FTM L29 Connective Tissue Handout PDF

BASIC PRINCIPLES OF MEDICINE 1: FOUNDATIONS TO MEDICINE Lecture No: 29 Connective Tissue Dr Davina C M Simoes MSc PhD FHEA Assistant Professor in Cellular Pathology, Dept. Applied Sciences, Faculty of Health and Life Sciences [email protected] Session ID: davina1 Copyright All year 1 c...

BASIC PRINCIPLES OF MEDICINE 1: FOUNDATIONS TO MEDICINE Lecture No: 29 Connective Tissue Dr Davina C M Simoes MSc PhD FHEA Assistant Professor in Cellular Pathology, Dept. Applied Sciences, Faculty of Health and Life Sciences [email protected] Session ID: davina1 Copyright All year 1 courses materials, whether in print or online, are protected by copyright. The work, or parts of it, may not be copied, distributed or published in any form, printed, electronic or otherwise. As an exception, students enrolled in year 1 of St. George’s University School of Medicine and their faculty are permitted to make electronic or print copies of all downloadable files for personal and classroom use only, provided that no alterations to the documents are made and that the copyright statement is maintained in all copies. View only files, such as lecture recordings, are explicitly excluded from download and creating copies of these recordings by students and other users are strictly illegal. The author of this document has made the best effort to observe current copyright law and the copyright policy of St George's University. Users of this document identifying potential violations of these regulations are asked to bring their concern to the attention of the author. Session ID: davina1 Assigned Reading 1. Histology – A Text and Atlas Pawlina 8th Edition Chapter 6: Connective Tissue Pg. 170-209 https://meded-lwwhealthlibrarycom.periodicals.sgu.edu/content.aspx?sectionid=20 9563493&bookid=2583 2. DLA - Connective tissue Session ID: davina1 Your Objectives will show here! Objectives SOM.MK.I.BPM1.1.FTM.3.HCB.0111 SOM.MK.I.BPM1.1.FTM.3.HCB.0112 List the function(s) of the different types of connective tissue Identify the major components of connective tissue SOM.MK.I.BPM1.1.FTM.3.HCB.0113 Identify and describe the microanatomical features of the resident cell types in connective tissue including fibroblast, myofibroblast, macrophage, adipocyte, stem cell and mast cell. SOM.MK.I.BPM1.1.FTM.3.HCB.0114 BPM1 | FTM | Lecture 29 | Connective Tissue SOM.MK.I.BPM1.1.FTM.3.HCB.0115 Describe the role of connective tissue cells in anaphylactic shock. Describe the two components that make up the extracellular matrix of connective tissue. SOM.MK.I.BPM1.1.FTM.3.HCB.0116 Define ground substance and list & describe the structure of the three main components (proteoglycans, glycosaminoglycans, multiadhesive glycoproteins) with at least 2 examples of each main component. SOM.MK.I.BPM1.1.FTM.3.HCB.0117 Identify & describe the cell type responsible for the production of ground substance. SOM.MK.I.BPM1.1.FTM.3.HCB.0118 List the cell types responsible for the production of collagen, elastic and reticular fibers. SOM.MK.I.BPM1.1.FTM.3.HCB.0119 Identify & describe the basic structure, function and histological staining of collagen, elastic & reticular fibers. SOM.MK.I.BPM1.1.FTM.3.HCB.0120 List common locations of types I, II and III collagen and elastic fibers. SOM.MK.I.BPM1.1.FTM.3.HCB.0121 Define “argyrophillic” and state the fiber of connective tissue that exhibit this property. SOM.MK.I.BPM1.1.FTM.3.HCB.0122 List the common types of fibrillar and basement membrane collagens. SOM.MK.I.BPM1.1.FTM.3.HCB.0123 Describe the alterations in collagen synthesis that results in abnormal scar formation. SOM.MK.I.BPM1.1.FTM.3.HCB.0124 List