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1.
Nephron ; 148(4): 264-272, 2024.
Article in English | MEDLINE | ID: mdl-36617405

ABSTRACT

The clinical features of cerebellar vermis hypoplasia, oligophrenia, ataxia, coloboma, and hepatic fibrosis (COACH) characterize the rare autosomal recessive multisystem disorder called COACH syndrome. COACH syndrome belongs to the spectrum of Joubert syndrome and related disorders (JSRDs) and liver involvement distinguishes COACH syndrome from the rest of the JSRD spectrum. Developmental delay and oculomotor apraxia occur early but with time, these can improve and may not be readily apparent or no longer need active medical management. Congenital hepatic fibrosis and renal disease, on the other hand, may develop late, and the temporal incongruity in organ system involvement may delay the recognition of COACH syndrome. We present a case of a young adult presenting late to a Renal Genetics Clinic for evaluation of renal cystic disease with congenital hepatic fibrosis, clinically suspected to have autosomal recessive polycystic kidney disease. Following genetic testing, a reevaluation of his medical records from infancy, together with reverse phenotyping and genetic phasing, led to a diagnosis of COACH syndrome.


Subject(s)
Abnormalities, Multiple , Brain/abnormalities , Cerebellar Vermis , Cerebellum/abnormalities , Cholestasis , Coloboma , Genetic Diseases, Inborn , Intellectual Disability , Liver Diseases , Nervous System Malformations , Polycystic Kidney, Autosomal Recessive , Young Adult , Humans , Coloboma/diagnosis , Coloboma/genetics , Polycystic Kidney, Autosomal Recessive/diagnosis , Polycystic Kidney, Autosomal Recessive/genetics , Delayed Diagnosis , Genotype , Liver Cirrhosis/genetics , Ataxia/diagnosis , Ataxia/genetics , Intellectual Disability/genetics , Developmental Disabilities
2.
Eur Rev Med Pharmacol Sci ; 27(11): 4951-4959, 2023 Jun.
Article in English | MEDLINE | ID: mdl-37318469

ABSTRACT

OBJECTIVE: Percutaneous nephrolithotomy (PCNL) is a common endourological procedure for patients with large kidney stones, but the management of postoperative pain is still a major challenge. The aim of this clinical trial was to evaluate the efficacy of 0.25% bupivacaine infiltration along the nephrostomy tract on postoperative pain scores and analgesia consumption in patients who underwent PCNL. PATIENTS AND METHODS: A total of 50 patients who underwent PCNL were enrolled in this prospective, randomized controlled trial (NCT04160936). Patients were prospectively randomized into two equal groups: the study group (n=25) received 20 mL of 0.25% bupivacaine infiltration along the nephrostomy tract, whereas patients in the control group (n=25) did not. Postoperative pain as the primary outcome was assessed by using a visual analogue scale (VAS) and a dynamic visual analogue scale (DVAS) at different time points. The secondary outcomes were the time for first opioid demands, the number of opioid demands, and the total opioid consumption over the 48 h postoperatively. RESULTS: There were no significant differences between the two groups regarding demographics, surgery, and stone characteristics. Patients in the study group had significantly less VAS and DVAS pain scores compared to the control group. The mean time of the first opioid demand in the study group was significantly longer as compared to the control group (7.1 ± 2.5h vs. 3.2 ± 1.8 h, p<0.001). The mean number of doses and total consumption of opioids were significantly less in the study group compared to the control group over 48 h (1.5 ± 0.8 vs. 2.9 ± 0.7 and 122.82 ± 62.5 mg vs. 223 ± 70 mg, respectively) (p<0.0001). CONCLUSIONS: Local anesthetic infiltration of 0.25% bupivacaine along the nephrostomy tract is efficient in alleviating post-operative pain and reducing opioid consumption after PCNL.


Subject(s)
Nephrolithotomy, Percutaneous , Nephrostomy, Percutaneous , Humans , Bupivacaine/therapeutic use , Analgesics, Opioid/therapeutic use , Nephrolithotomy, Percutaneous/adverse effects , Anesthetics, Local/therapeutic use , Prospective Studies , Nephrostomy, Percutaneous/adverse effects , Nephrostomy, Percutaneous/methods , Pain, Postoperative/drug therapy , Nephrotomy
3.
Heliyon ; 9(6): e16436, 2023 Jun.
Article in English | MEDLINE | ID: mdl-37292363

