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1.
Indian J Thorac Cardiovasc Surg ; 40(1): 111-113, 2024 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-38125314

RESUMO

A patient with a history of endocarditis developed a fistula between the aorta and right atrium requiring surgical repair. The patient underwent surgical intervention with closure of the fistula using an autologous pericardial patch and primary repair. This report is significant because a rare surgical pathology is visualized clearly and provides an educational value to aid other clinicians in the recognition and management of this unusual diagnosis.

2.
Br J Anaesth ; 132(2): 312-319, 2024 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-38114355

RESUMO

BACKGROUND: Neurofilament light is a blood-based biomarker of neuroaxonal injury that can provide insight into perioperative brain vulnerability and injury. Prior studies have suggested that increased baseline and postoperative concentrations of neurofilament light are associated with delirium after noncardiac surgery, but results are inconsistent. Results have not been reported in cardiac surgery patients, who are among those at highest risk for delirium. We hypothesised that perioperative blood concentrations of neurofilament light (both baseline and change from baseline to postoperative day 1) are associated with delirium after cardiac surgery. METHODS: This study was nested in a trial of arterial blood pressure targeting during cardiopulmonary bypass using cerebral autoregulation metrics. Blood concentrations of neurofilament light were measured at baseline and on postoperative day 1. The primary outcome was postoperative delirium. Regression models were used to examine the associations between neurofilament light concentration and delirium and delirium severity, adjusting for age, sex, race, logistic European System for Cardiac Operative Risk Evaluation, bypass duration, and cognition. RESULTS: Delirium occurred in 44.6% of 175 patients. Baseline neurofilament light concentration was higher in delirious than in non-delirious patients (median 20.7 pg ml-1 [IQR 16.1-33.2] vs median 15.5 pg ml-1 [IQR 12.1-24.2], P<0.001). In adjusted models, greater baseline neurofilament light concentration was associated with delirium (odds ratio, 1.027; 95% confidence interval, 1.003-1.053; P=0.029) and delirium severity. From baseline to postoperative day 1, neurofilament light concentration increased by 42%, but there was no association with delirium. CONCLUSIONS: Baseline neurofilament light concentration, but not change from baseline to postoperative day 1, was associated with delirium after cardiac surgery.


Assuntos
Procedimentos Cirúrgicos Cardíacos , Delírio , Humanos , Delírio/diagnóstico , Delírio/etiologia , Filamentos Intermediários , Estudos Prospectivos , Encéfalo , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Complicações Pós-Operatórias/diagnóstico
3.
Curr Cardiol Rep ; 24(11): 1699-1709, 2022 11.
Artigo em Inglês | MEDLINE | ID: mdl-36063349

RESUMO

PURPOSE OF REVIEW: Cardiovascular autonomic control is an intricately balanced dynamic process. Autonomic dysfunction, regardless of origin, promotes and sustains the disease processes, including in patients with heart failure (HF). Autonomic control is mediated through the two autonomic branches: parasympathetic and sympathetic (P&S). HF is arguably the disease that stands to most benefit from P&S manipulation to reduce mortality risk. This review article briefly summarizes some of the more common types of autonomic dysfunction (AD) that are found in heart failure, suggests a mechanism by which AD may contribute to HF, reviews AD involvement in common HF co-morbidities (e.g., ventricular arrhythmias, AFib, hypertension, and Cardiovascular Autonomic Neuropathy), and summarizes possible therapy options for treating AD in HF. RECENT FINDINGS: Autonomic assessment is important in diagnosing and treating CHF, and its possible co-morbidities. Autonomic assessment may also have importance in predicting which patients may be susceptible to sudden cardiac death. This is important since most CHF patients with sudden cardiac death have preserved left ventricular ejection fraction and better discriminators are needed. Many life-threatening cardiovascular disorders will require invasive testing for precise diagnoses and therapy planning when modulating the ANS is important. In cases of non-life-threatening disorders, non-invasive ANS testing techniques, especially those that individually assess both ANS branches simultaneously and independently, are sufficient to diagnose and treat serially.


