不分化的连接组织疾病中的超粘性综合征:诊断和治疗的挑战

Connective tissues are one of the four main tissue types in humans that are extensively present in the body. They are characterized by cells embedded in an extracellular matrix (ECM) composed of a ground substance and three main types of protein fibers— collagen, elastic, and reticular fibers. The ground substance of connective tissues can range from a watery and jelly-like consistency to mineralized and hard. The wide variety of cells in the connective tissues include fibroblasts,...

Connective tissue develops from the mesoderm of a developing embryo and consists of cells, fibers, and ground substance: a gel-like material containing large complexes of carbohydrates and proteins. Connective tissue was first identified as a separate tissue family in the 18th century, and Johannes Peter Muller coined the term connective tissue.
Fat cells (adipocytes), smooth muscle cells (myoblasts), and bone cells (osteoblasts) are some connective tissue cell types. Some immune system cells...

The connective tissues have different properties and functions in the human body. They are broadly categorized into proper, supporting, or fluid connective tissues.
Connective Tissue Proper
Connective tissue proper is the most abundant class of connective tissues. As its name implies, it predominantly connects different tissues in the body. Depending on the cell types, ground substance, viscosity, and fiber types in the ECM, connective tissue proper is further categorized into loose and dense....

Blood and lymph are fluid connective tissues. They contain cells, also known as formed elements, circulating in a liquid extracellular matrix, the plasma. The formed elements are derived from hematopoietic stem cells in the bone marrow. Blood and lymph connect all vital parts and carry nutrients, oxygen, and other essential molecules like antibodies.
Blood
The blood flows through blood vessels— arteries, capillaries, and veins. Blood plasma is primarily made of proteins, solutes, and...

Fascia, a thin layer of fibrous connective tissue, is distributed throughout the body. It demarcates and forms a supportive covering over skeletal muscles, bones, blood vessels, and organs. There are three main types of facia— superficial fascia, deep fascia, and subserous fascia. These are all present at different depths in the body. Fascia reduces the friction and permits muscles, joints, and organs to easily slide against each other, facilitating movement of the body and preventing...

One of the significant functions of connective tissue is connecting tissues and organs. Unlike epithelial tissue that is composed of cells closely packed with little or no extracellular space in between, connective tissue cells are dispersed in a matrix. The matrix usually includes a large amount of extracellular material produced by the connective tissue cells that are embedded within it. It plays a significant role in the functioning of this tissue. The major component of the matrix is a...