ABSTRACT

Extraction of cellulose nanocrystals (CNCs) from agro-residues has received much attention, not only for their unique properties supporting a wide range of potential applications, but also their limited risk to global climate change. This research was conducted to assess Nile roses (Eichhornia crassipes) fibers as a natural biomass to extract CNCs through an acid hydrolysis approach. Nile roses fibers (NRFs) were initially subjected to alkaline (pulping) and bleaching pretreatments. Microcrystalline cellulose (MCC) was used as control in comparison to Nile rose based samples. All samples underwent acid hydrolysis process at a mild temperature (45 °C). The impact of extraction durations ranging from 5 to 30 min on the morphology structure and crystallinity index of the prepared CNCs was investigated. The prepared CNCs were subjected to various characterization techniques, namely: X-ray diffraction (XRD), FT-IR analysis, Transmission electron microscopy (TEM), and X-ray Photoelectron spectroscopy (XPS). The outcomes obtained by XRD showed that the crystallinity index increased as the duration of acid hydrolysis was prolonged up to 10 min, and then decreased, indicating optimal conditions for the dissolution of amorphous zones of cellulose before eroding the crystallized domains. These data were confirmed by FT-IR spectroscopy. However, a minor effect of hydrolysis duration on the degree of crystallinity was noticed for MCC based samples. TEM images illustrated that a spherical morphology of CNCs was formed as a result of 30 min acid hydrolysis, highlighting the optimal 20 min acid hydrolysis to obtain a fibrillar structure. The XPS study demonstrated that the main constituents of extracted CNCs were carbon and oxygen.

4.
Heliyon ; 8(2): e08877, 2022 Feb.
Article in English | MEDLINE | ID: mdl-35198759

ABSTRACT

The integration of photovoltaic into a greenhouse has been implemented to maximize the energy output and crop production yield from the same land space. The effect of greenhouse external shading of opaque crystalline silicon photovoltaic (PV) panels at 13-26% of the roof area on the microclimate and growth of Chili pepper Capsicum annuum cv. (omega) was investigated. The PV panels were divided into two arrays (each of 4 PVs) and fixed separately on two external pillars of 4 m height in the Southeast and Northwest directions, respectively. Each array of 4 PVs could be tracked from the East-West direction to North-South. There were two greenhouses, one was used as control without shading and the other had the external shading. The results revealed that the external shading of PV slightly decreased air temperatures (1-2 °C) and light intensities (25-40%) but had no effect on the dew point temperatures and relative humidity as compared to the control (unshaded greenhouse). Furthermore, the yield and growth of Chili pepper in the shaded greenhouse was insignificantly higher than that in the unshaded greenhouse. Moreover, the simulation results revealed that the estimated electric energy of photovoltaic panels was 3705 kWh/year at a tilt angle of 25° facing South.

5.
Helminthologia ; 58(3): 281-291, 2021 Sep.
Article in English | MEDLINE | ID: mdl-34934391

ABSTRACT

Veterinary inspection of fish caught at the western side of the Arabian Gulf off the shore of Saudi Arabia in 2017 revealed abnormal irregular zigzag black lines in the skins of 135 out of 1500 (prevalence = 9 %) whitecheek sharks (Carcharhinus dussumieri). These lesions have been noticed in the dorsal and ventral part of the head near the snout and around the eyes, as well as in the skin of the pectoral fins. The parasitological, pathological and ultramorphological aspects of the sampled fish were studied using a light microscope, as well as scanning and transmission electron microscopes. Trichosomoidid-type eggs identified as Huffmanela sp. were found in the superficial layer of the skin. The mature eggs containing larvae (n = 75) were ellipsoidal or spindle-shaped, smooth-shelled, amber-brown to dark brown or even black in colour. The width of the eggs ranged from 34 to 49µm with lengths ranging from 95 to 107µm. The polar plugs of the immature eggs were slightly protruding, whereas mature eggs had clearly protruding polar plugs. The width of the polar plugs ranged from 4.5 - 9.5µm. The shells of the eggs were smooth and there was a thin layer seen near the polar areas. The infected skin exhibited desquamated epithelium, with inflammatory cell aggregations and melanophores in the debris. The underlying musculatures of the skin were free from any eggs, but showed mild pathological changes, including congestion of blood vessels, haemorrhages and oedema. The prevalence of infection showed seasonality, peaking significantly in the spring (18.18 %), with comparable prevalence rates in winter and summer (9.43 % and 8.57 %, respectively). However, infections dropped significantly during the autumn months (3.75 %). It is believed that this is the first study reporting the presence of Huffmanela sp. in whitecheek sharks at El-Jubail Province, Saudi Arabia, Arabian Gulf.

