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Taiwanese Journal of Obstetrics &... Sep 2021Necrotizing funisitis (NF) is defined as the presence of an arc (i.e., crescent/band/ring/halos) of infiltrated neutrophils and/or associated debris in Wharton's jelly...
Necrotizing funisitis is an indicator that intra-amniotic inflammatory response is more severe and amnionitis is more frequent in the context of the extension of inflammation into Wharton's jelly.
OBJECTIVE
Necrotizing funisitis (NF) is defined as the presence of an arc (i.e., crescent/band/ring/halos) of infiltrated neutrophils and/or associated debris in Wharton's jelly (WJ) of umbilical-cord (UC). However, no information exists about the comparison in intra-amniotic inflammatory-response (IAIR) and inflammation in extra-placental membranes between the presence and absence of NF in the context of inflammation in WJ among spontaneous preterm births (PTBs). The objective of current study is to examine this issue.
MATERIALS AND METHODS
We examined IAIR and the frequency of amnionitis according to the progression of inflammation in UC (i.e. stage-1, umbilical phlebitis [inflammation in umbilical-vein(UV)] only; stage-2, involvement of at least one umbilical-artery[UA] and either the other UA or UV without extension into WJ; stage-3, the extension of inflammation into WJ without NF; stage-4, the extension of inflammation into WJ with NF) in 120singleton spontaneous PTBs (<37weeks). IAIR was gauged by AF MMP-8 (ng/ml) within 3days before birth.
RESULTS
1) Stage-1, stage-2, stage-3, and stage-4 were present in 20%(24/120), 6%(7/120), 61%(73/120), and 13%(16/120) of cases respectively; 2) AF MMP-8 continuously increased (stage-1 vs. stage-2 vs. stage-3 vs. stage-4; median[ng/ml], range[ng/ml]; 207.2[16.8-1196.5] vs. 444.1[8.5-2608.0] vs. 458.8[0.4-3116.7] vs. 1859.7[912.3-5304.8]; Spearman's rank correlation-test, α = 0.454, P = 0.006), and the frequency of increased AF MMP-8 (≥854.1 ng/ml) elevated (stage-1 vs. stage-2 vs. stage-3 vs. stage-4; 13%[1/8] vs. 33%[1/3] vs. 32%[6/19] vs. 100%[5/5]; Linear-by-linear-association, P = 0.012) with the progression of inflammation in UC; 3) Moreover, there was a stepwise increase in the frequency of amnionitis according to the progression of inflammation in UC (stage-1, 33%[8/24]; stage-2, 43%[3/7]; stage-3, 62%[45/73]; stage-4, 81%[13/16]; Linear-by-linear-association, P = 0.001).
CONCLUSION
NF is an indicator that IAIR is more severe and amnionitis is more frequent in the context of the extension of inflammation into WJ. Therefore, current study confirms that NF is the most advanced stage in the progression of inflammation within UC.
Topics: Adult; Amnion; Amniotic Fluid; Chorioamnionitis; Female; Humans; Inflammation; Matrix Metalloproteinase 8; Placenta; Pregnancy; Pregnancy Complications, Infectious; Premature Birth; Umbilical Cord; Wharton Jelly
PubMed: 34507659
DOI: 10.1016/j.tjog.2021.07.011 -
Medicine Jul 2021Amniotic band syndrome (ABS) is a congenital malformation that results in abnormalities in many parts of the body. Most surgical treatments for ABS used multi-stage... (Clinical Trial)
Clinical Trial
BACKGROUND
Amniotic band syndrome (ABS) is a congenital malformation that results in abnormalities in many parts of the body. Most surgical treatments for ABS used multi-stage Z-plasties. The purpose of this study was to assess the clinical results of one-stage circular incision techniques for limb ring constriction due to amniotic band syndrome.
METHODS
We reviewed 27 patients with limb ring constriction in ABS from 2010 to 2020. The mean ages of the patients were 11.7 months (range, 0-72 months). The complete circular incision release the ABS. All patients' operations were used one-stage circular incision surgical techniques, including patients with multiple bands. All the patients were followed up with a period ranges from 2 years to 10 years. Patient-reported visual analog scale (VAS) scar ranking on a scale of 0 (minimum satisfaction) to 10 (maximum satisfaction) were used to evaluate esthetic outcomes.
RESULTS
After our surgery, all the limbs, toes, and fingers were rescued, and the lymphedema reduced remarkably. The VAS scores (mean ± SD) for patient satisfaction were 7.55 ± 1.89. The surgical treatment of amniotic band syndrome in a one-stage circular incision is safe and effective.
CONCLUSION
The one-stage circular incision surgical techniques have many advantages, including reduced surgical invasiveness, scar formation, and the cost of treatment.
