177,345 research outputs found
Ebstein anomaly with blood cyst
No full textBlood cysts are congenital, located on the endocardium, particularly along the lines of closure of heart valves. The cysts are thin walled, lined by flattened, cobblestone shaped, endothelium and filled with non-organized blood. They are rarely found in adults and in some cases may be mistaken for malignancies. Ebstein anomaly is a rare congenital malformation, the clinical presentation of which varies from her form and anatomical severity, and also with other associated heart diseases. The association between EA and blood cyst is rare but reported in the medical literature.We are presenting the case of 32 years old woman with Ebstein anomaly and new tumor formation adopted and considered to be malignant. There is discussion of the probability that the formation is associated with endothelial injury in the course of the preceding RFA in 2010The patient was directed to cardiac surgery. The treatment included extirpation of the tumor formation from the right ventricle and tricuspid valve and tricuspid valve replacement in order to correct Ebstein anomaly. Morphological description fulfills criteria for Blood cyst: thin-walled cystic formation with myxoid stroma fiber upholstered in single-endothelium of small focuses of endothelial proliferation, filled with blood clot attached to the wall with 2-3 seats.Key words: Ebstein anomaly, blood cyst, congenital malformatio
Anaesthetic management in a parturient with Ebstein′s anamoly
No full textEbstein′s anamoly is a rare congenital heart defect comprising less than 1% of patients with congenital heart disease. Among the congenital heart lesions, Ebstein′s anamoly is one of the most diverse in presentation, severity and management. Even though pregnancy in women with Ebstein′s anamoly is usually well-tolerated, they are at increased risk of developing ventricular and supraventricular tachycardias, congestive cardiac failure, sudden collapse and even death. Here, we report a case of the pregnant woman with mild pre-eclampsia, known case of Ebstein′s anamoly posted for emergency caesarean section
Ebstein Anomaly
No full textKey Facts
Incidence: 1–5/20,000 live births.
Inheritance: 6 %, but occurs sporadically, related to maternal age and maternal
exposure to benzodiazepines or lithium therapy.
Medication: Change antiarrhythmic drugs; a beta-blocker as an alternative is
the first choice.
World Health Organization class: Class II or III.
Risk of pregnancy: Atrial arrhythmias, heart failure, thromboembolic events
(possibly paradoxical in the presence of a shunt).
Life expectancy: Reduced.
Key Management
Preconception: ECG, echo, exercise test; consider cMR.
Pregnancy: Careful detection of arrhythmias and cardiac failure; consider
thromboprophylaxis.
Labor: Vaginal delivery.
Postpartum: Consider thromboprophylaxis
Hyperosmolarity Induces Armanni-Ebstein-like Renal Tubular Epithelial Swelling and Cytoplasmic Vacuolization
No full textArmanni-Ebstein lesions have been considered pathognomonic for diabetes mellitus and appear as markedly swollen renal tubular epithelial cells with cytoplasmic clearing and glycogen accumulation. However, the extent to which hyperosmolarity contributes to the Armanni-Ebstein phenotype is unclear. Ten sheep were injected intravenously with 20% mannitol at 11 mOsm/kg, and subsequent histological evaluation of the kidneys showed variable degrees of osmotic nephrosis and cytoplasmic clearing of renal tubular epithelial cells similar to that seen with Armanni-Ebstein lesions. However, although morphological changes similar to Armanni-Ebstein lesions could be produced, no intracytoplasmic glycogen was demonstrated with periodic Acid-Schiff (PAS) stain. This suggests that while hyperosmolarity may contribute to the development of an Armanni-Ebstein phenotype, glycogen accumulation may result from the more complex metabolic effects of glucose on renal tubular epithelial cells. Thus, when Armanni/Ebstein-like vacuolizations are seen at autopsy, a confirmatory PAS stain is recommended because of the potential effect of hyperosmolar states
Armanni-Ebstein phenomenon and hypothermia
No full textRetrospective review was undertaken of 46 cases of lethal hypothermia for the presence of subnuclear vacuolization of renal tubular epithelial cells. Fifteen of the 46 cases (33%) had renal tubular vacuolization typical of the Armanni-Ebstein phenomenon. The age range was 30-87 years (average 59 years) with a male to female ratio of 6:9. Nine of the 15 cases with Armanni-Ebstein changes (60%) had a history of diabetes mellitus, and in seven of these vitreous humour biochemical analyses were performed, all of which revealed diabetic ketoacidosis (vitreous glucose levels = 32.9-85.3 mmol/L; β-hydroxybutyrate = 7.4-20 mmol/L). This study has confirmed the association between hypothermia and renal tubular epithelial vacuolization, but in addition raises the prospect that this may be contributed to in some cases by underlying diabetic ketoacidosis. Hypothermic deaths should, therefore, raise the possibility of diabetes mellitus and initiate postmortem biochemical measurement of vitreous humor glucose and β-hydroxybutyrate levels.Chong Zhou, Roger W. Byar
QRS duration and QRS fractionation on surface electrocardiogram are markers of right ventricular dysfunction and atrialization in patients with Ebstein anomaly
