Virtual planning as a resource for correcting severe facial asymmetry ­ case report / Planejamento virtual como recurso para correção de assimetria facial severa ­ relato de caso

A hiperplasia hemimandibular é responsável por prejuízos estéticos, funcionais, motores e psicossociais. Com etiologia incerta, ocorre frente ao desequilíbrio de fatores regulatórios de crescimento presentes na camada cartilaginosa do côndilo. O relato objetiva descrever a tomada de decisões baseada em exames complementares específicos aliados à adequada intervenção cirúrgica da lesão. Paciente gênero feminino, 33 anos de idade, compareceu à clínica particular com queixa principal de "face assimétrica", foi requerido uma avaliação cintilográfica objetivando e confirmando a interrupção do crescimento condilar, descartando a hipótese de Osteocondroma e condilectomia. Após preparo ortodôntico prévio, os exames tomográficos foram utilizados na criação de um protótipo que foi impresso após a realização dos movimentos ósseos planejados. Mediante à reconstrução, foi concluído que a assimetria presente não seria totalmente corrigida somente através da intervenção ortognática, sendo necessária também uma osteotomia removendo parte da base do corpo e ângulo mandibular, através da confecção de um guia de corte, promovendo à reanatomização sem a necessidade de acesso extra oral submandibular, evitando uma cicatriz em face feminina. Paciente encontra-se em pós-operatório de 60 meses, sem queixas e satisfeita. Portanto, é evidenciado cada vez mais a influência positiva que o planejamento virtual pode trazer aos profissionais na otimização dos resultados cirúrgicos.

Hemimandibular hyperplasia is responsible for aesthetic, functional, motor, and psychosocial impairments. With an uncertain etiology, it occurs due to the imbalance of regulatory growth factors present in the cartilaginous layer of the condyle. The report aims to describe decision-making based on specific complementary exams combined with the appropriate surgical intervention for the condition. A 33-year-old female patient presented at a private clinic with the main complaint of "asymmetric face." A scintigraphic evaluation was requested to objectively confirm the interruption of condylar growth, ruling out the hypothesis of Osteochondroma and condylectomy. After prior orthodontic preparation, tomographic exams were used to create a prototype that was printed after planned bone movements. Through the reconstruction, it was concluded that the existing asymmetry would not be entirely corrected through orthognathic intervention alone, necessitating also an osteotomy to remove part of the base of the body and mandibular angle. This was done through the creation of a cutting guide, allowing for reanatomization without the need for submandibular extraoral access, thus avoiding a scar on the female face. The patient is 60 months postoperative, with no complaints and satisfied. Therefore, the increasingly positive influence of virtual planning on optimizing surgical outcomes for professionals is evident.

Clinical application of the UroLift® prostatic urethral lift in Spain: consensus on the treatment of lower urinary tract symptoms associated with urinary flow obstruction and secondary to benign prostatic hyperplasia (BPH).

Benign prostatic hyperplasia (BPH) is an increasingly common pathology in the adult male. BPH increases after the age of 40-45 years, and its management consumes an enormous amount of resources. The UroLift® System is an approved technology designed to treat lower urinary tract symptoms (LUTS) secondary to BPH and is used to perform the prostatic urethral lift (PUL) procedure. Various urology specialists in Spain with experience in PUL have prepared this consensus document. Endorsed by the Spanish Urology Association, its information is based on the most recent findings. The main objective of this document is to disseminate the consensus recommendations among all professionals treating patients with LUTS/BPH. Both primary care physicians and urologists can assess and offer PUL as an effective, minimally invasive treatment.

Papillary endothelial hyperplasia (Masson's tumor) of the breast: A diagnostic challenge.

Papillary endothelial hyperplasia (PEH) or Masson's tumor is a rare benign vascular tumor that usually appears in the soft tissues of the head and neck, trunk and extremities, being extremely rare in the breast. Its diagnosis can be a challenge, especially in the follow-up of patients with previous disease of breast carcinoma. We present the case of a 65-year-old patient, with a history of bilateral breast cancer and reconstruction with implants, who presented a Masson's tumor during follow-up. An ultrasound scan was performed, showing a well-circumscribed mass in the left breast, located in the posterior contour of the implant. Subsequently, magnetic resonance imaging (MR) depicted an enhancing tumor, without infiltration of adjacent structures. Finally, the definitive anatomopathological diagnosis was obtained after surgical excision.

