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mucopolysaccharidosis (mucopolysaccharidosis, MPS) are a group of lysosomal accumulation disease, is due to defects in lysosomal enzymes, resulting in acid mucopolysaccharide (glucose amino glycans) degradation blocked accumulation of mucopolysaccharides in the body caused by a series of clinical symptoms. Mucopolysaccharidosis will be divided into 7 major types, each type is further divided into 2 to 4 subtypes of which mucopolysaccharidosis type Ⅰ Ⅳ most common and more characteristic, and especially the most typical type Ⅰ, the viscosity polysaccharide storage disease of the prototype. According to clinical history, laboratory tests and X ray, CT MRI, B-, can be diagnosed by means of prenatal care. The lack of complete cure for disease. The most promising treatment of mucopolysaccharidosis disease is more specific enzyme replacement therapy and gene therapy, both to improve clinical performance and survival.
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monocytes - phagocyte system MPS (mucopolysaccharidosis) outlined the causes of the pathogenesis of the main features of pathological diagnosis of clinical diagnosis and treatment prognosis of scientific references of professional services marketing environmental programs Dutch ornamental plant production Economics master production schedule defines MPS MPS (Man Pusi) early pregnancy processing MPS Power Systems Company Profile Products mononuclear myofascial pain syndrome - before the phagocytic cell system including the bone marrow mononuclear phagocytes, peripheral blood mononuclear cells, and tissue macrophages, with the most active in vivo biological activity of the cell types. MPS (mucopolysaccharidosis) Overview Chen Jiqing: polysaccharide deposition and mucopolysaccharide excretion in urine caused by the disease. GAG is a polysaccharide containing nitrogen, constitutes the main component of connective tissue cells, is also widely present in various mammalian cells. Important to have dermatan sulfate glycosaminoglycan (DS), heparin sulfate (HS), keratin sulfate (KS), chondroitin sulfate (CS) and hyaluronic acid (HA), etc. The first three are closely related with this group of diseases .
multiple bone development disorder
mucopolysaccharide degradation of these must be carried out in the lysosomes, there are 10 currently known enzymes involved in the degradation process in which an enzyme defect of any will result in glycosaminoglycan chain accumulation of body decomposition barriers, causing abnormal cell structure and function abnormalities. GAG accumulation in the organs of various systems that lead to pathological changes of these organs and clinical symptoms. According to clinical manifestations and enzyme defects, MPS can be classified into type Ⅰ ~ Ⅶ of 6, in which work is divided into H-type Ⅰ, Ⅰ S-type, Ⅴ type has been renamed Ⅰ H / S type. In addition to type Ⅱ ######-linked recessive, the rest are autosomal recessive disease. Like other lysosomal accumulation disease, like most all types of MPS disease in about 1 year old, the course is progressive, and involved multiple systems, with similar clinical symptoms, but the severity of each type vary, and have their own features, of which the most typical type Ⅰ H, worst prognosis, patients often die before the age of 10; Ⅰ S-type condition lightest. Yang Xijiang: . Based mostly bone lesions, also involving the central nervous system, cardiovascular system and liver, spleen, joints, tendons, skin and so on. Mucopolysaccharide glycosaminoglycan real name is (glucosaminoglycan), is a bone matrix and connective tissue cells are major components, it is by uronic acid and N-acetyl-hexosamine sulfate or disaccharide units composed of large repeat molecule, is a multi-anionic polymer of GAG, which are the main component of dermatan sulfate (dermatin sulfate, DS), heparan sulfate (heparan sulfate, HS), keratin sulfate (keratan sulfate, KS),air force one low nike, sulfuric acid cartilage Su (chondroitin sulfate, CS) and hyaluronic acid (hyaluronic acid, HA) and so on. The degradation of these polysaccharides to be carried out in the lysosomes, 10 species are known to have lysosomal glycosidase, and sulfatase acetyltransferase involved in the degradation process, an enzyme defect of any glycosaminoglycan chain will cause barriers to the accumulation of body decomposition, and since the urine. According to clinical manifestations and enzyme defects, MPS can be classified into type Ⅰ ~ Ⅶ of 6, which has been renamed type Ⅴ Ⅰ H / S type, there are several subtypes of each type, the characteristics of various types of MPS are, for the more common type Ⅰ , is also the most typical clinical manifestations. In addition to type Ⅱ Ⅹ - linked recessive inheritance, the rest are autosomal recessive disease.