the three major classifications of connective tissue with their subclassification. SOM.MK.I.BPM1.1.FTM.3.HCB.0125 Describe the two subtypes of embryonic connective tissue. SOM.MK.I.BPM1.1.FTM.3.HCB.0126 SOM.MK.I.BPM1.1.FTM.3.HCB.0127 SOM.MK.I.BPM1.1.FTM.3.HCB.0128 SOM.MK.I.BPM1.1.FTM.3.HCB.0129 Describe the two general subtypes of connective tissue proper. Describe the structure and location of lamina propria. List the types of specialized connective tissue. List common location sites for each type of connective tissue. SOM.MK.I.BPM1.1.FTM.3.HCB.0130 Compare and contrast white and brown adipose tissue in relation to its appearance, location and function. SOM.MK.I.BPM1.1.FTM.3.HCB.0131 List at least two commonly used stains to identify connective tissue under light microscopy Describe the factors that lead to formation of interstitial fluid, the formation of lymph, and development of edema. SOM.MK.I.BPM1.1.FTM.3.HCB.0132 Session ID: connect23 4 Primary Tissue Types Muscle tissue Epithelium Connective Session ID: davina1 tissue Nerve tissue Lecture Outline - Connective Tissue 1. 2. 3. 4. 5. Definition Function General components Classification Clinical Correlation I. Embryonic connective tissue 1. Mesenchyme 2. Mucous connective tissue II. Connective tissue proper 1. Loose connective tissue 2. Dense connective tissue a) Irregular b) Regular III. Specialized connective tissue 1. Cartilage 2. Bone 3. Blood & Hematopoietic tissue 4. Reticular tissue 5. Elastic tissue 6. Adipose tissue (White and Brown) Session ID: davina1 SOM.1ai.BPM1.1.FTM.3.HCB.BT.1001 A. What is connective tissue? “An aggregation of loosely packed cells with wide intercellular spaces filled with extracellular matrix providing support” Gr. stroma = mattress Parenchyma are functional cellular units (epithelium, nerve, muscle) Stroma (connective tissue) Supporting structures A Why is connective tissue important? Function: Support, defense (immune function) protection (physical), repair, nutrition (storage & transport), insulation Session ID: davina1 Thyroid gland 500x H&E 2. Function: Foreign Support substance Defense (physical and immune) Nutrition (storage & transport) Repair pathogen Basement membrane Ground substance functions: 1. Glue 2. Lubricant 3. Barrier (defense) 4. Tensile strength CELLs: Permanent Transient Fibers: Collagen laminin elastic Connective tissue is directly supplied by blood, lymphatic vessels and nerves Session ID: davina1 SOM.1ai.BPM1.1.FTM.3.HCB.BT.1001 SOM.1ai.BPM1.1.FTM.3.HCB.BT.1116 3. Components A) Cells Permanent Transient B) Extracellular matrix (ECM) i. Fibers ii. Ground substance (GAGs, proteoglycans and glycoproteins) iii. Tissue fluid Connective tissue is directly supplied by blood, lymphatic vessels and nerves *Exception: Cartilage (avascular connect tissue) Classification is based on its components: types amounts proportions Session ID: davina1 SOM.MK.I.BPM1.1.FTM.3.HCB.0113 A. Cells Resident/permanent BPM1 | FTM | Lecture 29 | Connective Tissue Fibroblasts Myofibroblasts Macrophages Adipocytes Stem cells Mast cells Wandering/transient – discussed in greater details in the CPR Module – This is your Required Reading Assignment Session ID: Lymphocyte Plasma cell Neutrophil Monocyte Basophil Eosinophil SOM.MK.I.BPM1.1.FTM.3.HCB.0113//0117 Cells of Connective Tissue: Fibroblasts Most common cell in connective tissue Synthesize all the extracellular fibers Synthesize and maintain the ground substance Fibroblast BPM1 | FTM | Lecture 29 | Connective Tissue Spindle-shaped, may be active (fibroblasts) or inactive (fibrocytes) Has thin, pale staining cytoplasmic processes Not visible in routine preparation as they get