Gingivitis espongiótica. Manejo clínico no quirúrgico. Reporte de un caso / Spongiotic gingivitis. Non-surgical clinical management. Report of a case

Objetivo: La gingivitis espongiótica es una patología benigna poco común y una entidad clínica patológica poco frecuente que los odontólogos pueden observar durante la atención odontológica. Es importante conocer la misma para poder diagnosticarla adecuadamente, describir sus características clínicas y elaborar un plan para su tratamiento. El objetivo de este artículo es presentar un caso clínico de esta patología poco conocida, describir sus características y su manejo clínico. Caso clínico: Un niño de 7 años que ingresó derivado por su odontólogo a la cátedra de Estomatología de la Facultad de Odontología de la Universidad Nacional de Córdoba, Argentina, presentaba en la encía libre e insertada del maxilar superior una lesión con ausencia de sintomatología y evolución crónica, benigna y con cambios estéticos notorios. Se deriva a la cátedra de Periodoncia para realizar un tratamiento interdisciplinario. Tuvo una respuesta favorable al tratamiento de la lesión, y se realizó un seguimiento periódico de la misma evitando la técnica quirúrgica convencional para su tratamiento.(AU)

Aim: Spongiotic gingivitis is a rare benign pathology and a rare clinical pathological entity that dentists can observe during dental care. It is important to know it to be able to properly diagnose it, describe its clinical characteristics and develop a plan for its treatment. The aim of this article is to present a clinical case of this little-known pathology and describe its characteristics as well as its clinical management. Clinical case: A 7-yeard-old child who was referred by his dentist to the Department of Stomatology of the Faculty of Dentistry of the National University of Córdoba, Argentina, presented a lesion in the free and inserted gingiva of the upper jaw, with absence of symptoms and chronic, benign evolution with notable aesthetic changes. He is referred to the Periodontics department to perform interdisciplinary treatment. He had a favorable response to the treatment of the lesion, and a periodic follow-up was carried out, avoiding the conventional surgical technique for its treatment.(AU)

Benign prostatic hyperplasia. / Hiperplasia benigna de próstata.

Benign prostatic hyperplasia (BPH) is a histopathologic definition associated with enlargement of the prostate gland that causes obstruction of the lower urinary tract and manifests clinically with characteristic symptoms that are what bring patients for consultation. Urinary tract symptoms are common, especially in an increasingly aging population. Diagnosis and the decision on when and how to treat depend on the patient's quality of life and objective clinical parameters. An individualized, risk-based approach is necessary to guide conservative, pharmacologic, or surgical treatment.

Hiperplasia prostática benigna (HPB), sintomas do trato urinário inferior (LUTS) e função sexual / Benign Prostatic Hyperplasia (BPH), Lower Urinary Tract Symptoms (LUTS), and Sexual Function

A hiperplasia prostática benigna (HPB) representa o crescimento não maligno do tecido da próstata. Proliferação de células estromais e epiteliais na zona de transição da próstata causa compressão uretral e obstrução do fluxo vesical. Isso pode levar a manifestações de LUTS (lower urinary tract symptoms): urgência urinária, noctúria, dificuldades de micção, esvaziamento incompleto da bexiga, menor força e/ ou interrupção do jato e inflamações. Mecanismos do LUTS/HPB compartilhados afetam a função sexual masculina. Atividade aumentada de subtipos de receptores α1-adrenérgicos na próstata está associada à HPB. Tais receptores, também presentes no pênis, podem inibir a ereção, devido à HPB. Quanto à correlação entre LUTS e disfunção sexual, aventa-se que ambas resultem da contração anômala da musculatura lisa, por ativação dos receptores α1-adrenérgicos. LUTS/HPB causam desconforto nos homens, devido à obstrução urinária, ejaculação dolorosa, disfunção erétil (DE), distúrbios ejaculatórios e baixa libido, que prejudicam a qualidade de vida, deles e de suas parcerias. A noctúria interfere negativamente na qualidade do sono e na disposição para a atividade sexual. Tratamentos para LUTS/HPB podem induzir disfunções sexuais. Entre eles, ressecção transuretral (RTU), α1-bloqueadores, inibidores da 5α-redutase e terapia combinada (α1-bloqueador e 5α-redutase). Os efeitos prejudiciais do LUTS/HPB e de seu tratamento sobre a função sexual ainda são subdiagnosticados e insuficientemente tratados. A atividade sexual deve ser investigada antes e durante o tratamento, também orientando o paciente sobre os possíveis efeitos de cada opção terapêutica sobre a função sexual, evitando-se assim o abandono do tratamento.

Familial bilateral macronodular adrenal hyperplasia due to a novel ARMC 5 germline mutation: Clinical status and possible association with other neoplasms.