β-galactosidase
cause (a) In addition to these diseases and type Ⅱ-linked recessive inheritance, but are autosomal recessive. (B) of the mucopolysaccharide metabolic disease called Hurler syndrome type Ⅰ, an autosomal recessive disease, from α-L-Aidoo caused by defects in enzymes of uronic acid. (C) MPS type Ⅳ metabolic disease: also known as Morguio-Brailsford syndrome, is an autosomal recessive disease, due to N-acetyl amino galactose 6 - sulfatase defect can not participate in the decomposition of disease mucopolysaccharidosis . In addition to the pathogenesis of MPS Ⅱ was X-linked recessive genetic type, the other mode of inheritance of various types are autosomal recessive defective gene is located on autosomes, and only the incidence of homozygous genotypes in the parents are mixed before homozygous for the gene mutation in children with normal probability is 25%, and the remaining 50% are heterozygous carriers of the MPS Ⅱ type of defect gene is located on chromosome X disease therefore only men, women are gene carriers , Prevalence of male and female offspring being a carrier chance for each of 50%
X chromosome
has a wide range of proven gene mutation, and the large difference between different groups. Mucopolysaccharide, including 4 -, chondroitin sulfate, 6 - chondroitin sulfate,nike air force one, chondroitin sulfate, heparin sulfate, keratin sulfate, heparin and hyaluronic acid and other ingredients, for the cornea, cartilage, bone, skin, fascia, heart valves and blood vessels structural components of connective tissue. MPS Ⅰ type of uronic acid α-Aidoo enzyme deficiency, MPS Ⅱ type Aidoo uronic acid sulfatase deficiency and MPS Ⅶ type of β-glucuronidase deficiency, all lead to chondroitin sulfate and heparan sulfate degradation blocked. MPS Ⅲ type of enzyme deficiency can cause a variety of heparan sulfate degradation of obstacles. MPS Ⅳ type of β-galactosidase enzyme deficiency mainly affects the degradation of keratin sulfate. MPS Ⅵ B-type acyl sulfatase lack of major disruption to the degradation of chondroitin sulfate. Not all sticky polysaccharide degradation in the body a lot of savings and deposits in the tissues, causing organ damage and dysfunction. At the same time, excessive mucopolysaccharide been discharged from the urine. Pathological changes in mucopolysaccharide deposition in the fiber cells, staining a balloon-like cells, known as Hurler cells, present in the liver, spleen, lymphoid tissue of the reticular cells, in chondrocytes and osteoblasts, central nervous system and peripheral nerve section , retinal cells and corneal cells also have similar material accumulation. Deposition of the formation of macular thickening endocardium, aorta, pulmonary artery, coronary artery and brain, kidney, liver, spleen, and limbs of the arterial wall are deposited [1]. Most of the clinical performance of normal children at birth, 1 year of age the growth and development are normal. Age of onset of symptoms due to an increase in the type of mucopolysaccharide different varies. Onset symptoms are usually ear infections, runny nose and cold. Although various types of mucopolysaccharidosis with increased progression of disease severity quite different, but the clinical performance of children have some common characteristics such as: special face and short stature abnormal skeletal system. Most children have joint changes and activity limitation. Multiple organ involvement seen in all patients. Some children with corneal opacity, and can therefore lead to impaired vision or even blindness. Hepatosplenomegaly, and cardiovascular involvement is common. Some children may have mental retardation ######ual development, umbilical hernia and inguinal hernia, slow growth, hydrocephalus, skin thickening, hair growing, chronic runny nose, recurrent ear infections, can cause hearing loss and so on. Various types of mucopolysaccharidosis characteristics (Table 1).
