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Giant Bilateral Adrenal Myelolipomas in a Non-Compliant Patient with Congenital Adrenal Hyperplasia.The American Journal of Case Reports Apr 2024BACKGROUND 21-hydroxylase deficiency, an essential enzyme for glucocorticoid and mineralocorticoid synthesis, is the cause of congenital adrenal hyperplasia (CAH) in...
BACKGROUND 21-hydroxylase deficiency, an essential enzyme for glucocorticoid and mineralocorticoid synthesis, is the cause of congenital adrenal hyperplasia (CAH) in more than 95% of cases. It is an autosomal recessive disorder encoded by the CYP21A2 gene, categorized into classical forms, which encompass the salt-wasting (SW) and simple virilizing (SV) forms, as well as the nonclassical form (NC). The aim of medical treatment is to replace missing glucocorticoids and, if necessary, mineralocorticoids, while also reducing elevated adrenal androgens. CASE REPORT We present the case of a 42-year-old woman with CAH who discontinued therapy during adolescence and was admitted to hospital with fatigue, nausea, and severe abdominal pain. A CT scan showed an extreme enlargement of the adrenal glands. Laboratory tests revealed elevated levels of 17-hydroxyprogesterone and other adrenal androgens, along with normal plasma metanephrine levels. Decreased morning cortisol levels suggested partial adrenal insufficiency requiring glucocorticoid replacement therapy. Due to the development of several serious complications and clinical deterioration, the multidisciplinary team recommended bilateral removal of masses measuring 300×250×200 mm on the right side and 250×200×200 mm on the left side. Histological and immunochemical examination confirmed the presence of giant myelolipomas with adrenal cortex hyperplasia. CONCLUSIONS Adrenal tumors, particularly myelolipomas, have a higher prevalence in patients with CAH. Our case report provides further evidence of the suspected link between non-compliant CAH therapy and the development of myelolipomas, along with promotion of their pronounced growth.
Topics: Adult; Female; Humans; Adrenal Gland Neoplasms; Adrenal Glands; Adrenal Hyperplasia, Congenital; Glucocorticoids; Lipoma; Myelolipoma; Steroid 21-Hydroxylase
PubMed: 38582958
DOI: 10.12659/AJCR.943005 -
Journal of Plastic, Reconstructive &... May 2024The increasing number of esthetic procedures emphasizes the need for effective evaluation methods of outcomes. Current practices include the individual practitioners'... (Comparative Study)
Comparative Study
BACKGROUND
The increasing number of esthetic procedures emphasizes the need for effective evaluation methods of outcomes. Current practices include the individual practitioners' judgment in conjunction with standardized scales, often relying on the comparison of before and after photographs. This study investigates whether comparative evaluations influence the perception of beauty and aims to enhance the accuracy of esthetic assessments in clinical and research settings.
OBJECTIVE
To compare the evaluation of attractiveness and gender characteristics of faces in group-based versus individual ratings.
METHODS
A sample of 727 volunteers (average age of 29.5 years) assessed 40 facial photographs (20 male, 20 female) for attractiveness, masculinity, and femininity using a 5-point Likert scale. Each face was digitally edited to display varying ratios in four lip-related proportions: vertical lip position, lip width, upper lip esthetics, and lower lip esthetics. Participants rated these images both in an image series (group-based) and individually.
RESULTS
Differences in the perception of the most attractive/masculine/feminine ratios for each lip proportion were found in both the group-based and individual ratings. Group ratings exhibited a significant central tendency bias, with a preference for more average outcomes compared with individual ratings, with an average difference of 0.50 versus 1.00. (p = 0.033) CONCLUSION: A central tendency bias was noted in evaluations of attractiveness, masculinity, and femininity in group-based image presentation, indicating a bias toward more "average" features. Conversely, individual assessments displayed a preference for more pronounced, "non-average" appearances, thereby possibly pointing toward a malleable "intrinsic esthetic blueprint" shaped by comparative context.