INTRODUCTION/OBJECTIVES: Mutations in the ARMC5 (armadillo repeat containing 5, OMIM 615549) gene, a putative tumor suppressor gene, have recently been identified as a common cause of sporadic and familial bilateral macronodular adrenal hyperplasia (BMAH). Familial BMAH is thought to be caused by two mutations, one germline and the other somatic, as suggested by the 2-hit theory. The objective is to describe a new mutation and develop its clinical characteristics and implications. METHODS, RESULTS AND CONCLUSIONS: We present an affected family with 11 members carrying a novel mutation of the ARMC5 gene (NM_001288767.1): c.2162T>C p. (Leu721Pro). Two of the carriers developed clinical Cushing's syndrome (CS), two mild autonomous cortisol secretion (MACS) and one presented with autonomous cortisol secretion (ACS). Four patients developed other tumors, three of whom died from this cause. It is not known whether these tumors could be related to the described mutation.

[Portosinusoidal vascular disorder: A paradigm shift]. / Enfermedad vascular portosinusoidal: un cambio de paradigma.

The term portosinusoidal vascular disorder (PSVD) refers to a clinical-pathological entity that encompasses those patients with intrahepatic vascular damage without cirrhosis at risk of developing severe complications of portal hypertension. Numerous systemic diseases, genetic disorders, and toxic agents have been associated with this pathology, making its diagnosis an important clinical challenge. The recent description of uniform diagnostic criteria and a better understanding of its pathophysiology will allow for better identification of patients, even in early stages of the disease. Although there is currently no effective etiological treatment available, early diagnosis allows for the development of preventive strategies for some severe complications of portal hypertension.

Changes in benign prostatic hyperplasia management in Valencia: a real-world evidence analysis.

INTRODUCTION AND OBJECTIVES: A training program was developed to increase general practitioners' engagement in the optimal management of Benign Prostatic Hyperplasia (BPH). The goal of this study was to evaluate changes in BPH management after the implementation of a training program. MATERIAL AND METHODS: This observational retrospective cohort study was conducted between 2019 and 2020. Aggregated data were analyzed in three evaluation periods (2010, 2012 and 2015), addressing quality indicators for diagnosis, treatment, and treatment outcomes. RESULTS: Overall, 118 795 patients who presented any data points were included. All quality indicators (number of IPSS and PSA determinations) increased between the first period and the last. Combination (α-blocker + 5-ARI) therapy was increasingly prescribed during the study periods whereas the proportion of prescriptions for single-agent α-blocker showed no significant differences among the periods analyzed. However, the total number of patients eligible for combination therapy who actually received this treatment was low in all periods (7.5%, 17.9%, and 20.1%, in 2010, 2012, and 2015, respectively). The outcome indicators revealed a decrease in referrals to the urology unit mostly among newly diagnosed patients. Even though the proportion of patients who underwent BPH-related surgeries increased significantly from the first to the second period, the number of surgeries remained stable between the second and third periods. CONCLUSIONS: The training program had a generally positive impact on the management of BPH patients in PC, but the overall study period may be insufficient to show an effect on some outcome indicators such as the number of surgeries.

Initial experience with thulium fiber laser for prostate enucleation: Analysis of the intraoperative and short-term outcomes in a prospective, multicenter cohort.

INTRODUCTION: Holmium laser enucleation of the prostate has rapidly become the gold standard for the surgical treatment of benign prostate hyperplasia, although thulium fiber laser (TFL) has also been postulated as an effective and safe alternative for prostate enucleation. The aim of this study is to describe our initial experience with the TFL for endoscopic enucleation of the prostate. MATERIAL AND METHODS: All patients proposed to TFL prostate enucleation were included in the analysis, regardless their prostate volume, catheter status and severity of symptoms, in 3 centers. Preoperative characteristics, intraoperative times and functional 3-months follow-up variables were collected, along with complications. RESULTS: Fifty-six patients were available, with a mean age of 68.7 years. Enucleation and morcellation efficiencies were 2.04 and 7.47 g/min, respectively. Median hospital stay was one day. Comparable functional data, pre and 3-month post-surgery was: mean prostate volume 88.9 vs 21.3 g, maximum urinary flow 13.2 vs 27.3 ml/s, post-void residual volume 149 vs 7.8 ml, prostatic specific antigen level 11.2 vs 1 ng/ml, and International Prostate Symptom Score 20.75 vs 3.96. Fourteen out of 56 (25%) patients presented with complications grade ≤2, according to the Clavien-Dindo classification. DISCUSSION: With wider evidence for other urological indications, very recent evidence about the suitability of TFL for prostate enucleation has arisen, since the first case described in 2021. Our results seem to back up these previous successful experiences as long as we obtained good intraoperative and short term follow-up functional results. However, there is still a need of longer follow-up data. CONCLUSIONS: TFL represents a novel technology for prostate enucleation, with a good intraoperative and short follow-up functional results, and a safety profile similar to the observed for those techniques that have been wider used for this indication. Further studies with longer follow-up periods and comparative with these other techniques are necessary.

