Intervenciones dietéticas en el tratamiento del síndrome del ovario poliquístico. Una revisión bibliográfica / Dietary interventions in the treatment of polycystic ovary syndrome: A bibliographic review

El síndrome del ovario poliquístico (SOP), es una endocrinopatía femenina reconocida como un trastorno heterogéneo caracterizado por un hiperandrogenismo y una disfunción ovulatoria que conlleva problemas de fertilidad. Además, las pacientes suelen presentar una sintomatología asociada como la resistencia a la insulina, la intolerancia a la glucosa, la obesidad central y/o el síndrome metabólico que pueden inducir a un aumento del riesgo de enfermedad cardiovascular. Dado que uno de los principales objetivos del tratamiento del SOP es reducir las consecuencias metabólicas relacionadas con la obesidad, la resistencia a la insulina y el síndrome metabólico, las intervenciones dietéticas dirigidas a este propósito pueden resultar eficaces en el tratamiento de este padecimiento. Se ha llevado a cabo una búsqueda bibliográfica en diferentes bases de datos como Web of Science (WOS), PubMed y Google Académico estableciendo unos criterios de búsqueda previamente definidos. Se han elegido 11 trabajos para su revisión completa y análisis crítico. Entre las diferentes intervenciones que se han utilizado, se han seguido estrategias dietéticas como la Dietary Approaches to Stop Hypertension (DASH), modificaciones en los hidratos de carbono (HC), la inclusión de algún alimento determinado en el patrón dietético habitual y/o los cambios en el estilo de vida. De los resultados obtenidos, destacan las mejoras propiciadas en los marcadores corporales con un régimen DASH, los beneficios promovidos por dietas con modificaciones en los HC, en la resistencia insulínica (IR) y los marcadores hormonales, así como los efectos favorables en las manifestaciones clínicas relacionadas con el hiperandrogenismo, fomentados por el consumo de soja y las modificaciones en el estilo de vida (LSM).(AU)

Polycystic ovary syndrome (PCOS) is a female endocrinopathy recognized as a heterogeneous disorder characterized by hyperandrogenism and ovulatory dysfunction that leads to fertility problems. In addition, patients usually present with associated symptoms such as insulin resistance, glucose intolerance, central obesity and/or metabolic syndrome that can induce an increased risk of cardiovascular disease. Since one of the main goals of PCOS is to reduce the metabolic consequences related to obesity, insulin resistance, and the metabolic syndrome, targeted dietary interventions may be effective in treating PCOS.A bibliographic search has been carried out in different databases such as Web of Science, Pubmed and Google Scholar, establishing previously defined search criteria. Eleven have been chosen for full review and critical analysis. Among the different interventions that have been used, dietary strategies have been followed such as the dietary approaches to stop hypertension (DASH), modifications in carbohydrates, the inclusion of a certain food in the usual dietary pattern and/or lifestyle modifications. Of the results obtained, we highlight the improvements in body markers with a DASH diet, the benefits promoted by diets with modifications in carbohydrates, in insulin resistance and hormonal markers and favorable effects on clinical manifestations related to hyperandrogenism, fostered by soy consumption and lifestyle modifications.(AU)

Association of free testosterone, glucose level and obesity among women with polycystic ovary syndrome in Soran city, Kurdistan-Iraq / Asociación de testosterona libre, nivel de glucosa y obesidad entre mujeres con síndrome de ovario poliquístico en la ciudad de Soran, Kurdistán-Irak

Background: Polycystic ovary syndrome (PCOS) is the most common endocrinopathy affecting approximately 5–10% of women of reproductive age and it is also a major cause of anovulatory infertility. PCOS is associated with obesity and conditions like hirsutism, acne, diabetes, and irregular periods. Aim: The present study aimed to evaluate the serum-free testosterone (FT) levels of women afflicted with hirsutism, one of the main physical manifestations of PCOS versus healthy women and determine whether their serum testosterone levels correlate with polycystic ovaries, glucose levels, menstrual abnormalities, and obesity. Methods: This study assessed 180 women; this included 140 females who suffered from excessive and unwanted hair growth on the chin and 40 healthy women as a control group. Free testosterone levels and fasting blood glucose levels were taken. Prior to the study, ultrasonographic (US) tests were performed for all patients to diagnose polycystic ovaries. Results: Patients with hirsutism exhibited a significant elevation in free testosterone (FT) compared to the control group. Approximately half of these women were confirmed to have a polycystic ovary, and 42.8% of them were overweight and obese. Additionally, women between 18 and 20 years old (G1) present with the highest level of FT. Conclusion: Serum FT levels were significantly increased in hirsute women, and this positively correlated with BMI and glucose levels in women with PCOS. Glucose levels may serve as a potentially effective biomarker in evaluating the severity of hirsutism in women suspected of having PCOS.(AU)

Antecedentes: El síndrome de ovario poliquístico (SOP) es la endocrinopatía más común que afecta aproximadamente al 5-10% de las mujeres en edad reproductiva y también es una de las principales causas de infertilidad anovulatoria. El SOP está asociado con la obesidad y condiciones como hirsutismo, acné, diabetes y períodos irregulares. Objetivo: El presente estudio tuvo como objetivo evaluar los niveles séricos de testosterona libre (FT, del inglés) de mujeres con hirsutismo, una de las principales manifestaciones físicas del síndrome de ovario poliquístico, en comparación con mujeres sanas y determinar si los niveles séricos de testosterona se correlacionan con ovarios poliquísticos, niveles de glucosa, anomalías menstruales y obesidad. Métodos: Este estudio evaluó a 180 mujeres; esto incluyó a 140 mujeres que sufrían de un crecimiento de vello excesivo e indeseado en la barbilla y 40 mujeres sanas como grupo de control. Se midieron el nivel de FT y el nivel de azúcar en sangre en ayunas. Antes del estudio, se realizaron pruebas ultrasonográficas a todas las pacientes para diagnosticar ovarios poliquísticos. Resultados: Los pacientes con hirsutismo exhibieron una elevación significativa en la FT en comparación con el grupo de control. Se confirmó que aproximadamente la mitad de estas mujeres tenían un ovario poliquístico y el 42,8% de ellas tenían sobrepeso y obesidad. Adicionalmente, las mujeres entre 18 y 20 años (G1) presentan el mayor nivel de FT. Conclusión: Los niveles séricos de FT aumentaron significativamente en mujeres hirsutas, y esto se correlacionó positivamente con el IMC y los niveles de glucosa en mujeres con SOP. Los niveles de glucosa pueden servir como un biomarcador potencialmente eficaz para evaluar la gravedad del hirsutismo en mujeres con sospecha de SOP.(AU)

