The Prevalence of Noonan Spectrum Disorders in Pediatric Patients with Pulmonary Valve Stenosis.

OBJECTIVE: To investigate the prevalence of Noonan spectrum disorders in a pediatric population with pulmonary valve stenosis (PVS) and explore other characteristics of Noonan spectrum disorders associated with PVS. STUDY DESIGN: A retrospective medical record review was completed for patients with a diagnosis of PVS seen at the Children's Hospital Colorado Cardiology clinic between 2009 and 2019. Syndromic diagnoses, genotypes, cardiac characteristics, and extracardiac characteristics associated with Noonan spectrum disorders were recorded; statistical analysis was conducted using R. RESULTS: Syndromic diagnoses were made in 16% of 686 pediatric patients with PVS, with Noonan spectrum disorders accounting for 9% of the total diagnoses. Individuals with Noonan spectrum disorders were significantly more likely to have an atrial septal defect and/or hypertrophic cardiomyopathy than the non-Noonan spectrum disorder individuals. Supravalvar pulmonary stenosis was also correlated significantly with Noonan spectrum disorders. Extracardiac clinical features presenting with PVS that were significantly associated with Noonan spectrum disorders included feeding issues, failure to thrive, developmental delay, short stature, and ocular findings. The strongest predictors of a Noonan spectrum disorder diagnosis were cryptorchidism (70%), pectus abnormalities (66%), and ocular findings (48%). The presence of a second characteristic further increased this likelihood, with the highest probability occurring with cryptorchidism combined with ocular findings (92%). CONCLUSIONS: The 9% prevalence of Noonan spectrum disorder in patients with PVS should alert clinicians to consider Noonan spectrum disorders when encountering a pediatric patient with PVS. The presence of PVS with 1 or more Noonan spectrum disorder-related features should prompt a genetic evaluation and genetic testing for RAS pathway defects. Noonan spectrum disorders should also be included in the differential when a patient presents with supravalvar pulmonary stenosis.

Registered nurses' knowledge and practice of preoperative fasting and medication administration.

Background: Knowledge of fasting or Nil Per Os (NPO) guidelines is an essential component of nursing care in the preoperative period. Aim: To describe registered nurses' (RNs) knowledge and management of the preoperative NPO period. Setting: Selected surgical wards in a tertiary hospital in the Western Cape, South Africa. Methods: Quantitative descriptive, cross-sectional study utilising a structured questionnaire. The population consisted of RNs working in selected surgical wards. Convenience sampling was used and adequate knowledge was determined as ≥ 90%. Results: The response rate was 100%. Of the 68 participants, 48 (70.6%) held a diploma and 20 (29.4%) held a degree as the highest academic qualification achieved. Sixty-one (89.7%) participants knew the correct reason for keeping patients NPO. Sixty-five (95.6%) knew the correct answer for the NPO time for solids while only 27 (39.7%) knew the correct answer for clear fluids. Only 30 (44.1%), 26 (38.2%) and 33 (48.5%) participants, respectively, answered the questions about oral analgesia, oral antibiotics and chronic medication administration during the NPO period correctly. Significantly more degree participants knew the correct answer for the fasting time for non-human milk (p = 0.005) and more diploma participants would administer chronic medication during the NPO period (p = 0.037). Conclusion: Inadequate knowledge of NPO times for various fluids and unsatisfactory practice of medication administration for oral and chronic medication require attention. Contribution: This study highlights the importance that ongoing education is needed to ensure that patients receive the most up-to-date evidence-based care during the NPO period.

Exploring the culturally sensitive sexual and reproductive health information communication skill needs of parents in Ghana.

BACKGROUND: Parents play a vital role in the sexual and reproductive health (SRH) of adolescents. Parents' communication with their adolescents regarding SRH is considered an important part of adolescent development, as this contributes to optimising safe SRH. AIM: This phase of the study explored the culturally sensitive SRH information communication skill needs of parents, based on their personal and social motivation, within the Ghanaian context. SETTING: The study was conducted at the Asante Akyem North Municipality of Ghana. METHODS: This article describes the second phase of an explanatory, sequential, mixed-method study. Following on from the first phase systematic review, this second phase comprised a qualitative descriptive study where 10 purposively sampled parents of adolescents participated in semi-structured interviews to elicit data. Braun and Clarke's thematic data analysis process was applied. Data were saved and managed in Atlas.ti (version 23.0.7). RESULTS: Four themes emerged on communication skills: SRH information, parent and adolescent factors, contextual factors and communication skill needs. Parent and adolescent communication on SRH takes place occasionally. Parents lack the skills to communicate with adolescents regarding SRH. CONCLUSION: Parents in this context require skills to communicate SRH information with their adolescent children. A culturally appropriate intervention that supports SRH information communication between parents and adolescents may have value in guiding this communication process.Contribution: The findings of this study can contribute to the adaptation of a culturally sensitive SRH information communication intervention in Ghana which will promote adolescent SRH.

Additional feeding assistance improves the energy and protein intakes of hospitalised elderly patients. A health services evaluation.

Malnutrition is a serious issue that is prevalent in elderly hospitalised patients. Traditionally the role of feeding was designated to the nurse; however competing tasks mean that additional support for feeding assistance is needed. A program that utilises volunteers during weekday lunchtimes to assist, feed and socialise with patients at a Sydney hospital began during 2005. Twenty-three patients (mean age: 83.2±8.9years) participated in this study. Observations and weighed plate waste were recorded for each patient for all meals on two weekdays (when volunteers present) and two weekend days (when volunteers not present). Grip strength, Mini-Nutritional Assessments and interviews were conducted with patients, and surveys with volunteers and staff. Lunchtime energy and protein intakes increased significantly (396 kJ and 4.3g respectively) when volunteers were present. Volunteers spent an average of 12.3 min with each patient at lunchtime, compared to 4.7 min for nurses. Nurses indicated time barriers to feeding patients but were positive about the value of the program. Volunteers were commonly observed feeding, setting up meals and providing encouragement to patients. Additional feeding assistance is one effective strategy to increase the energy and protein intakes and combat malnutrition in elderly inpatients.

