A novel NSDHL variant in CHILD syndrome with gastrointestinal manifestations and localized skin involvement.

Background: CHILD syndrome is an X-linked dominant disorder associated with pathogenic mutations in the NSDHL gene. The condition is predominantly found in females as it is lethal in males. Most cases present at birth with extensive unilateral ichthyosiform erythroderma involving the trunk and limbs.

Amphiphilic and Hydrophilic Block Copolymers from Aliphatic N-Substituted 8-Membered Cyclic Carbonates: A Versatile Macromolecular Platform for Biomedical Applications.

Introduction of hydrophilic components, particularly amines and zwitterions, onto a degradable polymer platform, while maintaining precise control over the polymer composition, has been a challenge. Recognizing the importance of these hydrophilic residues in multiple aspects of the nanobiomedicine field, herein, a straightforward synthetic route to access well-defined amphiphilic and hydrophilic degradable block copolymers from diethanolamine-derived functional eight-membered N-substituted aliphatic cyclic carbonates is reported. By this route, tertiary amine, secondary amine, and zwitterion residues can be incorporated across the polymer backbone.

Cryptococcosis mimicking lung carcinoma with brain metastases in an immunocompetent patient.

Cryptococcosis is a fungal infection caused by Cryptococcus spp. that enters the body via inhalation. This ubiquitous yeast has gained notoriety as an opportunistic pathogen in the immunosuppressed population.

A Simple and Facile Approach to Aliphatic N-Substituted Functional Eight-Membered Cyclic Carbonates and Their Organocatalytic Polymerization.

Aliphatic N-substituted functional eight-membered cyclic carbonates were synthesized from N-substituted diethanolamines by intramolecular cyclization. On the basis of the N-substituent, three major subclasses of carbonate monomers were synthesized (N-aryl, N-alkyl and N-carbamate). Organocatalytic ring opening polymerization (ROP) of eight-membered cyclic carbonates was explored as a route to access narrowly dispersed polymers of predictable molecular weights.

Codelivery of dual drugs from polymeric micelles for simultaneous targeting of both cancer cells and cancer stem cells.

Aim: Phenformin-loaded micelles (Phen M) were used in combination with gemcitabine-loaded micelles (Gem M) to study their combined effect against H460 human lung cancer cells and cancer stem cells (CSCs) in vitro and in vivo.

Materials & Methods: Gem M and Phen M were prepared via self-assembly of a mixture of a diblock copolymer of PEG and urea-functionalized polycarbonate (PEG-PUC) and a diblock copolymer of PEG and acid-functionalized polycarbonate (PEG-PAC) through hydrogen bonding and ionic interactions. Gem M and Phen M were characterized and tested for efficacy both in vitro and in vivo against cancer cells and CSCs.

d(1) Oxosulfido-Mo(V) Compounds: First Isolation and Unambiguous Characterization of an Extended Series.

Reaction of Tp(iPr)Mo(VI)OS(OAr) with cobaltocene in toluene results in the precipitation of brown, microcrystalline oxosulfido-Mo(V) compounds, [CoCp2][Tp(iPr)Mo(V)OS(OAr)] (Cp(-) = η(5)-C5H5(-), Tp(iPr)(-) = hydrotris(3-isopropylpyrazol-1-yl)borate, OAr(-) = phenolate or 2-(s)Bu, 2-(t)Bu, 3-(t)Bu, 4-(s)Bu, 4-Ph, 3,5-(s)Bu2, 2-CO2Me, 2-CO2Et or 2-CO2Ph derivative thereof). The compounds are air- and water-sensitive and display ν(Mo═O) and ν(Mo[Formula: see text]S) IR absorption bands at ca. 890 and 435 cm(-1), respectively, 20-40 cm(-1) lower in energy than the corresponding bands in Tp(iPr)MoOS(OAr).

Injectable biodegradable hydrogels from vitamin D-functionalized polycarbonates for the delivery of avastin with enhanced therapeutic efficiency against metastatic colorectal cancer.

Humanized vascular endothelial growth factor (VEGF) antibody (bevacizumab; Avastin) is a highly effective monoclonal antibody against metastatic colorectal cancer and several other advanced late stage cancers. However, limited aqueous solubility and short circulation half-life of the antibody result in long infusion time (30-90 min) and frequent injections. Such direful medical procedures often cause considerable patient inconvenience and prolonged pharmacy preparation.

Antimicrobial hydrogels: a new weapon in the arsenal against multidrug-resistant infections.

