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Leaf-level gas exchange data support the mechanistic understanding of plant fluxes of carbon and water. These fluxes inform our understanding of ecosystem function, are an important constraint on parameterization of terrestrial biosphere models, are necessary to understand the response of plants to global environmental change, and are integral to efforts to improve crop production. Collection of these data using gas analyzers can be both technically challenging and time consuming, and individual studies generally focus on a small range of species, restricted time periods, or limited geographic regions. The high value of these data is exemplified by the many publications that reuse and synthesize gas exchange data, however the lack of metadata and data reporting conventions make full and efficient use of these data difficult. Here we propose a reporting format for leaf-level gas exchange data and metadata to provide guidance to data contributors on how to store data in repositories to maximize their discoverability, facilitate their efficient reuse, and add value to individual datasets. For data users, the reporting format will better allow data repositories to optimize data search and extraction, and more readily integrate similar data into harmonized synthesis products. The reporting format specifies data table variable naming and unit conventions, as well as metadata characterizing experimental conditions and protocols. For common data types that were the focus of this initial version of the reporting format, i.e., survey measurements, dark respiration, carbon dioxide and light response curves, and parameters derived from those measurements, we took a further step of defining required additional data and metadata that would maximize the potential reuse of those data types. To aid data contributors and the development of data ingest tools by data repositories we provided a translation table comparing the outputs of common gas exchange instruments. Extensive consultation with data collectors, data users, instrument manufacturers, and data scientists was undertaken in order to ensure that the reporting format met community needs. The reporting format presented here is intended to form a foundation for future development that will incorporate additional data types and variables as gas exchange systems and measurement approaches advance in the future. The reporting format is published in the U.S. Department of Energy's ESS-DIVE data repository, with documentation and future development efforts being maintained in a version control system.

Aim: To assess the awareness and knowledge levels about glaucoma and its determinants in an urban population of Chennai in south India.Materials and Methods: Chennai glaucoma study (CGS) was a population based prevalence study to estimate the prevalence of glaucoma in a rural and urban south Indian population. A total of 3850 subjects aged 40 years or above participated in the urban arm of CGS. A systematic random sample of 1926 (50.0%) subjects completed a questionnaire that assesses their awareness and knowledge level of glaucoma. Respondents "having heard of glaucoma" even before they were contacted/recruited for the study were defined as "aware" and respondents having some understanding of the eye disease were defined as "knowledgeable".Results: Overall 13.5% were aware of glaucoma, the age-gender adjusted rate for awareness was 13.3% (95% CI: 11.57 to 15.03). Two clinicians graded knowledge on glaucoma, based on the subject's knowledge of risk factors, definitions and treatment aspects of glaucoma. Overall 8.7% had some knowledge about glaucoma. Among those who had knowledge 0.5% had good knowledge about glaucoma, 4% had fair knowledge and 4.2% had poor knowledge. We observed a very good agreement between the clinicians in grading knowledge (k =0.92). Determinants of glaucoma awareness and knowledge were higher levels of education, females, age, religion and family history of glaucoma.Conclusion: Awareness and knowledge about glaucoma was very low among the urban population of Chennai. We have found that younger subjects and men were less aware of glaucoma. Subjects with lower levels of education were less aware and knew less about glaucoma than their counterparts. The study findings stress the need for health education for effective prevention of blindness due to glaucoma. [ABSTRACT FROM AUTHOR]

AbstractBackground: Stress reduction and comprehensive lifestyle modification programs have improved atherosclerosis and cardiac risk factors in earlier trials. Little is known about the impact of such programs on quality-of-life (QoL) and psychological outcomes. Given recent significant improvements in cardiac care, we evaluated the current benefit of stress reduction/lifestyle modification on QoL and emotional distress in patients with coronary artery disease (CAD). Methods: 101 patients (59.4 ± 8.6 years, 23 female) with CAD were randomized to a 1-year lifestyle/stress management program (n = 48) or written advice (n = 53). QoL and psychological outcomes were assessed with the SF-36, Beck Depression, Spielberger State/Trait Anxiety, Spielberger State/Trait Anger and Perceived Stress Inventories. Group repeated-measures analyses of variance were performed for all measures. Results: Adherence to the program was excellent (daily relaxation practice 39 ± 5vs. 5 ± 8 min, respectively; p < 0.001). Both groups improved comparably in most dimensions of QoL, and significantly greater improvements for the lifestyle group were found for physical function and physical sum score (p = 0.046 and p = 0.045). Depression, anxiety, anger and perceived stress were reduced similarly in both groups. However, intervention × gender interaction effects revealed greater benefits among women in the lifestyle intervention vs. advice group for depression and anger (p = 0.025 and p = 0.040), but no effects for men. Conclusions: A comprehensive lifestyle modification and stress management program did not improve psychological outcomes in medically stable CAD patients. The program did appear to confer psychological benefits for women but not men. Further trials should investigate gender-related differences in coronary patient responses to behavioral interventions.Copyright © 2005 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

