Résultats De 5ans De Thyroïdectomie Au Service D’orl Et Chirurgie Cervico-Faciale De l’Hôpital National De Niamey (Niger)

Purpose: To evaluate the results of thyroidectomy in the Oto-RhinoLaryngology and Neck Surgery office of the National Hospital of Niamey in Niger. Materials and methods: we analyzed the epidemiological aspects, the indications, the technics and the evolution of thyroidectomy through a retrospective and descriptive study conducted from January 2010 to December 2015. Results: Thyroidectomy represents 8, 51% (n = 236/2773) of the ENT operative activities and an average of 47.2 cases per year for 5 years. Among the patients, there were 15 men and 221 women, a sex ratio of 0.17. The mean age was 37.25 years (extreme 22 and 61 years). The operative indications are dominated by multinodular goiter euthyroid (43.22%), thyroid nodules (39.40%), hyperthyroidism (goiter and Basedow) and substernal goiters, respectively, representing 8.90% and 6.35%. The gestures performed were subtotal thyroidectomy in 56 cases (23.73%), complete thyroidectomy in 85 cases (36.02%) and lobo-isthmectomy in 95 cases (40.25%). The recurrent nerve was searched in 235 cases (99%) and found in 215 cases (91, 10%). It was found 1 case (0.42%) of splitting of the left nerve recurrent. 2 cases (0.84%) of immediate postoperative hemorrhage was recorded. Morbidity was 0.29% with 4 cases of definitive unilateral recurrent paralysis and 3 cases of transient hypoparathyroidism. No mortality was encountered. Conclusion: The thyroidectomy is a frequent intervention in the ENT and Neck Surgery office of the National Hospital of Niamey. Multinodular goiter remains the first surgical indication. Recurrent and hypoparathyroid morbidity was very low

Utilisation des lambeaux dans les pertes de substances maxillo-faciales: à propos de 32 cas au Niger

Diverses lésions peuvent rompre l’harmonie anatomique, esthétique et fonctionnelle de la face, parmi lesquelles les pertes de substances (PDS) qui peuvent être d’origine traumatique, infectieuse ou tumorale. L’objectif de ce travail est de décrire les aspects épidémiologiques, cliniques et thérapeutiques des patients ayant bénéficiés de prise en charge par lambeaux dans les pertes de substances (PDS) maxillo-faciales. Il s’agit d’une étude rétrospective (du 1er janvier 2016 au 15 juillet 2019) et prospective (du 16 juillet 2019 au 31 octobre 2020), soit une période de 4 ans 10 mois (58mois) réalisée dans les services de Stomatologie et Chirurgie Maxillo-Faciale de l’hôpital national et l’hôpital général de référence de Niamey. Pendant la durée de l’étude, 32 patients de tout âge et des deux sexes ont été enregistré dans le service. La moyenne d’âge était de 34 ans avec des âges inférieur et supérieur de 02 ans et 65 ans. Le sexe féminin était prédominant de 59% des cas. la tranche d’âge de 31-45 ans est la plus touchée avec 31,25% des cas. L’étiologie tumorale était la plus fréquente dans 43,75% suivi des (PDS) post- traumatique dans 34,38% des cas. la région parotidienne représentait la partie la plus atteinte avec 21,88% des cas. Les lambeaux régionaux et ceux à distance étaient les plus utilisés dans 35,29% des cas chacun, et les locaux dans 29,42%. Les nécroses partielles étaient les complications les plus fréquentes soit 15,63% des cas. Les PDS maxillo-faciale sont souvent responsables de certains préjudice fonctionnel et esthétique qui peuvent altérer la personnalité. Ces PDS font appel à des techniques de reconstruction par lambeaux locorégionaux et à distance avec des indications bien précises.   Various lesions can disrupt the anatomical, aesthetic and functional harmony of the face, including loss of substances that can be of traumatic, infectious or tumor origin. The objective of this work is to describe the epidemiological, clinical and therapeutic aspects of patients who have benefited from flap management in maxillofacial loss of substances. This is a retrospective (from January 1, 2016 to July 15, 2019) and prospective (from July 16, 2019 to October 31, 2020), i.e. 4 years 10 months (58 months) carried out in the departments of Stomatology and Maxillofacial surgery at the national hospital and the general reference hospital of Niamey. During the study period, 32 patients of all ages and two sexes were registered in the department. The average age was 34 years with lower and upper ages of 02 years and 65 years. The female sex was predominant in 59% of cases. the 31- 45 age group is the most affected with 31.25% of cases. Tumor etiology was the most frequent in 43.75% followed by post-traumatic (PDS) in 34.38% of cases. the parotid region represented the most affected part with 21.88% of cases. Regional and remote flaps were used the most in 35.29% of cases each, and local ones in 29.42%. Partial necrosis was the most frequent complication, i.e. 15.63% of cases. Maxillofacial loss of substance is often responsible for certain functional and aesthetic damage that can alter the personality. These losses of substance use locoregional and remote flap reconstruction techniques with very precise indications

Long-term follow up of families with pathogenic NFKB1 variants reveals incomplete penetrance and frequent inflammatory sequelae

Publisher Copyright: © 2022 The AuthorsNuclear factor κ light-chain enhancer of activated B cells (NF-κB) family of evolutionarily conserved transcription factors are involved in key cellular signaling pathways. Previously, hypogammaglobulinemia and common variable immunodeficiency (CVID)-like phenotypes have been associated with NFKB1 variants and loss-of-function NFKB1 variants have been reported as the most common monogenic cause for CVID among Europeans. Here, we describe a Finnish cohort of NFKB1 carriers consisting of 31 living subjects in six different families carrying five distinct heterozygous variants. In contrast to previous reports, the clinical penetrance was not complete even with advancing age and the prevalence of CVID/hypogammaglobulinemia was significantly lower, whereas (auto)inflammatory manifestations were more common (42% of the total cohort). At current stage of knowledge, routine genetic screening of asymptomatic individuals is not recommended, but counseling of potential adult carriers seems necessary.Peer reviewe