embedded between the fibers Myofibroblasts - wound healing. Presence of contractile filaments Session ID: connect23 processes SOM.MK.I.BPM1.1.FTM.3.HCB.0113 Macrophages Derived from monocytes Phagocytic; produce cytokines Irregular cell membrane / cytoplasmic extensions (pseudopodia) Antigen presenting cells; multinuclear giant cells BPM1 | FTM | Lecture 29 | Connective Tissue Migrate to connective tissue and differentiate to macrophages Session ID: Connect Tissue - histiocytes Liver – Kupffer cells, Skin – Langehans cells Brain – microglia Bone – osteoclasts SOM.MK.I.BPM1.1.FTM.3.HCB.0113 Mast Cells Originate in the bone marrow At this stage, they lack cytoplasmic granules Migrate to connective tissue or lamina propria (mucosa) Proliferate and accumulate cytoplasmic granules in connective tissue Granules are large and intensely basophilic Requires special stains to visualize BPM1 | FTM | Lecture 29 | Connective Tissue Secretory products contained within the granules include histamine, heparin, proteases, and eosinophil and neutrophil chemotactic factors Mast cells and basophils circulating in the blood are derived from the same progenitor in the bone marrow Has IgE antibodies on their surface (allergic reactions) Exhibits metachromasia Granules stain a different color from the color of the dye initially used to stain Eg toluidine blue (a blue dye) stains mast cells purple-red instead of blue This phenomenon is determined by a change in the electronic structure of the dye molecule after binding to the granular material In addition, mast cell granules are PAS positive because of their glycoprotein nature SOM.1ai.BPM1.1.FTM.3.HCB.BT.1001 SOM.1ai.BPM1.1.FTM.3.HCB.BT.1116 2. Components A) Cells Permanent Transient B) Extracellular matrix (ECM) i. Fibers ii. Ground substance (GAGs, proteoglycans and glycoproteins) iii. Tissue fluid Connective tissue is directly supplied by blood, lymphatic vessels and nerves *Exception: Cartilage (avascular connect tissue) Classification is based on its components: types amounts proportions Session ID: davina1 Session ID: connect23 SOM.MK.I.BPM1.1.FTM.3.HCB.0118//0119//0120 Fibers of Connective Tissue: Collagen, Elastic… Strong and resilient to pulling and stretch Collagen fibrils - EM Most abundant protein in connective tissue Long polymer: Unbranched, thick and wavy (under light microscope) Many different types with type I being the most abundant Appears eosinophilic with hematoxylin & eosin dyes BPM1 | FTM | Lecture 29 | Connective Tissue Function: a) b) c) d) Provides mechanical strength: E.g. tendons, bones – type I Provides resistance to pressure: E.g. cartilage – type II Provides scaffolding for cells: E.g. reticular – type III Component of the basement membrane: Collagens type IV collagen, VI, VII Collagen fibrils – Light microscopy Collagen types Type l collagen cartilage Type ll collagen Type lll collagen Type lV collagen (Reticular) Basement membrane Session ID: davina1 Collagen fibers Collagen fibrils have 68-nm banding pattern Capsule of the testis, EM x9,500 X = collagen fibers bundles Inset x 75,000 Collagen fibrils Atomic force microscopic x 65,000 Collagen fibrils often consist of more than one type of collagen. Session ID: davina1 SOM.MK.I.BPM1.1.FTM.3.HCB.0115//0116 Ground Substance Ground Substance BPM1 | FTM | Lecture 29 | Connective Tissue Clear, viscous substance with a slippery feel and high-water content Found between the cells and fibers – space filler Can be fluid, semi-fluid, gelatin-like or calcified – depending on the tissue Supports, bind, stores water, gives the tissue tensile strength and provides a medium for transport Contains numerous proteins and