Cardiac rhabdomyoma in childhood tuberous sclerosis

1995 ◽  
Vol 5 (2) ◽  
pp. 166-171 ◽  
Author(s):  
Jung Yun Choi ◽  
Eun Jung Bae ◽  
Chung Il Noh ◽  
Yong-Soo Yoon ◽  
Yong Seung Hwang

AbstractWe studied 52 children in order to assess the prevalence, natural history, and electrophysiologic effects of cardiac rhabdomyoma in tuberous sclerosis. Their ages ranged from one day to 12 years (median age 1.33 year). The tumors in the heart were found in 22 patients through cross-sectional echocardiography. The overall prevalence of cardiac rhabdomyoma was 42%. The prevalence of cardiac tumors in patients first seen in infancy was 77%, and that in patients over one year of age was 31%. Those with cardiac tumors diagnosed in infancy had more tumors per person than did the patients diagnosed after infancy, and the tumors were smaller in the patients diagnosed over one year of age. Follow-up cross-sectional echocardiography showed that almost all tumors showed spontaneous regression. Complete regression occurred more commonly in younger patients, and in those with smaller tumors. About 70% of patients with cardiac tumors had either normal findings or infrequent premature contractions on 24hour Holter monitorings. The rest of the patients with cardiac tumors showed either frequent premature contractions, atrioventricular block, or atrial tachycardia, but antiarrhythmic medication was rarely required. Cross-sectional echocardiography, therefore, seems to be the best diagnostic tool for screening cardiac rhabdomyomas in patients with tuberous sclerosis. The tumors may be managed more conservatively in view of their high rate of spontaneous regression and rather rare association with symptomatic arrhythmias.

2021 ◽  
Vol 8 (Supplement_1) ◽  
pp. S312-S312
Author(s):  
Sonali D Advani ◽  
Sonali D Advani ◽  
Andrea Cromer ◽  
Brittain A Wood ◽  
Esther Baker ◽  
...  

Abstract Background Early assessments of COVID19 preparedness reported resource shortages, use of crisis capacity strategies, variations in testing, personal protective equipment (PPE), and policies in US hospitals. One year later, we performed a follow-up survey to assess changes in infection prevention practice and policies in our diverse network of community and academic hospitals. Methods This was a cross-sectional electronic survey of infection preventionists in 58 hospitals within the Duke Infection Control Outreach Network (community) and Duke/UNC Health systems (academic) in April-May 2021 to follow-up our initial survey from April 2020. The follow-up survey included 26 questions related to resource availability, crisis capacity strategies, procedures, changes to PPE and testing, and staffing challenges. Results We received 54 responses (response rate, 93%). Facilities reported significantly fewer PPE and resource shortages in the follow-up survey compared to our initial survey (Figure 1, P< 0.05). Only 32% of respondents were still reprocessing N95 respirators (compared to 73% in initial survey, P< 0.05). All hospitals performed universal masking, universal symptom screening on entry, and 30% required eye protection. In 2020, most hospitals suspended elective surgical procedures in March-April, and restarted in May-June. Approximately 92% reported in-house testing for SARS-COV-2 by April 2020, at least a third of which had a weekly capacity of >100 tests. Almost 80% performed universal pre-operative testing, while 61% performed universal preadmission testing for SARS-COV-2. Almost all hospitals switched from test-based to time-based strategy for discontinuing isolation precautions, majority in August-September 2020. Twenty-five percent hospitals reported infection prevention furloughs, staffing cuts, and or reassignments, while 81% reported increased use of agency nursing during the pandemic. Conclusion Our follow-up survey reveals improvement in resource availability, evolution of PPE guidance, increase in testing capacity, and burdensome staffing changes. Our serial surveys suggest increasing uniformity in infection prevention policies, but also highlight the increase in staff turnover and infection prevention staffing shortages. Disclosures Sonali D. Advani, MBBS, MPH, Nothing to disclose David J. Weber, MD, MPH, PDI (Consultant)


2001 ◽  
Author(s):  
M Brzosko ◽  
I Fiedorowicz-Fabrycy ◽  
J Fliciñski ◽  
H Przepiera-Bêdzak ◽  
K Prajs

2021 ◽  
pp. 219256822110223
Author(s):  
Grant Riew ◽  
Francis Lovecchio ◽  
Dino Samartzis ◽  
Philip K. Louie ◽  
Niccole Germscheid ◽  
...  