Table 1
1. mucopolysaccharidosis type Ⅰ Ⅰ mucopolysaccharidosis Although there are 3 subtypes, but are the same enzyme deficiency, but a different degree of enzyme deficiency. Hurler syndrome, which is more common clinical manifestations of the most serious, Scheie syndrome, the symptoms appear late, the lightest condition, and Hurler-Scheie syndrome is between. Normal at birth was normal. 6 months to 1 year old children began to emerge after the slow growth, expression indifferent, unresponsive, and even mental retardation language naive idiot. Large head, prominent forehead, showed a boat-shaped, eyes away from the broadening, collapse or flat nose, nostril increases, thick and everted lips, mouth, tongue, and often extended in large estuary, small and dull teeth sparse dentition uneven corneal opacity common, severe cases can cause blindness. Otitis media often occurs, and lead to hearing loss or deafness. Involvement of the heart valves and tendons can cause cardiac enlargement and cardiac insufficiency. Bronchial cartilage lesions can be easily induced airway stenosis complicated by infection. Bulging abdomen, hepatosplenomegaly, and more with inguinal hernia or umbilical hernia, may have diarrhea or constipation. Thick hair, thick black. Short neck, shrug, short limbs and trunk, kyphosis, arched hump. Most joint flexion-like tonic activity was limited, often knee-ankle valgus and flatfoot deformities such as palm, that can occur with short carpal tunnel syndrome. Hurler syndrome often die in childhood, Scheie syndrome and Hurler-Scheie syndrome, can survive to adulthood. 2. Mucopolysaccharidosis type Ⅱ rare. According to the severity of the disease is divided into A, B2 subtypes, including A type of serious condition. All the patients were male and more than 2 to 6 years of age onset.
bone deformities
Hurler syndrome with similar clinical presentation, but there late, slow progress in mental retardation and severe short stature as Hurler syndrome. Serious illness from early childhood that is the beginning of retinitis pigmentosa and optic disc edema, but no corneal opacity. Hearing was conducted damage and, ultimately, for the deaf. Minor skeletal abnormalities. Cardiac involvement is more common, mainly as heart valve disease, coronary heart disease and congestive heart failure most of obstructive apnea syndrome, hepatosplenomegaly, diarrhea or constipation. Patients often die before the age of 15 patients with mild type B, and some may have normal hearing and no corneal skeletal deformities. 3. Mucopolysaccharidosis type Ⅲ clinical Although this type can be very rare there are 4 different enzyme deficiency but their clinical manifestations are very similar, mainly for the purpose of mental decline, which mucopolysaccharidosis type Ⅲ A clinical rapid progress. Usually 4 to 5 years old with normal intelligence then gradually appear before the slow, mental retardation, was progressively increased. In severe cases, 2 to 3 years old can have a mental retardation. More than a hair more. Changes in other areas such as special face, short stature and bone deformities such as not serious or even normal. Usually have hearing loss but no corneal opacity. Generally not involving the heart. No hernia may have slightly enlarged liver and spleen. Body Shaoai or nearly normal, a very small number can be expressed as short stature. Can be limited or even on Festival Day tonic on hand and other joints can be a flexion deformity. 4. Mucopolysaccharidosis type Ⅳ showed prominent growth retardation, general adult height not exceeding 160cm. Face, and later walking toddlers with normal intelligence staggering gait instability. Short neck, shrugging. Teething late, irregular dentition, teeth lack luster. Corneal opacity can be as early childhood began to appear. Damage hearing was conducted. Often without cardiac involvement. Slightly enlarged liver and spleen was no hernia, including chicken skeletal deformities, kyphosis, genu valgum, flat feet and joint deformities such as flexion contracture, and significant joint laxity, but no ankylosis. Cervical subluxation can occur, causing spinal cord compression symptoms. Most patients can survive 20 to 30 years old. 5. Mucopolysaccharidosis type Ⅴ is that the type is the mucopolysaccharidosis type Ⅰ Seheie type, and Hrular syndrome showed no difference between severe corneal opacity, and opacity for the peripheral, patients of normal intelligence tall, normal or Shaoai, life is normal, but the hairy ankylosis. Dorsal column, skull X-ray showed only a slight change. 