The added value of the electrocardiogram in Noonan syndrome

Abstract Noonan syndrome is a genetic disorder characteried by short stature, typical facial features, developmental delay, and CHD. In this single-centre retrospective study, we analysed typical Noonan syndrome-related electrocardiographic features in 95 patients with clinically and molecularly confirmed Noonan syndrome. Typical Noonan syndrome-related electrocardiographic features are left axis deviation, small left precordial R-waves, large right precordial S-waves, abnormal Q-wave, and abnormal wide QRS complex. In this representative cohort, CHD was found in 59 patients (62.1%) and typical Noonan syndrome-related electrographic features in 60 patients (63.2%). The typical Noonan syndrome-related electrographic features were also increased over baseline in patients without CHD (41.7%). Of all 95 patients, left axis deviation was seen in 46.3%, small left precordial R-waves in 30.5%, large right precordial S-waves in 5.3%, and abnormal Q-wave and wide QRS complex in 2.1%. There was no significant difference in the frequency of the individual-specific electrographic features between the group with CHD and the group without CHD. However, there were significantly more patients with a small left precordial R-wave in the subgroup with pulmonary stenosis compared to patients without pulmonary stenosis. Conclusion: Specific Noonan syndrome-related electrographic features are frequently present in patients with Noonan syndrome, also in the absence of CHD. These results suggest that there may be a continuum of cardiac anomalies from overt CHD to milder abnormalities that are only seen on electrocardiogram.

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Case Presentation - Biventricular Hypertrophy and Valvular Pulmonary Stenosis in Adult Patient with Noonan Syndrome: A Rare Case

Cardiovascular and Cardiometabolic Journal (CCJ) ◽

10.20473/ccj.v2i2.2021.77-83 ◽

2021 ◽

Vol 2 (2) ◽

pp. 77

Author(s):

Tinton Pristianto ◽

Rosi Amrilla Fagi

Keyword(s):

Noonan Syndrome ◽

Pulmonary Valve ◽

Genetic Disorder ◽

Pulmonary Stenosis ◽

Pulmonary Regurgitation ◽

Nasolabial Fold ◽

Pulmonary Valve Stenosis ◽

Case Presentation ◽

Valvular Pulmonary Stenosis ◽

Chest Deformities

Introduction: Noonan syndrome (NS) is a genetic disorder often accompanied by multiple congenital abnormalities. The prevalence of NS at live birth has been reported as one in 1000-2500 individuals. About 80% of patients with Noonan syndrome have abnormalities in the cardiovascular system.Case presentation:41-year-old Javanese male presented with chief complaint shortness of breath. His Body Mass Index (BMI) was 18,3. He had an oval-shaped face with a short neck, thin hair, and prominent nasolabial fold. Echocardiography showed biventricular hypertrophy alongside pulmonary valve stenosis, pulmonary regurgitation and minimal pericardial effusion. Discussion: In 1962, Jacqueline Noonan, a pediatric cardiologist, identified 9 patients whose faces were very similar, had short stature, significant chest deformities, and with pulmonary stenosis. Noonan syndrome is a relatively common non-chromosomal syndrome that is similar to the phenotype of Turner's syndrome and presents with cardiovascular malformations. Adult with NS has distinctive facial features such as ptosis, wide eyes, low posterior rotation of ears and helical thickening, and a wide neck.Pulmonary stenosis is the most common heartdefect found in NS, besides HCM isalsoquitecommon inabout20% of patients. We reported a case of a patient with typical characteristics of NS such as pulmonary valve stenosis accompanied by biventricular ventricular hypertrophyand its typical face who survived through adulthood.Conclusion: Syndrome Noonan in the adult is quite rare and difficult to diagnose. We reported a case of an adult man with facial appearance and echocardiographic findings identical with Noonan Syndrome.

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Electrocardiography may be a useful tool in the diagnosis of early mild canine heartworm disease

Acta Veterinaria Brno ◽

10.2754/avb201180010081 ◽

2011 ◽

Vol 80 (1) ◽

pp. 81-85

Author(s):

Edward M. Onyango

Keyword(s):