polysaccharides Glycosaminoglycans (GAGs) Proteoglycans (combination of GAGs with a protein core) Multi-adhesive glycoproteins (MAGs) Session ID: connect23 Compounds of the Ground Substance 1. Glucosaminoglycans (GAGs) Hyaluronic acid (non sulfated polymer) dermatan sufate, chondroitin sulfate, heparan sulfate 2. Proteoglycans (GAGs bound to protein core), basophilic sufated proteoglycans - basophilic “Bottle brush” like structure “Bottle brush” like structure “bristle” Proteoglycans (GAGs attached to core protein) Session ID: davina1 3. Glycoproteins (“Glue”) – multiadhesive (MAGs) adhesion molecules: fibronectin, laminin, chondronectin, osteopontin integrins - cell surface receptors that bind cells to matrix Function: 1. Glue –binds cell to fibers 2. Lubricant – allows ease of motion nutrients, metabolites and cells 3. Barrier – physical prevention of microorganisms 4. Tissue tensile strength MAGs Session ID: davina1 SOM.MK.I.BPM1.1.FTM.3.HCB.0116 Components of the Ground Substance Glycosaminoglycans (GAGs) Most abundant polysaccharide in ground substance Long chain, unbranched polysaccharides Posttranslational modification of proteoglycans Highly negatively charged Possess carboxyl and sulfate groups on sugar molecules Allows it to attract water, forming a hydrated gel Stains well with basic dyes Permits rapid diffusion of water-soluble molecules Egs. BPM1 | FTM | Lecture 29 | Connective Tissue Hyaluronan Extremely long and rigid Can hold large volumes of water Only GAG that is not sulfated, and not modified post translation Abundant in cartilage Acts as a shock absorber Chondroitin-4-Sulphate Chondroitin-6-Sulphate Heparan sulphate Keratan sulphate Session ID: connect23 SOM.MK.I.BPM1.1.FTM.3.HCB.0116 Components of the Ground Substance Proteoglycans (PGs) GAGs covalently attached to a core protein Extend perpendicularly from the core protein in a brush-like manner Core protein has sites for multiple GAG attachments BPM1 | FTM | Lecture 29 | Connective Tissue Can have a single GAG attached (eg versican) or multiple different GAGS (syndecan or aggrecan) Aggrecan contains both chondroitin sulfate and keratan sulfate GAGs attachment can vary from 1 (as in the case of decorin) to hundreds (as in the case of aggrecan) Functions to link cells to ECM Session ID: connect23 Bottle-brush like appearance SOM.MK.I.BPM1.1.FTM.3.HCB.0116 Components of the Ground Substance Multi-adhesive Glycoproteins (MAGs) Have binding sites for ECM proteins such as collagen Have binding sites for other ECM components such as GAGs and proteoglycans Functions Stabilize the ECM and link it to the surface of connective tissue cells Regulate cell movement and migration Regulate cell differentiation and proliferation BPM1 | FTM | Lecture 29 | Connective Tissue Egs. Fibronectin Most abundant glycoprotein Aids in cell to ECM attachment Laminin Present in basal lamina Osteoponin Present in bone tissue Helps attach osteoclasts to the bone surface Tenascin Important in wound healing Most important during embryogenesis Session ID: connect23 Basement membrane Basal BM BL Layers: Laminin (glycoprotein) and collagen IV Session ID: davina1 SOM.1ai.BPM1.1.F lamina SOM.1ai.BPM1.1.F SOM.1ai.BPM1.1.FTM.3.HCB.0119 Renal corpuscle and convoluted tubules of kidney G Which type of collagen fibers are found here? Periodic Acid Schiff (PAS) staining of glycoproteins & proteoglycans D. Classification of connective tissue I. Embryonic connective tissue 1. Mesenchyme 2. Mucous connective tissue II. Connective tissue proper 1. Loose connective tissue 2. Dense connective tissue a) Irregular b) Regular III. Specialized connective tissue 1. Cartilage 2. Bone 3. Blood & Hematopoietic tissue 4. Reticular tissue 5. Elastic tissue 6. Adipose tissue (White and Brown) Session ID: davina1 Classification is based on the composition and organization of its ECM components and on its functions. Embryogenesis of basic tissues: Ectoderm Almost all connective tissues Mesoderm Endoderm Session ID: davina1 SOM.1ai.BPM1.1.FTM.3.HCB.BT1112 I. Embryonic Connective Tissue Components:  Few cells A. Cells B. Extracellular matrix (ECM) i. a. Fibers  Few fibers ii. b. Ground substance iii. c. Tissue fluid Abundant ground substance Types: 1. Mesenchymal 2. Mucus Session ID: davina1 ☺ Embryonic Connective Tissue cont. 1. Mesenchymal connective tissue. Location: embryo Components - Mesenchymal cells Stem cells Tapered spindle appearance - Sparsely arranged reticular fibers - Abundant ground substance Mesenchyme (H&E X480) SOM.1ai.BPM1.1.FTM.3.HCB.BT1112 Session ID: davina1 Embryonic Connective Tissue cont. SOM.1ai.BPM1.1.FTM.3.HCB.BT1112 2. Mucous connective tissue Location: Umbilical cord Components - Mesenchymal cells - Ground substance: Wharton’s jelly, gelatin like GS rich in hyaluronan Other examples – cardiac jelly, vitreous humor in eye Wharton’s jelly (GS) BV Cells nuclei Cytoplasmic process give a spindle like appearance to the cell Sparse reticular fibres Abundant ground substance Session ID: davina1 Mucous (H&E X480) 😀 Have you heard about Blood Bank? 👍 Clinical importance Session ID: davina1 II. Connective Proper 1. Loose or Areolar connective tissue Location: - Beneath the epithelia lining - Lamina propria Components  Abundant cells of various types  Few fibers  Abundant ground substance More cells than fibers ☺ fibroblasts Features: Flexible, not resistant to stress Rich in blood supply: - Enables exchange of O₂, CO2 and nutrients - Enables diffusion of hormones and growth factors. Fill spaces between other tissues, loosely thin and relatively sparse collagen fibers It surrounds glands and the smallest blood vessels Session ID: davina1 SOM.1ai.BPM1.1.FTM.3.HCB.BT1113 SOM.1ai.BPM1.1.FTM.3.HCB.BT.1118 Loose Connective Tissue Ellastic fiber nuclei BV collagen GS mast cells μΦ Mesentery Verhoeff ’s hematoxylin Session ID: davina1 Loose Connective Tissue (F-Fibroblast) intestinal glands of the colon Location:  Surrounds and suspends vessels and nerves processes  underlies and supports most epithelia  fills spaces between other tissues Session ID: davina1 SOM.1ai.BPM1.1.FTM.3.HCB.BT.1113 Session ID: davina1 Lamina propria “It is a thin layer of loose areolar connective tissue part of the mucosa” Found lining - respiratory tract - GI tract - urogenital tract Ep = Pseudostratified ciliated columnar epithelium G = Goblet cells H&E x400 submucosa SOM.1ai.BPM1.1.FTM.3.HCB.0156 Session ID: davina1 LAMINA PROPRIA Lamina propria Ep epithelial cells C collagen fibers LCT loose connective tissue = lamina propria MM muscularis mucosa DICT dense irregular connective tissue Colon, Mallory trichrome X 250 Session ID: davina1 II.2.a. Dense Irregular Connective Tissue Location: Organ capsule, periosteum, dermis Components:  Fewer cells, sparse and typically of a single type (fibroblasts) Abundant fibers, mostly collagen fibers  Little ground substance  More fibers than cells ☺ Features:  No orientation of collagen fibers - resistance to stress in all directions  Confers significant strength to organs and structures (organs capsules, periosteum, dermis) Session ID: davina1 SOM.1ai.BPM1.1.FTM.3.HCB.BT.1118 SOM.1ai.BPM1.1.FTM.3.HCB.BT.1113 H&E fibroblasts Cytoplasmic extensions Pararosaniline-toluidine blue stain Deep layer Or DICT Session ID: davina1 Connective Tissue Proper - Loose x Dense Glandular epithelium