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Archivos Argentinos de Pediatria Aug 2024Herlyn-Werner-Wunderlich syndrome, also known as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), is a rare, congenital Müllerian duct anomaly...
Herlyn-Werner-Wunderlich syndrome, also known as obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), is a rare, congenital Müllerian duct anomaly characterized by the association of septate uterus, obstructed hemivagina, and ipsilateral renal agenesis. The most common clinical presentation is an abdominal mass secondary to hematocolpos, pain, and dysmenorrhea. It is associated with infertility, endometriosis, and menstrual and obstetric alterations. The ultrasound is the technique of choice for the initial assessment, while the magnetic resonance imaging remains the most accurate method for diagnosis. The resection of the vaginal septum is the recommended treatment. Here we describe 2 clinical cases to highlight the importance of an early diagnosis to prevent potential complications in the future.
Topics: Humans; Female; Vagina; Syndrome; Kidney; Abnormalities, Multiple; Mullerian Ducts; Uterus; Child
PubMed: 38197594
DOI: 10.5546/aap.2023-10138.eng -
European Journal of Cell Biology Jun 2024Epithelial tissues cover the surfaces and lumens of the internal organs of multicellular animals and crucially contribute to internal environment homeostasis by... (Review)
Review
Epithelial tissues cover the surfaces and lumens of the internal organs of multicellular animals and crucially contribute to internal environment homeostasis by delineating distinct compartments within the body. This vital role is known as epithelial barrier function. Epithelial cells are arranged like cobblestones and intricately bind together to form an epithelial sheet that upholds this barrier function. Central to the restriction of solute and fluid diffusion through intercellular spaces are occluding junctions, tight junctions in vertebrates and septate junctions in invertebrates. As part of epithelial tissues, cells undergo constant renewal, with older cells being replaced by new ones. Simultaneously, the epithelial tissue undergoes relative rearrangement, elongating, and shifting directionally as a whole. The movement or shape changes within the epithelial sheet necessitate significant deformation and reconnection of occluding junctions. Recent advancements have shed light on the intricate mechanisms through which epithelial cells sustain their barrier function in dynamic environments. This review aims to introduce these noteworthy findings and discuss some of the questions that remain unanswered.
Topics: Animals; Humans; Epithelial Cells; Tight Junctions; Epithelium
PubMed: 38579602
DOI: 10.1016/j.ejcb.2024.151410 -
European Journal of Radiology Dec 2017Papillary glioneuronal tumors (PGNT) are a rare and recently recognized tumor entity. The neuroimaging findings were reviewed to determine if any specific findings...
OBJECTIVE
Papillary glioneuronal tumors (PGNT) are a rare and recently recognized tumor entity. The neuroimaging findings were reviewed to determine if any specific findings emerge to assist a preoperative diagnosis of PGNT.
MATERIALS AND METHODS
Seven histologically confirmed cases of PGNT were evaluated from 2004 to 2014. Clinical, neuroimaging and histological findings were reviewed and tabulated.
RESULTS
Headache and seizures were observed in 4 patients (57.1%) each. The majority (n=5, 71.4%) of lesions were periventricular and located in temporal lobe with 57.1% cases being solid cystic (n=4), and 42.9% being purely solid (n=3). Calcification and hemorrhage were noted in 3 cases (42.9%) and 5 cases (71.4%) respectively. The most frequent imaging feature was the presence of septations in the cystic component that enhanced on contrast which correlated with long pseudopapillary projections into the cyst cavity on histopathology. The solid inner component demonstrated heterogeneous enhancement. One case with tumor recurrence demonstrated hemorrhage with superficial siderosis, patchy diffusion restriction, raised choline and focal areas of raised perfusion which correlated on histopathology with increased cellularity and anaplasia.
CONCLUSION
Presence of cystic mass in periventricular location with septations and a solid inner component should raise a suspicion of PGNT on neuroimaging.
Topics: Adolescent; Adult; Brain Neoplasms; Calcinosis; Central Nervous System Cysts; Child; Child, Preschool; Female; Headache Disorders; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Neoplasm Recurrence, Local; Neoplasms, Neuroepithelial; Neuroimaging; Retrospective Studies; Seizures; Temporal Lobe; Young Adult
PubMed: 29153366
DOI: 10.1016/j.ejrad.2017.10.004 -
Journal of Minimally Invasive Gynecology Dec 2023To study the impact of Müllerian anomalies on reproductive outcomes in a recurrent pregnancy loss (RPL) population and to evaluate the effect of surgical correction of...
