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23. Combined glue embolization and surgical excision for management of pediatric and adolescent patients assigned female at birth with genitourinary and perineal vascular anomalies
IF 1.7 4区 医学 Q3 OBSTETRICS & GYNECOLOGY Pub Date : 2025-02-28 DOI: 10.1016/j.jpag.2025.01.056
Zara Khan , Anna Ujvary , Maya Younoszai , Giri Shivaram , Katherine Debiec

Background

Vascular anomalies (VA) encompass a broad range of tumors and malformations of the capillaries, arteries, veins or lymphatics. Often diagnosed in birth to childhood, VA affect around 5% of children, of whom an estimated 2-2.6% have genitourinary and perineal (GUP) involvement, which may contribute to pain, debility, and aesthetic concerns. A recent innovation in surgical management for VA is n-BCA glue embolization followed by surgical excision. This study reviews the technical success and clinical outcomes of this combined surgical approach for management of GUP-VA.

Case

For this IRB-approved retrospective chart review, patients assigned female at birth with GUP-VA who underwent glue embolization and surgical excision were identified from a large database of patients with VA who presented for care to a tertiary-care children's hospital from 1998-2024. Demographics, operative procedure and complications, and prior and subsequent treatment history were reviewed. Five female patients with GUP-VA underwent glue embolization and excision (mean age 13 years, range 3-22) between 2014-2023. Four patients presented with congenital vulvar arteriovenous or venous malformations, while one patient developed a perineal body venous malformation secondary to saddle injury. Indications for treatment included swelling (100%), pain (40%) and bleeding (20%). Two patients had undergone prior therapies, including sclerotherapy (n=1) and prior surgical excision with eventual symptom recurrence (n=1). Embolization was performed with 1:3 or 1:4 n-BCA glue in ethiodized oil. Mean lesion size was 2.9 × 2.1 × 1.4 cm. Three patients underwent excision immediately post-embolization, while two patients underwent excision the following day. The procedure was technically successful in 100% of patients: symptomatic improvement was achieved in all patients, and none experienced VA recurrence. There was one instance of nontarget glue thromboembolism to a segmental pulmonary artery; suction thrombectomy removed the majority of the glue thrombus without any change in patient's oxygenation status and no large filling defects on pulmonary arteriography. Excision procedures were uncomplicated; mean and median surgical blood loss volumes were 46 and 50 mL. One patient experienced wound separation and was monitored for wound healing by secondary intention. Mean and median follow-up duration were six and three months (range 1-14 months).

Comments

Combined glue embolization and excision is a safe and effective treatment option for pediatric and adolescent patients presenting with GUP-VA.
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引用次数: 0
12. Needle-less Local Anesthetic Device for Subcutaneous Etonogestrel Insertion
IF 1.7 4区 医学 Q3 OBSTETRICS & GYNECOLOGY Pub Date : 2025-02-28 DOI: 10.1016/j.jpag.2025.01.024
Amitha Ganti , Megan Sumida , Olivia Winfrey , Jennifer Dietrich

Background

The subcutaneous etonogestrel implant is a first-line option for long-acting reversible contraceptive in adolescents but is often avoided due to fear of procedural pain. This early randomized control trial aimed to evaluate how a novel, needle-less device (J-Tip TM) for local anesthesia affected pain perception and overall patient satisfaction during implant insertion.

Methods

This IRB approved study recruited adolescents 13-21 years of age interested in the etonogestrel implant. Participants were randomized to receive intradermal lidocaine via the needle-less device or the traditional needle-based approach (control group). They were asked to complete a modified Amsterdam Preoperative Anxiety and Information Scale before and after the procedure and to report their pain scores via a 10-point Visual Analogue Scale throughout the procedure. Demographic and basic medical information were collected. Statistical analysis included student t-tests, correlation analysis, and effect size analysis with Cohen's d.

Results

Twenty-three participants were consented (11 controls and 12 interventions). Both groups had similar demographics and pre-procedure anxiety levels. Most were 16 years of age and denied having pre-existing mood disorders. Pain scores during lidocaine injection were significantly higher in the control group compared to the intervention group (mean = 5.8 ± 2.0 vs 0.8 ± 1.3, p< 0.001, range = 3-9 vs 0-3, d = 2.95). Pain scores during Nexplanon insertion were significantly lower in the control group compared to the intervention group (mean= 0.8 ± 0.9 vs 4.1 ± 2.5, p < 0.001, range = 0-2 vs range = 1-8, d = -1.81). In the post-procedure survey, recalled pain during insertion and overall satisfaction with the procedure among patients, parents and providers were not significantly different. Pre-procedure anxiety and expecting the procedure to be uncomfortable were positively correlated with increased stress during the procedure (r = 0.6, r=0.45) but not significantly correlated with any pain scores during the procedure.

