Health-related quality of life and cognitive function in children with Crigler-Najjar syndrome type 1.

BACKGROUND: The aim of the study was to assess the health-related quality of life (HRQOL) and cognitive function in patients with Crigler-Najjar syndrome (CNS) type I and its impact on their lives. METHODS: Twenty-one patients diagnosed with CNS type I aged 1 month to 18 years in the Paediatric Hepatology Unit of Cairo University Children's Hospital were enrolled in this cross-sectional observational study. The patients' health-related quality of life (HRQOL) was assessed using the World Health Organization Quality Of Life BREF questionnaire (WHOQOL-BREF) and the Short Form 36 Health Survey Questionnaire (SF-36). Cognitive function was assessed using the Stanford-Binet Intelligence Scale: Fifth Edition (SB5). RESULTS: All patients had a history of admission to a neonatal intensive care unit, 17 were managed by phototherapy only and 5 also underwent exchange transfusion. According to the WHOQOL questionnaire, 11 cases (52.4%) had a low QOL score, and 7 of 13 patients had an average score for their total IQ test. Cases with poor compliance to phototherapy had statistically significantly lower QOL scores (p=0.001), while, according to the SF36 survey, cases who received exchange transfusion had statistically significantly higher cognitive function (p=0.03). There was a positive correlation between the neurological effect as a complication of the disease and poor physical QOL. CONCLUSION: Paediatric patients with CNS have significantly lower HRQOL, especially physically, psychologically and environmentally. It is recommended that assessment of HRQOL should be a routine part of follow-up in CNS patients. Patients whose HRQOL is affected receive regular psychiatric counselling, social support and rehabilitation.Abbreviations: CNS: Crigler-Najjar syndrome; HRQOL: health-related quality of life; IQ: intelligence quotient; NICU: neonatal intensive care unit; QOL: quality of life; SB5: Stanford-Binet intelligence scale: 5th edition; SF-36: Short Form 36 Health Survey Questionnaire; UDGT: uridine diphosphate glucuronosyl transferase; UGT1A1: uridine 5'-diphosphate glucuronosyltransferase; WHOQOL-BREF: World Health Organization Quality of Life Brief Version.

Egyptian propolis extract for functionalization of cellulose nanofiber/poly(vinyl alcohol) porous hydrogel along with characterization and biological applications.

Bee propolis is one of the most common natural extracts and has gained significant interest in biomedicine due to its high content of phenolic acids and flavonoids, which are responsible for the antioxidant activity of natural products. The present study report that the propolis extract (PE) was produced by ethanol in the surrounding environment. The obtained PE was added at different concentrations to cellulose nanofiber (CNF)/poly(vinyl alcohol) (PVA), and subjected to freezing thawing and freeze drying methods to develop porous bioactive matrices. Scanning electron microscope (SEM) observations displayed that the prepared samples had an interconnected porous structure with pore sizes in the range of 10-100 µm. The high performance liquid chromatography (HPLC) results of PE showed around 18 polyphenol compounds, with the highest amounts of hesperetin (183.7 µg/mL), chlorogenic acid (96.9 µg/mL) and caffeic acid (90.2 µg/mL). The antibacterial activity results indicated that both PE and PE-functionalized hydrogels exhibited a potential antimicrobial effects against Escherichia coli, Salmonella typhimurium, Streptococcus mutans, and Candida albicans. The in vitro test cell culture experiments indicated that the cells on the PE-functionalized hydrogels had the greatest viability, adhesion, and spreading of cells. Altogether, these data highlight the interesting effect of propolis bio-functionalization to enhance the biological features of CNF/PVA hydrogel as a functional matrix for biomedical applications.

Effect of Soybean Oil Supplementation on Milk Production, Digestibility, and Metabolism in Dairy Goats under Thermoneutral and Heat Stress Conditions.

In a previous work, we observed that heat-stressed goats suffer reductions in milk yield and its contents of fat and protein. Supplementation with soybean oil (SBO) may be a useful strategy to enhance milk quality. In total, eight multiparous Murciano-Granadina dairy goats (42.8 ± 1.3 kg body weight; 99 ± 1 days of lactation) were used in a replicated 4 × 4 Latin square design with four periods; 21 d each (14 d adaptation, 5 d for measurements and 2 d transition between periods). Goats were allocated to one of four treatments in a 2 × 2 factorial arrangement. Factors were no oil (CON) or 4% of soybean oil (SBO), and controlled thermal neutral (TN; 15 to 20 °C) or heat stress (HS; 12 h/d at 37 °C and 12 h/d at 30 °C) conditions. This resulted in four treatment combinations: TN-CON, TN-SBO, HS-CON, and HS-SBO. Compared to TN, HS goats experienced lower (p < 0.05) feed intake, body weight, N retention, milk yield, and milk protein and lactose contents. However, goats in HS conditions had greater (p < 0.05) digestibility coefficients (+5.1, +5.2, +4.6, +7.0, and +8.9 points for dry matter, organic matter, crude protein, neutral detergent fiber, and acid detergent fiber, respectively) than TN goats. The response to SBO had the same magnitude in TN and HS conditions. Supplementation with SBO had no effects on feed intake, milk yield, or milk protein content. However, SBO supplementation increased (p < 0.05) blood non-esterified fatty acids by 50%, milk fat by 29%, and conjugated linoleic acid by 360%. In conclusion, feeding 4% SBO to dairy goats was a useful strategy to increase milk fat and conjugated linoleic acid without any negative effects on intake, milk yield, or milk protein content. These beneficial effects were obtained regardless goats were in TN or HS conditions.