Assuntos
Insuficiência Cardíaca , Função Ventricular Esquerda , Humanos , Volume Sistólico , Morte Súbita Cardíaca/etiologia , Morte Súbita Cardíaca/prevenção & controle
4.
Anesthesiology ; 137(3): 303-314, 2022 09 01.
Artigo em Inglês | MEDLINE | ID: mdl-35984933

RESUMO

BACKGROUND: Neurofilament light is a marker of neuronal injury and can be measured in blood. Postoperative increases in neurofilament light have been associated with delirium after noncardiac surgery. However, few studies have examined the association of neurofilament light changes with postdischarge cognition in cardiac surgery patients, who are at highest risk for neuronal injury and cognitive decline. The authors hypothesized that increased neurofilament light (both baseline and change) would be associated with worse neuropsychological status up to 1 yr after cardiac surgery. METHODS: This observational study was nested in a trial of cardiac surgery patients, in which blood pressure during bypass was targeted using cerebral autoregulation monitoring. Plasma concentrations of neurofilament light were measured at baseline and postoperative day 1. Neuropsychological testing was performed at baseline, 1 month after surgery, and 1 yr after surgery. Primary outcomes were baseline and change from baseline in a composite z-score of all cognitive tests. RESULTS: Among 167 patients, cognitive outcomes were available in 80% (134 of 167) and 61% (102 of 167) at 1 month and 1 yr after surgery, respectively. The median baseline concentration of neurofilament light was 18.2 pg/ml (interquartile range, 13.4 to 28.1), and on postoperative day 1 was 28.5 pg/ml (interquartile range, 19.3 to 45.0). Higher baseline log neurofilament light was associated with worse baseline cognitive z-score (adjusted slope, -0.60; 95% CI, -0.90 to -0.30; P < 0.001), no change in z-score from baseline to 1 month (0.11; 95% CI, -0.19 to 0.41; P = 0.475), and improvement in z-score from baseline to 1 yr (0.56; 95% CI, 0.31 to 0.81; P < 0.001). Whereas some patients had an improvement in cognition at 1 yr and others a decline, an increase in neurofilament light from baseline to postoperative day 1 was associated with a greater decline in cognition at 1 yr. CONCLUSIONS: Higher baseline neurofilament light concentration was associated with worse baseline cognition but improvement in cognition at 1 yr. A postoperative increase in neurofilament light was associated with a greater cognitive decline at 1 yr.


Assuntos
Assistência ao Convalescente , Procedimentos Cirúrgicos Cardíacos , Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Cognição , Estudos de Coortes , Humanos , Filamentos Intermediários , Alta do Paciente , Complicações Pós-Operatórias/etiologia , Estudos Prospectivos
6.
Case Rep Endocrinol ; 2021: 8479193, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34497729

RESUMO

Sporadic reports of factitious elevations of thyroid hormones related to laboratory interference from autoantibodies and multiple myeloma paraproteins have appeared in the literature. Such clinically confusing laboratory results can lead to erroneous diagnoses and inappropriate treatments. We report an additional case of a patient with multiple myeloma and an IgG paraproteinemia who had such a spurious elevation of total T3 complicating her levothyroxine management of hypothyroidism. In addition, we alert clinicians that differences in performance characteristics between various manufacturers' test platforms may also cause spurious reports.

7.
Sci Rep ; 11(1): 9421, 2021 May 03.
Artigo em Inglês | MEDLINE | ID: mdl-33941798

RESUMO

Electromagnetic (EM) metasurfaces are essential in a wide range of EM engineering applications, from incorporated into antenna designs to separate devices like radome. Near-field manipulators are a class of metasurfaces engineered to tailor an EM source's radiation patterns by manipulating its near-field components. They can be made of all-dielectric, hybrid, or all-metal materials; however, simultaneously delivering a set of desired specifications by an all-metal structure is more challenging due to limitations of a substrate-less configuration. The existing near-field phase manipulators have at least one of the following limitations; expensive dielectric-based prototyping, subject to ray tracing approximation and conditions, narrowband performance, costly manufacturing, and polarization dependence. In contrast, we propose an all-metal wideband phase correcting structure (AWPCS) with none of these limitations and is designed based on the relative phase error extracted by post-processing the actual near-field distributions of any EM sources. Hence, it is applicable to any antennas, including those that cannot be accurately analyzed with ray-tracing, particularly for near-field analysis. To experimentally verify the wideband performance of the AWPCS, a shortened horn antenna with a large apex angle and a non-uniform near-field phase distribution is used as an EM source for the AWPCS. The measured results verify a significant improvement in the antenna's aperture phase distribution in a large frequency band of 25%.

