Incidence of type 1 diabetes in Navarra, 2009-2020. Evidence of a stabilization.

INTRODUCTION: The evolution of the incidence of type 1 diabetes (T1D) in all groups is controversial. Our objective is to study the incidence from 2009 to 2020, based on the Type 1 Diabetes Registry of Navarra, and to analyze the clinical presentation as diabetic ketoacidosis (DKA), and HbA1c at onset. MATERIALS AND METHODS: Descriptive study of all cases diagnosed as T1D and included in the Population Registry of T1D of Navarra in the period 1/1/2009 to 12/31/2020. Data are obtained from primary and secondary sources with an ascertainment rate of 96%. Incidence rates are expressed per 100,000 person-years of risk, by age group and sex. Likewise, a descriptive analysis of the HbA1c and DKA at diagnosis of each patient is performed. RESULTS: 627 new cases are registered, which represents an incidence of 8.1 (10 in men, 6.3 in women), without differences throughout the analyzed period. The group with the highest incidence is the 10-14 years old children (27.8), followed by that of 5-9 years old (20.6). The incidence in people older than 15 years is 5.8. Twenty six percent of patients present DKA at onset. The global mean of HbA1c is 11.6%, without changes throughout the studied period. CONCLUSIONS: The population registry of T1D of Navarra shows a stabilization in the incidence of T1D at all ages in the 2009-2020 period. The percentage of presentation as severe forms is high, even in adulthood.

A national survey on the efficacy and safety of continuous subcutaneous insulin infusion in patients with type 1 diabetes in Spain.

AIMS: To assess safety and benefits of continuous subcutaneous insulin infusion (CSII) therapy in a cohort of type 1 diabetes patients in Spain. METHODS: A web-based national registry was created by the Working Group of the Spanish Diabetes Association. All patients on CSII being followed at selected referral centers were included. A cross-sectional analysis was performed. RESULTS: A total of 1275 patients were included. Data completion for patients on CSII was 67 ±â€¯32%. Indications for treatment were suboptimal glycemic control (32%), high glucose variability (24%), preconception care (14%) and hypoglycemia (11%). In the patients on CSII for ≥1 year (n = 843, mean CSII duration of 5 years), HbA1c decreased by 5 mmol/mol (0.5%) in the whole population and by 8 mmol/mol (0.7%) in subjects with suboptimal glycemic control as CSII indication. Percentage of patients achieving HbA1c ≤ 53 mmol/mol (7%) increased from 20% before CSII to 34% at the end of follow-up. Severe hypoglycemia decreased from 29% to 5%. The rate of discontinuation was 9.5%. HbA1c was lower in patients using bolus advisor and temporary basal rates. CONCLUSIONS: CSII was associated with a sustained improvement in glycemic control and a reduction in severe hypoglycemia. The use of advanced CSII settings was related to better glycemic control.

Incidence and temporal trends of childhood type 1 diabetes between 1975 and 2012 in Navarre (Spain) / Incidencia y tendencia temporal de la diabetes tipo 1 en la infancia, entre 1975 y 2012, en Navarra (España)

Objective: To determine trends in the incidence of type 1 diabetes in Navarre (Spain) between 1975 and 2012 by age and sex. Patients and methods: The study population comprised residents of Navarre under 15 years of age. A Poisson regression model was fitted to analyze changes in the incidence over time, adjusted by year of diagnosis, age group and sex. Results: A total of 494 patients were registered, representing an adjusted incidence rate of 13.2/100,000 person-years. The annual relative increase in the incidence rate was 3.7%. The highest incidence was found in the group aged 10-14 years. The incidence among boys aged 10-14 tended to be higher than that in girls of the same age. Conclusions: Since the year 2000, the incidence of type 1 diabetes among persons younger than 15 years in Navarre has been very high and has quadrupled over the last four decades (AU)