Conclusions

While the new device may effectively reduce pain during administration of local anesthesia, its efficacy compared to traditional needle-based injection methods prior to implant insertion warrants further investigation. Our findings also emphasize the importance of managing patient expectations regarding comfort and pain, which may enhance overall satisfaction and reduce perceived pain and stress during the procedure. This study serves as a pilot to inform a larger randomized controlled trial aimed at improving the patient experience during etonogestrel implant insertions.
{"title":"12. Needle-less Local Anesthetic Device for Subcutaneous Etonogestrel Insertion","authors":"Amitha Ganti ,&nbsp;Megan Sumida ,&nbsp;Olivia Winfrey ,&nbsp;Jennifer Dietrich","doi":"10.1016/j.jpag.2025.01.024","DOIUrl":"10.1016/j.jpag.2025.01.024","url":null,"abstract":"<div><h3>Background</h3><div>The subcutaneous etonogestrel implant is a first-line option for long-acting reversible contraceptive in adolescents but is often avoided due to fear of procedural pain. This early randomized control trial aimed to evaluate how a novel, needle-less device (J-Tip TM) for local anesthesia affected pain perception and overall patient satisfaction during implant insertion.</div></div><div><h3>Methods</h3><div>This IRB approved study recruited adolescents 13-21 years of age interested in the etonogestrel implant. Participants were randomized to receive intradermal lidocaine via the needle-less device or the traditional needle-based approach (control group). They were asked to complete a modified Amsterdam Preoperative Anxiety and Information Scale before and after the procedure and to report their pain scores via a 10-point Visual Analogue Scale throughout the procedure. Demographic and basic medical information were collected. Statistical analysis included student t-tests, correlation analysis, and effect size analysis with Cohen's d.</div></div><div><h3>Results</h3><div>Twenty-three participants were consented (11 controls and 12 interventions). Both groups had similar demographics and pre-procedure anxiety levels. Most were 16 years of age and denied having pre-existing mood disorders. Pain scores during lidocaine injection were significantly higher in the control group compared to the intervention group (mean = 5.8 ± 2.0 vs 0.8 ± 1.3, p&lt; 0.001, range = 3-9 vs 0-3, d = 2.95). Pain scores during Nexplanon insertion were significantly lower in the control group compared to the intervention group (mean= 0.8 ± 0.9 vs 4.1 ± 2.5, p &lt; 0.001, range = 0-2 vs range = 1-8, d = -1.81). In the post-procedure survey, recalled pain during insertion and overall satisfaction with the procedure among patients, parents and providers were not significantly different. Pre-procedure anxiety and expecting the procedure to be uncomfortable were positively correlated with increased stress during the procedure (r = 0.6, r=0.45) but not significantly correlated with any pain scores during the procedure.</div></div><div><h3>Conclusions</h3><div>While the new device may effectively reduce pain during administration of local anesthesia, its efficacy compared to traditional needle-based injection methods prior to implant insertion warrants further investigation. Our findings also emphasize the importance of managing patient expectations regarding comfort and pain, which may enhance overall satisfaction and reduce perceived pain and stress during the procedure. This study serves as a pilot to inform a larger randomized controlled trial aimed at improving the patient experience during etonogestrel implant insertions.</div></div>","PeriodicalId":16708,"journal":{"name":"Journal of pediatric and adolescent gynecology","volume":"38 2","pages":"Page 227"},"PeriodicalIF":1.7,"publicationDate":"2025-02-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143520019","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
56. Informing Educational Initiatives: Sources and Satisfaction of Menstrual Education Among Young Women in a Multi-State Study
IF 1.7 4区 医学 Q3 OBSTETRICS & GYNECOLOGY Pub Date : 2025-02-28 DOI: 10.1016/j.jpag.2025.01.089
Jacquelyn Golden, Ana tomlinson, Garrett Forman, Lisa Gwynn

Background

Period poverty (inadequate access to resources and knowledge regarding menstruation management) has been observed in college students, despite their access to higher education. This study aims to explore experiences seeking guidance for menstrual problems and education preferences, while investigating correlations between guidance sources and satisfaction. The findings may inform future menstrual health education initiatives for young women.

Methods

A Qualtrics survey was distributed via snowball sampling to females enrolled in U.S. undergraduate programs. Information collected included demographics, preferences on menstrual education, and experiences with seeking help with menstrual problems. Chi-square goodness of fit and independence tests were performed using SPSS v29.

Results

Results from 102 participants across 9 states were analyzed. The majority were White (91.2%) and Non-Hispanic (89.2%), with ages ranging from 18 to 28 (average 19.6, SD±1.62). Of the respondents, 76.5% graduated from public high school, 19.6% from private high school, 2.9% foreign high school, and 0.9% trade school. The significantly preferred setting for menstrual education was doctors’ offices (25.48%, p< 0.001), followed by online (18.25%), home (14.83%), and social media (14.83%). School was significantly least preferred, with small groups (14.07%, p=0.025) favored over auditoriums (12.55%, p=0.002). Top topics of interest for learning about in school included when to see a doctor for menstrual health issues (15.33%, p< 0.001) and signs of common menstrual problems (14.18%, p=0.010). A preference for learning about free or discounted period products in small groups approached significance (p=0.059). Satisfaction levels varied by source of help. Higher satisfaction was reported by those consulting mothers (p< 0.001) or healthcare providers (p=0.02). Interestingly, despite a slight preference for online learning, satisfaction decreased for those seeking help online (p=0.014), and women were dissatisfied if they did not seek help at all (p< 0.001).

Conclusions

To address period poverty among college students, it is crucial to prioritize accessible education from trusted sources, particularly healthcare providers and family, while fostering supportive environments in both academic and home settings. Key initiatives in schools should focus on recognizing common menstrual issues and knowing when to seek medical help. Additionally, information about free or discounted menstrual products should be provided in small groups.
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引用次数: 0
50. How Well are we Writing to Patients? A Review of Clinical Correspondence from a Tertiary Paediatric and Adolescent Gynaecology Centre
IF 1.7 4区 医学 Q3 OBSTETRICS & GYNECOLOGY Pub Date : 2025-02-28 DOI: 10.1016/j.jpag.2025.01.083
Charlotte Porter-Hope , Ker Yi Wong , Hazel Isabella Learner

Background

It's recognised that patient engagement with healthcare is improved when letters are addressed and written directly to them. Adolescents and Young adults have different communication requirements; providing clinical communication with accessible language is thought to improve patient outcomes. Since 2018, clinicians in the UK have been advised to address all clinic letters directly to patients – this audit aimed to identify compliance with this national guidance.

Methods

A retrospective audit of outpatient clinic letters written to patients in January 2024, from a tertiary Paediatric and Adolescent gynaecology service, was undertaken. The clinical concern, patient age, learning needs, and who the letter was addressed to was recorded. For letters written to the patient, there was a subjective review of readability with assessment for presence of un-explained medical jargon and caculation of Flesch Reading Ease scores.