8.
JACC Clin Electrophysiol ; 7(4): 463-470, 2021 04.
Artigo em Inglês | MEDLINE | ID: mdl-33812839

RESUMO

OBJECTIVES: This study sought to explore the long-term arrhythmic outcomes of bilateral cardiac sympathetic denervation (BCSD). BACKGROUND: BCSD has been associated with improved arrhythmic outcomes in patients with refractory ventricular arrhythmias. However, whether BCSD antiarrhythmic effects are sustained long after the procedure is still uncertain. METHODS: We included consecutive patients who underwent BCSD because of refractory ventricular tachycardia (VT) and had at least 18 months of follow-up. VT recurrence after BCSD was evaluated to assess arrhythmic outcomes. The occurrence of VT episodes within the first 12 weeks after the procedure was assessed to explore the impact of early VT recurrence on late arrhythmia-free survival. RESULTS: Twenty patients (42 ± 16 years; 55% male) were included in the analysis. Nineteen (95%) patients had structural heart disease (left ventricular ejection fraction: 0.46 ± 0.14). Class I or class III drugs failed for all patients, and the mean number of VT ablation procedures was 2.5 ± 1.6. Over a mean follow-up of 1,300 ± 321 days (median: 1,276 days [Interquartile range (IQR): 1,181 to 1,480 days), 11 (55%) patients remained VT free after sympathectomy. Freedom from sustained VT or implantable cardioverter-defibrillator shock was 60% (95% confidence interval: 0.35 to 0.77) and 54.5% (95% confidence interval: 0.31 to 0.73) after BCSD at 1 and 4 years. Early VT recurrence was not associated with worse late arrhythmia-free survival rates. CONCLUSIONS: BCSD was associated with longstanding antiarrhythmic effects in patients with refractory ventricular arrhythmias. The occurrence of VT episodes early after the procedure was not associated with worse late arrhythmic outcomes.


Assuntos
Taquicardia Ventricular , Arritmias Cardíacas/cirurgia , Feminino , Humanos , Masculino , Estudos Retrospectivos , Simpatectomia , Taquicardia Ventricular/cirurgia , Resultado do Tratamento
9.
Indian Pediatr ; 58(1): 30-33, 2021 01 15.
Artigo em Inglês | MEDLINE | ID: mdl-33452774

RESUMO

OBJECTIVE: To examine the common and specific clinical features, mutation spectrum and genotype-phenotype correlation in Noonan syndrome and related RASopathies. PARTICIPANTS: Records of 30 patients with clinical diagnosis of Noonan syndrome and related RASopathies presenting over a six-year period at a tertiary care medical genetics centre were reviewed. Detailed clinical phenotype evaluation and genetic testing (PTPN11 sequencing or next generation sequencing) was done. The genetic results were used to classify the patients. RESULTS: Noonan syndrome was confirmed in 22 patients, 5 had cardiofaciocutaneous syndrome and 3 had Noonan syndrome like disorder with loose anagen hair. The molecular diagnosis was confirmed in 27 patients. Mutations in PTPN11 gene were confirmed in 57.8 % patients. Developmental delay, cardiac defects, ectodermal abnormalities and coarse face was the predominant phenotype. Noonan syndrome like disorder with loose anagen hair was clinically identifiable by the sparse, slow growing hair and caused by one recurrent SHOC2, c.4A>G mutation. CONCLUSION: Noonan syndrome and other RASopathies should be suspected in patients with short stature, cardiac defects, typical facial dysmorphism with or without ectodermal involvement.


