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1.
Cell ; 184(14): 3762-3773.e10, 2021 07 08.
Artigo em Inglês | MEDLINE | ID: mdl-34133943

RESUMO

Sneezing is a vital respiratory reflex frequently associated with allergic rhinitis and viral respiratory infections. However, its neural circuit remains largely unknown. A sneeze-evoking region was discovered in both cat and human brainstems, corresponding anatomically to the central recipient zone of nasal sensory neurons. Therefore, we hypothesized that a neuronal population postsynaptic to nasal sensory neurons mediates sneezing in this region. By screening major presynaptic neurotransmitters/neuropeptides released by nasal sensory neurons, we found that neuromedin B (NMB) peptide is essential for signaling sneezing. Ablation of NMB-sensitive postsynaptic neurons in the sneeze-evoking region or deficiency in NMB receptor abolished the sneezing reflex. Remarkably, NMB-sensitive neurons further project to the caudal ventral respiratory group (cVRG). Chemical activation of NMB-sensitive neurons elicits action potentials in cVRG neurons and leads to sneezing behavior. Our study delineates a peptidergic pathway mediating sneezing, providing molecular insights into the sneezing reflex arc.


Assuntos
Tronco Encefálico/fisiopatologia , Neuropeptídeos/metabolismo , Nariz/fisiopatologia , Reflexo/fisiologia , Espirro/fisiologia , Animais , Modelos Animais de Doenças , Hipersensibilidade/fisiopatologia , Masculino , Camundongos Endogâmicos C57BL , Neurocinina B/análogos & derivados , Neurocinina B/metabolismo , Neurônios/metabolismo , RNA Interferente Pequeno/metabolismo , Células Receptoras Sensoriais/fisiologia , Canais de Cátion TRPV/metabolismo , Gravação em Vídeo
2.
Development ; 151(10)2024 May 15.
Artigo em Inglês | MEDLINE | ID: mdl-38646822

RESUMO

The precise assembly of tissues and organs relies on spatiotemporal regulation of gene expression to coordinate the collective behavior of cells. In Drosophila embryos, the midgut musculature is formed through collective migration of caudal visceral mesoderm (CVM) cells, but how gene expression changes as cells migrate is not well understood. Here, we have focused on ten genes expressed in the CVM and the cis-regulatory sequences controlling their expression. Although some genes are continuously expressed, others are expressed only early or late during migration. Late expression relates to cell cycle progression, as driving string/Cdc25 causes earlier division of CVM cells and accelerates the transition to late gene expression. In particular, we found that the cell cycle effector transcription factor E2F1 is a required input for the late gene CG5080. Furthermore, whereas late genes are broadly expressed in all CVM cells, early gene transcripts are polarized to the anterior or posterior ends of the migrating collective. We show this polarization requires transcription factors Snail, Zfh1 and Dorsocross. Collectively, these results identify two sequential gene expression programs bridged by cell division that support long-distance directional migration of CVM cells.


Assuntos
Divisão Celular , Movimento Celular , Proteínas de Drosophila , Regulação da Expressão Gênica no Desenvolvimento , Animais , Movimento Celular/genética , Proteínas de Drosophila/metabolismo , Proteínas de Drosophila/genética , Divisão Celular/genética , Mesoderma/metabolismo , Mesoderma/citologia , Fatores de Transcrição/metabolismo , Fatores de Transcrição/genética , Drosophila melanogaster/genética , Drosophila melanogaster/metabolismo , Drosophila melanogaster/embriologia , Fator de Transcrição E2F1/metabolismo , Fator de Transcrição E2F1/genética , Embrião não Mamífero/metabolismo , Embrião não Mamífero/citologia , Drosophila/genética , Drosophila/metabolismo , Drosophila/embriologia , Fatores de Transcrição da Família Snail/metabolismo , Fatores de Transcrição da Família Snail/genética
3.
Development ; 150(7)2023 04 01.
Artigo em Inglês | MEDLINE | ID: mdl-36938965

RESUMO

Blood vessels form elaborate networks that depend on tissue-specific signalling pathways and anatomical structures to guide their growth. However, it is not clear which morphogenetic principles organize the stepwise assembly of the vasculature. We therefore performed a longitudinal analysis of zebrafish caudal fin vascular assembly, revealing the existence of temporally and spatially distinct morphogenetic processes. Initially, vein-derived endothelial cells (ECs) generated arteries in a reiterative process requiring vascular endothelial growth factor (Vegf), Notch and cxcr4a signalling. Subsequently, veins produced veins in more proximal fin regions, transforming pre-existing artery-vein loops into a three-vessel pattern consisting of an artery and two veins. A distinct set of vascular plexuses formed at the base of the fin. They differed in their diameter, flow magnitude and marker gene expression. At later stages, intussusceptive angiogenesis occurred from veins in distal fin regions. In proximal fin regions, we observed new vein sprouts crossing the inter-ray tissue through sprouting angiogenesis. Together, our results reveal a surprising diversity among the mechanisms generating the mature fin vasculature and suggest that these might be driven by separate local cues.