Hipertecosis estromal ovárica: reporte de un caso / Ovaric stromal hypertecosis: a case report

Resumen ANTECEDENTES: La hipertecosis estromal ovárica es un tumor androgenizante del ovario, raro, que puede manifestarse como: hiperandrogenismo, carcinoma de endometrio, obesidad e hipertensión. En la bibliografía solo se encuentran algunos reportes de casos y pequeñas series de casos. CASO CLÍNICO: Paciente de 20 años, con sangrado vaginal continuo, hirsutismo, clitoromegalia, implantación androide del vello púbico y facial. En la ecografía del ovario derecho se advirtió una lesión sólida en su interior. Concentraciones elevadas de testosterona libre, testosterona e hidroxiprogesterona. Se sospechó un tumor androgenizante del ovario. Se practicó la salpingooforectomía derecha por vía laparoscópica. El reporte de patología fue: parénquima ovárico con extensos nidos de células estromales luteinizadas con hiperplasia estromal. Diagnóstico: hipertecosis estromal. CONCLUSIÓN: Ante la coexistencia de signos de virilización, alteración en la regularidad y frecuencia del ciclo menstrual, con altas concentraciones de andrógenos y una lesión tumoral ovárica en el estudio ecográfico debe sospecharse hipertecosis estromal ovárica. El tratamiento de elección es la resección quirúrgica del ovario afectado. Debido al bajo potencial de malignidad de estas lesiones puede considerarse el acceso laparoscópico.

Abstract BACKGROUND: Ovarian stromal hyperthecosis is a rare ovarian androgenizing tumor that can manifest as hyperandrogenism, endometrial carcinoma, obesity and hypertension. Only a few case reports and small case series are found in the literature. CLINICAL CASE: 20-year-old patient with continuous vaginal bleeding, hirsutism, clitoromegaly, and android implantation of pubic and facial hair. Ultrasonography of the right ovary showed a solid lesion inside the ovary. Elevated concentrations of free testosterone, testosterone and hydroxyprogesterone. An ovarian androgenizing tumor was suspected. A laparoscopic right salpingo-oophorectomy was performed. The pathology report was ovarian parenchyma with extensive nests of luteinized stromal cells with stromal hyperplasia. Diagnosis: stromal hyperthecosis. CONCLUSION: In the coexistence of signs of virilization, alteration in the regularity and frequency of the menstrual cycle, with high concentrations of androgens and an ovarian tumor lesion in the ultrasound study, ovarian stromal hyperthecosis should be suspected. The treatment of choice is surgical resection of the affected ovary. Due to the low malignant potential of these lesions, laparoscopic access can be considered.

Spironolactone in Dermatology: Uses in Acne, Hidradenitis Suppurativa, Female Pattern Baldness, and Hirsutism. / Uso de la espironolactona en dermatología: acné, hidradenitis supurativa, alopecia femenina e hirsutismo.

Spironolactone is an economical potassium-sparing diuretic with an anti-androgenic effect and a good safety profile. Our experience suggests that this diuretic is underexploited in dermatology even though there is evidence supporting its use in several skin conditions. When prescribed for acne in female patients (level 1-2 evidence; strength of recommendation, B), for example, it can reduce the need for antibiotics and possibly isotretinoin. Other diseases in which spironolactone is potentially useful are hidradenitis suppurativa and female androgenetic alopecia. We discuss the indications for spironolactone, dosing in dermatology, precautions to consider, and adverse effects. We also review new evidence that stresses the safety of long-term therapy and supports the use of this drug without the need for complementary testing in young women. We think that spironolactone merits a place among the medications commonly used in routine clinical practice.

Presentación de un caso de síndrome de Cooke-Apert-Gallais / Presentation of a case of Cooke-Apert-Gallais syndrome

Se estudió una paciente de 47 años de edad con antecedentes de buena salud, sin antecedentes atópicos personales ni familiares, que acude a la consulta de endocrinología por presentar aumento del vello corporal con dos años de evolución. Al examen físico se le constató rubicundez facial, escudo pubiano masculino, predominio de la cintura escapular sobre la cintura abdominal e hipertrofia de 3,5 cm del clítoris. Referente a los exámenes complementarios presentó un marcado aumento de la testosterona en sangre así que se le practicó una histerectomía con doble anisectomía y los resultados de la biopsia fueron: un tumor benigno de células de Leydig. Se concluyó que se trataba de un Síndrome de Cooke-Apert-Gallais por un tumor productor de testosterona en el ovario(AU)

A study was conducted of a female 47-year-old patient with a history of good health and no personal or family atopic antecedents, who attends endocrinology consultation due to increased growth of body hair of two years' evolution. Physical examination revealed facial reddishness, a male pubic shield, predominance of the scapular waist over the abdominal waist, and 3.5 cm hypertrophy of the clitoris. Complementary tests found a marked increase in blood testosterone, which led to the performance of hysterectomy with double adnexectomy. The result of the biopsy was benign Leydig cell tumor. The final diagnosis was Cooke-Apert-Gallais syndrome due to a testosterone-producing tumor in the ovary(AU)

Oral Contraceptives in Dermatology. / Anticonceptivos orales en dermatología.