The rapid emergence of antibiotic resistance in pathogenic microbes is becoming an imminent global public health problem. Treatment with conventional antibiotics often leads to resistance development as the majority of these antibiotics act on intracellular targets, leaving the bacterial morphology intact. Thus, they are highly prone to develop resistance through mutation.

Co-delivery of antiviral and antifungal therapeutics for the treatment of sexually transmitted infections using a moldable, supramolecular hydrogel.

In this investigation, a therapeutic co-delivery hydrogel system is developed to provide effective HIV prophylaxis, alongside the prevention and/or treatment of candidiasis. Two components-a HIV reverse transcriptase inhibitor, tenofovir, and a cationic macromolecular antifungal agent derived from a vitamin D-functionalized polycarbonate (VD/BnCl (1:30))-are formulated into biodegradable vitamin D-functionalized polycarbonate/PEG-based supramolecular hydrogels. The hydrogels exhibit thixotropic properties and can be easily spread across surfaces for efficient drug absorption.

Phenformin-loaded polymeric micelles for targeting both cancer cells and cancer stem cells in vitro and in vivo.

Conventional cancer chemotherapy often fails as most anti-cancer drugs are not effective against drug-resistant cancer stem cells. These surviving cancer stem cells lead to relapse and metastasis. In this study, an anti-diabetic drug, phenformin, capable of eliminating cancer stem cells was loaded into micelles via self-assembly using a mixture of a diblock copolymer of poly(ethylene glycol) (PEG) and urea-functionalized polycarbonate and a diblock copolymer of PEG and acid-functionalized polycarbonate through hydrogen bonding.

Role of non-covalent and covalent interactions in cargo loading capacity and stability of polymeric micelles.

Polymeric micelles self-assembled from biodegradable amphiphilic block copolymers have been proven to be effective drug delivery carriers that reduce the toxicity and enhance the therapeutic efficacy of free drugs. Several reviews have been reported in the literature to discuss the importance of size/size distribution, stability and drug loading capacity of polymeric micelles for successful in vivo drug delivery. This review is focused on non-covalent and covalent interactions that are employed to enhance cargo loading capacity and in vivo stability, and to achieve nanosize with narrow size distribution.

Antimicrobial polycarbonates: investigating the impact of balancing charge and hydrophobicity using a same-centered polymer approach.

Biodegradable antimicrobial polymers are a promising solution for combating drug resistant microbes. When designing these materials, the balance between charge and hydrophobicity significantly affects the antimicrobial activity and selectivity toward microbes over mammalian cells. Furthermore, where the charge and hydrophobicity is located on the molecules has also proven to be significant.

Block copolymer mixtures as antimicrobial hydrogels for biofilm eradication.

Current antimicrobial strategies have mostly been developed to manage infections due to planktonic cells. However, microbes in their nature state will tend to exist by attaching to and growing on living and inanimate surfaces that result in the formation of biofilms. Conventional therapies for treating biofilm-related infections are likely to be insufficient due to the lower susceptibility of microbes that are embedded in the biofilm matrix.

Synergistic co-delivery of membrane-disrupting polymers with commercial antibiotics against highly opportunistic bacteria.

A series of vitamin E-containing biodegradable antimicrobial cationic polycarbonates is designed and synthesized via controlled organocatalytic ring-opening polymerization. The incorporation of vitamin E significantly enhances antimicrobial activity. These polymers demonstrate broad-spectrum antimicrobial activity against various microbes, e.

Structural characterization and unusual reactivity of oxosulfido-Mo(V) compounds: implications for the structure and electronic description of the very rapid form of xanthine oxidase.

The first structural characterization of a mononuclear, EPR-active, oxosulfido-Mo(V) compound related to the very rapid form of xanthine oxidase (VR-XnO) is reported. The compound, [CoCp2][Tp(iPr)Mo(V)OS(2-OC6H4CO2Et)] [Cp = cyclopentadienyl; Tp(iPr) = hydrotris(3-isopropylpyrazol-1-yl)borate], exhibits a distorted octahedral geometry with Mo═O and Mo=/--S distances of 1.761(5) and 2.

Bipyridine Adducts of Molybdenum Imido Alkylidene and Imido Alkylidyne Complexes.