6.
Eur Rev Med Pharmacol Sci ; 25(17): 5412-5423, 2021 Sep.
Article in English | MEDLINE | ID: mdl-34533816

ABSTRACT

OBJECTIVE: To investigate the effect of mirror therapy (MT) together with taping compared to modified constraint-induced movement therapy (mCIMT) and MT alone on the quality of upper extremity (UE) function, dexterity, and grip strength in children with hemiplegic cerebral palsy (CP). PATIENTS AND METHODS: Sixty children with hemiplegic CP ranging in age from 6 to 8 years were enrolled. The participants were randomly distributed into three groups. The three groups underwent the same suggested upper limb (UL) exercise programme for 1h/5 days/week for 12 successive weeks. Group A performed the programme with MT and taping. Group B performed the same programme using mCIMT alone, while group C performed this programme with MT alone. In addition, the three groups underwent a routine physical therapy programme for 1 h. The quality of UE function, dexterity, and grip strength was measured using the Quality of Upper Extremity Skills Test (QUEST), Box and Block Test (BBT), and hand-held dynamometer before and after 12 successive weeks of treatment. RESULTS: After treatment, the measurement of all variables in the three groups showed significant improvements with superior effects seen in group A. CONCLUSIONS: Based on the results obtained in this study, MT with taping, mCIMT alone, and MT alone are good supplements to traditional physical therapy programmes in improving the quality of UE function, dexterity, and grip strength in children with hemiplegic CP with more superior effects seen after using MT together with taping.


Subject(s)
Cerebral Palsy/therapy , Exercise Therapy/methods , Hemiplegia/therapy , Mirror Movement Therapy/methods , Athletic Tape , Child , Female , Hand Strength/physiology , Humans , Male , Muscle Strength Dynamometer , Physical Therapy Modalities , Upper Extremity/physiology
7.
Heliyon ; 7(1): e06062, 2021 Jan.
Article in English | MEDLINE | ID: mdl-33537487

ABSTRACT

Bio-matrix of rice husk and Mobil Composition of Matter No. 41 (MCM-41) was modified with alizarin red S for preconcentration of Pd2+ prior flame atomic absorption spectrometric determination. The prepared bio-matrix (RH@MCM-41@ARS) was characterized by Fourier-transform infrared spectroscopy (FTIR), X-ray diffraction (XRD), transmission electron microscope (TEM), scanning electron microscope, energy dispersive X-ray spectrometer (SEM/EDX) and surface area studies. The impact of different parameters (solution pH, amount of sorbent, contact time, sample volume, initial Pd2+ concentration and diverse ions) on the uptake of Pd2+ were evaluated. The maximum adsorption capacity of Pd2+ onto RH@MCM-41@ARS was 198.2 mg g-1 at optimum conditions. Applying the optimized procedure as a preconcentration step led to limit of detection of 0.13 µg L-1 and dynamic analytical range up to 500 µg L-1. The sorbent was regenerated by 0.5 mol L-1 thiourea for at least 10 cycles without significant reduction of adsorption capacity. The method was applied for preconcentration of Pd2+ from real samples.

8.
RSC Adv ; 11(63): 39728-39741, 2021 Dec 13.
Article in English | MEDLINE | ID: mdl-35494162

ABSTRACT

Microtubules have become an appealing target for anticancer drug development including mainly colchicine binding site inhibitors (CBSIs). A new series of novel trimethoxypyridine derivatives were designed and synthesized as tubulin targeting agents. In vitro anti-proliferative activities of the tested compounds compared to colchicine against hepatocellular carcinoma (HepG-2), colorectal carcinoma (HCT-116), and breast cancer (MCF-7) was carried out. Most of compounds showed significant cytotoxic activities. Compounds Vb, Vc, Vf, Vj and VI showed superior anti-proliferative activities to colchicine. Where compound VI showed IC50 values of 4.83, 3.25 and 6.11 µM compared to colchicine (7.40, 9.32, 10.41 µM) against HCT 116, HepG-2 and MCF-7, respectively. The enzymatic activity against tubulin enzyme was carried out for the compounds that showed high anti-proliferative activity. Also, compound VI exhibited the highest tubulin polymerization inhibitory effect with an IC50 value of 8.92 nM compared to colchicine (IC50 value = 9.85 nM). Compounds Vb, Vc, Vf, Vj, & VIIIb showed promising activities with IC50 values of 22.41, 17.64, 20.39, 10.75, 31.86 nM, respectively. Cell cycle and apoptosis test for compound VI against HepG-2 cells, indicated that compound VI can arrest cell cycle at G2/M phase, and can cause apoptosis at pre-G1 phase, with high apoptotic effect 18.53%. Molecular docking studies of the designed compounds confirmed the essential hydrogen bonding with CYS241 beside the hydrophobic interaction at the binding site compared to reference compounds which assisted in the prediction of the structure requirements for the detected antitumor activity.

