RESUMO
Due to the low-power consumption, self-driven ultraviolet (UV) photodetectors have great potentials in a broad range of applications, such as optical communication, ozone monitoring, bio-medicine, and flame detection. In this Letter, it is pretty novel to enhance the photocurrent and responsivity of self-driven UV photodetectors by (Al,Ga)N nanowire/graphene/polyvinylidene fluoride (PVDF) heterojunction successfully. Compared to those of the photodetector with only nanowire/graphene heterojunction, it is found that both the photocurrent and responsivity of the photodetector with nanowire/graphene/PVDF heterojunction can be enhanced more than 100%. It is proposed that PVDF could maintain the internal gain by increasing the number of carrier cycles. Furthermore, this photodetector can also have a high detectivity of 5.3×1011 Jones and fast response speed under 310 nm illumination. After preserving for one month without any special protection, both photocurrent and responsivity of the photodetector with nanowire/graphene/PVDF heterojunction are demonstrated to be quite stable. Therefore, this work paves an effective way to improve the performance of photodetectors for their applications in the fields of next-generation optoelectronic devices.
RESUMO
Swine acute diarrhea syndrome coronavirus (SADS-CoV) is a novel porcine enteric coronavirus that causes acute watery diarrhea, vomiting, and dehydration in newborn piglets. The type III interferon (IFN-λ) response serves as the primary defense against viruses that replicate in intestinal epithelial cells. However, there is currently no information available on how SADS-CoV modulates the production of IFN-λ. In this study, we utilized IPI-FX cells (a cell line of porcine ileum epithelium) as an in vitro model to investigate the potential immune evasion strategies employed by SADS-CoV against the IFN-λ response. Our results showed that SADS-CoV infection suppressed the production of IFN-λ1 induced by poly(I:C). Through screening SADS-CoV-encoded proteins, nsp1, nsp5, nsp10, nsp12, nsp16, E, S1, and S2 were identified as antagonists of IFN-λ1 production. Specifically, SADS-CoV nsp1 impeded the activation of the IFN-λ1 promoter mediated by MAVS, TBK1, IKKε, and IRF1. Both SADS-CoV and nsp1 obstructed poly(I:C)-induced nuclear translocation of IRF1. Moreover, SADS-CoV nsp1 degraded IRF1 via the ubiquitin-mediated proteasome pathway without interacting with it. Overall, our study provides the first evidence that SADS-CoV inhibits the type III IFN response, shedding light on the molecular mechanisms employed by SADS-CoV to evade the host immune response.
Assuntos
Alphacoronavirus , Infecções por Coronavirus , Doenças dos Suínos , Animais , Suínos , Complexo de Endopeptidases do Proteassoma , Interferon lambda , Alphacoronavirus/fisiologia , Ubiquitinas , Infecções por Coronavirus/veterináriaRESUMO
Because of wide range of applications, the flexible artificial synapse is an indispensable part for next-generation neural morphology computing. In this work, we demonstrate a flexible synaptic device based on a lift-off (In,Ga)N thin film successfully. The synaptic device can mimic the learning, forgetting, and relearning functions of biological synapses at both flat and bent states. Furthermore, the synaptic device can simulate the transition from short-term memory to long-term memory successfully under different bending conditions. With the high flexibility, the excitatory post-synaptic current of the bent device only shows a slight decrease, leading to the high stability. Based on the experimental conductance for long-term potentiation and depression, the simulated three-layer neural network can achieve a high recognition rate up to 90.2%, indicating that the system comprising of flexible synaptic devices could have a strong learning-memory capability. Therefore, this work has a great potential for the development of wearable intelligence devices and flexible neuromorphic systems.
