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1.
Emerg Radiol ; 31(4): 613-617, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38797776

RESUMO

BACKGROUND: Splenic sequestration crisis is a potentially fatal complication of sickle cell disease, mainly seen in young children. Only a few case series describe the acute splenic sequestration crisis in adults and its management, which primarily consists of supportive care and, in some cases, splenectomy. Splenic artery embolization has seldom been described in sickle cell disease. This is probably the first case in which an adult with sickle cell disease presented with an acute splenic sequestration crisis was managed successfully through splenic artery embolization. RESULTS: This 22-year-old female, a known case of sickle cell disease, presented with severe pain in the abdomen and low-grade intermittent fever for two days, secondary to an acute splenic sequestration crisis. The diagnosis of acute splenic sequestration was made based on clinical and blood parameters, ultrasonography, and computed tomography. Even with adequate supportive care and blood transfusions, the patient's condition worsened with a rapid fall in the hemoglobin and total platelet count. Considering splenectomy to be a high-risk procedure for this patient, a decision of rescue splenic artery embolization was taken, which was successful. CONCLUSION: Splenic artery embolization may be considered a lifesaving procedure in patients with acute splenic sequestration, where the risk of splenectomy can be high. Adequate post-procedure supportive care is vital for preventing complications.


Assuntos
Anemia Falciforme , Embolização Terapêutica , Artéria Esplênica , Humanos , Embolização Terapêutica/métodos , Feminino , Anemia Falciforme/complicações , Anemia Falciforme/terapia , Artéria Esplênica/diagnóstico por imagem , Adulto Jovem , Esplenopatias/diagnóstico por imagem , Esplenopatias/terapia , Esplenopatias/etiologia , Doença Aguda , Tomografia Computadorizada por Raios X
2.
Neuroophthalmology ; 48(4): 240-248, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38933744

RESUMO

We wanted to evaluate if optical coherence tomography angiography OCTA findings could predict the functional outcome in extracranial carotid artery atherosclerotic disease (ECAD) associated stroke. This exploratory study was performed on adults with acute ischaemic stroke due to ECAD at 3-6 weeks following stroke onset with risk factor matched controls without carotid artery stenosis. Twenty-three stroke patients (cases) and 23 controls were enrolled. There was significant difference between cases and controls in deep vessel density at the macula (p = .0007) and in radial peripapillary capillary perfusion density (RPCPD) at the optic nerve head (ONH) (p = .0007). Statistically significant difference was noted in the total superficial vessel density (SVD) at the macula (SVD within 1 standard deviation [SD] versus SVD beyond 1 SD of control data) in the ipsilateral eye and functional outcome at 3 months (poor versus very good outcome, modified Rankin scale [mRS] 0-1 versus mRS 2-6, respectively; p = .0361). There was statistically insignificant correlation between the RPCPD at the ONH and the National Institutes of Health Stroke Scale score at admission, mRS at discharge, and mRS at 3 months following stroke onset (r = .33, r = .35, r = .39; p = .11, p = .09, p = .06, respectively). The findings of this exploratory study suggested that OCTA findings may predict 3 month outcomes in cases of ECAD-related stroke and could be useful in decision making in future intervention studies as to whether intervene or not in patients having critical or non-critical ECAD for preventing stroke.

3.
J Food Sci Technol ; 61(8): 1492-1502, 2024 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-38966786

RESUMO

In this study, the impact of formulated emulsion was studied on strawberries which were coated using dip and electrostatic spray coating methods. The coated strawberries were kept at room temperature for a period of 12 days. A significant level of chargeability of w/o emulsion was achieved in terms of charge-to-mass ratio of 2.81 mC/kg at an applied high voltage of 2.0 kV, applied air pressure of 0.3 MPa, and liquid flow rate of 33.6 ml/min. The distance of 170 mm from the nozzle tip to Faraday cage was maintained during the measurements. As compared to uncoated and dip coated strawberries, the water-in-oil based electrostatically charged sprays considerably (p < 0.05) reduced the weight loss, decay rate, pH, titrable acidity, TSS, and antioxidant activity. In both the cases, i.e. strawberries coated with dip and electrostatic spray coating methods, the same weight loss was observed, however, there was a considerably less weight loss as compared to uncoated samples. The textures of the uncoated (9.02 N) and dip coated (12.58 N) samples were significantly different from the electrostatic spray coated (15.85 N) samples. Since, the coating formulation had no impact on the sensory attributes, the samples were considered as acceptable at the end of the storage. Furthermore, compared to uncoated, water-in-oil based electrostatically charged spray coating was more effective at delaying the decay by 12 days.