Assuntos
Síndrome de Noonan , Fácies , Estudos de Associação Genética , Humanos , Peptídeos e Proteínas de Sinalização Intracelular , Mutação , Síndrome de Noonan/diagnóstico , Síndrome de Noonan/genética , Fenótipo
10.
Indian J Thorac Cardiovasc Surg ; 36(5): 492-501, 2020 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-33061160

RESUMO

Mitral valve surgery has evolved through the ages, in response to prevalent epidemiology of mitral pathologies. In the modern era, advances in technology has allowed physicians to help a wider spectrum on increasingly sicker patients. This review summarises these advances and its associated evidence base for safety and efficacy.

11.
Innovations (Phila) ; 15(4): 379-381, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32628080

RESUMO

Acute type A aortic dissection (TAAD) with malperfusion syndrome remains a challenging diagnosis and optimal surgical management remains unsettled. We present a case and surgical approach employed for a patient with TAAD and malperfusion syndrome who presented with pulseless bilaterally extremities. Satisfactory outcomes can be achieved with early multidisciplinary collaboration and urgent repair of the aorta and simultaneous efforts to restore blood flow to ischemic tissue.


Assuntos
Aorta Torácica/cirurgia , Aneurisma da Aorta Torácica/cirurgia , Dissecção Aórtica/cirurgia , Isquemia/etiologia , Extremidade Inferior/irrigação sanguínea , Procedimentos Cirúrgicos Vasculares/métodos , Doença Aguda , Dissecção Aórtica/complicações , Dissecção Aórtica/diagnóstico por imagem , Aorta Torácica/diagnóstico por imagem , Aneurisma da Aorta Torácica/diagnóstico por imagem , Circulação Sanguínea , Tamponamento Cardíaco/etiologia , Angiografia por Tomografia Computadorizada , Humanos , Masculino , Pessoa de Meia-Idade
12.
Anesth Analg ; 130(6): 1534-1544, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32384343

RESUMO

BACKGROUND: Although frailty has been associated with major morbidity/mortality and increased length of stay after cardiac surgery, few studies have examined functional outcomes. We hypothesized that frailty would be independently associated with decreased functional status, increased discharge to a nonhome location, and longer duration of hospitalization after cardiac surgery, and that delirium would modify these associations. METHODS: This was an observational study nested in 2 trials, each of which was conducted by the same research team with identical measurement of exposures and outcomes. The Fried frailty scale was measured at baseline. The primary outcome (defined before data collection) was functional decline, defined as ≥2-point decline from baseline in Instrumental Activities of Daily Living (IADL) score at 1 month after surgery. Secondary outcomes were absolute decline in IADL score, discharge to a new nonhome location, and duration of hospitalization. Associations were analyzed using linear, logistic, and Poisson regression models with adjustments for variables considered before analysis (age, gender, race, and logistic European Score for Cardiac Operative Risk Evaluation [EuroSCORE]) and in a propensity score analysis. RESULTS: Data were available from 133 patients (83 from first trial and 50 from the second trial). The prevalence of frailty was 33% (44 of 133). In adjusted models, frail patients had increased odds of functional decline (primary outcome; odds ratio [OR], 2.41 [95% confidence interval {CI}, 1.03-5.63]; P = .04) and greater decline at 1 month in the secondary outcome of absolute IADL score (-1.48 [95% CI, -2.77 to -0.30]; P = .019), compared to nonfrail patients. Delirium significantly modified the association of frailty and change in absolute IADL score at 1 month. In adjusted hypothesis-generating models using secondary outcomes, frail patients had increased discharge to a new nonhome location (OR, 3.25 [95% CI, 1.37-7.69]; P = .007) and increased duration of hospitalization (1.35 days [95% CI, 1.19-1.52]; P < .0001) compared to nonfrail patients. The increased duration of hospitalization, but no change in functional status or discharge location, was partially mediated by increased complications in frail patients. CONCLUSIONS: Frailty may identify patients at risk of functional decline at 1 month after cardiac surgery. Perioperative strategies to optimize frail cardiac surgery patients are needed.