AIM: To develop a customised, portable, cost-effective (logarithmic minimal angle resolution) LogMAR chart with adjustable illumination for use as a mass vision-screening device in the rural population. MATERIALS AND METHODS: Visual acuity of 100 individuals was evaluated with a customised chart and compared with the standard Early Treatment Diabetic Retinopathy Study (ETDRS) chart and Snellen's Chart. Bland and Altman analytical techniques were used for analysis. RESULTS: Test-retest variability of the customised chart was just a one-line difference (95% CI for agreement), and so were the results with the standard ETDRS charts; a variability of 3-line was noted with Snellen's chart. Two-line differences were observed when comparison was made with Standard ETDRS chart and 2 to 3-line differences with Snellen's chart. CONCLUSION: The customised portable LogMAR chart with adjustable illumination shows less test-retest variability and better agreement with standard ETDRS chart; therefore, it can be used as a mass vision-screening device in rural settings. [ABSTRACT FROM AUTHOR]

Objectives: To present cases of rapid onset obesity with hypothalamic dysfunction, hypoventilation, and autonomic dysregulation (ROHHAD) and discuss management insights., Methods: Case records of patients fulfilling the criteria for ROHHAD and presenting to the Pediatric Endocrinology Division of All India Institute of Medical Sciences, New Delhi, between July 2019 and June 2024 were reviewed for clinical features, treatments, and outcomes., Results: Five patients (4 boys, 1 girl) presented at a median age of 4 y (range 3.3-6) with rapidly progressive weight gain as the initial symptom. Autonomic dysregulation and hyperprolactinemia were present in all patients (100%). Sleep disturbances were noted in 4 patients (80%), respiratory support was required for 4 patients (80%), temperature instability in 3 (60%), dysnatremia in 3 (60%), pituitary hormone deficiencies in 3 (60%), behavioral issues in 2 (40%), and neuroectodermal tumor (NET) in 1 patient (20%). Treatment included IVIg for 3 patients with no improvement, home BiPAP support for 1 patient, and unilateral adrenalectomy for a ganglioneuroma in another. Four (80%) patients died during follow-up (mean age at death 5 y); 1 due to sudden cardiorespiratory arrest, and 3 from respiratory tract infections., Conclusions: ROHHAD management requires a multimodal approach, addressing pituitary hormone deficiencies, autonomic dysregulation, and screening for hypoventilation and NETs. Emphasis should be on early home respiratory support and monitoring for cardiac rhythm disturbances. Further studies are needed to evaluate the efficacy of immunomodulatory agents., Competing Interests: Declarations. Conflict of Interest: None., (© 2024. The Author(s), under exclusive licence to Dr. K C Chaudhuri Foundation.)