Study Design: Cross-sectional, anonymous, international survey. Objectives: The COVID-19 pandemic has resulted in the rapid adoption of telemedicine in spine surgery. This study sought to determine the extent of adoption and global perspectives on telemedicine in spine surgery. Methods: All members of AO Spine International were emailed an anonymous survey covering the participant’s experiences with and perceptions of telemedicine. Descriptive statistics were used to depict responses. Responses were compared among regions. Results: 485 spine surgeons participated in the survey. Telemedicine usage rose from <10.0% to >39.0% of all visits. A majority of providers (60.5%) performed at least one telemedicine visit. The format of “telemedicine” varied widely by region: European (50.0%) and African (45.2%) surgeons were more likely to use phone calls, whereas North (66.7%) and South American (77.0%) surgeons more commonly used video ( P < 0.001). North American providers used telemedicine the most during COVID-19 (>60.0% of all visits). 81.9% of all providers “agreed/strongly agreed” telemedicine was easy to use. Respondents tended to “agree” that imaging review, the initial appointment, and postoperative care could be performed using telemedicine. Almost all (95.4%) surgeons preferred at least one in-person visit prior to the day of surgery. Conclusion: Our study noted significant geographical differences in the rate of telemedicine adoption and the platform of telemedicine utilized. The results suggest a significant increase in telemedicine utilization, particularly in North America. Spine surgeons found telemedicine feasible for imaging review, initial visits, and follow-up visits although the vast majority still preferred at least one in-person preoperative visit.


2020 ◽  
Vol 33 (9) ◽  
pp. 1147-1153
Author(s):  
Fatima Ali Mazahir ◽  
Manal Mustafa Khadora

AbstractObjectivesWe evaluated the spectrum of diseases accompanying congenital hypothyroidism (CH) in the United Arab Emirates and compared them with internationally studied patterns.MethodsThe presented retrospective cross-sectional study took place in two government tertiary care centres. In total, 204 patients with a confirmed diagnosis of CH and a minimum period of follow-up of 1 year were included. Patients with Down syndrome, infants born at <35 weeks of gestation, and babies with TORCH (Toxoplasma gondii, Other viruses [HIV, measles, etc.], Rubella, Cytomegalovirus, and Herpes simplex) infections were subsequently excluded from the study.ResultsOf the subjects with CH, 39% had associated extrathyroidal anomalies (ETAs); among these, 25% had a single anomaly. A significant proportion of Arab males were affected by CH as compared to other ethnic groups. Dyshormonogenesis was the commonest aetiological cause (55%) of CH. Males with an ectopic lingual thyroid gland had significant ETAs as compared to females of the same cohort. The most common ETAs were congenital heart disease (16%), followed by urogenital tract anomalies (14%).ConclusionsDetection of a high rate and variability of ETAs associated with CH necessitates the formulation of a structured screening programme including appropriate clinical, laboratory, and imaging tools to detect ETAs at an earlier stage.


