Focal segmental glomerulosclerosis
Focal segmental glomerulosclerosis, or sometimes focal glomerular sclerosis, or just sometimes FSGS, is a type of kidney disease that affects the kidney’s glomeruli, which is where small molecules are first filtered out of blood and into the urine. From the name, you haveglomerulosclerosis, which indicates sclerosis, or scar tissue, forming in the glomeruli. Segmentalmeans that only a segment, or part of the glomeruli is affected, and focal means that among all those glomeruli in the kidney, only some are affected.
局灶节段性肾小球硬化 /局灶肾小球硬化， 或称FSGS，是一种累及肾小球的肾病。肾小球是小分子物质最从血液中先滤过入尿的地方。从名字上看，肾小球硬化指示肾小球中有硬化或瘢痕。节段性意思是只有肾小球的一部分受累，局灶性是指在肾组织所有肾小球中只有部分肾小球受累。
Those glomeruli that are affected, though, allow proteins to filter through into the urine, and ultimately people with FSGS develop nephrotic syndrome.
当这些肾小球受累，蛋白质可滤过入尿，最终，FSGS患者出现肾病综合征。
But what exactly is nephrotic syndrome? Well usually the glomerulus only lets small molecules, like sodium and water, move from the blood into the kidney nephron, where it eventually makes its way into the urine. But with nephrotic syndromes, the glomeruli are damaged and they become more permeable, so they start letting plasma proteins come across from the blood to the nephron and then into the urine, which causes proteinuria, typically greater than 3.5 grams per day.
究竟什么是肾病综合征嘞？嗯...正常的肾小球只允许小分子物质，如钠离子和水， 从血中进入肾单位，最终进入尿液。但肾病综合征时，肾小球受损，通透性增加， 从而允许血清蛋白质越过屏障从血中进入肾单位， 然尿，导致蛋白尿，通常高于3.5克/天
An important protein in the blood is albumin, and so when it starts leaving the blood, people gethypoalbuminemia—low albumin in the blood. With less protein in the blood, the oncotic pressure falls, which lowers the overall osmotic pressure, which drives water out of the blood vessels and into the tissues, called edema.
白蛋白是血液中一种重要的蛋白质，当白蛋白离开血液，可能导致罹患低蛋白血症——即血清白蛋白水平低。随着血清蛋白质减少，胶体渗透压也减少， 即总渗透压下降， 这导致（血液中）水分从血管流出进入组织，称为水肿。
Finally, it’s thought that as a result of either losing albumin or losing some protein or proteins that inhibit the synthesis of lipids, or fat, you get increased levels of lipids in the blood, calledhyperlipidemia.
最后，人们认为白蛋白或其他蛋白质的丢失，以及抑制脂类合成的蛋白丢失，会导致血液脂肪含量增高——即高脂血症。
Just like the proteins, these lipids can also get into the urine, causing lipiduria. And those are the hallmarks of nephrotic syndrome—
和蛋白质同理，这些脂肪也可以进入尿液，导致脂尿症。这些属于肾病综合征的5个特点：
Proteinuria, hypoalbuminemia, edema, hyperlipidemia, and lipiduria. So focal segmental glomerulosclerosis is a type of nephrotic syndrome, that’s helpful.
蛋白尿、低蛋白血症、水肿、高脂血症、脂尿症。所以局灶节段性肾小球肾炎是肾病综合征的一种。
原发性FSGS
But why does the glomerulus develop segmental sclerosis in the first place? Well, primary FSGSis when it’s idiopathic, or there’s no clear underlying cause. What is known, though, is that thepodocytes, which are the cells that have these long tentacle-like projections, called foot processes, that wrap around the capillaries in the glomeruli, are damaged. These damaged podocytes allow some plasma proteins and lipids to sneak by, which then go on to get into the urine.
但肾小球是如何原发性地产生节段性硬化的呢？原发性FSGS特发（于肾），或者无潜在原因。但我们已经知道，有着长长的、触手样足突的、包绕肾小球毛细血管簇的足细胞受损。这些受损的足细胞允许一些血浆蛋白和脂肪通过，进入尿液。
Not only that though, over time, some of these proteins and lipids get trapped and build up in the glomerulus, resulting in hyalinosis, where the tissue has a hyaline or glassy appearance on histology, and it’s thought that over time these areas move on to develop sclerosis, or scar tissue.