Objective: To surgically demonstrate preconceptional laparoscopic repair of a chronic myometrial defect with mesh reinforcement, resulting in a successful pregnancy outcome., Design: Video case report. The Institutional Ethical Committee was consulted, and the requirement for approval was waived because the video describes a modified surgical technique. The patient included in this video gave consent for publication of the video and posting of the video online, including on social media, the journal website, scientific literature websites (such as PubMed, ScienceDirect, Scopus, and others), and other applicable sites., Setting: A referral advanced gynecological endoscopy center., Patient: A 27-year-old woman (P 0 A 1 ) was diagnosed with myomas during pregnancy, resulting in miscarriage at 22 weeks. Laparotomy and myomectomy were performed 2 months later, and three 8-cm myomas were removed. The endometrial cavity opened posteriorly during surgery, and retained products of conceptions were removed. Periconceptional imaging done after two years showed few intramural myomas and a deficient myometrium in the posterior fundal region. Laparoscopy revealed a defect in the posterior fundal aspect of the uterus with leakage of dye, which was converted to laparotomy and myomectomy with the repair of the myometrial defect. After 1 year, follow-up magnetic resonance imaging showed thinned-out posterior myometrium with a focal area of absent myometrium in the midline and endometrial prolapse. The patient was advised on surrogacy, but she wanted to repair the defect again and try for pregnancy, so she was referred to our center. With the background of a few case reports using mesh to reinforce myometrial repair (1, 2), we counseled the patient about the myometrial repair with the additional use of mesh as an off-label use., Intervention: The risk of uterine rupture after myomectomy is rare (<1%) (3), but it is a severe complication. High-risk cases, like significant myometrial defects or previous ruptures, may require surgical correction. Native repair may not achieve optimal results in all cases. Alternative approaches, like the additional use of mesh or biological materials, have been reported (4). In this case, we demonstrate the use of dual mesh for scar repair. Synthetic mesh over the uterus is used in laparoscopic procedures like sacrohysteropexy and cerclage. We used Parietex (Covidien, New Haven, CT, USA) mesh, a composite macroporous polyester mesh usually used for ventral hernia repair. It has an outer hydrophilic, absorbable collagen barrier that reduces adhesion formation. Laparoscopically, after adhesiolysis, a significant defect was demonstrated on the posterior wall of the uterus (Fig. 1). A complete resection of the fibrotic tissue along the edges of the scar defect was done to expose healthy myometrium. Myometrium was repaired in two layers, excluding the endometrium, with a V-Loc (Covidien, Dublin, Ireland) No. 1-0 suture. Parietex mesh was sutured over the repaired posterior myometrium to reinforce it (Fig. 2)., Main Outcome Measures: The postoperative myometrial thickness on imaging and pregnancy outcome., Results: Postoperative ultrasound scan after 6 weeks demonstrated restoration of posterior wall myometrial thickness of 14 mm. The patient was conceived through in vitro fertilization techniques 4 months after surgery. Antenatal follow-up was uneventful except for suspicion of posterior placenta accreta. She underwent an elective cesarean section with uterine artery embolization at 34 weeks and delivered a healthy infant weighing 1,950 g. Placental removal was uneventful. On inspection, the posterior surface of the uterus was intact without dehiscence, meshing in situ with minimal adhesions (Fig. 3)., Conclusion: Myometrial scar defects can cause potential obstetric complications. Native repair of scar defects may not achieve optimal results, as in our case. Mesh repair of myomectomy scar defects can be used as an alternative approach, as exemplified in this case. However, further studies are required to establish the safety and efficacy of this approach., Competing Interests: Declaration of interests P.G.P. has nothing to disclose. N.J. has nothing to disclose. M. Shah has nothing to disclose. M. Sudhakar has nothing to disclose. G.P. has nothing to disclose., (Copyright © 2023 American Society for Reproductive Medicine. Published by Elsevier Inc. All rights reserved.)

Background: Abnormal uterine bleeding (AUB) is a common gynaecological condition. The levonorgestrel-releasing Intrauterine device (LNG-IUD) is an effective medical treatment. option which carries a small risk of device expulsion. For those who experience expulsion, some may benefit from a more robust surgical approach., Objectives: To demonstrate the technique for suture fixation of an LNG-IUD under hysteroscopic guidance. Materials and methods: Stepwise video demonstration of the technique using a 5mm hysteroscope and a 3mm laparoscopic needle holder. The Institutional Ethical Committee was consulted, and the requirement for approval was waived because the video described a modified surgical technique. Informed consent was obtained from the patient., Main Outcome Measures: A 35yr old parous woman with a nine-month history of AUB and severe dysmenorrhoea had an LNG-IUD sited with effective symptom relief. Unfortunately, the device was expelled six months after insertion, and she responded poorly to other medical treatments. Transvaginal ultrasonography (TVUS) suggested posterior wall adenomyosis. Considering her relief of symptoms with the LNG-IUD and history of expulsion, the patient was counselled regarding suture-fixation of the LNG-IUD., Results: She was followed-up at 6 months post insertion. The LNG-IUD was noted in the uterine cavity without displacement or expulsion., Conclusion: Hysteroscopy-guided suture fixation of an LNG-IUD is a minimally invasive, effective option for patients with a history of expulsion of an IUD. However, further studies are required to establish the safety and efficacy of this approach., Learning Objective: To demonstrate LNG -IUD suture fixation technique using hysteroscopy for patients diagnosed with AUB and a history of device expulsion.

