scholarly journals The spectrum of congenital malformations of the lung at a rural

2021 ◽  
Vol 1 (2) ◽  
Author(s):  
Dr. Pooja Bhole ◽  
Dr. Vivek Gharpure
Keyword(s):  
Congenital Malformations ◽  
Pulmonary Sequestration ◽  
Rural Hospital ◽  
Rural Setting ◽  
Open Lung ◽  
Congenital Lung Malformation ◽  
Number Of Patients ◽  
Lobar Emphysema ◽  
Lung Resections

ABSTRACT Background: Congenital malformations of lung, such as congenital lobar emphysema, cystic adenomatoid malformation, bronchial cyst, pulmonary sequestration are of rare occurrence. However, our hospital being in a rural area attracts a large number of patients with complex malformations. Method: This retrospective analysis is done to evaluate diagnostic accuracy, safety and efficacy and outcomes of open lung resections at a rural hospital. Results: 16 children with congenital lung malformation underwent open resection in a rural hospital, with no mortality and minimal morbidity, no complications and more than 24 months follow-up. Conclusion: Open lung resections are found to be safe, effective in a rural setting also.

scholarly journals A case report: Type II Abernethy malformation complicated with congenital polydactyly and enlargement of all cardiac chambers.

2019 ◽  
Author(s):  
Cheng Zhu ◽  
Min Wang ◽  
Qian Hao
Keyword(s):  
Congenital Malformations ◽  
Portacaval Anastomosis ◽  
Test Results ◽  
Abernethy Malformation ◽  
Form And Function ◽  
Laboratory Test Results ◽  
Number Of Patients ◽  
Patient Reported ◽  
And Function

Abstract Background: Abernethy malformation is a kind of congenital malformation of portal vein system caused by abnormal portacaval anastomosis. It can be in combination with other congenital malformations. The major therapy of Abernethy malformation is surgery. There has been a limited number of patients since the first patient reported, leading to a limited view towards this kind of disease until now.Results: In August 2018 we treated a patient diagnosed with typeII Abernethy malformation complicated with both congenital polydactyly and enlargement of all cardiac chambers, which is extremely rare and can be a supplementary to the existing cases. Besides, the low white blood cell and platelet, the arrested megakaryocytic maturation and the positive platelet autoantibody in serum may result in misdiagnosis as immune thrombocytopenia, so we analyze the differential points between these two diseases. We treated this patient with silybin orally and advised him to make follow-up visits because of his mild liver function disorder, normal cardiac function and no other malformations or complications complicated. At the latest follow-up, we knew the condition of the patient was generally satisfactory, whether in terms of laboratory test results or his daily life experience.Conclusions: Because of some changes of spleen in form and function secondary to Abernethy malformation, in some cases, this disease has similarities with a part of blood diseases, which we should take into consideration for differential diagnosis, especially when other congenital malformations are found in combination at the same time. This case also suggests that simply conservative treatment with regular follow-up visits can be suitable for certain patients.

scholarly journals A Case Report: Type II Abernethy Malformation Complicated with Congenital Polydactyly and Enlargement of all Cardiac Chambers

2020 ◽  
Vol 7 (1) ◽  
pp. 32-35
Author(s):  
Cheng Zhu ◽  
Min Wang ◽  
Qian Hao
Keyword(s):  
Congenital Malformations ◽  
Life Experience ◽  
Daily Life ◽  
Health Condition ◽  
Type Ii ◽  
Test Results ◽  
Abernethy Malformation ◽  
Laboratory Test Results ◽  
Number Of Patients

Background: Abernethy malformation is a kind of congenital malformation of the portal vein system caused by abnormal portacaval shunts. It can be in combination with many other congenital malformations. There has been a limited number of patients since the first patient was reported, leading to limited knowledge of this kind of disease. Methods: In August 2018, we treated a patient diagnosed with type II Abernethy malformation complicated with both congenital polydactyly and enlargement of all cardiac chambers, which is extremely rare and can be supplementary to the existing cases. According to a comprehensive and adequate assessment of patients' condition, we treated him with oral silybin (70 mg every time and 3 times a day) for 3 months, and advised him to make follow-up visits. Results: At the latest follow-up, we knew the health condition of this patient was generally satisfactory, whether in terms of laboratory test results or his daily life experience. Conclusion: Although the major therapy for Abernethy malformation is surgery, this case suggests that simple conservative treatment with regular follow-up visits can be suitable for certain patients.

