Implementing a Transition Program from Paediatric to Adult Services in Phenylketonuria: Results after Two Years of Follow-Up with an Adult Team

We aimed to report the implementation of a phenylketonuria (PKU) transition program and study the effects of follow-up with an adult team on metabolic control, adherence, and loss of follow-up. Fifty-five PKU patients were analysed in the study periods (SP): 2 years before (SP1) and after the beginning of adult care (SP2). Retrospective data on metabolic control and number of clinic appointments were collected for each SP, and protein intakes were analysed. In SP2, three patients (6%) were lost to follow-up. There was a small but statistically significant increase in median number of annual blood spots from SP1 to SP2: 11 (7–15) vs. 14 (7–20); p = 0.002. Mean ± SD of median blood Phe remained stable (525 ± 248 µmol/L vs. 552 ± 225 µmol/L; p = 0.100); median % of blood Phe < 480 µmol/L decreased (51 (4–96)% vs. 37 (5–85)%; p = 0.041) and median number of clinic appointments increased from SP1 to SP2: (5 (4–6) vs. 11 (8–13); p < 0.001). No significant differences were found regarding any parameter of protein intake. Our results suggest that the implementation of an adult service was successful as impact on metabolic control was limited and attendance remained high. Continuous dietetic care likely contributed to these results by keeping patients in follow-up and committed to treatment.     

Adherence to Mediterranean Diet and Selected Lifestyle Elements among Young Women with Type 1 Diabetes Mellitus from Northeast Poland: A Case-Control COVID-19 Survey

An appropriate balanced diet and dietary patterns are important at every stage of life, but in the case of young patients with type 1 diabetes mellitus (T1DM), it is especially crucial during the COVID-19 pandemic. The aim of the study was to assess health and nutritional behaviors, mainly adherence to the Mediterranean diet (MD), during the second wave of the COVID-19 pandemic in Poland among women with T1DM, and to compare them with a healthy population. This survey (based on a questionnaire) was conducted in December 2020 and included 219 young women, healthy (n = 106) and with T1DM (n = 113), from northeast Poland. Over 30% of the study group admitted that they did not engage in any physical activity. A large proportion declared that their screen time was 5–7 h a day (48% in control and 40% in T1DM group). High intakes of sweet-beverages, sweets and red meat, but also low intakes of olive oil, fish and nuts were observed. The vast majority of participants (60% vs. 71%) were moderately adherent to the Mediterranean Diet Adherence Screener (MEDAS). The study demonstrated that despite the similarity between the behaviors of healthy people and those with T1DM, negative health and nutritional practices, such as low physical activity, long screen time, medium and high levels of stress and inappropriate eating habits were observed.     

A Three-Year Longitudinal Study Comparing Bone Mass,Density, and Geometry Measured by DXA,pQCT, and Bone Turnover Markers in Children with PKU Taking L-Amino Acid or Glycomacropeptide Protein Substitutes

In patients with phenylketonuria (PKU), treated by diet therapy only, evidence suggests that areal bone mineral density (BMDa) is within the normal clinical reference range but is below the population norm. Aims: To study longitudinal bone density, mass, and geometry over 36 months in children with PKU taking either amino acid (L-AA) or casein glycomacropeptide substitutes (CGMP-AA) as their main protein source. Methodology: A total of 48 subjects completed the study, 19 subjects in the L-AA group (median age 11.1, range 5–16 years) and 29 subjects in the CGMP-AA group (median age 8.3, range 5–16 years). The CGMP-AA was further divided into two groups, CGMP100 (median age 9.2, range 5–16 years) (n = 13), children taking CGMP-AA only and CGMP50 (median age 7.3, range 5–15 years) (n = 16), children taking a combination of CGMP-AA and L-AA. Dual X-ray absorptiometry (DXA) was measured at enrolment and 36 months, peripheral quantitative computer tomography (pQCT) at 36 months only, and serum blood and urine bone turnover markers (BTM) and blood bone biochemistry at enrolment, 6, 12, and 36 months. Results: No statistically significant differences were found between the three groups for DXA outcome parameters, i.e., BMDa (L2–L4 BMDa g/cm²), bone mineral apparent density (L2–L4 BMAD g/cm³) and total body less head BMDa (TBLH g/cm²). All blood biochemistry markers were within the reference ranges, and BTM showed active bone turnover with a trend for BTM to decrease with increasing age. Conclusions: Bone density was clinically normal, although the median z scores were below the population mean. BTM showed active bone turnover and blood biochemistry was within the reference ranges. There appeared to be no advantage to bone density, mass, or geometry from taking a macropeptide-based protein substitute as compared with L-AAs.     

Drug-Resistant Tuberculosis in Pet Ring-Tailed Lemur,Madagascar

We diagnosed tuberculosis in an illegally wild-captured pet ring-tailed lemur manifesting lethargy, anorexia, and cervical lymphadenopathy. Whole-genome sequencing confirmed the Mycobacterium tuberculosis isolate belonged to lineage 3 and harbored streptomycin resistance. We recommend reverse zoonosis prevention and determination of whether lemurs are able to maintain M. tuberculosis infection.     

Efficacy of vaccination with StroVac for recurrent urinary tract infections in women: a comparative single-centre study

International Urology and Nephrology - To assess the efficacy of prophylaxis for urinary tract infections (UTI) in a two-year follow-up in women with StroVac compared to a therapy with...     

Factors Influencing the Sex Ratio at Birth in India: A New Analysis based on Births Occurring between 2005 and 2016

Previous research on sex ratio at birth (SRB) in India has largely relied on macro‐analysis of census data that do not contain the breadth of factors needed to explain patterns in SRB. Additionally, no previous research has examined the differentiation of factors associated with SRB across birth orders, a key determinant in societies affected by son preference. This study aims to fill these gaps using micro‐data related to 553,461 births occurring between 2005 and 2016 collected as part of the 2015–2016 National Family Health Survey. Analyses used multivariable logistic regressions stratified by birth order to examine associations with SRB at the national level. The SRB at birth order 1 was outside the biological normal limit, and generally increased with birth order. First births in households with wealth in the middle and richest quintiles, with mothers who desired a higher ideal number of sons than daughters, and in lower fertility communities had a higher probability of being male. Most SRB correlates were visible at birth orders 3 or higher. Programs and policies designed to address India's male‐skewed SRB must consider the diverse factors that influence SRB, particularly for higher order births.