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Administração de Componentes Sanguíneos e Derivados em Segurança: A importância da Enfermagem
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Sarcoidosis and Multiple Myeloma: A Case Report and Literature Review
Get PDFThe existence of a sarcoidosis-lymphoma syndrome has been previously proposed since the relation between sarcoidosis and an increased risk of lymphoproliferative disorders is well established. Multiple myeloma is a malignant multifocal proliferation of clonal plasma cells within the bone marrow, and its association with sarcoidosis has been rarely described. We present a concurrent diagnosis of sarcoidosis and multiple myeloma and make a brief analysis of the reported cases in the literature. A 65-year-old woman underwent surgery for the excision of a wrist mass that presented 3 years before. Histological analysis showed sarcoid-type epithelioid granulomas without necrosis, establishing soft tissue sarcoidosis. Further evaluation revealed marked interstitial lung parenchyma lesions and large intrathoracic adenopathies. Bronchofibroscopy with transbronchial biopsy confirmed lung sarcoidosis. In addition, blood analysis showed monoclonal IgG kappa gammopathy. A bone marrow biopsy confirmed hypercellularity with 60% plasma cells and plasmocyte infiltration. Thus, the diagnosis of systemic sarcoidosis and multiple myeloma was established simultaneously. In a brief review of the literature, we identified 33 reports of cases with both sarcoidosis and multiple myeloma. We point out the importance of a high level of suspicion for the association of sarcoidosis with malignant haematological diseases such as multiple myeloma.info:eu-repo/semantics/publishedVersio
Upper limb vascular mapping with Doppler ultrasound: Technique precision evaluated in healthy volunteers.
No full textIntroduction: Doppler ultrasound is recommended by international societies for preoperative vascular mapping in vascular access surgery. Literature is scarce regarding data on Doppler ultrasound-associated errors.
Objectives: Our aim was to evaluate Doppler ultrasound precision for upper limb vascular mapping.
Methods: Fifty-two adult healthy volunteers were evaluated for superficial vein diameter, brachial artery flow and diameter in the lower third of non-dominant arm by a dedicated vascular access radiologist blinded for the identification of the participants. Each participant was scheduled for three evaluations one week apart. Friedman test and multivariate analysis of variance for repeated measures were used.
Results: There were no statistical differences within subjects across the three weeks except for brachial artery flow in participants who had basilic vein as the dominant vein.
Discussion: Repeated anatomical and haemodynamic parameters measured by Doppler ultrasound performed by an experienced medical sonographer, according to our protocol, did not show statistical differences within subjects, independently of age, gender and body mass index.info:eu-repo/semantics/publishedVersio
Extensive Linear Scleroderma en Coup De Sabre With Exertion-Induced Hemiplegic Migraine
No full textWe report the case of a 9-year-old girl with linear scleroderma en coup de sabre (LSCS) who developed progressive white matter involvement, presenting as intractable hemiplegic migraine-like attacks induced by exercise. After a period of severely aggressive course, clinical and radiological stabilization was achieved under immunosuppressant treatment. Intrathecal synthesis of IgG and lymphocytic pleocytosis provided indirect evidence of a chronic inflammatory process of the central nervous system. We discuss the possible immunopathogenic mechanisms responsible for the neurocutaneous involvement in LSCS, favouring the hypothesis of an autoimmune and inflammatory vasculopathy. The singular occurrence of hemiplegic migraine triggered by exertion add further insight to the currently unknown pathogenesis of scleroderma disorder. In addition, we highlight the importance of intensive immunosuppression approaches in selected cases, contrasting with the classic benign course of LCSC.info:eu-repo/semantics/publishedVersio
Genetic modulators of fetal hemoglobin expression and ischemic stroke occurrence in African descendant children with sickle cell anemia