LEVEL OF EVIDENCE
Level IV-retrospective case series.
Topics: Amniotic Band Syndrome; Child; Child, Preschool; Female; Humans; Infant; Infant, Newborn; Male; Surgical Procedures, Operative
PubMed: 34397722
DOI: 10.1097/MD.0000000000026764 -
European Journal of Medical Research Jun 2021Since 2011, screening maternal blood for cell-free foetal DNA (cffDNA) fragments has offered a robust clinical tool to classify pregnancy as low or high-risk for Down,...
BACKGROUND
Since 2011, screening maternal blood for cell-free foetal DNA (cffDNA) fragments has offered a robust clinical tool to classify pregnancy as low or high-risk for Down, Edwards, and Patau syndromes. With recent advances in molecular biology and improvements in data analysis algorithms, the screening's scope of analysis continues to expand. Indeed, screening now encompassess additional conditions, including aneuploidies for sex chromosomes, microdeletions and microduplications, rare autosomal trisomies, and, more recently, segmental deletions and duplications called copy number variations (CNVs). Yet, the ability to detect CNVs creates a new challenge for cffDNA analysis in couples in which one member carries a structural rearrangement such as a translocation or inversion.
CASE PRESENTATION
We report a segmental duplication of the long arm of chromosome 3 and a segmental deletion of the short arm of chromosome 5 detected by cffDNA analysis in a 25-year-old pregnant woman. The blood sample was sequenced on a NextSeq 550 (Illumina) using the VeriSeq NIPT Solution v1 assay. G-band karyotyping in amniotic fluid only detected an abnormality in chromosome 5. Next-generation sequencing in amniocytes confirmed both abnormalities and identified breakpoints in 3q26.32q29 and 5p13.3p15. The foetus died at 21 weeks of gestation due to multiple abnormalities, and later G-band karyotyping in the parents revealed that the father was a carrier of a balanced reciprocal translocation [46,XY,t(3;5)(q26.2;p13)]. Maternal karyotype appeared normal.
CONCLUSION
This case provides evidence that extended cffDNA can detect, in addition to aneuploidies for whole chromosomes, large segmental aneuploidies. In some cases, this may indicate the presence of chromosomal rearrangements in a parent. Such abnormalities are outside the scope of standard cffDNA analysis targeting chromosomes 13, 18, 21, X, and Y, potentially leading to undiagnosed congenital conditions.
Topics: Adult; Biomarkers; Cell-Free Nucleic Acids; Chromosomes, Human, Pair 3; DNA Copy Number Variations; Female; Fetal Diseases; Fetus; Genetic Testing; Humans; Karyotyping; Pregnancy; Trisomy
PubMed: 34187576
DOI: 10.1186/s40001-021-00535-5 -
BMC Pregnancy and Childbirth Jun 2021Amniotic band syndrome is a rare phenomenon, but it can result in serious complications. We report herein our experience of amniotic band syndrome in a monochorionic...
BACKGROUND
Amniotic band syndrome is a rare phenomenon, but it can result in serious complications. We report herein our experience of amniotic band syndrome in a monochorionic diamniotic twin pregnancy where rupture of the dividing membrane occurred early in the second trimester.
CASE PRESENTATION
A 29-year-old nulliparous woman was referred to us for management of her monochorionic diamniotic twin pregnancy at 10 weeks of gestation. When we were unable to identify a dividing membrane at 15 weeks of gestation using two-dimensional ultrasonography, we used three-dimensional ultrasonography to confirm its absence. Both modalities showed that the left arm of baby B was swollen and attached to a membranous structure originating from the placenta at 18 weeks of gestation. Tangled umbilical cords were noted on magnetic resonance imaging at 18 weeks of gestation. Emergency cesarean delivery was performed at 30 weeks of gestation because of the nonreassuring fetal status of baby A. The left arm of baby B had a constrictive ring with a skin defect. Both neonates had an uncomplicated postnatal course and were discharged around 2 months after delivery.
CONCLUSIONS
Attention should be paid to the potential for amniotic band syndrome if rupture of the dividing membrane between twins is noted during early gestation.
Topics: Adult; Amniotic Band Syndrome; Cesarean Section; Female; Fetal Membranes, Premature Rupture; Humans; Infant, Newborn; Live Birth; Pregnancy; Pregnancy Trimester, Second; Pregnancy, Twin; Premature Birth; Twins, Monozygotic; Ultrasonography, Prenatal
PubMed: 34182926
DOI: 10.1186/s12884-021-03948-6 -
Plastic and Reconstructive Surgery.... May 2021Congenital amniotic band syndrome (ABS) is an anomaly with no proven etiology occurring in 0.7 per 10,000 live births. This defect mostly concerns the extremities and is...