Oral contraceptives combine estrogen and progesterone to suppress ovulation. Synthetic forms are usually used. In dermatology, oral contraceptives are prescribed for 2 main reasons: to prevent pregnancy when teratogenic drugs must be taken and to treat skin manifestations of hyperandrogenism. Most oral contraceptives improve both acne and hirsutism, but the androgenic effect of progestogens - particularly if the contraceptive contains first- or second-generation progestogens- can trigger or exacerbate acne. One of the most serious side effects of oral contraceptives, thrombosis, is mainly caused by the estrogen component and its dose. If we mainly consider a contraceptive's thrombotic profile when prescribing, the choice would be to have 30µg or less of ethinyl estradiol combined with levonorgestrel. On the other hand, if our main objective is to treat signs of androgenization, we would prefer contraceptives containing progestogens with antiandrogenic effects.

Síndrome dos ovários policísticos: tratamento das manifestações androgênicas / Polycystic ovary syndrome: treatment of androgenic manifestations

A síndrome dos ovários policísticos (SOP) é um distúrbio endócrino-metabólico muito frequente no período reprodutivo. Quando associado ao distúrbio metabólico, as mulheres com SOP podem ter ainda risco acrescido para doença cardiovascular. O objetivo deste manuscrito é descrever as repercussões metabólicas, incluindo quais as principais, como investigar e as consequências desse distúrbio sobre a saúde da mulher. É uma revisão narrativa mostrando a implicação da resistência insulínica, das dislipidemias e da síndrome metabólica sobre o sistema reprodutor e sobre o risco cardiovascular da mulher com SOP, bem como do uso de sensibilizadores de insulina no seu tratamento. Conclui-se que a correção dos distúrbios metabólicos na SOP é benéfica tanto para o sistema reprodutor quanto para o cardiovascular. A primeira linha de tratamento é a mudança de estilo de vida e a perda de peso. Na resposta inadequada, o tratamento medicamentoso está recomendado. Nas mulheres com obesidade mórbida que não tiveram bons resultados com o tratamento clínico, a cirurgia bariátrica é uma opção.(AU)

Tumor ovárico de células de Leydig en una niña de 7 años que se presentó como pubarquia precoz con elevación de 17 hidroxiprogesterona / Ovarian tumor of Leydig cells in a 7-year-old girl who presented as precocious pubarche with elevation of 17 hydroxyprogesterone

Clinical case: a girl of 7 ½ years who consulted for early pubarche without thelark, with a percentile size of 75 for a genetic target size in the 10th percentile, overweight with a 90th percentile BMI, and normal blood pressure. The biochemical study showed high levels of androgens: testosterone: 7.2 ng/dL, androstenedione of 5.1 ng / ml, 17OHP: 15 ng / dL with low normal DHEAS (0.26 ug/ml), Plasma Renin Activity normal low: 0.22 ng/mL/h. Initial imaging study showed a bone age of 10 years 6 months and normal abdominal and pelvic ultrasound. Molecular study showed no pathogenic variants in the CYP21A2 gene (21 Hydroxylase). With a probable diagnosis of non-classical congenital adrenal hyperplasia (HSRNC) and no known mutation, he started treatment with hydrocortisone (12 mg/m2). At 8.7 years, pubertal development begins and braking begins with LHRH analogues, which are administered for 18 months. Despite the treatment, signs of virilization and elevation of androgens (testosterone up to 130 ng/ml) are progressively accentuated, which do not diminish when trying different corticosteroid schemes. MRI of the abdomen and pelvis shows the normal adrenal glands and a solid nodular image of 2.1 x 1.6 cm in the right ovary (Figure 2), later demonstrated with pelvic ultrasound (Figure 2). Right laparoscopic oophorectomy was performed, whose biopsy demonstrated a Leydig cell tumor. One month after surgery, all androgenic levels were normalized, so the gradual suspension of corticosteroids began. Conclusion: Although HSRNC is the most frequent pathological cause of early pubarche, when it is associated with progressive clinical and biochemical hyperandrogenism despite adequate treatment and without pathogenic variants in the CYP21A2 gene, even with high levels of 17OHP, other causes should be considered, specifically, androgen producing tumors.

Caso clínico: niña de 7½ años que consulta por pubarquia precoz sin telarquia, con talla en percentil 75 para una talla objetivo genético en percentil 10, sobrepeso con IMC percentil 90 y presión arterial normal. El estudio bioquímico mostró niveles elevados de andrógenos: testosterona: 7,2 ng/dL, androstenediona de 5,1 ng/ml, 17OHP: 15 ng/dL con DHEAS normal baja (0,26 ug/ml), Actividad de Renina Plasmática normal baja: 0.22 ng/ mL/h. Estudio de imágenes inicial mostró una edad ósea de 10 años 6 meses y ecografía abdominal y pelviana normales. Estudio molecular no mostró variantes patogénicas en el gen CYP21A2 (21 Hidroxilasa). Con diagnosticó probable de hiperplasia suprarrenal congénita no clásica (HSRNC) y sin mutación conocida,inició el tratamiento con hidrocortisona (12 mg/m2). A los 8.7 años comienza desarrollo puberal y se inicia frenación con análogos de LHRH, los cuales se administran por 18 meses. A pesar del tratamiento se acentúan progresivamente los signos de virilización y hayelevación de los andrógenos (testosterona hasta 130 ng/ml), que no disminuyen intentando diferentes esquemas de corticoides. Se realiza RM de abdomen y pelvis que muestra las glándulas suprarrenales normales y una imagen nodular sólida de 2.1 x 1.6 cm en el ovario derecho (Figura 2), demostrada posteriormente con Ecografía pelviana (Figura 2). Se realiza ooforectomía derecha por vía laparoscópica, cuya biopsia demostró un tumor de células de Leydig. Un mes después de la cirugía, se normalizan todos los niveles androgénicos por lo que se inició la suspensión gradual de los corticoides. Conclusión: Aunque la HSRNC es la causa patológica más frecuente de la pubarquia precoz, cuando se asocia con un hiperandrogenismo clínico y bioquímico progresivo a pesar de un tratamiento adecuado y sin variantes patógenicas en el gen CYP21A2, incluso con niveles elevados de 17OHP, otras causas deben ser consideradas, específicamente tumores productores de andrógenos.