Mammary gland,H&E 175; insets 350 Session ID: davina1 Mammary gland, Masson’s trichrome Session ID: davina1 II.2.b. Dense Regular Connective Tissue Location: Tendons/aponeuroses and ligaments Components:  Fewer cells of single type, mostly fibroblasts, aligned between fiber bundles  Abundant fibers, mostly collagen (type I). Little ground substance  More fibers than cells ☺ Feature:  Imparts tensile strength  Regular (specific) orientation of collagen fibers Session ID: davina1 SOM.1ai.BPM1.1.FTM.3.HCB.BT.1113 SOM.1ai.BPM1.1.FTM.3.HCB.BT.1118 SOM.1ai.BPM1.1.FTM.3.HCB.BT.1113 SOM.1ai.BPM1.1.FTM.3.HCB.BT.1118 Fibroblast/tendon cell/ tendinocyte Tendinocyte thin process A C processes C rER cytoplasm C = collagen fibers Fibroblast EM x 25,000 B Euchromatin x Heterochromatin Tendon, TEM Fibroblasts responsible for maintaining the fibers and ground substance Session ID: davina1 De-tour 👍👎 Have you heard Greek vs Trojans war? Session ID: davina1 tendon Covering an entire tendon: - Epitendineum (Ept) Covering around a group of fascicles: - Peritendineum Connective tissue covering around a group of fibers: - Endotendineum (Ent) SOM.1ai.BPM1.1.FTM.3.HCB.0157 TF = tendon fascicle Session ID: davina1 Dense irregular connective tissues vs SOM.1ai.BPM1.1.FTM.3.HCB.BT.1118 Session ID: davina1 Dense regular connective tissues III.4. Reticular Connective Tissue or Argyrophilic Function: Forms a supporting framework for cellular constituents of various tissues and organs: haematopoietic and lymphatic organs Location: bone marrow, lymph nodes, liver, spleen, endocrine tissue, nerve tissue Components: Reticular cells (modified fibroblasts), cytoplasmic extensions cover reticular fibers Reticular fibers (collagen III fibers) Note: Reticular fibers are also secreted by Schwann cells and smooth muscle (Gr silver + friend) 😀 What stains are used to identify reticular fibers in light microscopy? liver SOM.1ai.BPM1.1.FTM.3.HCB.BT.1114 SOM.1ai.BPM1.1.FTM.3.HCB.BT.1118 Session ID: davina1 III.5. Elastic Connective Tissue Location: Elastic arteries, elastic cartilage, vocal ligament, suspensory ligament of the penis Composed of cross-linked elastin molecules forming the central core of elastic fiber and surrounding network of fibrillin microfibrils and associated proteins (EMILIN-1, MAGP-1) Elastin fiber is connected by amino acids (aa) desmosine and isodesmosine. Produced by fibroblasts and smooth muscle cells Special staining required: Orcein, Resorcin or Verhoeff’s Recoil function. Extremely elastic - 5x more than rubber (150% their resting length) Session ID: davina1 SOM.1ai.BPM1.1.FTM.3.HCB.BT.1114 SOM.1ai.BPM1.1.FTM.3.HCB.BT.1118 SOM.1ai.BPM1.1.FTM.3.HCB.BT.1114 SOM.1ai.BPM1.1.FTM.3.HCB.BT.1115 SOM.1ai.BPM1.1.FTM.3.HCB.BT.1118 Elastic artery Which unique amino acids are found in elastic fibers that give them their elasticity? Elastic stain highlights the large number of elastic fibers in aorta. Elastin makes tissues elastic, so that the tissue will recoil after stretch. Elastic fibres are 5 times more extensible than an elastic band! Mixing up collagen (less extensible) and elastin, prevents overstretch. Session ID: davina1 Comparison slide Elastic fiber Reticular fiber compare Note the short thin and branching nature of the fibers. Specially stained for reticular fibers. Session ID: davina1 Review The following stains are commonly used to identify connective tissues for observation with light microscopy Hematoxylin and Eosin: most used stain in Histology and Cell Biology Trichrome: differentiates between nucleus, cytoplasm and connective tissue fibers. Silver stain: used to stain reticular fibers, stains them black Elastic stains: