关于纤维状淋巴结膜炎的诊断和管理的最新情况
Rose Mary Attieh1, Yihe Yang2, Jordan L Rosenstock3
1Division of Kidney Diseases and Hypertension, Glomerular Center at Northwell Health, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, New York.
Advances in kidney disease and health
|July 31, 2024
概括
纤维状丸炎 (FGN) 是一种罕见的病,其特征是有特色的纤维状. 诊断依赖于免疫组织化学染色,这种渐进的疾病的治疗结果仍然是可变的.
科学领域:
- 腎臟病學 (nephrology) 是一種醫學.
- 病理学 病理学 病理学
- 免疫学 免疫学 免疫学
背景情况:
- 纤维状丸炎 (FGN) 是一种罕见的病.
- 它经常表现为晚期功能衰竭,蛋白尿和高血压.
- FGN与自身免疫性疾病,C型肝炎感染和恶性瘤有关.
研究的目的:
- 总结一下FGN的主要特点.
- 讨论诊断方法和治疗策略.
- 为了突出疾病的异质性和预后.
主要方法:
- 对脏活检结果的审查,包括刚果红色染色和纤维素特征.
- 作为诊断标准的DnaJ同源亚家族B成员9的免疫组织化学染色的讨论.
- 用免疫抑制和rituximab治疗结果的分析.
主要成果:
- FGN显示的是刚果红阴性,非分支的纤维素 (约. 20 nm) 的距离.
- DnaJ同类子家族B成员9染色对于诊断至关重要.
- 存在非典型的变种,如共性,单型性和半月性FGN.
- 治疗的成功是可变的,没有确立的标准治疗.
- 在2-4年内,FGN经常发展为末期病.
- 移植后的复发是令人担忧的.
结论:
- FGN是一种异质性病,预后不佳.
- 通过免疫组织化学方法进行准确的诊断是必不可少的.
- 需要进一步的研究,以建立有效的治疗方法和改善结果.
相关概念视频
Amyloid Fibrils
9.5K
Amyloid fibrils are aggregates of misfolded proteins. Under most circumstances, misfolded proteins are either refolded by chaperone proteins or degraded by the proteasome. However, in the case of a mutation or a disease, these proteins can accumulate to form large clusters and often further assemble to form elongated fibers, called fibrils.
Amyloid deposits were observed as early as 1639 in the liver and the spleen. In 1854, Rudolph Virchow performed iodine staining,...
Amyloid deposits were observed as early as 1639 in the liver and the spleen. In 1854, Rudolph Virchow performed iodine staining,...
9.5K
Renal Failure: Dose Adjustments
77
In patients with renal impairment, drugs undergo significant changes in their pharmacokinetics, which require dosage adjustments to ensure safe and effective therapy.
Reduced renal clearance and elimination rate are common outcomes of renal impairment. These alterations lead to a prolonged elimination half-life and an altered apparent volume of distribution for drugs. As a result, dosage adjustments are typically necessary to maintain optimal drug levels in the body.
However, dosage adjustments...
Reduced renal clearance and elimination rate are common outcomes of renal impairment. These alterations lead to a prolonged elimination half-life and an altered apparent volume of distribution for drugs. As a result, dosage adjustments are typically necessary to maintain optimal drug levels in the body.
However, dosage adjustments...
77
Pulmonary Tuberculosis V
176
Medical management of tuberculosis (TB) patients involves a comprehensive approach that includes diagnosis, treatment, and monitoring. The specific strategies can vary depending on the type of tuberculosis (latent or active), the patient's overall health status, and other considerations.
Latent tuberculosis infection occurs when TB bacteria are present in a person's body, but are not causing illness or symptoms. It is not contagious, and preventive treatment is crucial to avoid the...
Latent tuberculosis infection occurs when TB bacteria are present in a person's body, but are not causing illness or symptoms. It is not contagious, and preventive treatment is crucial to avoid the...
176
Glomerular Filtration Rate and its Regulation
2.4K
The Glomerular Filtration Rate (GFR) is a measure of kidney function, reflecting the volume of filtrate formed per minute in the kidneys. On average, GFR is approximately 125 mL/min in males and 105 mL/min in females. Maintaining a relatively constant GFR is essential for the kidneys to effectively regulate body fluid homeostasis and maintain extracellular stability.
GFR regulation involves two primary intrinsic controls: the myogenic and tubuloglomerular feedback mechanisms.
The myogenic...
GFR regulation involves two primary intrinsic controls: the myogenic and tubuloglomerular feedback mechanisms.
The myogenic...
2.4K
Cystic Fibrosis: Management
151
Cystic fibrosis (CF) is an autosomal recessive disorder that predominantly affects individuals of Northern European descent, occurring at a rate of 1 in 3500. It is caused by a genetic mutation in a gene on chromosome 7, most commonly the ΔF508 mutation, that codes for the cystic fibrosis transmembrane conductance regulator (CFTR) protein. This results in thicker mucus secretions and obstruction pathologies in multiple organs, including the lungs and sinuses.
Sinus disease and chronic...
Sinus disease and chronic...
151
Open Angle Glaucoma: Treatment
420
In open-angle glaucoma, the iridocorneal angle remains open, but the trabecular meshwork becomes stiff, slowing down the outflow of aqueous humor. This causes a buildup of aqueous humor in the anterior chamber, leading to a sudden increase in intraocular pressure. The treatment for open-angle glaucoma focuses on reducing the elevated intraocular pressure by either decreasing the secretion of aqueous humor or increasing its outflow.
Drugs such as carbonic anhydrase inhibitors, α2- and...
Drugs such as carbonic anhydrase inhibitors, α2- and...
420