4.
Environ Res ; 233: 116496, 2023 09 15.
Artigo em Inglês | MEDLINE | ID: mdl-37380008

RESUMO

The elimination of pathogenic bacteria from water sources is currently crucial for obtaining drinkable water. Therefore, the development of platforms with the ability to interact with pathogens and remove them is a potential future tool for medicine, food and water safety. In this work, we have grafted a layer of NH2-MIL-125 (Ti) on Fe3O4@SiO2 magnetic nanospheres for the removal of multiple pathogenic bacteria from water. The synthesized Fe3O4@SiO2@NH2-MIL-125 (Ti) nano adsorbent was characterized by FE-SEM, HR-TEM, FT-IR, XRD, BET surface analysis, magnetization tests, respectively, which illustrated its well-defined core-shell structure and magnetic behaviour. The prepared magnetic-MOF composite sorbent was attractive towards capturing a wide range of pathogens (S. typhimurium, S. aureus, E. coli, P. aeruginosa and K. pneumoniae) under experimental conditions. Influence factors such as adsorbent dosage, bacterial concentration, pH and incubation time were optimized for enhanced bacterial capture. The application of an external magnetic field removed Fe3O4@SiO2@NH2-MIL-125 (Ti) nano adsorbent from the solution along with sweeping the attached pathogenic bacteria. The non-specific removal efficiency of S. typhimurium for magnetic MOF composite was 96.58%, while it was only 46.81% with Fe3O4@SiO2 particles. For specific removal, 97.58% of S. typhimurium could be removed selectively from a mixture with monoclonal anti- Salmonella antibody conjugated magnetic MOF at a lower concentration of 1.0 mg/mL. The developed nano adsorbent may find great potential in microbiology applications and water remediation.


Assuntos
Dióxido de Silício , Titânio , Espectroscopia de Infravermelho com Transformada de Fourier , Escherichia coli , Staphylococcus aureus , Adsorção , Bactérias , Água , Fenômenos Magnéticos
5.
BMC Genomics ; 22(1): 65, 2021 Jan 20.
Artigo em Inglês | MEDLINE | ID: mdl-33472593

RESUMO

BACKGROUND: The lesser grain borer, Rhyzopertha dominica is a serious pest of stored grains. Fumigation and contact insecticides play a major role in managing this pest globally. While insects are developing genetic resistance to chemicals, hormonal analogues such as s-methoprene play a key role in reducing general pest pressure as well as managing pest populations that are resistant to fumigants and neurotoxic contact insecticides. However, resistance to s-methoprene has been reported in R. dominica with some reports showing a remarkable high resistance, questioning the use of this compound and other related analogues in grain protection. The current study attempts to identify possible molecular mechanisms that contribute in resistance to s-methoprene in R. dominica. RESULTS: Transcriptome analysis of resistant and susceptible strains of this pest species identified a set of differentially expressed genes related to cytochrome P450s, indicating their potential role in resistance to s-methoprene. Laboratory bioassays were performed with s-methoprene treated wheat grains in presence and absence of piperonyl butoxide (PBO), a cytochrome P450 inhibitor. The results indicate that PBO, when applied alone, at least at the concentration tested here, had no effect on R. dominica adult emergence, but has a clear synergistic effect to s-methoprene. The number of produced progeny decreased in presence of the inhibitor, especially in the resistant strain. In addition, we also identified CYP complement (CYPome) of R. dominica, annotated and analysed phylogenetically, to understand the evolutionary relationships with other species. CONCLUSIONS: The information generated in current study suggest that PBO can effectively be used to break resistance to s-methoprene in R. dominica.


Assuntos
Besouros , Inseticidas , Animais , Besouros/genética , Dominica , Perfilação da Expressão Gênica , Inseticidas/farmacologia , Metoprene , Butóxido de Piperonila/farmacologia , Transcriptoma
6.
Pestic Biochem Physiol ; 171: 104717, 2021 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-33357567