STUDY OBJECTIVE
To study the impact of Müllerian anomalies on reproductive outcomes in a recurrent pregnancy loss (RPL) population and to evaluate the effect of surgical correction of uterine septum on the odds of achieving live birth in RPL patients with a septate uterus.
DESIGN
A retrospective cohort study.
SETTING
A specialized RPL clinic at a tertiary center.
PATIENTS
RPL patients with ≥ 2 pregnancy losses before 20 weeks' gestation who attended a specialized RPL clinic.
INTERVENTION
We aimed to assess the association between a possible risk factor (Müllerian anomalies) and reproductive outcomes and that between having surgery for septate uterus and achieving a live birth.
MEASUREMENTS AND MAIN RESULTS
The primary outcome is live birth rate in RPL patients with Müllerian anomalies compared with those without; secondary outcome measures include rates of full-term live birth, preterm live birth, first and second trimester pregnancy loss, and stillbirth. After adjusting for patient age at the initial RPL visit, the number of pregnancy losses, and the presence of any other abnormal RPL investigation, the odds of achieving live birth were on average 49.4% lower for patients with a septate uterus than those without Müllerian anomalies (odds ratio, 0.51; 95% confidence interval, 0.30-0.86) in the studied cohort (n = 377). A subanalysis of 72 patients with septate uterus demonstrated a higher likelihood of live birth in those who underwent septum resection (46/72; 63.9%) than those who elected to go for expectant management (26/72; 36.1%), yet this study was underpowered to establish a significant difference (52.2% vs 34.6%; p = .22).
CONCLUSION
In RPL patients, having a septate uterus significantly decreased the chances of achieving live birth. Patients with septate uterus who received hysteroscopic septum division had a higher tendency to achieve more live births than those who elected expectant management. However, our study was underpowered to detect a statistically significant difference.
Topics: Pregnancy; Infant, Newborn; Female; Humans; Septate Uterus; Hysteroscopy; Retrospective Studies; Uterus; Abortion, Habitual; Premature Birth
PubMed: 37506876
DOI: 10.1016/j.jmig.2023.07.012 -
Journal of Gynecology Obstetrics and... Feb 2022To compare the reproductive outcome following hysteroscopic resection versus usual care in nulliparous women with a septate uterus. (Comparative Study)
Comparative Study
OBJECTIVE
To compare the reproductive outcome following hysteroscopic resection versus usual care in nulliparous women with a septate uterus.
METHODS
A retrospective cohort study of nulliparous women with a history of uterine septum and had singleton pregnancies at >20 weeks gestation between Jan. 2016 and Dec. 2019 were conducted. Follow-up was performed through medical record reviews and telephone conversations. The primary outcome was preterm birth at<37weeks gestation, and the secondary outcomes include cesarean delivery, malpresentation, preeclampsia, and birth weight<10th percentile for gestational age. Multivariate logistic regressions were performed to evaluate the effect of hysteroscopic resection on primary and secondary outcomes, after controlling for potential confounding factors.
RESULTS
Totally 198 women were included in this study, among which 112 women underwent a hysteroscopic resection and 86 women received usual care. Preterm birth incidence (7.1% vs. 18.6%, P = 0.03) and malpresentation (10.7% vs. 23.3%, P = 0.03) was significantly lower in women treated with hysteroscopic resection compared with those received usual care. Multivariate logistic regression indicated that hysteroscopic resection was significantly associated with decreased risks of preterm birth (OR = 0.36, 95% CI: 0.13-0.68; P < 0.01) and malpresentations (OR = 0.47, 95% CI: 0.25-0.71; P < 0.01), after controlling for potential confounding factors.
CONCLUSION
Hysteroscopic resection could significantly reduce the risks of preterm birth and malpresentations compared to usual care for nulliparous women with a septate uterus.
Topics: Adult; Cohort Studies; Female; Humans; Hysteroscopy; Pregnancy; Pregnancy Complications; Pregnancy Outcome; Retrospective Studies; Uterus
PubMed: 34852309
DOI: 10.1016/j.jogoh.2021.102275 -
Journal of Fungi (Basel, Switzerland) May 2023To explore the species diversity of the genus , specimens from Henan, Hubei and Jiangsu Provinces in China are examined, and three undescribed taxa are encountered. The...