Estudios de laboratorio de diversos parámetros orientados a tratar de dilucidar la fisiopatología del síndrome de ovarios poliquísticos. Parte 1 / Laboratory studies of various parameters aimed at trying to elucidate the pathophysiology of polycystic ovary syndrome. Part 1

Esta revisión fue realizada con el fin de evaluar nuestros resultados de laboratorio así como aquellos de la literatura que constituyen, a nuestro entender, aportes significativos en el síndrome de ovarios poliquísticos (SOP). Nuestro especial énfasis será presentar las limitaciones de las metodologías empleadas por nuestro grupo, comparativamente a las reportadas por otros investigadores. La determinación de andrógenos, en particular de Testosterona (TT), es quizá la de mayor complejidad dado que los resultados con los diferentes inmunoensayos empleados en nuestro medio producen resultados muy variables por los diferentes métodos y aún entre laboratorios que usan la misma metodología. La técnica de referencia es la cromatografía líquida en tándem con espectrometría de masa (LC-MSMS), de difícil aplicación en laboratorios de análisis clínicos debido a su alto costo y la imposibilidad de resolver numerosas muestras. En estudios previos demostramos que de los métodos habitualmente usados para evaluar la TT circulante, solo en 2 inmunoensayos los resultados obtenidos fueron satisfactoriamente validados indirectamente según el criterio del Consenso de los Centros para el Control y Prevención de Enfermedades (CDC, USA) contra LC-MSMS, los cuales fueron comparables a dicha metodología con niveles superiores a 0,5 ng/ml. El SOP puede presentar factores de riesgo aumentados para la enfermedad cardiovascular y la diabetes II. Estos factores no están debidamente categorizados en función de los distintos fenotipos del SOP. Se evaluarán los principales analitos empleados con este objetivo y los nuevos que aporten elementos de mayor especificidad en este sentido

This review was performed in order to evaluate our laboratory results as well as those of the literature that constitute, in our opinion, significant contributions in these pathophysiologies. Our special emphasis will be on presenting the limitations of the methodologies used by our group, compared to those reported by other researchers. The determination of androgens, in particular Testosterone (TT), is perhaps the most complex since the results with the different immunoassays used in our environment produce very variable results by the different methods and even between laboratories that use the same methodology. The reference technique is LC-MSMS, difficult to apply in clinical analysis laboratories because of its high cost and the inability to solve numerous samples. In previous studies, we demonstrated that, in comparison to LC-MSMS with the usual methods for evaluating circulating TT, the results obtained in only 2 immunoassays were satisfactorily validated indirectly according to the criteria of CDC against LC-MSMS, which were comparable to that methodology with levels higher than 0.5 ng/ml. PCOS may have increased risk factors for cardiovascular disease and diabetes II. These factors are not properly categorized according to the different phenotypes of PCOS. The main analytes used for this purpose will be evaluated and new ones that contribute elements of greater specificity in this sense

Criterios diagnósticos y tratamiento integral terapéutico del síndrome de ovarios poliquísticos / Diagnostic criteria and therapeutic integral treatment of polycystic ovary syndrome

Introducción: el síndrome de ovarios poliquísticos es un cuadro clínico caracterizado por la presencia de ovarios con pequeños quistes, amenorrea, hirsutismo y obesidad. Objetivo: proporcionar criterios diagnósticos vigentes y tendencias de tratamiento integral mediante una revisión sistemática de la literatura que permita un direccionamiento de los criterios médicos en atención a las mujeres con síndrome de ovario poliquístico. Métodos: estudio descriptivo retrospectivo, realizado mediante búsquedas en base de datos electrónicos como, Pubmed, Google Scholar, Cochrane Library, Science Direct y Elsevier. Se consultaron 25 artículos publicados desde el año 2011 para realizar esta revisión en torno al síndrome de ovario poliquístico, los cuales presentan evidencias científicas, en relación a la utilidad de la misma con contribuciones en el diagnóstico y manejo terapéutico de esta patología. Resultados: el abordaje diagnóstico implica ciertas dificultades en relación con el momento de presentación de los síntomas y la tendencia actual es considerar como un diagnóstico de exclusión, empleándose los cuatros fenotipos propuestos en Rotterdam. El estudio encontró que los cambios en el estilo de vida, la reducción del peso, dieta, ejercicios, medicina alternativa, tratamientos cosméticos, prevención de factores epigenéticos, la terapia quirúrgica y farmacológica contribuyen a mejorar la calidad de vida y sintomatología en las pacientes afectadas con esta entidad nosológica. Conclusión: el síndrome de ovario poliquístico sigue siendo una enfermedad con diagnóstico de exclusión, pero la tendencia actual es investigar la hormona antimulleriana como predictor de esta enfermedad. El tratamiento está orientado a corregir el hiperandrogenismo, los trastornos menstruales, las alteraciones metabólicas asociadas y la anovulación(AU)