Objective: To describe the vaginoscopic management of longitudinal vaginal septum in the case of obstructive hemivagina and ipsilateral renal agenesis (OHVIRA) syndrome., Design: Surgical video describing step-by-step management., Setting: OHVIRA syndrome also known as Herlyn-Werner-Wunderlich syndrome is a triad of obstructed hemivagina, uterus didelphys, and ipsilateral renal anomaly [1] (Supplimentary Video 1). Patients usually present after menarche with progressive dysmenorrhea, lower abdominal pain, a paravaginal mass, foul mucopurulent discharge, and intermenstrual bleeding due to hemi hematocolpos [2]. Magnetic resonance imaging is the choice of investigation [3]. Surgical resection of the septum is the choice of treatment, which can be done vaginoscopically to reduce postoperative pain and promote enhanced recovery [4]. In this video, we will demonstrate a case of a 28-years old, nulliparous woman diagnosed with uterine didelphys having lower abdominal pain and persistent vaginal discharge., Interventions: The video demonstrates the technique of vaginoscopic excision of the right hemi-vaginal septum that resulted in complete visualization of both cervices. Diagnostic laparoscopy confirmed uterine didelphys. The left cervix was visualized and the hysteroscope was negotiated into the cervical canal (Supplimentary Video 2). The left cavity was normal with left ostia. Intraoperative transrectal-ultrasound was done to localize the cystic collection in the right hemivagina. Needle aspiration of cystic collection was done over the bulging portion of the right hemivagina and mucoid material was aspirated. Longitudinal obstructive vaginal septum was incised using a collins knife and mucoid secretions were drained (Supplimentary Video 3). Hysteroscope inserted into opened right hemivagina, negotiated through the right cervix and right hemiuterus with right ostia was visualized. The residual septum was resected with a loop electrode and hemostasis was ensured. Cystoscopy done, left ureteric orifice with urine reflux visualized. Vaginal examination showed both cervices with near normal reconstructed vagina., Conclusion: The possibility of OHVIRA syndrome should be considered in all cases of uterine didelphys. Vaginoscopic management is a safe and effective method with a minimally invasive approach., (Copyright © 2023 AAGL. Published by Elsevier Inc. All rights reserved.)

Context: Patients with mutations in thyroid hormone transporter MCT8 have developmental delay and chronic thyrotoxicosis associated with being underweight and having cardiovascular dysfunction., Objective: Our previous trial showed improvement of key clinical and biochemical features during 1-year treatment with the T3 analogue Triac, but long-term follow-up data are needed., Methods: In this real-life retrospective cohort study, we investigated the efficacy of Triac in MCT8-deficient patients in 33 sites. The primary endpoint was change in serum T3 concentrations from baseline to last available measurement. Secondary endpoints were changes in other thyroid parameters, anthropometric parameters, heart rate, and biochemical markers of thyroid hormone action., Results: From October 15, 2014 to January 1, 2021, 67 patients (median baseline age 4.6 years; range, 0.5-66) were treated up to 6 years (median 2.2 years; range, 0.2-6.2). Mean T3 concentrations decreased from 4.58 (SD 1.11) to 1.66 (0.69) nmol/L (mean decrease 2.92 nmol/L; 95% CI, 2.61-3.23; P < 0.0001; target 1.4-2.5 nmol/L). Body-weight-for-age exceeded that of untreated historical controls (mean difference 0.72 SD; 95% CI, 0.36-1.09; P = 0.0002). Heart-rate-for-age decreased (mean difference 0.64 SD; 95% CI, 0.29-0.98; P = 0.0005). SHBG concentrations decreased from 245 (99) to 209 (92) nmol/L (mean decrease 36 nmol/L; 95% CI, 16-57; P = 0.0008). Mean creatinine concentrations increased from 32 (11) to 39 (13) µmol/L (mean increase 7 µmol/L; 95% CI, 6-9; P < 0.0001). Mean creatine kinase concentrations did not significantly change. No drug-related severe adverse events were reported., Conclusions: Key features were sustainably alleviated in patients with MCT8 deficiency across all ages, highlighting the real-life potential of Triac for MCT8 deficiency., (© The Author(s) 2021. Published by Oxford University Press on behalf of the Endocrine Society.)

