Current treatments for the management of homozygous familial hypercholesterolaemia: a systematic review and commentary.

Aims: Homozygous familial hypercholesterolaemia (HoFH) is a rare disorder characterized by markedly elevated circulating low-density lipoprotein cholesterol (LDL-C) from birth. This review aimed to critically evaluate treatments for HoFH with respect to their efficacy, safety, accessibility, overall context and position within the treatment pathway.

Methods And Results: A mixed-methods review was undertaken to systematically identify and characterize primary interventional studies on HoFH, with a focus on LDL-C reduction as the primary outcome.

Whole Body, Whole Life, Whole Family: Patients' Perspectives on X-Linked Hypophosphatemia.

The rare genetic disorder X-linked hypophosphatemia (XLH) is often exclusively considered to impact children, and, as such, adult patients with XLH may receive inadequate care because their symptoms are not associated with XLH. However, studies have shown that XLH has long-term adverse health consequences that continue throughout adulthood requiring comprehensive lifelong care. Indeed, XLH impacts patients' whole body, whole life, and whole family.

Glycogen Storage Disease Type Ia: Current Management Options, Burden and Unmet Needs.

Glycogen storage disease type Ia (GSDIa) is caused by defective glucose-6-phosphatase, a key enzyme in carbohydrate metabolism. Affected individuals cannot release glucose during fasting and accumulate excess glycogen and fat in the liver and kidney, putting them at risk of severe hypoglycaemia and secondary metabolic perturbations. Good glycaemic/metabolic control through strict dietary treatment and regular doses of uncooked cornstarch (UCCS) is essential for preventing hypoglycaemia and long-term complications.

Studies on effect of co-processing on palmyrah and maize starch mixtures using DOE approach.

Starch mixture is an important approach in designing drug delivery system with modulated properties. The aim of the present work is to study the effect of pregelatinization on starch mixture by varying the concentration of palmyrah and maize starch. All the starch mixtures were statistically designed using 3 full factorial design.

Perioperative self-reflection among surgical residents.

Background: We studied prevalence and predictors of meaningful self-reflection among surgical residents and with prompting/structured interventions, sought to improve/sustain resident skills.

Methods: Residents from six programs recorded 1032 narrative self-reflective comments (120 residents), using a web-based platform. If residents identified something learned or to be improved, self-reflection was deemed meaningful.

Do female surgeons learn or teach differently?

Background: Gender and/or gender-stereotypes might influence surgical education. We hypothesized that female surgeons might focus their learning and teaching differently from male surgeons.

Methods: Residents and surgeons (multi-institutional) individually recorded preoperatively discussed learning objectives (LO) for matching cases.

A taxonomy of perioperative surgical learning: Trending resident skill acquisition.

Background: Resident and curriculum evaluation require tracking surgical resident operative performance, yet what and when to measure remains unclear.

Methods: From a multi-institutional database, we reviewed 611 resident/surgeon-paired assessments of ACGME Milestones and modified OPRS ratings for different cases and postgraduate years.

Results: Faculty Milestone ratings increased with each PGY (p=<0.

Control and support models of help-seeking behavior in women experiencing domestic violence in India.

In India, there is limited prioritization of domestic violence, which is seen as a private and family matter, and handled as a social responsibility rather than a complaint or crime. Despite the Domestic Violence Act, implemented in 2006, the widespread phenomenon of domestic violence across Indian states goes unreported. Using control and support models, this article aims to examine women's behavior in seeking help while dealing with partner violence.

Development, in vitro and in vivo characterization of Eudragit RL 100 nanoparticles for improved ocular bioavailability of acetazolamide.

Glaucoma is characterized by increased intra ocular pressure (IOP) which results in blindness if left untreated. Acetazolamide (ACZ) is used to treat glaucoma since long back. Since it is a Class IV drug [According to Biopharmaceutics Classification System (BCS)], so its topical delivery results in poor ocular bioavailability.

Impact of objectively assessing surgeons' teaching on effective perioperative instructional behaviors.

Importance: Advancing surgical technology and decreasing resident learning hours have limited exposure to perioperative training, necessitating more effective and efficient perioperative teaching by faculty surgeons. Participation in collaborative efforts and process improvement can change behaviors and enhance teaching.

Objectives: To promote deliberate teaching of residents, change resident perception of their teachers, and produce sustainable improvements by objectively measuring surgeons' perioperative teaching performance.

The risk factor of domestic violence in India.

Background: It is over the last decade that research in this field of domestic violence has led to greater recognition of the issue as public health problem. The paper aims to study the prevalence of physical, psychological, and sexual violence and potential risk factors of the women confronting violence within the home in India.

Materials And Methods: A multicentric study with analytical cross-sectional design was applied.

Immune-stimulating potential of cell envelope proteins from Vibrio cholerae associated to chitosan microparticles: an in vitro study.

Context: Cholera is a severe diarrheal disease that remains an important cause of illness and death in many parts of the world.

Objective: This study has been designed to check the immune-stimulating potential of antigens in their native and associated form as chitosan microparticles in vitro.