Topics: Humans; Female; Male; Adult; Beauty; Esthetics; Face; Photography; Masculinity; Femininity; Young Adult; Adolescent; Middle Aged; Lip; Surveys and Questionnaires
PubMed: 38582052
DOI: 10.1016/j.bjps.2024.03.012 -
American Journal of Men's Health 2024Mental ill-health and suicide represent a significant proportion of the burden of global disease among men. Connell's relational theory of masculinities provides a... (Review)
Review
Mental ill-health and suicide represent a significant proportion of the burden of global disease among men. Connell's relational theory of masculinities provides a useful framework to explore how mental health literacy, mental health stigma, and delayed help-seeking and help-offering behaviors are associated with mental ill-health among men, particularly within male-dominated industries. To address the high incidences of mental ill-health in male-dominated industries, several workplace interventions targeting these outcomes have been implemented. No review to date has examined the current state of evidence for these interventions or identified the behavior change techniques used. This review was restricted to empirical, quantitative research reporting on psychosocial interventions targeting mental health literacy, stigma, and help-seeking and help-offering behaviors in male-dominated industries. Quality appraisal was completed using the Effective Public Health Practice Project and a narrative synthesis was conducted. Twelve articles were included for review which reported on four distinct interventions. The methodological quality of two articles was strong, three moderate and seven weak. The strongest evidence of intervention effects related to mental health literacy and help-seeking intentions. There was less evidence relating to help-offering and help-seeking behaviors and mental health stigma. Sixteen behavior change techniques were identified across interventions that are discussed in relation to the wider men's health literature. The evidence on psychosocial interventions in male-dominated industries is limited due to methodological and conceptual issues. Recommendations for future research include standardized reporting of intervention descriptions, the use of theory to guide intervention development, and utilizing validated and reliable outcome measures.
Topics: Humans; Male; Mental Health; Health Literacy; Workplace; Social Stigma; Masculinity; Help-Seeking Behavior
PubMed: 38581228
DOI: 10.1177/15579883241236223 -
Anales de Pediatria May 2024
Topics: Humans; Adrenal Gland Neoplasms; Virilism; Female; Diagnosis, Differential; Adrenal Hyperplasia, Congenital
PubMed: 38580598
DOI: 10.1016/j.anpede.2024.03.019 -
Endocrinology, Diabetes & Metabolism... Apr 2024Total testosterone, which is peripherally converted to its biologically active form dihydrotestosterone (DHT), is the first-line hormone investigation in hyperandrogenic...
SUMMARY
Total testosterone, which is peripherally converted to its biologically active form dihydrotestosterone (DHT), is the first-line hormone investigation in hyperandrogenic states and infertility in premenopausal women. Polycystic ovary syndrome (PCOS), the most common cause of hyperandrogenism and infertility in young women, is often associated with mild elevations of total testosterone. Whereas very high levels of total testosterone (>2-3 SD of normal reference), are most often associated with hyperandrogenic signs, menstrual irregularity, rapid onset of virilization, and demand a prompt investigation. Herein, we report a case of a 32-year-old woman who was referred to the endocrinology outpatient clinic due to secondary amenorrhea and extremely high testosterone levels without any virilization signs. We initially suspected pitfalls in the testosterone laboratory test. Total serum testosterone decreased after a diethyl-ether extraction procedure was done prior to the immunoassay, but testosterone levels were still elevated. An ovarian steroid-cell tumor (SCT) was then revealed, which was thereby resected. Twenty-four hours post surgery, the total testosterone level returned to normal, and a month later menstruation resumed. This case emphasizes that any discrepancy between laboratory tests and the clinical scenario deserves a rigorous evaluation to minimize misinterpretation and errors in diagnosis and therapeutic approach. Additionally, we describe a possible mechanism of disease: a selective peripheral target-tissue response to high testosterone levels that did not cause virilization but did suppress ovulation and menstruation.
LEARNING POINTS
Total testosterone is the most clinically relevant hormone in investigating hyperandrogenic states and infertility in premenopausal women. Very high total testosterone levels in women (>2-3 SD of normal reference) are most often associated with hyperandrogenic signs, menstrual irregularities, and a rapid onset of virilization. In women with very elevated testosterone levels and the absence of clinical manifestations, laboratory interference should be suspected, and diethyl ether extraction is a useful technique when other methods fail to detect it. Ovarian steroid cell tumors (SCT) encompass a rare subgroup of sex cord-stromal tumors and usually secrete androgen hormones. SCTs are clinically malignant in 25-43% of cases. A selective response of peripheral target tissues to testosterone levels, with clinical manifestations in some tissues and no expression in others, may reflect differences in the conformation of tumor-produced testosterone molecules.