Aim and Objectives: 1. To study the clinical outcome, growth and glycaemic control, 2. To study the frequency and type of genetic mutations., Methods: This is a retrospective study with a review of data of medical records from 2008 till date., Results: Twelve patients (six males) with neonatal diabetes mellitus (NDM) were identified. Median (interquartile range - (IQR)) age at diagnosis was 72 (31-95) days with a history of consanguinity in 75%. The median birth weight (range) was 2345 (900-3300) g. Follow-up data were available for eight patients with a median age at (IQR) follow-up of 3.3 (3-5.3) years. At follow-up, the mean annual HbA1c was 8.2% at a mean insulin dose of 1.1 U/kg/d. One patient with Wolcott-Rallison syndrome (WRS) and 21α-hydroxylase deficiency had poor growth and intellectual difficulty. The rest demonstrated satisfactory growth with an increase of mean weight centile from 2 nd to 13 th , height centile from 6.5 th to 20 th and normal neuro-cognitive development. Eleven patients underwent genetic testing with a molecular diagnosis in 54% (6/11): EIF2AK3 ( n = 2) and one each in INS , PDX1 , IL2RA and FOXP3 . None had variants in ABCC8 or KCNJ11 . One with immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX) syndrome underwent haematopoietic stem cell transplant (HSCT) and later succumbed., Conclusion: Our study demonstrates good clinical outcomes among NDM patients without immune dysfunction. Molecular diagnosis was attained only in around half of the patients (54%) with a great genetic heterogeneity., Competing Interests: There are no conflicts of interest., (Copyright: © 2022 Indian Journal of Endocrinology and Metabolism.)

Study Objective: To study the reproductive outcomes after laparoscopic myomectomy comparing conventional (nonbarbed) suture with barbed suture used for myometrial defect closure., Design: Monocentric retrospective cohort study with prospective follow-up survey for reproductive outcomes conducted in April and May 2020., Setting: Tertiary care center (center for advanced gynecologic laparoscopy and infertility)., Patients: Women who underwent laparoscopic myomectomy for uterine leiomyomas from January 2004 to December 2017., Interventions: Laparoscopic myomectomy with closure of the myometrium using either conventional (nonbarbed suture) or barbed suture and follow-up survey regarding reproductive outcomes., Measurements and Main Results: The outcomes measured included the rate of conception, pregnancy complications, mode of delivery, and perioperative complications for both kinds of suture materials used. Of the 399 women who underwent laparoscopic myomectomy, 343 satisfied the inclusion criteria and were followed up; 235 patients responded. A total of 120 patients were included in the nonbarbed group (group A), and 115 patients were included in the barbed group (group B). A total of 182 (group A: 97 vs group B: 85; p = .204) women had actively sought pregnancy postoperatively, of whom 93 (51.09%) in total and 51 (54.8%) in group A vs 42 (45.1%) in group B reported at least 1 pregnancy with no significant difference in the incidence rate ratios between the 2 groups. Of the recorded pregnancies84.9% (group A: 88.2% vs group B: 80.9%) live births, 6.4% (group A: 5.8% vs group B: 7.1%) had first-trimester miscarriages, 2.1% (group A: 1.9% vs group B: 2.3%) had an ectopic pregnancy, and 6 were ongoing pregnancies at the time of the study analysis, which were compared statistically between both study groups. Pregnancy-related complications were noted in 12 of the 93 pregnant women (12.9%), which were comparable in both groups. No case of uterine rupture was reported., Conclusion: Our study supports good reproductive outcomes in women after laparoscopic myomectomy with barbed sutures. Furthermore, the inclusion of nonbarbed sutures as a control group in our study reinforces that barbed sutures in myomectomy are as safe as, and an easier alternative to, conventional sutures without affecting pregnancy outcomes., (Copyright © 2021 AAGL. Published by Elsevier Inc. All rights reserved.)

Background and Objectives: In our institution, we have an ongoing newborn thyroid screening (NBS) program since July 2001. In the initial 9 months, we used cord blood thyroid-stimulating hormone (TSH) (CBTSH) cutoff of 20 mIU/L and thereafter the cutoff was increased to 25 mIU/L. Our objective was to evaluate whether a CBTSH cutoff of 25 mIU/L is sensitive and cost-effective in NBS of congenital hypothyroidism (CH)., Materials and Methods: All in-born babies are screened and those with CBTSH ≥25 mIU/L are recalled for confirmatory TSH/T4/FT4 tests. CH is confirmed with elevated TSH and low T4/FT4. Those with CBTSH 20-24.99 mIU/L were recalled for confirmatory tests in initial period of our NBS and prospectively between January and August 2017. Statistical analysis was done to derive positive predictive value and sensitivity to diagnose CH for each CBTSH between 20 and 30 mIU/L., Results: A total of 164,163 neonates were screened from July 2001 to August 2017. Of the 2352 babies with CBTSH ≥25-30 mIU/L, 1763 returned for retesting and 5 confirmed as CH (4 gland-in-situ and 1 absent uptake on nuclear scan). Of the 14,742 screened during the study period, 195 of the 293 babies with CBTSH 20-24.99 mIU/L returned for retesting and none diagnosed as CH. A CBTSH of 25 mIU/L has 99.2% sensitivity and 97.5% specificity. A lower screen TSH cutoff 20 mIU/L would result in recall of additional 300 babies/year with no definite improvement in sensitivity., Conclusions: Our data justify the continuation of using screen TSH cutoff of 25 mIU/L while using cord blood for NBS in our population. With a diverse and large population, it is important that we use feasible regional screen cutoffs for optimal use of our resources., Competing Interests: There are no conflicts of interest., (Copyright: © 2021 Indian Journal of Endocrinology and Metabolism.)