Somatic mutations and T-cell clonality in patients with immunodeficiency

Common variable immunodeficiency (CVID) and other late-onset immunodeficiencies often co-manifest with autoimmunity and lymphoproliferation. The pathogenesis of most cases is elusive, as only a minor subset harbors known monogenic germline causes. The involvement of both B and T cells is, however, implicated. To study whether somatic mutations in CD4(+) and CD8(+) T cells associate with immunodeficiency, we recruited 17 patients and 21 healthy controls. Eight patients had late-onset CVID and nine patients other immunodeficiency and/or severe autoimmunity. In total, autoimmunity occurred in 94% and lymphoproliferation in 65%. We performed deep sequencing of 2,533 immune-associated genes from CD4(+) and CD8(+) cells. Deep T-cell receptor b-sequencing was used to characterize CD4(+) and CD8(+) T-cell receptor repertoires. The prevalence of somatic mutations was 65% in all immunodeficiency patients, 75% in CVID, and 48% in controls. Clonal hematopoiesis-associated variants in both CD4(+)and CD8(+) cells occurred in 24% of immunodeficiency patients. Results demonstrated mutations in known tumor suppressors, oncogenes, and genes that are critical for immuneand proliferative functions, such as STAT5B (2 patients), C5AR1 (2 patients), KRAS (one patient), and NOD2 (one patient). Additionally, as a marker of T-cell receptor repertoire perturbation, CVID patients harbored increased frequencies of clones with identical complementarity determining region 3 sequences despite unique nucleotide sequences when compared to controls. In conclusion, somatic mutations in genes implicated for autoimmunity and lymphoproliferation are common in CD4(+) and CD8(+) cells of patients with immunodeficiency. They may contribute to immune dysregulation in a subset of immunodeficiency patients.Peer reviewe

Relation between Anemia and Vulnerable Coronary Plaque Components in Patients with Acute Coronary Syndrome: Virtual Histology-Intravascular Ultrasound Analysis

The aim of the present study was to evaluate the plaque components and the predictors of thin-cap fibroatheroma (TCFA) in anemic patients with acute coronary syndrome using virtual histology-intravascular ultrasound (VH-IVUS). Anemia was defined according to criteria of the World Health Organization, (i.e. , hemoglobin levels < 13 g/dL in men and < 12 g/dL in women) and we compared VH-IVUS findings between anemia group (171 patients, 260 lesions) and non-anemia group (569 patients, 881 lesions). Anemia group had greater % necrotic core (NC) volume (21% ± 9% vs 19% ± 9%, P = 0.001) compared with non-anemia group. Hemoglobin level correlated negatively with absolute NC volume (r = -0.235, P < 0.001) and %NC volume (r = -0.209, P < 0.001). Independent predictors of TCFA by multivariate analysis were diabetes mellitus (odds ratio [OR], 2.213; 95% confidence interval [CI], 1.403-3.612, P = 0.006), high-sensitivity C-reactive protein (OR, 1.143; 95% CI, 1.058-1.304, P = 0.012), microalbuminuria (albumin levels of 30 to 300 mg/g of creatinine) (OR, 2.124; 95% CI, 1.041-3.214, P = 0.018), and anemia (OR: 2.112; 95% CI 1.022-3.208, P = 0.028). VH-IVUS analysis demonstrates that anemia at the time of clinical presentation is associated with vulnerable plaque component in patients with acute coronary syndrome

Enoxaparin versus unfractionated heparin with fibrinolysis for ST-elevation myocardial infarction

Background: Unfractionated heparin is often used as adjunctive therapy with fibrinolysis in patients with ST-elevation myocardial infarction. We compared a low-molecular-weight heparin, enoxaparin, with unfractionated heparin for this purpose. Methods: We randomly assigned 20,506 patients with ST-elevation myocardial infarction who were scheduled to undergo fibrinolysis to receive enoxaparin throughout the index hospitalization or weight-based unfractionated heparin for at least 48 hours. The primary efficacy end point was death or nonfatal recurrent myocardial infarction through 30 days. Results: The primary end point occurred in 12.0 percent of patients in the unfractionated heparin group and 9.9 percent of those in the enoxaparin group (17 percent reduction in relative risk, P<0.001). Nonfatal reinfarction occurred in 4.5 percent of the patients receiving unfractionated heparin and 3.0 percent of those receiving enoxaparin (33 percent reduction in relative risk, P<0.001); 7.5 percent of patients given unfractionated heparin died, as did 6.9 percent of those given enoxaparin (P=0.11). The composite of death, nonfatal reinfarction, or urgent revascularization occurred in 14.5 percent of patients given unfractionated heparin and 11.7 percent of those given enoxaparin (P<0.001); major bleeding occurred in 1.4 percent and 2.1 percent, respectively (P<0.001). The composite of death, nonfatal reinfarction, or nonfatal intracranial hemorrhage (a measure of net clinical benefit) occurred in 12.2 percent of patients given unfractionated heparin and 10.1 percent of those given enoxaparin (P<0.001). Conclusions: In patients receiving fibrinolysis for ST-elevation myocardial infarction, treatment with enoxaparin throughout the index hospitalization is superior to treatment with unfractionated heparin for 48 hours but is associated with an increase in major bleeding episodes. These findings should be interpreted in the context of net clinical benefit