e.g. Orcein, Verhoeff’s→ Stain elastic fibers. SOM.1ai.BPM1.1.FTM.3.HCB.0154 III.6. White adipose Connective Tissue Cells Nucleus Lipid droplet Function -large -peripheral -one -fat storage Session ID: davina1 SOM.1ai.BPM1.1.FTM.3.HCB.BT.1114 SOM.1ai.BPM1.1.FTM.3.HCB.BT.1119 Brown Adipose Tissue brown adipocyte Brown adipose tissue (Central nucleus) Cells -smaller Nucleus -central Lipid droplet -many Function -energy release Rich in mitochondria Session ID: davina1 SOM.1ai.BPM1.1.FTM.3.HCB.BT.1114 SOM.1ai.BPM1.1.FTM.3.HCB.BT.1119 white adipocyte Specialized Connective Tissue 1. 2. 3. Cartilage cells – chondroblasts/chondrocytes Bone cells – osteoblasts/osteoclasts/osteocytes Blood cells – erythrocytes and leukocytes 1 SOM.1ai.BPM1.1.FTM.3.HCB.BT.1114 2 Session ID: davina1 3 Clinical Correlations SOM.1aiii.BPM1.1.FTM.3.HCB.BT.1124 SOM.1aiii.BPM1.1.FTM.3.HCB.BT.1125 Anaphylactic shock - increased mast cell release of histamine. Edema - increased tissue fluid NORMAL (equilibrium) Hydrostatic pressure Oncotic pressure Plasma proteins Edema (balance) Oncotic pressure Hydrostatic pressure Favored by: a) Increased intravascular(capillary) hydrostatic pressure b) Decreased intravascular oncotic pressure c) Increased vascular permeability Session ID: davina1 Protein deficiency Ehlers-Danlos syndrome (EDS) Group of CT inherited disorders attributed to mutations of collagen and other ECMs. It affects the joints (hypermobility, dislocations), skin (pail, thin), blood vessels (easy rupture). Characterised by either poor strength of collagen or absence of sufficient amounts of structurally normal collagen. early morbidity and mortality due to rupture of vessels and internal organs SIX types: classical, hypermobility, vascular, kyphoscoliotic , arthrochalasis (COL 1), dermatosparaxis SOM.1aiii.BPM1.1.FTM.1.HCB.0159 Session ID: davina1 Type EDS form Clinical feature Collagen abnormality I Gravis Classic Collagen type V and Col type I (rare) II Mitis Mild Collagen type V and Col type I (rare) III Hypermobile hEDS -Marked joint laxity Collagen type III IV Vascular Severe life-threatening vascular haemorrhage Collagen type III V X-linked Similar to Type II EDS but xlinked VI Ocular/scoliosis Significant eye problems VII Arthrocalasis Joint herperlaxity & congenital dislocations VIII Peridontal Dental problems & classic EDS Session ID: davina1 Collage type I Marfan’s syndrome Decreased elastic fibers (fibrillin gene) Autosomal dominant disorder involving the deficiency of the protein fibrillin 1 and increase in TGF-β This results in tall stature, long limbs, fingers and toes. Features of the disorder are most often found in the heart, blood vessels, bones, joints, and eye. Cardiovascular manifestations can include aortic aneurysm and mitral valve prolapse SOM.1ai.BPM1.1.FTM.1.HCB.BT1121 Session ID: davina1 Hypertrophic scar, keloids - increased collagen Hypertrophic scar Raised scar tissue, but within original wound boundary Keloid scar When in excess of the boundary, extending into surrounding tissue Caused by Increased collagen production NEW – ‘Injury should reach the reticular dermis (DICT)’ Ogawa, 2017 SOM.1ai.BPM1.1.FTM.3.HCB.0161 Session ID: davina1 Keloid x Hypertrophic scars Session ID: davina1 Scurvy - decreased collagen (vitamin C) Ascorbate (Vit C) is essential for the hydroxylation of the amino acid proline which forms hydroxyproline - a major component of collagen. Collagen is an essential structural protein in the body accounting for around a quarter of all the protein in our body and is critical to the structural integrity of our body. Patients usually develop gum disease and poor wound healing. Session ID: davina1