scholarly journals Lung Ultrasound: A New Tool in the Management of Congenital Lung Malformation

2019 ◽  
Vol 36 (S 02) ◽  
pp. S99-S105 ◽  
Author(s):  
Michele Quercia ◽  
Raffaella Panza ◽  
Grazia Calderoni ◽  
Antonio Di Mauro ◽  
Nicola Laforgia
Keyword(s):  
Ct Scan ◽  
Lung Ultrasound ◽  
Case Series ◽  
Pulmonary Sequestration ◽  
Type I ◽  
Congenital Pulmonary Airway Malformation ◽  
Congenital Lung Malformation ◽  
First Case ◽  
Pulmonary Airway

Objective This study was aimed to evaluate effectiveness of lung ultrasound (LUS) in the management of congenital pulmonary airway malformation and pulmonary sequestration in NICUs. Study Design This is a nonconsecutive case series of neonates admitted to the academic NICU of Policlinico of Bari, Italy, from 2010 to 2018, for suspected lung malformations and examined by LUS. Results Seven neonates were admitted for suspected pulmonary malformations, four neonates were diagnosed with pulmonary sequestration and three with congenital pulmonary airway malformation either type I (two cases) or type II (one case) according to Adzick classification. Prenatal scans had described lung malformations in six patients. Two underwent surgical intervention during the 1st month of life. All were successfully discharged home and their follow-up has been uneventful thereafter. In all the seven neonates, LUS easily detected the lesion showing a significantly high correspondence with computed tomography (CT) scan findings. Conclusion We described the first case series of neonates affected by complex pulmonary malformations, assessed by LUS. In our experience, LUS was safe and effective for the diagnosis with high degree of consistency with CT scan findings. We suggest that LUS might be an important diagnostic method for lung malformations in newborns and a useful technique for their follow-up and late management, avoiding multiple exposures to radiations.

STRUCTURAL AND DYNAMIC FEATURES OF EAR-LY DISABILITY DUE TO MENTAL DISEASES

2020 ◽  
pp. 62-67
Author(s):  
Shmakova O.P.
Keyword(s):  
People With Disabilities ◽  
Child And Adolescent Psychiatry ◽  
Child Psychiatrist ◽  
Childhood And Adolescence ◽  
Disabled Children ◽  
Dynamic Features ◽  
Factors Affecting ◽  
Number Of Children ◽  
Number Of Patients

Prevention of disability is one of the most significant tasks of child and adolescent psychiatry. Obtaining data on the dynamics of the number of people with disabilities and the factors affecting this indicator seems to be one of the relevant aspects. Aim: to trace the dynamics of the number of children with disabili-ties and to assess the change in the structure of early disability over the past decades. Materials and Meth-ods. A comparative analysis of two cohorts of patients was carried out: 1st - patients born in 1990-1992. (1203 patients (men - 914, 76%; women - 289, 24%)) who applied to the district neuropsychiatric dispensa-ry for outpatient care in childhood and adolescence; II - children and adolescents born in 2005 - 2018 (602 patients (male - 410, 68%; female - 192, 32%), ob-served at the time of the study by a child psychiatrist in the neuropsychiatric dispensary. Research methods: clinical and psychopathological; follow-up; statisti-cal. Results. Comparison of the number and nosologi-cal distribution of disabled children in two cohorts showed that over the 15th year there has been a shift towards an increase in the proportion of disabled children among patients observed by child and ado-lescent psychiatrists. The increase in the number of children with disabilities was due to those suffering from childhood autism and other disorders of general development. There were no statistically significant differences in the number of people with disabilities who received benefits before the age of 7, as well as differences in gender ratios among disabled people in the two cohorts. Conclusion. Early disability is a mul-tifactorial phenomenon, prevalence, dynamics, the structure of which depends not only on clinical, but also on socio-administrative realities. Children with autism require increased attention, since there has been a multiple increase in the number of patients with this diagnosis.