Assuntos
Células Endoteliais , Peixe-Zebra , Animais , Peixe-Zebra/genética , Fator A de Crescimento do Endotélio Vascular/metabolismo , Neovascularização Fisiológica , Veias/metabolismo
4.
Development ; 150(14)2023 07 15.
Artigo em Inglês | MEDLINE | ID: mdl-37390294

RESUMO

Caudal developmental defects, including caudal regression, caudal dysgenesis and sirenomelia, are devastating conditions affecting the skeletal, nervous, digestive, reproductive and excretory systems. Defects in mesodermal migration and blood supply to the caudal region have been identified as possible causes of caudal developmental defects, but neither satisfactorily explains the structural malformations in all three germ layers. Here, we describe caudal developmental defects in transmembrane protein 132a (Tmem132a) mutant mice, including skeletal, posterior neural tube closure, genitourinary tract and hindgut defects. We show that, in Tmem132a mutant embryos, visceral endoderm fails to be excluded from the medial region of early hindgut, leading directly to the loss or malformation of cloaca-derived genitourinary and gastrointestinal structures, and indirectly to the neural tube and kidney/ureter defects. We find that TMEM132A mediates intercellular interaction, and physically interacts with planar cell polarity (PCP) regulators CELSR1 and FZD6. Genetically, Tmem132a regulates neural tube closure synergistically with another PCP regulator Vangl2. In summary, we have identified Tmem132a as a new regulator of PCP, and hindgut malformation as the underlying cause of developmental defects in multiple caudal structures.


Assuntos
Defeitos do Tubo Neural , Camundongos , Animais , Defeitos do Tubo Neural/metabolismo , Tubo Neural/metabolismo , Neurulação , Camadas Germinativas/metabolismo , Polaridade Celular/fisiologia , Proteínas de Membrana/genética , Proteínas de Membrana/metabolismo
5.
Development ; 149(7)2022 04 01.
Artigo em Inglês | MEDLINE | ID: mdl-35297968

RESUMO

Vascular networks comprise endothelial cells and mural cells, which include pericytes and smooth muscle cells. To elucidate the mechanisms controlling mural cell recruitment during development and tissue regeneration, we studied zebrafish caudal fin arteries. Mural cells colonizing arteries proximal to the body wrapped around them, whereas those in more distal regions extended protrusions along the proximo-distal vascular axis. Both cell populations expressed platelet-derived growth factor receptor ß (pdgfrb) and the smooth muscle cell marker myosin heavy chain 11a (myh11a). Most wrapping cells in proximal locations additionally expressed actin alpha2, smooth muscle (acta2). Loss of Pdgfrb signalling specifically decreased mural cell numbers at the vascular front. Using lineage tracing, we demonstrate that precursor cells located in periarterial regions and expressing Pgdfrb can give rise to mural cells. Studying tissue regeneration, we did not find evidence that newly formed mural cells were derived from pre-existing cells. Together, our findings reveal conserved roles for Pdgfrb signalling in development and regeneration, and suggest a limited capacity of mural cells to self-renew or contribute to other cell types during tissue regeneration.


Assuntos
Miócitos de Músculo Liso , Pericitos , Receptor beta de Fator de Crescimento Derivado de Plaquetas , Proteínas de Peixe-Zebra , Peixe-Zebra , Animais , Células Endoteliais/metabolismo , Miócitos de Músculo Liso/metabolismo , Pericitos/metabolismo , Receptor beta de Fator de Crescimento Derivado de Plaquetas/genética , Receptor beta de Fator de Crescimento Derivado de Plaquetas/metabolismo , Peixe-Zebra/metabolismo , Proteínas de Peixe-Zebra/genética , Proteínas de Peixe-Zebra/metabolismo
6.
J Cardiovasc Electrophysiol ; 35(3): 433-437, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-38205869