不仅如此，随着时间推移，这些（漏出的）蛋白质和脂类一部分被困住并在肾小球中积累，导致玻璃样变性，即积累物在组织学染色上像透明物或玻璃，人们认为随着时间推移这些积累区域继续发展成硬化或瘢痕。
Some research suggests that these changes in FSGS are a continuation from another nephrotic syndrome called minimal change disease which is also characterized by podocyte injury, other research suggests that minimal change disease and FSGS have different mechanisms of podocyte injury.
一些研究表明FSGS的病变是由另一种肾病综合征——微小病变性肾小球肾炎发展而来的。微小病变性肾小球肾炎也是由足细胞损伤介导的，另一些研究表明微小病变性肾小球肾炎和FSGS足细胞损伤有不同的机制。
Fundamentally, FSGS is a histopathologic description, basically what it looks like under a microscope, which could be the end result of a number of different pathophysiologic mechanisms.
从根本上讲，FSGS是一个从组织学上的描述性病变，即反映其镜下特点，它可以是众多不同的（肾小球）病变的最终结果。
继发性FSGS
As opposed to primary FSGS, Secondary FSGS is when there’s a clear underlying cause, and there are several important conditions that can be associated. Sickle cell disease, where blood cells are sickle-shaped due to a structurally abnormal hemoglobin protein that can cause tissue ischemia, has been linked to FSGS. HIV, a retrovirus that infects various human cell types, can lead to FSGS and is called HIV-associated nephropathy. Heroin abuse has also been linked to FSGS, which is called heroin nephropathy. Also, other conditions like kidney hyperperfusion or increased perfusion of blood, as well as increased pressure in the glomerular capillaries have been linked to FSGS as well.
与原发性FSGS不同，继发性FSGS有其明确的病因，有几个病理过程可能与之有关。镰状细胞贫血症，血细胞因血红蛋白结构异常而整体呈现镰刀形可导致组织缺血，与FSGS有关。HIV，感染人类不同细胞的逆转录病毒可能导致FSGS，称为HIV相关肾病。海洛因滥用也与FSGS有关，称为海洛因肾病。除此之外，如肾高灌注或肾血流量增加，以及肾毛细血管球内压力增高也与FSGS有关。
Alright so on histology, there’ll be segmental sclerosis and hyalinosis of the glomeruli, and under electron microscope, there’s effacement of the foot processes of the podocytes. And onimmunofluorescence, there might be nonspecific focal deposits of IgM and complement proteins, and these get trapped in the areas of hyalinosis, but these isn’t always seen.
患者组织活检中，会出现肾小球节段性硬化和玻璃样变，在电镜下可见足细胞足突消失。在免疫荧光电镜下，可能有非特异性病灶，源于IgM和补体蛋白沉积。这些沉积局限于玻璃样变区域，但不总是出现。
诊治
FSGS is the most common cause of nephrotic syndrome in African-Americans and Hispanics, and can affect both children and adults. FSGS is usually treated with steroids, although it has an inconsistent response, and for that reason, especially in adults— FSGS can involve more and more segments of the kidney’s glomeruli to the point where it causes chronic renal failure.
FSGS是非裔美国人和西班牙裔美国人肾病综合征最主要的病因。成人儿童都可患病。FSGS通常用类固醇治疗，尽管会产生抗药性。因此，尤其在成人中—— FSGS可累及越来越多的肾小球， 直至慢性肾衰。
小结
Alright, so as a quick recap, focal segmental glomerulosclerosis is a nephrotic syndrome characterized by sclerosis and hyalinosis in some, but not all, glomeruli, and usually only affects part of the glomeruli that it affects.
医理闪充时间！局灶节段性肾小球肾炎是一种肾病综合征，以部分肾小球的硬化和玻璃样变为特征，通常只影响肾小球的一小部分。
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