L20- Glomerular diseases Year 4 Paediatrics Notes OmpathStudy

L20- Glomerular diseases Year 4 · Paediatrics Collection: 4TH YEAR ALL NOTES / PAEDS / OTHER NOTES -- Lecture 20 \nEditing file\n\n Glomerular diseases \nObjectives:\n★ Understand the physiology / pathophysiology of Glomerular\nstructure.\n★ Recognize Normal & abnormal urine analysis in making diagnosis\nof Glomerular Disease vs Non-Glomerular disease\n★ Recognize the differences between Nephritic & Nephrotic\nGlomerular diseases.\n★ To recognize the early features of Glomerular diseases before it is\ntoo late! Early Dx & Rx makes a huge difference\n★ To learn the common causes of Nephrotic & Nephritic renal\ndiseases. \nColor index: Color index:\nOriginal text Females slides Males slides\nDoctor’s notes Text book Important Golden notes Extra \n\n Glomerular Diseases1 2\n\n ◄ Normal structure is needed to:\n●01 Keeps the glomerular filtration normal, thus maintains normal kidney function. \n●02 Maintain urine volume and hence, preventing fluid retention in the body which causes edema and high\nblood pressure. \n●03 Prevents the blood components (cells, proteins) from leaving the bloodstream and appearing in the\nurine.\n★ renal cortex is the most important functional part of the kidney because it has the glomeruli\n\n if the Glomerular structure is intact the urine will show:\n● No protein ● No heme\n● No RBCs ● No cellular casts\n(Accept: <2 RBCs/high power field) ● Devoid of fats\n\n ◄ How glomerular diseases start?\n● The insult to the glomeruli is either an autoimmune attack or is the result of deposition of antibody-antigen complex in the\nkidney which will attack the glomeruli which will lead to a local inflammation there. The pathology depends on the\ncomponent of the glomeruli that is affected (basement membrane, mesangium…)\n● Here we are talking about primary glomerular diseases that are mostly caused by immune system\ndysfunction.\n● Auto-antibodies targeting glomerular structure or immune- complexes (antigen-antibody) depositing\nand traumatizing the glomerular components.\n● Glomerular diseases are named based on their histopathological characteristics seen under the\nmicroscope. So, almost always a kidney biopsy is needed to diagnose any suspected primary\nglomerular disease. Urinalysis & blood tests are also used. \nThe manifestations of a glomerular disease are usually indicative of which\ncomponents of glomerulus structure was affected mainly by the disease process:\n\n If endothelial cells OR Mesangial cells OR GBM\nOR all of them together were targeted; then\nIf Podocytes were the main target of the disease\nGlom Capillary wall will be damaged by\nprocess this leads mainly to proteinuria ( at\ninflammation so blood components will leak to\nlarge amount) due to foot process effacement;\nthe urine space causing: hematuria, proteinuria\nthus Nephrotic Syndrome will be the main\nand abnormal renal function; thus Nephritic\nfinding.\npattern of renal disease will be present\n( Clinically called: Glomerulonephritis or GN )\n\n1. Glomerular disorders are characterized by impairment in selective filtration of blood, resulting in excretion of larger substances\nsuch as plasma proteins and blood cells. As disease advances, GFR decreases proportionally, leading to renal failure and the\npossible need for dialysis and/or transplantation. The classic features are proteinuria, hematuria, or both.\n- Glomerular diseases are generally chronic and all of them can cause nephrotic syndrome.\n- Biopsy is the most accurate test to establish a diagnosis (though not always needed)\n- Often treated with steroids (several resolve spontaneously)\n- Additional immunosuppressive medications (cyclophosphamide, mycophenolate) are frequently used. \n\n Glomerular Diseases 3\n\n Disease of The Kidney \nTubular Vascular Glomerular1 Obstructive Cysts and tumors\nBut to make things easier, we can put\nGlomerular diseases in two main clinical\ncategories\n\n Nephrotic syndrome Nephritic syndrome\n\n Primary causes IgA nephropathy \nPoststreptococcal glomerulonephritis\nMinimal change disease\n(PSGN) \nMembranous nephropathy Lupus nephritis\n\n Focal segmental glomerulosclerosis\nRapidly progressive glomerulonephritis\n\n secondary causes Anti-GBM (Goodpasture's disease)\n\n IgA nephropathy ANCA vasculitis ( e.g. Wegner’s\nGranulomatosis) \nmembranoproliferative\nglomerulonephritis (MPGN) Membranoproliferative GN (MPGN)\n\n Diabetic nephropathy\nAlport’s syndrome2\n\n Amyloidosis\n\n1. The degree or amount of proteinuria is the main difference between glomerulonephritis and nephrotic syndrome. 2. Alport’s syndrome (hereditary nephritis): X-linked or autosomal dominant inheritance with variable penetrance. It is a congenital defect of collagen. Features include\nhematuria, pyuria, proteinuria, high-frequency hearing loss without deafness, visual disturbance, progressive renal failure. No effective treatment. \n\n Nephrotic syndrome 4\n\n ◄ Pathological findings:\n● Normal foot processes Diffuse Foot Processes Effacement\n\n ●