No full textEbstein anomaly is a rare and heterogeneous congenital heart defect affecting the tricuspid valve and right ventricular (RV) myocardium. Few studies have analysed the electrocardiographic features of Ebstein anomaly and none has addressed correlations with disease severity. Patients with Ebstein anomaly who had undergone electrocardiography and cardiac magnetic resonance (CMR) within 6 weeks between 2001 and 2009 were included. Exclusion criteria were: associated congenital cardiac defect, previous RV myoplasty and/or reduction surgery, class I anti-arrhythmic drug therapy, and paced/pre-excited QRS. Standard electrocardiogram (ECG) findings were correlated with CMR-based RV measures and clinical profile. The mean age of the 63 study patients was 22 13 years. An RV conduction delay (rsR pattern in right precordial leads) was present in 45 patients (71). The QRS duration correlated with anatomic RV diastolic volume (r 0.56, P 0.0001) and inversely with RV ejection fraction (EF; r 0.62, P 0.0001). The presence of QRS fractionation predicted greater atrialized RV volume (80 31 vs. 45 37 mL/m(2), P 0.001). Normal QRS duration was associated with smaller anatomic RV diastolic volume (150 57 vs. 256 100 mL/m(2); P 0.0001), higher RV EF (48 6 vs. 34 14; P 0.0001), higher oxygen consumption (VO2) at cardiopulmonary exercise (25.8 vs. 21.8 mL/kg/min, P 0.05) and lower incidence of oxygen desaturation with exercise (25 vs. 65, P 0.02). Delayed and prolonged depolarization of the RV is common in patients with Ebstein anomaly. The QRS duration is a marker of RV enlargement and dysfunction. QRS fractionation is associated with a greater atrialized RV volume. A preserved surface ECG identifies a subset of patients with Ebstein anomaly with mild morphological and functional abnormalities and better clinical profile
Erosive gastritis, Armanni-Ebstein phenomenon and diabetic ketoacidosis
No full textThe Armanni-Ebstein phenomenon, which is found in the kidneys in diabetic ketoacidosis, has also been proposed as an independent diagnostic postmortem marker for hypothermia. A case is reported to demonstrate the possibility of a more complex inter-related etiology in certain instances. A 44-year-old man with a past history of hospital admission for hypothermia, alcoholism and insulin dependent diabetes mellitus was found dead at his home address. At autopsy there were prominent superficial erosive gastritis (Wischnewsky spots) in keeping with terminal hypothermia. In addition there was also marked cortical pallor of the kidneys due to subnuclear renal tubular epithelial vacuolization (Armanni-Ebstein phenomenon). Thus there was evidence for both hypothermia and Armanni-Ebstein phenomenon, suggesting a relationship. Subsequent biochemical testing of vitreous humor, however, demonstrated markedly elevated levels of glucose (36.5 mmol/l; N = 3.6–6.0 mmol/l), β-hydroxybutyrate (23.2 mmol/l; N < 0.3 mmol/l), and lactate (29.4 mmol/l; N = 0.2–2.0 mmol/l). Death was, therefore, due to diabetic ketoacidosis complicated by hypothermia. Diabetes mellitus has a known association with both hypothermia and Armanni-Ebstein phenomenon, thus, before renal tubular vacuolization can be taken as a marker of hypothermia in isolation, it is important to consider the possibility that in certain cases underlying diabetic ketoacidosis may be present.Roger W. Byard and Chong Zho
Armanni–Ebstein lesions: A need for clarification
No full textArmanni-Ebstein lesions were first described by Luciano Armanni, a pathologist at the University of Naples, during autopsy studies undertaken in 1872, as a unique vacuolar nephropathy associated with poorly controlled diabetes that involves selective renal epithelial cell glycogen accumulation. However, within the last two decades, a broader range of vacuolar changes, including lipid deposition, have also been termed Armanni-Ebstein (AE) lesions, creating some confusion on possible etiology. We would suggest that the term AE phenomenon would be best reserved for the original clear cell change associated with glycogen deposition, and that this should be clearly distinguished from subnuclear lipid vacuolization ("basal vacuolization"). Although there is obvious inter-relation between these two types of vacuoles, they appear morphologically and biochemically distinct from each other. More precise classification may assist in clarifying the causal processes and possible diagnostic significance of different types of renal epithelial vacuolization at autopsy.Chong Zhou, Andrea J. Yool, James Nolan and Roger W. Byar
Ebstein Anomaly in the Fetus: Preparing for the Perfect Storm
No full textThe diagnosis and management of Ebstein anomaly from fetal to neonatal life is discussed in this review. Echocardiographic findings can be particularly helpful in understanding the physiologic impact of the disease and to guide management.</jats:p
Appropriate Similarity Measures for Author Cocitation Analysis
No full textWe provide a number of new insights into the methodological discussion about author cocitation analysis. We first argue that the use of the Pearson correlation for measuring the similarity between authors’ cocitation profiles is not very satisfactory. We then discuss what kind of similarity measures may be used as an alternative to the Pearson correlation. We consider three similarity measures in particular. One is the well-known cosine. The other two similarity measures have not been used before in the bibliometric literature. Finally, we show by means of an example that our findings have a high practical relevance.information science;Pearson correlation;cosine;similarity measure;author cocitation analysis
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