Intellectual and Developmental Status in Children With Hyperphenylalaninemia and PKU Who Were Screened in a National Program

Background:

Hyperphenylalaninemia (HPA) and Phenylkeonuria (PKU) are metabolic errors caused by deficiency of phenylalanine hydroxylase enzyme, which results in increased level of phenylalanine. This increase is toxic to the growing brain.

Objectives:

The purpose of this study was to compare the intellectual and developmental status in HPA and PKU children with normal population in national screening program.

Patients and Methods:

In a historical cohort study, 41 PKU patients who had the inclusion criteria and 41 healthy children were evaluated. Wechsler preschool and primary scale of intelligence-3rd edition (WPPI-3) was used in order to assess the intellectual status of children 4 years and older and Ages and stages questionnaire (ASQ) was used to assess the developmental status of children 5 years and younger.

Results:

In intellectual test comparison, the two groups showed significant difference in Wechsler’s performance intelligence score and some performance subscales (P-value < 0.01). In comparison of developmental status, no significant difference was observed between the two groups (P-value > 0.05).

Conclusions:

Even with early diagnosis and treatment of PKU patients, these children show some deficiencies intellectually compared to normal children. This study emphasizes on necessity for screening intellectual and developmental status of PKU patients so that effective medical or educational measures can taken in case of deficiencies.     

Cu-MOF: an efficient heterogeneous catalyst for the synthesis of symmetric anhydrides via the C–H bond activation of aldehydes

In this paper, an efficient and straightforward synthetic approach for the preparation of a number of symmetric carboxylic anhydrides was reported using Cu₂(BDC)₂(DABCO) as an efficient heterogeneous catalyst via the C–H bond activation of aldehydes with excellent yields and simple work up. This C–H bond activation reaction appears simple and convenient, has a wide substrate scope and makes use of cheap, abundant, and easily available reagents. The Cu-MOF catalyst was recycled and reused four times without any loss of catalytic activity.

In this paper, an efficient synthetic method for the preparation of symmetric carboxylic anhydrides was reported using Cu₂(BDC)₂(DABCO) as an efficient heterogeneous catalyst via the C–H bond activation of aldehydes with good yields and simple work up.     

Protective effects of Scutellaria lindbergii root extract against oxidative-induced cell and DNA damage in mouse fibroblast-like cells

Context: Scutellaria lindbergii Rech. f. (Lamiaceae) is an Iranian species of Scutellaria which has been shown to exert antimicrobial, antioxidant and cytotoxic effects. Objective: The protective properties of total methanol extract (TME) of S. lindbergii and its fractions (defatted and CH₂Cl₂) were investigated against cytotoxic and genotoxic effects of H₂O₂ in NIH 3T3 cell line as non-malignant cells. Materials and methods: The cells were incubated with different concentrations of S. lindbergii root extracts [TME (15–250?μg ml^?1), defatted fraction (15–500?μg ml^?1) and CH₂Cl₂ fraction (5–40?μg ml^?1)] and toxic concentration of H₂O₂ (200?µM) at 37?°C for 2?h concurrently and Cell viability was quantitated by MTT assay. The antigenotoxic effect of extracts was investigated using comet assay. The cells were incubated with extracts [TME (25–250?μg ml^?1), defatted fraction (25–500?μg ml^?1) and CH₂Cl₂ fraction (5–40?μg ml^?1)] and H₂O₂ (25?µM) at 4?°C for 20?min, then the comet assay was performed. DNA damage was expressed as percentage tail DNA. Results: Total methanol extract of S. lindbergii and its fractions had a significant inhibitory effect on DNA damage. The IC₅₀ values of TME, defatted fraction and CH₂Cl₂ fraction against DNA damage were determined as 48, 138 and 8?μg ml^?1, respectively. Conclusion: S. lindbergii extracts can prevent oxidative DNA damage, which is likely due to its flavonoids and phenolic compounds as antioxidant constituents.     

Cost-Effectiveness of Endovascular Versus Open Repair of Abdominal Aortic Aneurysm: A Systematic Review

Cardiovascular Drugs and Therapy - Abdominal aortic aneurysm (AAA) is a life-threatening condition which, in the absence of increasing diameter or rupture, often remains asymptomatic, and a...     

Histological and mucin histochemical study of the small intestine of the Persian squirrel (Sciurus anomalus)

This article describes the histological and mucin histochemical properties of the small intestine of the Persian squirrel (Sciurus anomalus). This species is widely distributed in the Middle East and can be found as a companion animal. The histological studies revealed that the plicae circulares were not visible in the tunica mucosa. The maximum height and width of the villi were observed in the duodenum, which then decreased toward the ileum. The muscularis mucosa was scattered, whereas the tunica submucosa was composed of dense connective tissue. The lymphatic nodules were seen in the submucosa of the distal part of the jejunum and ileum, and Brunner’s glands were embedded in the initial portion of the duodenum. The tunica muscularis was significantly thicker in the ileum, and the circular muscle layer was thicker than the longitudinal muscle layer throughout the entire length of the small intestine. The mucin histochemistry, which was examined using the periodic acid-Schiff (PAS) and alcian blue (AB) (pH 1.0 and 2.5) and also PAS–AB (pH 2.5) and aldehyde fuchsin-AB (pH 2.5) techniques coupled with methylation and saponification reaction for some sections, showed that the small intestine mucous content included both carboxylated and sulfated acidic mucins with few neutral mucins. The results of this study contribute to the knowledge of the histological and histochemical characteristics of the gastrointestinal tracts of exotic mammals and provide data for comparison with other mammals.     

Does the Nature of Chondrolabral Injury Affect the Results of Open Surgery for Femoroacetabular Impingement?

BackgroundThe degree to which patient characteristics, clinical outcomes, and the nature, severity, and corresponding treatment of chondrolabral injury in femoroacetabular impingement (FAI) is associated with failure after surgery is incompletely understood.Questions/purposes(1) Are patient factors associated with failure (age, sex, body mass index, and preoperative modified Harris hip score [mHHS]) in the open surgical treatment of FAI? (2) Is the nature of chondrolabral injury associated with failure? (3) Are any specific chondrolabral injury treatment methods superior?MethodsBetween 2000 and 2008, 172 open surgical procedures in 167 patients were performed for the treatment of FAI by two surgeons at two separate academic medical centers. Ultimately, 142 patients were included in this retrospective study. Mean followup was 3 years (range, 1–12 years). Patient and clinical factors along with the nature, severity, and treatment of chondrolabral injuries were assessed for an association with failure, defined as conversion to THA or the inability to achieve the minimum clinically important difference of the mHHS. Thirty-two percent (45 of 142) of patients failed open surgical treatment of FAI.ResultsPatient factors associated with failure included age (odds ratio [OR], 1.04; p = 0.036) and preoperative mHHS (OR, 4.42; p = 0.033). Neither the nature of the labral lesion nor the severity of the chondral lesion demonstrated a relationship with failure (p > 0.05). Surgically, labral refixation was associated with a decrease in the risk of failure (OR, 0.31; p = 0.039).ConclusionsWe were unable to identify an increased risk of poor outcomes based on sex, body mass index, or severity of chondrolabral lesions. We did find an increased risk of poorer outcomes associated with age. Labral refixation was associated with a decrease in the risk of failure indicating that treatment methodology, rather than the nature of the chondrolabral injury, may be associated with clinical failure. Future studies will be needed to help determine optimal treatment strategies for chondrolabral injuries.

Level of Evidence

Level IV, therapeutic study.