Introduction: the polycystic ovarian syndrome (PCOS) is a clinical condition characterized by the presence of ovaries with small cysts, amenorrhea, hirsutism and obesity. Objective: Provide current diagnostic criteria and comprehensive treatment trends, through a systematic review, which allows addressing the medical criteria in care for women with polycystic ovarian syndrome (PCOS). Methods: A retrospective descriptive study was conducted by searching electronic database as Pubmed, Google Scholar, Cochrane Library, Science Direct and Elsevier. Twenty-five articles published since 2011 are addressed for this review on SOP, which present scientific evidence, in relation to its utility with contributions in the diagnosis and therapeutic management of this pathology. Results: the diagnostic approach implies certain difficulties in relation to the time of presentation of the symptoms. The current tendency is to consider it as a diagnosis of exclusion, using the four phenotypes proposed in Rotterdam. The study found that changes in lifestyle, weight reduction, diet, exercise, alternative medicine, cosmetic treatments, and prevention of epigenetic factors, surgical and pharmacological therapy contribute to improve the quality of life and symptoms in affected patients with this nosological entity. Conclusion: Polycystic ovarian syndrome remains a disease with a diagnosis of exclusion, but the current trend is to investigate the anti-mullerian hormone as a predictor of this disease. The treatment aimed at correcting hyperandrogenism, menstrual disorders, associated metabolic disorders and anovulation(AU)

Hirsutismo: aproximación al diagnóstico y tratamiento / Hirsutism: approach to the diagnosis and treatment

El hirsutismo en una endocrinopatía frecuente en mujeres en edad reproductiva, caracterizada por un crecimiento excesivo del vello corporal en áreas dependientes de andrógenos, con diferentes grados de severidad y percepción subjetiva. Clínicamente el hirsutismo es un reflejo de la concentración local y circulante de androgénos y de la sensibilidad de la unidad pilo-sebácea a los mismos. Esta revisión se enfocará en la fisiopatología, etiología, diagnóstico diferencial y tratamiento del hirsutismo.

Hirsutism is a common endocrinopathy among women of reproductive age characterized by the excessive growth of body hair in androgen-dependent areas with different grades of severity and subjective perception. Hirsutism is an expression of circulating and local androgen concentrations and the sensitivity of the pilosebaseous unit to androgens. This review will focus on the pathophysiology, etiology, differential diagnosis and treatment of hirsutism.

Síndrome cornelia de lange, fenotipo III / Cornelia de lange syndrome, phenotype III

El Síndrome de Cornelia de Lange es un trastorno del desarrollo poco frecuente, heterogéneo genéticamente, que se define en su forma clásica por hipocrecimiento antenatal y postnatal, microcefalia, rasgos faciales distintivos, sinofridia e hirsutismo, retraso mental y micromelia. Objetivo: dar a conocer las características clínicas de los pacientes con este síndrome para realizar diagnósticos oportunos, estudios pertinentes y manejos multidisciplinarios en los pacientes afectados. Caso Clínico, lactante femenina, 19 meses de edad, con antecedentes maternos: 17 años, embarazo de 34 semanas de gestación, con un control prenatal, atendida en Hospital Santa Bárbara Integrado, ingresada para uteroinhibición sin lograr respuesta y desencadena trabajo de parto. Recién nacida, femenina, cefálica, peso de 1,625 g, perímetro cefálico 29.5 cm, malformaciones en miembros superiores, ingresada a sala de neonatos por distrés respiratorio y prematurez. Evaluada por pediatra, quien describe características dismórficas. Al examen físico: focomelia, micromelia, pestañas largas y abundantes, sinofridia e hirsutismo. En la radiografía se observó: agenesia de ambos cubitos, todos los hallazgos clínicos y laboratoriales compatibles con Síndrome de Cornelia de Lange. No se realizó ecocardiograma ni estudios genéticos, el diagnóstico de este síndrome fue clínico. A los 19 meses fue reevaluada y se encontró: peso 3,600 g, perímetro cefálico 34.5 cm y talla 55 cm, evidente retraso del crecimiento, mental y psicomotor. Conclusión: los pacientes con este síndrome deben ser diagnosticados fundamentalmente por clínica, luego realizar estudios imagenológicos para descartar otras anomalías. Además, el tratamiento debe ser integral...(AU)

Association between Lipid Accumulation Product and Hirsutism in Patients with Polycystic Ovary Syndrome / Associação entre lipid accumulation product (LAP) e hirsutismo na síndrome do ovário policístico

Objective Polycystic ovary syndrome (PCOS) is the most common endocrine metabolic disorder in women between menarche and menopause. Clinical hyperandrogenism is the most important diagnostic criterion of the syndrome, which manifests as hirsutism in 70% of cases. Hirsute carriers of PCOS have high cardiovascular risk. Lipid accumulation product (LAP) is an index for the evaluation of lipid accumulation in adults and the prediction of cardiovascular risk. The aim of this study was to evaluate the association between LAP and hirsutism in women with PCOS. Methods This was a cross-sectional observational study of a secondary database, which included 263 patients who had visited the Hyperandrogenism Outpatient Clinic from November 2009 to July 2014. The exclusion criteria were patients without Ferriman-Gallwey index (FGI) and/or LAP data. We used the Rotterdam criteria for the diagnosis of PCOS. All patients underwent medical assessment followed by measurement and recording of anthropometric data and the laboratory tests for measurement of the following: thyroid-stimulating hormone, follicle-stimulating hormone, prolactin, total testosterone, sex hormone binding globulin, 17-α-hydroxyprogesterone (follicular phase), glycohemoglobin A1c, and basal insulin. In addition, the subjects underwent lipid profiling and oral glucose tolerance tests. Other laboratory measurements were determined according to clinical criteria. LAP and the homeostatic model assessment index (HOMA-IR) were calculated using the data obtained. We divided patients into two groups: the PCOS group with normal LAP (< 34.5) and the PCOS group with altered LAP (> 34.5) to compare the occurrence of hirsutism. For statistical analysis, we used SPSS Statistics for Windows(r) and Microsoft Excel programs, with descriptive (frequencies, percentages, means, and standard deviations) and comparative analyses (Student's t-test and Chi-square test). We considered relations significant when the p-value was≤0.05. Results LAP was high in most patients (n = 177; 67.3%) and the FGI indicated that 58.5% of the patients (n = 154) had hirsutism. The analysis by LAP quartiles showed a positive correlation (p = 0.04) among patients with a high FGI and an upper quartile LAP (> 79.5) when compared with those with LAP < 29.0 (lower quartile). Conclusion This study demonstrated an association between high LAP and hirsutism. The FGI could represent a simple and low-cost tool to infer an increased cardiovascular risk in women with PCOS.