Results

There were 125 clinic consultations reviewed. The median patient age was 17 years old (2-51years). Clinical context varied and included 53/125 appointments about menstrual dysfunction, 27/125 mullerian anomalies, 20/125 Differences of Sex Development conditions and 10/125 vulval issues. The majority of letters were addressed directly to the patient (100/125 80%). The rest (25/125 20%) were addressed to the referring doctor. In 9/25 this was appropriate given a child's young age or a young person with learning needs. In 10/25 consultation notes were sent out as clinical correspondence, 5 consultations did not have a clinic letter for the appointment, and one letter was written directly to family doctor requesting referral to a service more local to the patient. Most letters written to patients were analysed subjectively to have recipient appropriate language (92%). Unexplained jargon included in letters included: contraindicated, hirsutism, malignant and laparoscopy. Flesch Reading Ease score was on average 51.5, which corresponds to being fairly difficult to read.

Conclusions

Generally, clinic letters were found to have good adherence to national guidance and addressed the patient. Although largely correspondence was written to patients was jargon free, objective assessment of Flesch Reading Ease score suggests that letters readability could be improved.
{"title":"50. How Well are we Writing to Patients? A Review of Clinical Correspondence from a Tertiary Paediatric and Adolescent Gynaecology Centre","authors":"Charlotte Porter-Hope ,&nbsp;Ker Yi Wong ,&nbsp;Hazel Isabella Learner","doi":"10.1016/j.jpag.2025.01.083","DOIUrl":"10.1016/j.jpag.2025.01.083","url":null,"abstract":"<div><h3>Background</h3><div>It's recognised that patient engagement with healthcare is improved when letters are addressed and written directly to them. Adolescents and Young adults have different communication requirements; providing clinical communication with accessible language is thought to improve patient outcomes. Since 2018, clinicians in the UK have been advised to address all clinic letters directly to patients – this audit aimed to identify compliance with this national guidance.</div></div><div><h3>Methods</h3><div>A retrospective audit of outpatient clinic letters written to patients in January 2024, from a tertiary Paediatric and Adolescent gynaecology service, was undertaken. The clinical concern, patient age, learning needs, and who the letter was addressed to was recorded. For letters written to the patient, there was a subjective review of readability with assessment for presence of un-explained medical jargon and caculation of Flesch Reading Ease scores.</div></div><div><h3>Results</h3><div>There were 125 clinic consultations reviewed. The median patient age was 17 years old (2-51years). Clinical context varied and included 53/125 appointments about menstrual dysfunction, 27/125 mullerian anomalies, 20/125 Differences of Sex Development conditions and 10/125 vulval issues. The majority of letters were addressed directly to the patient (100/125 80%). The rest (25/125 20%) were addressed to the referring doctor. In 9/25 this was appropriate given a child's young age or a young person with learning needs. In 10/25 consultation notes were sent out as clinical correspondence, 5 consultations did not have a clinic letter for the appointment, and one letter was written directly to family doctor requesting referral to a service more local to the patient. Most letters written to patients were analysed subjectively to have recipient appropriate language (92%). Unexplained jargon included in letters included: contraindicated, hirsutism, malignant and laparoscopy. Flesch Reading Ease score was on average 51.5, which corresponds to being fairly difficult to read.</div></div><div><h3>Conclusions</h3><div>Generally, clinic letters were found to have good adherence to national guidance and addressed the patient. Although largely correspondence was written to patients was jargon free, objective assessment of Flesch Reading Ease score suggests that letters readability could be improved.</div></div>","PeriodicalId":16708,"journal":{"name":"Journal of pediatric and adolescent gynecology","volume":"38 2","pages":"Page 253"},"PeriodicalIF":1.7,"publicationDate":"2025-02-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143520219","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
32. Endometrial hyperplasia in an adolescent with secondary amenorrhoea
IF 1.7 4区 医学 Q3 OBSTETRICS & GYNECOLOGY Pub Date : 2025-02-28 DOI: 10.1016/j.jpag.2025.01.065
Brooke Vandermolen , Sophie Clarke , Ephia Yasmin , Hazel Isabella Learner

Background

Endometrial cancer is the most common gynaecological malignancy globally. The incidence is increasing, particularly in younger women with obesity but has the potential to be missed in adolescents as the presenting features, such as abnormal menstrual bleeding, are common. This case describes an adolescent who was found to have endometrial hyperplasia and explores the risk factors and management of this condition.

Case

An 18-year-old female was referred to adolescent gynaecology with a 4 year history of chronic pelvic pain. A 9 month history of secondary amenorrhea with raised body mass index (BMI) (BMI 53kg/m2) was noted. Previous spinal MRI imaging showed suggestion of a thickened endometrial stripe [Fig1] so repeat pelvic imaging was arranged which reported endometrial thickening (19mm). Hysteroscopy and endometrial biopsy confirmed endometrial hyperplasia without atypia, and a Mirena Intrauterine System (IUS) was placed in the endometrial cavity. Repeat hysteroscopy and endometrial biopsy 7 months later showed resolution of endometrial hyperplasia but subsequent hysteroscopy and endometrial biopsy at 12 months again found endometrial hyperplasia without atypia. [Fig2]. At 15 months repeat hysteroscopy showed a more typical appearing cavity with thin endometrim, endometrial biopsy with only yielded a scanty sample but with no atypia reported. A repeat hysteroscopy and biopsy in another 6 months is planned and they are currently awaiting bariatric surgery.