RESUMO

The rusty grain beetle, Cryptolestes ferrugineus, a major pest of stored commodities, has developed very high levels (>1000×) of resistance to the fumigant phosphine. Resistance in this species is remarkably stronger than reported in any other stored product pests demanding the need to understand the molecular basis of this trait. Previous genetic studies in other grain insect pests identified specific variants in two major genes, rph1 and rph2 in conferring the strong resistance trait. However, in C. ferrugineus, although the gene, rph1 was identified as cytochrome-b5-fatty acid desaturase, the rph2 gene has not been reported so far. We tested the candidate gene for rph2, dihydrolipoamide dehydrogenase (dld) using the recently published transcriptome of C. ferrugineus and identified three variants, L73N and A355G + D360H, a haplotype, conferring resistance in this species. Our sequence analysis in resistant strain and phosphine selected resistant survivors indicates that these variants occur either alone as a homozygote or a mixture of heterozygotes (i.e complex heterozygotes) both conferring strong resistance. We also found that one of the three variants, possibly L73N expressing "dominant" trait at low frequency in resistant insects. Comparison of dld sequences between Australian and Chinese resistant strain of this species confirmed that the identified variants are highly conserved. Our fitness analysis indicated that resistant insects may not incur significant biological costs in the absence of phosphine selection for 19 generations. Thus, we propose that the observed high levels of resistance in C. ferrugineus could be primarily due to the characteristics of three unique variants, L73N and A355G + D360H within dld.


Assuntos
Besouros , Inseticidas , Fosfinas , Animais , Austrália , Besouros/genética , Di-Hidrolipoamida Desidrogenase/genética , Resistência a Inseticidas/genética , Inseticidas/farmacologia , Fosfinas/farmacologia
7.
Annu Rev Entomol ; 65: 333-350, 2020 01 07.
Artigo em Inglês | MEDLINE | ID: mdl-31610132

RESUMO

Development of resistance in major grain insect pest species to the key fumigant phosphine (hydrogen phosphide) across the globe has put the viability and sustainability of phosphine in jeopardy. The resistance problem has been aggravated over the past two decades, due mostly to the lack of suitable alternatives matching the major attributes of phosphine, including its low price, ease of application, proven effectiveness against a broad pest spectrum, compatibility with most storage conditions, and international acceptance as a residue-free treatment. In this review, we critically analyze the published literature in the area of phosphine resistance with special emphasis on the methods available for detection of resistance, the genetic basis of resistance development, key management strategies, and research gaps that need to be addressed.


Assuntos
Insetos/genética , Inseticidas , Fosfinas , Animais , Parasitologia de Alimentos , Fumigação , Controle de Insetos , Resistência a Inseticidas/genética
9.
J Food Sci Technol ; 54(6): 1538-1544, 2017 May.
Artigo em Inglês | MEDLINE | ID: mdl-28559613

RESUMO

This paper reports the prediction capacity of various microbial inactivation models to prefigure the bactericidal effect using pulsed electric field (PEF) on liquid food. The aim of study was to compare the various inactivation models based on accuracy and bias factor to find out the most accurate inactivation model for Escherichia coli present in carrot juice treated with PEF. In this study, E. coli suspended in carrot juice was treated with varying pulsed electric field strength for different intervals. The obtained data were utilized for the evaluation of parameters of Bigelow, Peleg, Hülsheger and Weibull inactivation models. Furthermore, secondary models were developed for Hülsheger and Weibull to predict the microbial inactivation at any level of field strength and treatment time. The secondary model for Hülsheger exhibits 5.8% error as compared to the Weibull model having 8.5% error in prediction of death kinetics of E. coli suspended in carrot juice by means of PEF. The comparative analysis of secondary models to forecast the unknown data set unveiled the superior functioning of Hülsheger model.

10.
Pestic Biochem Physiol ; 127: 67-75, 2016 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-26821660

RESUMO

Inheritance of resistance to phosphine fumigant was investigated in three field-collected strains of rusty grain beetle, Cryptolestes ferrugineus, Susceptible (S-strain), Weakly Resistant (Weak-R) and Strongly Resistant (Strong-R). The strains were purified for susceptibility, weak resistance and strong resistance to phosphine, respectively, to ensure homozygosity of resistance genotype. Crosses were established between S-strain×Weak-R, S-strain×Strong-R and Weak-R×Strong-R, and the dose mortality responses to phosphine of these strains and their F1, F2 and F1-backcross progeny were obtained. The fumigations were undertaken at 25°C and 55% RH for 72h. Weak-R and Strong-R showed resistance factors of 6.3× and 505× compared with S-strain at the LC50. Both weak and strong resistances were expressed as incompletely recessive with degrees of dominance of -0.48 and -0.43 at the LC50, respectively. Responses of F2 and F1-backcross progeny indicated the existence of one major gene in Weak-R, and at least two major genes in Strong-R, one of which was allelic with the major factor in Weak-R. Phenotypic variance analyses also estimated that the number of independently segregating genes conferring weak resistance was 1 (nE=0.89) whereas there were two genes controlling strong resistance (nE=1.2). The second gene, unique to Strong-R, interacted synergistically with the first gene to confer a very high level of resistance (~80×). Neither of the two major resistance genes was sex linked. Despite the similarity of the genetics of resistance to that previously observed in other pest species, a significant proportion (~15 to 30%) of F1 individuals survived at phosphine concentrations higher than predicted. Thus it is likely that additional dominant heritable factors, present in some individuals in the population, also influenced the resistance phenotype. Our results will help in understanding the process of selection for phosphine resistance in the field which will inform resistance management strategies. In addition, this information will provide a basis for the identification of the resistance genes.