6. Mucopolysaccharidosis type Ⅵ extremely rare. Clinical manifestations and mucopolysaccharidosis type Ⅰ similar, but patients with normal intelligence. Generally from 2 to 3 years of growth retardation began to appear. Skull sewn closed early, there may be hydrocephalus, and intracranial hypertension symptoms and cause spastic paralysis. Corneal opacity appeared earlier, had progressive hearing loss has serious heart valve disease in the blind and the deaf inguinal hernia Dengjun hepatosplenomegaly and more common. Bone deformities are similar to the MPS Ⅰ type, but relatively mild, usually long bones of upper limb than the lower extremities involved. Joints was limited. May have mild joint stiffness. Most of the patients life is not more than 10 years of age. 7. Mucopolysaccharidosis type Ⅶ extremely rare. Special face soon after birth began to appear. Corneal opacity usually of normal intelligence and hearing damage are more common. Many have hepatosplenomegaly, usually not involving the heart, no hernia. Short arm, bone dysplasia can have chicken breast, knee valgus and other skeletal deformities. Yang Xijiang main features: Type: Ⅰ H (Hurler), Ⅰ S (Scheia), Ⅰ H / S (Hurler / Scheie): 2. Enzyme defects (gene locus): α-L-Aidoo uronic acid glycosidase (4p16, 3); 3. Urine sugar polysaccharide row: DS, HS; 4. Clinical manifestations: the growth of backward, intelligent backward, hepatosplenomegaly, bone and joint deformities, cardiovascular disease,air force one low, the first large, ugly face, corneal spot, deafness, multiple bone dysplasia (such as spinal vertebrae as clubbing, beak-like, pulp-shaped ribs, such as ribbon-like, etc.), the most important condition to Ⅰ H, Ⅰ S lightest, Ⅰ H / Ⅰ S for the intermediate; (b) Ⅱ type (Hunter): 1. Type: Ⅱ A (heavy), Ⅱ B (light): 2. Enzyme defects (gene locus): Ai bar sugar sulfate sulfatase (Xq27-q28); 3. Urine sugar polysaccharide row: DS, HS; 4. Clinical manifestations: the growth of backward, intelligent behind the ugly face, joint stiffness, multiple bone dysplasia light than Ⅰ H, Ⅱ B-type lighter than type Ⅱ A; (c) Ⅲ type (Sanfilippo): 1. Type and enzyme defect (locus): (1) Ⅲ A enzyme defects (gene locus): sulfur lactamase; (2) Ⅲ B enzyme defects (gene locus): α-N-acetyl has glycosidase; (3) Ⅲ C enzyme defects (gene locus): acetyl-CoA-α-glucosamine-N-acetyltransferase; (4) Ⅲ D enzyme defects (gene locus): N-acetyl glucosamine -6-- sulfate sulfatase (12q14); 2. Urine sugar polysaccharide row: HS; 3. Clinical manifestations: the ugly face, bone deformities than Ⅰ, Ⅱ type is light, the gradual emergence of growth behind the rapid degeneration of nervous system function, serious intelligence behind, with hepatosplenomegaly, joint stiffness, etc.; (d) Ⅳ type (Morquio): 1. Type and enzyme defect (locus): (1) Ⅳ A enzyme defects (gene locus): GalNAc -6-- sulfate sulfatase (16q24.3); (2) Ⅳ B; enzyme defects (gene locus): β - galactosidase (3p21.3); 2. Urine sugar polysaccharide row: KS, CS; 3. Clinical manifestations: normal intelligence, short stature, joint laxity, bone deformities significantly, facial hypoplasia, corneal opacity, deafness, mild liver and spleen increased; (e) Ⅵ type (Maroteaux-Lamy): 1. Enzyme defects (gene loci): aromatic sulfatase (5q13-q14); 2. Urine sugar polysaccharide row: DS, HS; 3. Clinical manifestations: Clinical and two types, A-type like Ⅰ H-type, but of normal intelligence; B-type had no significant bone deformities; (f) Ⅶ type (Sly): 1. Enzyme defects (gene locus): β-glucuronidase (7q21.1-q22); 2. Urine sugar polysaccharide row: HS, DS, CS; 3. Clinical manifestations: Ⅰ H-type with similar, but slightly abnormal or normal intelligence; diagnosis (a) physical development disorders: Children 1 year old after the show most of the growth in the backward, short stature; joints aberration, kyphosis or scoliosis, common knee valgus, claw-like hands and other changes. Children with large heads, ugly face, prominent forehead and two zygomatic, hair, hair more than the low, small eye fissure, eye away from the wide, low, flat nose, big nose, small chin, lips thick. (B) mental retardation: the spirit of neural development in children gradually slow in the 1 year old, but most of Ⅰ S-type children of normal intelligence. (C) Eye diseases: Most of the children around 1 year old appears in the corneal opacity, corneal mucopolysaccharide in the main to KS and DS, Ⅲ type enzyme defect without corneal lesions. Ⅰ S-type of glaucoma can occur. (D) other: Common hepatosplenomegaly, deafness, heart valve damage, arteriosclerosis and so on. With the progress of the disease, pulmonary insufficiency can occur, cervical nerve compression symptoms such as communicating hydrocephalus and secondary lesions. (E) X-ray examination of bone: the skull increases, shallow sella long; spine after scoliosis; vertebral wedge, thoracic and lumbar vertebral