Objetivo: Determinar la tendencia en la incidencia de diabetes tipo 1 en Navarra entre 1975 y 2012 por edad y sexo. Pacientes y métodos: La población objeto de estudio comprende a los residentes en Navarra menores de 15 años de edad. Para analizar la evolución de la incidencia a lo largo del tiempo, se ha utilizado un modelo de regresión de Poisson ajustado por año de diagnóstico, grupo de edad y sexo. Resultados: Se han diagnosticado 494 pacientes, lo que supone una incidencia ajustada de 13,2/100.000 personas-año. El incremento relativo anual en la tasa de incidencia ha sido del 3,7%. El grupo de edad con mayor incidencia fue el de 10 a 14 años. En este mismo grupo, la incidencia en niños tiende a ser mayor que en niñas. Conclusiones: Desde el año 2000, la incidencia de diabetes tipo 1 en menores de 15 años, en Navarra, es muy alta y se ha cuadriplicado en las últimas cuatro décadas (AU)

Incidence and temporal trends of childhood type 1 diabetes between 1975 and 2012 in Navarre (Spain).

OBJECTIVE: To determine trends in the incidence of type 1 diabetes in Navarre (Spain) between 1975 and 2012 by age and sex. PATIENTS AND METHODS: The study population comprised residents of Navarre under 15 years of age. A Poisson regression model was fitted to analyze changes in the incidence over time, adjusted by year of diagnosis, age group and sex. RESULTS: A total of 494 patients were registered, representing an adjusted incidence rate of 13.2/100,000 person-years. The annual relative increase in the incidence rate was 3.7%. The highest incidence was found in the group aged 10-14 years. The incidence among boys aged 10-14 tended to be higher than that in girls of the same age. CONCLUSIONS: Since the year 2000, the incidence of type 1 diabetes among persons younger than 15 years in Navarre has been very high and has quadrupled over the last four decades.

Hipoparatiroidismo hipomagnesémico asintomático inducido por pantoprazol / Hipomagnesémico asymptomatic hypoparathyroidism induced by pantoprazole

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[Cushing's syndrome: special issues]. / Síndrome de Cushing: situaciones especiales.

The present article reviews: Corticotrophin (ACTH) independent bilateral macronodular adrenal hyperplasia, which is characterized by aberrant adrenal receptors due to either ectopic expression or to overexpression (eutopic expression). Micronodular adrenal hyperplasia, which provokes small pigmented nodules in the adrenal gland with atrophy of the internodal tissue. These nodules may not be visible on imaging tests. The term subclinical Cushing's syndrome, coined in 1981, should be used in patients with clinically non-functioning adrenal adenomas but who show autonomous cortisol production that is insufficient to generate overt Cushing's syndrome. This entity must be distinguished from preclinical Cushing's syndrome, given that the subclinical form does not necessarily herald the development of symptoms of hypercortisolism. Cushing's syndrome is uncommon in children and adolescents. Regarding the general incidence of the disease, only 10% of cases are diagnosed in this age group. The most common cause of endogenous Cushing's disease in children older than 7 years is ACTH-dependent Cushing's disease (85%). The association of Cushing's syndrome and pregnancy is highly uncommon, since hypercortisolism usually causes amenorrhea, oligomenorrhea and infertility due to inhibition of gonadotropin secretion. One hundred thirty-six pregnancies have been described in 122 women, with a gestational age at diagnosis of 18.4+/-1 weeks.

Síndrome de Cushing: situaciones especiales / Cushing's syndrome: Special Issues

Se revisan: la hiperplasia suprarrenal macronodular bilateral independiente de corticotropina (ACTH), caracterizada por la expresión aberrante de receptores en la corteza adrenal. Como expresión ectópica o hay una expresión amplificada (expresión eutópica). La hiperplasia adrenal micronodular, que cursa con pequeños nódulos pigmentados en la adrenal con atrofia del tejido internodular. Estos nódulos pueden no ser vistos en las pruebas de imagen. El síndrome de Cushing subclínico, introducido en 1981, debe aplicarse a los pacientes que tienen adenomas adrenales clínicamente no funcionantes, pero con secreción de cortisol autónoma, aunque insuficiente para producir la aparición de un cuadro florido. Conviene distinguirlo del síndrome de Cushing preclínico, dado que no se trata necesariamente de un estado previo a la clínica de hipercortisolismo. El síndrome de Cushing es poco frecuente en niños y adolescentes. De la incidencia general de la enfermedad, sólo el 10% de los casos son diagnosticados en esas edades. La causa más frecuente de síndrome de Cushing endógeno en niños mayores de 7 años es la enfermedad de Cushing dependiente de ACTH (85%). La combinación síndrome de Cushing y embarazo es muy poco frecuente. Su rareza se debe que el hipercortisolismo habitualmente cursa con amenorrea, oligomenorrea e infertilidad por inhibición de la secreción de gonadotropinas. Se han descrito 136 gestaciones en 122 mujeres, con una edad gestacional al diagnóstico de 18,4 ± 1 semanas (AU)