Lactational Responses of Heat-Stressed Dairy Goats to Dietary L-Carnitine Supplementation.

Heat stress causes significant losses in milk production, and nutritional strategies are needed to alleviate its effects. Endogenous carnitine synthesis is also reduced by heat stress (HS). Carnitine plays a central role in fatty acid oxidation and buffers the toxic effects of acyl groups. We hypothesized that carnitine supplementation would make up for any carnitine deficiencies during HS and improve lipid metabolism. The objective was to evaluate rumen-protected L-carnitine (CAR) supplementation in dairy goats under thermo-neutral (TN) or HS conditions. Four Murciano-Granadina dairy goats were used in a four × four Latin square design. Goats were allocated to one of four treatments in a two × two factorial arrangement. Factors were 1) diet: control (CON) or supplementation with CAR (1 g/d); and 2) ambient conditions: TN (15 to 20 °C) or HS (0900 to 2100 h at 35 °C, 2100 to 0900 h at 28 °C). Blood free-, acetyl-, and total-carnitine concentrations increased almost three times by supplementation. Despite this efficient absorption, CAR had no effect on feed intake, milk production or blood metabolites in TN or HS conditions. Heat stress increased rectal temperature and respiratory rate. Additionally, HS goats experienced 26% loss in feed intake, but they tended to eat longer particle sizes. Compared to TN, heat-stressed goats lost more subcutaneous fat (difference in fat thickness measured before and after each period = -0.72 vs. +0.64 mm). In conclusion, supplemented L-carnitine was efficiently absorbed, but it had no lactational effects on performance of goats under thermo-neutral or heat stress conditions.

Effect of Direct-Acting Agents on Fibrosis Regression in Chronic Hepatitis C Virus Patients' Treatment Compared with Interferon-Containing Regimens.

Hepatitis C virus (HCV) treatment is aiming to cure and prevent the development, progression of fibrosis, and related complications. Interferon-based therapy was claimed to reduce or even reverse fibrosis. Although direct-acting agents have a better cure rate, we still lack the knowledge of their effect on fibrosis regression. We aim to assess fibrosis regression in direct-acting agents compared with interferon-based treatment regimens in the treatment of chronic HCV patients. The 204 chronic HCV patients were divided into 3 groups; group 1(N = 68) received Peg-IFN and ribavirin, group 2 (N = 69) received sofosbuvir and ribavirin, and group 3 (N = 67) received Peg-IFN, ribavirin, and sofosbuvir. Fibrosis assessment was performed by transient elastography (TE), APRI and FIB 4, in the pretreatment and at least 3 months after end of treatment. Of these, 66.2% of the patients did not show significant fibrosis changes, 6.4% fibrosis progressed, and 27.5% of fibrosis regressed (P < 0.0001) by TE. Similar results were detected in the different treatment regimens with no statistically significant difference between the regimens. Fibrosis regression was detected in 43.3% of cirrhotic patients who achieved sustained virological response (SVR) and only in 27.4% with significant fibrosis. Significant improvement of posttreatment aspartate transaminase, alanine transaminase, and alpha fetoprotein as well as APRI and FIB 4 scores were detected. Fibrosis regression (TE, APRI and FIB 4) was detected with direct-acting agents and interferon-based therapy. Treated patients with significant fibrosis will benefit of fibrosis regression irrespective to their treatment response, whereas fibrosis regression was associated with SVR in cirrhotic patients.

Acupuncture efficacy in the treatment of persistent primary nocturnal enuresis.