To explore the species diversity of the genus , specimens from Henan, Hubei and Jiangsu Provinces in China are examined, and three undescribed taxa are encountered. The morphological characteristics and DNA sequence analyses of the combined , ITS, LSU, and regions support their placement in and their recognition as new species. sp. nov. is distinguished by the formation of abundant aerial mycelia on PDA, falcate, (1-)3-septate macroconidia 16-35 × 1.5-2.8 μm and subcylindrical, aseptate microconidia 7.5-13 × 0.8-1.1 μm. sp. nov. has a coralloid colony on PDA, falcate, 2-5-septate macroconidia 38-70 × 2-4.5 μm and rod-shaped to ellipisoidal, aseptate microconidia 2-7 × 1-1.9 μm. sp. nov. is characterized by filiform, 2-6-septate macroconidia 28-58 × 1.5-2.3 μm and lacking microconidia. Morphological differences between these novel species and their close relatives are compared in detail. The previously recorded species of the genus in China are listed and a key to these taxa is provided.
PubMed: 37233283
DOI: 10.3390/jof9050572 -
Molecular dissection of smooth septate junctions: understanding their roles in arthropod physiology.Annals of the New York Academy of... Jun 2017Smooth septate junctions (sSJs) are cell-cell junctions that are thought to regulate the paracellular pathway of the intestine and renal system in arthropods. The... (Review)
Review
Smooth septate junctions (sSJs) are cell-cell junctions that are thought to regulate the paracellular pathway of the intestine and renal system in arthropods. The detailed mechanism of action of sSJs is not well understood, because their molecular organization has remained elusive for a long time. Recently, two sSJ-specific membrane proteins, Ssk and Mesh, were identified by screening monoclonal antibodies raised against sSJ-containing membrane fractions isolated from the silkworm midgut. Furthermore, a genetic screen in Drosophila based on microscopic observation of sSJ formation identified Tsp2A as a novel sSJ-specific membrane protein. Together with Tsp2A, Ssk and Mesh form a protein complex, and all three proteins are required for sSJ formation, as well as intestinal barrier function in Drosophila. Additional studies are likely to elucidate their roles in (1) the formation and reorganization of sSJs, (2) paracellular barrier functions and permselectivity, and (3) short-term and long-term regulation of paracellular functions in arthropod epithelia.
Topics: Animals; Arthropods; Drosophila Proteins; Drosophila melanogaster; Intestinal Mucosa; Membrane Proteins; Models, Biological; Signal Transduction; Tetraspanins; Tight Junctions
PubMed: 28636800
DOI: 10.1111/nyas.13366 -
JGH Open : An Open Access Journal of... Sep 2021Multiseptated gallbladder (MSG) (also known as "Honeycomb gallbladder") is a rare condition that was first described by Knetsch in 1952, and there are around 150 cases... (Review)
Review
Multiseptated gallbladder (MSG) (also known as "Honeycomb gallbladder") is a rare condition that was first described by Knetsch in 1952, and there are around 150 cases described over the world. MSG has been described as a congenital anomaly in most of the cases and as acquired in a few. Moreover, the phenomenon was described with a variety of different symptoms and management. The aim of this article is to have better understanding of this condition and management approach. We are reporting a 4-year-old girl, who presented to Sidra Medicine, Qatar with MSG. We have also included 97 cases for review and analysis. The median age of presentation of the condition was 27 years but may present in neonates and in the elderly, while gender was not a risk factor. Abdominal pain is the most common presenting symptom, but it can present without symptoms. Certain congenital anomalies were detected in the pancreaticobiliary system in few patients with MSG. Medical treatment was reported in eight symptomatic patients, four of whom failed therapy. Cholecystectomy was performed in 40 patients, which resulted in resolutions of symptoms in 13 of them. Based on the available literature, congenital MSG is probably due to in-pouching of gallbladder wall to its own cavity forming septa containing muscular fibers. MSG can be diagnosed solely via imaging, and ultrasound appears to be an effective and feasible mode of diagnosis. Medical treatment efficacy is not well-known, but cholecystectomy has resulted in complete resolution in symptomatic patients.
