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Manifestação Incomum de uma Entidade Rara: Doença de Erdheim-Chester
Get PDFA 69-year-old woman presented with sudden left hemiparesis. Computed tomography (CT) scan excluded acute brain
injuries. Patient was admitted with acute ischemic stroke of
right hemisphere. There was full recovery within 24 hours and
etiologic studies were normal. There was a concomitant history of ingestion of 6 litres of water per day over the previous
2 years. Water deprivation test followed by the administration
of desmopressin confirmed central diabetes insipidus diagnosis. Brain magnetic resonance imaging (MRI) was normal.
Full body CT scan showed arterial wall thickening suggestive
of large and medium vessels vasculitis, retroperitoneal fibrosis, perinephric infiltration, pleural and pericardial thickening and diffuse densification of greater omentum. Angio-MRI
showed occlusive micro-arterial injuries. Laparoscopic biopsy of greater omentum revealed multinucleated giant Touton
CD68+, CD1A- cells. This finding is pathognomonic of Erdheim-Chester Disease, a rare non-Langerhans histiocytosis.info:eu-repo/semantics/publishedVersio
Predictive factors of discordance between the instantaneous wave-free ratio and fractional flow reserve.
No full textOBJECTIVES:
To identify clinical, angiographic and hemodynamic predictors of discordance between instantaneous wave-free ratio (iFR) and fractional flow reserve (FFR).
BACKGROUND:
The iFR was found to be non-inferior to the gold-standard FFR for guiding coronary revascularization, although it is discordant with FFR in 20% of cases. A better understanding of the causes of discordance may enhance application of these indices.
METHODS:
Both FFR and iFR were measured in the prospective multicenter CONTRAST study. Clinical, angiographic and hemodynamic variables were compared between patients with concordant values of FFR and iFR (cutoff ≤0.80 and ≤0.89, respectively).
RESULTS:
Out of the 587 patients included, in 466 patients (79.4%) FFR and iFR agreed: both negative, n = 244 (41.6%), or positive, n = 222 (37.8%). Compared with FFR, iFR was negative discordant (FFR+/iFR-) in 69 (11.8%) patients and positive discordant (FFR-/iFR+) in 52 (8.9%) patients. On multivariate regression, stenosis location (left main or proximal left anterior descending) (OR: 3.30[1.68;6.47]), more severe stenosis (OR: 1.77[1.35;2.30]), younger age (OR: 0.93[0.90;0.97]), and slower heart rate (OR: 0.59[0.42;0.75]) were predictors of a negative discordant iFR. Absence of a beta-blocker (OR: 0.41[0.22;0.78]), older age (OR: 1.04[1.00;1.07]), and less severe stenosis (OR: 0.69[0.53;0.89]) were predictors of a positive discordant iFR.
CONCLUSIONS:
During iFR acquisition, stenosis location, stenosis degree, heart rate, age and use of beta blockers influence concordance with FFR and should be taken into account when interpreting iFR.info:eu-repo/semantics/publishedVersio
Hypertensive Disorders during Pregnancy and Risk of Bronchopulmonary Dysplasia in Very Preterm Infants.
Get PDF It is not yet fully known whether hypertensive disorders (HTD) during pregnancy impose an increased risk of development of bronchopulmonary dysplasia (BPD) in preterm newborn infants.
OBJECTIVE:
To test the hypothesis that preeclampsia and other HTD are associated with the development of BPD in preterm infants.
MATERIALS AND METHODS:
Data on mothers and preterm infants with gestational age 24 to 30 weeks were prospectively analyzed in 11 Portuguese level III centers. Statistical analysis was performed using IBM SPSS statistics 23.