RESUMO

BACKGROUND: Pneumothorax is a well-recognized complication of cardiac implantable electronic device (CIED) insertion. While AP fluoroscopy alone is the most commonly imaging technique for subclavian or axillary access, caudal fluoroscopy (angle 40°) is routinely used at our institution. The caudal view provides additional separation of the first rib and clavicle and may reduce the risk of pneumothorax. We assessed outcomes at our institution of AP and caudal fluoroscopic guided pacing lead insertion. METHODS: Retrospective cohort study of consecutive patients undergoing transvenous lead insertion for pacemakers, defibrillators, and cardiac resynchronization therapy devices between 2011 and 2023. Both de novo and lead replacement/upgrade procedures were included. Data were extracted from operative, radiology, and discharge reports. All patients underwent postprocedure chest radiography. RESULTS: Three thousand two hundred fifty-two patients underwent insertion of pacing leads between February 2011 and March 2023. Mean age was 71.1 years (range 16-102) and 66.7% were male. Most (n = 2536; 78.0%) procedures used caudal guidance to obtain venous access, while 716 (22.0%) procedures used AP guidance alone. Pneumothoraxes occurred in five (0.2%) patients in the caudal group and five (0.7%) patients in the AP group (p = .03). Subclavian contrast venography was performed less frequently in the caudal group (26.2% vs. 42.7%, p < .01). CONCLUSION: Caudal fluoroscopy for axillary/subclavian access is associated with a lower rate of pneumothorax and contrast venography compared with an AP approach.


Assuntos
Desfibriladores Implantáveis , Marca-Passo Artificial , Pneumotórax , Humanos , Masculino , Adolescente , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Idoso , Idoso de 80 Anos ou mais , Feminino , Veia Axilar , Marca-Passo Artificial/efeitos adversos , Estudos Retrospectivos , Fluoroscopia/métodos , Desfibriladores Implantáveis/efeitos adversos
7.
J Anat ; 245(2): 289-302, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38613221

RESUMO

The salt marsh harvest mouse (Reithrodontomys raviventris) is an endangered species, endemic to the San Francisco Bay Estuary, that co-occurs with the more broadly distributed species, the western harvest mouse (Reithrodontomys megalotis). Despite their considerable external morphological similarities, the northern subspecies of salt marsh harvest mice have relatively longer and thicker tails than do western harvest mice, which may be related to their abilities to climb emergent marsh vegetation to avoid tidal inundation. We used micro-CT to compare post-cranial skeletal anatomy between the salt marsh and western harvest mouse, to examine whether the salt marsh harvest mouse's restriction to brackish marshes is associated with skeletal adaptations for scansorial locomotion. We found that salt marsh harvest mice exhibited a deeper 3rd caudal vertebra, a more caudally located longest tail vertebra, craniocaudally longer tail vertebrae, and a longer digit III proximal phalanx than western harvest mice. These phalangeal and vertebral characteristics are known to decrease body rotations during climbing, increase contact with substrates, and decrease fall susceptibility in arboreal mammals, suggesting that the salt marsh harvest mouse may be morphologically specialized for scansorial locomotion, adaptive for its dynamic wetland environment.


Assuntos
Locomoção , Animais , Locomoção/fisiologia , Áreas Alagadas , Microtomografia por Raio-X , Osso e Ossos/anatomia & histologia , Osso e Ossos/fisiologia
8.
FASEB J ; 37(7): e23053, 2023 07.
Artigo em Inglês | MEDLINE | ID: mdl-37342918

RESUMO

The genetic control and signaling pathways of vascular development are not comprehensively understood. Transcription factors Islet2 (Isl2) and nr2f1b are critical for vascular growth in zebrafish, and further transcriptome analysis has revealed potential targets regulated by isl2/nr2f1b. In this study, we focused on the potential activation gene signal-transducing adaptor protein 2b (stap2b) and revealed a novel role of stap2b in vascular development. stap2b mRNA was expressed in developing vessels, suggesting stap2b plays a role in vascularization. Knocking down stap2b expression by morpholino injection or Crispr-Cas9-generated stap2b mutants caused vascular defects, suggesting a role played by stap2b in controlling the patterning of intersegmental vessels (ISVs) and the caudal vein plexus (CVP). The vessel abnormalities associated with stap2b deficiency were found to be due to dysregulated cell migration and proliferation. The decreased expression of vascular-specific markers in stap2b morphants was consistent with the vascular defects observed. In contrast, overexpression of stap2b enhanced the growth of ISVs and reversed the vessel defects in stap2b morphants. These data suggest that stap2b is necessary and sufficient to promote vascular development. Finally, we examined the interaction between stap2b and multiple signaling. We showed that stap2b regulated ISV growth through the JAK-STAT pathway. Moreover, we found that stap2b was regulated by Notch signaling to control ISV growth, and stap2b interacted with bone morphogenetic protein signaling to contribute to CVP formation. Altogether, we demonstrated that stap2b acts downstream of the isl2/nr2f1b pathway to play a pivotal role in vascular development via interaction with multiple signaling pathways.