The present article reviews: Corticotrophin (ACTH) independent bilateral macronodular adrenal hyperplasia, which is characterized by aberrant adrenal receptors due to either ectopic expression or to overexpression (eutopic expression). Micronodular adrenal hyperplasia, which provokes small pigmented nodules in the adrenal gland with atrophy of the internodal tissue. These nodules may not be visible on imaging tests. The term subclinical Cushing’s syndrome, coined in 1981, should be used in patients with clinically non-functioning adrenal adenomas but who show autonomous cortisol production that is insufficient to generate overt Cushing’s syndrome. This entity must be distinguished from preclinical Cushing’s syndrome, given that the subclinical form does not necessarily herald the development of symptoms of hypercortisolism. Cushing’s syndrome is uncommon in children and adolescents. Regarding the general incidence of the disease, only 10% of cases are diagnosed in this age group. The most common cause of endogenous Cushing’s disease in children older than 7 years is ACTH-dependent Cushing’s disease (85%). The association of Cushing’s syndrome and pregnancy is highly uncommon, since hypercortisolism usually causes amenorrhea, oligomenorrhea and infertility due to inhibition of gonadotropin secretion. One hundred thirty-six pregnancies have been described in 122 women, with a gestational age at diagnosis of 18.4 ± 1 weeks (AU)

Inédita coincidencia: feocromocitoma sobre glándula suprarrenal atrófica por enfermedad de Addison / An unusual association: pheochromocytoma on an atrophied adrenal gland due to Addison´s disease

El síndrome pluriglandular autoinmunitario (SPGA) tipo II es el síndrome inmunoendocrinopatológico más frecuente. Se define por la aparición de2 o más de las siguientes entidades: insuficiencia suparrenal primaria(enfermedad de Addison), enfermedad de Graves, diabetes mellitus tipo1A, tiroiditis autoinmunitaria, hipogonadismo primario, enfermedad celíaca o miastenia grave. Asimismo, es frecuente que aparezcan también vitíligo, alopecia, anemia perniciosa y/o serositis. La insuficiencia suprarrenal primaria con la que cursan estos pacientes afecta a la corteza adrenal, y ésta es destruida por los auto anticuerpos contra la 21-hidroxilasa. A diferencia de las demás etiologías de la insuficiencia suprarrenal (enfermedades infecciosas, enfermedades infiltrativas, hemorragia, enfermedades tumorales), respeta la médula adrenal. Los feocromocitomas son tumores derivados de las células cromafines del sistema nervioso simpático situadas en la médula adrenal. Pueden cursar con manifestaciones clínicas muy variadas, desde una hipertensión arterial (HTA) aislada o acompañada de episodios paroxísticos, que incluyen la clásica tríada de cefalea, palpitaciones y diaforesis, hasta cuadros potencialmente graves, como edema agudo de pulmón, arritmias o muerte súbita. No hay que olvidar que hasta el 40% son asintomáticos. A continuación, presentamos el caso de una paciente diagnosticada de SPGA tipo II, que desarrolla un feocromocitoma. En esta ocasión, en una glándula adrenal cortical atrofiada se desarrolla un tumor dependiente de la médula adrenal. Esta coexistencia de endocrinopatías, sin conexión etiológica alguna, no deja de ser cuando menos un hallazgo sorprendente, no descrito hasta el momento en la literatura actual (AU)