Assuntos
Procedimentos Cirúrgicos Cardíacos/efeitos adversos , Delírio/etiologia , Fragilidade/complicações , Fragilidade/cirurgia , Complicações Pós-Operatórias/diagnóstico , Atividades Cotidianas , Idoso , Delírio/cirurgia , Feminino , Idoso Fragilizado , Avaliação Geriátrica , Cardiopatias/complicações , Cardiopatias/cirurgia , Hospitalização , Humanos , Tempo de Internação , Masculino , Pessoa de Meia-Idade , Distribuição de Poisson , Índice de Gravidade de Doença , Resultado do Tratamento
13.
Indian Pediatr ; 56(12): 1017-1019, 2019 12 15.
Artigo em Inglês | MEDLINE | ID: mdl-31884430

RESUMO

OBJECTIVE: To assess the utility of computer-aided facial analysis in identifying dysmorphic syndromes in Indian children. METHODS: Fifty-one patients with a definite molecular or cytogenetic diagnosis and recognizable facial dysmorphism were enrolled in the study and their facial photographs were uploaded in the Face2Gene software. The results provided by the software were compared with the molecular diagnosis. RESULTS: Of the 51 patients, the software predicted the correct diagnosis in 37 patients (72.5%); predicted as the first in the top ten suggestions in 26 (70.2%). In 14 patients, the software did not suggest a correct diagnosis. CONCLUSIONS: Computer-aided facial analysis is a method that can aid in diagnosis of genetic syndromes in Indian children. As more clinicians start to use this software, its accuracy is expected to improve.


Assuntos
Anormalidades Craniofaciais/diagnóstico por imagem , Face/diagnóstico por imagem , Fácies , Interpretação de Imagem Assistida por Computador/métodos , Adolescente , Criança , Pré-Escolar , Anormalidades Craniofaciais/classificação , Anormalidades Craniofaciais/patologia , Face/patologia , Feminino , Transtornos do Espectro Alcoólico Fetal/diagnóstico por imagem , Transtornos do Espectro Alcoólico Fetal/patologia , Humanos , Lactente , Recém-Nascido , Masculino , Fotografação
14.
Regul Toxicol Pharmacol ; 108: 104471, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31493441

RESUMO

Multi-locational supervised field trials were conducted in different agro-climatic regions in India to study dissipation of trifloxystrobin and tebuconazole in tomato after spraying a combination formulation (trifloxystrobin 25% + tebuconazole 50%, 75WG) at recommended doses: (i) single (trifloxystrobin 87.5 g a.i. ha-1 + tebuconazole 175 g a.i. ha-1) and (ii) double (trifloxystrobin 175 g a.i. ha-1 + tebuconazole 350 g a.i. ha-1). Fruit samples were extracted with ethyl acetate using a modified QuEChERS method. The residues (parent fungicides + metabolite) were analyzed and confirmed by GC-ECD and GC-MS, respectively. The half-life (t1/2) of trifloxystrobin and tebuconazole in tomato varied from 1.08 to 1.72 and 1.13 -to 1.64 days at single; and 1.27 to 2.13 and 1.24 to 1.96 days at double dose, respectively. Since maximum residue limit (MRL) at pre-harvest interval (PHI) of 5 days is impractical, as tomato is usually harvested and consumed almost everyday after the last spray, the risk assessment was performed at minimum PHI of 1 day. Accordingly, on the basis of supervised field trial data and using OECD MRL calculator, MRL of 0.5 and 1.5 mg kg-1 at single dose were proposed for trifloxystrobin and tebuconazole in/on tomato, respectively.


