-
Rare : Open Research in Rare Diseases 2024POEMS Syndrome is a constellation of findings including Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal plasma cell disorder, and Skin changes. Calciphylaxis, a...
POEMS Syndrome is a constellation of findings including Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal plasma cell disorder, and Skin changes. Calciphylaxis, a microangiopathy involving vascular calcification and thrombotic occlusions, occurs rarely in POEMS. We present a case of prominent calciphylaxis that antedated the diagnosis of POEMS. The patient presented with extensive ecchymoses progressing to necrotic lesions in the setting of acute renal injury. Previously, she had chronic slowly progressive polyneuropathy, splenomegaly, hypothyroidism, amenorrhea, and ascites. Calciphylaxis was diagnosed on skin biopsy, and POEMS was diagnosed based upon clinical findings plus a bone marrow biopsy showing 15% lambda chain restricted plasma cells. Treatment for the calciphylaxis was supportive with fluids, tissue debridement, wound vacuum devices and antibiotics for secondary infection. Myeloma was treated with bortezomib and steroids. All aspects of the patient's manifestations improved. We conclude that calciphylaxis can be a prominent feature of POEMS and can appear prior to recognition of the full-blown syndrome.
PubMed: 38435320
DOI: 10.1016/j.rare.2024.100019 -
Frontiers in Public Health 2024Among adolescents, amenorrhea is a common reason for medical consultation. Despite the variety of underlying etiologies, the prevalence of the causes is incompletely...
OBJECTIVE
Among adolescents, amenorrhea is a common reason for medical consultation. Despite the variety of underlying etiologies, the prevalence of the causes is incompletely understood. This study aimed to assess the demographic and etiological factors among patients with amenorrhea treated in a single specialist unit of adolescent gynecology.
DESIGN
Retrospective register study.
METHODS
Medical records of 438 girls evaluated for primary or secondary amenorrhea in a single tertiary care center between 2015 and 2019 were retrospectively reviewed. In all, 423 patients-171 with primary amenorrhea and 252 with secondary amenorrhea-were included in the study. Data on underlying conditions, anthropometric variables, and selected hormonal markers were analyzed.
RESULTS
Functional hypogonadotropic hypogonadism was the most frequent reason for primary (56%) and secondary (78%) amenorrhea. It was mostly explained by lifestyle-related functional hypothalamic amenorrhea caused by disordered eating, intense exercise, energy deficiency, psychological stress, and their combinations.
CONCLUSION
Menstrual pattern is a significant indicator of overall health and well-being among adolescent girls and young women. Functional reasons behind primary and secondary amenorrhea are important to recognize. Treatment often requires long-term lifestyle modifications. The frequency of functional causes also implies that most amenorrhea cases are preventable.
Topics: Adolescent; Humans; Female; Amenorrhea; Retrospective Studies; Anthropometry; Behavior Therapy; Exercise
PubMed: 38435281
DOI: 10.3389/fpubh.2024.1304277 -
Cureus Jan 2024Interstitial ectopic pregnancy is rare (2%-4% of ectopic pregnancies). The atypical clinical presentation of interstitial ectopic pregnancy associated with massive...
Interstitial ectopic pregnancy is rare (2%-4% of ectopic pregnancies). The atypical clinical presentation of interstitial ectopic pregnancy associated with massive vaginal bleeding is extremely rare and makes early preoperative diagnosis even more difficult. The presentation of our case concerns the early diagnosis and surgical treatment of a patient with an interstitial ectopic pregnancy without rupture, which presented atypically with painless, severe vaginal bleeding. A 27-year-old fourth-term pregnant woman presented with massive painless vaginal bleeding. Secondary amenorrhea was calculated at eight weeks and four days. Transvaginal ultrasound and transvaginal Doppler ultrasound combined with the quantification of beta-chorionic gonadotropin hormone raised the suspicion of interstitial ectopic pregnancy. Intraoperatively, the presence of a large swelling of the right horn of the uterus was established, and a wedge resection was performed with the removal of the corresponding fallopian tube. Three weeks after surgery, the serum beta-chorionic gonadotropin hormone value was zero. In this paper, the rarity of interstitial ectopic pregnancy, the difficulties related to early and correct preoperative diagnosis, and the selection of the appropriate available therapeutic procedures are emphasized, the correct application of which can significantly contribute to reducing the morbidity and mortality of these patients.
