隐藏在缺席中:临床病理学见解与选择性IgA缺乏相关的脏疾病
Bangchen Wang1, Harpreet Singh2, Matthew Ellis2
1Department of Pathology, Duke University, Durham, North Carolina.
概括
选择性IgA缺乏症 (sIgAD) 是一种常见的原发性免疫缺陷. 缺少脏活检上的IgA染色可能表明sIgAD,通常与感染和自身免疫性疾病有关.
科学领域:
- 腎臟病學 (nephrology) 是一種醫學專業.
- 免疫学 免疫学 免疫学
- 病理学 病理学 病理学
背景情况:
- 选择性IgA缺乏症 (sIgAD) 是最常见的原发性免疫缺陷症.
- 在活检上缺少IgA免疫光是sIgAD的罕见诊断线索.
- 此前关于这一发现的报道仅限于两名患者.
研究的目的:
- 根据缺席的IgA免疫光,调查疑似SIgAD患者的临床,人口和活检结果.
- 确定缺席IgA染色与临床表现和免疫球蛋白测试结果之间的关联.
主要方法:
- 从15名完全缺乏IgA免疫光的患者的脏活检上回顾性收集数据.
- 对临床表现,病史,免疫球蛋白检测结果和活检诊断的分析.
- 发现与疑似sIgAD诊断的相关性.
主要成果:
- 大多数患者呈现急性损伤和/或蛋白尿.
- 临床病史经常包括复发性感染,自身免疫性疾病,过敏性疾病和癌症.
- 免疫球蛋白测试在有结果的10名患者中,有9名患者证实了sIgAD.
- 脏活检通常显示出与免疫相关的淋巴细胞疾病,最常见的是狼性炎.
结论:
- 在活检上完全没有IgA染色,这可能是sIgAD的迹象.
- 这一发现与一系列的临床条件有关,包括感染,自身免疫性疾病和过敏性疾病.
- 识别这种诊断线索对于管理潜在的sIgAD及其相关并发症的患者至关重要.
更多相关视频
09:43Analyses of Proteinuria, Renal Infiltration of Leukocytes, and Renal Deposition of Proteins in Lupus-prone MRL/lpr Mice
Published on: June 8, 2022
2.8K
14:18Author Spotlight: Investigating the Potential of Chinese Herbal Medicinal Active Dioscin in Treating IgA Nephropathy
Published on: October 13, 2023
1.6K
相关概念视频
01:20Renal Corpuscle
1.3K
The glomerulus and Bowman's capsule are two essential components of the nephron, which is the functional unit of the kidney. These microscopic structures play a critical role in the process of blood filtration to produce urine.
Glomerulus: Structure and Function
The glomerulus is a tiny, intricate network of capillaries located at the beginning of the nephron. It's enveloped by the Bowman's capsule and receives its blood supply from an afferent arteriole, which divides into numerous...
Glomerulus: Structure and Function
The glomerulus is a tiny, intricate network of capillaries located at the beginning of the nephron. It's enveloped by the Bowman's capsule and receives its blood supply from an afferent arteriole, which divides into numerous...
1.3K
01:10Nephrons
1.7K
The kidneys are intricate organs with millions of working units known as nephrons. Each nephron features two major structures: the renal corpuscle, which facilitates blood plasma filtration, and the renal tubule, which handles the glomerular filtrate. Blood supply is directly linked to the nephrons. The renal corpuscle consists of the glomerulus, a capillary network, and the Bowman's capsule, a double-walled epithelial structure that encases the glomerulus. The filtering of blood plasma...
1.7K
01:24Renal Tubule and Collecting Duct
560
The renal tubule is divided into three parts: the proximal convoluted tubule (PCT), the Loop of Henle (LOH), and the distal convoluted tubule (DCT).
Proximal Convoluted Tubule (PCT):
The PCT is the initial segment of the renal tubule, extending from the Bowman's capsule that encloses the glomerulus. Its convoluted structure and microvilli-lined cells increase the surface area for reabsorption. The PCT reabsorbs glucose, amino acids, sodium, and water from the filtrate, ensuring essential...
Proximal Convoluted Tubule (PCT):
The PCT is the initial segment of the renal tubule, extending from the Bowman's capsule that encloses the glomerulus. Its convoluted structure and microvilli-lined cells increase the surface area for reabsorption. The PCT reabsorbs glucose, amino acids, sodium, and water from the filtrate, ensuring essential...
560
01:17Gastritis-II: Pathophysiology
185
Gastritis is marked by disruption of the mucosal barrier that usually protects the stomach tissue from digestive juices and manifests in acute and chronic forms.
In acute gastritis, the gastric mucosa becomes swollen and red and undergoes superficial erosion. Superficial ulceration may lead to bleeding.
In chronic gastritis, persistent or repeated insults lead to chronic inflammatory changes and, eventually, thinning or atrophy of the gastric tissue.
Gastritis can stem from various causes, each...
In acute gastritis, the gastric mucosa becomes swollen and red and undergoes superficial erosion. Superficial ulceration may lead to bleeding.
In chronic gastritis, persistent or repeated insults lead to chronic inflammatory changes and, eventually, thinning or atrophy of the gastric tissue.
Gastritis can stem from various causes, each...
185
01:12Internal Anatomy of the Kidney
1.1K
The kidneys are essential organs in the human body, performing a myriad of tasks that maintain homeostasis and overall health.
Anatomical Position and Dimensions
The kidneys are retroperitoneal organs positioned against the posterior abdominal wall on either side of the spine, roughly between the twelfth thoracic and third lumbar vertebrae. Each kidney is typically 10-12 cm long, 5-6 cm wide, and 3-4 cm thick, weighing about 150 grams.
Renal Cortex
The outermost region of the kidney is the...
Anatomical Position and Dimensions
The kidneys are retroperitoneal organs positioned against the posterior abdominal wall on either side of the spine, roughly between the twelfth thoracic and third lumbar vertebrae. Each kidney is typically 10-12 cm long, 5-6 cm wide, and 3-4 cm thick, weighing about 150 grams.
Renal Cortex
The outermost region of the kidney is the...
1.1K
01:45Kidney Structure
67.2K
The kidneys are two large bean-shaped organs located in the upper abdomen. They filter the blood several times a day to remove toxins and rebalance water and electrolytes of the circulatory system via the renal veins. The kidneys receive blood directly from the heart via the renal arteries. These arteries enter the kidney at the hilum, the concave surface of the bean, where they branch and divide into smaller vessels and capillaries.
67.2K