Síndrome de Cushing secundario a adenoma adrenocortical ectópico

Ectopic secretion of cortisol by adrenocortical adenomas is an extremely rare cause of independent ACTH Cushing syndrome (CS). A clinical case is presented where, due to poorly controlled diabetes mellitus type 2, the presence of hypercortisolism was demonstrated. ACTH levels suppressed determined that it was ACTH independent. Imaging tests and surgery showed that it was a Cushing’s syndrome secondary to ectopic adrenocortical adenoma of the left broad ligament. There are few published cases in the literature, some of them in the ovary, testes or in the renal hilum; so we think it could be interesting and we add another one.La secreción ectópica de cortisol por adenomas adrenocortico les suponen una causa extremadamente rara de síndrome de Cushing (SC) ACTH independiente. Se presenta un caso clínico donde a raíz de una diabetes mellitus tipo 2 mal controlada se demostró la presencia de hipercortisolismo. Los niveles de ACTH suprimidos determinaron que era ACTH independiente. Las pruebas de imagen junto con la cirugía aclararon que se trataba de un adenoma corticosuprarrenal originado por tejido adrenal ectópico del área del ligamento ancho izquierdo. Son pocos los casos publicados en la literatura, alguno de ellos en ovario, testículos o en el hilio renal; gracias a este caso aportamos uno más

Effectiveness of lanreotide autogel 120 mg at extended dosing intervals for acromegaly

Purpose: Recent data indicate that extended dosing intervals (EDIs) with lanreotide autogel 120 mg are effective and well-received among patients with acromegaly who have achieved biochemical control with monthly injections of long-acting somatostatin analogues (SSAs). We further evaluated the effectiveness of lanreotide autogel 120 mg delivered at EDIs (>4 weeks) in routine clinical practice. Methods: Cross-sectional, multicentre, observational study conducted to determine the effectiveness—measured by control of serum insulin-like growth factor 1 (IGF-1)—of lanreotide autogel 120 mg at dosing intervals >4 weeks for ≥6 months in selected patients with acromegaly treated in routine clinical practice (NCT02807233). Secondary assessments included control of growth hormone (GH) levels, treatment adherence, patient satisfaction, and quality of life (QoL) using validated questionnaires (EQ-5D, AcroQoL, and TSQM-9). Patients who received radiotherapy within the last 6 months were excluded. Results: Among 109 patients evaluated, mean (SD) age was 59.1 (13.2) years. IGF-1 values were normal (mean [SD]: 175.0 [74.5], 95% CI: 160.8 –189.1) in 91.7% of cases and normal in 91.4% of patients without previous radiotherapy treatment (n = 81). GH levels were ≤2.5 and ≤1 ng/mL, respectively, in 80.6% and 58.3%. Most patients were treated either every 5–6 (57.8%) or 7–8 weeks (38.5%), with 2.8% treated greater than every 8 weeks. The mean AcroQoL score was 63.0 (20.1). The mean global treatment satisfaction score (TSQM-9) was 75.1 (16.6). Treatment adherence (defined as no missed injections) was 94.5%. Conclusion: Lanreotide autogel 120 mg at intervals of >4 weeks provided IGF-1 control in more than 90% of patients with acromegaly. Treatment satisfaction and adherence were good. These findings support use of extended dosing intervals in patients who have achieved good biochemical control with long-acting SSAs

The Apoptotic Microtubule Network During the Execution Phase of Apoptosis

Apoptosis is a regulated energy-dependent process of cell death characterized by specific morphological and biochemical features in which caspase activation has a central role. During apoptosis, cells undergo characteristic morphological rearrangements in which the cytoskeleton participates actively. From a historical point of view, this reorganization has been assigned mainly to actinomyosin ring contraction with microtubule and intermediate filaments, both reported to be depolymerized at early stages of apoptosis. However, recent results have shown that the microtubule cytoskeleton is reformed during the execution phase of apoptosis, forming an apoptotic microtubule network (AMN). AMN is closely associated with the plasma membrane, forming a cortical ring or cellular “cocoon.” Apoptotic microtubules’ reorganization has been reported in many cell types and under many apoptotic inducers. Recently, it has been proposed that AMN is essential for preserving plasma membrane permeability and cell morphology during the execution phase of apoptosis. Apoptotic microtubules’ depolymerization leads cells to secondary necrosis and the release of toxic intracellular contents that can harm surrounding cells and initiate inflammation. Therefore, microtubules’ reorganization in physiological apoptosis during development and in the adult organism or in pathological apoptosis induced by anticancer treatments or chronic inflammation is essential for tissue homeostasis, preventing cell damage and inflammation

Revisiting the usefulness of the short acute octreotide test to predict treatment outcomes in acromegaly