9.
Neuropeptides ; 72: 65-74, 2018 Dec.
Article in English | MEDLINE | ID: mdl-30396594

ABSTRACT

The female ventromedial hypothalamic nucleus (VMN) is a focal substrate for estradiol (E) regulation of energy balance, feeding, and body weight, but how E shapes VMN gluco-regulatory signaling in each sex is unclear. This study investigated the hypothesis that estrogen receptor-alpha (ERα) and/or -beta (ERß) control VMN signals that inhibit [γ-aminobutyric acid] or stimulate [nitric oxide, steroidogenic factor-1 (SF-1)] counter-regulation in a sex-dependent manner. VMN nitrergic neurons monitor astrocyte fuel provision; here, we examined how these ER regulate astrocyte glycogen metabolic enzyme, monocarboxylate transporter, and adrenoreceptor protein responses to insulin-induced hypoglycemia (IIH) in each sex. Testes-intact male and E-replaced ovariectomized female rats were pretreated by intracerebroventricular ERα antagonist (MPP) or ERß antagonist (PHTPP) administration before IIH. Data implicate both ER in hypoglycemic inhibition of neuronal nitric oxide synthase protein in each sex and up-regulation of glutamate decarboxylase65/67 and SF-1 expression in females. ERα and -ß enhance astrocyte AMPK and glycogen synthase expression and inhibit glycogen phosphorylase in hypoglycemic females, while ERß suppresses the same proteins in males. Differential VMN astrocyte protein responses to IIH may partially reflect ERα and -ß augmentation of ERß and down-regulation of alpha1, alpha2, and beta1 adrenoreceptor proteins in females, versus ERß repression of GPER and alpha2 adrenoreceptor profiles in males. MPP or PHTPP pretreatment blunted counter-regulatory hormone secretion in hypoglycemic males only, suggesting that in males one or more VMN neurotransmitters exhibiting sensitivity to forebrain ER may passively regulate this endocrine outflow, whereas female forebrain ERα and -ß are apparently uninvolved in these contra-regulatory responses.


Subject(s)
Astrocytes/metabolism , Glycogen/metabolism , Hypoglycemia/metabolism , Prosencephalon/metabolism , Receptors, Estrogen/metabolism , Sex Characteristics , Ventromedial Hypothalamic Nucleus/metabolism , Animals , Blood Glucose/metabolism , Female , Male , Neurons/metabolism , Ovariectomy , Rats , Rats, Sprague-Dawley
10.
Bone Joint J ; 100-B(7): 831-838, 2018 07.
Article in English | MEDLINE | ID: mdl-29954207

ABSTRACT

Aims: What represents clinically significant acetabular undercoverage in patients with symptomatic cam-type femoroacetabular impingement (FAI) remains controversial. The aim of this study was to examine the influence of the degree of acetabular coverage on the functional outcome of patients treated arthroscopically for cam-type FAI. Patients and Methods: Between October 2005 and June 2016, 88 patients (97 hips) underwent arthroscopic cam resection and concomitant labral debridement and/or refixation. There were 57 male and 31 female patients with a mean age of 31.0 years (17.0 to 48.5) and a mean body mass index (BMI) of 25.4 kg/m2 (18.9 to 34.9). We used the Hip2Norm, an object-oriented-platform program, to perform 3D analysis of hip joint morphology using 2D anteroposterior pelvic radiographs. The lateral centre-edge angle, anterior coverage, posterior coverage, total femoral coverage, and alpha angle were measured for each hip. The presence or absence of crossover sign, posterior wall sign, and the value of acetabular retroversion index were identified automatically by Hip2Norm. Patient-reported outcome scores were collected preoperatively and at final follow-up with the Hip Disability and Osteoarthritis Outcome Score (HOOS). Results: At a mean follow-up of 2.7 years (1 to 8, sd 1.6), all functional outcome scores significantly improved overall. Radiographically, only preoperative anterior coverage had a negative correlation with the improvement of the HOOS symptom subscale (r = -0.28, p = 0.005). No significant difference in relative change in HOOS subscale scores was found according to the presence or absence of radiographic signs of retroversion. Discussion: Our study demonstrated the anterior coverage as an important modifier influencing the functional outcome of arthroscopically treated cam-type FAI. Cite this article: Bone Joint J 2018;100-B:831-8.