Cerebellotrigeminal Dermal Dysplasia (Gómez-López-Hernández Syndrome)

2018 ◽  
Vol 16 (05) ◽  
pp. 362-368 ◽  
Author(s):  
Federica Sullo ◽  
Agata Polizzi ◽  
Stefano Catanzaro ◽  
Selene Mantegna ◽  
Francesco Lacarrubba ◽  
...  
Keyword(s):  
De Novo ◽  
Chromosomal Rearrangements ◽  
Number Of Patients ◽  
Wide Range ◽  
Visual Problems ◽  
Neurodevelopmental Abnormalities ◽  
Clinical Triad ◽  
High Degree ◽  
Motor Handicap

Cerebellotrigeminal dermal (CTD) dysplasia is a rare neurocutaneous disorder characterized by a triad of symptoms: bilateral parieto-occipital alopecia, facial anesthesia in the trigeminal area, and rhombencephalosynapsis (RES), confirmed by cranial magnetic resonance imaging. CTD dysplasia is also known as Gómez-López-Hernández syndrome. So far, only 35 cases have been described with varying symptomatology. The etiology remains unknown. Either spontaneous dominant mutations or de novo chromosomal rearrangements have been proposed as possible explanations. In addition to its clinical triad of RES, parietal alopecia, and trigeminal anesthesia, CTD dysplasia is associated with a wide range of phenotypic and neurodevelopmental abnormalities.Treatment is symptomatic and includes physical rehabilitation, special education, dental care, and ocular protection against self-induced corneal trauma that causes ulcers and, later, corneal opacification. The prognosis is correlated to the mental development, motor handicap, corneal–facial anesthesia, and visual problems. Follow-up on a large number of patients with CTD dysplasia has never been reported and experience is limited to few cases to date. High degree of suspicion in a child presenting with characteristic alopecia and RES has a great importance in diagnosis of this syndrome.

scholarly journals Biomarkers-based personalized follow-up in chronic heart failure improves patient’s outcomes and reduces care associate cost

2021 ◽  
Vol 19 (1) ◽  
Author(s):  
Antonio Leon-Justel ◽  
Jose I. Morgado Garcia-Polavieja ◽  
Ana Isabel Alvarez-Rios ◽  
Francisco Jose Caro Fernandez ◽  
Pedro Agustin Pajaro Merino ◽  
...  
Keyword(s):  
Heart Failure ◽  
Hospital Admissions ◽  
Left Ventricular ◽  
Left Ventricular Ejection ◽  
Secondary Outcome ◽  
Ventricular Ejection Fraction ◽  
Number Of Patients ◽  
Ed Visits ◽  
The Impact

Abstract Background Heart failure (HF) is a major and growing medical and economic problem, with high prevalence and incidence rates worldwide. Cardiac Biomarker is emerging as a novel tool for improving management of patients with HF with a reduced left ventricular ejection fraction (HFrEF). Methods This is a before and after interventional study, that assesses the impact of a personalized follow-up procedure for HF on patient’s outcomes and care associated cost, based on a clinical model of risk stratification and personalized management according to that risk. A total of 192 patients were enrolled and studied before the intervention and again after the intervention. The primary objective was the rate of readmissions, due to a HF. Secondary outcome compared the rate of ED visits and quality of life improvement assessed by the number of patients who had reduced NYHA score. A cost-analysis was also performed on these data. Results Admission rates significantly decreased by 19.8% after the intervention (from 30.2 to 10.4), the total hospital admissions were reduced by 32 (from 78 to 46) and the total length of stay was reduced by 7 days (from 15 to 9 days). The rate of ED visits was reduced by 44% (from 64 to 20). Thirty-one percent of patients had an improved functional class score after the intervention, whereas only 7.8% got worse. The overall cost saving associated with the intervention was € 72,769 per patient (from € 201,189 to € 128,420) and €139,717.65 for the whole group over 1 year. Conclusions A personalized follow-up of HF patients led to important outcome benefits and resulted in cost savings, mainly due to the reduction of patient hospitalization readmissions and a significant reduction of care-associated costs, suggesting that greater attention should be given to this high-risk cohort to minimize the risk of hospitalization readmissions.

scholarly journals 288. Follow-Up Blood Cultures in Gram-Negative Bacteremia: How Do They Impact Outcomes

