaluación nutricional, vitamina D (25-OHD) y estado inmunológico. La DMO se midió mediante densitometría. Se utilizó análisis descriptivo, comparación de medias y regresión lineal simple y múltiple. Resultados: El 88,7% estaban en estadio B y C, 57% eran eutróficos y 18,9% tenían talla baja. El 33,3% presentaba niveles de 25-OHD < 20 ng/ml. El 11%, 6% y 4% de los niños tenían DMO < 2DE en cadera, columna y cuerpo entero, respectivamente. La DMO se correlacionó con IMC, talla, severidad de enfermedad y años de tratamiento. Sólo inhibidores de las proteasas (IP) mantuvieron su significancia al ajustar por otras variables. Conclusión: Los niños infectados con VIH tuvieron DMO más baja por edad comparados con datos de NHANES III. La severidad de la enfermedad, talla, zIMC, los años de tratamiento con anti-retrovirales, principalmente IP, están relacionados con la reducción de la masa ósea.

Changes in bone mineral density (BMD) are common in adults infected with human immunodeficiency virus (HIV). There are few studies evaluating bone involvement in children infected. Objective: To evaluate BMD in vertically HIV-infected children. Methods: We studied 53 infected children (8-18 years) from five hospitals. Disease status, nutritional assessment, vitamin D (25-OHD) levels and immunological status were recorded. BMD was measured by densitometry. Descriptive analysis, comparison of means and simple and multiple linear regression were used. Results: 88.7% children were in stage B and C, 57% were eutrophic and 18.9% had short stature. 33.3% had 25-OHD levels < 20 ng / ml. 11%, 6% and 4% of the children had BMD <-2DE in hip, spine and whole body respectively. BMD was correlated with BMI, height, disease stage and years of treatment. Only protease inhibitors (PIs) maintained their significance when adjusted for other variables. Conclusion: children infected with HIV had lower BMD by age compared to NHANES III data. The severity of the disease, height, zBMI, years of treatment with antiretrovirals, mainly IP, are related to the reduction of bone mass.

[Tenofovir-associated Fanconi`s syndrome and rickets in a HIV infected girl]. / Síndrome de Fanconi y raquitismo hipofosfatémico asociado al uso de tenofovir en una niña infectada con VIH.

Tenofovir (TDF) is an inhibitor of reverse transcriptase nucleotide analogue, although it has good tolerability and high anti-retroviral activity, its effect on the kidney has been a concern. OBJECTIVE: To describe a girl infected with HIV who presented Fanconi syndrome during antiretroviral therapy with TDF. CLINICAL CASE: We describe a HIV-1-infected girl, who after 18 months treatment with TDF presented loss of strength and pain of the lower extremities with functional impairment. Laboratory findings were consistent with Fanconi syndrome. Radiographs showed bilateral hip fracture and wrists. Full recovery of Fanconi syndrome was achieved four months after changing antiretroviral therapy. CONCLUSIONS: TDF-prescribing physicians must be prepared to detect signs and symptoms of renal dysfunction and immediately consider switching to another antiviral drug.

Síndrome de Fanconi y raquitismo hipofosfatémico asociado al uso de tenofovir en una niña infectada con VIH / Tenofovir-associated Fanconi`s syndrome and rickets in a HIV infected girl

El tenofovir (TDF) es un inhibidor de la transcriptasa reversa análogo de nucleótidos, aunque tiene buena tolerabilidad y alta actividad antirretroviral, su efecto sobre el riñón ha sido un motivo de preocupación. Objetivo: Describir el caso de una niña infectada por VIH que presenta síntomas y hallazgos de laboratorio compatibles con un síndrome de Fanconi durante el tratamiento con TDF como parte de su terapia antirretroviral. Caso clínico: Niña infectada por el VIH-1, que después de 18 meses con el tratamiento con TDF presentó pérdida de fuerza y dolor de las extremidades inferiores con deterioro funcional. Los hallazgos de laboratorio fueron compatibles con el síndrome de Fanconi. Las radiografías mostraron fractura bilateral de cadera y muñecas. El síndrome de Fanconi se recuperó por completo cuatro meses después del cambio de terapia antirretroviral. Conclusiones: Los médicos que prescriben TDF deben estar preparados para detectar signos y síntomas indicativos de disfunción renal y considerar de inmediato el cambio del fármaco a otro antirretroviral.