Study Objective: Cervical insufficiency occurs in 0.1% to 1 % of all pregnancies and is associated with a high risk of second-trimester abortion and/or preterm delivery [1]. Laparoscopic encerclage is highly recommended for a previous failed vaginal encerclage and is superior to the laparotomy approach in terms of low morbidity and faster recovery [2]. Laparoscopic encerclage in pregnancy is more challenging than that in the nonpregnant state. This is because of the enlarged uterine size, engorged uterine vessels, and infeasibility of using a uterine manipulator. The standardization and description of the technique are the main objectives of this video (Video 1). We have described the surgery in 6 steps that could make this procedure easier and safer., Design: A step-by-step video demonstration of the technique., Setting: Paul's Hospital, Centre for Advanced Endoscopy & Infertility Treatment, Kochi, India. A 29-year-old pregnant woman, gravida 3 abortions 2, at 13 weeks period of gestation, with a history of 2 second-trimester abortions owing to cervical insufficiency. The patient had a failed vaginal cervical encerclage at 18 weeks in the second pregnancy., Interventions: This is a step-wise laparoscopic approach for successful cervical encerclage in pregnancy. In this video, we demonstrate our technique for laparoscopic cervical encerclage in a pregnant woman's uterus in 6 steps using a Mersilene tape (Ethicon US, LLC, Somerville, NJ) as follows: (1) Opening the uterovesical fold and dissecting the bladder, (2) opening the left broad ligament and creating a window, (3) opening the right broad ligament and creating a window, (4) placing the Mersilene tape on the left side medial to the uterine vessels at the cervicoisthmic junction, (5) placing the Mersilene tape on the right side medial to the uterine vessels at the cervicoisthmic junction, (6) tying the Mersilene tape anteriorly., Conclusion: The standardization of laparoscopic cervical encerclage in pregnancy using the above 6 steps could make this procedure easier and safer to perform. Moreover, the standardization of the surgical technique could shorten the learning curve., (Copyright © 2020 AAGL. Published by Elsevier Inc. All rights reserved.)

Study Objective: To demonstrate a technique of laparoscopy-assisted neocervicovaginal reconstruction in a case of cervicovaginal aplasia., Design: Step-by-step demonstration of the surgery in an instructional video., Setting: Cervicovaginal aplasia is a rare congenital anomaly that occurs in 1 in 80 000 to 100 000 live births [1]. Occasionally, there can be other associated anomalies. Epigenetic modifications of homeobox and Wnt genes (wingless-type mouse mammary tumor virus integration site family) are hypothesized to cause defects in the development of the müllerian reproductive tract by interfering with cell migration during organogenesis [2]. Our patient was a 15-year-old girl who had a unicornuate uterus along with cervicovaginal aplasia (American Society for Reproductive Medicine class Ia, Ib, and IIc and European Society of Human Reproduction and Embryology class U4a, C4, and V4). The most common approach to treat this particular anomaly is hysterectomy, but there are many reports of neocervicovaginal reconstruction with good results [3]., Interventions: Laparoscopic assessment showed a right unicornuate uterus with hematometra, right hematosalpinx, and a left noncavitary rudimentary horn with endometriosis. A vertical incision was made over the most prominent bulging part of the uterine fundus, and the hematometra was drained. Laparoscopic inspection of the uterine cavity showed an irregular cavity with thickened endometrium. The cervical canal could not be identified. The inspection of the external genitalia revealed complete vaginal aplasia. An inverted T incision was made over the vestibule and neovagina created by blunt digital dissection. The bladder was laparoscopically mobilized down. A Maryland dissector was inserted into the uterine cavity through the incision in the fundus and directed toward the neovagina. The myometrium was then punctured while simultaneously visualizing the neovagina to create a neocervix. A 16-F Foley's catheter was then pulled into the uterine cavity with the Maryland dissector from the vaginal end. The neocervix was enlarged around the catheter and then sutured to the vestibular epithelium with 6 interrupted 1-0 polyglactin sutures. The Foley's catheter was anchored to the myometrium laparoscopically, and the uterine incision was closed with interrupted 1-0 polyglactin sutures. The right hematosalpinx was then excised. The intravaginal plastic mold was removed after 4 days. The patient was advised to use the bulb of a plastic pipette to help maintain vaginal patency. She resumed her menses 3 weeks after the surgery. Follow-up at 8 months revealed a normal-sized uterus on transabdominal ultrasound and a vaginal length of 5.5 cm on speculum examination. She currently reports regular cyclic menstruation with mild dysmenorrhea and has not yet begun sexual activity., Conclusion: Cervicovaginal aplasia can be successfully treated by laparoscopy-assisted neocervicovaginal reconstruction as demonstrated in the video., (Copyright © 2019 AAGL. Published by Elsevier Inc. All rights reserved.)