Assuntos
Acetatos/análise , Frutas/química , Fungicidas Industriais/análise , Iminas/análise , Resíduos de Praguicidas/análise , Solanum lycopersicum , Estrobilurinas/análise , Triazóis/análise , Adulto , Criança , Exposição Dietética , Cromatografia Gasosa-Espectrometria de Massas , Meia-Vida , Humanos , Nível de Efeito Adverso não Observado , Medição de Risco
16.
JAMA Surg ; 154(9): 819-826, 2019 09 01.
Artigo em Inglês | MEDLINE | ID: mdl-31116358

RESUMO

Importance: Delirium occurs in up to 52% of patients after cardiac surgery and may result from changes in cerebral perfusion. Using intraoperative cerebral autoregulation monitoring to individualize and optimize cerebral perfusion may be a useful strategy to reduce the incidence of delirium after cardiac surgery. Objective: To determine whether targeting mean arterial pressure during cardiopulmonary bypass (CPB) using cerebral autoregulation monitoring reduces the incidence of delirium compared with usual care. Design, Setting, and Participants: This randomized clinical trial nested within a larger trial enrolled patients older than 55 years who underwent nonemergency cardiac surgery at a single US academic medical center between October 11, 2012, and May 10, 2016, and had a high risk for neurologic complications. Patients, physicians, and outcome assessors were masked to the assigned intervention. A total of 2764 patients were screened, and 199 were eligible for analysis in this study. Intervention: In the intervention group, the patient's lower limit of cerebral autoregulation was identified during surgery before CPB. On CPB, the patient's mean arterial pressure was targeted to be greater than that patient's lower limit of autoregulation. In the control group, mean arterial pressure targets were determined according to institutional practice. Main Outcomes and Measures: The main outcome was any incidence of delirium on postoperative days 1 through 4, as adjudicated by a consensus expert panel. Results: Among the 199 participants in this study, mean (SD) age was 70.3 (7.5) years and 150 (75.4%) were male. One hundred sixty-two (81.4%) were white, 26 (13.1%) were black, and 11 (5.5%) were of other race. Of 103 patients randomized to usual care, 94 were analyzed, and of 102 patients randomized to the intervention 105 were analyzed. Excluding 5 patients with coma, delirium occurred in 48 of the 91 patients (53%) in the usual care group compared with 39 of the 103 patients (38%) in the intervention group (P = .04). The odds of delirium were reduced by 45% in patients randomized to the autoregulation group (odds ratio, 0.55; 95% CI, 0.31-0.97; P = .04). Conclusions and Relevance: The results of this study suggest that optimizing mean arterial pressure to be greater than the individual patient's lower limit of cerebral autoregulation during CPB may reduce the incidence of delirium after cardiac surgery, but further study is needed. Trial Registration: ClinicalTrials.gov identifier: NCT00981474.


Assuntos
Pressão Arterial/fisiologia , Ponte Cardiopulmonar/efeitos adversos , Delírio/etiologia , Monitorização Intraoperatória/métodos , Complicações Pós-Operatórias/prevenção & controle , Centros Médicos Acadêmicos , Fatores Etários , Idoso , Ponte Cardiopulmonar/métodos , Circulação Cerebrovascular , Delírio/epidemiologia , Delírio/fisiopatologia , Feminino , Avaliação Geriátrica , Homeostase/fisiologia , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Complicações Pós-Operatórias/fisiopatologia , Estudos Prospectivos , Valores de Referência , Índice de Gravidade de Doença , Fatores Sexuais
17.
Indian J Endocrinol Metab ; 23(1): 140-149, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31016169

RESUMO

Type 2 diabetes mellitus (T2DM) is a family of metabolic disorders characterized by hyperglycemia as a consequence of abnormalities in insulin secretion and insulin sensitivity. It affects hundreds of millions of people worldwide and leads to increased morbidity, compromised quality of life, higher mortality sodium glucose co-transporter 2 (SGLT2) inhibitors, a new class of oral antidiabetic drugs, have garnered considerable attention in the recent past and are considered potential first-line candidates for the management of T2DM. This review outlines the evidence-based therapeutic efficacy, safety, limitations, and advantages of SGLT2 inhibitors in the management of T2DM. SGLT2 inhibitors work by preventing the kidneys from reabsorbing glucose back into the blood, leading to increase in excretion of glucose through urine, thereby lowering hyperglycemia. Treatment with SGLT2 inhibitors improves A1C levels, reduces blood pressure and body weight, and is overall well tolerated by patients with T2DM. However, additional data on long-term cardiovascular safety are still needed. Characteristic adverse events include mild genital - urinary tract infection more commonly seen in women than in men, but serious infection is uncommon. Their use should be exercised with extra caution in patients suffering from renal impairment. Further, advancing to dual/triple combinational therapies with SGLT2 inhibitors and existing oral antidiabetic options may prove to be a breakthrough in the management of T2DM.