INTRODUCTION: This study aimed to assess the therapeutic efficacy of traditional Chinese acupuncture in the treatment of persistent primary nocturnal enuresis (PNE). METHODS: Fifty children and adolescents suffering from persistent PNE were recruited from the Pediatrics and Urology Outpatient Clinic of Zagazig University Hospital during the year 2010. They included 31 boys and 19 girls, with a mean age of 116 ± years (range 9-17 years). The children and their parents underwent thorough counseling followed by two courses of treatment, each course administered over 10 consecutive days. Patients were instructed to continue regular follow-up, every three months, after starting the therapy. The response rate (cure, improvement, or failure) was monitored by recording dry nights and the number of spontaneous arousals to void per week on a calendar. Children who showed partial or no response after six months of observation received another two courses of acupuncture therapy, and were followed up for a further six months period. RESULTS: After the initial two courses of treatment, the cure rate at six months was 76% with an additional 18% achieving partial improvement. Twelve children needed another two courses of treatment. After one year of follow-up, 92% of patients showed complete cure and 8% showed failure of acupuncture therapy. CONCLUSION: Acupuncture treatment in patients with PNE appears effective in increasing the percentage of dry nights, with stable results even after the end of treatment courses. Further controlled studies are needed to confirm these results and to elucidate the therapeutic mechanism of acupuncture. KEYWORDS: Acupuncture Therapy; Nocturnal Enuresis; Traditional Medicine.

Effects of dietary supplements of zinc-methionine on milk production, udder health and zinc metabolism in dairy goats.

Twenty-two Murciano-Granadina dairy goats were used to investigate the effects of organic Zn supplementation of a diet containing a high level of inorganic Zn. Goats were kept in pens, machine milked once a day throughout lactation and fed a diet based on a dehydrated mixture of whole-plant maize and alfalfa ad libitum, alfalfa pellets, barley grain and a concentrate mixture. Treatments were: (1) control, and (2) supplemented with 1 g/d Zn-Methionine (Zn-Met) included in the concentrate mixture. After parturition, goats were blocked in week 3 and dietary treatments were applied until week 23. From weeks 3-20, feed intake, milk yield, milk composition, milk somatic cell count (SCC), and udder health were measured. In week 21, all goats were injected intraperitoneally with 1 g/d DL-methionine for 5 d to establish the effects of methionine under the conditions of udder stress induced by hand milking on the second day. During weeks 22 and 23, diet digestibility, and N and Zn balance were determined. Dry matter intake, milk yield, and milk contents of total solids, fat, total and true protein, and casein did not differ between treatments, but whey protein and non-protein nitrogen contents were significantly lower for the Zn-Met group. Milk SCC tended to decrease as a result of Zn-Met supplementation but differences between treatments were not significant when halves with persistent infection were excluded. Hand milking increased SCC in both groups, but udders of supplemented goats showed a lower reaction. Apparent absorption of N significantly increased and Zn retention tended to increase in Zn-Met supplemented goats. We conclude that Zn-Met supplementation can enhance resistance to udder stress in dairy goats. Effects were attributed to the organic Zn and not to the methionine component. Zn retention and protein utilization were also improved by the Zn-Met supplement.

Efecto abortivo, aislamiento e identificación de principios activos de Alnus acuminata / Abortive effect, isolation and identification of actives principes of Alnus acuminata

Se aislaron tres sustancias puras de la fracción clorofórmica del extracto alcohólico de las hojas de Alnus acuminata, que fueron identificados por métodos espectroscópicos como Olean-13(18)-en-ona; 5-hidroxi-4,ï7-dimetoxiflavona y un alcohol alifático cuya estructura química esta en estudio. El estudio farmacológico mostró un efecto abortivo alto (80-100 por ciento) en ratones hembras preñadas. El estudio histopatológico mostró cambios significativos en las células del útero tanto a nivel del trofoblasto como del endotelio vascular

Fito y bioanálisis de algunas plantas utilizadas en medicina popular con posible actividad farmacológica / Phyto and Bioassay of some plants utilized in popular medicine with possible activity pharmacology

El bioanálisis de 29 especies medicinales y seis sustancias patrones para determinar la CL 50 por el método de Artemia salina, demostró que 22 de las 29 plantas estudiadas presentan CL 50 menor a 500µg/ml. Las especies que presentan CL 50 menor a 100 µg/ml son Lantana camara, Bauhinia picta, Pitiviria alliacea, Caléndula officinalis, Rumex obtusifolius, Salvia palaefolia, taraxacum officinale, Senecio formosus, Malvia rotundifolia, Solanum nigrum y Plantago major. Según el análisis fitoquímico en el presente trabajo la mayoría de las plantas estudiadas presentan alcaloides, esteroles, triterpenos, flavonoides, taninos y saponinas. Los derivados de antraquinonas se encontraron únicamente en Rumex obtusifolius

Contribución al estudio farmacognóstico de algunas especies de coca cultivadas en Colombia / Contribution to the pharmacognostic study of some species of coca grown in Colombia

En el presente trabajo se realizó un estudio macroscópico y microscópico de las hojas de cinco taxa del género Erythroxylum, que permite una identificación adecuada de las mismas