Assuntos
Sinapses , Dispositivos Eletrônicos Vestíveis , Redes Neurais de ComputaçãoRESUMO
BACKGROUND AND AIMS: Sarcopenia is a disease characterized by loss of skeletal muscle mass and function that is closely associated with cardiovascular disease. The serum creatinine/cystatin C (Cr/CysC) ratio has been shown to be a simplified indicator for identifying low muscle mass (LMM) or sarcopenia. The aim of this study was to investigate whether the Cr/CysC ratio helps to predict prognostic information in hypertensive patients. METHODS AND RESULTS: This cohort study included 2509 patients with hypertension from the National Health and Nutrition Survey 1999-2002. To evaluate the association between Cr/CysC ratio and mortality, we used Kaplan Meier estimates to calculate cumulative survival probabilities for all-cause mortality and cardiovascular mortality, Cox regression analyses, and hazard ratio (HR) and 95% confidence interval (CI) were calculated. Over a median follow-up of 11.76 years, lower Cr/CysC ratio was associated with lower risk of all-cause mortality (per 0.1 increase, HR:0.81, 95% CI: 0.77-0.85, P < 0.001) and cardiovascular mortality (per 0.1 increase, HR:0.80, 95% CI: 0.72-0.89, P < 0.001). Compared with patients with normal muscle mass, all-cause mortality, and cardiovascular mortality HR for patients with LMM diagnosed by Cr/CysC ratio were 1.57 (95% CI: 1.36-1.82, P < 0.001) and 1.64 (95% CI: 1.12-2.42, P = 0.012), respectively. CONCLUSION: We found that low muscle mass shown by lower Cr/CysC ratio was an independent risk factor for poor prognosis in hypertensive patients. We recommend routine screening of Cr/CysC ratio in hypertensive patients and early intervention for low muscle mass or sarcopenia.
Assuntos
Doenças Cardiovasculares , Hipertensão , Sarcopenia , Humanos , Estudos de Coortes , Creatinina/metabolismo , Cistatina C , Hipertensão/diagnóstico , Sarcopenia/diagnósticoRESUMO
Rheumatoid arthritis (RA) is a chronic autoimmune disease marked by synovitis and cartilage destruction. The active compound, icariin (ICA), derived from the herb Epimedium, exhibits potent anti-inflammatory properties. However, its clinical utility is limited by its water insolubility, poor permeability, and low bioavailability. To address these challenges, we developed a multifunctional drug delivery system-adipose-derived stem cells-exosomes (ADSCs-EXO)-ICA to target active macrophages in synovial tissue and modulate macrophage polarization from M1 to M2. High-performance liquid chromatography analysis confirmed a 92.4 ± 0.008% loading efficiency for ADSCs-EXO-ICA. In vitro studies utilizing cellular immunofluorescence (IF) and flow cytometry demonstrated significant inhibition of M1 macrophage proliferation by ADSCs-EXO-ICA. Enzyme-linked immunosorbent assay, cellular transcriptomics, and real-time quantitative PCR indicated that ADSCs-EXO-ICA promotes an M1-to-M2 phenotypic transition by reducing glycolysis through the inhibition of the ERK/HIF-1α/GLUT1 pathway. In vivo, ADSCs-EXO-ICA effectively accumulated in the joints. Pharmacodynamic assessments revealed that ADSCs-EXO-ICA decreased cytokine levels and mitigated arthritis symptoms in collagen-induced arthritis (CIA) rats. Histological analysis and micro computed tomography confirmed that ADSCs-EXO-ICA markedly ameliorated synovitis and preserved cartilage. Further in vivo studies indicated that ADSCs-EXO-ICA suppresses arthritis by promoting an M1-to-M2 switch and suppressing glycolysis. Western blotting supported the therapeutic efficacy of ADSCs-EXO-ICA in RA, confirming its role in modulating macrophage function through energy metabolism regulation. Thus, this study not only introduces a drug delivery system that significantly enhances the anti-RA efficacy of ADSCs-EXO-ICA but also elucidates its mechanism of action in macrophage function inhibition.