PubMed: 38579789
DOI: 10.1530/EDM-23-0117 -
American Journal of Men's Health 2024Type II diabetes is increasingly becoming a problem in Latin American countries such as Peru. People living with diabetes must incorporate several behavioral changes in...
Type II diabetes is increasingly becoming a problem in Latin American countries such as Peru. People living with diabetes must incorporate several behavioral changes in their everyday lives, which are done outside the purview of medical professionals. Support from friends and family members is essential to the successful management of any chronic condition. Our study discusses the role of family involvement in supporting the management of diabetes among Peruvian men and examines how masculine norms play a role in the way such support is received and perceived, and their influence in motivation to adhere to treatment recommendations. In-depth interviews with 20 men from a low socioeconomic status, aged 27 to 68 with a diagnosis of Type II diabetes were conducted. Our analysis suggests the importance of the close, complex, and integrated experience that connects family members and patients with a chronic condition. Participant accounts demonstrate they receive multiple forms of support from a diverse range of social relationships. The overwhelming majority of the people giving the support were female and were especially significant in supporting management practices. The participants' accounts were able to demonstrate how living with a chronic condition, such as diabetes, affects the whole family-physically, mentally, and emotionally-and they experience the disease as one unit. Our study demonstrates the need for a family health experience approach that considers masculine gender norms around health and provides relevant insights to inform family-based treatments and therapies to allow for more and better targeted health care for men.
Topics: Humans; Male; Female; Peru; Family Support; Diabetes Mellitus, Type 2; Chronic Disease; Perception; Masculinity
PubMed: 38567927
DOI: 10.1177/15579883241239552 -
Acta Medica Indonesiana Jan 2024Hypogonadism is a condition characterized by diminished or absent production of sex hormones by the testicles in men and the ovaries in women. Hypogonadism is classified...
Hypogonadism is a condition characterized by diminished or absent production of sex hormones by the testicles in men and the ovaries in women. Hypogonadism is classified into primary and secondary hypogonadism. Each type of hypogonadism can be caused by congenital and acquired factors. There are many factors that contribute to the occurrence of hypogonadism, including genetic and developmental disorders, infection, kidney disease, liver disease, autoimmune disorders, chemotherapy, radiation, surgery, and trauma. This represents the considerable challenge in diagnosing hypogonadism.The goals of treatment include restore sexual functionality and well-being, initiating and sustaining virilization, osteoporosis prevention, normalize growth hormone levels in elderly men if possible, and restoring fertility in instances of hypogonadotropic hypogonadism. The main approach to treating hypogonadism is hormone replacement therapy. Male with prostate cancer, breast cancer, and untreated prolactinoma are contraindicated for hormone replacement therapy. When selecting a type of testosterone therapy for male with hypogonadism, several factors need to be considered, such as the diversity of treatment response and the type of testosterone formulation. The duration of therapy depends on individual response, therapeutic goals, signs and symptoms, and hormonal levels. The response to testosterone therapy is evaluated based on symptoms and signs as well as improvements in hormone profiles in the blood. Endocrine Society Clinical Practice Guideline recommend therapeutic goals based on the alleviation of symptoms and signs, as well as reaching testosterone levels between 400 - 700 ng/dL (one week after administering testosterone enanthate or cypionate) and maintaining baseline hematocrit.Hormone therapy is the primary modality in the management of hypogonadism. The variety of signs and symptoms makes early diagnosis of this condition challenging. Moreover, administering hypogonadism therapy involves numerous considerations influenced by various patient factors and the potential for adverse effects. This poses a challenge for physicians to provide targeted hypogonadism therapy with minimal complications.
Topics: Humans; Male; Female; Aged; Hypogonadism; Testosterone; Testis; Hormone Replacement Therapy
PubMed: 38561883
DOI: No ID Found -
Cureus Mar 2024Hyperandrogenism in postmenopausal females may arise from either ovarian or adrenal sources and can pose a challenging diagnostic dilemma. We present the case of a...