body before the lower edge of the lips was like protrusion; ribs and spine end small and sternum end wider, showing ribbon-like; forearm chunky, metacarpal base sharpened, distal phalanx narrow circle. (F) urine mucopolysaccharide detection: 1. Positive for toluidine blue colorimetric method as a screening test for this disease; 2. Cellulose acetate membrane electrophoresis can distinguish the type of urinary excretion of mucopolysaccharide in order to help classification; 3. Ammonium chloride, cetyl pyridine 24-hour urine test shows increased total glycosaminoglycan (normal is 3 ~ 25mg). (G) cytology: Bone marrow or peripheral blood lymphocytes by Wright's or Giemsa staining, can be seen in the cytoplasm stained deep purple granules (Reilly bodies), on the diagnosis of secondary value. (H) Enzymatic analysis: peripheral blood leukocytes, serum, or cultured fibroblasts enzymatic analysis, the diagnosis of various types of MPS activity determination should be based on subject. (Ix) Analysis of gene diagnosis of mucopolysaccharide metabolism in a variety of DNA coding for the enzyme gene mutations. With the above paragraph (a) ~ (g) of the disease can be diagnosed based with paragraph (h) or (IX) can be diagnosed and the disease type diagnosis. Science diagnosis based on clinical history, laboratory tests and X ray, CT MRI, B-, can be diagnosed by means of prenatal care. Differential diagnosis is sometimes required between the various types of differential diagnosis based primarily on clinical features of children check with the relevant enzymes Mucopolysaccharidosis Table 2 also increased disease still need to be identified with the following diseases: 1. Multiple acid lipase deficiency The clinical manifestations of this disease and mucopolysaccharidosis polycythemia are similar, but the symptoms of mental retardation and neurological disorders occur more quickly mucopolysaccharide often more similar to metachromatic white matter atrophy. Patients often have hepatomegaly and fixed laboratory ichthyosis skin sticky and more diabetes and cell-free enzyme deficiency. 2. Glycosides systemic deposition of lipid disorders ganglion (GML ganglion lipid glycosides disease) both mucopolysaccharide storage diseases of fat and clinical characteristics. Children in infancy that is severe systemic lipid glycosides ganglion deposition, smart growth retardation, hypotonia, hepatosplenomegaly, more than half of patients with macular skin and Yinghong points. 3. Mannosidase histiocytosis with psychomotor retardation, hearing loss ugly face hepatosplenomegaly, hypotonia, mild dysplasia and other multiple bone. A large number of urine mannose oligosaccharides, non-sticky and more diabetes. 4. Fucose patients ugly face, hepatosplenomegaly, severe mental, motor retardation, multiple bone dysplasia. Urinary excretion of fucose containing oligosaccharides, non-sticky and more diabetes. 5. Acylamino glucose aspartic aciduria and Hurler syndrome and Hunter easily confused syndrome. Children with normal birth, the gradual emergence of wide nose, collapsed bridge of the nose, flexion nose, thick lips so ugly face, and a short neck, skull asymmetry, scoliosis, hepatosplenomegaly, urine contains large amounts of aspartic acid Ammonia glucose. 6. Sticky sticky fat fat disease type Ⅰ clinical manifestations of disease and X-ray changes have much in common with the Hurler syndrome. However, most patients with adhesive resin myoclonic seizures, muscle atrophy, chorea-like athetosic, nystagmus, and macular skin and Yinghong points. Sialic acid-binding urinary excretion of oligosaccharides increased proteoglycan levels were normal. Sticky resin disease type Ⅱ early psychomotor retardation occurs, and the early stage of rapid development of gingival hyperplasia, narrow thorax, heart valve disease more common, no corneal opacity, can be known for about six months of periosteal bone formation, often in children who died in infancy . No increase in urinary mucopolysaccharide. Sticky resin type Ⅳ disease may have mental retardation, corneal opacity, etc. but not sticky and more diabetes. 7.Kneist syndrome Morquio syndrome with similar clinical manifestations, including the bulk of collapsed bridge of the nose, cleft palate, short neck, bell-shaped chest, retinal detachment, hearing loss hernia short limbs and trunk, arched tibial kyphosis, joint stiffness, etc. . Children can also have keratin sulfate in urine, but no N-acetyl-galactosidase -6-- sulfatase or β-galactosidase lack of laboratory tests: 1. Urine Test (1) sticky polysaccharides: ① Urine mucopolysaccharide qualitative test: urine was purple blue spot at the ring or spots as positive, normal urine patches no color change.