PubMed: 38425637
DOI: 10.7759/cureus.53225 -
PLoS Computational Biology Feb 2024The hypothalamus is the central regulator of reproductive hormone secretion. Pulsatile secretion of gonadotropin releasing hormone (GnRH) is fundamental to physiological...
The hypothalamus is the central regulator of reproductive hormone secretion. Pulsatile secretion of gonadotropin releasing hormone (GnRH) is fundamental to physiological stimulation of the pituitary gland to release luteinizing hormone (LH) and follicle stimulating hormone (FSH). Furthermore, GnRH pulsatility is altered in common reproductive disorders such as polycystic ovary syndrome (PCOS) and hypothalamic amenorrhea (HA). LH is measured routinely in clinical practice using an automated chemiluminescent immunoassay method and is the gold standard surrogate marker of GnRH. LH can be measured at frequent intervals (e.g., 10 minutely) to assess GnRH/LH pulsatility. However, this is rarely done in clinical practice because it is resource intensive, and there is no open-access, graphical interface software for computational analysis of the LH data available to clinicians. Here we present hormoneBayes, a novel open-access Bayesian framework that can be easily applied to reliably analyze serial LH measurements to assess LH pulsatility. The framework utilizes parsimonious models to simulate hypothalamic signals that drive LH dynamics, together with state-of-the-art (sequential) Monte-Carlo methods to infer key parameters and latent hypothalamic dynamics. We show that this method provides estimates for key pulse parameters including inter-pulse interval, secretion and clearance rates and identifies LH pulses in line with the widely used deconvolution method. We show that these parameters can distinguish LH pulsatility in different clinical contexts including in reproductive health and disease in men and women (e.g., healthy men, healthy women before and after menopause, women with HA or PCOS). A further advantage of hormoneBayes is that our mathematical approach provides a quantified estimation of uncertainty. Our framework will complement methods enabling real-time in-vivo hormone monitoring and therefore has the potential to assist translation of personalized, data-driven, clinical care of patients presenting with conditions of reproductive hormone dysfunction.
Topics: Male; Female; Humans; Bayes Theorem; Luteinizing Hormone; Gonadotropin-Releasing Hormone; Follicle Stimulating Hormone; Hypothalamus
PubMed: 38422116
DOI: 10.1371/journal.pcbi.1011928 -
Archives of Gynecology and Obstetrics May 2024The Kyleena™ Satisfaction Study (KYSS) provided the first data on 19.5 mg levonorgestrel-releasing intrauterine system (LNG-IUS-19.5 mg) use in routine clinical... (Observational Study)
Observational Study
PURPOSE
The Kyleena™ Satisfaction Study (KYSS) provided the first data on 19.5 mg levonorgestrel-releasing intrauterine system (LNG-IUS-19.5 mg) use in routine clinical practice. Here we report results from the German participants in KYSS.
METHODS
This prospective, observational, single-arm cohort study recruited women who independently chose to use LNG-IUS-19.5 mg during routine counseling in Germany. Overall satisfaction and bleeding profile satisfaction, continuation rates, and safety profile were evaluated at 12 months or premature end of observation (EoO).
RESULTS
In the German study population, LNG-IUS-19.5 mg placement was attempted in 508 women and successful in 506 women. Mean age was 32.3 years, and 60.0% (n = 305/508) were parous. Placement was considered easy and associated with no more than mild pain, even in younger and nulliparous participants. Of those with satisfaction data available, 87.6% (n = 388/443) were satisfied with LNG-IUS-19.5 mg at 12 months/EoO. Satisfaction was similar for parous (86.9%, n = 238/274) and nulliparous (88.8%, n = 150/169) women, and was independent of age, prior contraceptive method, or reason for choosing LNG-IUS-19.5 mg. Most participants (73.6%, n = 299/406) were also satisfied with their bleeding profile at 12 months/EoO, independent of parity, age, prior contraceptive method, presence of amenorrhea or dysmenorrhea severity. The 12-month continuation rate was 84.1% (n = 427/508). Most discontinuations were due to loss to follow-up (8.5%, n = 43/508) or treatment-emergent adverse events (TEAEs) (4.7%, n = 24/508). TEAEs were reported in 12.6% (n = 64) of participants, with 9.3% (n = 47) considered to have an LNG-IUS-19.5 mg-related TEAE.