Axis Deviation ◽

Qrs Complex ◽

S Wave ◽

Additional Indication ◽

Canine Heartworm ◽

Canine Heartworm Disease ◽

Heartworm Disease ◽

Lead Avr ◽

Significant Attenuation ◽

Q Wave

This study was carried out to determine the usefulness of standard electrocardiography for the diagnosis of early canine heartworm disease. Baseline electrocardiograms were recorded in 12 dogs. Thirty artificial Dirofilaria immitis worms were inserted in the pulmonary artery of each dog. New electrocardiograms were recorded on days 1-4, 7, 14, 21, 28 and 35 after insertion of worms. A significant attenuation of amplitude of Q wave in lead I was recorded on days 1-4, 7, 14, 21, 28 and 35; of R wave in lead II on days 21, 28 and 35; and of S wave in lead aVR on days 7, 14, 21, 28 and 35. Duration of the QRS complex in lead AVF was significantly prolonged on day 14. In 41.6% of the dogs, right axis deviation was recorded. These results suggest that for a dog whose normal baseline standard electrocardiogram is known, a comparison of the QRS complex of the baseline with those of subsequent standard electrocardiograms may suggest early canine heartworm disease when there is an attenuation of amplitude of Q wave in lead I, R wave in lead II and S wave in lead aVR. An additional indication can be right axis deviation.

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Noonan syndrome and pregnancy outcomes

Cardiology in the Young ◽

10.1017/s104795112100514x ◽

2022 ◽

pp. 1-5

Author(s):

Christopher A. Chow ◽

Katherine H. Campbell ◽

Josephine C. Chou ◽

Robert W. Elder

Keyword(s):

Caesarean Section ◽

Noonan Syndrome ◽

Neonatal Intensive Care ◽

Pulmonary Valve ◽

Heart Defects ◽

Genetic Disorder ◽

Pulmonary Stenosis ◽

Retrospective Chart Review ◽

Maternal Complications ◽

Cardiac Procedures

Abstract Background: Noonan syndrome is a genetic disorder with high prevalence of congenital heart defects, such as pulmonary stenosis, atrial septal defect and hypertrophic cardiomyopathy. Scarce data exists regarding the safety of pregnancy in patients with Noonan syndrome, particularly in the context of maternal cardiac disease. Study design: We performed a retrospective chart review of patients at Yale-New Haven Hospital from 2012 to 2020 with diagnoses of Noonan syndrome and pregnancy. We analysed medical records for pregnancy details and cardiac health, including echocardiograms to quantify maternal cardiac dysfunction through measurements of pulmonary valve peak gradient, structural heart defects and interventricular septal thickness. Results: We identified five women with Noonan syndrome (10 pregnancies). Three of five patients had pulmonary valve stenosis at the time of pregnancy, two of which had undergone cardiac procedures. 50% of pregnancies (5/10) resulted in pre-term birth. 80% (8/10) of all deliveries were converted to caesarean section after a trial of labour. One pregnancy resulted in intra-uterine fetal demise while nine pregnancies resulted in the birth of a living infant. 60% (6/10) of livebirths required care in the neonatal intensive care unit. One infant passed away at 5 weeks of age. Conclusions: The majority of mothers had pre-existing, though mild, heart disease. We found high rates of prematurity, conversion to caesarean section, and elevated level of care. No maternal complications resulted in long-term morbidity. Our study suggests that women with Noonan syndrome and low-risk cardiac lesions can become pregnant and deliver a healthy infant with counselling and risk evaluation.

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Writing in Aphasia Rehabilitation: Cursive vs Manuscript

Journal of Speech and Hearing Disorders ◽

10.1044/jshd.4104.523 ◽

1976 ◽

Vol 41 (4) ◽

pp. 523-529 ◽

Cited By ~ 3

Author(s):

Daniel R. Boone ◽

Harold M. Friedman

Keyword(s):

Correct Response ◽

The Other ◽

Read Aloud ◽

Writing Style ◽

Reading And Writing ◽

Manual Responses ◽

Significant Difference ◽

Written Form ◽

The Individual ◽

Read Number

Reading and writing performance was observed in 30 adult aphasic patients to determine whether there was a significant difference when stimuli and manual responses were varied in the written form: cursive versus manuscript. Patients were asked to read aloud 10 words written cursively and 10 words written in manuscript form. They were then asked to write on dictation 10 word responses using cursive writing and 10 words using manuscript writing. Number of words correctly read, number of words correctly written, and number of letters correctly written in the proper sequence were tallied for both cursive and manuscript writing tasks for each patient. Results indicated no significant difference in correct response between cursive and manuscript writing style for these aphasic patients as a group; however, it was noted that individual patients varied widely in their success using one writing form over the other. It appeared that since neither writing form showed better facilitation of performance, the writing style used should be determined according to the individual patient’s own preference and best performance.