Seven bipyridine adducts of molybdenum imido alkylidene bispyrrolide complexes of the type Mo(NR)(CHCMe(2)R')(Pyr)(2)(bipy) (1a-1g; R = 2,6-i-Pr(2)C(6)H(3) (Ar), adamantyl (Ad), 2,6-Me(2)C(6)H(3) (Ar'), 2-i-PrC(6)H(4) (Ar(iPr)), 2-ClC(6)H(4) (Ar(Cl)), 2-t-BuC(6)H(4) (Ar(t) (Bu)), and 2-MesitylC(6)H(4) (Ar(M)), respectively; R' = Me, Ph) have been prepared using three different methods. Up to three isomers of the adducts are observed that are proposed to be the trans and two possible cis pyrrolide isomers of syn alkylidenes. Sonication of a mixture containing 1a-1g, HMTOH (2,6-dimesitylphenol), and ZnCl(2)(dioxane) led to the formation of MAP species of the type Mo(NR)(CHCMe(2)R')(Pyr)(OHMT) (3a-3g).

Reactivity studies of oxo-Mo(IV) complexes containing potential hydrogen-bond acceptor/donor phenolate ligands.

Reactivity studies of oxo-Mo(IV) complexes, Tp(iPr)MoO{2-OC(6)H(4)C(O)R-κ(2)O,O'} (R = Me, Et, OMe, OEt, OPh, NHPh), containing chelated hydrogen-bond donor/acceptor phenolate ligands are reported. Hydrolysis/oxidation of Tp(iPr)MoO(2-OC(6)H(4)CO(2)Ph-κ(2)O,O') in the presence of methanol yields tetranuclear [Tp(iPr)MoO(μ-O)(2)MoO](2)(μ-OMe)(2) (1), while condensation of Tp(iPr)MoO{2-OC(6)H(4)C(O)Me-κ(2)O,O'} and methylamine gives the chelated iminophenolate complex, Tp(iPr)MoO{2-OC(6)H(4)C(Me)NMe-κ(2)O,N} (2), rather than the aqua complex, Tp(iPr)MoO{2-OC(6)H(4)C(Me)NMe-κO}(OH(2)). The oxo-Mo(IV) complexes are readily oxidized by dioxygen or hydrogen peroxide to the corresponding cis-dioxo-Mo(VI) complexes, Tp(iPr)MoO(2){2-OC(6)H(4)C(O)R}; in addition, suitable one-electron oxidants, e.

Synthesis of cis,syndiotactic ROMP polymers containing alternating enantiomers.

Ring-opening metathesis polymerization (ROMP) of rac-endo,exo-5,6-dicarbomethoxynorbornene (inter alia) yields a cis,syndio,alt-polymer, one in which the sequential units in the cis,syndiotactic polymer consist of alternating enantiomers. Cis selectivity arises through addition of the monomer to produce an all-cis-metallacyclobutane intermediate, while syndioselectivity and alternating enantiomer structures arise as a consequence of inversion of configuration at the metal center with each metathesis step.

cis-Dioxo- and cis-(hydroxo)oxo-Mo(V) complexes stabilized by intramolecular hydrogen-bonding.

The reactions of Tp(iPr)Mo(VI)O(2)Cl with salicylanilides and NEt(3) produce cis-Tp(iPr)Mo(VI)O(2)(2-OC(6)H(4)CONHR) (Tp(iPr) = hydrotris(3-isopropylpyrazol-1-yl)borate, R = Ph, 4-C(6)H(4)Cl, 4-C(6)H(4)OMe). The N-methyl complex, Tp(iPr)MoO(2){2-OC(6)H(4)CON(Me)Ph}, is similarly prepared. Reduction of the amido complexes by cobaltocene produces green, EPR-active compounds, [CoCp(2)][Tp(iPr)Mo(V)O(2)(2-OC(6)H(4)CONHR)], that exhibit strong, low energy, ν(MoO(2)) IR bands at ∼ 895 and 790 cm(-1) (cf.

Synthesis and characterization of TpiPrMoO(S2PR2) (R = Pri, Ph, OEt, OPri, (-)-mentholate) and {HB(OMe)(Pripz)2}MoO(S2PPri2), including isomers of known 1,2-borotropically-shifted complexes.

Green/blue TpiPrMoO(S2PR2) (TpiPr = hydrotris(3-isopropylpyrazolyl)borate; R = Pri, Ph, OEt, OPri, (-)-mentholate) complexes were synthesized and characterized by elemental analysis, mass spectrometry, IR and NMR spectroscopy, and X-ray crystallography. The diamagnetic, six-coordinate, oxo-Mo(IV) complexes possess distorted octahedral geometries defined by terminal oxo, bidentate dithio acid, and tridentate TpiPr ligands. The R = Pri and Ph derivatives are isomers of previously reported 1,2-borotropically shifted complexes, TpiPr*MoO(S2PR2) (TpiPr* = hydrobis(3-isopropylpyrazolyl)(5-isopropylpyrazolyl)borate; ref: Inorg.