18.
Indian J Endocrinol Metab ; 23(1): 159-164, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31016171

RESUMO

Von Hippel-Lindau (VHL) disease is an autosomal dominant disorder characterized by various endocrine, nonendocrine, benign, and malignant tumors in various organs. VHL tumor suppressor gene, located on short arm of chromosome 3 is responsible for this. Pheochromocytoma (PCC) is one of the important endocrine manifestations that needs to be ruled out in case of VHL suspicion. In this review, we summarize the endocrine manifestations of VHL disease and their management while giving case history of five such cases.

19.
Heart Surg Forum ; 22(2): E103-E106, 2019 03 04.
Artigo em Inglês | MEDLINE | ID: mdl-31013218

RESUMO

OBJECTIVES: Management of acute aortic intramural hematomas (IMHs) involving the ascending aorta and root remains controversial. Some series have suggested that delaying operative intervention beyond the first 24-hours may be beneficial. METHODS: A retrospective single-institution analysis was performed to identify patients presenting with type A IMH. These patients were classified by whether they underwent surgery within 24 hours or delayed operative intervention. Patients with additional indications for emergent operation, such as acute aortic regurgitation or malperfusion syndromes, were excluded. Outcomes were assessed with logistic regression, and the Kaplan-Meier method was used to analyze long-term survival. RESULTS: Of the 129 patients with acute type A aortic pathology, 36 (27.9%) presented with isolated IMH. IMH patients were less likely to present with acute aortic regurgitation (8.6% versus 27.9%, P = .020) or limb ischemia (0% versus 12.6%, P = .027). Of the IMH patients without other emergent operative indications, 23 (67.6%) underwent surgery within 24 hours. Delayed operative repair was not associated with increased risk of mortality, stroke, or renal failure (all P >.05). Survival analysis showed no difference in survival at 1 year. CONCLUSIONS: In well-selected patients, delayed operation for type An intramural hematoma is not associated with adverse outcomes.


Assuntos
Aneurisma Aórtico/cirurgia , Dissecção Aórtica/cirurgia , Hematoma/cirurgia , Tempo para o Tratamento , Dissecção Aórtica/mortalidade , Aneurisma Aórtico/mortalidade , Feminino , Hematoma/mortalidade , Humanos , Masculino , Pessoa de Meia-Idade , Seleção de Pacientes , Estudos Retrospectivos , Fatores de Risco , Análise de Sobrevida
20.
Indian J Pediatr ; 86(7): 645-647, 2019 07.
Artigo em Inglês | MEDLINE | ID: mdl-30879238

RESUMO

Congenital chylothorax is a relatively uncommon condition seen in newborn period. Treatment comprises of adequate pleural fluid drainage, octreotide infusion, total parenteral nutrition followed by medium chain triglyceride (MCT) based low fat milk preparations. The authors present a case of Cornelia de Lange syndrome with a rare presentation of antenatally diagnosed chlyothorax presenting at birth with respiratory distress. The baby was managed with skimmed milk formulation fortified with coconut oil providing low fat and high proteins.


Assuntos
Quilotórax/congênito , Síndrome de Cornélia de Lange/complicações , Leite , Animais , Quilotórax/complicações , Quilotórax/diagnóstico , Quilotórax/dietoterapia , Síndrome de Cornélia de Lange/diagnóstico , Alimentos Formulados , Humanos , Recém-Nascido , Masculino , Leite/química , Octreotida/administração & dosagem , Octreotida/uso terapêutico , Síndrome do Desconforto Respiratório do Recém-Nascido , Triglicerídeos
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