Subject(s)
Acetabulum/surgery , Arthroscopy/methods , Femoracetabular Impingement/surgery , Hip Joint/surgery , Orthopedic Procedures/methods , Acetabulum/diagnostic imaging , Adolescent , Adult , Arthroscopy/adverse effects , Disability Evaluation , Female , Follow-Up Studies , Hip Joint/diagnostic imaging , Humans , Imaging, Three-Dimensional/methods , Male , Middle Aged , Orthopedic Procedures/adverse effects , Osteoarthritis, Hip/etiology , Patient Reported Outcome Measures , Registries , Retrospective Studies , Treatment Outcome , Young Adult
11.
3 Biotech ; 7(6): 373, 2017 Dec.
Article in English | MEDLINE | ID: mdl-29071170

ABSTRACT

Isolation of high-quality RNA from weed plants such as Parthenium hysterophorus is a difficult task due to the hindrance caused by numerous secondary metabolites. Such metabolites not only affect the quality and yield of RNA, but also limit the quality of downstream applications. Therefore, the present study was undertaken to design a protocol for yielding RNA with better quality and quantity from P. hysterophorus leaf which could be suitable for functional genomics. To achieve the objective, four different important RNA extraction protocols, viz. acid guanidinium thiocyanate-phenol-chloroform, phenol-LiCl precipitation, TRIzol®, and PVP-ethanol were tested. The PVP-ethanol method proved to be best among the tested protocols. This method was further modified for obtaining improved quality and yield of RNA. The modified method successfully enhanced the yield of RNA from 280 to 334 µg g-1 fresh weight. The absorbance ratio (A260/A280) was in the purity range of 1.9 that indicated the good quality of RNA. To prove the feasibility of the extracted RNA in PCR-based cDNA synthesis, actin transcripts were targeted and successfully amplified using suitable primers. The improved protocol thus not only improved the yield and quality of RNA, but also gave better results in reverse transcriptase PCR.

12.
J Trace Elem Med Biol ; 36: 52-6, 2016 Jul.
Article in English | MEDLINE | ID: mdl-27259352

ABSTRACT

Deficiency of essential trace elements (such as Cu or Zn) and accumulation of potentially toxic trace elements (as Cd or Pb) are both known to have adverse effects in hemodialysis (HD) patients. Up to our knowledge, no studies about the permeability of low and high flux polysulfone membranes on metal ions during hemodialysis are available. Therefore, the aim of the present study was to address this issue. Forty one hemodialysis patients (19 were using high flux polysulfone membrane while the remaining were using low flux one) participated in the study. Blood levels of Cu, Zn, Cd and Pb were determined by graphite furnace atomic absorption spectrometry among HD patients, before and after dialysis session, as well as among matched 40 healthy persons. Blood concentrations of Cu and Zn in the whole hemodialysis group was significantly lower than those of the healthy control group, on the other hand the toxic metals (Cd and Pb) levels were observed to be significantly higher among HD patients compared to the normal persons. Among the hemodialysis group, there were no significant differences between the low and high flux dialyzer groups in terms of pre-dialysis blood levels of Cu, Zn, Cd and Pb. In addition, significantly decreased levels of all metal ions were observed after dialysis sessions using either low or high flux membranes. An exception was Pb which did not show any difference between pre-dialysis and post-dialysis values in the low flux groupIn conclusion Zn and Cu deficiencies should be considered in the treatment of these patients. High flux membranes are more efficient than low flux ones in removing excess Cd and Pb. Therefore, when high flux membranes are used, chelation therapy might not be required for Cd and Pb overload.


Subject(s)
Renal Dialysis/instrumentation , Trace Elements/blood , Trace Elements/isolation & purification , Adult , Case-Control Studies , Female , Humans , Male , Middle Aged
13.
Anal Sci ; 32(4): 395-400, 2016.
Article in English | MEDLINE | ID: mdl-27063710

ABSTRACT

A simple mixed-micelle mediated extraction was elaborated for the preconcentration and determination of scandium(III) by inductively coupled plasma optical emission spectrometry. Scandium(III) was complexed with Alizarin Red S and cetyltrimethylammonium bromide at pH 3 to form hydrophobic chelates, which could be extracted with Triton X-114 at room temperature (25°C) in the presence of KI as a salting-out electrolyte. The main parameters of the extraction procedure were investigated in regard to the extraction efficiency of scandium(III). Under the optimum conditions, a linear range of 0.5 - 150 ng mL(-1) and a detection limit of 0.2 ng mL(-1), along with a preconcentration factor of 100, were achieved. Furthermore, the interference of diverse ions accompanying scandium(III) was extensively studied. The obtained results indicate the high selectivity of the proposed procedure. The accuracy of the procedure was verified through recovery experiments on spiked water samples and synthetic mixtures. The procedure was successfully applied to a scandium(III) determination in clay samples.