No full textSickle cell anemia (SCA) is an autosomal recessive monogenic disease with significant clinical variability. Cerebrovascular disease, particularly ischemic stroke, is one of the most severe complications of SCA in children. This study aimed to investigate the influence of genetic variants on the levels of fetal hemoglobin (Hb F) and biochemical parameters related with chronic hemolysis, as well as on ischemic stroke risk, in ninety-one unrelated SCA patients, children of sub-Saharan progenitors. Our results show that a higher Hb F level has an inverse relationship with the occurrence of stroke, since the group of patients who suffered stroke presents a significantly lower mean Hb F level (5.34 ± 4.57% versus 9.36 ± 6.48%; p = 0.024). Furthermore, the co-inheritance of alpha-thalassemia improves the chronic hemolytic pattern, evidenced by a decreased reticulocyte count (8.61 ± 3.58% versus 12.85 ± 4.71%; p < 0.001). In addition, our findings have confirmed the importance of HBG2 and BCL11A loci in the regulation of Hb F expression in sub-Saharan African SCA patients, as rs7482144_A, rs11886868_C, and rs4671393_A alleles are significantly associated with a considerable increase in Hb F levels (p = 0.019, p = 0.026, and p = 0.028, respectively). Concerning KLF1, twelve different variants were identified, two of them novel. Seventy-three patients (80.2%) presented at least one variant in this gene. However, no correlation was observed between the presence of these variants and Hb F level, severity of hemolysis, or stroke occurrence, which is consistent with their in silico-predicted minor functional consequences. Thus, we conclude that the prevalence of functional KLF1 variants in a sub-Saharan African background does not seem to be relevant to SCA clinical modulation.info:eu-repo/semantics/publishedVersio
Unilateral leukemic infiltration and acute angle closure as the first sign of B-cell acute lymphoblastic leukemia relapse
Get PDFObjective: Unilateral ocular leukemic infiltration with acute angle closure is an infrequent complication of B-cell acute lymphoblastic leukemia (ALL-B). We present a clinical case of leukemic ocular infiltration as the sole manifestation of ALL-B relapse. Methods: Case description Results: A 15-year-old female with a history of acute lymphoblastic leukemia in remission for 2 years and pulmonary tuberculosis treated in the past year presented with ocular redness and decreased visual acuity in the left eye (LE) with 5 days of evolution. Visual acuity was 20/20 in the right eye (RE) and absence of light perception in the left eye (LE). Biomicroscopy of LE showed a small hypopion, anterior chamber cells 4+, vitreous cells 3+, and a large white mass in the vitreous with associated vitreous hemorrhage in organization. In LE fundoscopy, the vitreous mass occupying most of the vitreous cavity and associated hemorrhage prevented retina visualization. B-scan ultrasound showed a multilobulated mass occupying virtually the entire vitreous cavity with associated choroidal detachment. Forty-eight hours later, she developed acute angle closure of LE with an IOP of 55 mmHg. A flow cytometric analysis of the anterior chamber and vitreous showed leukemic tumor cells. The microbiologic exam and PCR for Mycobacterium tuberculosis were negative. No other signs of relapse of the disease were identified after investigation by the oncology department. Rescue treatment of the underlying disease was started, with symptomatic improvement. Conclusion: Leukemic ocular infiltration can be the only manifestation of ALL-B relapse.info:eu-repo/semantics/publishedVersio
Effectiveness of delayed-release dimethyl fumarate on patient-reported outcomes and clinical measures in patients with relapsing-remitting multiple sclerosis in a real-world clinical setting: PROTEC.
Get PDFEnsaio clínico PROTEC, Protocolo nº 109MS408Abstract
BACKGROUND:
Patient-reported outcomes (PRO) and clinical outcomes give a broad assessment of relapsing-remitting multiple sclerosis (RRMS) disease.
OBJECTIVE:
The aim is to evaluate the effectiveness of delayed-release dimethyl fumarate (DMF) on disease activity and PROs in patients with RRMS in the clinic.
METHODS:
PROTEC, a phase 4, open-label, 12-month observational study, assessed annualized relapse rate (ARR), proportion of patients relapsed, and changes in PROs. Newly diagnosed and early MS (≤3.5 EDSS and ≤1 relapse in the prior year) patient subgroups were evaluated.