Introduction: We previously described that a short version of the acute octreotide test (sAOT) can predict the response to first-generation somatostatin receptor ligands (SRLs) in patients with acromegaly. We have prospectively reassessed the sAOT in patients from the ACROFAST study using current ultra-sensitive GH assays. We also studied the correlation of sAOT with tumor expression of E-cadherin and somatostatin receptor 2 (SSTR2) .Methods: A total of 47 patients treated with SRLs for 6 months were evaluated with the sAOT at diagnosis and correlated with SRLs' response. Those patients whose IGF1 decreased to = 3SDS, were considered non-responders. The 2 hours GH value (GH2h) after s.c. administration of 100 mcg of octreotide was used to define predictive cutoffs. E-cadherin and SSTR2 immunostaining in somatotropinoma tissue were investigated in 24/47 and 18/47 patients, respectively.Results: In all, 30 patients were responders and 17 were non-responders. GH(2h) was 0.68 (0.25-1.98) ng/mL in responders vs 2.35 (1.59-9.37) ng/mL in non-responders (p<0.001). GH(2h) = 1.4ng/mL showed the highest ability to identify responders (accuracy of 81%, sensitivity of 73.3%, and specificity of 94.1%). GH(2h) = 4.3ng/mL was the best cutoff for non-response prediction (accuracy of 74%, sensitivity of 35.3%, and specificity of 96.7%). Patients with E-cadherin-positive tumors showed a lower GH(2h) than those with E-cadherin-negative tumors [0.9 (0.3-2.1) vs 3.3 (1.5-12.1) ng/mL; p<0.01], and patients with positive E-cadherin presented a higher score of SSTR2 (7.5 +/- 4.2 vs 3.3 +/- 2.1; p=0.01).Conclusion: The sAOT is a good predictor tool for assessing response to SRLs and correlates with tumor E-cadherin and SSTR2 expression. Thus, it can be useful in clinical practice for therapeutic decision-making in patients with acromegaly

Osteogénesis imperfecta forma clásica no deformante. Comunicación de una nueva mutación en el gen COL1A1 en dos casos de la misma familia

Osteogenesis imperfecta (OI), is a rare condition which is heterogeneous in clinical and genetic terms. Several types have been described and its main feature is bone fragility. It is generally caused by gene mutations in those genes which codify for the α1 and α2 of the type 1 collagen (COL1A1 and COL1A2) with dominant autosomal heredity. We report the case of two relatives (father and daughter) with OI whose genetic study shows a mutation in COL1A1 previously undetected: the deletion of a Guanine, G(c.3524delG). Clinical aspects, heredity and reproductive options of the patients affected are considered.La osteogénesis imperfecta (OI), es una patología poco frecuente y muy heterogénea desde el punto de vista clínico y genético. Su característica principal es la fragilidad ósea, habiéndose descrito varios tipos. Generalmente es causada por mutaciones en los genes que codifican para las cadenas α1 y α2 del procolágeno tipo 1 (COL1A1 y COL1A2) con herencia autosómica dominante. Comunicamos los casos de dos pacientes (padre e hija) con OI cuyo estudio genético muestra una mutación en COL1A1 no conocida previamente: la deleción de una Guanina, G(c.3524delG). Se repasan aspectos clínicos, de herencia y opciones reproductivas de los pacientes afectados

Effect on sebaceous glands of hamsters ears and pre-clinic toxicological evaluation of creams containing bioglea