Assuntos
Proteínas de Peixe-Zebra , Peixe-Zebra , Animais , Proteínas Adaptadoras de Transdução de Sinal/metabolismo , Janus Quinases/metabolismo , Neovascularização Fisiológica/genética , Transdução de Sinais/fisiologia , Fatores de Transcrição STAT/metabolismo , Peixe-Zebra/genética , Peixe-Zebra/metabolismo , Proteínas de Peixe-Zebra/metabolismo
9.
Am J Obstet Gynecol ; 2024 May 22.
Artigo em Inglês | MEDLINE | ID: mdl-38782230

RESUMO

Developments in preconception and prenatal technologies have led to undeniable advances in how health-care providers screen and treat patients. Despite these advances, at any point errors can occur leading to misdiagnosis or a missed diagnosis. In some instances, the missed information can lead to the birth of a child with health issues where short of the error, the decision to avoid conception or terminate the pregnancy might have been made. When these lapses unfold, there exists the potential for a wrongful birth or wrongful life lawsuit to ensue. While these 2 actions are based on the same set of events, they are distinct legal claims with varying degrees of judicial permissibility. Global legal acceptability of wrongful birth and life lawsuits tends to resemble patterns in the United States. Analyzing prior wrongful birth and wrongful life claims can reveal common trends in events leading to these types of lawsuits, as well as an understanding of their potential outcomes. A familiarity with wrongful birth and wrongful life lawsuits demonstrates how these cases are unique from other forms of prenatal or birth injury tort lawsuits and can provide insights to common shortcomings in clinical practice. Applying these lessons to clinical practice highlights key approaches towards limiting the risk of certain errors leading to wrongful birth and wrongful life lawsuits, with the goal of health-care providers offering high quality health care.

10.
Anal Bioanal Chem ; 416(14): 3389-3399, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38632130

RESUMO

As one of the most common iron-chelating agents, deferoxamine (DFO) rapidly chelates iron in the body. Moreover, it does not compete for the iron characteristic of hemoglobin in the blood cells, which is common in the clinical treatment of iron poisoning. Iron is a trace element necessary to maintain organism normal life activities. Iron deficiency can lead to anemia, whereas iron overload can cause elevated levels of cellular oxidative stress and cell damage. As a consequence, detection of the iron content in tissues and blood is of great significance. The traditional techniques for detecting the iron content include inductively coupled plasma-mass spectrometry and atomic absorption spectrometry, which cannot be used for imaging purposes. Laser ablation-ICP-MS and synchrotron radiation micro-X-ray fluorescence can map the concentration and distribution of iron in tissues. However, these methods can only be used to measure the total iron levels in blood or tissues. In recent years, due to the deepening understanding of iron metabolism, diseases related to iron overload have attracted increasing attention. Therefore, we took advantage of the properties of DFO in terms of chelating iron and investigated different sampling times following DFO injection in the tail vein of mice. We used mass spectrometry imaging (MSI) technology to detect the DFO and ferrioxamine content in the blood and different tissues to indirectly characterize the non-heme iron content.


Assuntos
Desferroxamina , Ferro , Espectrometria de Massas por Ionização e Dessorção a Laser Assistida por Matriz , Animais , Ferro/metabolismo , Ferro/análise , Camundongos , Espectrometria de Massas por Ionização e Dessorção a Laser Assistida por Matriz/métodos , Injeções Intravenosas , Quelantes de Ferro , Masculino , Distribuição Tecidual
11.
Pediatr Dev Pathol ; : 10935266241281797, 2024 Sep 26.
Artigo em Inglês | MEDLINE | ID: mdl-39324207

RESUMO

Casamassima-Morton-Nance syndrome (CMNS) is a rare disorder characterized by spondylocostal dysostosis (SCD), anal atresia, and urogenital anomalies. We describe a fetus with CMNS associated with a limb-body wall defect (LBWD), the second such case in the literature. We compare the phenotypic differences with previously reported cases, including those with segmentation anomalies of the axial skeleton, body wall defects, or absent/abnormal genitalia, revealing the consistent presence of SCD in CMNS. However, as expected, a wide phenotypic spectrum emerges, providing useful observations for fetal/neonatal screening relevant to differential diagnoses. Advanced diagnostic methods using imaging and next-generation skeletal dysplasia multi-gene panels are advisable, as they enable timely, actionable, well-informed decisions for parental counseling, potential elective termination of pregnancy, and prenatal and/or postnatal care. Most reported cases do not mention the recurrence of these usually lethal anomalies.