RESULTS:
Unadjusted ARR at 12 months post-DMF versus 12 months before DMF initiation was 75% lower (0.161 vs. 0.643, p < 0.0001) overall (n = 1105) and 84%, 77%, and 71% lower in newly diagnosed, ≤3.5 EDSS, and ≤1 relapse subgroups, respectively. Overall, 88% of patients were relapse-free 12 months after DMF initiation (84%, newly diagnosed; 88%, ≤3.5 EDSS; 88%, ≤1 relapse). PRO measures for fatigue, treatment satisfaction, daily living, and work improved significantly over 12 months of DMF versus baseline.
CONCLUSION:
At 12 months after versus 12 months before DMF initiation, ARR was significantly lower, the majority of patients were relapse-free, and multiple PRO measures showed improvement (overall and for subgroups), suggesting that DMF is effective based on clinical outcomes and from a patient perspective.Clinical trial: A Study Evaluating the Effectiveness of Tecfidera (Dimethyl Fumarate) on Multiple Sclerosis (MS) Disease Activity and Patient-Reported Outcomes (PROTEC), NCT01930708,info:eu-repo/semantics/publishedVersio
Neonatal Brachial Plexus Palsy: Risk Factors and Its Prognostic Value
Get PDFIntroduction: Neonatal brachial plexus palsy affects 0.7
to 5.8 per 1,000 newborns and is characterised by upper
limb paresis detected in the immediate neonatal period.
Shoulder dystocia, instrumental delivery and foetal
macrosomia are well-known risk factors. Most neonatal
brachial plexus palsy evolve favourably, while 3%-27% of
newborns have sequelae.
Methods: A retrospective cross-sectional study was
conducted to characterise neonatal brachial plexus
palsy in the newborn population of a hospital with
differentiated perinatal support and to assess the rela -
tionship between the risk factors and lesion prognosis.
The authors reviewed the newborn medical records
referred to the physical medicine and rehabilitation
clinic between January 2006 and December 2016.
Results: During the study period, 137 cases of neo-
natal brachial plexus palsy were identified in 36,833
births, which translate into an incidence of 3.7/1,000
live births. Foetal macrosomia was found in 41% and
shoulder dystocia in 40%. According to the Narakas clas-
sification, 58% were included in group I, 30% in group
II, 9% in group III and 3% in group IV. The majority of
patients were discharged without sequelae. Newborns
with group II, III and IV lesions as well as macrosomic
newborns were more likely to develop sequelae (p <
0.05). Shoulder dystocia and operative delivery did not
present a statistically significant relationship with the
prognosis of the lesion.
Discussion: The incidence of neonatal brachial plexus
palsy in this population was similar to is described in
other series. The relationship between macrosomia and
neonatal brachial plexus palsy with sequelae found may
be of importance in the attempt to prevent this lesioninfo:eu-repo/semantics/publishedVersio
Transmesocolic hernia with sigmoid colon strangulation without surgical history: a series of two case reports.
Get PDFThe incidence of internal hernias is rare (0.2-0.9%). The prevalence of intestinal obstruction for an internal hernia is low (0.5-5%), however if strangulation is present the overall mortality is higher than 50%. There are multiple places where an internal hernia may be localized, with transmesenteric: transmesocolic (8%) and transomental (1-4%) as the rarest. We report a series of two cases (men with 40 years-old and women with 92 years old) of volvulus of colon sigmoid in a strangulated transverse and descendent transmesocolic hernia, with one case associated also to a transomental hernia. Both patients were submitted to a Hartmann procedure and on follow-up remained free of complains. In conclusion, transmesenteric internal hernia should be included as diagnosis hypothesis for intestinal occlusion and if the diagnosis is made, the patient should be submitted to emergency surgery due to high rates of complications, high morbidity and mortality.info:eu-repo/semantics/publishedVersio
Artemether/Lumefantrine for the Treatment of P. malariae in a Patient on Hemodialysis
Get PDFThe combination of artemether/lumefantrine is indicated for the treatment of acute uncomplicated Plasmodium falciparum malaria. There have been no clinical trials to assess the efficacy of this medication in patients with renal impairment. While it is unlikely that artemether/lumefantrine would be removed during dialysis, clinical experience regarding drug use in this setting is limited. In this article, the authors report successful treatment of Plasmodium malariae malaria on a patient with end-stage kidney disease undergoing hemodialysis.info:eu-repo/semantics/publishedVersio