Protein Substitute Requirements of Patients with Phenylketonuria on BH4 Treatment: A Systematic Review and Meta-Analysis.

The traditional treatment for phenylketonuria (PKU) is a phenylalanine (Phe)-restricted diet, supplemented with a Phe-free/low-Phe protein substitute. Pharmaceutical treatment with synthetic tetrahydrobiopterin (BH4), an enzyme cofactor, allows a patient subgroup to relax their diet. However, dietary protocols guiding the adjustments of protein equivalent intake from protein substitute with BH4 treatment are lacking. We systematically reviewed protein substitute usage with long-term BH4 therapy. Electronic databases were searched for articles published between January 2000 and March 2020. Eighteen studies (306 PKU patients) were eligible. Meta-analyses demonstrated a significant increase in Phe and natural protein intakes and a significant decrease in protein equivalent intake from protein substitute with cofactor therapy. Protein substitute could be discontinued in 51% of responsive patients, but was still required in 49%, despite improvement in Phe tolerance. Normal growth was maintained, but micronutrient deficiency was observed with BH4 treatment. A systematic protocol to increase natural protein intake while reducing protein substitute dose should be followed to ensure protein and micronutrient requirements are met and sustained. We propose recommendations to guide healthcare professionals when adjusting dietary prescriptions of PKU patients on BH4. Studies investigating new therapeutic options in PKU should systematically collect data on protein substitute and natural protein intakes, as well as other nutritional factors.

Effects of active/passive interventions on pain, anxiety, and quality of life in women with fibromyalgia: Randomized controlled pilot trial.

The authors of this study compared the effects of pilates exercises and connective tissue massage (CTM) on pain intensity; pain-pressure threshold; and tolerance, anxiety, progress, and health-related quality of life in females with fibromyalgia. It was a pilot, assessor masked, randomized controlled trial conducted between January and August of 2013. Twenty-one women with fibromyalgia were randomly assigned to the pilates exercise program (six of whom did not complete the program), and 22 were randomly assigned to CTM (one of whom did not complete this program). Each group received the assigned intervention three times per week during a 4-week period. The Visual Analogue Scale, algometry, State-Trait Anxiety Inventory, Fibromyalgia Impact Questionnaire, and Nottingham Health Profile were used at baseline and at the end of treatments. Significant improvements were found in both groups for all parameters. However, the scores for pain-pressure threshold were significantly elevated and the symptoms of anxiety were significantly diminished in the exercise group compared to the massage group. Thus, exercise and massage might be used to provide improvements in women with fibromyalgia. The exercise group showed more advantages than the massage group and thus might be preferred for patients with fibromyalgia. However, an adequately powered trial is required to determine this with certainty.

The questioning for routine monthly monitoring of proteinuria in patients with ß-thalassemia on deferasirox chelation.

BACKGROUND: Iron chelation therapy is one of the mainstays of the management of the patients with ß-thalassemia (BT) major. Deferasirox is an oral active iron chelating agent. Proteinuria is one of the potential renal adverse effects of deferasirox, and monthly follow-up for proteinuria is suggested by Food and Drug Administration and European Medicine Agency. METHODS: We aimed to investigate the necessity for monthly monitoring for proteinuria among patients with BT on deferasirox. A retrospective laboratory and clinic data review was performed for patients with BT major or intermedia who were treated with deferasirox chelation therapy. All patients were monitored for proteinuria for every 3 or 4 weeks after the initiation of deferasirox with serum creatinine and spot urine protein/creatinine ratios. RESULTS: The median follow-up time of the 37 (36 BT major and one BT intermedia) patients was 44 months. Seven patients (18.9%) developed significant proteinuria (ratio ≥0.8). Of the 1490 measurements, 12 tests (0.8%) were proteinuric. Urine proteinuria resolved in all of the patients during the follow-up. The risk of proteinuria was higher at ages below a cut-off point of 23 years (p = 0.019). Patients, who were on deferasirox at doses above a cut-off dose of 29 mg/kg/day, were found to have higher risk of proteinuria development (p = 0.004). CONCLUSION: Proteinuria resolves without any complication or major intervention according to our results. Potentially more risky groups (age below 23 years old and receivers above a dose of 29 mg/kg/day) might be suggested to be followed monthly, besides monitoring all of the patients.

Investigating the effects of a multidimensional exercise program on symptoms and antiinflammatory status in female patients with ankylosing spondylitis.

OBJECTIVE: The purpose of this study is to investigate the effects of a multidimensional exercise program on symptoms and antiinflammatory status in female patients with ankylosing spondylitis (AS). METHODS: The BATH Indexes, Dougados Functional Index (DFI), Health Assessment Questionnaire in Spondyloarthopathies (HAQ-S), Ankylosing Spondylitis Quality of Life (ASQoL) and Beck Depression Inventory (BDI) were used to evaluate twenty-four female AS patients. ESR, CRP, TNF-α and IL-6 were also analyzed. All patients were assessed at baseline and with 3 weeks intervals till 12 week. A multidimensional exercise program was applied for three times a week. RESULTS: There were significant differences in Bath Ankylosing Spondylitis Global Index (BAS-G) and Disease Activity Index (BASDAI), HAQ-S, ASQoL and BDI scores (p < 0.05). The level of the ESR, CRP and IL-6 fluctuated slightly. There was only significant difference at 3 and 12 weeks as compared to baseline levels in TNF-α values (p = 0.048, p < 0.001). CONCLUSION: We concluded that multidimensional exercise program should be taken into consideration for AS patients due to its positive effects on symptoms and antiinflammatory effects.

