Postgraduate Medicine最新文献

This review aims to describe the epidemiology, pathogenesis, clinical manifestations, diagnosis, treatment, and prognosis of eosinophilic granulomatosis with polyangiitis (EGPA). Eosinophilic granulomatosis with polyangiitis is a small to medium vessel necrotizing vasculitis, typically classified with granulomatosis with polyangiitis (GPA) and microscopic polyangitis (MPA) as antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). However, less than 50% of patients with EGPA have a positive ANCA test. Among all the vasculitides, asthma and eosinophilia are unique features of EGPA. Eosinophilic granulomatosis with polyangiitis is very rare and the diagnosis may be missed as the disease evolves over time. Polyneuropathies are common and may be severe, requiring aggressive immunosuppressive therapy. Heart involvement is the most common cause of death in EGPA. Biopsy of involved tissue supports a clinically suspected diagnosis but is not always feasible. Treatment of EGPA is primarily dictated by the severity of disease and prognostic factors. More severe disease frequently requires the use of aggressive therapy such as cyclophosphamide. Once treatment is initiated, patients can achieve good control of symptoms; unfortunately, disease relapses are common and prolonged treatment with corticosteroids is often necessary for asthma management. A better understanding of the disease heterogeneity is needed for the development of better therapies.

This review discusses the clinical manifestations of cutaneous small vessel vasculitis. The etiologies and work up will be explored as well as the treatment considerations. This entity is multifactorial and usually involves multiple specialties. The presentation can range from self-limited to life threatening, multi-organ failure. It is essential to be able to diagnose vasculitis and proceed with the appropriate laboratory studies and work-up. Finally, investigation of associated etiologies such as infection and drugs will guide additional diagnostic studies.

Polyarteritis nodosa (PAN) is a primary form of vasculitis characterized by inflammation of primarily medium-sized arteries. Several key events have shaped the current spectrum of the disease including the separation of a subgroup with microscopic polyangiitis, the discovery of the association of hepatitis B, and the discovery of adenosine deaminase 2 deficiency (DADA2). With the discovery of secondary causes of PAN and changing nomenclature, the incidence of PAN has declined over time. Common manifestations include constitutional symptoms, skin involvement, peripheral neuropathy, gastrointestinal disease, and renal involvement. DADA2 is a genetic cause of medium vessel vasculitis that is important to distinguish from primary PAN as treatment with TNF inhibitors can prevent morbidity and mortality in those with a vasculitis phenotype. Treatment of systemic primary PAN involves the use of systemic immunosuppressive therapy largely guided by the severity of disease. With current treatment regimens, the prognosis has changed from a once uniformly fatal disease to a 5-year survival rate above 80%.

Behçet Disease is a relapsing and remitting variable vessel vasculitis characterized by recurrent mucocutaneous ulcers that can involve almost every organ system in the body. Indeed, the presence of recurrent oral or genital ulcers with other auto-inflammatory symptoms should raise suspicion for this elusive disease. It is unique among the vasculitides in that it can affect vessels of small, medium, and large size and tends to involve venous rather than arterial circulation, and its effects on the pulmonary venous circulation are particularly notable for their role in disease mortality. Classically seen in Mediterranean, Middle-Eastern, and eastern Asian countries, and relatively rare in the United States, prevalence has been increasing, prompting an increased need for internists to be aware of Behcet's clinical presentation and treatment. As early recognition and diagnosis of the disease is key to successful treatment and better prognosis, this review provides a brief summary of the current etiological theories, important clinical manifestations, and treatments including newer biologic alternatives.

Objectives: This study aimed to evaluate the clinical features, management, and outcomes of patients with animal bites presented to the pediatric emergency department of a tertiary center.

Methods: Patients with ICD-10 code W54 (contact with dog) and W55 (contact with other mammals) between March 1^st, 2017, and July 1^st, 2021, were included in the study. Demographic characteristics of the patients, type of contacted animal, wound characteristics (muscle involvement, soft tissue defect, vascular injury, type of nerve injury, and Lackmann's classification), wound care measurements, tetanus prophylaxis, administration of rabies immunoglobulin and antibiotics, location of the injury, existing fractures, suturing, splinting, surgical consultations and hospitalization status were recorded.

Results: Four hundred and nineteen incidents of animal bites (240 males and 179 females) occurred over a four-year period. 51% was due to a dog bite; 47% was by a cat. The median age was nine years (IQR: 5-14 years). Most bites (91.6%) involved only a single anatomical site. The extremities were the commonly involved part (right upper limb [35.3%], left upper limb [21.2%], right lower limb [12.6%], left lower limb [16%]). Head-neck and face injury ratio was 17.6%. Torso (5.7%) and genitalia (5.2%) were uncommonly involved. A consultation was requested from at least one surgical department for 8% of the patients. 97.1% of patients received a rabies vaccine. Most attacks were trivial and did not require hospitalization.