urine
② 24h Determination of urinary mucopolysaccharides: normal daily urinary excretion of mucopolysaccharides was 3 ~ 25mg. Mucopolysaccharidosis increased in patients with urinary mucopolysaccharide often than 100mg/24h. As a result of increased disease mucopolysaccharidosis type lacking the enzyme is different from the urine of their composition and quantity of the viscous polysaccharides were different. MPS Ⅰ, MPS Ⅱ and MPS Ⅶ type of urinary chondroitin sulfate proteoglycan and heparan sulfate, Hurler syndrome, of which the most significant in patients with MPS Ⅲ type heparan sulfate in urine only. MPS Ⅳ type of keratin sulfate, increases with decreasing age trend. MPS Ⅵ based mainly chondroitin sulfate. (2) Activity Assay: Determination of urine can be a variety of enzymes, various types of mucopolysaccharidosis have a corresponding increase in disease activity decreased. 2. Blood test (1) Reilly bodies: the type of disease can be increased mucopolysaccharides in the peripheral blood or bone marrow lymphocytes and neutrophils saw sizes and shapes of purple sticky polysaccharide particles, Reilly bodies MPS Ⅵ type addition to other white blood cells, platelets can still be seen in the Reilly bodies. (2) Activity Assay: Determination of peripheral blood leukocytes in activity in the diagnosis and identification of various types of mucopolysaccharidosis main basis for polycythemia. Other laboratory examinations: 1.X-ray examination (1) MPS Ⅰ type: MPS Ⅰ type in all subtypes, X-ray findings of bone changes are also in the most severe Hurler syndrome. ① head: within 6 months after birth is normal then the gradual emergence of craniosynostosis, the former fontanelle closure delay. Increased anteroposterior diameter of the skull was scaphoid. Meningeal thickening can cause obstructive hydrocephalus before and after the sella can further increase the head diameter increases, the shoe was lying; a subarachnoid cyst, could occur sella increased. Diploe dense skull plate thickening, hardening of the skull base and also orbital roof. Sphenoid, mastoid and paranasal Doufa Yu and gasification of the mandible bad chunky, hook sudden bad education, the ankle was flat or depression-like fossa shallow and irregular. Missing teeth, sparse array of small, often located in the mandible the molars. ② spine: vertebral body upper and lower double-convex or oval-shaped odontoid short, may have atlantoaxial subluxation. Lower thoracic and upper lumbar spine (chest 12, waist or waist 1 1, 2 lumbar) vertebrae short oval, its anterior edge sharpened, showing . ③ thorax: rib, small end of the spine, gradually widened the middle to the end of the sternum, was Significantly thicker medial clavicular segment, lateral segment smaller and upturned. Scapular position increased slightly equilateral triangle, the next angle sharpened scapulae, shallow and small, or even disappear. Small flat humeral head and neck - shaft angle becomes smaller, or even at right angles, can have varus deformity. ④ pelvis: iliac wing and outside, within the base below the narrowed iliac, ischial obturator oval, widened pubic symphysis. Outside the upper edge of the acetabulum was sloping, shallow acetabulum, acetabular angle increases. Small dense flat femoral head, femoral epiphyseal nucleus flat and small or irregular, and there late, slender neck and neck - shaft angle was increased valgus. ⑤ long bone: changes in upper limb than obvious. Shaping the backbone of the barrier, resulting in the backbone of rough and short, tapered at both ends, thinning of cortical bone, bone marrow cavity increased. Developmental disorders seen bar-shaped metaphyseal lines, small epiphyses, irregular, or there delay. ⑥ short tube of bone and wrist: palmar (plantar), finger (toe) thickening proximal, distal sharpened, showing bullet-like. Distal phalanges (especially the thumb) Remote variable taper, showing claw-like flexion deformity. Carpal irregular ossification delay, ossification center is small, and the number of children less than age distal radius and ulna developmental disorders, wrist joints appear before end (2) MPS Ⅱ type: changes in the skeletal system is similar to Hurler syndrome, but appears relatively late, progress is slow, change less often. Main changes include: broadening the backbone of long bones, multiple bone development disorder. Sella was (3) MPS Ⅲ Type: The type of minor skeletal abnormalities, may have parietal, posterior temporal and occipital mastoid gasification thickening bad; upper and lower vertebral body is slightly elevated, or oval; clavicle widened medial side , some patients before the rib was Straight line on the edge of the acetabulum; bone chunky, slightly widened metaphysis,air force 1 high, may be associated with bone