Objetivo A síndrome dos ovários policísticos (SOP) é o distúrbio endócrino mais comumemmulheres entre amenarca e a menopausa. O hiperandrogenismo clínico é o critério diagnóstico mais importante da síndrome, que se manifesta como hirsutismo em70% dos casos. Portadores de SOP hirsutas têmelevado risco cardiovascular. O Lipid accumulation product (LAP) é um índice para a avaliação da acumulação de lípidos nos adultos e é um preditor de risco cardiovascular. O objetivo deste estudo é avaliar a associação entre LAP e hirsutismo em portadoras da SOP. Métodos Estudo observacional transversal de banco de dados secundário, que incluiu 263 pacientes do Ambulatório de Hiperandrogenismo no período de novembro de 2009 a julho de 2014. Foram excluídas pacientes sem o índice de Ferriman-Gallwey e/ ou LAP. Foram utilizados como critérios diagnósticos da SOP os critérios de Rotterdam. Todas as pacientes foram submetidas à avaliação médica seguida da aferição e registro dos dados antropométricos e a realização dos seguintes exames laboratoriais: hormônio estimulante da tireoide (TSH), hormônio folículo estimulante (FSH), prolactina (PRL), Testosterona total, globulina ligadora dos hormônios sexuais (SHBG), 17-α- hidroxiprogesterona, (fase folicular), perfil lipídico, teste oral de tolerância à glicose, glico-hemoglobina a1C, insulina basal. Outras dosagens laboratoriais foram determinadas à critério clínico. O LAP e o HOMA-IR (homeostatic model assessment) foram calculados com os dados obtidos. As pacientes foram divididas em dois grupos: grupo das portadoras de SOP com LAP normal (LAp < 34,5) e grupo das portadoras de SOP com LAP alterado (LAP >34,5) para as comparações do hirsutismo. Para a análise estatística, foram utilizados os programas SPSS Statistics for Windows(r) e Microsoft Excel(r), sendo feitas análises descritivas (frequências, percentuais, médias, desviospadrão) e comparativas (t-Student e qui-quadrado). Foram consideradas relações significativas quando p-valor foi menor ou igual a 0,05. Resultados O LAP foi elevado na maioria das pacientes (n = 177; 67,3%) e o índice de Ferriman-Gallwey (IF) demonstrou que 58,5% das pacientes (n = 154) eram hirsutas. A análise por quartis de LAP, demonstrou correlação positiva (p = 0,04) entre pacientes comIF elevado e LAP no quartil superior (>79,5) quando comparadas àquelas com LAP menor que 29,0 (quartil inferior). Conclusão O estudo demonstrou associação do LAP elevado e hirsutismo. O escore de Ferriman-Gallwey poderia representar uma ferramenta simples e de baixo custo para inferir risco cardiovascular aumentado em portadoras da síndrome.

EVALUACIÓN CLÍNICA DE VELLO CORPORAL TERMINAL Y PREVALENCIA DE HIRSUTISMO EN ESTUDIANTES UNIVERSITARIAS DE LA CIUDAD DE MANIZALES (CALDAS), 2012 / CLINICAL EVALUATION OF TERMINAL BODY HAIR AND HIRSUTISM PREVALENCE IN UNIVERSITY STUDENTS FROM MANIZALES (CALDAS), 2012

Objetivos: Determinar la prevalencia del hirsutismo en la ciudad de Manizales, caracterizar la patología desde su perspectiva demográfica y clínica, y relacionar las diferentes variables con la presencia o no de hirsutismo. Metodología: Investigación de corte transversal de nivel analítico, basada en el estudio de una población de acceso de 830 estudiantes de primer año de las universidades de la ciudad, quienes fueron encuestadas y examinadas aplicando la escala estandarizada de Ferriman y Gallwey modificada (F-Gm), previo consentimiento informado. Resultados: La prevalencia de hirsutismo encontrada para un puntaje de 8 de la escala de F-Gm es del 20,5%, el percentil 95 hallado equivale a 12 puntos. Se encontró mayor frecuencia de hirsutismo en los diferentes puntos de corte evaluados en las mujeres: con edad entre los 20 y 24 años, mestizas, con antecedente y presencia de acné, con padres con calvicie, con hermanas con hirsutismo. Existe correlación negativa entre el puntaje de la escala de F-Gm y edad de la pubarca, presión arterial sistólica y estrato socioeconómico; y correlación positiva con el perímetro abdominal. Conclusiones: Para la población estudiada el signo hirsutismo presenta prevalencia similar a la encontrada en otros estudios publicados, el percentil 95 como parámetro de normalidad de 12 en la escala F-Gm es superior a los puntos de corte informados en la literatura.