14.
Hum Exp Toxicol ; 35(6): 654-65, 2016 Jun.
Article in English | MEDLINE | ID: mdl-26231422

ABSTRACT

Because of the widespread use of dichlorvos (DDVP) for domestic applications, evaluation of their toxic effects is of major concern to public health. Lycopene may lower oxidative stress by a mechanism that is not fully elucidated. The present study was undertaken to evaluate the protective efficacy of lycopene in terms of normalization of altered biochemical parameters following DDVP treatment in rats. Animals were divided into four groups. The first group was used as control, while groups 2, 3, and 4 were orally treated with lycopene (10 mg kg(-1) body weight (b.w.)), DDVP (1.6 mg kg(-1) b.w.), and DDVP plus lycopene, respectively. Results showed that oral administration of DDVP for 30 days increased the levels of lipid peroxidation markers such as malondialdehyde, 4-hydroxynonanal, and protein carbonyl content in liver. Also, a decrease in levels of vitamin C, vitamin E, and reduced glutathione was detected due to DDVP administration. These were accompanied by a decrease in the activities of antioxidant enzymes superoxide dismutase, catalase, glutathione peroxidase, and glutathione-S-transferase in the liver tissue. Moreover, DDVP increased the activities of serum transaminases, alkaline phosphatase, lactate dehydrogenase, and lipoxygenase, and the levels of bilirubin, total cholesterol, low-density lipoprotein cholesterol, triglyceride and DNA-protein crosslinks, and 8-hydroxy-2-deoxyguanosine, while decreased the level of high-density lipoprotein cholesterol. Our results provide new insights into the biochemical studies of relation between DDVP hepatotoxicity and lycopene treatment. Administration of lycopene to DDVP-treated rats reverted the status of hepatic markers to near-normal levels. These data suggest that lycopene can protect against the liver damage induced by DDVP.


Subject(s)
Antioxidants/therapeutic use , Carotenoids/therapeutic use , Chemical and Drug Induced Liver Injury/prevention & control , Environmental Pollutants/toxicity , Oxidative Stress/drug effects , Animals , Antioxidants/administration & dosage , Biomarkers/analysis , Carotenoids/administration & dosage , Chemical and Drug Induced Liver Injury/etiology , Chemical and Drug Induced Liver Injury/metabolism , Dichlorvos , Lipid Peroxidation/drug effects , Liver Function Tests , Lycopene , Male , Rats, Wistar
15.
Osteoporos Int ; 27(1): 81-92, 2016 Jan.
Article in English | MEDLINE | ID: mdl-26138583

ABSTRACT

UNLABELLED: Treatment with zoledronic acid (ZA) over 2 years, among 33 children with osteogenesis imperfecta (OI) and five Bruck syndrome cases, showed reduction in fracture rates, pain, and improvement in bone mineral density (BMD) and motor milestones of development. This is the first study reporting the use of bisphosphonates in patients with Bruck syndrome (BS). INTRODUCTION: OI and BS are genetic disorders that result in bone fragility and reduced BMD. There is little literature describing the efficacy and safety of ZA in this population. In this study, we assess the response to treatment with ZA at six monthly intervals in Egyptian children with OI and BS for a period of 2 years. METHODS: Thirty-three patients with OI and five patients with BS were treated with 0.1 mg/kg ZA intravenously every 6 months for 2 years during which they were followed up using different parameters. A clinical severity score (CSS) was applied to the patients before and 2 years after the start of therapy. Comparison of disease severity and response to ZA treatment between autosomal-dominant (AD) and autosomal-recessive (AR) OI patients was also done. RESULTS: After 6 months of treatment, OI and BS patients showed a significant increase in BMD Z-scores (P < 0.003 in the spine and P < 0.004 in the hip), together with a significant drop in fracture rate (P < 0.001), relief of pain (P < 0.001), and improvement in ambulation (P < 0.001). CSS was significantly reduced after 2 years of treatment in both OI and BS patients. AR-OI patients were more severely affected than AD-OI patients and showed more significant improvement. CONCLUSION: Zoledronic acid proved to be safe and effective in the treatment of OI and BS. The biannual infusion protocol was convenient to patients. There was a positive correlation between disease severity and benefits of the treatment. The use of the CSS proved to be of value in the assessment of the degree of severity in OI, and with some modifications, it was a valuable tool for the assessment of response to treatment.