Conclusion: Animal bites often cause minor injuries. However, multiple dog attacks can be seen related to a high number of stray animals in our country. Therefore, these patients may present with major traumas. Surgical intervention and hospitalization may be required. Emergency physicians play an essential role in acute management and rabies prophylaxis in these patients.

Objective: The relevance of this study is conditioned by the need for urgent search and implementation of effective methods of treatment of urinary system diseases in people of different ages, as well as addressing issues of quality treatment of connective tissue diseases in general and its dysplasia in particular. The aim of the article is to identify congenital defects as visceral markers of connective tissue dysplasia.

Methods: The methodology of this study includes a survey of a group of children with considerable problems in the development and functioning of the urinary system at the age of 2 weeks to 3 years, in order to qualitatively select and determine the most effective methods of treatment. Children who took part in this study had a set of phenotypic and clinical properties of undifferentiated connective tissue dysplasia.

Results: The considerable prevalence of undifferentiated connective tissue dysplasia in young children with congenital malformations of the urinary system, especially in children with abnormal development and functioning of kidney tissue, which substantially influences the course of the disease was determined. Also, treatment of undifferentiated connective tissue dysplasia was predicted.

Conclusions: It was concluded that the presence of a malformation of the urinary system, which is acquired by a child from birth, can be considered as a visceral manifestation of undifferentiated connective tissue dysplasia.

Objectives: The aim of this study is to compare the demographic, clinical features, treatment results and outcomes in pediatric patients with idiopathic uveitis and uveitis due to juvenile idiopathic arthritis (JIA) and Behçet's disease (BD).

Methods: 97 pediatric uveitis patients were divided into three groups according to the etiology of uveitis: Group 1 comprised idiopathic uveitis patients, Group 2 uveitis patients who had JIA, and Group 3 uveitis patients with BD.

Results: Symptomatic presentation and intermediate uveitis were more common in Group 1 (p < 0.005). Asymptomatic presentation and anterior uveitis in Group 2 (p < 0.005), whereas symptomatic presentation and posterior uveitis in Group 3 (p < 0.005). Erythrocyte sedimentation rate (ESR) was higher in patients with BD or JIA uveitis than those with idiopathic uveitis (p < 0.005). Biologic therapy was more commonly used in JIA group compared to other groups (p < 0.005). Patients who had a complication related with uveitis were more common in females, asymptomatic disease course, and needed use of biologic treatment than in those without any complication (p < 0.005).

Conclusion: Uveitis accompanying rheumatologic diseases may have asymptomatic and insidious course but have higher ESR as an important notice.

Objectives: Atrial fibrillation (AF) is the most common arrhythmias, which significantly jeopardizes global cardiovascular health through the complicated heart failure and stroke. Published studies have demonstrated the impact of insulin resistance on the genesis of AF. Hence, monitoring insulin resistance may be a possible way to improve the detection of early-stage AF. Accordingly, our work aimed to investigate the association between TyG, a surrogate of insulin resistance, and the prevalent AF, and to evaluate the potential of TyG to refine the detection of prevalent AF in a diabetic population.

Methods: This cross-sectional study was derived from the National Metabolic Management Center Program and included 3244 diabetic patients between September 2017 and December 2020. TyG was calculated as ln[fasting TG (mg/ dL)× FPG (mg/dL)/2]. AF was diagnosed according to electrocardiography and subjects' self-reports.

Results: The prevalence of AF was 6.57%. In the fully adjusted model, each SD elevation of TyG cast a 40.6% additional risk for prevalent AF. In the quartile analysis, the top quartile showed a 2.120 times risk of prevalent AF compared with the bottom quartile. Smooth curve fitting demonstrated that the association was linear in the full range of TyG, and subgroup analysis suggested that the association was robust in several common subpopulations of AF. Furthermore, ROC results displayed an improvement for the detection of prevalent AF when adding TyG into conventional cardiovascular risk factors (0.812vs.0.825, P = 0.019), and continuous net reclassification index (0.227, 95% CI: 0.088-0.365, P = 0.001) and integrated discrimination index (0.007, 95% CI: 0.001-0.012, P = 0.026) also showed the improvement achieved by TyG.

Conclusion: Our data supported a linear and robust correlation between TyG and the prevalent AF in a diabetic population. Moreover, our results implicated the potential usefulness of TyG to refine the detection of prevalent AF in a diabetic population.