Objectives: To determine the prevalence of hirsutism in the city of Manizales (Colombia), to characterize its pathology from its demographic and medical perspective and to relate the different variables with the presence or absence of hirsutism. Methodology: Transversal analytical research based on the study of a population composed by 830 female freshmen attending several local universities who were surveyed and examined applying Ferriman and Gallwey modified scale (F-Gm) previous informed consent. Results: The prevalence of hirsutism for a score of 8 in the F-Gm scale was 20.5%; the 95 percentile found is equivalent to 12 points. An increased frequency of hirsutism was found in the cut off points evaluated in women with the following characteristics: age 20 to 24, mestizo, with a history and presence of acne, bald parents and sisters with hirsutism. There is negative relation between the score in the F-Gm scale and the pubarche age, systolic blood pressure, socio-economic status. On the contrary, it positively correlated with abdominal circumference. Conclusions: For the population studied hirsutism showed a similar prevalence to that found in the population studied compared to published data. Nevertheless, the 95 percentile, as a normality parameter of 12 in the F-Gm scale, is higher than the cut off points informed in the literature.

Ferriman-Gallwey score correlates with obesity and insulin levels in polycystic ovary syndrome: an observational study / Correlação da escala de Ferriman-Gallwey com a obesidade e níveis de insulina na síndrome dos ovários policísticos: um estudo observacional

BACKGROUND: The Polycystic Ovary Syndrome (PCOS) is the most common endocrinopathy and one of the main causes of infertility in women. OBJECTIVES: This study aimed to evaluate the correlation between clinical hyperandrogenism assessed by modified Ferriman-Gallwey (F-G) score and metabolic parameters in Polycystic Ovary Syndrome women. METHODS: This observational study included fifty Polycystic Ovary Syndrome subjects. Detailed information about body mass index (BMI) and abdominal circumference (AC) were obtained from each subject. F-G score was applied to assess hirsutism through visual method. Serum levels of insulin, glucose and testosterone were measured. RESULTS: A positive correlation was observed between F-G score with body mass index, abdominal circumference and insulin. CONCLUSIONS: Obesity, mainly abdominal adipose tissue, and insulin levels correlate with hyperandrogenism in Polycystic Ovary Syndrome women, analyzed by F-G score. F-G score could be a marker to evaluate metabolic disorders in Polycystic Ovary Syndrome women.

JUSTIFICATIVA: A Síndrome dos Ovários Policísticos (SOP) é a endocrinopatia mais comum e uma das principais causas de infertilidade em mulheres. OBJETIVOS: O presente estudo teve como objetivo avaliar a correlação entre hiperandrogenismo clínico, avaliado pela escala de Ferriman-Gallwey (FG) modificada e parâmetros metabólicos em mulheres com a Síndrome dos Ovários Policísticos. MÉTODOS: Este estudo observacional incluiu cinquenta mulheres com Síndrome dos Ovários Policísticos. Informações detalhadas sobre o índice de massa corporal (IMC) e circunferência abdominal (CA) foram obtidas de cada participante. A escala FG foi aplicada para avaliar o hirsutismo através do método visual. Níveis séricos de insulina, glicose e testosterona foram também avaliados. RESULTADOS: Observou-se uma correlação positiva entre a escala FG e o índice de massa corporal, circunferência abdominal e insulina. CONCLUSÕES: A obesidade, principalmente o tecido adiposo abdominal, e os níveis de insulina se correlacionam com hiperandrogenismo em mulheres com Síndrome dos Ovários Policísticos, analisados por meio da escala FG. Desta forma, esta escala poderia ser um marcador para avaliar distúrbios metabólicos em mulheres com Síndrome dos Ovários Policísticos.

Hirsutismo pós-menopausa: caso clínico raro de hipertecose ovárica / Post-menopausal hirsutism: a rare case of ovarian hyperthecosis

Durante o climatério podem ocorrer sinais clínicos de hiperandroginismo. Contudo quadros de virilização exigem investigação para exclusão de uma fonte produtora de androgênios. Doente de 66 anos, com menopausa espontânea aos 50, referenciada à consulta de ginecologia por hirsutismo após a menopausa, com agravamento no último ano e episódios demetrorragia que não valorizava. Ao exame objetivo tinha masculinização da voz, alopécia androgênica, aumento da pilosidade na face, tronco e membros e hipertrofia do clitóris.Feita ecografia endovaginal que revelou espessamento endometrial e ovários aumentados de volume para a idade; estudo analítico que demonstrou um valor de testosterona total elevado (225ng/dL); tomografia computadorizada da suprarrenal e ressonância magnética crânio-encefálica que não revelaram alterações; e histeroscopia com remoção de pólipo endometrial, associado a hiperplasia endometrial simples sem atipia. Submetida a histerectomia total com anexectomia bilateral. O estudo histológico concluiu tratar-se de hipertecose ovárica. Seis meses após a cirurgia apresentava normalização da testosterona sérica, acentuada redução do hirsutismo e melhoria da alopécia.O diagnóstico de hiperandrogenismo em mulheres na pós-menopausa constitui um desafio. Os meios complementares de diagnóstico nem sempre permitem detectar a origem da hiperandrogenemia. O tratamento da hipertecose ovárica melhora o hirsutismo e pode reduzir o risco de patologia maligna hormonodependente.

tMild clinical signs of hyperandrogenism such as hirsutism may arise during the menopausal transition as part of the aging process. However, development of virilization may be interpreted as the presence a specific source of androgen excess. 66 year-old menopausal woman, with a record of progressive hirsutism since menopausal age (50 years-old) exacerbated over the past year. Episodes of metrorrhagia were not valued by the patient. Physical examination showed deepening of voice, frontotemporal alopecia, hirsutism in face, trunk and limbs and hypertrophy of the clitoris. A transvaginal ultrasound revealed a thickened endometrium and enlarged ovaries considering her age and analytical study showed an increase of total testosterone (225ng/dL). No changes were detected at computerized tomography of adrenals and cerebral magnetic resonance. Performed an hysteroscopy with removal of endometrial polyp, associated to simple endometrial hyperplasia without atypia. The histological diagnosis after hysterectomy and bilateral salpingo-oophorectomy, revealed a ovarian hyperthecosis. Six months after surgery it was observed a testosterone within the normal range, marked hirsutism reduction and alopecia improvement.Diagnosis of hyperandrogenism in postmenopausal is a challenging task. Imaging techniques do not always reveal the source of excess androgens. The ovarian hyperthecosis treatment effectively improves hirsutism and reduces the risk of hormone-dependent tumors.