Background: Disordered thyroid hormone transport, due to mutations in the SLC16A2 gene encoding monocarboxylate transporter 8 (MCT8), is characterised by intellectual and motor disability resulting from cerebral hypothyroidism and chronic peripheral thyrotoxicosis. We sought to systematically assess the phenotypic characteristics and natural history of patients with MCT8 deficiency., Methods: We did an international, multicentre, cohort study, analysing retrospective data from Jan 1, 2003, to Dec 31, 2019, from patients with MCT8 deficiency followed up in 47 hospitals in 22 countries globally. The key inclusion criterion was genetically confirmed MCT8 deficiency. There were no exclusion criteria. Our primary objective was to analyse the overall survival of patients with MCT8 deficiency and document causes of death. We also compared survival between patients who did or did not attain full head control by age 1·5 years and between patients who were or were not underweight by age 1-3 years (defined as a bodyweight-for-age Z score <-2 SDs or <5th percentile according to WHO definition). Other objectives were to assess neurocognitive function and outcomes, and clinical parameters including anthropometric characteristics, biochemical markers, and neuroimaging findings., Findings: Between Oct 14, 2014, and Jan 17, 2020, we enrolled 151 patients with 73 different MCT8 (SLC16A2) mutations. Median age at diagnosis was 24·0 months (IQR 12·0-60·0, range 0·0-744·0). 32 (21%) of 151 patients died; the main causes of mortality in these patients were pulmonary infection (six [19%]) and sudden death (six [19%]). Median overall survival was 35·0 years (95% CI 8·3-61·7). Individuals who did not attain head control by age 1·5 years had an increased risk of death compared with patients who did attain head control (hazard ratio [HR] 3·46, 95% CI 1·76-8·34; log-rank test p=0·0041). Patients who were underweight during age 1-3 years had an increased risk for death compared with patients who were of normal bodyweight at this age (HR 4·71, 95% CI 1·26-17·58, p=0·021). The few motor and cognitive abilities of patients did not improve with age, as evidenced by the absence of significant correlations between biological age and scores on the Gross Motor Function Measure-88 and Bayley Scales of Infant Development III. Tri-iodothyronine concentrations were above the age-specific upper limit in 96 (95%) of 101 patients and free thyroxine concentrations were below the age-specific lower limit in 94 (89%) of 106 patients. 59 (71%) of 83 patients were underweight. 25 (53%) of 47 patients had elevated systolic blood pressure above the 90th percentile, 34 (76%) of 45 patients had premature atrial contractions, and 20 (31%) of 64 had resting tachycardia. The most consistent MRI finding was a global delay in myelination, which occurred in 13 (100%) of 13 patients., Interpretation: Our description of characteristics of MCT8 deficiency in a large patient cohort reveals poor survival with a high prevalence of treatable underlying risk factors, and provides knowledge that might inform clinical management and future evaluation of therapies., Funding: Netherlands Organisation for Health Research and Development, and the Sherman Foundation., (Copyright © 2020 Elsevier Ltd. All rights reserved.)