Assuntos
Tecido Adiposo , Artrite Reumatoide , Exossomos , Flavonoides , Macrófagos , Animais , Flavonoides/farmacologia , Flavonoides/química , Exossomos/metabolismo , Ratos , Macrófagos/efeitos dos fármacos , Macrófagos/metabolismo , Tecido Adiposo/citologia , Masculino , Artrite Experimental/tratamento farmacológico , Ratos Sprague-Dawley , Sistemas de Liberação de Medicamentos/métodos , Células-Tronco/metabolismo , Células-Tronco/efeitos dos fármacos , Células-Tronco Mesenquimais/metabolismo , Células-Tronco Mesenquimais/efeitos dos fármacosRESUMO
Due to the requirements of oceanography exploration and detection, self-powered photodetectors (PDs) with low-power consumption are essential for the next-generation optoelectronic applications. In this work, we successfully demonstrate a self-powered photoelectrochemical (PEC) PD in seawater based on the (In,Ga)N/GaN core-shell heterojunction nanowires. Compared to those of the PD in pure water, it is found that the upward and downward overshooting features of current can be the key reason contributing to the much faster response speed of the PD in seawater. Thanks to the enhanced response speed, the rise time of PD can be reduced more than 80%, and the fall time remains only 30% by applying in seawater instead of pure water. The key factors of generating these overshooting features should be the instantaneous temperature gradient, carrier accumulation and elimination on the semiconductor/electrolyte interfaces at the moments of light on and off. By the analysis of experimental results, the Na+ and Cl- ions are proposed to be the main factors affecting the PD behavior in seawater, which can enhance the conductivity and accelerate the oxidation-reduction reaction significantly. This work paves an effective way to develop the new self-powered PDs for the wide applications in under-seawater detection and communication.
RESUMO
BACKGROUND: The triglyceride-glucose (TyG) index is a reliable alternative biomarker of insulin resistance (IR). However, whether the TyG index has prognostic value in critically ill patients with coronary heart disease (CHD) remains unclear. METHODS: Participants from the Medical Information Mart for Intensive Care III (MIMIC-III) were grouped into quartiles according to the TyG index. The primary outcome was in-hospital all-cause mortality. Cox proportional hazards models were constructed to examine the association between TyG index and all-cause mortality in critically ill patients with CHD. A restricted cubic splines model was used to examine the associations between the TyG index and outcomes. RESULTS: A total of 1,618 patients (65.14% men) were included. The hospital mortality and intensive care unit (ICU) mortality rate were 9.64% and 7.60%, respectively. Multivariable Cox proportional hazards analyses indicated that the TyG index was independently associated with an elevated risk of hospital mortality (HR, 1.71 [95% CI 1.25-2.33] P = 0.001) and ICU mortality (HR, 1.50 [95% CI 1.07-2.10] P = 0.019). The restricted cubic splines regression model revealed that the risk of hospital mortality and ICU mortality increased linearly with increasing TyG index (P for non-linearity = 0.467 and P for non-linearity = 0.764). CONCLUSIONS: The TyG index was a strong independent predictor of greater mortality in critically ill patients with CHD. Larger prospective studies are required to confirm these findings.
Assuntos
Doença das Coronárias , Estado Terminal , Masculino , Humanos , Feminino , Cuidados Críticos , Doença das Coronárias/diagnóstico , Glucose , Triglicerídeos , Glicemia , Biomarcadores , Fatores de RiscoRESUMO
Atomically dispersed catalysts (ADCs) with a well-defined structure are theoretically desirable for a high-selectivity photocatalytic reaction. However, achieving high product selectivity remains a practical challenge for ADCs-based photocatalysts. Herein, we reveal a spin polarization effect on achieving high product selectivity (95.0%) toward the photocatalytic nitrobenzene (PhNO2) hydrogenation to aniline (PhNH2) on atomically dispersed Fe site-loaded graphitic carbon nitride (Fe/g-C3N4). In combination with the Gibbs free energy diagram and electronic structure analysis, the origin of the photocatalytic performance is attributed not only to the strong metal-support interaction between the Fe site and g-C3N4, but more importantly to the strong spin polarization effect that promotes the potential-determining step (PDS) of *PhNO to *PhNOH. This work could be helpful for the design of ADCs-based photocatalysts in view of the spin polarization effect.