Assuntos
Besouros/efeitos dos fármacos , Inseticidas/farmacologia , Fosfinas/farmacologia , Animais , Besouros/genética , Heterozigoto
11.
Annu Rev Entomol ; 59: 279-97, 2014.
Artigo em Inglês | MEDLINE | ID: mdl-24160430

RESUMO

Previously regarded as minor nuisance pests, psocids belonging to the genus Liposcelis now pose a major problem for the effective protection of stored products worldwide. Here we examine the apparent biological and operational reasons behind this phenomenon and why conventional pest management seems to be failing. We investigate what is known about the biology, behavior, and population dynamics of major pest species to ascertain their strengths, and perhaps find weaknesses, as a basis for a rational pest management strategy. We outline the contribution of molecular techniques to clarifying species identification and understanding genetic diversity. We discuss progress in sampling and trapping and our comprehension of spatial distribution of these pests as a foundation for developing management strategies. The effectiveness of various chemical treatments and the availability and potential of nonchemical control methods are critically examined. Finally, we identify research gaps and suggest future directions for research.


Assuntos
Controle de Insetos , Insetos , Inseticidas , Animais , Controle de Insetos/métodos , Insetos/efeitos dos fármacos , Insetos/microbiologia , Insetos/fisiologia , Resistência a Inseticidas , Inseticidas/farmacologia
12.
Nanoscale ; 16(26): 12523-12533, 2024 Jul 04.
Artigo em Inglês | MEDLINE | ID: mdl-38888214

RESUMO

The bioaccumulation of various heavy metals in the environment and agriculture is posing serious hazards to human health. Hexavalent chromium is one of the most encountered heavy metal pollutants. The routine monitoring of Cr(VI) via simple methods assumes great analytical significance in sectors like environmental safety, food quality, etc. This study reports a novel biocompatible and luminescent metal-organic framework (ascorbic acid functionalized Bio-MOF-1) based "Turn-on" nanoprobe for rapid and sensitive optical detection of Cr(VI). Bio-MOF-1 has been synthesized, functionalized with ascorbic acid (AA), and then comprehensively characterized for its key material properties. The presence of Cr(VI) results in the photoluminescence recovery of Bio-MOF-1/AA. Using the above approach, Cr(VI) is detected over a wide concentration range of 0.02 to 20 ng mL-1, with the limit of detection being 0.01 ng mL-1. The nanoprobe is capable of detecting Cr(VI) in real water as well as in some spiked food samples. Hence, the ascorbic acid functionalized Bio-MOF-1 nanoprobe is established as a potential on-field detection tool for Cr(VI).

13.
Cureus ; 16(5): e60887, 2024 May.
Artigo em Inglês | MEDLINE | ID: mdl-38910704

RESUMO

Moebius syndrome is a rare disease characterized by unilateral or bilateral facial nerve palsies with/without other cranial nerve palsy. It manifests clinically with facial muscle weakness and/or ophthalmoplegia and can be associated with other physical anomalies such as various limb deformities and orofacial malformation. Herein, we have described the clinical and radiological features of Moebius syndrome in a 9-year-old female child who presented with left-side facial palsy and bilateral complete horizontal gaze palsy.

14.
J Neurosci Rural Pract ; 15(2): 381-383, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38746493

RESUMO

Simultaneous occlusion of both middle cerebral arteries (MCAs) is very rare and usually devastating. Few case reports are available in the literature where bilateral thrombectomy was done simultaneously to remove the clot. High NIH stroke scale with a low level of consciousness can be a clue for the diagnosis. Timely intervention is necessary to decrease morbidity and mortality in these patients. We also reviewed the existing literature where mechanical thrombectomies were done for bilateral MCA stroke in PubMed, Google Scholar, Cochrane, and Embase. Herein, we report a case of 47-year-old female having rheumatic heart disease presented with simultaneous bilateral MCAs occlusion, treated with mechanical thrombectomies successfully.