Congenital amniotic band syndrome (ABS) is an anomaly with no proven etiology occurring in 0.7 per 10,000 live births. This defect mostly concerns the extremities and is often accompanied by other developmental anomalies. There are many methods of treatment for this type of defect, such as simple excision and suturing, local V-Y plasty, Z-plasty, multiple Z "plasties" or multiple W plasties, plasty with deepithelized or non-deepithelized rectangular lobes, and rigottomies complemented with lipofilling. The literature most often describes cases of treated children. There are no case reports of ABS treatment in adults. However, failure to undertake such treatment in childhood may result in serious mental dysfunction. We present the case of a 39-year-old woman with congenital ABS, in whom failure to provide proper treatment in childhood resulted in persistent depressive disorder development. The applied treatment, consisting of multiple Z plasties, liposuction, and fat grafting, resulted in improved appearance of her lower extremity, as well as the cessation of mental symptoms.
PubMed: 34055562
DOI: 10.1097/GOX.0000000000003594 -
BMJ Case Reports May 2021Limb body wall complex (LBWC) is a rare, lethal malformation characterised by body wall defects, craniofacial and limb anomalies with or without various other organ...
Limb body wall complex (LBWC) is a rare, lethal malformation characterised by body wall defects, craniofacial and limb anomalies with or without various other organ anomalies. We report a case of dichorionic diamniotic twin pregnancy discordant for LBWC, diagnosed by ultrasound (US) and confirmed by MRI at 21 weeks' gestation, managed expectantly and delivered at 35 weeks by emergency caesarean section with a favourable outcome of the unaffected twin. The anomalous twin, who died soon after birth, had a sizeable thoracoabdominal wall defect, eviscerated liver and bowel loops attached to the placenta, short cord, ectopia cordis, lung hypoplasia, kyphoscoliosis, right upper limb amelia and left clubfoot with polydactyly. MRI helps to demonstrate the fetal morphology better when there are limitations to the US due to unfavourable fetal position, multifetal gestation, maternal obesity or reduced liquor. In twin pregnancies, the management will depend on ensuring the survival of the unaffected twin.
Topics: Cesarean Section; Female; Fetus; Humans; Magnetic Resonance Imaging; Pregnancy; Pregnancy Reduction, Multifetal; Pregnancy, Twin
PubMed: 33980568
DOI: 10.1136/bcr-2021-242783 -
BMC Anesthesiology Apr 2021Adams-Oliver syndrome is characterized by the combination of congenital scalp defects and terminal transverse limb defects. In some instances, cardiovascular... (Review)
Review
BACKGROUND
Adams-Oliver syndrome is characterized by the combination of congenital scalp defects and terminal transverse limb defects. In some instances, cardiovascular malformations and orofacial malformations have been observed. Little is written with regards to the anesthetic management and airway concerns of patients with Adams-Oliver syndrome.
CASE PRESENTATION
A five-year-old female with Adams-Oliver syndrome presented for repeat lower extremity surgery. Airway exam was significant for dysmorphic features, such as hypertelorism, deviated jaw, and retrognathia. Video laryngoscope was utilized for intubation due to the patients retrognathic jaw, cranial deformities, and facial dysmorphism. A vein finder with ultrasound guidance was needed to place the peripheral intravenous line due to her history of difficult intravenous access. The patient was successfully intubated with slight cricoid pressure applied to direct the endotracheal tube smoothly. Surgery and recovery were both unremarkable.
CONCLUSIONS
Due to varying presentations of Adams-Oliver syndrome, anesthetic and airway management considerations should be carefully assessed prior to surgery. Anesthesiologists must take into consideration possible orofacial abnormalities that may make intubation difficult. Amniotic band syndrome and other limb defects could potentially impact intravenous access as well.
Topics: Airway Management; Child, Preschool; Ectodermal Dysplasia; Female; Humans; Limb Deformities, Congenital; Scalp Dermatoses
PubMed: 33858352
DOI: 10.1186/s12871-021-01339-0 -
Nanotechnology, Science and Applications 2021Oxidative tissue damage caused by reactive oxygen species results in a significant decrease in the total antioxidant capacity of the biological system. The aim of this...
INTRODUCTION
Oxidative tissue damage caused by reactive oxygen species results in a significant decrease in the total antioxidant capacity of the biological system. The aim of this interdisciplinary study was to answer the question of whether active antioxidants modify, at a molecular and supramolecular level, the tissue of pathological amnion and the necrotic eschar degraded in thermal burn.
METHODS
A Nicolet 6700 Fourier-transform spectrophotometer with OMNIC software and the EasiDiff diffusion accessory were used in the FTIR spectroscopic analysis. A NICOLET MAGNA-IR 860 spectrometer with FT-Raman accessory was used to record the Raman spectra of the samples. The samples were exposed to bacteria capable of causing nosocomial infections, ie Gram-positive and Gram-negative and . Whereas samples of hypotrophic amnion interacted with and . The obtained flame retardant effect of placentas was evaluated using the method of the limiting oxygen index (LOI).