Actualización del síndrome de ovario poliquístico y su importancia en dermatología / Update of polycystic ovary syndrome and its importance in dermatology

El Síndrome de Ovario Poliquístico es un trastorno endocrino común que afecta a mujeres en edad reproductiva que puede causar problemas metabólicos y deterioro psicosocial importante. Las manifestaciones dermatológicas más frecuentes del hiperandrogenismo incluyen acné, hirsutismo y alopecia, cuyo reconocimiento es esencial para hacer un diagnóstico precoz. Las modalidades de tratamiento incluyen terapia hormonal con el objetivo de modular la producción de andrógenos y su acción, así como tratamientos no hormonales dirigidos a condiciones dermatológicas específicas.

Polycystic ovarian syndrome is a common endocrine disorder that affects women of reproductive age, which can cause metabolic, reproductive and psychosocial impairment. The most common skin manifestations of hyperandrogenism are acne, hirsutism and alopecia, whose recognition is essential for early diagnosis. There are several treatment modalities, including hormonal therapy in order to modulate androgen production and their effects, as well as other non hormonal treatments targeted to specific dermatologic conditions.

Calidad de vida relacionada con la salud en mujeres con ovario poliquístico / Health-related Quality of Life in Women with Polycystic Ovary Syndrome

El síndrome de ovario poliquístico es la alteración endocrino-metabólica que más frecuentemente afecta a las mujeres en edad reproductiva. Sus síntomas y signos característicos son las irregularidades menstruales, amenorrea, acné, hirsutismo, obesidad e infertilidad. El síndrome de ovario poliquístico tiene implicaciones significativas para la salud a largo plazo, que pueden conducir a morbilidad psicológica y a una calidad de vida disminuida. Este artículo revisa la literatura actual en relación con el síndrome de ovario poliquístico y la calidad de vida relacionada con la salud, y evalúa cómo algunas manifestaciones clínicas del síndrome de ovario poliquístico afectan la calidad de vida de las mujeres que lo padecen.

Polycystic ovary syndrome is the most frequent endocrine and metabolic disorder in women of reproductive age. Its characteristic signs and symptoms are menstrual irregularities, amenorrhea, acne, hirsutism, obesity and infertility. Polycystic ovary syndrome has major effects on long-term health, which can lead to psychological morbidity and decreased quality of life. This paper reviews the current literature on polycystic ovary syndrome and health-related quality of life; in addition, it assesses how some clinical manifestations of polycystic ovary syndrome affect the quality of life of women who suffer this disorder.

Hirsutism: diagnosis and treatment / Hirsutismo: diagnóstico e tratamento

Hirsutism is defined as excessive terminal hair growth in androgen-dependent areas of the body in women, which grows in a typical male distribution pattern. Hirsutism is a common clinical problem in women, and the treatment depends on the cause. The condition is often associated with a loss of self-esteem. Hirsutism reflects the interaction between circulating androgen concentrations, local androgen concentrations, and the sensitivity of the hair follicle to androgens. Polycystic ovary syndrome and idiopathic hirsutism are the most common causes of the condition. A woman’s history and, physical examination are particularly important in evaluating excess hair growth. The vast majority of women with hirsutism have the idiopathic variety, and the diagnosis is made by exclusion. Serum testosterone level > 200 ng/dL is highly suggestive of adrenal or ovarian tumor. Treatment of hirsutism should be based on the degree of excess hair growth presented by the patient and in the pathophysiology of the disorder. Treatment includes lifestyle therapies, androgen suppression, peripheral androgen blockage, and cosmetic treatments. The current review discusses definition, pathogenesis, physiopathology, differential diagnosis, diagnostic strategies, and treatment.

O hirsutismo é definido como o excesso de crescimento terminal de pelos em áreas dependentes de andrógenos no corpo de mulheres, com crescimento em um padrão de distribuição tipicamente masculino. O hirsutismo é um problema clínico comum em mulheres, e o tratamento depende da causa. A condição está geralmente associada com a perda de autoestima. O hirsutismo reflete a interação entre as concentrações de andrógenos circulantes, concentrações locais de andrógenos e a sensibilidade do folículo capilar aos androgênios. A síndrome dos ovários policísticos e o hirsutismo idiopático são as causas mais comuns do transtorno. O histórico e o exame físico são particularmente importantes na avaliação do excesso de crescimento de pelos. A maioria das mulheres com hirsutismo possui a variedade idiopática, e o diagnóstico é feito por exclusão. Uma concentração sérica de testosterona > 200 ng/dL é altamente sugestiva de tumor ovariano ou de adrenal. O tratamento do hirsutismo deve ser baseado no nível de excesso de crescimento dos pelos e a fisiopatologia do transtorno. O tratamento inclui alterações no estilo de vida, supressão de andrógenos, bloqueio periférico de andrógenos e tratamentos cosméticos. A presente revisão discute definição, patogênese, fisiopatologia, diagnóstico diferencial e estratégias de diagnóstico e tratamento.