Evaluation of dried blood spot testing using the Abbott Alinity i.

BACKGROUND: The West of Scotland Specialist Virology Centre currently uses the Abbott Architect for DBS serology. The new Abbott Alinity i will replace the Architect in our laboratory. In this study, mock and stored patient DBS samples were tested on both platforms and results compared. STUDY DESIGN: Mock DBS were made from whole blood where patient results were known (38 negative samples and 141 positive samples; 39 HIV Antigen/Antibody (Ag/Ab), 35 HCV IgG antibody (HCVG), 34 HBV core IgG (HBCG) and 33 HBsAg). Mock DBS were tested on both Abbott platforms. Stored patient DBS samples (132 negative and 263 positive: 9 HIVAg/Ab, 10 HBsAg, 52 HBCG and 60 HCVG) previously tested on the Architect were retested on the Alinity i. RESULTS: Mock DBS showed good correlation between the Architect and Alinity i for the HIV Ag/Ab,HBCG and HCVG assays. A poorer correlation occurred with HBsAg, the Alinity i reported HBsAg positives at a lower value compared to the Architect. The coefficient of variation for intra-assay variation was 1.69 % (HIVAg/Ab), 3.25 % (HCVG), 1.68 % (HBsAg) and 1.95 % (HBCG). The sensitivity and specificity was determined based on results from the mock and patient samples. At S/Co cut-off 1.0 both HIV and HBsAg had a sensitivity of 100 %. A cut-off 0.8 gave a sensitivity of 95.83 % (95 % CI 89.67%-98.85%) for HCVG and 0.3 gave a sensitivity of 98.8 % (CI 93.69%-99.97%) for HBCG. DISCUSSION: The alinity i compared well against the architect and can be used to test DBS samples.

Elderly women need dietary protein to maintain bone mass.

Excess dietary protein is considered a risk factor for osteoporosis owing to the potential for renal acid load. Researchers who conducted a recent prospective study of older adults reported that animal protein had a protective role for bone, especially in elderly women, whereas plant protein was negatively associated with bone mineral density. An interaction between protein and calcium suggested protein alone was not the important factor. Other studies confirm the beneficial effect of increasing dietary protein intake in older women to reduce bone mineral density loss and risk of fracture, suggesting that emphasis should be placed on promoting adequate protein intake in elderly women.

Effect of fruit on net acid and urinary calcium excretion in an acute feeding trial of women.

OBJECTIVE: Consumption of fruits and vegetables has been implicated in lowering net acid excretion (NAE), but few studies have directly examined NAE and urinary calcium effects. Further, there is no evidence that only fresh fruits and vegetables must be consumed for a beneficial effect on bone. METHODS: A crossover, acute-load study was designed to investigate whether processed fruit was as effective as fresh fruit in reducing NAE and protein-induced hypercalciuria. Fifteen women completed three dietary treatments on three different mornings. A fasting urine sample was collected before consuming one of the following three isocaloric high-protein treatments: control, fresh apples, and processed applesauce. The serving size for the applesauce treatment was 2.5 times that for fresh apples. Urine was collected at baseline (0 h) and at 1.5, 3.0, and 4.5 h. RESULTS: Compared with baseline, NAE increased after control treatment but decreased after fresh or processed apple treatment (P = 0.041). Calcium excretion increased with all treatments by 3 h; however, the increase was less for fresh apple and applesauce (P = 0.024). CONCLUSIONS: In an acute feeding model, fruit intake reduced NAE and urinary calcium excretion. Processed fruit appears to be effective, although a larger serving size was needed than with fresh fruit.

Health promotion for adolescents in primary care: randomised controlled trial.

OBJECTIVES: To evaluate the effectiveness of inviting teenagers to general practice consultations to discuss health behaviour concerns and appropriate follow up care. DESIGN: Randomised controlled trial, with participants randomised to a consultation (intervention) or usual care (control). Questionnaires completed at baseline, 3 months, and 12 months. SETTING: Eight general practices in Hertfordshire, England. PARTICIPANTS: 1516 teenagers aged 14-15 years. INTERVENTION: Consultations with practice nurses to discuss health concerns and develop plans for healthier lifestyles. MAIN OUTCOME MEASURES: Mental and physical health, "stage of change" for health related behaviour, and use of health services. RESULTS: At baseline 970 teenagers completed questionnaires; 23% smoked, 35% had been drunk in the previous three months, 64% considered they ate unhealthily, 39% took little exercise, and 36% had possible depression. 41% (304) of teenagers invited attended for a consultation; over one third (112) were offered follow up care. More intervention group teenagers reported positive movement in stage of change for diet and exercise and in at least one of four behaviours (diet, exercise, smoking, drinking alcohol) at 3 months (41% v 31%, P<0.01), but this did not persist at 12 months. There was marginally more positive change in actual behaviour by intervention teenagers at 3 months (16% v 12%, P=0.06). Recognition of possible depression resulted in improved mental health outcomes at 3 and 12 months. 97% of attenders said they would recommend the intervention to a friend. CONCLUSIONS: Change in behaviour was slight but encouraging, and the intervention was well received and relatively cheap.