Familial ApoB-specific familial hypobetalipoproteinemia in a patient with non-classical congenital adrenal hyperplasia. / Hipobetalipoproteinemia familiar ApoB específica en una paciente con hiperplasia suprarrenal congénita no clásica.

Familial hypobetalipoproteinaemia is a disorder of lipid metabolism characterized by low levels of total cholesterol, low-density lipoprotein cholesterol and apolipoprotein B. ApoB-related familial hypolipoproteinemia is an autosomal condition with a codominance inheritance pattern. Non-classical congenital adrenal hyperplasia is an autosomal recessive disorder due to mutations in the CYP21A2, a gene encoding for the enzyme 21-hydroxylase, which results in an androgen excess production from adrenal source. We here present the case of a 25-year-old woman with NCAH showing decreased levels of total-cholesterol, low-density lipoprotein cholesterol and triglycerides. Her parent had digestive symptoms and severe hepatic steatosis with elevated liver enzymes, as well as decreased levels of total and low-density lipoprotein cholesterol. A genetic-molecular study of the proband identified a mutation in the APOB gene, which allowed a diagnosis of heterozygous ApoB-related hypolipoproteinaemia to be made.

Evaluating the effects of preoperative treatment with 5-alpha reductase inhibitors in holmium laser enucleation of the prostate.

INTRODUCTION AND AIM: Patients treated with HoLEP are frequently treated with previous treatments, including 5-alpha-reductase inhibitors (5-ARIs). We investigated the impact of pretreatment with 5-ARIs on perioperative and immediate postoperative parameters in patients treated with HoLEP. MATERIAL AND METHODS: A retrospective study was performed using a prospectively collected database including all patients treated with HoLEP at our center between January 2017 and January 2023. The resected tissue weight, enucleation and morcellation efficiency (enucleation weight/time and morcellation weight/ time), postoperative complications, hospital stay and hemoglobin drop have been analyzed. RESULTS: A total of 327 patients were included. Of these, 173 (52.9%) were treated with 5-ARIs. No differences were found among the perioperative parameters investigated to determine efficiency. No differences were observed in peri- or postoperative complications, hospital stay or hemoglobin drop. CONCLUSIONS: Therapy with 5-ARIs had no impact on the immediate postoperative outcomes of patients treated with HoLEP. In our cohort, we observed that the use of 5-ARIs did not affect surgical efficiency, enucleation or morcellation. Further multicenter studies will be necessary to validate these findings.

Hiperplasia suprarrenal congénita clásica de presentación neonatal: reporte de un caso clínico / Neonatal classic congenital adrenal hyperplasia: clinical case study / Hiperplasia adrenal congênita clássica de apresentação neonatal: relato de caso clínico

Introducción: la hiperplasia suprarrenal congénita (HSC) comprende un conjunto de enfermedades hereditarias que involucran alteraciones en el ciclo del cortisol a nivel enzimático. La forma clásica tiene una incidencia de 1:14.000 a 1:18.000 nacimientos, mientras que la no clásica se presenta en 1:2.000 recién nacidos. Según la enzima involucrada, las manifestaciones clínicas varían desde asintomáticas a alteraciones en medio interno que comprometen la vida, por lo que debe tenerse un alto nivel de sospecha clínica para diagnosticarla en forma oportuna. En Uruguay, desde el año 2007, se cuenta con el pesquisaje de la 17-OH progesterona, producto aumentado en la forma más frecuente de HSC. El diagnóstico prenatal mediante la búsqueda de mutaciones en el gen CYP21A2, a través de punción de vellosidades coriales o amniocentesis, o del ADN fetal en sangre materna se recomienda en HSC con ambos padres portadores de la mutación severa y el antecedente de un hijo previo con la forma clásica. El tratamiento prenatal se considera en etapa experimental, con dexametasona en fetos femeninos con riesgo de enfermedad clásica, manteniéndose con la confirmación hasta el parto. Se presenta el caso clínico de una recién nacida de 11 días con HSC perdedora de sal y virilización de genitales externos, diagnosticada por la pesquisa neonatal. Se reporta su manejo interdisciplinario y evolución. Conclusiones: la hiperplasia suprarrenal es una enfermedad hereditaria potencialmente grave. La pesquisa neonatal constituye una herramienta efectiva para la detección de esta enfermedad. El manejo multidisciplinario es clave para el seguimiento y la optimización del tratamiento.