12.
BMC Pregnancy Childbirth ; 24(1): 105, 2024 Feb 02.
Artigo em Inglês | MEDLINE | ID: mdl-38308257

RESUMO

BACKGROUND: Although epidural anaesthesia and spinal anaesthesia are currently the general choices for patients undergoing caesarean section, these two neuraxial anaesthesia methods still have drawbacks. Caudal anaesthesia has been considered to be more appropriate for gynaecological surgery. The purpose of this study was to compare epidural anaesthesia combined with caudal anaesthesia, spinal anaesthesia and single-space epidural anaesthesia for caesarean section with respect to postoperative comfort and intraoperative anaesthesia quality. METHODS: In this clinical trial, 150 patients undergoing elective caesarean section were recruited and randomized into three groups according to a ratio of 1:1:1to receive epidural anaesthesia only, spinal anaesthesia only or epidural anaesthesia combined with caudal anaesthesia. The primary outcome was postoperative comfort in the three groups. Secondary outcomes included intraoperative anaesthesia quality and the incidences of nausea, vomiting, postdural puncture headache, maternal bradycardia, or hypotension. RESULTS: More patients were satisfied with the intraoperative anaesthesia quality in the EAC group than in the EA group (P = 0.001). The obstetrician was more significantly satisfied with the intraoperative anaesthesia quality in the SA and EAC groups than in the EA group (P = 0.004 and 0.020, respectively). The parturients felt more comfortable after surgery in the EA and EAC groups (P = 0.007). The incidence of maternal hypotension during caesarean section was higher in the SA group than in the EA and EAC groups (P = 0.001 and 0.019, respectively). CONCLUSIONS: Epidural anaesthesia combined with caudal anaesthesia may be a better choice for elective caesarean section. Compared with epidural anaesthesia and spinal anaesthesia, it has a higher quality of postoperative comfort and intraoperative anaesthesia.


Assuntos
Anestesia Caudal , Anestesia Epidural , Anestesia Obstétrica , Raquianestesia , Hipotensão , Humanos , Feminino , Gravidez , Cesárea/métodos , Anestesia Epidural/métodos , Raquianestesia/métodos , Hipotensão/epidemiologia , Hipotensão/etiologia , Ultrassonografia de Intervenção , Anestesia Obstétrica/métodos
13.
Gen Comp Endocrinol ; 349: 114468, 2024 04 01.
Artigo em Inglês | MEDLINE | ID: mdl-38325527

RESUMO

Adrenaline is one of the most important neurotransmitters in the central nervous system and is produced during stress. In this study, we investigated the modulatory role of adrenaline and adrenergic receptors on the neuroendocrine Dahlgren cells in the caudal neurosecretory system (CNSS) of olive flounder. Ex vivo electrophysiological recordings revealed that adrenaline significantly increased the firing frequency and altered the firing pattern of Dahlgren cells. Moreover, treatment with adrenaline led to a significant upregulation of ion channels and major hormone secretion genes in CNSS at the mRNA levels. Additionally, treatment with adrenaline resulted in a significantly elevation in the expression levels of α1- and ß3-adrenergic receptors. Furthermore, the ß3-adrenergic receptor antagonist exerts a significant inhibitory effect on adrenaline-induced enhancement firing activities of Dahlgren cells, whereas the α1-adrenergic receptor antagonist displays a comparatively weaker inhibitory effect. Additionally, the enhanced firing activity induced by adrenaline could be effectively suppressed by both α1- and ß3-adrenergic receptor antagonists. Taken together, these findings provide strong evidence in favor of the excitatory effects of adrenaline through α1 and ß3 adrenergic receptors in CNSS to stimulate the secretion of stress-related hormones, ß3-adrenergic receptor plays a more dominant role in the modulation of firing activities of Dahlgren cells by adrenaline and thereby regulates the stress response in olive flounder.


Assuntos
Epinefrina , Linguado , Animais , Epinefrina/farmacologia , Linguado/genética , Sistemas Neurossecretores/metabolismo , Receptores Adrenérgicos/metabolismo , Neurotransmissores/metabolismo
14.
Childs Nerv Syst ; 40(6): 1791-1797, 2024 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-38411707

RESUMO

INTRODUCTION: Sacral agenesis (SA) includes a range of clinical presentations of varying severity, with implications for function and quality of life (QoL). Diagnosis is often made perinatally, and prognostic discussions become an important aspect of parental counselling. This study engaged SA sufferers and their caregivers to obtain objective, long-term patient reported outcome data. METHOD: Patients with radiologically confirmed SA from a single tertiary spinal unit underwent retrospective medical record review. Patients were then contacted by telephone to complete QoL questionnaires including EQ-ED-5L for adults and EQ-ED-Y for < 16-year-olds. Additional information including Renshaw grade, employment, living situation and bladder function was also collected. RESULTS: Twenty-six patients with SA were identified. Mean age is 23.35 years (range 0.92-63.53), 13 M:17F. Renshaw grade ranged from 1 to 4. Sixty-eight percent had associated kyphoscoliotic deformities. The majority (70%) had either impaired or absent bladder control, and 80% need walking aids to mobilise. Twenty patients completed the questionnaire (10 adults and 10 < 16-year-olds). Mean EQ-ED-5L index for adults was +0.474 (range -0.1 to +0.089, 1 = best), with a lower mean value of +0.287 (range -0.54 to +1) for the < 16-year cohort. Those undergoing spinal fusion procedures had significantly lower scores (-0.08 v +0.44, p = 0.022). CONCLUSION: This study provides an objective record of the QoL of individuals with SA, illustrating a wide variety of outcomes, with differences between younger and older individuals which may reflect the results of a long-term adaptive process. The implications for individuals should be carefully tailored to the specific deformity and the likely underlying neurological deficits.