remodeling disorder. Bone marrow cavity narrow, irregular. (4) MPS Ⅳ type: skull, sella turcica normal. Early vertebral then gradually become slightly rounded, flat, front middle of a prominent tongue-like, widened intervertebral space; small or absence of the odontoid process, atlantoaxial joint can lead to instability. Increased anteroposterior diameter of the thorax, sternum shortening, and chicken-like protrusion bent; rib front-end sag, and have widened, outreach, rib side after thinning. Widening of the medial end of clavicle, was outside the top of the shoulder blade stretch Dieyi like small, elevated position, shallow glenoid or disappear. Outreach iliac wing, narrow iliac bone at the base, shallow acetabulum, was from the outside on the lower slopes inward like change, ischium and pubis stubby. Femoral metaphyseal enlargement, depression, irregular, femoral neck - shaft angle increases, may have dislocation of the hip. Distal femur and proximal tibia epiphysis flat and small, metaphyseal broadening, was double or wavy with a dense, narrow epiphyseal line. Feet, the small distal radial epiphysis without rules, or even disappear appear before sloping joints; carpal small, irregular. Generally stubby long bones, irregular metaphyseal widening, and a cusp-like protrusions; sparse trabecular bone cortical thinning and irregular, avascular necrosis of bone marrow samples may have changed. Metacarpophalangeal stubby, non-epiphyseal end narrower. (5) MPS Ⅵ type: Similar to Hurler syndrome. Some patients may have epiphyseal ischemic necrosis of femoral head epiphysis like changes in common. (6) MPS Ⅶ type: mainly for patients with multiple bone dysplasia, X-ray findings with Hurler syndrome similar. 2.CT and magnetic resonance imaging (MRI) can accurately understand, including the brain, spinal bone (cartilage), joints, respiratory and cardiovascular systems, the extent and scope of structural change. Both can be clearly shows the skull dysplasia, white matter changes, spinal stenosis hydrocephalus, arachnoid cyst, dural thickening of the craniocervical joint, spinal cord compression. However, inspections in the white matter,air force one shoes, MRI is more sensitive and reliable than the CT general, the longer the duration of CT and MRI are the more obvious changes. 3.B-mode ultrasound for fetal examination, whether the fetus can be found in bone and joint deformities, hepatosplenomegaly, and hydrocephalus and other abnormalities. 4. Biopsies biopsy showed liver cells, skin or connective tissue fibroblasts contained mucopolysaccharide metabolism enzyme activity were significantly reduced. 5. Antenatal checks as a normal pregnancy is not usually a routine examination. Mannosidase for increased birth to children of female patients, again during pregnancy amniotic fluid proteoglycan concentration and viable cell activity assay of amniotic fluid if the mucopolysaccharide concentration in amniotic fluid significantly increased activity in amniotic fluid cells was significantly reduced, then prenatal diagnosis can be determined. So far no effective treatment for treatment. (A) General treatment: attention to rest, to prevent respiratory infections. (B) basic drug therapy: high-dose corticosteroids can inhibit the synthesis of mucopolysaccharide in the skin, but the patient application invalid. Large doses of vitamin A can cultured fibroblasts mucopolysaccharide deposition reduced, the patient applications are invalid. Penicillamine daily 20mg/kg, orally can decrease urine GAG excretion. (C) other treatment: enzyme replacement and gene therapy are being studied. Bone marrow transplant may improve symptoms, especially for minor children smart damage. Infusion of fresh plasma to temporarily improve the normal condition. Prognosis of children with disabilities in accordance with the severity of the prognosis and spinal compression may be. Normal intelligence. Spinal deformity can be a special belt to prevent the further development of nerve compression symptoms may be surgery. References 1. Chen Jiqing: Yang Xijiang: Full name is the marketing professional services. MPS customers to: provide high quality services, access to and use of marketing information, online marketing, market segmentation, market selection and market positioning, understanding the customer as an individual, professional service pricing, to make services accessible, integrated marketing communication, customer Strategic relationships with our customers. Dutch ornamental plant production and environmental programs in recent years, the Dutch ornamental plant production and environmental protection plan (MPS) has been for the creation of a fair flower (Fair Flower & Plants, referred to as FFP) certification system and the International