CONCLUSION
Our real-world findings on LNG-IUS-19.5 mg use in German KYSS participants reflected its suitability for a broad population, including young and nulliparous women.
CLINICAL TRIAL REGISTRATION
NCT03182140 (date of registration: June 2017).
Topics: Pregnancy; Female; Humans; Adult; Levonorgestrel; Contraceptive Agents, Female; Prospective Studies; Cohort Studies; Intrauterine Devices, Medicated; Germany; Dysmenorrhea
PubMed: 38421421
DOI: 10.1007/s00404-024-07421-5 -
Journal of Surgical Case Reports Feb 2024An ectopic pregnancy occurs when the fertilized egg is implanted and develops outside the endometrium, i.e. in the fallopian tubes, cervix, ovary, or abdomen. It...
An ectopic pregnancy occurs when the fertilized egg is implanted and develops outside the endometrium, i.e. in the fallopian tubes, cervix, ovary, or abdomen. It commonly presents with a history of amenorrhoea, lower abdominal pain, and slight vaginal bleeding. The fallopian tube is the most typical location for ectopic pregnancy. Two percent of reported pregnancies are ectopic pregnancy. Ectopic pregnancy remains a public health threat for women in reproductive age, and a major cause of maternal mortalities in the first trimester. In East Africa, these reports are limited, despite a great need for documentation addressing key considerations for diagnosis and management of ectopic pregnancy in these resource limited settings. In this case study, we report on 26-years-old female Gravida 5 Para 4 Living 4, who reported history of amenorrhoea for 3 months complaining of slight per vagina bleeding and lower abdominal pain for 5 days more marked at left iliac region along with generalized weakness for 2 weeks. Her vitals were stable. Pelvic ultrasound revealed empty uterus and live fetus at the left adnexa corresponding to 14 weeks 6 days with minimal free fluid in the Douglas Cul-de-sac. The patient's final diagnosis was live extra-uterine pregnancy at 14 weeks 6 days that was managed by emergency laparotomy with salpingectomy. The patient recovered completely after surgery and was discharged in a stable condition. Ectopic pregnancy still remains one of the major causes of maternal morbidity and mortality. Early diagnosis and referral in hemodynamically state along with use of minimal access surgery or management can change the scenario of ectopic pregnancy in the developing world. Late attendance to first visit clinics is still a major concern in low resource limited settings as this could have been picked early and intervened. Management of broad ligament ectopic pregnancy in the second trimester is still challenging especially in low resource settings where the clients do not attend clinics and because of unavailability of ultrasound machines to diagnose it.
PubMed: 38404442
DOI: 10.1093/jscr/rjae084 -
Cell Reports. Medicine Mar 2024Transmasculine people usually reach amenorrhea within 6 months of adequate testosterone treatment. It is often assumed that no ovulation occurs during amenorrhea....
Transmasculine people usually reach amenorrhea within 6 months of adequate testosterone treatment. It is often assumed that no ovulation occurs during amenorrhea. However, in this study, we report recent ovulatory activity in amenorrheic transmasculine people on testosterone therapy at gender-affirming oophorectomy. Histological signs of recent ovulatory activity, including the presence of ovulatory follicles, corpus luteum, and corpus albicans, are observed in 17 of 52 individuals (33%). This is not significantly correlated to the duration, testosterone serum levels, or type of testosterone used. These results suggest that amenorrhea does not equal anovulation in transmasculine people on adequate testosterone therapy, emphasizing the importance of contraception for people who engage in sexual activity that can result in pregnancy.
Topics: Pregnancy; Female; Humans; Testosterone; Amenorrhea; Ovulation
PubMed: 38402622
DOI: 10.1016/j.xcrm.2024.101440