Assuntos
Qualidade de Vida , Humanos , Feminino , Masculino , Adolescente , Adulto , Adulto Jovem , Criança , Pré-Escolar , Pessoa de Meia-Idade , Estudos Retrospectivos , Lactente , Sacro/anormalidades , Inquéritos e Questionários , Resultado do Tratamento , Anormalidades Múltiplas , Meningocele , Região Sacrococcígea/anormalidades
15.
Childs Nerv Syst ; 40(5): 1415-1425, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38441629

RESUMO

PURPOSE: To analyze the relationship between spinal cord and vertebral abnormalities from the point of view of embryology. METHODS: We analyzed the clinical and radiological data of 260 children with different types of spinal cord malformations in combination with vertebral abnormalities. RESULTS: Among 260 individuals, approximately 109 presented with open neural tube defects (ONTDs), 83 with split cord malformations (SCMs), and 83 with different types of spinal lipomas. Pathological spina bifida emerged as the most frequent vertebral anomaly, affecting 232 patients, with a higher prevalence in ONTD. Vertebral segmentation disorders, including unsegmented bars, butterfly vertebrae, and hemivertebrae, were present in 124 cases, with a higher prevalence in SCM. The third most common spinal anomaly group consisted of various forms of sacral agenesis (58 cases), notably associated with blunt conus medullaris, spinal lipomas, and sacral myelomeningocele. Segmental aplasia of the spinal cord had a typical association with segmental spinal absence (N = 17). CONCLUSION: The association between SCM and neuroenteric cyst/canal and vertebral segmentation disorders is strong. High ONTDs often coincide with pathological spina bifida posterior. Type 1 spinal lipomas and focal spinal nondisjunction also correlate with pathologic spina bifida. Segmental spinal absence or dysgenesis involves localized spinal and spinal cord aplasia, sometimes with secondary filar lipoma.


Assuntos
Anormalidades Múltiplas , Hérnia Diafragmática , Lipoma , Meningocele , Defeitos do Tubo Neural , Disrafismo Espinal , Criança , Humanos , Coluna Vertebral/anormalidades , Medula Espinal/patologia , Meningocele/patologia , Defeitos do Tubo Neural/patologia , Imageamento por Ressonância Magnética
16.
Childs Nerv Syst ; 40(3): 715-728, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-37924337

RESUMO

OBJECTIVE: Conus region lumbosacral lipomas (LSLs) are highly heterogeneous in their morphology, clinical presentation, and outcome, with an incompletely understood natural history and often treacherous surgical anatomy. This systematic review aims to critically evaluate and assess the strength of the current LSL evidence base to guide management strategies. METHODS: According to a systematic review following PRISMA guidelines, a search was conducted using the key term "lumbosacral lipoma" across MEDLINE (OVID), Embase, Cochrane Library, and PubMed databases from January 1951 to April 2021. All studies containing ten or more paediatric conus lipomas were included. Data heterogeneity and bias were assessed. RESULTS: A total of 13 studies were included, containing 913 LSLs (predominantly transitional type-58.5%). Two-thirds (67.5%) of all patients (treated and non-treated) remained clinically stable and 17.6% deteriorated. Neuropathic bladder was present in 8.6% at final follow-up. Of patients managed surgically, near-total resection vs. subtotal resection deterioration-free survival rates were 77.2-98.4% and 10-67% respectively. 4.5% (0.0-27.3%) required re-do untethering surgery. Outcomes varied according to lipoma type. Most publications contained heterogeneous populations and used variable terminology. There was a lack of consistency in reported outcomes. CONCLUSION: Amongst published series, there is wide variability in patient factors such as lipoma type, patient age, and methods of (particularly urological) assessment. Currently, there is insufficient evidence base upon which to make clear recommendations for the management of children with LSL. There is an imperative for neurosurgeons, neuroradiologists, and urologists to collaborate to better standardise the terminology, assessment tools, and surgical interventions for this challenging group of conditions.