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Aftermath Effect Of GST On Consumer Purchasing Power

Restaurant Business ◽

10.26643/rb.v118i5.7657 ◽

2019 ◽

Vol 118 (5) ◽

pp. 122-131

Author(s):

S. Thowseaf ◽

M. Ayisha Millath ◽

K. Malik Ali

Keyword(s):

Living Standard ◽

Purchasing Power ◽

Percentage Increase ◽

Defense System ◽

Key Factors ◽

Predominant Role ◽

Tax System ◽

Significant Difference ◽

The Individual ◽

Taxation System

Tax is an important source of income for the country. It is through tax; country strengthens its defense system, infrastructure, and government. Hence, tax system plays a predominant role in developing country’s economy. The complication in taxation system and liberty for taxpayers are key factors generating loopholes for corruption. GST is superior taxation system over VAT but, if neither properly implemented nor scrutinized according to the economy, it is people residing get affected. GST taxation system is capable of increasing legal transaction, reducing corruption and complexity that exists in current taxation. India is 166th country to adopt GST and GST taxation slab in India is 0%, 5%, 12%, 18% and 28%. Although average Tax levied is 14.8750% in India, it is 28% tax that is levied for most of the commodities, which are directly or indirectly used in everyday life of common individuals. Despite, GST being favorable to distributor in-terms of profit and government to attain tax by increasing legal transaction through invoice. It is noted that for the same percentage of taxation, the amount does not vary for VAT and GST. The tax slab decreased for 71 commodities and no change in 21 commodities; there has been an increase in tax slab for 60 commodities. 26% taxation was levied for most commodities considered was currently levied by 28% taxation which is greater than before. It was found that average tax percentage reduced was calculated to be 6.07143. The average tax percentage increase was calculated to be 4.7833 percentage for the considered commodities. The overall tax average tax percentage is estimated to be 14.8750% which does not have a significant difference concerning tax levied before GST, which was calculated to be 15.7829% for considered commodities. Therefore, the consumer purchasing power and overall living standard of the individual in India will remain almost same.

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A comparison between Asians and Caucasians in the dimensions of the femoral isthmus based on a 3D-CT analysis of 1189 adult femurs

European Journal of Trauma and Emergency Surgery ◽

10.1007/s00068-021-01740-x ◽

2021 ◽

Author(s):

Darius M. Thiesen ◽

Dimitris Ntalos ◽

Alexander Korthaus ◽

Andreas Petersik ◽

Karl-Heinz Frosch ◽

...

Keyword(s):

Proximal Femur ◽

Strong Predictor ◽

Matched Pair ◽

3D Ct ◽

Femur Fractures ◽

Matched Pair Analysis ◽

Significant Difference ◽

Ct Analysis ◽

Ct Data ◽

The Individual

Abstract Introduction For successful intramedullary implant placement at the femur, such as nailing in unstable proximal femur fractures, the use of an implant that at least reaches or exceeds the femoral isthmus and yields sufficient thickness is recommended. A number of complications after intramedullary femoral nailing have been reported, particularly in Asians. To understand the anatomical features of the proximal femur and their ethnic differences, we aimed to accurately calculate the femoral isthmus dimensions and proximal distance of Asians and Caucasians. Methods In total, 1189 Asian and Caucasian segmented 3D CT data sets of femurs were analyzed. The individual femoral isthmus diameter was precisely computed to investigate whether gender, femur length, age, ethnicity or body mass index have an influence on isthmus diameters. Results The mean isthmus diameter of all femurs was 10.71 ± 2.2 mm. A significantly larger diameter was found in Asians when compared to Caucasians (p < 0.001). Age was a strong predictor of the isthmus diameter variability in females (p < 0.001, adjusted r2 = 0.299). With every year of life, the isthmus showed a widening of 0.08 mm in women. A Matched Pair Analysis of 150 female femurs showed a significant difference between isthmus diameter in Asian and Caucasian femurs (p = 0.05). In 50% of the cases the isthmus was found in a range of 2.4 cm between 16.9 and 19.3 cm distal to the tip of the greater trochanter. The female Asian femur differs from Caucasians as it is wider at the isthmus. Conclusions In absolute values, the proximal isthmus distance did not show much variation but is more proximal in Asians. The detailed data presented may be helpful in the development of future implant designs. The length and thickness of future standard implants may be considered based on the findings.