2020 ◽  
Vol 7 (Supplement_1) ◽  
pp. S144-S144
Author(s):  
Azza Elamin ◽  
Faisal Khan ◽  
Ali Abunayla ◽  
Rajasekhar Jagarlamudi ◽  
aditee Dash
Keyword(s):  
Clinical Outcomes ◽  
Antibiotic Treatment ◽  
Readmission Rate ◽  
Blood Cultures ◽  
Categorical Variables ◽  
Gram Negative ◽  
Number Of Patients ◽  
Significant Difference ◽  
The Impact

Abstract Background As opposed to Staphylococcus. aureus bacteremia, there are no guidelines to recommend repeating blood cultures in Gram-negative bacilli bacteremia (GNB). Several studies have questioned the utility of follow-up blood cultures (FUBCs) in GNB, but the impact of this practice on clinical outcomes is not fully understood. Our aim was to study the practice of obtaining FUBCs in GNB at our institution and to assess it’s impact on clinical outcomes. Methods We conducted a retrospective, single-center study of adult patients, ≥ 18 years of age admitted with GNB between January 2017 and December 2018. We aimed to compare clinical outcomes in those with and without FUBCs. Data collected included demographics, comorbidities, presumed source of bacteremia and need for intensive care unit (ICU) admission. Presence of fever, hypotension /shock and white blood cell (WBC) count on the day of FUBC was recorded. The primary objective was to compare 30-day mortality between the two groups. Secondary objectives were to compare differences in 30-day readmission rate, hospital length of stay (LOS) and duration of antibiotic treatment. Mean and standard deviation were used for continuous variables, frequency and proportion were used for categorical variables. P-value < 0.05 was defined as statistically significant. Results 482 patients were included, and of these, 321 (67%) had FUBCs. 96% of FUBCs were negative and 2.8% had persistent bacteremia. There was no significant difference in 30-day mortality between those with and without FUBCs (2.9% and 2.7% respectively), or in 30-day readmission rate (21.4% and 23.4% respectively). In patients with FUBCs compared to those without FUBCs, hospital LOS was longer (7 days vs 5 days, P < 0.001), and mean duration of antibiotic treatment was longer (14 days vs 11 days, P < 0.001). A higher number of patients with FUBCs needed ICU care compared to those without FUBCs (41.4% and 25.5% respectively, P < 0.001) Microbiology of index blood culture in those with and without FUBCs Outcomes in those with and without FUBCs FUBCs characteristics Conclusion Obtaining FUBCs in GNB had no impact on 30-day mortality or 30-day readmission rate. It was associated with longer LOS and antibiotic duration. Our findings suggest that FUBCs in GNB are low yield and may not be recommended in all patients. Prospective studies are needed to further examine the utility of this practice in GNB. Disclosures All Authors: No reported disclosures

Coronary sinus catalase and ceruloplasmin levels predict all-cause mortality in patients with end-stage heart failure awaiting heart transplantation

2020 ◽  
Vol 41 (Supplement_2) ◽  
Author(s):  
W Szczurek ◽  
M Gasior ◽  
M Skrzypek ◽  
G Kubiak ◽  
A Kuczaj ◽  
...  
Keyword(s):  
Heart Failure ◽  
Heart Transplantation ◽  
Coronary Sinus ◽  
Public Institution ◽  
Funding Source ◽  
Organ Shortage ◽  
Number Of Patients ◽  
End Stage ◽  
Medical University