RESULTS:
A total of 494 preterm infants from 410 mothers were enrolled, and 119 (28%) of the 425 babies, still alive at 36 weeks, developed BPD. The association between chronic arterial hypertension, chronic arterial hypertension with superimposed preeclampsia, and gestational hypertension in mothers and BPD in preterm infants was not significant (p = 0.115; p = 0.248; p = 0.060, respectively). The association between preeclampsia-eclampsia and BPD was significant (p = 0.007). The multivariate analysis revealed an association between preeclampsia-eclampsia and BPD (odds ratio [OR] = 4.6; 95% confidence interval [CI] 1.529-13.819; p = 0.007) and a protective effect for BPD when preeclampsia occurred superimposed on chronic arterial hypertension in mothers (OR = 0.077; 95%CI 0.009-0.632; p = 0.017).
CONCLUSION:
The results of this study support the association of preeclampsia in mothers with BPD in preterm babies and suggest that chronic hypertension may be protective for preterm babies.info:eu-repo/semantics/publishedVersio
Food-protein induced proctocolitis in an exclusive breastfed infant
Get PDFIntrodução: A protocolite induzida por proteína alimentar é uma reação de hipersensibilidade não -IgE mediada, mais frequentemente desencadeada pela ingestão de leite de vaca ou soja mas também já reconhecida no
contexto de aleitamento materno exclusivo. Pode manifestar -se nas primeiras semanas de vida e o seu diagnóstico constitui um desafio pela inespecificidade das manifestações clínicas, muitas vezes levando a intervenções
médicas desnecessárias e invasivas. Caso clínico: Os autores apresentam um lactente com um mês de vida sob
aleitamento materno exclusivo, avaliado por dejeções hemáticas, recusa alimentar e irritabilidade, cuja investigação clínica foi consecutivamente negativa e cujas manifestações clínicas reverteram após evicção do leite materno
e início de leite extensamente hidrolizado. Conclusões: A evicção da proteína alimentar desencadeante resulta
numa recuperação clínica geralmente completa e sem sequelas tardias. Pode ser necessária a suspensão do aleitamento materno em lactentes nos quais não seja identificada outra causa para a hipersensibilidade.info:eu-repo/semantics/publishedVersio
Intravenous thrombolysis in acute ischemic stroke due to intracranial artery dissection: a single-center case series and a review of literature
No full textEfficacy and safety of intravenous thrombolysis (IVT) in acute ischemic stroke (AIS) due to intracranial artery dissection (IAD) are currently not established. We aimed to present a single-center experience on IAD-related AIS treated by IVT. We selected all consecutive patients with IAD-related AIS treated by IVT from a prospectively constructed single-center acute stroke registry from 2003 to 2017. We reviewed demographical, clinical and neuroimaging data and recorded hemorrhagic complications, mortality within 7 days and modified Rankin Scale at 3-months. Out of 181 AISs related to cervicocephalic dissections, 10 (5.5%) were due to IAD and five of these patients received IVT. Among these five patients, median age was 62 years; hypertension and dyslipidemia were the most frequent vascular risk factors. IAD locations were distal internal carotid artery, middle cerebral artery (M1), anterior cerebral artery (A2), and, in two cases, the basilar artery. All anterior circulation IADs were occlusive or subocclusive, while the two basilar artery IADs caused arterial stenosis. After IVT, there were no subarachnoid or symptomatic intracranial hemorrhages. One patient had an asymptomatic hemorrhagic infarct type 1. Two patients died within 7 days from ischemic mass effect. The other three patients had favorable clinical outcomes at 3-months. In this small single-center case series of IAD-related AIS, thrombolysis seemed relatively safe. However, IVT efficacy and the likelihood of arterial recanalization are still uncertain in this context. Further studies are needed to assess the safety and efficacy of IVT in these patients.info:eu-repo/semantics/publishedVersio
Admissão de Doentes com Cancro do Pulmão nas Unidades de Cuidados Intensivos
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Hemiconvulsion-hemiplegia-epilepsy syndrome: case report and use of dextromethorphan
No full textinfo:eu-repo/semantics/publishedVersio