Introduction: congenital adrenal hyperplasia (CAH) involves a set of hereditary diseases that include alterations in the cortisol cycle, at enzymatic level. The classic variant has an incidence of 1:14,000 to 1:18,000 births, while the non-classic one occurs in 1:2,000 newborns. As a result of the enzyme involved, the clinical manifestations change from asymptomatic to alterations in the internal environment that compromise life, so clinical suspicion must be high in order to diagnose it in a timely manner. The diagnosis is more frequently made by neonatal screening than by physical examination, and it is a more sensitive method, especially in males, since there are no changes at the level of external genitalia. The implementation of screening has reduced the time prior to diagnosis. In Uruguay, since 2007, a universal screening has been carried out measuring 17-OH progesterone, which is increased in the most frequent form of CAH. Treatment is lifelong, consisting of oral glucocorticoids (hydrocortisone) and mineralocorticoids (fludrocortisone). We recommend prenatal diagnosis by searching for mutations in the CYP21A2 gene through chorionic villus puncture or amniocentesis, or fetal DNA in maternal blood in cases of CAH if both parents are carriers of the severe mutation and have a history of a previous classic case. Prenatal treatment with dexamethasone is considered in the experimental stage, in female fetuses at risk of the standard disease, which is maintained until birth if confirmed. We present a clinical case of an 11-day-old newborn with salt-wasting congenital adrenal hyperplasia and virilization of the external genitalia, diagnosed by neonatal screening. We report her management and interdisciplinary evolution. Conclusion: adrenal hyperplasia is a potentially serious inherited disease. Neonatal screening is an effective tool for detecting this disease. Multidisciplinary management is key to monitoring and optimizing treatment.

Introdução: a hiperplasia adrenal congênita (HAC) compreende um conjunto de doenças hereditárias que envolvem alterações no ciclo do cortisol, em nível enzimático. A forma clássica tem incidência de 1:14.000 a 1:18.000 nascimentos, enquanto a forma não clássica ocorre em 1:2.000 recém-nascidos. Dependendo da enzima envolvida, as manifestações clínicas variam de assintomáticas até alterações do ambiente interno que comprometem a vida, portanto é necessário ter um alto nível de suspeita clínica para diagnosticá-la em forma precoce. No Uruguai, desde 2007, existe triagem para 17-OH progesterona, produto aumentado na forma mais frequente de HAC. O diagnóstico pré-natal pela busca de mutações no gene CYP21A2 por meio de punção de vilosidades coriônicas ou amniocentese, ou DNA fetal no sangue materno é recomendado na HAC com ambos os pais portadores da mutação grave e história de filho anterior com a forma clássica. O tratamento pré-natal é considerado em fase experimental, com dexametasona em fetos femininos com risco de doença clássica, continuando com confirmação até o parto. É apresentado o caso clínico de um recém-nascido de 11 dias com hiperplasia adrenal congênita perdedora de sal e virilização da genitália externa, diagnosticado por triagem neonatal. Relatamos sua gestão interdisciplinar e evolução. Conclusões: a hiperplasia adrenal é uma doença hereditária potencialmente grave. A triagem neonatal é uma ferramenta eficaz para detectar esta doença. O manejo multidisciplinar é fundamental para monitorar e otimizar o tratamento.

Terapia termal con vapor de agua: variaciones técnicas entre hospitales universitarios españoles y eficacia a 2 años de seguimiento / Water vapor thermal therapy: technical variations among spanish hospitals and efficacy at 2-year follow-up