Toward multifunctional Mo(VI-IV) complexes: cis-dioxomolybdenum(VI) complexes containing hydrogen-bond acceptors or donors.

The complexes cis-TpiPrMoVIO2(OAr-R) (TpiPr=hydrotris(3-isopropylpyrazol-1-yl)borate, -OAr-R=hydrogen-bonding phenolate derivative) are formed upon reaction of TpiPrMoO2Cl, HOAr-R, and NEt3 in dichloromethane. The orange, diamagnetic, dioxo-Mo(VI) complexes exhibit strong nu(MoO2) IR bands at ca. 935 and 900 cm(-1) and NMR spectra indicative of Cs symmetry.

Coupling of CpCr(CO)3 and heterocyclic dithiadiazolyl radicals. synthetic, x-ray diffraction, dynamic NMR, EPR, CV, and DFT studies.

The reaction of the 1,2,3,5-dithiadiazolyls (4-R-C(6)H(4)CN(2)S(2))(2) (R = Me, 2a; Cl, 2b; OMe, 2c; and CF3, 2d) and (3-NC-5-tBu-C(6)H(3)CN(2)S(2))(2) (2e) with [CpCr(CO)(3)](2) (Cp = eta(5)-C(5)H(5)) (1) at ambient temperature respectively yielded the complexes CpCr(CO)(2)(eta(2)-S(2)N(2)CC(6)H(4)R) (R = 4-Me, 3a; 4-Cl, 3b; 4-OMe, 3c; and 4-CF(3), 3d) and CpCr(CO)(2)(eta(2)-S(2)N(2)CC(6)H(3)-3-(CN)-5-(tBu)) (3e) in 35-72% yields. The complexes 3c and 3d were also synthesized via a salt metathesis method from the reaction of NaCpCr(CO)(3) (1B) and the 1,2,3,5-dithiadiazolium chlorides 4-R-C(60H(4)CN(2)S(2)Cl (R = OMe, 8c; CF(3), 8d) with much lower yields of 6 and 20%, respectively. The complexes were characterized spectroscopically and also by single-crystal X-ray diffraction analysis.

Diagnosis of a metastatic phyllodes tumor of the pancreas using EUS-FNA.

Context: Phyllodes tumors are rare fibro-epithelial lesions which make up less than 1% of all breast neoplasms. After curative surgery, distant metastases may occur without local recurrence; the typical sites of the metastases being the lungs and the bones. Endoscopic ultrasound (EUS) and EUS-guided fine needle aspiration (EUS-FNA) has emerged as the leading modalities for the evaluation of pancreatic masses.

Heterocyclic thionates as a new class of bridging ligands in oxo-centered triangular cyclopentadienylchromium(III) complexes.

The interactions of the benzothiazolate complex, CpCr(CO)(2)(SCSN(C(6)H(4))) (2), and the tetrazole thiolate complex, CpCr(CO)(3)(eta(1)-SCN(4)Ph) (3), with controlled amounts of Me(3)OBF(4) and (MeO)(2)SO(2), respectively, produced the corresponding mu(3)-oxo trinuclear thionate-bridged complexes, [Cp(3)Cr(3)(mu(2)-OH)(mu(3)-O)(mu(2)-eta(2)-SCSN(C(6)H(4)))(2)](5)BF(4) (45%) and [Cp(3)Cr(3)(mu(2)-OH)(mu(3)-O)(mu(2)-eta(2)-SCN(4)Ph)(2)](9)(MeOSO(3)) (53%), together with their respective free dimethylated thiolate ligands, [MeSCSNMe(C(6)H(4))](4)BF(4) and (Me(2)SCN(4)Ph)(8)MeOSO(3). The reaction of 3 with Me(3)OBF(4) resulted in the isolation of a binuclear complex, [Cp(2)Cr(2)(mu-OH)(mu-eta(2)-SCN(4)Ph)(2)](7)BF(4) (43%), and (8)BF(4) (27%). The reaction of the thiopyridine complex, CpCr(CO)(2)(SPy) (4), with I(2) also produced a similar mu(3)-oxo complex 10 (31%), together with CpCrI(2)(THF) (11) and the disulfide (SPy)(2).