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Limited predictive value of illness perceptions for short-term poor recovery in musculoskeletal pain. A multi-center longitudinal study

BMC Musculoskeletal Disorders ◽

10.1186/s12891-021-04366-7 ◽

2021 ◽

Vol 22 (1) ◽

Author(s):

E. J. de Raaij ◽

H. Wittink ◽

J. F. Maissan ◽

P. Westers ◽

R. W. J. G. Ostelo

Keyword(s):

Prognostic Factors ◽

Pain Intensity ◽

Musculoskeletal Pain ◽

Physical Functioning ◽

Predictive Value ◽

Illness Perceptions ◽

Illness Perception ◽

Added Value ◽

Poor Recovery ◽

Significant Difference

Abstract Background Musculoskeletal pain (MSP) is recognized worldwide as a major cause of increased years lived with disability. In addition to known generic prognostic factors, illness perceptions (IPs) may have predictive value for poor recovery in MSP. We were interested in the added predictive value of baseline IPs, over and above the known generic prognostic factors, on clinical recovery from MSP. Also, it is hypothesized there may be overlap between IPs and domains covered by the Four-Dimensional Symptom Questionnaire (4DSQ), measuring distress, depression, anxiety and somatization. The aim of this study is twofold; 1) to assess the added predictive value of IPs for poor recovery and 2) to assess differences in predictive value for poor recovery between the Brief Illness Perception Questionnaire - Dutch Language Version (Brief IPQ-DLV) and the 4DSQ. Methods An eligible sample of 251 patients with musculoskeletal pain attending outpatient physical therapy were included in a multi-center longitudinal cohort study. Pain intensity, physical functioning and Global Perceived Effect were the primary outcomes. Hierarchical logistic regression models were used to assess the added value of baseline IPs for predicting poor recovery. To investigate the performance of the models, the levels of calibration (Hosmer-Lemeshov test) and discrimination (Area under the Curve (AUC)) were assessed. Results Baseline ‘Treatment Control’ added little predictive value for poor recovery in pain intensity [Odds Ratio (OR) 0.80 (Confidence Interval (CI) 0.66–0.97), increase in AUC 2%] and global perceived effect [OR 0.78 (CI 0.65–0.93), increase in AUC 3%]. Baseline ‘Timeline’ added little predictive value for poor recovery in physical functioning [OR 1.16 (CI 1.03–1.30), increase in AUC 2%]. There was a non-significant difference between AUCs in predictive value for poor recovery between the Brief IPQ-DLV and the 4DSQ. Conclusions Based on the findings of this explorative study, assessing baseline IPs, over and above the known generic prognostic factors, does not result in a substantial improvement in the prediction of poor recovery. Also, no recommendations can be given for preferring either the 4DSQ or the Brief IPQ-DLV to assess psychological factors.

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A Comparison of Colectomy Outcomes Utilizing Open, Laparoscopic, and Robotic Techniques

The American Surgeon ◽

10.1177/0003134820973384 ◽

2020 ◽

pp. 000313482097338

Author(s):

Elizabeth McCarthy ◽

Benjamin L. Gough ◽

Michael S. Johns ◽

Alexandra Hanlon ◽

Sachin Vaid ◽

...

Keyword(s):

Postoperative Recovery ◽

Logistic Regression Models ◽

Community Institution ◽

Physical Status Classification ◽

Estimated Blood Loss ◽

Comparative Review ◽

Significant Difference ◽

American Society ◽

The Individual ◽

American Society Of Anesthesiologists

Introduction Robotic colectomy could reduce morbidity and postoperative recovery over laparoscopic and open procedures. This comparative review evaluates colectomy outcomes based on surgical approach at a single community institution. Methods A retrospective review of all patients who underwent colectomy by a fellowship-trained colon and rectal surgeon at a single institution from 2015 through 2019 was performed, and a cohort developed for each approach (open, laparoscopic, and robotic). 30-day outcomes were evaluated. For dichotomous outcomes, univariate logistic regression models were used to quantify the individual effect of each predictor of interest on the odds of each outcome. Continuous outcomes received a similar approach; however, linear and Poisson regression modeling were used, as appropriate. Results 115 patients were evaluated: 14% (n = 16) open, 44% (n = 51) laparoscopic, and 42% (n = 48) robotic. Among the cohorts, there was no statistically significant difference in operative time, rate of reoperation, readmission, or major complications. Robotic colectomies resulted in the shortest length of stay (LOS) (Kruskal-Wallis P < .0001) and decreased estimated blood loss (EBL) (Kruskal-Wallis P = .0012). Median age was 63 years (interquartile range [IQR] 53-72). 54% (n = 62) were female. Median American Society of Anesthesiologists physical status classification was 3 (IQR 2-3). Median body mass index was 28.67 (IQR 25.03-33.47). A malignant diagnosis was noted on final pathology in 44% (n = 51). Conclusion Among the 3 approaches, there was no statistically significant difference in 30-day morbidity or mortality. There was a statistically significant decreased LOS and EBL for robotic colectomies.