La bioglea es un producto natural que se obtiene en balnearios de aguas minerales. Se realizó la evaluación del efecto antiacné e irritación dérmica y ocular de dos cremas cuyo principio activo es la bioglea a las concentraciones de 2,5 y 5%. En la evaluación toxicológica se realizaron los ensayos de irritación ocular y dérmica siguiendo la metodología descrita por las normas Nº 404 y 405 de OECD. En el ensayo de irritación dérmica fueron aplicados 0,5 g de las cremas sobre la piel de tres conejos albinos Nueva Zelandia machos. En el ensayo de irritación ocular se aplicaron 0,1 g de las cremas sobre las estructuras oculares de tres conejos. Para la actividad farmacológica fueron aplicados, vía dérmica, 0,1 g de las cremas sobre la oreja derecha de hámsteres Sirios Dorados hembras estimulados androgénicamente por inyección intramuscular de 80 µg/ml de propionato de testosterona, con el objetivo de evaluar su efecto antiacné. La crema de bioglea a las dos concentraciones calificaron como no irritantes para el ensayo de irritación dérmica y ocular. La estimulación androgénica produjo un aumento significativo (p<0,001) del área de las glándulas sebáceas respecto al grupo no estimulado androgénicamente. Las dos concentraciones de bioglea administradas produjeron una significativa disminución (p<0,05 y p<0,01) del tamaño de las glándulas sebáceas de la oreja de hámsteres estimulados androgénicamente. El efecto obtenido fue sistémico debido a la semejanza en el tamaño de las glándulas sebáceas entre las orejas tratadas con las cremas y las orejas no tratadas. De acuerdo con los resultados obtenidos con las cremas de bioglea pueden ser empleadas en la terapia antiacné.Bioglea is a natural product obtained from mineral water resorts. We evaluated anti-acne effect, eye and dermic irritation of two creams with bioglea as active substance at concentrations of 2.5 and 5 %. Eye and dermic irritation test was conduced according to the OECD Nº 404 and 405 Guidelines. In dermal irritation assay 0,5 g of the creams was applied on the skin of three male New Zealand albino rabbits. In eye irritation assay 0,1 g of the creams were applied on the ocular structures of three rabbits. For antiacne activity assay 0,1 g of the creams was applied on right pinna of female Syrian golden hamsters androgenic stimulated by 80 µg/ml testosterone propionate intramuscular injection. In eye and dermic irritation tests both concentrations of Bioglea creams were classified as non-irritant. Androgenic stimulation cause a significant increase (p<0,001) in sebaceous glands size when compared with the androgenic non-stimulated group. Both concentrations of Bioglea caused significant decrease (p<0,05 and p<0,01) in sebaceous glands size of androgenic stimulated hamsters pinna. The effect observed was systemic due to similar sebaceous glands size of creams treated side and untreated side. According to these results Bioglea creams will can be use in antiacne therapy.Colegio de Farmacéuticos de la Provincia de Buenos Aire

Toxicological evaluation and antiacne effect of mineral water and mud creams

Fue evaluado el efecto antiacné y el potencial tóxico de cuatro cremas conteniendo agua y fango mineral al 25% y 40% del Balneario de San Diego de los Baños ubicado en Pinar del Río, Cuba, a través de los ensayos de estimulación androgénica de las glándulas sebáceas en oreja de hámster y de irritación dérmica, ocular y fototoxicidad. En el ensayo de irritación dérmica fueron aplicados 0,5 g de cada una de las cremas sobre la piel de conejos albinos Nueva Zelanda machos. En el ensayo de irritación ocular se aplicaron 0,1 g de las cremas sobre las estructuras oculares de conejos. El ensayo de fototoxicidad se realizó aplicando 50 µg sobre la piel de cobayos albinos Hartley de ambos sexos. Para la actividad farmacológica fueron aplicados, vía dérmica, 0,1 g de las cremas sobre la oreja de hámster Sirios Dorados hembras estimulados androgénicamente por inyección intramuscular de 80 µg/mL de testosterona propionato, con el objetivo de evaluar su efecto antiacné. Las cremas ensayadas se clasificaron como no irritantes para el ensayo de irritación dérmica y ocular y no fototóxicas, mostrando la seguridad necesaria para su empleo clínico. La estimulación androgénica produjo un aumento significativo (p<0,01) del tamaño de las glándulas sebáceas respecto al grupo no estimulado androgénicamente. Las cuatro cremas administradas produjeron una significativa disminución (p<0,01) del tamaño de las glándulas sebáceas de la oreja de hámsters estimulados androgénicamente. El efecto obtenido fue sistémico, evidenciado por la semejanza en el tamaño de las glándulas sebáceas entre las orejas tratadas con las cremas y las orejas no tratadas.Four creams containing 25% and 40% of mineral water and mud from San Diego de los Baños Resort, located in Pinar del Río, Cuba, were evaluated for dermic and eye irritation, phototoxicity assay and antiacne properties. In dermal irritation assay 0.5 g of the creams was applied on the skin of male New Zealand albino rabbits. In eye irritation assay 0.1g of the creams were applied on the ocular structures of rabbits. The phototoxicity assay was carried out applying 50 µg on the skin of both sexes Hartley albino guinea pigs. For antiacne activity assay 0.1 g of the creams was applied on right pinna of female Syrian golden hamsters androgenic stimulated by 80 µg/mL testosterone propionate intramuscular injection. The creams were classified as no irritant for dermic and eye irritation assays and no phototoxic. Androgenic stimulation caused a significant size increase of the sebaceous glands (p<0.01) as compared to the group that did not receive androgenic stimulation. These four creams applied produced a significant decrease (p<0.01) of the size of the sebaceous glands of the hamster ears with androgenic stimulation. The effect was systemic due to the similarity in the size of sebaceous glands in both, treated and untreated side. The creams under study results no irritant to eye and skin and no phototoxic. Results prove the innocuity of four mud and mineral waters creams necessary for clinic used.Colegio de Farmacéuticos de la Provincia de Buenos Aire