Subject(s)
Bone Density Conservation Agents/therapeutic use , Diphosphonates/therapeutic use , Imidazoles/therapeutic use , Osteogenesis Imperfecta/drug therapy , Adolescent , Bone Density/drug effects , Child , Child, Preschool , Diphosphonates/administration & dosage , Drug Administration Schedule , Female , Fractures, Bone/etiology , Fractures, Bone/physiopathology , Fractures, Bone/prevention & control , Humans , Imidazoles/administration & dosage , Infant , Infusions, Intravenous , Male , Osteogenesis Imperfecta/complications , Osteogenesis Imperfecta/physiopathology , Pain Measurement/methods , Prospective Studies , Severity of Illness Index , Zoledronic Acid
16.
Biomed Pharmacother ; 74: 211-4, 2015 Aug.
Article in English | MEDLINE | ID: mdl-26349987

ABSTRACT

AD is a common neurodegenerative disease characterized by aggregated amyloid-beta (Aß) peptide, and oxidative stress, while uncoupling protein 2 (UCP2) is a member of the anion carrier family, predicted the existence of a protein-regulated proton leak with the main purpose of controlling mitochondrial oxidative stress, reduce the generation of superoxide anion. we use the primary hippocampal neurons and add the different doses of Aß1-40, then observe the change of UCP2 at different concentrations of Aß, activity of LDH and the content of NO. Our results provide novel insight that UCP2 may protect hippocampal neurons exposed to amyloid ß protein through decreasing ROS production. 20µmol/L Aß1-40 significantly increased the activity of LDH and the content of NO. According to the correlation analysis, NO was significantly correlation with LDH, and UCP2 was significantly correlation with NO. These results suggest the potential of UCP2 as a therapeutic candidate for treating neurodegenerative diseases such as AD.


Subject(s)
Alzheimer Disease/physiopathology , Amyloid beta-Peptides/administration & dosage , Ion Channels/metabolism , Mitochondrial Proteins/metabolism , Oxidative Stress , Peptide Fragments/administration & dosage , Animals , Cells, Cultured , Hippocampus/cytology , Hippocampus/metabolism , L-Lactate Dehydrogenase/metabolism , Mitochondria/metabolism , Neurons/pathology , Nitric Oxide/metabolism , Rats , Rats, Sprague-Dawley , Reactive Oxygen Species/metabolism , Uncoupling Protein 2
17.
Article in English | MEDLINE | ID: mdl-25953570

ABSTRACT

Hydroxyapatite nanorods (HAPNRs) were prepared from recycled eggshell by using precipitation method. The structure of the HAPNRs was physicochemically and morphologically characterized by X-ray diffraction, transmission electron microscopy and Fourier transform infrared spectroscopy. The resulting HAPNRs were used for solid phase extractive preconcentration of Cu(2+), Zn(2+) and Pb(2+) prior to its determination by flame atomic absorption spectrometry. Experimental variables that influence the quantitative extraction of metal ions were optimized by both batch and column methods. The analytes were quantitatively sorbed on the matrix between pHs6 and 9. The maximum sorption capacity of the HAPNRs has been found to be 2.43, 2.37 and 2.53 mmol g(-1) for Cu(2+), Zn(2+) and Pb(2+), respectively, with the preconcentration factor of 250. The 3σ detection limit and 10σ quantification limit for Cu(2+), Zn(2+) and Pb(2+) were found to be 0.72, 0.55 and 5.12 µg L(-1) and 2.40, 1.83 and 17.06 µg L(-1), respectively. The calibration curves were linear up to 250 µg L(-1) for Cu(2+), 300 µg L(-1) for Zn(2+) and 400 µg L(-1) for Pb(2+). Accuracy of the proposed method was verified using certified reference materials (NCS ZC85006 Tomato, Seronorm Trace Elements Whole Blood L-1, Seronorm Trace Elements Whole Blood L-3 and Seronorm Trace Elements Urine). The present method was successfully applied to the analysis of these metal ions in sea water, biological and food samples.