Assuntos
Lipoma , Neoplasias da Medula Espinal , Neoplasias da Coluna Vertebral , Criança , Humanos , Lactente , Neoplasias da Medula Espinal/cirurgia , Região Lombossacral/cirurgia , Imageamento por Ressonância Magnética/métodos , Neoplasias da Coluna Vertebral/cirurgia , Lipoma/cirurgia , Resultado do Tratamento
17.
Eur Spine J ; 33(4): 1675-1682, 2024 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-38459986

RESUMO

OBJECTIVE: Postoperative progressive coronal caudal curve (PCC) was characterized by a postoperative de novo caudal S-curve ≥ 20° following congenital cervicothoracic scoliosis (CTS) corrective osteotomies, and at least 20° greater than the preoperative measurement, while the incidence was uncertain and the pathogenesis was equivocal. The objective of this study was to investigate the morbidity and potential factors contributing to PCC following CTS surgery. METHODS: This study reviewed 72 CTS patients between 2005 and 2021. Patients were categorized into two groups according to the absence or presence of PCC at last follow-up, namely the nonprogressive curve group (NPC-group) and the progressive curve group (PC-group). Demographics, radiographic data and the Scoliosis Research Society-22 (SRS-22) questionnaire results were reviewed. Multivariate linear regression analyses were utilized to determine possible predictors for PCC. RESULTS: PCC was observed in 11 (15%) of the total 72 patients. Compared with the NPC-group, the PC-group exhibited greater postoperative residual local curve (24.0 ± 9.7° vs. 9.1 ± 4.4°, P < 0.001), upper instrumented vertebra (UIV) tilt (16.9 ± 7.4° vs. 6.2 ± 3.7°, P < 0.001), T1 tilt (14.3 ± 9.4° vs. 6.6 ± 3.9°, P = 0.022) and neck tilt (10.1 ± 6.7° vs. 3.7 ± 2.5, P = 0.009). The multivariable linear regression demonstrated that the larger postoperative UIV tilt, residual local curve and neck tilt were associated with PCC. In addition, patients with PCC showed lower SRS-22 scores in terms of pain, mental health, self-image and satisfaction (P < 0.05). CONCLUSIONS: The morbidity of PCC was 15% in CTS patients who underwent corrective osteotomies. Greater residual local curve, postoperative UIV tilt and neck tilt were identified as predictors for PCC.


Assuntos
Escoliose , Fusão Vertebral , Humanos , Escoliose/diagnóstico por imagem , Escoliose/cirurgia , Incidência , Fusão Vertebral/métodos , Estudos Retrospectivos , Vértebras Torácicas/diagnóstico por imagem , Vértebras Torácicas/cirurgia , Osteotomia/métodos , Seguimentos , Resultado do Tratamento
18.
Paediatr Anaesth ; 34(4): 293-303, 2024 04.
Artigo em Inglês | MEDLINE | ID: mdl-38146668

RESUMO

BACKGROUND AND OBJECTIVES: The aim of this narrative review is to evaluate the literature describing the use of caudal anesthetic-based techniques in premature and ex-premature infants undergoing lower abdominal surgery. METHODS: All available literature from inception to August 2023 was retrieved according to Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines from Medline, PubMed, Embase, and the Cochrane Library. Two authors reviewed all references for eligibility, abstracted data, and appraised quality. RESULTS: Of the 211 articles identified, 45 met our inclusion criteria yielding 1548 cases with awake caudal anesthesia. The review included 558 (36.0%) cases of awake caudal anesthesia, 837 cases (54.1%) of "awake" caudal anesthesia with sedation, and 153 cases (9.9%) of combined spinal caudal epidural anesthesia without sedation. The overall anesthetic failure rate was 7.2% (71.9:1000 caudals). Failure rates were highest for CSEA (13.7%, 7.7-18.4), intermediate for awake caudal (6.6%, 5.26-9.51), and lowest for sedated caudal anesthesia (5.85%, 4.48-7.82). The incidence (range) of perioperative apnea was highest for sedated caudal anesthesia (8.16, 0%-24%), intermediate for awake caudal (7.62%, 0%-60%), and lowest for CSEA (5.53%, 0%-14.3%). High spinal anesthesia occurred in 0.84%, or 8.35:1000 caudals overall. The incidence was highest in awake caudal anesthesia cases (1.97% or 19.7:1000 caudals), intermediate with caudal with sedation (1.07% or 10.7:1000 caudals), and lowest in CSEA (0.7% or 6.6:1000 caudals). Our review was confounded by incomplete data reporting and small sample sizes as most were case reports. There were no high-quality randomized controlled trials, and the eight single-center retrospective data reviews lacked sufficient data to perform meta-analysis. CONCLUSIONS: There is insufficient evidence to validate or refute the benefits of the use of "awake" caudal anesthesia in premature and ex-premature infants. The high doses of local anesthetics used, the high failure rate, and the increased incidence of high spinal anesthesia would suggest that the techniques offer no real advantages over awake spinal anesthesia or general anesthesia with a regional block.