Hyperandrogenism in postmenopausal females may arise from either ovarian or adrenal sources and can pose a challenging diagnostic dilemma. We present the case of a 66-year-old female with postmenopausal hyperandrogenism with virilization, adrenal incidentaloma, and concurrent finding of two extremely rare ovarian tumors, including bilateral Leydig cell tumor and Brenner tumor. Laboratory tests showed elevated testosterone and androstenedione and normal dehydroepiandrosterone sulfate (DHEAS). Response to 1 mg overnight dexamethasone suppression test demonstrated persistently elevated testosterone and incomplete suppression of androstenedione. Computed tomography (CT) scan showed a left adrenal nodule and an unremarkable appearance of the ovaries. The pelvic ultrasound did not show an ovarian tumor on the right ovary, and the left ovary was not seen. Adrenal and ovarian vein sampling suggested the ovaries as the source of the testosterone. Given the ovarian vein sampling results, a multidisciplinary discussion between endocrinology and gynecologic oncology concluded that bilateral salpingo-oophorectomy (BSO) was the next best step for diagnosis and management. Laparoscopic BSO was performed. Histopathology showed bilateral Leydig cell tumors and a left ovarian Brenner tumor. At one-year postoperative follow-up, alopecia improved, and testosterone level normalized. This case highlights the importance of diagnostic pathways and interdisciplinary collaboration in managing rare clinical scenarios of hyperandrogenism in postmenopausal females. As in our case, surgeons may be hesitant to remove normal-appearing ovaries. While the three presented tumor types in this case arise from distinct tissues and exhibit different histological characteristics, the presence of such a unique triad prompts consideration of potential unifying pathogenic mechanisms.
PubMed: 38559537
DOI: 10.7759/cureus.55334 -
Gynecological Endocrinology : the... Mar 2024To highlight the challenges in diagnosing 46, XY disorder of sex development related to mutation. (Review)
Review
OBJECTIVE
To highlight the challenges in diagnosing 46, XY disorder of sex development related to mutation.
METHODS
We present an unusual case of a 12-year-old female child came for enlargement of clitoris and initially diagnosed as partial androgen insensitivity syndrome (AIS).
RESULTS
On examination, the patient's vulva was found virilized with 3cm-long clitoris. Her peripheral blood karyotype was 46, XY. The ultrasound showed an empty pelvis and hormone results confirmed hyperandrogenism. Therefore, the partial AIS was suspected, but the following whole exon sequencing indicates a pathological missense mutation in . Further investigation and surgery did not reveal any brain, heart, lung or diaphragm lesions related to MYRF, but only maldeveloped internal genitalia and a persistent urachus. Her serum testosterone dropped to normal after surgical removal of the remaining ipsilateral testis and epididymitis without spermatogenesis as shown by pathology.
CONCLUSION
Due to the karyotype, hyperandrogenism, empty pelvis but a virilism after puberty, the patient was initially diagnosed as partial AIS. This misleading clinical diagnose will not be verified as the mutation if without the whole exon sequencing, particularly in the absence of obvious brain, heart, lung and diaphragm lesions as in this case.
Topics: Child; Female; Humans; Male; Androgen-Insensitivity Syndrome; Hyperandrogenism; Mutation; Receptors, Androgen; Sexual Development; Transcription Factors; Membrane Proteins
PubMed: 38547923
DOI: 10.1080/09513590.2024.2331072 -
Frontiers in Sociology 2024Recent movements like #MeToo and #TimesUp have surfaced and challenged ideas about masculinity in popular conversations. In particular, these ideas have centered around... (Review)
Review
Recent movements like #MeToo and #TimesUp have surfaced and challenged ideas about masculinity in popular conversations. In particular, these ideas have centered around "toxic masculinity"-a version of masculinity that reflects stereotyped, dated, and even dangerous expectations for manhood. This notion of masculinity can be reinforced in a number of ways, especially through pop culture, where it runs the risk of becoming commonly accepted or normalized. This study evaluates the narratives of masculinity in three different novels that are marketed toward high school-aged students in the United States: ; ; and . Using a critical literacy lens, this study considers the symbols, behaviors, expectations, and meanings given to masculinity in and through each novel and considers the implications of this analysis for adolescent readership inside and outside of schooling. The study concludes that the more contemporary novels showcase a range of masculine portrayals, including positive, affirming versions of masculinity, compared to a more singular and pessimistic one found in a novel traditionally used in schools. Thus, the study concludes that formal schooling may be an important way to address and disrupt unhealthy versions of masculinity.
PubMed: 38544790
DOI: 10.3389/fsoc.2024.1329041