PubMed: 34584965
DOI: 10.1002/jgh3.12621 -
The Cochrane Database of Systematic... Jan 2017Women with a septate uterus are at increased risk for subfertility, recurrent miscarriage, and preterm birth. Restoration of the anatomy of the uterus by hysteroscopic... (Review)
Review
BACKGROUND
Women with a septate uterus are at increased risk for subfertility, recurrent miscarriage, and preterm birth. Restoration of the anatomy of the uterus by hysteroscopic septum resection is an established intervention. This treatment has been assessed mainly in retrospective cohort studies, which suggested a positive effect on pregnancy outcomes. The major flaw in these studies is the before/after design, which will always favour the tested intervention.
OBJECTIVES
To determine whether hysteroscopic septum resection in women of reproductive age with a septate uterus improves live birth rates and to assess the safety of this procedure.
SEARCH METHODS
We searched the Cochrane Gynaecology and Fertility Group Specialised Register (inception to May 2016), the Cochrane Central Register of Controlled Trials (CENTRAL CRSO) (inception to May 2016), MEDLINE (1946 to May 2016), Embase (1974 to May 2016), PsycINFO (1806 to May 2016), and CINAHL database (1982 to May 2016). We also searched trial registers for ongoing and registered trials, reference lists, the Cochrane Library, unpublished dissertations and theses, conference abstracts, OpenGrey, LILACS, PubMed, and Google.
SELECTION CRITERIA
We planned to include randomised controlled trials that assessed the effect on reproductive outcomes and the safety of hysteroscopic septum resection in women of reproductive age with a septate uterus.
DATA COLLECTION AND ANALYSIS
If there had been studies to include, two review authors would have independently selected studies, assessed trial risk of bias, and extracted data. They would also have contacted study authors for additional information.
MAIN RESULTS
As in the 2011 version of this review, we identified no randomised controlled trials for inclusion in this update.
AUTHORS' CONCLUSIONS
Hysteroscopic septum resection in women of reproductive age with a septate uterus is performed worldwide to improve reproductive outcomes. At present, there is no evidence to support the surgical procedure in these women. Randomised controlled trials are urgently needed. Two trials are currently underway.
Topics: Abortion, Habitual; Adult; Female; Humans; Hysteroscopy; Pregnancy; Uterus
PubMed: 28093720
DOI: 10.1002/14651858.CD008576.pub4 -
Neurosurgical Review Apr 2024To analyse the imaging findings of papillary glioneuronal tumors (PGNTs), in order to improve the accuracy of preoperative diagnosis of this tumor. The clinical and...
To analyse the imaging findings of papillary glioneuronal tumors (PGNTs), in order to improve the accuracy of preoperative diagnosis of this tumor. The clinical and imaging manifestations of 36 cases of PGNT confirmed by pathology were analyzed retrospectively. A total of 17 males and 19 females, averaging 22.47 (± 11.23) years. Initial symptoms included epilepsy in ten, headache in seven, and others in 19 cases. 97.2% (35/36) of the lesions were located in the supratentorial area, and 80.5% (29/36) in the intraventricular or deep white matter adjacent to the lateral ventricles. Twenty-four of the lesions (66.7%) were mixed cystic and solid, four (11.1%) were cystic with mural nodules, four (11.1%) were cystic, and four (11.1%) were solid. Four cases of PGNT of cystic imaging showed a "T2-FLAIR mismatch" sign. 69.4% (25/36) had septations. Nine lesions (25%) were accompanied by edema, and 9 (25%) of the mixed cystic and solid lesions were accompanied by hemorrhage. Among the 18 patients who underwent computed tomography (CT) or susceptibility-weighted imaging (SWI), nine had lesions with calcification. PGNTs mostly manifest as cystic mass with mural nodules or mixed cystic and solid mass in the white matter around the supratentorial ventricle, and the cystic part of the lesion is mostly accompanied by septations. Pure cystic lesions may exhibit the sign of "T2-FLAIR mismatch". PGNT is rarely accompanied by edema but sometimes by calcification and hemorrhage. Patients often present with seizures, headaches, and mass effect symptoms.
Topics: Humans; Male; Female; Adult; Adolescent; Young Adult; Child; Magnetic Resonance Imaging; Retrospective Studies; Brain Neoplasms; Tomography, X-Ray Computed; Middle Aged; Ganglioglioma; Child, Preschool
PubMed: 38649515
DOI: 10.1007/s10143-024-02393-1