Hospital de Santa Maria
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Glucocorticoid Therapy and Suicidal Behavior – a case report
Os corticosteroides são medicamentos muito usados no alívio de sinais e
sintomas derivados de muitas doenças, incluindo as inflamatórias e autoimunes,
e são os anti-inflamatórios/imunossupressores mais frequentemente prescritos em
todo o mundo. Contudo, os corticosteroides podem também induzir efeitos adversos
significativos. Consequências neuropsiquiátricas graves, incluindo psicose, mania,
depressão, delirium, compromisso cognitivo, suicídio e tentativa de suicídio têm sido
reportadas. Estes efeitos adversos ocorrem em até 60% dos doentes sob corticoterapia.
Ainda assim, a previsão de tais efeitos é difícil, e os riscos variam com a idade, género,
posologia, antecedentes psiquiátricos e alguns marcadores biológicos.
Apresentamos um caso clínico de um doente sem antecedentes psiquiátricos,
que faz uma tentativa de suicídio durante o tratamento com corticosteroides, e realizamos
uma revisão da literatura usando a base de dados PubMed.
A educação, tanto dos doentes quanto das suas famílias, acerca destes eventos
adversos, e o aumento do nível de alerta por parte dos médicos acerca da sua
ocorrência, poderá facilitar uma monitorização mais rigorosa. Quando a redução
da dose de corticoterapia não for suficiente para o controlo da sintomatologia, será
importante o tratamento sintomático com psicotrópicos.info:eu-repo/semantics/publishedVersio
Benign follicular tumors
Benign follicular tumors comprise a large and heterogeneous group of neoplasms that share a common histogenesis and display morphological features resembling one or several portions of the normal hair follicle, or recapitulate part of its embryological development. Most cases present it as clinically nondescript single lesions and essentially of dermatological relevance. Occasionally, however, these lesions be multiple and represent a cutaneous marker of complex syndromes associated with an increased risk of visceral neoplasms. In this article, the authors present the microscopic structure of the normal hair follicle as a basis to understand the type and level of differentiation of the various follicular tumors. The main clinicopathological features and differential diagnosis of benign follicular tumors are then discussed, including dilated pore of Winer, pilar sheath acanthoma, trichoadenoma, trichilemmoma, infundibuloma, proliferating trichilemmal cyst/tumor, trichoblastoma and its variants, pilomatricoma, trichodiscoma/fibrofolliculoma, neurofollicular hamartoma and trichofolliculoma. In addition, the main syndromes presenting with multiple follicular tumors are also discussed, namely Cowden, Birt-Hogg-Dubé, Rombo and Bazex-Dupré-Christol syndromes, as well as multiple tumors of follicular infundibulum (infundibulomatosis) and multiple trichoepitheliomas. Although the diagnosis of follicular tumors relies on histological examination, we highlight the importance of their knowledge for the clinician, especially when in presence of patients with multiple lesions that may be the cutaneous marker of a cancer-prone syndrome. The dermatologist is therefore in a privileged position to recognize these lesions, which is extremely important to provide further propedeutic, appropriate referral and genetic counseling for these patients.info:eu-repo/semantics/publishedVersio
"Gunslinger's gait": a new cause of unilaterally reduced arm swing
OBJECTIVE:
To postulate a new possible cause of a unilaterally reduced arm swing in addition to the known medical conditions such as shoulder pathology, Erb's palsy, stroke, and Parkinson's disease.
METHODS:
Analysis of YouTube videos depicting the gait of highly ranked Russian officials.
RESULTS:
We found a similar walking pattern in President Vladimir Putin, Prime Minister Dmitry Medvedev and three other highly ranked Russian officials, all presenting with a consistently reduced right arm swing in the absence of other overt neurological abnormalities.
CONCLUSIONS:
We propose that this new gait pattern, which we term "gunslinger's gait," may result from a behavioural adaptation, possibly triggered by KGB or other forms of weapons training where trainees are taught to keep their right hand close to the chest while walking, allowing them to quickly draw a gun when faced with a foe. This should be included in the differential diagnosis of a unilaterally reduced arm swing.info:eu-repo/semantics/publishedVersio
Anestesia Pediátrica em Período de Imaturidade Cerebral e Perturbações do Neurodesenvolvimento
INTRODUCTION:
Several experimental and clinical studies suggest that drugs used in pediatric anesthesia may exert undesirable effects on the developing central nervous system. The objective of this review was to assess the results and conclusions of published studies on long lasting neurodevelopment disorders following exposure to anesthetics in children in a phase of brain immaturity.
MATERIAL AND METHODS:
We performed a literature search in several sources (PubMed, SciELO and Cochrane Library) using the terms 'Pediatric anesthesia OR Pediatric anesthetic OR Developing brain anesthetic OR Developing brain anesthesia AND behavior disorders'. We selected human studies, referring to long lasting neurodevelopment effects after exposure to anesthetics in the first four years of life.
RESULTS:
Ten retrospective studies met the inclusion criteria. Of these, seven suggest risk of neurobehavioral disorders after exposure of small children to anesthetics, as opposed to the results obtained by the other three.
DISCUSSION:
Although mostly using large databases, the studies found are retrospective, vary in test groups, include sometimes avoidable confounders and some present inaccuracies in the choice of the test and control populations that can compromise the reliability of the results.