Assuntos
Anestesia Caudal , Anestesia por Condução , Humanos , Lactente , Recém-Nascido , Anestesia Caudal/métodos , Anestesia por Condução/métodos , Anestésicos Locais , Recém-Nascido Prematuro , Vigília
19.
World J Surg Oncol ; 22(1): 187, 2024 Jul 22.
Artigo em Inglês | MEDLINE | ID: mdl-39039538

RESUMO

BACKGROUND: The cranial-caudal-medial approach (CCMA) has been proposed for laparoscopic right hemicolectomy nowadays. This study aimed to investigate the safety and oncological efficacy of CCMA in the treatment of right-sided colon cancer compared to the medial-lateral approach (MLA). METHODS: Patients diagnosed with right-sided colon cancer were included from February 2015 to June 2018, retrospectively, dividing into the CCMA group and the MLA group. We compared the basic characteristics and the short-term and long-term outcomes in two groups. RESULTS: Two hundred and ninety-six patients were included in this study. The baseline characteristics were similar in two groups. Compared with MLA group, CCMA group exhibited shorter operation time (136.3 ± 25.3 min vs. 151.6 ± 21.5 min, P < 0.001), lower estimated blood loss (44.1 ± 15.2 ml vs. 51.4 ± 26.9 min, P = 0.010), and more harvested lymph nodes (18.5 ± 7.1 vs. 16.5 ± 5.7, P = 0.021). The 5-year overall survival (OS) rate for the CCMA group was 76.5%, and the 5-year disease-free survival (DFS) rate was 72.3%, both of which were not inferior to the MLA group. No significant difference was found between two groups in terms of other clinical parameters. CONCLUSION: The CCMA in laparoscopic right hemicolectomy is safe and feasible, making the anatomical plane clearer. This approach can shorten the operation time, reduce intraoperative blood loss, harvest more lymph nodes, and yield satisfactory oncological outcomes.


Assuntos
Colectomia , Neoplasias do Colo , Laparoscopia , Pontuação de Propensão , Humanos , Colectomia/métodos , Feminino , Masculino , Laparoscopia/métodos , Estudos Retrospectivos , Neoplasias do Colo/cirurgia , Neoplasias do Colo/patologia , Pessoa de Meia-Idade , Taxa de Sobrevida , Seguimentos , Idoso , Duração da Cirurgia , Prognóstico
20.
Pediatr Radiol ; 54(9): 1451-1461, 2024 08.
Artigo em Inglês | MEDLINE | ID: mdl-38750326

RESUMO

Caudal regression syndrome is a form of segmental spinal dysgenesis involving the caudal spinal column, ranging from segmental coccygeal agenesis to extensive thoracolumbar agenesis with varying degrees of spinal cord dysgenesis. A majority of caudal regression cases are sporadic but maternal pre-gestational diabetes mellitus is an important risk factor. Imaging is an integral part of management of caudal regression syndrome. Antenatal diagnosis on obstetric ultrasound and evaluation with fetal MRI is ideal. Early postnatal diagnosis and/or detailed evaluation with MRI is essential for early management to improve outcomes. Pang classification categorizes caudal regression syndrome into two categories based on the position of the conus while Renshaw classification is based on the degree of vertebral column agenesis. Caudal regression syndrome may be associated with several additional anomalies, both spinal and extraspinal. A number of genitourinary and gastrointestinal anomalies have been described in association with caudal regression syndrome. The field of view of MRI of the lumbosacral spine in caudal regression syndrome needs to be extended to visualize the retroperitoneal structures without the use of a saturation band. Syndromic associations may be suspected, and additional imaging performed, based on findings of extended field of view MRI of the spine. Associated sacral masses and filar abnormalities need to be identified and may also require surgical treatment. The multisystem nature of this disease necessitates a multimodality approach to the evaluation and management of caudal regression syndrome with close cooperation between pediatric neuroradiologists and body radiologists as well as multiple clinical teams. Appropriate early management with surgical correction as necessary can significantly improve prognosis and survival in caudal regression syndrome.


Assuntos
Imageamento por Ressonância Magnética , Humanos , Recém-Nascido , Síndrome , Imageamento por Ressonância Magnética/métodos , Feminino , Anormalidades Múltiplas/diagnóstico por imagem , Diagnóstico Pré-Natal/métodos
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