Subject(s)
Copper/analysis , Durapatite/chemistry , Lead/analysis , Nanotubes/chemistry , Zinc/analysis , Solid Phase Extraction , Spectrophotometry, Atomic
18.
Spectrochim Acta A Mol Biomol Spectrosc ; 145: 487-492, 2015 Jun 15.
Article in English | MEDLINE | ID: mdl-25797223

ABSTRACT

Evaluation of the photocatalytic activity of ZnO-ZrO2 nanomaterials using fluorescence based technique has rarely been reported. In the present work, ZnO-ZrO2 mixed oxides coupled with various ZnO dosages (0, 10, 30, 50, 70wt%) were prepared by impregnation method. These nanomaterials were characterized by studying their structural, surface and optical properties. The photocatalytic activity in term of quantitative determination of the active oxidative species (OH) produced on the surface of binary oxide was evaluated using fluorescent probe method. The interaction between ZnO and ZrO2 was affected on the photocatalytic efficiency of mixture. The results show that, the addition of ZnO to ZrO2 decreased the electron-hole recombination and increased the rate of OH radicals formation. 50 wt% ZnO-ZrO2 photocatalyst exhibited much higher photocatalytic activity. The profound effect of binary oxide catalyst was generally considered due to the high surface area, small particle size, high monoclinic phase of ZrO2 content, low band gap and the presence of surface OH groups.


Subject(s)
Fluorometry/methods , Light , Nanostructures/chemistry , Zinc Oxide/chemistry , Zirconium/chemistry , Adsorption , Catalysis/radiation effects , Fluorescence , Kinetics , Nanostructures/ultrastructure , Nitrogen/chemistry , Spectrophotometry, Ultraviolet , Spectroscopy, Fourier Transform Infrared , Time Factors , X-Ray Diffraction
19.
Clin Chim Acta ; 448: 13-7, 2015 Aug 25.
Article in English | MEDLINE | ID: mdl-25636653

ABSTRACT

Enoyl-CoA hydratase (Ech) catalyzes the second step in the physiologically important beta-oxidation pathway of fatty acid metabolism. The enzyme was reported to be associated with the progression, metastasis and drug resistance of cancers. It might function as a tumor promoter or a tumor suppressor for certain cancers depending on the particular type or stage of tumor cells/tissues. In this review, Ech's association with malignant tumors as well as its potential mechanisms is discussed and summarized. The enzyme might be useful in the diagnosis, treatment and prognosis determination of certain tumors.


Subject(s)
Enoyl-CoA Hydratase/metabolism , Neoplasms/enzymology , Animals , Humans , Neoplasms/diagnosis , Neoplasms/metabolism
20.
Endocr Regul ; 48(3): 126-34, 2014 Jul.
Article in English | MEDLINE | ID: mdl-25110211

ABSTRACT

OBJECTIVE: We aimed to investigate the effect of ghrelin antagonist (D-Lys3) GHRP-6 on the treatment of ovariectomy-induced obesity as compared to hormone replacement therapy with estradiol. METHODS: Twenty eight rats were divided into four groups: control sham operated (C), ovariectomized non-treated (OVX), ovariectomized+estradiol-treated (OVX+E) groups, and ovariectomized+ghrelin antagonist-treated group (OVX+GA). Rats were allowed free water and commercial standard diet ad libitum for 5 weeks after surgery. Body mass index (BMI) was determined at the beginning and the end of the experiment. Rats were sacrificed by decapitation and blood samples were collected for measurements of serum lipid profile, insulin, and glucose levels. Gastrocolic omental fat (GCOF) was removed and weighed. RESULTS: Ovariectomy was accompanied with a significantly higher body weight, food intake, BMI, GCOF, serum total cholesterol (TC), low density lipoprotein-cholesterol (LDL-C), glucose, insulin, and homeostatic model assessment - insulin resistance (HOMA-IR), with a significant decrease in high density lipoprotein-cholesterol (HDL-C) and triglycerides (TGs) in comparison with C group. Estradiol reversed the ovariectomy-induced changes except that of TGs. Administration of ghrelin antagonist was effective in treating the ovariectomy-induced obesity as evidenced by normalization of body weight, food intake, BMI, and GCOF weight, serum levels of insulin, glucose, HOMA-IR, and HDL-C. The serum levels of TC, LDL-C and TGs were improved but did not reach the control values. CONCLUSION: Although estradiol succeeded in the prevention of almost all ovariectomy-induced disturbances, it had a potential cardiovascular risk due to a marked increase in serum TGs. Ghrelin antagonist was effective in ameliorating ovariectomy-induced obesity, so it may be used as a promising treatment for postmenopausal obesity, irrespective of hormonal replacement.


Subject(s)
Anti-Obesity Agents/therapeutic use , Ghrelin/antagonists & inhibitors , Hormone Replacement Therapy , Obesity/drug therapy , Obesity/etiology , Oligopeptides/therapeutic use , Ovariectomy/adverse effects , Animals , Blood Glucose/drug effects , Blood Glucose/metabolism , Body Weight/drug effects , Eating/drug effects , Estradiol/analogs & derivatives , Estradiol/therapeutic use , Female , Insulin/blood , Insulin Resistance , Lipids/blood , Rats
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