2014 ◽  
Vol 4 (4) ◽  
Author(s):  
Alexandre González-Rodríguez ◽  
Oriol Molina-Andreu ◽  
Rafael Penadés ◽  
María Luisa Imaz Gurrutxaga ◽  
Rosa Catalán

2017 ◽  
Vol 5 (2) ◽  
pp. 193-196 ◽  
Author(s):  
Ramush Bejiqi ◽  
Ragip Retkoceri ◽  
Hana Bejiqi

BACKGROUND: Cardiac rhabdomyoma (CRs) are the most common primary tumour of the heart in infants and children. Usually are multiple and, basing on the location can cause a haemodynamic disturbance, dysrhythmias or heart failure during the fetal and early postnatal period. CRs have a natural history of spontaneous regression and are closely associated with tuberous sclerosis complex (TSC). It has an association with tuberous sclerosis (TS), and in those, the tumour may regress and disappear completely, or remain consistent in size. AIM: We aimed to evaluate the prenatal diagnosis, clinical presentation and outcome of CRs and their association with TSC in a single centre. The median follow-up period was three years (range: 6 months - 5 years). MATERIAL AND METHODS: We reviewed medical records of all fetuses diagnosed prenatally with cardiac rhabdomyoma covering the period January 2010 to December 2016 which had undergone detailed ultrasound evaluation at a single centre with limited technical resources. RESULTS: Twelve fetuses were included in the study; mostly had multiple tumours and a total of 53 tumours were identified in all patients - the maximum was one fetus with16 tumours. All patients were diagnosed prenatally by fetal echocardiography. In two patient's haemodynamic disturbances during the fetal period was noted and pregnancies have been terminated. After long consultation termination of pregnancy was chosen by the parents in totally 8 cases. In four continuing pregnancies during the first year of live tumours regressed. TSC was diagnosed in all patients during the follow-up. CONCLUSIONS: Cardiac rhabdomyoma are benign from the cardiovascular standpoint in most affected fetuses. An early prenatal diagnosis may help for an adequate planning of perinatal monitoring and treatment with the involvement of a multidisciplinary team. Large tumour size, the number of tumours and localisation may cause hydrops, and they are significantly associated with poor neonatal outcome.


2021 ◽  
Vol 9 (T4) ◽  
pp. 267-278
Author(s):  
Pramitha Esha Nirmala Dewi ◽  
Montarat Thavorncharoensap

BACKGROUND: The early use of statin with intensive regimen has been recommended by the recent guidelines as the prevention of acute coronary syndrome (ACS) related events among the high-risk patients. Meanwhile, the inconsistent statin utilization for targeted patient in current practice is still an issue. AIM: This study aims to review the utilization rate of statin among patients with ACS. METHODS: A systematic search of relevant studies published between inceptions to June 2020 was conducted in PubMed. Patients and intervention domains were used to build up the searching formula. A study was eligible for inclusion if it was an original study of patients with ACS and it examined the utilization of statin. The risk of bias was assessed using Axis and NOS checklist. RESULTS: Among the 49 eligible studies, 38 were cohort studies while the others were cross-sectional studies. The utilization rate of statin at hospital admission ranged from 16% to 61% while 25% to 75% during the hospitalization. Of the total studies, 35 studies reported the statin rate at discharge ranging from 58% to 99%. Almost all studies revealed the reduction of statin utilization rate along the follow-up period. The number of statins prescribed was found to be lower among female and elderly patients. CONCLUSION: Despite the established benefits of statin among patients with ACS, our study revealed that statin was underutilized for secondary prevention after ACS. To improve patients’ clinical outcomes with ACS, efforts should be made to increase optimal treatment and compliance with a statin.


2003 ◽  
Vol 13 (3) ◽  
pp. 258-263 ◽  
Author(s):  
Junko Shiono ◽  
Hitoshi Horigome ◽  
Seiyo Yasui ◽  
Tomoyuki Miyamoto ◽  
Miho Takahashi-Igari ◽  
...  