CONCLUSION:
Because of the numerous limitations of the few studies available, the reported results are still deemed insufficient to change current clinical practice. However, although it is undisputable that anesthesia should be provided when needed, regardless of age, the warnings found in literature are worrisome, therefore whenever surgery is unavoidable in small children, alternatives that may help reduce the risks of anesthetic exposure should be sought.info:eu-repo/semantics/publishedVersio
MÓDULO 8 - 2º Curso de Formação para Internos 2013 - 2014:Pediatria do Internamento de Medicina
L’utilisation combinée des scores GRACE et CRUSADE pour la stratification du risque d’infarctus du myocarde
BACKGROUND:
Risk assessment is fundamental in the management of acute coronary syndromes (ACS), enabling estimation of prognosis.
AIMS:
To evaluate whether the combined use of GRACE and CRUSADE risk stratification schemes in patients with myocardial infarction outperforms each of the scores individually in terms of mortality and haemorrhagic risk prediction.
METHODS:
Observational retrospective single-centre cohort study including 566 consecutive patients admitted for non-ST-segment elevation myocardial infarction. The CRUSADE model increased GRACE discriminatory performance in predicting all-cause mortality, ascertained by Cox regression, demonstrating CRUSADE independent and additive predictive value, which was sustained throughout follow-up. The cohort was divided into four different subgroups: G1 (GRACE<141; CRUSADE<41); G2 (GRACE<141; CRUSADE≥41); G3 (GRACE≥141; CRUSADE<41); G4 (GRACE≥141; CRUSADE≥41).
RESULTS:
Outcomes and variables estimating clinical severity, such as admission Killip-Kimbal class and left ventricular systolic dysfunction, deteriorated progressively throughout the subgroups (G1 to G4). Survival analysis differentiated three risk strata (G1, lowest risk; G2 and G3, intermediate risk; G4, highest risk). The GRACE+CRUSADE model revealed higher prognostic performance (area under the curve [AUC] 0.76) than GRACE alone (AUC 0.70) for mortality prediction, further confirmed by the integrated discrimination improvement index. Moreover, GRACE+CRUSADE combined risk assessment seemed to be valuable in delineating bleeding risk in this setting, identifying G4 as a very high-risk subgroup (hazard ratio 3.5; P<0.001).
CONCLUSIONS:
Combined risk stratification with GRACE and CRUSADE scores can improve the individual discriminatory power of GRACE and CRUSADE models in the prediction of all-cause mortality and bleeding. This combined assessment is a practical approach that is potentially advantageous in treatment decision-making
Intellectual disability, coarse face, relative macrocephaly, and cerebellar hypotrophy in two sisters
We report on two Portuguese sisters with a very similar phenotype characterized by severe intellectual disability, absent speech, relative macrocephaly, coarse face, cerebellar hypotrophy, and severe ataxia. Additional common features include increased thickness of the cranial vault, delayed dental eruption, talipes equino-varus, clinodactyly, and camptodactyly of the fifth finger. The older sister has retinal dystrophy and the younger sister has short stature. Their parents are consanguineous. We suggest this condition constitutes a previously unreported autosomal recessive entity
Gender mismatch between donor and recipient is a factor of morbidity but does not condition survival after cardiac transplantation
We intended to evaluate the influence of sex mismatch between donor and recipient, which is still under much debate, on survival and comorbidities after cardiac transplantation. From November 2003 to December 2013, a total of 258 patients were transplanted in our center. From these, 200 receptors were male (77.5%) and constituted our study population, further divided into those who received the heart from a female donor (Group A) - 44 patients (22%) and those who received it from a male donor (Group B) - 156 (78%). Median follow-up was 4.2 ± 3.0 years (1-10 years). The two groups were quite comparable with each other, except for body mass index, systolic pulmonary artery pressure, and transpulmonary gradient, which were significantly lower in Group A. A low donor/recipient weigh ratio (<0.8) was avoided whenever possible. Hospital mortality was not different in the two groups. During follow-up, global survival was similar, as was survival free from acute cellular rejection and cardiac allograft vasculopathy. However, patients in Group A had decreased survival free from serious infections and malignant tumors. Allocation of female donors to male receptors can be done safely, at least in receptors without pulmonary hypertension and when an adequate donor/recipient weigh ratio is ensured
Molecular and cytogenetic characterization of expanded B-cell clones from multiclonal versus monoclonal B-cell chronic lymphoproliferative disorders.
Chronic antigen-stimulation has been recurrently involved in the earlier stages of monoclonal B-cell lymphocytosis, chronic lymphocytic leukemia and other B-cell chronic lymphoproliferative disorders. Among these individuals, expansion of ≥2 B-cell clones has been frequently reported; potentially, such coexisting clones have a greater probability of interaction with common immunological determinants. Here, we comparatively analyzed the B-cell receptor repertoire and the molecular profile, as well as the phenotypic, cytogenetic and hematological features of 228 chronic lymphocytic leukemia-like and non-chronic lymphocytic leukemia-like clones between multiclonal (n=85 clones from 41 cases) versus monoclonal (n=143 clones) monoclonal B-cell lymphocytosis, chronic lymphocytic leukemia and other B-cell chronic lymphoproliferative disorders. The B-cell receptor of B-cell clones from multiclonal cases showed a slightly higher degree of HCDR3 homology than B-cell clones from monoclonal cases, in association with unique hematological (e.g. lower B-lymphocyte counts) and cytogenetic (e.g. lower frequency of cytogenetically altered clones) features usually related to earlier stages of the disease. Moreover, a subgroup of coexisting B-cell clones from individual multiclonal cases which were found to be phylogenetically related, showed unique molecular and cytogenetic features: they more frequently shared IGHV3 gene usage, shorter HCDR3 sequences with a greater proportion of IGHV mutations and del(13q14.3), than other unrelated B-cell clones. These results would support the antigen-driven nature of such multiclonal B-cell expansions, with potential involvement of multiple antigens/epitopes