Objetivo El objetivo de este trabajo es conocer experiencia inicial con la terapia térmica con vapor de agua (TTVA) para hiperplasia benigna de próstata (HBP) en los hospitales universitarios españoles, así como describir las diferencias en cuanto a técnica y seguimiento observadas entre los centros. Materiales y métodos Este estudio multicéntrico observacional retrospectivo recogió características basales, datos quirúrgicos, posoperatorios y seguimiento a los uno, tres, seis, 12 y 24 meses, incluyendo cuestionarios validados, variaciones flujométricas, complicaciones y la necesidad de tratamiento farmacológico y quirúrgico tras el procedimiento. También se analizaron los posibles desencadenantes de retención aguda de orina (RAO) en el posoperatorio. Resultados Se incluyeron un total de 105 pacientes. No se observaron diferencias entre los grupos con y sin RAO con respecto a tiempo de sondaje (cinco y 4,3 días respectivamente, p = 0,178), ni volumen prostático (47,9 y 41,4 g, respectivamente, p = 0,147). La mejoría media a los tres, seis, 12 y 24 meses en cuanto de flujo máximo fue de 5,3, 5,2, 4,2 y 3,8 mL/s, respectivamente. En cuanto a la eyaculación, se observa una mejoría en la misma a partir de los tres meses de seguimiento que se mantiene en el tiempo. Conclusiones El tratamiento mínimamente invasivo de HBP con TTVA presenta buenos resultados funcionales a 24 meses de seguimiento, sin afectación significativa de la función sexual y con una baja incidencia de complicaciones. Existen pequeñas variaciones principalmente en cuanto al posoperatorio inmediato entre los hospitales participantes en el estudio. (AU)

Aim The aim of this work is to evaluate the initial experience with water vapor thermal therapy (WVTT) for benign prostatic hyperplasia (BPH) in Spanish university hospitals, as well as to describe the differences in technique and follow-up between centers. Materials and Methods This retrospective observational multicenter study collected baseline characteristics, surgical, postoperative and follow-up data at 1, 3, 6, 12 and 24 months, including validated questionnaires, flowmetric variations, complications, and the need for pharmacological or surgical treatment following the procedure. Possible triggers for postoperative acute urinary retention (AUR) were also analyzed. Results A total of 105 patients were included. No differences were observed between the groups with and without AUR with respect to catheterization time (5 and 4.3 days respectively, p = 0.178), or prostate volume (47.9 gr and 41.4 gr respectively, p = 0.147). The mean improvement at 3, 6, 12 and 24 months in terms of peak flow was 5.3, 5.2, 4.2 and 3.8 ml/s, respectively. As for ejaculation, an improvement was observed after 3 months of follow-up and was maintained over time. Conclusions Minimally invasive treatment for BPH with WVTT shows good functional outcomes at 24 months follow-up, without significant impairment of sexual function and a low incidence of complications. There are minor inter-hospital variations, mainly in the immediate postoperative period. (AU)

Função reprodutiva em portadoras de hiperplasia adrenal congênita: revisão narrativa da literatura / Reproductive health of patients with congenital adrenal hyperplasia: a narrative review

A hiperplasia adrenal congênita (HAC) pode cursar com redução da fertilidade na mulher. Entretanto, nos casos em que ocorre gestação, os recém-nascidos das por- tadoras de hiperplasia adrenal congênita exibem risco de hiperandrogenismo, com todas as suas consequências. A presente revisão atualiza o tema, considerando também as necessidades da assistência a essas pacientes. A busca identificou 294 artigos na base de dados MEDLINE/PubMed de 1961 a março/2023, e os resultados mostraram que as portadoras de hiperplasia adrenal congênita exibem significativa redução da fertilidade. Nos casos de interesse de gestação, as portadoras de hiper- plasia adrenal congênita devem fazer um planejamento reprodutivo, envolvendo a fase antenatal, o acompanhamento pré-natal especializado, o parto e o aleitamento.

Congenital adrenal hyperplasia may lead to reduced male and female fertility. Mo- reover, when the pregnancy occurs, the newborns of patients with congenital adrenal hyperplasia are at risk of hyperandrogenism with all its consequences. This review updates the theme and emphasizes assistance needs. The search identified 294 ar- ticles in the MEDLINE/PubMed database from 1961 to March/2023, and the results showed that patients with congenital adrenal hyperplasia truly exhibit a significant reduction in fertility. In cases of interest in pregnancy, patients with congenital adre- nal hyperplasia should carry out reproductive planning, involving the antenatal pha- se, specialized prenatal care, till delivery and breastfeeding.

Bilateral giant adrenal myelolipoma associated with congenital adrenal hyperplasia / Mielolipoma adrenal gigante bilateral associado a hiperplasia adrenal congênita