Tenofovir (TDF) is an inhibitor of reverse transcriptase nucleotide analogue, although it has good tolerability and high anti-retroviral activity, its effect on the kidney has been a concern. Objective: To describe a girl infected with HIV who presented Fanconi syndrome during antiretroviral therapy with TDF. Clinical case: We describe a HIV-1-infected girl, who after 18 months treatment with TDF presented loss of strength and pain of the lower extremities with functional impairment. Laboratory findings were consistent with Fanconi syndrome. Radiographs showed bilateral hip fracture and wrists. Full recovery of Fanconi syndrome was achieved four months after changing antiretroviral therapy. Conclusions: TDF-prescribing physicians must be prepared to detect signs and symptoms of renal dysfunction and immediately consider switching to another antiviral drug.

Malignant and benign thyroid nodules after total body irradiation preceding hematopoietic cell transplantation during childhood.

BACKGROUND: The risk of radiation-induced benign and malignant thyroid nodules is well known. OBJECTIVE: The aim of this study was to determine the occurrence of thyroid nodules and carcinomas after fractionated total body irradiation (TBI) preceding hematopoietic stem cell transplantation (HSCT) for malignant hematological disease during childhood. METHODS: We conducted a retrospective university hospital-based observational study. The participants were 76 patients receiving fractionated TBI between 1989 and 2009 as part of the conditioning regimen for HSCT to treat malignant hematological disease, with a median age of 8.2 (5.7-11.4) years, for whom the last ultrasound examination was performed at a median age of 14.2 (11.2-17) years. The main outcome measure was cumulative incidence of thyroid nodules detected by ultrasound scans followed by biopsy if necessary. RESULTS: Thyroid nodules were examined in 21 (28%) patients, six (29%) of whom were diagnosed with thyroid carcinoma at the age of 2.2-18.6 years after TBI. The cumulative incidence of nodule occurrence increased with increasing time from diagnosis. The 10-year cumulative incidence of benign and malignant thyroid nodules was 16% (95% confidence interval (CI) 4-27%) and 8% (95% CI 0-16%) respectively. Seventeen (22%) patients had hypothyroidism (compensated n=12, in five patients it was transient). No significant independent risk factors were identified in the multivariable competing risk model as a function of nodule occurrence. CONCLUSION: Short-term and life-long monitoring, with screening for nodules of the thyroid gland using ultrasound scans, is recommended for survivors subjected to TBI for HSCT during childhood.

Menstrual cycle irregularities and their relationship with HbA1c and insulin dose in adolescents with type 1 diabetes mellitus.

OBJECTIVE: To evaluate the prevalence and risk factors of menstrual cycle irregularities in adolescents with type 1 diabetes mellitus. DESIGN: Prospective diary of menstrual cycle. SETTING: Pediatric diabetes clinics and nearby schools. PATIENT(S): Adolescents with type 1 diabetes mellitus treated with multiple daily insulin doses (n = 56) and 56 healthy adolescents. MAIN OUTCOME MEASURE(S): Duration and variability of menstrual cycle. RESULT(S): Duration of the menstrual cycle was 48 ± 39 and 32 ± 7 days in girls with type 1 diabetes mellitus and controls, respectively. Oligomenorrhea (58.9% vs. 19.6%) and amenorrhea (10.7% vs. 1.8%) were more prevalent in girls with type 1 diabetes mellitus than in controls. Oligomenorrhea was observed in 53.3% of the girls with type 1 diabetes mellitus with optimal metabolic control. Girls with an HbA1c level of 7.6% to 8.9% exhibited increased cycle duration, menstrual cycle variability, and prevalence of oligomenorrhea compared with controls. Regression analysis showed that, for each point of increase in HbA1c, the menstrual cycle duration increased by 5.1 days. Cycle variability was associated with a higher daily insulin dose. CONCLUSION(S): Despite optimal metabolic control, a higher prevalence of oligomenorrhea was observed in girls with type 1 diabetes mellitus compared with controls. This is the first report to describe the high variability of the menstrual cycle in type 1 diabetes mellitus. HbA1c and insulin dose are important factors related to menstrual irregularities in type 1 diabetes mellitus.