15.
Cureus ; 16(7): e64209, 2024 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-39130918

RESUMO

Uterine arteriovenous malformations (AVM) are rare and usually present in women of reproductive age. Clinical presentation may overlap with early pregnancy, retained products of conception (RPOC), or gestational trophoblastic disease (GTD) if it occurs in a pregnant patient or the immediate postpartum period and becomes challenging to manage. Here, we present two cases of uterine AVM that presented with vaginal bleeding after miscarriages. In these cases, the presentation was vaginal bleeding with raised serum beta-human chorionic gonadotropin (ß-hCG) levels. The uterine AVM was diagnosed with ultrasound and contrast-enhanced CT and subsequently managed with uterine artery embolization. Although rare, uterine AVM should be kept in the differentials in a premenopausal patient with abnormal vaginal bleeding and positive serum ß-hCG levels. It should be differentiated from other common causes of vaginal bleeding with raised serum ß-hCG levels, such as early pregnancy, GTD, and RPOC, as early diagnosis and proper treatment are crucial for favorable outcomes.

16.
Artigo em Inglês | MEDLINE | ID: mdl-39110286

RESUMO

Insect growth regulators (IGRs) have been playing a major role in the effective management of a range of stored product insect pests including species that have developed resistance to major groups of insecticides, such as organophosphates (OPs) and synthetic pyrethroids (SPs). In the present study, we evaluated the efficacy of S-methoprene alone and in combination with piperonyl butoxide (PBO), an adjuvant component of insecticides for synergy, against two strains, Lab-S (susceptible) and Met-R (Methoprene resistant) of an economically important pest species, the lesser grain borer, Rhyzopertha dominica (F.) (Coleoptera: Bostrychidae). Adults of both Lab-S and Met-R strains were exposed to wheat treated with multiple concentrations of S-methoprene ranging from 0.001 to 0.01 and 10 to 60 mg/kg, respectively, alone and in combination with PBO. A variety of concentrations, including 0.27, 0.53, 0.80, and 1.07 g/kg, were evaluated for PBO. Mortality of adults and percent reduction in progeny were assessed after 14 and 65 days of treatment, respectively. As anticipated, the adult mortality rates of both strains were not significantly affected by S-methoprene alone. However, the number of progeny produced confirmed that the Met-R strain exhibited a high level of resistance to S-methoprene alone, with over 50 F1 progeny adults surviving in wheat treated with the maximal rate, 10 mg/kg. In contrast, the toxicity of S-methoprene was increased against the same resistant strain (Met-R), by 0.80 or 1.07 g/kg of PBO in combination treatment, resulting in a significant reduction in progeny numbers (25 adults per container). Although the tested concentrations of S-methoprene and PBO were well above the currently registered rate globally, our results highlight the fact that PBO enhances the toxicity of S-methoprene to some extent, reaffirming that the mode of action of the latter involves the inhibition of mixed-function oxidases (MFOs) and highlights the need for further research into developing potential binary or triplet formulations containing these two active ingredients (AIs).

17.
Cureus ; 16(1): e52370, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38361698

RESUMO

Background Moyamoya disease (MMD) can be a major cause of hemorrhagic stroke. Though extensive angiographic studies have been undertaken, the understanding of the association between aneurysms and MMD remains unanswered. In this study, we explore the association of the aneurysm with MMD and its management. We have also reviewed such associations described in the literature and how the present cases differ from those previously described. Materials and methods The clinical and radiologic data of moyamoya disease cases were accessed from medical and radiological records between January 2010 and July 2017. Two neuroradiologists independently analyzed the data and imaging details. Results Out of 103 patients with MMD, eight patients (7.77%) had associated intracranial aneurysms with eleven aneurysms. Out of the 11 aneurysms, five were the tip of the basilar artery aneurysms and were the most common location for aneurysm (45.5%), followed by lenticulostriate artery, PCA perforator, and distal ACA (DACA) in the P1 PCA, P2 PCA, and P3 PCA artery aneurysms. Out of eight patients, five (62.5%) had a hemorrhage on a non-contrast computed tomography (NCCT) scan of the brain, whereas three (37.5%) had an ischemic presentation. Out of 11 aneurysms, seven aneurysms, including three basilar tip aneurysms (unruptured) and one PCA perforator (ruptured), and three saccular PCA (P1, P2, and P3) (ruptured) were treated by endovascular coiling. Follow-up angiography showed stable aneurysmal occlusion except in one basilar tip, where recurrence was observed. Conclusions MMD-intracranial aneurysm is commonly observed in patients with intracranial hemorrhage and carries a higher risk of rupture. Therefore, identification of the aneurysm is essential for management. Endovascular treatment, either with coil or glue embolization, can be a safe and effective treatment method for such aneurysms with long-term good results.