RESUMO
BACKGROUND: Triglyceride-glucose (TyG) index as a reliable surrogate of insulin resistance (IR) has been shown to be related to adverse clinical outcomes in patients with acute coronary syndrome, heart failure, ischemic stroke and so on. However, the relationship between TyG index and all-cause mortality in intensive care unit (ICU) patients remains unknown. The purpose of this study was to investigate the correlation between TyG index and all-cause mortality to evaluate the impact of IR on the prognosis of this population. METHODS: This was a retrospective observational study that included 3026 patients who had an initial triglyceride and glucose data on the first day of ICU admission, and all data were extracted from the Medical Information Mart for Intensive Care III (MIMIC-III) database. These patients were grouped into quartiles (Q1-Q4) according to TyG index. The Kaplan-Meier analysis was used to compare all-cause mortality among the above four groups. Cox proportional hazards analyses were performed to examine the association between TyG index and all-cause mortality. RESULTS: During 10.46 years of follow-up, 1148 (37.9%) patients died, of which 350 (11.6%) occurred during the hospital stay and 258 (8.5%) occurred during the ICU stay. Kaplan-Meier analysis showed that the risk of all-cause mortality was significantly higher in patients with higher TyG index (log-rank P = 0.021). Multivariable Cox proportional hazards analyses showed that the TyG index was an independent risk predictor of ICU death (HR: 1.72, 95% CI 1.18-2.52, P = 0.005) and hospital death (HR: 2.19, 95% CI 1.59-3.03, P < 0.001), and each 1-unit increased in the TyG index, a 1.19-fold increase in the risk of death during the hospital stay. CONCLUSIONS: TyG index is strongly related to the all-cause mortality increasing in critically ill patients. This finding indicates that the TyG index might be useful in identifying people at high risk of ICU death and hospital death.
Assuntos
Glicemia , Resistência à Insulina , Biomarcadores , Glicemia/análise , Estado Terminal , Glucose , Humanos , Medição de Risco , Fatores de Risco , TriglicerídeosRESUMO
As an attractive and valuable platform chemical, 3-hydroxypropionic acid (3-HP) can be used to produce a variety of industrially important commodity chemicals and biodegradable polymers. Moreover, the biosynthesis of 3-HP has drawn much attention in recent years due to its sustainability and environmental friendliness. Here, we focus on recent advances, challenges, and metabolic engineering strategies in the biosynthesis of 3-HP. While glucose and glycerol are major carbon sources for its production of 3-HP via microbial fermentation, other carbon sources have also been explored. To increase yield and titer, synthetic biology and metabolic engineering strategies have been explored, including modifying pathway enzymes, eliminating flux blockages due to byproduct synthesis, eliminating toxic byproducts, and optimizing via genome-scale models. This review also provides insights on future directions for 3-HP biosynthesis.
Assuntos
Ácido Láctico , Engenharia Metabólica , Carbono , Glicerol/metabolismo , Ácido Láctico/análogos & derivados , Ácido Láctico/metabolismoRESUMO
BACKGROUND: Red blood cell distribution width (RDW) is highly associated with adverse clinical outcomes in many diseases. The present study aimed to evaluate the relationship between RDW and gastrointestinal bleeding (GIB) after isolated coronary artery bypass grafting (CABG). METHODS: This was a retrospective observational study that included 4473 patients who received CABG, and all the data were extracted from the Medical Information Mart for Intensive Care III database. Data collected included patient demographics, associated comorbid illnesses, laboratory parameters, and medications. The receiver operating characteristic (ROC) curve was used to determine the best cutoff value of RDW for the diagnosis of GIB. Multivariable logistic regression analysis was used to analyze the relationship between RDW and GIB. RESULTS: The incidence of GIB in patients receiving CABG was 1.1%. Quartile analyses showed a significant increase in GIB incidence at the fourth RDW quartile (> 14.3%; P < 0.001). The ROC curve analysis revealed that an RDW level > 14.1% measured on admission had 59.6% sensitivity and 69.4% specificity in predicting GIB after CABG. After adjustment for confounders, high RDW was still associated with an increased risk of GIB in patients with CABG (odds ratio = 2.83, 95% confidence interval 1.46-5.51, P = 0.002). CONCLUSIONS: Our study indicates that the elevated RDW level is associated with an increased risk of GIB after CABG, and it can be an independent predictor of GIB. The introduction of RDW to study GIB enriches the diagnosis method of GIB and ensures the rapid and accurate diagnosis of GIB.