Background: Glycogen storage disease (GSD) is typified by early morning seizures. Absence of this results in delayed diagnosis, especially the non-GSD 1 group. Data are limited to few patients with unclear outcome., Objectives: 1. Study the common presentation and types of GSD. 2. Study the clinical and biochemical outcome. 3. Review genetic mutations., Methods: Observational study from May 2016-April 2019 at metabolic clinic at our center., Results: Total of 30 patients were diagnosed with GSD. Ten were excluded-Fanconi-Bickel (3) and <4 months follow-up (7). Data were analyzed for 20 patients (16 males). Mean age at presentation was 4.3 yrs. All had hepatomegaly, 90% had short stature, and 40% had early morning seizures. Mean follow-up was 22 months. There was a statistically significant improvement in metabolic parameters on treatment (mean)-fasting glucose from 50.4 to 79.5 mg/dl, SGPT from 416 to 199 U/L. Lipid profile showed reduction in triglycerides (318-225 mg/dl) but minimal increase in cholesterol (178-188 mg/dl). Mean weight centile improved from 14.1 to 20.3 and height centile from 2.3 to 7.9. Genetic testing confirmed types VI (3), III (3), IXa (1), IXc (1), and Ia (1). Liver biopsy confirmed GSD in 15/20. All were managed with uncooked corn starch. In addition, omega-3 fatty acid was used in 8/20 and high protein diet in 2 with GSD type III., Conclusion: Awareness of GSD needs to improve among pediatricians and hepatologists. The most common symptoms are asymptomatic hepatomegaly and short stature. Dietary therapy with uncooked corn starch remains mainstay of treatment. Mixed hyperlipidemia is difficult to control despite good metabolic improvement. Role of omega-3 fatty acid needs to be explored further. Genetic mutation analysis can assist with tailoring treatment and should get precedence over liver biopsy., (© 2019 Indian National Association for Study of the Liver. Published by Elsevier B.V. All rights reserved.)

Adenomyosis is the presence of endometrial glands and stroma within the myometrium. The focal and localized form of adenomyosis is known as adenomyoma. It is rarely located outside the uterus which is termed as extrauterine adenomyoma. We describe three cases of extrauterine adenomyomas which were located in pararectal space, round ligament and ovary. These cases were treated by laparoscopic excision and diagnosis was confirmed by histopathological examination. A review of the literature identified 34 cases of extrauterine adenomyomas. The most common locations were pararectal space, ovary and broad ligament. Other pelvic locations included the round ligament, paraovarian, parametrial and pelvic wall. Extrapelvic adenomyomas were located in the liver, upper abdomen, inguinal scar, appendix and small bowel mesentery. The abdominopelvic pain was the most common clinical presentation of extrauterine adenomyoma. Various imaging modalities were used to identify extrauterine masses, but a definitive diagnosis could not be made preoperatively in any of the cases. Although rare, a possible diagnosis of adenomyoma must be contemplated while dealing with extrauterine masses. Surgical excision is the mainstay of treatment. Since malignancy has been reported in extrauterine adenomyomas, this possibility must be kept in mind while offering treatment., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Background: Opportunistic bilateral salpingectomy (OBS), also called as prophylactic salpingectomy or risk-reducing salpingectomy is the concurrent removal of the fallopian tubes in premenopausal women. Though there are some studies comparing the intraoperative complications and effect of salpingectomy on ovarian reserve, limited data are available on long-term follow-up after OBS., Purpose of the Study: To evaluate the surgical outcome of routine bilateral salpingectomy during total laparoscopic hysterectomy (TLH) in terms of intraoperative and postoperative complications. We also evaluated the incidence and reoperation rate for adnexal pathology after TLH., Methods: A retrospective study of 1470 patients undergoing total laparoscopic hysterectomy (TLH) with opportunistic bilateral salpingectomy (OBS) over 13 years was carried out at Paul's Hospital., Results: The mean age of the subjects was 43.6 ± 4.2 years, mean body mass index was 27 ± 5.4 kg/m 2 , and median parity was 2 (range 0-7). 43% of women had at least one previous surgery. The most common indication for surgery was fibroid uterus (67%, n  = 985). The total complication rate was 4.4% ( n  = 65). One specimen showed paratubal borderline serous malignancy. The follow-up period ranged from 6 months to 13 years during which 17 (1.1%) women had adnexal pathology, and eight women (1.1%) needed resurgery for it. No ovarian malignancies were reported on follow-up., Conclusions: OBS is a simple and short surgical step during TLH without increasing morbidity. OBS eliminates the risk of future diseases of tubal origin, and there might be a possible reduction in incidence and reoperation rate for future ovarian pathologies., Competing Interests: Compliance with Ethical StandardsThe authors declared that they have no conflict of interest.Written informed consent was obtained from the patients for publication. The retrospective observational nature of the study did not necessitate the local institutional ethics committee approval.