RESULTS
The infrared spectroscopy analysis proved that after modification of the amniotic samples in graphene oxide and ortho-silicic acid, the amide II band is split into two components. Incubation of samples in modifier solutions: graphene oxide, sodium ascorbate and L-ascorbic acid results in shifts and changes of intensity within the broadly understood lipid band 1743-1745-1747 cm. The oxidising changes observed within the lipid and amide bands are affected by the incubation effect of graphene oxide as a modifier, possibly adsorbing on the surface of the amniotic membrane. On the basis of microbiological studies, pathogenic bacteria commonly causing amniotic infections and growing in burn wounds were found to have particularly good resistance to stabilized ortho-silicic acid () and lactoferrin ().
CONCLUSION
This thermogravimetric study found the highest stability of the analysed tissues (hypotrophic amnion and burnt epidermis) after modification with graphene oxide and sodium ascorbate.
PubMed: 33727805
DOI: 10.2147/NSA.S294412 -
Molecular Genetics & Genomic Medicine Apr 2021A heterozygous natriuretic peptide receptor 2 (NPR2) gene c.2455C>T mutation was identified as a cause of familial idiopathic short stature (ISS). Only two cases with...
De novo c.2455C>T mutation of NPR2 gene in a fetus with shortened long bones and a ventricular septal defect conceived by a mother with a fragile site at 16q22.1 and a father with a rare heterochromatic variant of chromosome 4 from Vietnam.
BACKGROUND
A heterozygous natriuretic peptide receptor 2 (NPR2) gene c.2455C>T mutation was identified as a cause of familial idiopathic short stature (ISS). Only two cases with this mutation were reported previously, and the probands with ISS had no organ system defects.
METHODS
Next-generation sequencing (NGS) was performed on an amniotic fluid DNA sample of a fetus with shortened long bones and a small ventricular septal defect detected by an obstetric ultrasound examination. The pathogenic variant of the fetus was confirmed by Sanger sequencing. Sanger sequencing, G-banded, and C-banded karyotyping of the fetus's parents were subsequently performed.
RESULTS
A de novo NPR2 gene c.2455C>T, p.(Arg819Cys) mutation was identified in the fetus. No microdeletion or microduplication was identified in the fetus by copy number variation sequencing with a maximum resolution of 400 kb. The two previous miscarriages experienced by the fetus's parents were interpreted as a result of chromosomal aberrations, including a maternal fragile site at 16q22.1 and a rare paternal variant involving in a large G-band-positive and C-band-positive block of paracentric heterochromatin of chromosome 4p.
CONCLUSION
This report provides clinical signs of a de novo heterozygous NPR2 gene c.2455C>T mutation in the fetus and shows paternal chromosomal aberrations causing repeated pregnancy loss.
Topics: Adult; Amniocentesis; Chromosome Fragile Sites; Chromosomes, Human, Pair 16; Chromosomes, Human, Pair 4; Female; Fetus; Heart Septal Defects, Ventricular; Heterochromatin; Humans; Karyotype; Leg Bones; Mutation; Pregnancy; Receptors, Atrial Natriuretic Factor; Sequence Analysis, DNA; Ultrasonography, Prenatal
PubMed: 33713577
DOI: 10.1002/mgg3.1637 -
Hand (New York, N.Y.) Jan 2022Urgent surgical intervention for amniotic band sequence (ABS) is currently indicated for concerns of vascular compromise and progressive lymphedema. Peripheral motor...
Urgent surgical intervention for amniotic band sequence (ABS) is currently indicated for concerns of vascular compromise and progressive lymphedema. Peripheral motor nerve palsies are rare, and reports of surgical intervention in these cases describe persistent motor dysfunction. We report band release and ulnar, median, and radial nerve decompression in a 1-week-old with a severe upper extremity constriction band and signs of ulnar nerve motor dysfunction. A literature review on nerve exploration and outcomes of patients with motor nerve palsy from ABS was performed. Early evidence of ulnar motor function was observed at 5.5-month follow-up. Previous reports of nerve decompression for upper extremity constriction bands with motor nerve palsy document poor recovery after interventions beginning at 3 months of age. In this case, band release and nerve decompression were undertaken at 7 days of age, and we observed early motor recovery. This finding suggests that very early surgical intervention in the neonate may facilitate nerve recovery in appropriate candidates.
Topics: Amniotic Band Syndrome; Humans; Infant, Newborn; Paralysis; Ulnar Nerve; Upper Extremity
PubMed: 33703924
DOI: 10.1177/1558944721994260