Assuntos
Ponte de Artéria Coronária , Índices de Eritrócitos , Ponte de Artéria Coronária/efeitos adversos , Eritrócitos , Hemorragia Gastrointestinal/diagnóstico , Hemorragia Gastrointestinal/epidemiologia , Hemorragia Gastrointestinal/etiologia , Humanos , Prognóstico , Curva ROC , Estudos Retrospectivos , Fatores de RiscoRESUMO
Conventional photocatalysts generate numerous active species-primarily hydroxyl radicals (â¢OH)-under solar light excitation to exert photocatalytic activity for especially antibacterial effects. However, the light dependence limits their competitiveness against other antimicrobial materials since they do not work at night. Herein, a P-g-C3N4/Sr2MgSi2O7:Eu2+,Dy3+ (P-g-C3N4/SMSO) composite day-night photocatalyst is synthesized, using a model methyl orange (MO) substrate, and the impacts of trace P doping and the SMSO composite on the activity of the photocatalyst in MO degradation is investigated; Its antibacterial effect against Escherichia coli and Staphylococcus aureus on ceramic surfaces is further examined. The morphology, structure, and composition of the photocatalyst are characterized by SEM, TEM, XRD, FT-IR, and UV-vis DRS. Finally, the photocatalytic mechanism is elucidated through active species capture experiments and ESR testing. P doping and the SMSO heterojunction structure reduce the width of the forbidden band of g-C3N4 and broaden its visible-light-response range. Moreover, SMSO acts as a light source to realize long-lasting photocatalytic performance of the composite, even in the dark. The photocatalytic process produces â¢O2-, 1O2, and h+ active species, with â¢O2- and 1O2 playing the dominant role-instead of â¢OH as previously thought.
Assuntos
Nitrilas , Fósforo , Antibacterianos/química , Antibacterianos/farmacologia , Catálise , Cerâmica/farmacologia , Escherichia coli , Nitrilas/química , Fósforo/farmacologia , Espectroscopia de Infravermelho com Transformada de FourierRESUMO
Photocatalytic conversion of CO2to produce fuel is considered a promising approach to reduce CO2emissions and tackle energy crisis. GaN-based materials have been studied for CO2reduction because of their excellent optical properties and band structure. However, low photocatalytic activity and severe photocorrosion of GaN-based photoelectrode greatly limit their applications. In this work, photocatalytic activity was improved by adopting InGaN quantum dots (QDs) combined with C3N4nano-sheets as photoanode, and thus the efficiency of CO2reduction and the selectivity of hydrogen production were increased significantly. In addition, the photoelectron-chemical corrosion of photoelectrodes has been apparently controlled. InGaN QDs/C3N4has the highest CO and H2productions rates of 14.69µmol mol-1h-1and 140µmol mol-1h-1which were 2.2 times and 14.5 times than that of InGaN film photoelectrode, respectively. The enhancement of photocatalytic activity is attributed to C3N4modification and a large electric dipole forming on the surface of InGaN QDs, which facilitate the separation and transfer of photo-generated carriers and thus promote CO2reduction reaction. This work provides a promising strategy for the development of GaN-based photoanodes with superior stability and efficiency.
RESUMO
A unified catalytic asymmetric (N+1) (N=4, 5) annulation reaction of oxindoles with bifunctional peroxides has been achieved in the presence of a chiral phase-transfer catalyst (PTC). This general strategy utilizes peroxides as unique bielectrophilic four- or five-atom synthons to participate in the C-C and the subsequent umpolung C-O bond-forming reactions with one-carbon unit nucleophiles, thus providing a distinct method to access the valuable chiral spirooxindole-tetrahydrofurans and -tetrahydropyrans with good yields and high enantioselectivities under mild conditions. DFT calculations were performed to rationalize the origin of high enantioselectivity. The gram-scale syntheses and synthetic utility of the resultant products were also demonstrated.
RESUMO
Talaromyces marneffei is an important opportunistic pathogen mainly afflicting the HIV-infected patients, in rare instance, it could cause infection in non-HIV-infected individuals. We report a 51-year-old Chinese woman who, with histories of SLE for 14 years and disseminated talaromycosis for 4 years, occurred partial intestinal obstruction that was demonstrated to be caused by Talaromyces marneffei infection. The randomly amplified polymorphic DNA results of paraffin-embedded tissues from both the present episode and the previous infection suggested that the present infection was a recurrent. The patient was performed excision of involved intestine and treated with oral itraconazole at a daily dose of 400 mg for 3 months, leading to an excellent response. However, she died with unknown reason more than a year later. We also reviewed the literature on Talaromyces marneffei infection associated with SLE as well as intestinal talaromycosis alone.