18.
J Neurosci Rural Pract ; 15(1): 126-129, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38476428

RESUMO

Fenestrated aneurysm at vertebrobasilar junction (VBJ) is very rare and can occur due to non-fusion of longitudinal neural axis forming basilar artery in the early stage of embryonic life. Due to defects in tunica media and weakness in its wall, these fenestrations are more likely to develop an aneurysm. Various treatment strategies are required for the management of these types of aneurysms including simple coiling, stent-assisted coiling, balloon remodeling technique, and more recently kissing flow diverters. Herein, we report the case of ruptured fenestrated VBJ aneurysm which was managed successfully with novel reverse Y stenting with coiling.

19.
BMJ Case Rep ; 17(3)2024 Mar 15.
Artigo em Inglês | MEDLINE | ID: mdl-38490704

RESUMO

A term neonate with history of ventriculomegaly in the fetal period was diagnosed with a central nervous system tumour after radiological investigations. It was confirmed as an immature teratoma after histopathological examination. He underwent left frontal craniotomy with tumour excision. Intraoperatively, massive haemorrhage (venous bleed) occurred due to the high vascularity of the tumour and led to haemodynamic instability. A massive transfusion protocol was initiated. Despite multiple transfusions and shock management, he succumbed at 2 weeks of life. This case report highlights the importance of antenatal diagnosis and fetal MRI in prognostication and also the possible role of neoadjuvant chemotherapy in reducing tumour vascularity and, hence, bleeding.


Assuntos
Neoplasias Encefálicas , Hidrocefalia , Teratoma , Masculino , Recém-Nascido , Humanos , Gravidez , Feminino , Neoplasias Encefálicas/diagnóstico por imagem , Neoplasias Encefálicas/cirurgia , Teratoma/diagnóstico por imagem , Teratoma/cirurgia , Diagnóstico Pré-Natal , Feto/patologia
20.
J Clin Imaging Sci ; 14: 3, 2024.
Artigo em Inglês | MEDLINE | ID: mdl-38469176

RESUMO

Objectives: Neural crest cells (NCCs) are transient structures in the fetal life in vertebrates, which develop at the junctional site of the non-neural and neural ectoderm, sharing a common developmental origin for diverse diseases. After Epithelio-mesenchymal (EMT) of the NCCs within the neural tube, delamination of NCCs occurs. After delamination, the transformation of these cells into various cell lineages produces melanocytes, bones, and cartilage of the skull, cells of the enteric and peripheral nervous system. After the conversion, these cells migrate into various locations of the entire body according to the cell lineage. Abnormalities in neural crest (NC) formation and migration result in various malformations and tumors, known as neurocristopathy. Material and Methods: Herein, this case series describes a single-center experience in cephalic NC disorders over the past 3 years, including 17 cases of varying composition (i.e., vascular, dysgenetic, mixed, and neoplastic forms) involving the brain and occasionally skin, eyes, and face of the patients. Results: In our study of 17 patients with cephalic NC disease, 6 (35.3%) patients had vascular form, 5 (29.4%) had dysgenetic form, 4 (23.5%) had mixed form, and 2 (11.7%) had neoplastic form. Brain involvement in the form of vascular or parenchyma or both vascular and parenchymal was seen in all of our patients (100%), skin in 6 (35.3%) patients, eye in 2 (11.7%), and face in 1 (5.9%) patient. Treatment was planned according to the various manifestations of the disease. Conclusion: Neural crest diseases (NCDs) are a rare and under-recognized group of disorders in the literature and may have been under-reported due to a lack of awareness regarding the same. More such reporting may increase the repertoire of these rare disorders such that clinicians can have a high degree of suspicion leading to early detection and timely counseling and also improve preventive strategies and help in developing new drugs for these disorders or prevent them.

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