Material And Methods: Chitosan microparticles were prepared by the ionic gelation technique.

Domestic violence during pregnancy in India.

Domestic violence can result in many negative health consequences for women's health and well-being. Studies on domestic violence illustrate that abused women in various settings had increased health problems such as injury, chronic pain, gastrointestinal, and gynecological signs including sexually transmitted diseases, depression, and posttraumatic stress disorder. This article tries to understand the association between domestic violence and pregnancy outcomes and other health consequences.

Study of glucose uptake activity of Helicteres isora Linn. fruits in L-6 cell lines.

The effect of hot water extract of fruits of Helicteres isora on glucose uptake was studied in rodent skeletal muscle cells (L-6 cells) involved in glucose utilization. H. isora is an antidiabetic medicinal plant being used in Indian traditional medicine.

Improved and convenient method of RNA isolation from polyphenols and polysaccharide rich plant tissues.

It has been difficult to extract a good quality total RNA from the plant parts (such as seeds) which contain high levels of phenolic compounds, carbohydrates and other compounds that bind and/or co-precipitate with RNA. A simple, rapid and efficient method for isolating total RNA from polyphenols and polysaccharide rich plant tissues has been developed. Seeds of leguminosae family were chosen for the study.

Correlates of relationship, psychological, and sexual behavioral factors for HIV risk among Indian women.

The rate of HIV/AIDS among women in India is expected to rise yet few studies have examined factors related to HIV risk among Indian women. The objective of this prospective, cross-sectional study was to characterize similarities and differences in the relationships, psychological well-being, and sexual behaviors among Indian women (N=459). Both HIV positive (N=216) and negative (N=243) women from urban and rural areas in India were included in this study.

Use of gold mill tailings in making bricks: a feasibility study.

Mill tailings dumps at Kolar Gold Fields, Karnataka, are creating environmental problems. One of the solutions to these problems is to use the mill tailings for some useful purpose. This study examined the possibility of making bricks from the mill tailings with some additives in laboratory experiments.

Biosynthesis of Vitamin B(6) in yeast. Incorporation pattern of trioses.

The biosynthetic origin of the C(3) unit, C-6,5,5', of pyridoxamine was investigated in two yeasts, Candida utilis ATCC 9256 and Saccharomyces cerevisiae ATCC 7752. The incorporation patterns within pyridoxamine bishydrochloride derived from variously multiply (13)C- and (2)H-labeled samples of glycerol and glyceraldehyde, established by NMR spectroscopy, indicate that the three-carbon unit C-6,5,5' of pyridoxamine is derived intact from a triose.

2'-hydroxypyridoxol, a biosynthetic precursor of vitamins B(6) and B(1) in yeast.

In separate experiments cultures of the yeast Saccharomyces cerevisiae ATTC 7752 were grown in the presence of [5',5'-2H2]- or of [2',2',5',5'-2H4]-3-hydroxy-2,4,5-tri(hydroxymethyl)pyridine (i.e., 2'-hydroxypyridoxol).

Biosynthesis of vitamin B(6) in yeast: incorporation pattern of glucose.

Two yeasts, Saccharomyces cerevisiae ATCC 7752 and Candida utilis ATCC 9256, were incubated in the presence of variously multiply (13)C-labeled samples of D-glucose. The (13)C incorporation pattern within pyridoxamine dihydrochloride, established by (13)C NMR spectroscopy, differed from that which had previously been found within pyridoxine, isolated from Escherichia coli. Thus, the origin of the carbon skeleton of vitamin B(6) in yeast differs substantially from its origin in E.

Evaluating contraceptive choice through the method-mix approach. An Indian Council of Medical Research (ICMR) task force study.

The method-mix approach was used to evaluate informed contraceptive choices in the present study. A total of 8,077 potential clients were given a balanced presentation of all available contraceptive methods in the national program, ie, the CuT 200 intrauterine device (IUD), low-dose combined oral pills (OC), condom, and sterilization (female/male) along with a new method, Norplant(R).(1) The majority of women opted for spacing methods; among them, the IUD was preferred by about 60% of clients, followed by condoms (9%), OC (6%), and Norplant (5%).

Serum bupivacaine concentrations during continuous extrapleural infusion.

Purpose: To determine the rate of increase in serum bupivacaine concentration during continuous extrapleural infusion.

Methods: After thoracotomy for lobectomy under general anaesthesia, nine patients had an extrapleural catheter inserted, before chest closure, in a costovertebral gutter constructed surgically by lifting the parietal pleural. Bupivacaine 0.

Optimization of the determination of 4-aminopyridine in human serum and urine by column liquid chromatography.

Two convenient reversed-phase column liquid chromatographic procedures are described for the determination of 4-aminopyridine in human serum and urine. A 0.5-ml aliquot of serum after the addition of a 0.

Column liquid chromatographic determination of clozapine and N-desmethylclozapine in human serum using solid-phase extraction.

A 0.5-ml aliquot of a serum sample, after the addition of a 50-microliters aliquot of a 5 microgram/ml solution of amoxapine as the internal standard, is vortex-mixed with 0.5 ml of acetonitrile and centrifuged.