Assuntos
Obstrução Intestinal , Lúpus Eritematoso Sistêmico , Micoses , Talaromyces , Feminino , Humanos , Obstrução Intestinal/microbiologia , Lúpus Eritematoso Sistêmico/complicações , Pessoa de Meia-Idade , Micoses/diagnóstico , RecidivaRESUMO
To enhance the electron-hole separation and boost the practical performance of commercial titania (Degussa P25) under natural solar light, in this work, P25 was modified with Co(II) species (CoP25) through post-treatment with decomposition of Co-ethylenediaminetetraacetic acid precursors in a wet chemical anchoring process. With appropriate Co(II) loading amount as molecular cocatalyst, the resulted CoP25-4 showed significantly improved photocatalytic performance for Cr(VI) reduction and bisphenol A (BPA) oxidation under UV-light irradiation. The coexistence of Cr(VI) and BPA promoted mutually the degradation of both pollutants. Under simulated solar light (AM 1.5G) illumination, the Cr(VI) reduction rate over CoP25-4 was 8.5 times enhanced compared with that over P25, whereas the simultaneous degradation rate of BPA over CoP25-4 was 8 times higher than that over P25. Further investigations indicated that the covalent atomic Co(II) anchoring on P25 significantly promoted the photogenerated electron-hole separation and facilitated Cr(VI) reduction via the formation of a Co(I) intermediate and simultaneously boosted BPA oxidation. Our results demonstrated a facile strategy to modify P25 with remarkably improved performance for the practical application in environmental pollution management under natural light excitation.
RESUMO
PURPOSE: Morbidity due to cutaneous adverse drug reactions (CADRs) is quite common. The specific culprit drugs change over time and clinicians must be kept informed with updated knowledge, thus preventing potential CADRs. This retrospective study is a survey of CADRs encountered in a hospital-based population in Southern China during three time intervals, from 1984 to 2015. MATERIALS AND METHODS: The clinical records were review of 306 patients with CADRs who were admitted to our hospital from 2011 to 2015. These data were compared with patients visiting our hospital during 1984-1994 and 2003-2010. RESULTS: From 2011 to 2015, the most common CADRs were exanthematous reactions (40.8%) and Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN; 17.0%). There were eight cases (2.6%) of CADRs related to targeted therapy in oncology. In the 205 CADR cases that were due to single medications, the most common offending drugs were allopurinol (21.5%), cephalosporins (10.7%) and carbamazepine (10.2%). The percentages of CADR cases due to allopurinol, carbamazepine, or epidermal growth factor receptor inhibitors were significantly higher from 2011 to 2015 compared with 1984-1994 or 2003-2010. The rate of SJS/TEN occurrence was significantly higher in the two recent periods compared with 1984-1994. CONCLUSIONS: Changes in drug prescriptions are a major factor that affects the CADRs seen in clinical records. Newer drugs can be culpable for CADRs, and more CADRs are now documented with increased severity at clinical presentation. Reliable screening tests for specific drugs are urgently required to eliminate possible fatalities.
Assuntos
Toxidermias/epidemiologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Alopurinol/efeitos adversos , Anti-Inflamatórios não Esteroides/efeitos adversos , Carbamazepina/efeitos adversos , Cefalosporinas/efeitos adversos , Criança , Pré-Escolar , China/epidemiologia , Toxidermias/etiologia , Feminino , Fluoroquinolonas/efeitos adversos , Humanos , Lactente , Masculino , Medicina Tradicional Chinesa/efeitos adversos , Pessoa de Meia-Idade , Penicilinas/efeitos adversos , Adulto JovemRESUMO
Aquagenic cutaneous disorders, which include aquagenic urticaria, aquagenic pruritus, and aquagenic acrokeratoderma, are a group of rare diseases characterized by skin lesions or discomfort induced by brief contact with water. Aquagenic urticaria is characterized by pruritic wheals that occur at the sites of water contact, either immediately or within minutes, and disappear within 30-60 min after water removal. Aquagenic pruritus presents with severe pruritus or a stinging, tingling, or burning sensation without any visible skin changes. These symptoms occur immediately or within minutes after the skin is exposed to water, lasting for one hour or longer. Aquagenic acrokeratoderma usually presents with whitish papules and plaques, edema, and hyperwrinkling within 2-20 min after brief exposure to water; symptoms disappear within minutes to one hour after drying. The pathomechanisms of these diseases are not yet fully understood, and their diagnosis is primarily based on clinical features. Treatment remains a challenge. Herein, we review the literature regarding these unusual disorders.