Adrenal myelolipomas are rare benign tumors, often non-functioning, located in the adrenal cortex, consisting mainly of mature adipose tissue and hematopoietic tissue. Although uncommon, the number of reported cases has increased due to the greater use of diagnostic imaging techniques. This tumor is usually unilateral and found as an adrenal incidentaloma, although there is a predominance of bilaterality in patients with congenital adrenal hyperplasia (CAH). In this study, we report the case of a 33-year-old male patient with CAH due to 21-hydroxylase deficiency, in non-regular use of the control medication, with bilateral giant adrenal myelolipoma and subsequent evolution of bilateral testicular adrenal rest tumors. He underwent bilateral adrenalectomy by video laparoscopy. The anatomopathological analysis, which confirmed myelolipomas' diagnosis, revealed the right adrenal with 430 g and 12.5 x 9.3 cm and the left with 257 g and 11.5 x 10.4 cm. This tumor may be accompanied by adrenocortical adenoma and carcinoma, ganglioneuroma, pheochromocytoma, Addison's disease, Cushing's syndrome, or CAH. Among the hypotheses of its pathogenesis, we highlight an association between the development of adrenal myelolipoma and chronic hormonal stimulation by the adrenocorticotrophic hormone (ACTH), especially in CAH. The non-regular treatment of CAH with glucocorticoids may have contributed to the chronic and elevated secretion of ACTH and, consequently, to the development of bilateral giant adrenal myelolipoma (AU).

Mielolipomas adrenais são tumores benignos raros, com frequência não-funcionantes, localizados no córtex da adrenal, constituídos, principalmente, por tecido adiposo maduro e tecido hematopoético. Apesar de incomum, o número de casos relatados tem aumentado devido ao maior uso de técnicas diagnósticas de imagens. Esse tumor é geralmente unilateral e encontrado como um incidentaloma adrenal, embora haja predominância de bilateralidade em casos de portadores de hiperplasia adrenal congênita (HAC). Neste estudo, relatamos o caso de um paciente do sexo masculino, de 33 anos, portador de HAC por deficiência de 21-hidroxilase, em uso não-regular da medicação de controle, com mielolipoma adrenal gigante bilateral e posterior evolução de tumor bilateral testicular de restos de adrenais. Ele foi submetido à adrenalectomia bilateral por videolaparoscopia. A análise anátomo-patológica, que confirmou o diagnóstico de mielolipomas, revelou adrenal direita com 430 g e 12,5 x 9,3 cm, e esquerda com 257 g e 11,5 x 10,4 cm. Esse tumor pode vir acompanhado de adenoma e carcinoma adrenocortical, glanglioneuroma, feocromocitoma, doença de Addison, Síndrome de Cushing ou HAC. Dentre as hipóteses de sua patogênese, des-tacamos uma associação entre o desenvolvimento do mielolipoma adrenal e a estimulação hormonal crônica pelo hormônio adrenocorticotrófico (ACTH), especialmente na HAC. O tratamento não-regular da HAC com glicocorticoides pode ter contribuído para a secreção crônica e elevada de ACTH e, consequentemente, para o desenvolvimento do mielolipoma adrenal gigante bilateral (AU).

Comparison of bipolar radiofrequency thermotherapy and transurethral prostate resection in treatment of benign prostate hyperplasia.

OBJECTIVES: Lower urinary tract symptoms due to benign prostatic hyperplasia in men increase with aging. Risks related to anesthesia and surgery have led a search for alternative treatments. Bipolar radiofrequency (RF) thermotherapy is one of the methods adopted in patients with high surgical risks. The aim of this study is to compare the effect of bipolar RF thermotherapy and transurethral resection of the prostate (TURP) methods on voiding symptoms and on post-operative complication rates especially in patients carrying high surgical risks. METHODS: Pre-operative, post-operative 1st and 6th month International Prostate Symptom Score (IPSS), Qmax, quality of life, prostate volumes, and postoperative complications of the patients underwent TURP and RF for benign prostatic hyperplasia (BPH) were compared. RESULTS: In the RF group, the pre-operative median IPSS was 30, prostate volume 41.5 cc, post-void residual (PVR) 80 ml, and Qmax is 5.85 ml/s.; In the TURP group, these were 29, 40 cc, 85 ml, and 5.3 ml/sec, respectively. In the Bipolar RF group, post-operative 1st- and 6th-month median values were IPSS 18, 21; prostate volume 40, 40; PVR 40, 35; Qmax 10.9, 9.15 and in the TURP group IPSS 9, 8; prostate volume 20, 20; PVR 30, 10; Qmax 17.25, 19.1, respectively. CONCLUSION: Bipolar RF thermotherapy is an applicable treatment method for BPH patients with high surgical risks.