Comments

This case report describes a rare case of confirmed endometrial hyperplasia without atypia in an adolescent. Given its premalignant potential, endometrial hyperplasia is an important diagnosis to consider in adolescents, particularly in those presenting with risk factors such as raised BMI. Ultrasound scan whilst not diagnostic for polycystic ovarian syndrome in adolescence still has an important role in considering differential diagnoses and for endometrial assessment. Ultrasonographic endometrial assessment enables risk-stratifying for adolescents who would benefit from endometrial biopsy to identify endometrial hyperplasia.
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引用次数: 0
5. A rare case of 45X/46XY mosaic Turner syndrome in a neonate
IF 1.7 4区 医学 Q3 OBSTETRICS & GYNECOLOGY Pub Date : 2025-02-28 DOI: 10.1016/j.jpag.2025.01.038
Akanksha Garg, Adam Hittelman, Christina Lepore, Jasmine Gujral, Nada Derar, Alla Vash-Margita

Background

Ambiguous genitalia in a newborn can be challenging to diagnose and manage. We present a case of a 2-month-old infant with atypical genitalia and genetic (45X/46XY) mosaicism.

Case

A healthy infant, delivered after an uncomplicated pregnancy at 39 weeks’ gestation was observed to have ambiguous genitalia. Prenatal cell-free DNA showed 40% risk for Turner's syndrome. A physical exam demonstrated an enlarged clitoris and fusion of the labioscrotal fold with a palpable mass in the left inguinal canal. Pelvic ultrasound showed a left inguinal testicle, no obvious ovarian tissue, and the presence of rudimentary Mullerian structures. Serum hormonal evaluation at 6 weeks of life included a testosterone of 118.3, ng/dL, inhibin B of 139 pg/ml, AMH of 32 ng/ml, and dihydrotestosterone level of 30 ng/dL suggesting testicular function . Estradiol levels were 3 pg/mL. Cytogenetics revealed 45X/46XY mosaicism with an SRY gene. Genitogram demonstrated a urogenital sinus. Concern was raised for potential dysgenetic gonads, which carry a risk for malignancy and a shared decision was made to pursue an exam under anesthesia and diagnostic laparoscopy to assess gonads and mullerian structures. Surgical findings included a short urogenital sinus and a 1 cm vaginal canal. A normal-appearing single cervix was visualized, and the left testicle was identified in the inguinal canal. A right-sided streak gonad (Figure 1A, black arrow), fallopian tube (Figure 1B, green arrow) and small right hemi-uterus (Figure 1D, Pink arrow) were visualized. A left inguinal orchiopexy, left inguinal hernia repair (Figure 1C) and right gonadectomy and salpingectomy (Figure 1) was performed. Pathology showed benign ovarian stroma, with rete-testes like structures. We describe one of the few cases of a neonatal diagnosis of Mosiac Turner's syndrome with the presence of both testicular and Mullerian tissue. The diagnostic challenge of such cases is that imaging modalities are limited, and surgical exploration is often necessary for definitive phenotype analysis. Furthermore, it is important to counsel parents on the potential outcomes and encourage shared decision-making to consider future options for the child's sex of rearing whilst reducing the risk of possible malignancies.

Comments

-We highlight a rare case of neonatal 45X/46XY Mosaic Turner's syndrome with surgical findings of a normal cervix, urogenital sinus, a left semi-descended testicle, right streak gonad and fallopian tube, and hemi-uterus -We highlight the role of shared decision-making when managing rare, complex presentations of mosaic Turner's, including reducing the risk of gonadoblastomas
{"title":"5. A rare case of 45X/46XY mosaic Turner syndrome in a neonate","authors":"Akanksha Garg,&nbsp;Adam Hittelman,&nbsp;Christina Lepore,&nbsp;Jasmine Gujral,&nbsp;Nada Derar,&nbsp;Alla Vash-Margita","doi":"10.1016/j.jpag.2025.01.038","DOIUrl":"10.1016/j.jpag.2025.01.038","url":null,"abstract":"<div><h3>Background</h3><div>Ambiguous genitalia in a newborn can be challenging to diagnose and manage. We present a case of a 2-month-old infant with atypical genitalia and genetic (45X/46XY) mosaicism.</div></div><div><h3>Case</h3><div>A healthy infant, delivered after an uncomplicated pregnancy at 39 weeks’ gestation was observed to have ambiguous genitalia. Prenatal cell-free DNA showed 40% risk for Turner's syndrome. A physical exam demonstrated an enlarged clitoris and fusion of the labioscrotal fold with a palpable mass in the left inguinal canal. Pelvic ultrasound showed a left inguinal testicle, no obvious ovarian tissue, and the presence of rudimentary Mullerian structures. Serum hormonal evaluation at 6 weeks of life included a testosterone of 118.3, ng/dL, inhibin B of 139 pg/ml, AMH of 32 ng/ml, and dihydrotestosterone level of 30 ng/dL suggesting testicular function . Estradiol levels were 3 pg/mL. Cytogenetics revealed 45X/46XY mosaicism with an SRY gene. Genitogram demonstrated a urogenital sinus. Concern was raised for potential dysgenetic gonads, which carry a risk for malignancy and a shared decision was made to pursue an exam under anesthesia and diagnostic laparoscopy to assess gonads and mullerian structures. Surgical findings included a short urogenital sinus and a 1 cm vaginal canal. A normal-appearing single cervix was visualized, and the left testicle was identified in the inguinal canal. A right-sided streak gonad (Figure 1A, black arrow), fallopian tube (Figure 1B, green arrow) and small right hemi-uterus (Figure 1D, Pink arrow) were visualized. A left inguinal orchiopexy, left inguinal hernia repair (Figure 1C) and right gonadectomy and salpingectomy (Figure 1) was performed. Pathology showed benign ovarian stroma, with rete-testes like structures. We describe one of the few cases of a neonatal diagnosis of Mosiac Turner's syndrome with the presence of both testicular and Mullerian tissue. The diagnostic challenge of such cases is that imaging modalities are limited, and surgical exploration is often necessary for definitive phenotype analysis. Furthermore, it is important to counsel parents on the potential outcomes and encourage shared decision-making to consider future options for the child's sex of rearing whilst reducing the risk of possible malignancies.</div></div><div><h3>Comments</h3><div>-We highlight a rare case of neonatal 45X/46XY Mosaic Turner's syndrome with surgical findings of a normal cervix, urogenital sinus, a left semi-descended testicle, right streak gonad and fallopian tube, and hemi-uterus -We highlight the role of shared decision-making when managing rare, complex presentations of mosaic Turner's, including reducing the risk of gonadoblastomas</div></div>","PeriodicalId":16708,"journal":{"name":"Journal of pediatric and adolescent gynecology","volume":"38 2","pages":"Page 232"},"PeriodicalIF":1.7,"publicationDate":"2025-02-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143519929","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
20. Bleeding Profiles, Iron Deficiency, and Quality of Life in Two Cohorts of Adolescents and Young Adults with Type 1 Von Willebrand Disease and Joint Hypermobility/Ehlers-Danlos Syndrome
IF 1.7 4区 医学 Q3 OBSTETRICS & GYNECOLOGY Pub Date : 2025-02-28 DOI: 10.1016/j.jpag.2025.01.053
Anindita Varshneya , Maria Barrett , Marie-Teresa Colbert , Kendra Hutchens , Genevieve Moyer , Lauryn Roth , Patricia Huguelet