Abstract   Background, As a consequence of the worldwide increase in life expectancy and due to significant progress in the pharmacological and interventional treatment of heart failure (HF), the proportion of patients that reach an advanced phase of disease is steadily growing. Hence, more and more numerous group of patients is qualified to the heart transplantation (HT), whereas the number of potential heart donors has remained invariable since years. It contributes to deepening in disproportion between the demand for organs which can possibly be transplanted and number of patients awaiting on the HT list. Therefore, accurate identification of patients who are most likely to benefit from HT is imperative due to an organ shortage and perioperative complications. Purpose The aim of this study was to identify the factors associated with reduced survival during a 1.5-year follow-up in patients with end-stage HF awating HT. Method We propectively analysed 85 adult patients with end-stage HF, who were accepted for HT at our institution between 2015 and 2016. During right heart catheterization, 10 ml of coronary sinus blood was additionally collected to determine the panel of oxidative stress markers. Oxidative-antioxidant balance markers included glutathione reductase (GR), glutathione peroxidase (GPx), glutathione transferase (GST), superoxide dismutase (SOD) and its mitochondrial isoenzyme (MnSOD) and cytoplasmic (Cu/ZnSOD), catalase (CAT), malondialdehyde (MDA), hydroperoxides lipid (LPH), lipofuscin (LPS), sulfhydryl groups (SH-), ceruloplasmin (CR). The study protocol was approved by the ethics committee of the Medical University of Silesia in Katowice. The endpoint of the study was mortality from any cause during a 1.5 years follow-up. Results The median age of the patients was 53.0 (43.0–56.0) years and 90.6% of them were male. All included patients were treated optimally in accordance with the guidelines of the European Society of Cardiology. Mortality rate during the follow-up period was 40%. Multivariate logistic regression analysis showed that ceruloplasmin (odds ratio [OR] = 0.745 [0.565–0.981], p=0.0363), catalase (OR = 0.950 [0.915–0.98], p=0.0076), as well as high creatinine levels (OR = 1.071 [1.002–1.144], p=0.0422) were risk factors for death during 1.5 year follow-up. Conclusions Coronary sinus lower ceruloplasmin and catalase levels, as well as higher creatinine level are independently associated with death during 1.5 year follow-up. Funding Acknowledgement Type of funding source: Public Institution(s). Main funding source(s): Medical University of SIlesia, Katowice, POland

scholarly journals Routine clinical care data from thirteen cardiac outpatient clinics: design of the Cardiology Centers of the Netherlands (CCN) database

2021 ◽  
Vol 21 (1) ◽  
Author(s):  
Sophie H. Bots ◽  
Klaske R. Siegersma ◽  
N. Charlotte Onland-Moret ◽  
Folkert W. Asselbergs ◽  
G. Aernout Somsen ◽  
...  
Keyword(s):  
Risk Factors ◽  
General Practitioner ◽  
Data Collection ◽  
The Netherlands ◽  
Strong Base ◽  
Daily Care ◽  
Cardiac Symptoms ◽  
Number Of Patients ◽  
Patient Groups

Abstract Background Despite the increasing availability of clinical data due to the digitalisation of healthcare systems, data often remain inaccessible due to the diversity of data collection systems. In the Netherlands, Cardiology Centers of the Netherlands (CCN) introduced “one-stop shop” diagnostic clinics for patients suspected of cardiac disease by their general practitioner. All CCN clinics use the same data collection system and standardised protocol, creating a large regular care database. This database can be used to describe referral practices, evaluate risk factors for cardiovascular disease (CVD) in important patient subgroups, and develop prediction models for use in daily care. Construction and content The current database contains data on all patients who underwent a cardiac workup in one of the 13 CCN clinics between 2007 and February 2018 (n = 109,151, 51.9% women). Data were pseudonymised and contain information on anthropometrics, cardiac symptoms, risk factors, comorbidities, cardiovascular and family history, standard blood laboratory measurements, transthoracic echocardiography, electrocardiography in rest and during exercise, and medication use. Clinical follow-up is based on medical need and consisted of either a repeat visit at CCN (43.8%) or referral for an external procedure in a hospital (16.5%). Passive follow-up via linkage to national mortality registers is available for 95% of the database. Utility and discussion The CCN database provides a strong base for research into historically underrepresented patient groups due to the large number of patients and the lack of in- and exclusion criteria. It also enables the development of artificial intelligence-based decision support tools. Its contemporary nature allows for comparison of daily care with the current guidelines and protocols. Missing data is an inherent limitation, as the cardiologist could deviate from standardised protocols when clinically indicated. Conclusion The CCN database offers the opportunity to conduct research in a unique population referred from the general practitioner to the cardiologist for diagnostic workup. This, in combination with its large size, the representation of historically underrepresented patient groups and contemporary nature makes it a valuable tool for expanding our knowledge of cardiovascular diseases. Trial registration: Not applicable.

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