OBJETIVOS: La termoterapia bipolar por radiofrecuencia es uno de los métodos adoptados en pacientes con alto riesgo quirúrgico. El objetivo de este estudio es comparar el efecto de la termoterapia de radiofrecuencia bipolar y los métodos de RTUP en los síntomas de vaciado y en las tasas de complicaciones posoperatorias, especialmente en pacientes con alto riesgo quirúrgico. MÉTODOS: Se compararon el IPSS, el Qmax, la calidad de vida, los volúmenes de próstata y las complicaciones posoperatorias de los pacientes sometidos a RTUP y RF para la HBP preoperatorios, posoperatorios al primer y sexto mes. RESULTADOS: En el grupo de RF, la mediana preoperatoria del IPSS fue de 30, el volumen prostático de 41.5 cc, el PVR de 80 ml y el Qmax de 5.85 ml/seg.; En el grupo RTUP estos fueron 29, 40 cc, 85 ml y 5.3 ml/seg, respectivamente. En el grupo de RF bipolar, los valores medianos postoperatorios del primer y sexto mes fueron IPSS 18, 21; volumen de próstata 40, 40; PVR 40, 35; Qmax 10.9, 9.15 y en el grupo TURP IPSS 9, 8; volumen de próstata 20, 20; PVR 30, 10; Qmax 17.25, 19.1, respectivamente. CONCLUSIÓN: La termoterapia de RF bipolar es un método de tratamiento aplicable para pacientes con HPB con alto riesgo quirúrgico.

Importância do diagnóstico por imagem em pacientes com sangramento uterino anormal / Importance of diagnostic imaging in patients with abnormal uterine bleeding

O sangramento uterino anormal é diagnóstico sindrômico comum no consultório do ginecologista e pode comprometer substancialmente a qualidade de vida. O objetivo no diagnóstico de sangramento uterino anormal é distinguir pacientes com causas estruturais (anatômicas), como pólipo, adenomiose, leiomioma, malignidade e hiperplasia, de pacientes que apresentam anatomia normal, nas quais o sangramento pode ser devido a alteração dos mecanismos de coagulação, distúrbios ovulatórios, distúrbios primários do endométrio, iatrogenia, ou ter outra causa não classificada. O diagnóstico se inicia a partir de anamnese detalhada e exame físico geral e ginecológico completos, seguidos da solicitação de exames complementares (laboratoriais e de imagem), conforme indicado. O exame de imagem de primeira linha para identificação das causas estruturais inclui a ultrassonografia pélvica. Histerossonografia, histeroscopia, ressonância magnética e amostragem endometrial para exame de anatomia patológica são opções que podem ser incluídas no diagnóstico a depender da necessidade. O objetivo deste artigo é apresentar a relevância dos exames de imagem na investigação das causas de sangramento uterino anormal.

Abnormal uterine bleeding is one of the commonest presenting complaints encountered in a gynecologist's office and may substantially affect quality of life. The aim in the diagnosis of abnormal uterine bleeding is to distinguish women with anatomic causes such as polyp, adenomyosis, leiomyoma, malignancy and hyperplasia from women with normal anatomy where the cause may be coagulopathy, ovulatory disorders, endometrial, iatrogenic and not otherwise classified. Diagnosis begins with a thorough history and physical examination followed by appropriate laboratory and imaging tests as indicated. The primary imaging test for the identification of anatomic causes include ultrasonography. Saline infusion sonohysterography, magnetic resonance, hysteroscopy, endometrial sampling are options that can be included in the diagnosis depending on the need. The aim of this article is to present the relevance of imaging exams in the investigation of the causes of abnormal uterine bleeding.

Water vapor thermal therapy: Technical variations among spanish hospitals and efficacy at 2-year follow-up.

AIM: The aim of this work is to evaluate the initial experience with water vapor thermal therapy (WVTT) for benign prostatic hyperplasia (BPH) in Spanish university hospitals, as well as to describe the differences in technique and follow-up between centers. MATERIALS AND METHODS: This retrospective observational multicenter study collected baseline characteristics, surgical, postoperative and follow-up data at 1, 3, 6, 12 and 24 months, including validated questionnaires, flowmetric variations, complications, and the need for pharmacological or surgical treatment following the procedure. Possible triggers for postoperative acute urinary retention (AUR) were also analyzed. RESULTS: A total of 105 patients were included. No differences were observed between the groups with and without AUR with respect to catheterization time (5 and 4.3 days respectively, P=.178), or prostate volume (47.9g and 41.4g respectively, P=.147). The mean improvement at 3, 6, 12 and 24 months in terms of peak flow was 5.3, 5.2, 4.2 and 3.8ml/s, respectively. As for ejaculation, an improvement was observed after 3 months of follow-up and was maintained over time. CONCLUSIONS: Minimally invasive treatment for BPH with WVTT shows good functional outcomes at 24 months follow-up, without significant impairment of sexual function and a low incidence of complications. There are minor inter-hospital variations, mainly in the immediate postoperative period.