Background

Patients with Type 1 Von Willebrand Disease (VWD) experience heavy menstrual bleeding (HMB) and other bleeding symptoms that negatively impact their quality of life. Individuals with Generalized Joint Hypermobility (GJH) and hypermobile Ehlers-Danlos Syndrome (hEDS) also experience HMB and non-menstrual bleeding, likely due to abnormalities of collagen production. Limited research exists regarding the sequelae of HMB among these populations, including iron deficiency, and impact on school attendance and behavioral health. We aim to describe HMB, rates of iron deficiency, need for intravenous iron, and impact on school attendance and mood in adolescents and young adults with Type 1 VWD and GJH/hEDS.

Methods

We performed an IRB-approved, retrospective chart review of patients aged 10-25, presenting with HMB to a combined gynecology/hematology clinic at a tertiary children's hospital from 2019-2024. Cohort 1 included patients with Type 1 VWD. Cohort 2 included patients with GJH (Beighton score ≥ 4) or hEDS (confirmed by a geneticist). Extracted data included baseline demographics, bleeding assessment scores, baseline hemoglobin and ferritin levels, presence of depression or anxiety, and treatments prescribed. Descriptive data are summarized using mean and standard deviation, median and ranges.

Results

Cohort 1 (VWD) included 111 patients; Cohort 2 (GJH, hEDS) included 26 patients. Table 1 reports baseline demographics in both cohorts. Patients in both cohorts had high scores on baseline Bleeding Assessment Tool (BAT) and pictorial blood loss assessment chart (PBAC) (Table 2). Anxiety and depression were reported by 51% of individuals with VWD and 65% GJH/hEDS patients. Intravenous iron was needed for 54% VWD and 65% GJH/hEDS. Median total lines of treatment were 2 (VWD) and 3 (hEDS). Among patients who achieved bleeding improvement, defined by PBAC < 100, the LNG-IUD (alone or with supplemental therapy) was successful for 62% GJH/hEDS and 33% VWD.

Conclusions

Patients with GJH/hEDS and Type 1 VWD demonstrate similar bleeding profiles. These patients attempt several lines of therapy to control HMB. Patients with GHJ/hEDS demonstrate a trend towards higher rates of depression and anxiety and a greater need for intravenous iron. Our results suggest greater awareness and treatment guidelines are needed for patients with GJH/hEDS. Future analyses will compare the two cohorts and will assess the most effective treatment modalities.
{"title":"20. Bleeding Profiles, Iron Deficiency, and Quality of Life in Two Cohorts of Adolescents and Young Adults with Type 1 Von Willebrand Disease and Joint Hypermobility/Ehlers-Danlos Syndrome","authors":"Anindita Varshneya ,&nbsp;Maria Barrett ,&nbsp;Marie-Teresa Colbert ,&nbsp;Kendra Hutchens ,&nbsp;Genevieve Moyer ,&nbsp;Lauryn Roth ,&nbsp;Patricia Huguelet","doi":"10.1016/j.jpag.2025.01.053","DOIUrl":"10.1016/j.jpag.2025.01.053","url":null,"abstract":"<div><h3>Background</h3><div>Patients with Type 1 Von Willebrand Disease (VWD) experience heavy menstrual bleeding (HMB) and other bleeding symptoms that negatively impact their quality of life. Individuals with Generalized Joint Hypermobility (GJH) and hypermobile Ehlers-Danlos Syndrome (hEDS) also experience HMB and non-menstrual bleeding, likely due to abnormalities of collagen production. Limited research exists regarding the sequelae of HMB among these populations, including iron deficiency, and impact on school attendance and behavioral health. We aim to describe HMB, rates of iron deficiency, need for intravenous iron, and impact on school attendance and mood in adolescents and young adults with Type 1 VWD and GJH/hEDS.</div></div><div><h3>Methods</h3><div>We performed an IRB-approved, retrospective chart review of patients aged 10-25, presenting with HMB to a combined gynecology/hematology clinic at a tertiary children's hospital from 2019-2024. Cohort 1 included patients with Type 1 VWD. Cohort 2 included patients with GJH (Beighton score ≥ 4) or hEDS (confirmed by a geneticist). Extracted data included baseline demographics, bleeding assessment scores, baseline hemoglobin and ferritin levels, presence of depression or anxiety, and treatments prescribed. Descriptive data are summarized using mean and standard deviation, median and ranges.</div></div><div><h3>Results</h3><div>Cohort 1 (VWD) included 111 patients; Cohort 2 (GJH, hEDS) included 26 patients. Table 1 reports baseline demographics in both cohorts. Patients in both cohorts had high scores on baseline Bleeding Assessment Tool (BAT) and pictorial blood loss assessment chart (PBAC) (Table 2). Anxiety and depression were reported by 51% of individuals with VWD and 65% GJH/hEDS patients. Intravenous iron was needed for 54% VWD and 65% GJH/hEDS. Median total lines of treatment were 2 (VWD) and 3 (hEDS). Among patients who achieved bleeding improvement, defined by PBAC &lt; 100, the LNG-IUD (alone or with supplemental therapy) was successful for 62% GJH/hEDS and 33% VWD.</div></div><div><h3>Conclusions</h3><div>Patients with GJH/hEDS and Type 1 VWD demonstrate similar bleeding profiles. These patients attempt several lines of therapy to control HMB. Patients with GHJ/hEDS demonstrate a trend towards higher rates of depression and anxiety and a greater need for intravenous iron. Our results suggest greater awareness and treatment guidelines are needed for patients with GJH/hEDS. Future analyses will compare the two cohorts and will assess the most effective treatment modalities.</div></div>","PeriodicalId":16708,"journal":{"name":"Journal of pediatric and adolescent gynecology","volume":"38 2","pages":"Pages 239-240"},"PeriodicalIF":1.7,"publicationDate":"2025-02-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143519934","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
46. Genital differences with Congenital Adrenal Hyperplasia- adolescent choices. A retrospective review of new patients seen in a specialist multi-disciplinary service between 2018-2024.
IF 1.7 4区 医学 Q3 OBSTETRICS & GYNECOLOGY Pub Date : 2025-02-28 DOI: 10.1016/j.jpag.2025.01.079
Hazel Isabella Learner , Manju Netto , Sophie Clarke