Background:Cardiac rhabdomyomas associated with tuberous sclerosis induce various abnormalities in the electrocardiogram. Electrocardiographic evidence of ventricular hypertrophy may appear if the tumour is electrically active. To our knowledge, electrocardiographic evidence of ventricular hypertrophy has been reported only in association with congestive heart failure. Follow-up studies of changes in electrocardiographic findings are also lacking.Methods:We studied 21 consecutive patients with cardiac rhabdomyoma associated with tuberous sclerosis, 10 males and 11 females, aged from the date of birth to 9 years at diagnosis. The mean period of follow-up was 53 months. None of the patients developed congestive heart failure. We evaluated the electrocardiographic changes during the follow-up, and their association with echocardiographic findings.Results:Of the 21 patients, 12 showed one or more abnormalities on the electrocardiogram at presentation, with five demonstrating right or left ventricular hypertrophy. In all of these five cases, the tumours were mainly located in the respective ventricular cavity. In one patient with a giant tumour expanding exteriorly, there was marked left ventricular hypertrophy on the electrocardiogram. Followup studies showed spontaneous regression of the tumours in 12 of 19 patients, with abnormalities still present in only 7 patients. A gradual disappearance of left ventricular hypertrophy as seen on the electrocardiogram was noted in the patient with marked left ventricular hypertrophy at presentation in parallel with regression of the tumour.Conclusions:The presence of cardiac rhabdomyomas in patients with tuberous sclerosis might explain the ventricular hypertrophy seen on the electrocardiogram through its electrically active tissue without ventricular pressure overload or ventricular enlargement, although pre-excitation might affect the amplitude of the QRS complex. Even in cases with large tumours, nonetheless, the electric potential might not alter the surface electrocardiogram if the direction of growth of the tumour is towards the ventricular cavity. In many cases, electrocardiographic abnormalities tend to disappear, concomitant with regression of the tumours.


2018 ◽  
Vol 29 (2) ◽  
pp. 47-56
Author(s):  
MR Haque ◽  
K Ghosh ◽  
H Sultana ◽  
E Saha ◽  
A Kafi ◽  
...  

Objective: The main objective of the study was to assess the level of awareness on prevention and control of sexually transmitted diseases among 120 sex workers.Material and Methods: The cross-sectional study was conducted for one year. Data were collected through face to face interview using Bengali questionnaire. Data analysis was done using SPSS 17.0 software.Place and Period of Study: The study was conducted from January 1st to December 31st of 2014 among 120 sex workersliving in brothel of Daulodia,Rajbariin Bangladesh.Results: More than half (58.7%) of the respondents had children in the brothels. All the respondents (100%) heard about the sexually transmitted diseases. Majority (86.7%) of the respondents had knowledge on AIDS. Only a few (4.2%) respondents had knowledge on AIDS and Gonorrhea. Majority (91.7 %) of the respondents had knowledge on STD transmission from pregnant women. Almost all (97.5%, 98.3%)) of the respondents had the knowledge about STD transmissionnot from toilet or shaking hands respectively.Almost all of the (95.8%, 94.2%, 94.2%, 85.8% )respondents had the knowledge on STD transmission through sexual intercourse,injection,through mosquito,sharing same razor respectively. Most of them (81.7%) had good level of knowledge on STD transmission. Only above one tenth (11.7%) had moderate and poor level of knowledge.Almost all of them (96.7%, 95.9%, 95.9%) were found touse condom, agreed to keep one partner at a time,to reduce the number of sex partner respectively.Most of them (89.3%)did not knowthe HIV status of partner before sex,almost all (97.5%, 94.2% and 97.5%) agreed to avoid risky sex,to screen blood during transfusion, to avoid sharing needles & blade respectively. All (100%)of the respondents agreed for need of sexuality education for preventing STD.Almost all (98.30%)of the respondents had good awareness on Prevention and Control of Sexually Transmitted Diseases. There was no association between level of education and level of awareness (P> 0.005), between age and level of awareness (P> 0.005) and between duration of work and level of awareness (P> 0.005).Conclusion: Education is the most consistent and important determinant for prevention of STDs among all classes of people.TAJ 2016; 29(2): 47-56


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