Flower Wholesalers Association (Union Fleurs), retail organizations and relevant chambers of commerce organizations. Recently, the Dutch Horticultural Product Board (Dutch Product Board f or Horticulture) announced the creation of a fair flower authentication system will work to give a lot of financial aid. Meanwhile, the European Commission will decide in November whether the European countries give financial assistance to this work. This work is the creation of a fair flower into a new stage. Common commitment to social responsibility, whether in Europe or in areas outside of Europe is an irreversible trend. In the retail sector, it is always hard to find merchandise to reflect the self-personality, and commitment to the common responsibility of society is precisely such a class of commodity goods. Flowers received a fair flower logo, the Netherlands ornamental flowers such environmental program will be linked to the common responsibility of society together. Fair flowers with flowers to represent the label, this flower is based on sustainable development and socially responsible attitude produced, consumers see the label, it will naturally flower producers in respect. Those who receive a fair flower label products, also guarantee the product in the production process, not only to fully protect the legitimate rights and interests of employees, but also to maximize the protection of the environment. The most basic conditions to obtain the label ornamental plants in the Netherlands A-level environmental programs (MPS-A) standard and meet the terms of the scheme of social (MPS-GAP) or the corresponding standards. Soon after, the fair flower in Sweden, Germany, Austria and the United Kingdom implemented. Dutch ornamental plant environmental programs will soon provide this service. Another meaning of MPS: MPS MultiProcessins System. The multi-processor systems. SMP MPS constitute a technology. MPS another meaning: the main product schedule MPS (masterproductionschedule) concept: According to the marketing plans, BOM and process planning decisions of various parts of time and finished product manufacturing progress. He determines the finished products and spare parts production in each time period, including production time, assembly time and the amount or quantity. MPS (Master Production Schedule,air force 1 shoes, referred to as MPS) ERP management of the first stage. Master production schedule is to determine the final products in each specific period of time the production quantity of each specific program. Here's the final product is finalized for the enterprise to the factory's finished product, it should be specific to the product varieties, models. Here's the specific time period, usually weekly basis, in some cases, it can be days, ten days, months. Master production schedule provides details of what is produced, what time should be output, which is independent of demand planning. Master production schedule based on customer contracts and market forecasts, the business plan or outline in the production of specific products, making it expand the main basis for material requirements planning, comprehensive plan to play a specific plan from the nexus role in the transition. MPS re a meaning: material product balance system (System of Material Product Balances). 20 from the mid 20th century, large-scale production requirements in the community, the former Soviet Union to meet the needs of planned economy and began the preparation of national balance sheet, forming a system of the early 30s, the basic shape of the late 50s, and gradually extended to former CMEA countries. MPS economic definition marginal propensity to save MPS (marginal propensity to save) 1 yuan each change in national income brought about by changes in savings. Namely: the marginal propensity to save = the ratio of increment and the incremental revenues. Mathematical formula: MPS = Δs / Δy = ds / dy mathematical meaning of the marginal propensity to save: MPS is the saving function of the first order derivative; savings curve is tangent at any point. Saving function is linear, MPS is a constant (1-β). Nonlinear time, MPS has increasing trend with income. MPS and APS have increased, but the MPS> APS. For example: income from 100 yuan to 120 yuan, consumption by the 80 to 94 yuan. MPS =  S /  Y = 26-20/120-100 = 0.3MPS (Man Pusi) early pregnancy processing MPS (Man Pusi) flow introduces MPS (Manpu Si) technology is a minimally invasive treatment of early pregnancy (Minimally invasive), Painless (Painless), Private (Private), Security (Security), convenience (Speedy) abbreviation, by the China Senior Professors Association, recommended by thousands of gynecologic experts agreed the standard procedure to resolve an unwanted pregnancy.