Background

Congenital Adrenal Hyperplasia(CAH) is associated with genital differences including urogenital sinus anomalies and clitoral enlargement. Surgery in childhood is criticised as non-consensual genital surgery associated with risks and without functional indication in children. In adolescent and adult services surgery can be considered in mature individuals. There is limited data on the decisions of patients in these services considering genitoplasty. This project aimed to describe the decisions of patients with CAH referred to a specialist multi-disciplinary service for genital differences.

Methods

A 6-year retrospective review of new adolescent patients(aged 10-25) referred with genital differences associated with CAH to a specialist multi-disciplinary clinic with gynaecology, psychology, clinical nurse specialist and endocrinology. Data was extracted from electronic patient records on demographics, medical history and clinic outcomes. The data was analysed on Excel.

Results

Fifty-eight patients were identified with a median age at first appointment of 17years (10-25years). There was no issue with menstrual obstruction or recurrent urinary tract infections in any patients. The majority (34/58 58%) of patients had undergone genital surgery in infancy: 24/58 vaginal and clitoral surgery, 6/58 vaginoplasty, 2/58 clitoral reduction. Of these, three had had repeat procedures in childhood on either their vagina (2/3) or clitoris (1/3). There were 8/34 who were unaware, and 8/34 unsure,of what genital surgery they had previously had. One third of patients (23/58 39%) did not want to proceed with review of their genital differences preferring to defer the option of examination. 8/23 of these young people had not previously undergone surgery (with 1/8 late onset). Of those wanting to explore genital differences, 22/35 were planned for examination under anaesthesia (11/22 also having psychology input to support them considering clitoral concerns and options). There were 7/35 patients with clitoral issues and raised testosterone were planned for psychology and endocrinology input ahead of further gynaecology review. Six (6/635) patients following examination in clinic were offered vaginal dilation therapy, 4/35 felt ready to start this.

Conclusions

Genital differences with CAH, can be explored with the support of a specialised MDT service, at a time that is led by the individual affected.
{"title":"46. Genital differences with Congenital Adrenal Hyperplasia- adolescent choices. A retrospective review of new patients seen in a specialist multi-disciplinary service between 2018-2024.","authors":"Hazel Isabella Learner ,&nbsp;Manju Netto ,&nbsp;Sophie Clarke","doi":"10.1016/j.jpag.2025.01.079","DOIUrl":"10.1016/j.jpag.2025.01.079","url":null,"abstract":"<div><h3>Background</h3><div>Congenital Adrenal Hyperplasia(CAH) is associated with genital differences including urogenital sinus anomalies and clitoral enlargement. Surgery in childhood is criticised as non-consensual genital surgery associated with risks and without functional indication in children. In adolescent and adult services surgery can be considered in mature individuals. There is limited data on the decisions of patients in these services considering genitoplasty. This project aimed to describe the decisions of patients with CAH referred to a specialist multi-disciplinary service for genital differences.</div></div><div><h3>Methods</h3><div>A 6-year retrospective review of new adolescent patients(aged 10-25) referred with genital differences associated with CAH to a specialist multi-disciplinary clinic with gynaecology, psychology, clinical nurse specialist and endocrinology. Data was extracted from electronic patient records on demographics, medical history and clinic outcomes. The data was analysed on Excel.</div></div><div><h3>Results</h3><div>Fifty-eight patients were identified with a median age at first appointment of 17years (10-25years). There was no issue with menstrual obstruction or recurrent urinary tract infections in any patients. The majority (34/58 58%) of patients had undergone genital surgery in infancy: 24/58 vaginal and clitoral surgery, 6/58 vaginoplasty, 2/58 clitoral reduction. Of these, three had had repeat procedures in childhood on either their vagina (2/3) or clitoris (1/3). There were 8/34 who were unaware, and 8/34 unsure,of what genital surgery they had previously had. One third of patients (23/58 39%) did not want to proceed with review of their genital differences preferring to defer the option of examination. 8/23 of these young people had not previously undergone surgery (with 1/8 late onset). Of those wanting to explore genital differences, 22/35 were planned for examination under anaesthesia (11/22 also having psychology input to support them considering clitoral concerns and options). There were 7/35 patients with clitoral issues and raised testosterone were planned for psychology and endocrinology input ahead of further gynaecology review. Six (6/635) patients following examination in clinic were offered vaginal dilation therapy, 4/35 felt ready to start this.</div></div><div><h3>Conclusions</h3><div>Genital differences with CAH, can be explored with the support of a specialised MDT service, at a time that is led by the individual affected.</div></div>","PeriodicalId":16708,"journal":{"name":"Journal of pediatric and adolescent gynecology","volume":"38 2","pages":"Page 250"},"PeriodicalIF":1.7,"publicationDate":"2025-02-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143520215","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
49. Hormonal contraceptive considerations in the setting of venous thromboembolism and absent inferior vena cava: A case report
IF 1.7 4区 医学 Q3 OBSTETRICS & GYNECOLOGY Pub Date : 2025-02-28 DOI: 10.1016/j.jpag.2025.01.082
Elizabeth Baldor , Madeline Ross , Emily Orscheln , Tazim Dowlut-McElroy

Background

Venous thromboembolism (VTE) risk among users of contraception varies based on the hormonal composition of each method. Controversy exists regarding the use of hormonal contraception in those with a history of thrombosis or thrombophilia and require anticoagulation therapy.