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Computed tomography angiography and microsurgical flaps for traumatic wounds: What is the added value?

Clinical Hemorheology and Microcirculation ◽

10.3233/ch-200986 ◽

2021 ◽

pp. 1-9

Author(s):

Lucas Sousa Macedo ◽

Renato Polese Rusig ◽

Gustavo Bersani Silva ◽

Alvaro Baik Cho ◽

Teng Hsiang Wei ◽

...

Keyword(s):

Computed Tomography ◽

Upper Limb ◽

Computed Tomography Angiography ◽

Lower Limb ◽

High Energy ◽

Lower Limbs ◽

Added Value ◽

Vascular Lesions ◽

Limb Injuries ◽

Significant Difference

BACKGROUND: Microsurgical flaps are widely used to treat complex traumatic wounds of upper and lower limbs. Few studies have evaluated whether the vascular changes in preoperative computed tomography angiography (CTA) influence the selection of recipient vessel and type of anastomosis and the microsurgical flaps outcomes including complications. OBJECTIVE: The aim of this study was to evaluate if preoperative CTA reduces the occurrence of major complications (revision of the anastomosis, partial or total flap failure, and amputation) of the flaps in upper and lower limb trauma, and to describe and analyze the vascular lesions of the group with CTA and its relationship with complications. METHODS: A retrospective cohort study was undertaken with all 121 consecutive patients submitted to microsurgical flaps for traumatic lower and upper limb, from 2014 to 2020. Patients were divided into two groups: patients with preoperative CTA (CTA+) and patients not submitted to CTA (CTA–). The presence of postoperative complications was assessed and, within CTA+, we also analyzed the number of patent arteries on CTA and described the arterial lesions. RESULTS: Of the 121 flaps evaluated (84 in the lower limb and 37 in the upper limb), 64 patients underwent preoperative CTA. In the CTA+ group, 56% of patients with free flaps for lower limb had complete occlusion of one artery. CTA+ patients had a higher rate of complications (p = 0.031), which may represent a selection bias as the most complex limb injuries and may have CTA indicated more frequently. The highest rate of complications was observed in chronic cases (p = 0.034). There was no statistically significant difference in complications in patients with preoperative vascular injury or the number of patent arteries. CONCLUSIONS: CTA should not be performed routinely, however, CTA may help in surgical planning, especially in complex cases of high-energy and chronic cases, since it provides information on the best recipient artery and the adequate level to perform the microanastomosis, outside the lesion area.

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Safety climate and related factors in rehabilitation nurses of hospitals in Iran

Work ◽

10.3233/wor-203368 ◽

2021 ◽

pp. 1-8

Author(s):

Javad vatani ◽

Zahra Khanikosarkhizi ◽

Mohammad Ali Shahabi Rabori ◽

mohammad khandan ◽

Mohsen aminizadeh ◽

...

Keyword(s):

Safety Climate ◽

Cross Sectional Study ◽

P Value ◽

Related Factors ◽

Cross Sectional ◽

Climate Assessment ◽

Safety Work ◽

Significant Difference ◽

Training Courses ◽

The Individual

BACKGROUND: Safety climate is a common insight of staff that indicates individuals’ attitudes toward safety and priority of safety at work. OBJECTIVES: Nursing is a risky job where paying attention to safety is crucial. The assessment of the safety climate is one of the methods to measure the safety conditions in this occupation. The aim of this study was to assess the safety climate of rehabilitation nurses working in hospitals in Tehran. METHODS: This is a cross-sectional study which was carried out on 140 rehabilitation nurses selected from all hospitals and clinics in Tehran in 2019. To collect the required data, a two-section questionnaire was used. The first section was related to demographic factors and the second part (22 statements) was to measure the safety climate using nurses’ safety climate assessment questionnaire. The collected data were analyzed by SPSS V16 using independent t-test, ANOVA, Kruskal-Wallis and Mann-Whitney U test at the 5% level. RESULTS: Findings showed that the total mean of safety climate was 3.06±0.56. According to the results, a significant difference was found between the positive and negative satisfaction of nurses with safety climates (P-value = 0.03), communication with nurses (P-value = 0.01) and supervisors’ attitude (P-value = 0.02). Furthermore, a significant difference in safety climate between the individual with the second job and the individual without second could be observed (P-value = 0.01). CONCLUSIONS: The results indicated that the safety climate was not at an acceptable level. Thus, it is essential to introduce safety training courses (e.g. safety, work-rest balance, and so on) and to improve the safety performance at work.

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