The effect of glutamine and arginine-enriched nutritional support on quality of life in head and neck cancer patients treated with IMRT.

BACKGROUND AND AIMS: Oral mucositis and esophagitis are common acute toxicities of radiotherapy (RT) for head and neck cancer (HNC). In order to decrease the rates of these toxicities, we compared quality of life in HNC patients that did and did not receive a glutamine and arginine-enriched solution (GAES) during RT. METHODS: A total of 29 patients received intensity-modulated radiotherapy (IMRT); 15 used GAES b.i.d. during the treatment, and a matched cohort of 14 patients did not. Patients were administered the EORTC QLQ-C30, QLQ-H&N35 and QLQ-OES18 questionnaires on the 1st, 15th, and last days of IMRT. RESULTS: The global health status, functional and symptom scale scores were similar in both groups on the 1st day of IMRT. On the 15th and last days, the scores of social functions (p = 0.01 and p = 0.012), pain (p = 0.002 and p = 0.002), appetite (p = 0.01 and p = 0.02), dry mouth (p = 0.001 and p = 0.03), sticky saliva (p = 0.003 and p = 0.04), trouble with taste (p = 0.001 and p = 0.03), trouble with social eating (p = 0.004 and p = 0.006), and swallowing problems (p = 0.002 and p = 0.046) were significantly worse in the control group. CONCLUSIONS: Quality of life is negatively affected by IMRT; however, use of GAES may mediate this negative effect.

Pollen counts and their relationship to meteorological factors in Ankara, Turkey during 2005-2008.

Pollen plays an important role in the development and exacerbation of allergic diseases. We aimed to investigate the days with highest counts of the most allergenic pollens and to identify the meteorological factors affecting pollen counts in the atmosphere of Ankara, Turkey. Airborne pollen measurements were carried out from 2005 to 2008 with a Burkard volumetric 7-day spore trap. Microscope counts were converted into atmospheric concentrations and expressed as pollen grains/m(3). Meteorological parameters were obtained from the State Meteorological Service. All statistical analyses were done with pollen counts obtained from March to October for each year. The percentages of tree, grass and weed pollens were 72.1% (n = 24,923), 12.8% (n = 4,433) and 15.1% (n = 5,219), respectively. The Pinaceae family from tree taxa (39% to 57%) and the Chenopodiaceae/Amaranthaceae family from weed taxa, contributed the highest percentage of pollen (25% to 43%), while from the grass taxa, only the Poaceae family was detected from 2005 to 2008. Poaceae and Chenopodiaceae/Amaranthaceae families, which are the most allergenic pollens, were found in high numbers from May to August in Ankara. In multiple logistic regression analysis, wind speed (OR = 1.18, CI95% = 1.02-1.36, P = 0.023) for tree pollen, daily mean temperature (OR = 1.10, CI95% = 1.04-1.17, P = 0.001) and sunshine hours (OR = 1.15, CI95% = 1.01-1.30, P = 0.033) for grass pollen, and sunshine hours (OR = 3.79, CI95% = 1.03-13.92, P = 0.044) for weed pollen were found as significant risk factors for high pollen count. The pollen calendar and its association with meteorological factors depend mainly on daily temperature, sunshine hours and wind speed, which may help draw the attention of physicians and allergic patients to days with high pollen counts.

Effect of one-step polishing systems on surface roughness of different flowable restorative materials.

The purpose of this study was to investigate the influence of one-step polishing systems on the surface roughness of different flowable composites and a microhybrid composite. A total of 120 disks were fabricated and divided into six groups according to the different composite restorative materials tested (n = 20). Each group was further divided into four subgroups according to the polishing system (n = 5). For the control group, samples were left undisturbed after removal of Mylar strip. For the other three subgroups, samples were polished with PoGo, OptraPol, or Sof-Lex disks. Surface roughness was determined using a profilometer and observed under scanning electron microscope (SEM). Data were analyzed by one-way ANOVA and Duncan's multiple range test. For Tetric Flow, Grandio Flow, Filtek Supreme XT Flow, and Admira Flow, their lowest surface roughness values were obtained in Mylar Strip and PoGo groups. For Compoglass Flow, there were no significant differences between Mylar Strip, PoGo, and OptraPol. For Filtek Z250, the lowest surface roughness value was obtained with Mylar Strip. In light of the surface roughness results obtained, one-step polishing systems seemed to be a good choice for polishing flowable composites.

Effects of lidocaine with and without epinephrine on plasma epinephrine and lidocaine concentrations and hemodynamic values during third molar surgery.

Lidocaine with epinephrine is currently the most common local anesthetic agent used for impacted third molar surgery. The purpose of the present study was to define the adverse hemodynamic effects and plasma concentrations of lidocaine and epinephrine on 17 healthy patients during the impacted teeth operations. Arterial blood pressure (systolic blood pressure, diastolic blood pressure), heart rate, peripheral oxygen saturation range, and electrocardiography were measured by an automatic noninvasive pressure device and monitor. High-performance liquid chromatography was used to measure the changes of plasma concentrations of epinephrine and lidocaine from blood samples taken 5 different times during the operation. We concluded that lidocaine-epinephrine is effective local anesthetic and had no important adverse events in healthy patients during the third molar surgery.