Case

A 17 y.o. girl presented with persistent pain and swelling in both of her legs. Ultrasound confirmed bilateral femoral vein thromboses; CT revealed absence of the inferior vena cava (AIVC) with extensive thromboses through the iliac and femoral veins. She received therapeutic anticoagulation and mechanical thrombectomy. She reported a history of heavy menses and desired to initiate hormonal contraception for mitigation of heavy menstrual bleeding ultimately choosing the drospirenone 4mg contraceptive pill.

Comments

AIVC is a rare anomaly that affects < 1% of the population but commonly presents with lower extremity DVTs in young adults, requiring long-term or indefinite anticoagulation due to high rates of recurrence. Anticoagulation use in menstruating individuals has been associated with increased rates of abnormal uterine bleeding. Yet, there is some reluctance amongst health care providers to prescribe hormonal contraception in these circumstances due to increased risk of VTE, particularly with estrogen-containing methods. 2024 guidelines stratify risk based on: 1) current or history of VTE receiving therapeutic dosing of anticoagulants, 2) history of VTE receiving prophylactic dosing of anticoagulants without or with higher risk of recurrent VTE, and 3) history of VTE not receiving prophylactic dosing of anticoagulants. Though combination hormonal contraceptives (CHCs) do not appear to be associated with increased risk of recurrent VTE in those receiving anticoagulation therapy, CHCs are considered to have unacceptable risk in those with a high risk of recurrent VTE. Vascular malformations such as AIVC result in venous stasis and an increased risk of VTE with CHC use. In contrast, the menstrual and contraceptive benefits of progestin-only pills (POPs), the etonorgestrel implant, and the 52-mg levonorgestrel-releasing IUD (LnGIUD) usually outweigh the risk of recurrent VTE. A POP was deemed the most favorable option when prioritizing management of heavy bleeding in our patient given the possibility of breakthrough bleeding after initiation of LnGIUD, and the risk of irregular menstrual bleeding with the etonogestrel implant. Our patient chose the drospirenone 4mg contraceptive pill which may provide a more favorable bleeding profile with significantly lower rates of unscheduled bleeding compared to traditional POPs.
{"title":"49. Hormonal contraceptive considerations in the setting of venous thromboembolism and absent inferior vena cava: A case report","authors":"Elizabeth Baldor ,&nbsp;Madeline Ross ,&nbsp;Emily Orscheln ,&nbsp;Tazim Dowlut-McElroy","doi":"10.1016/j.jpag.2025.01.082","DOIUrl":"10.1016/j.jpag.2025.01.082","url":null,"abstract":"<div><h3>Background</h3><div>Venous thromboembolism (VTE) risk among users of contraception varies based on the hormonal composition of each method. Controversy exists regarding the use of hormonal contraception in those with a history of thrombosis or thrombophilia and require anticoagulation therapy.</div></div><div><h3>Case</h3><div>A 17 y.o. girl presented with persistent pain and swelling in both of her legs. Ultrasound confirmed bilateral femoral vein thromboses; CT revealed absence of the inferior vena cava (AIVC) with extensive thromboses through the iliac and femoral veins. She received therapeutic anticoagulation and mechanical thrombectomy. She reported a history of heavy menses and desired to initiate hormonal contraception for mitigation of heavy menstrual bleeding ultimately choosing the drospirenone 4mg contraceptive pill.</div></div><div><h3>Comments</h3><div>AIVC is a rare anomaly that affects &lt; 1% of the population but commonly presents with lower extremity DVTs in young adults, requiring long-term or indefinite anticoagulation due to high rates of recurrence. Anticoagulation use in menstruating individuals has been associated with increased rates of abnormal uterine bleeding. Yet, there is some reluctance amongst health care providers to prescribe hormonal contraception in these circumstances due to increased risk of VTE, particularly with estrogen-containing methods. 2024 guidelines stratify risk based on: 1) current or history of VTE receiving therapeutic dosing of anticoagulants, 2) history of VTE receiving prophylactic dosing of anticoagulants without or with higher risk of recurrent VTE, and 3) history of VTE not receiving prophylactic dosing of anticoagulants. Though combination hormonal contraceptives (CHCs) do not appear to be associated with increased risk of recurrent VTE in those receiving anticoagulation therapy, CHCs are considered to have unacceptable risk in those with a high risk of recurrent VTE. Vascular malformations such as AIVC result in venous stasis and an increased risk of VTE with CHC use. In contrast, the menstrual and contraceptive benefits of progestin-only pills (POPs), the etonorgestrel implant, and the 52-mg levonorgestrel-releasing IUD (LnGIUD) usually outweigh the risk of recurrent VTE. A POP was deemed the most favorable option when prioritizing management of heavy bleeding in our patient given the possibility of breakthrough bleeding after initiation of LnGIUD, and the risk of irregular menstrual bleeding with the etonogestrel implant. Our patient chose the drospirenone 4mg contraceptive pill which may provide a more favorable bleeding profile with significantly lower rates of unscheduled bleeding compared to traditional POPs.</div></div>","PeriodicalId":16708,"journal":{"name":"Journal of pediatric and adolescent gynecology","volume":"38 2","pages":"Pages 252-253"},"PeriodicalIF":1.7,"publicationDate":"2025-02-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143520218","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
18. Bilateral Borderline Ovarian Tumors in a 15-Year-Old: A Rare Pediatric Case
IF 1.7 4区 医学 Q3 OBSTETRICS & GYNECOLOGY Pub Date : 2025-02-28 DOI: 10.1016/j.jpag.2025.01.051
Jennifer Silk, Katelyn Day, Laura Stafman

Background

Ovarian malignancy in pediatrics, with germ cell tumors being most common, is reported in 3–8% of patients. Borderline tumors, a type of tumor with low malignant potential showing cytologic traits of malignancy but lacking clear invasive growth, occur at even lower rates with no consistent statistical data reported. Pediatric bilateral borderline ovarian tumors (BOTs) have not been well described in the literature beyond case reports.