Autoimmune polyendocrine syndrometype II (APS-II) is the most commonimmunoendocrinopathy syndrome. APS-IIis defined by the development of two or more of the following entities: primaryadrenal insufficiency (Addison’s disease),Graves’ disease, type 1A diabetes mellitus,autoimmune thyroiditis, primaryhypogonadism, celiac disease, and myasthenia gravis. Other frequent clinical findings are vitiligo, alopecia, pernicious anemia and/or serositis. Primary adrenal insufficiency in these patients affects the adrenal cortex, which is destroyed byautoantibodies against 21-hydroxylase.Unlike other causes of adrenal insufficiency (infectious diseases, infiltrative diseases, bleeding, tumors), the adrenal medulla is not involved. Pheochromocytomas are tumors arising from the chromaffin cells of the sympathetic nervous system in the adrenal medulla. The clinical symptoms of these tumors vary from isolated hypertension or hypertension accompanied by paroxysmal episodes —including the classical triad of headache, palpitations and diaphoresis—to potentially serious manifestations such as acute pulmonary edema, arrhythmias and sudden death. Nevertheless, up to 40%of affected patients are asymptomatic. We present the case of a patient diagnosed with APS-II who developed apheochromocytoma. In this patient, the adrenal gland cortex was atrophied andthe tumor was attached to the adrenal medulla. This coexistence ofendocrinopathies, with no etiologic connection, is a surprising finding, which has not previously been described in the current literatura (AU)

An unusual association: pheochromocytoma on an atrophied adrenal gland due to addison's disease.

Autoimmune polyendocrine syndrome type II (APS-II) is the most common immunoendocrinopathy syndrome. APS-II is defined by the development of two or more of the following entities: primary adrenal insufficiency (Addison's disease), Graves' disease, type 1A diabetes mellitus, autoimmune thyroiditis, primary hypogonadism, celiac disease, and myasthenia gravis. Other frequent clinical findings are vitiligo, alopecia, pernicious anemia and/or serositis. Primary adrenal insufficiency in these patients affects the adrenal cortex, which is destroyed by autoantibodies against 21-hydroxylase. Unlike other causes of adrenal insufficiency (infectious diseases, infiltrative diseases, bleeding, tumors), the adrenal medulla is not involved. Pheochromocytomas are tumors arising from the chromaffin cells of the sympathetic nervous system in the adrenal medulla. The clinical symptoms of these tumors vary from isolated hypertension or hypertension accompanied by paroxysmal episodes -including the classical triad of headache, palpitations and diaphoresis-to potentially serious manifestations such as acute pulmonary edema, arrhythmias and sudden death. Nevertheless, up to 40% of affected patients are asymptomatic. We present the case of a patient diagnosed with APS-II who developed a pheochromocytoma. In this patient, the adrenal gland cortex was atrophied and the tumor was attached to the adrenal medulla. This coexistence of endocrinopathies, with no etiologic connection, is a surprising finding, which has not previously been described in the current literature.

Prognostic value of thyroglobulin serum levels and 131I whole-body scan after initial treatment of low-risk differentiated thyroid cancer.

Diagnostic iodine-131 whole-body scan ((131)I-WBS) and serum thyroglobulin values (Tg) performed 6 to 12 months after thyroid ablation for differentiated thyroid carcinoma were evaluated in 194 consecutive patients at the Hospital de Navarra, (Pamplona, Spain). All patients underwent near-total thyroidectomy and (131)I ablation with 3.7 GBq. Patients with positive anti-Tg antibodies or with (131)I uptake outside the neck were previously excluded. Uptake of (131)I in the thyroid bed was detected in 27 patients (13.9%). Serum Tg levels were below 0.5 ng/mL in 133 patients, ranged from 0.5-10 ng/mL in 39 patients, and was above 10 ng/mL in 22 patients. After a follow-up of 7.7 +/- 3.3 years, persistence of the illness has been observed in 2 patients with undetectable Tg (1.5%), but metastases were not detected in any case. In those with Tg higher than 0.5 ng/mL, 29 of 61 patients had persistence of the disease (47.5%) with evidence of metastases in 15 (24.5%), irrespective of the initial total body scan (131)I uptake. In conclusion, serum Tg levels obtained after thyroid ablation has a good prognostic value and permits the selection of patients for further diagnostic studies, while diagnostic (131)I-WBS performed at that time did not correlate with results of Tg and scarcely provides additional information.