Case

A 15-year-old female presented to her pediatrician for a routine annual exam which revealed abdominal distention on physical exam. This prompted an abdominal x-ray and CT abdomen/pelvis which identified a 23 × 17.5 × 10.3cm cystic mass stemming from the left ovary. She initially did not report any complaints, but upon reflection, she stated that she felt distended one month prior and reported a ten-pound weight gain, fatigue, urinary frequency, light-headedness, and discomfort when stooling over the last two months. She underwent menarche at age 12 and denied any significant past medical or surgical history. She was referred to pediatric surgery for further evaluation. Tumor markers were significant for a slightly elevated CA125 (87) and inhibin-A just above normal limits (106). AFP, bHCG, LDH, and inhibin B were within normal limits. Her pelvic ultrasound noted a normal uterus, no free fluid, and a large multicystic and solid mass arising from the pelvis to the level of the liver. Due to the size of the mass, her ovaries were difficult to specifically delineate. The patient subsequently underwent an exploratory laparotomy that unexpectedly revealed masses on both ovaries, with the right mass appearing benign in the operating room and the left ovarian mass with concerning solid components and increased vascularity. Pelvic washings were obtained followed by ovarian-sparing excision of bilateral ovarian masses. Pathologic specimens were reviewed at the primary institution and sent for secondary review at an institution that specializes in pediatric ovarian pathology. The patient's pelvic washings were negative. The final diagnoses of the left (208.2g 18.5 × 14.8 × 3.2cm) and right (365 g 9.6 × 9.0 × 7.2cm) ovarian masses were proliferative serous BOTs.

Comments

This is an example of an extremely rare clinical scenario of bilateral serous BOTs in a pediatric patient. The case demonstrates the importance of an extensive preoperative workup for an abdominopelvic mass including imaging and laboratory assessment, collaboration of teams in the operating room and postoperatively, removal of masses without spillage in the abdominal cavity, and the significance of fertility preservation with ovarian-sparing mass excisions.
{"title":"18. Bilateral Borderline Ovarian Tumors in a 15-Year-Old: A Rare Pediatric Case","authors":"Jennifer Silk,&nbsp;Katelyn Day,&nbsp;Laura Stafman","doi":"10.1016/j.jpag.2025.01.051","DOIUrl":"10.1016/j.jpag.2025.01.051","url":null,"abstract":"<div><h3>Background</h3><div>Ovarian malignancy in pediatrics, with germ cell tumors being most common, is reported in 3–8% of patients. Borderline tumors, a type of tumor with low malignant potential showing cytologic traits of malignancy but lacking clear invasive growth, occur at even lower rates with no consistent statistical data reported. Pediatric bilateral borderline ovarian tumors (BOTs) have not been well described in the literature beyond case reports.</div></div><div><h3>Case</h3><div>A 15-year-old female presented to her pediatrician for a routine annual exam which revealed abdominal distention on physical exam. This prompted an abdominal x-ray and CT abdomen/pelvis which identified a 23 × 17.5 × 10.3cm cystic mass stemming from the left ovary. She initially did not report any complaints, but upon reflection, she stated that she felt distended one month prior and reported a ten-pound weight gain, fatigue, urinary frequency, light-headedness, and discomfort when stooling over the last two months. She underwent menarche at age 12 and denied any significant past medical or surgical history. She was referred to pediatric surgery for further evaluation. Tumor markers were significant for a slightly elevated CA125 (87) and inhibin-A just above normal limits (106). AFP, bHCG, LDH, and inhibin B were within normal limits. Her pelvic ultrasound noted a normal uterus, no free fluid, and a large multicystic and solid mass arising from the pelvis to the level of the liver. Due to the size of the mass, her ovaries were difficult to specifically delineate. The patient subsequently underwent an exploratory laparotomy that unexpectedly revealed masses on both ovaries, with the right mass appearing benign in the operating room and the left ovarian mass with concerning solid components and increased vascularity. Pelvic washings were obtained followed by ovarian-sparing excision of bilateral ovarian masses. Pathologic specimens were reviewed at the primary institution and sent for secondary review at an institution that specializes in pediatric ovarian pathology. The patient's pelvic washings were negative. The final diagnoses of the left (208.2g 18.5 × 14.8 × 3.2cm) and right (365 g 9.6 × 9.0 × 7.2cm) ovarian masses were proliferative serous BOTs.</div></div><div><h3>Comments</h3><div>This is an example of an extremely rare clinical scenario of bilateral serous BOTs in a pediatric patient. The case demonstrates the importance of an extensive preoperative workup for an abdominopelvic mass including imaging and laboratory assessment, collaboration of teams in the operating room and postoperatively, removal of masses without spillage in the abdominal cavity, and the significance of fertility preservation with ovarian-sparing mass excisions.</div></div>","PeriodicalId":16708,"journal":{"name":"Journal of pediatric and adolescent gynecology","volume":"38 2","pages":"Page 238"},"PeriodicalIF":1.7,"publicationDate":"2025-02-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143520842","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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Journal of pediatric and adolescent gynecology
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