脂肪营养不良综合征课件.ppt
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1、Lipodystrophy syndromes1 definition and classificationClinical Findings of the Major Lipodystrophy SubtypesClinical CharacteristicsstructureDiagnosisTherapies2 definition andClassification34 A heterogeneous group of congenital or acquired disorders characterized by either complete or partial lack of
2、 adipose tissue,which may occur in conjunction with pathological accumulation of fat in other distinct regions of the body,Belonged to the autonomic nervous system diseases definitionclassification congenital generalized lipodystrophy(CGL)acquired generalized lipodystrophy(AGL)acquired partial lipod
3、ystrophy(APL)familial partial lipodystrophy(FPL)HAART-associated lipodystrophy syndrome56 CGL(先天性全身脂肪营养不良),or Berardinelli-Seip syndrome(伯拉迪尼-塞普综合征),is an autosomal recessive常染色体隐性遗传 disorder characterized by a generalized lack of adipose tissue at birth or shortly thereafter(within the first year o
4、f life),and is accompanied by prominent muscularity and subcutaneous veins.Congenital Generalized Lipodystrophy7 In early childhood,patients with CGL may exhibit hyperphagia 食欲过盛(possibly a manifestation of underlying leptin deficiency),accelerated linear growth,advanced bone age骨龄超前,or acromegaloid
5、 features(enlarged hands,feet,and mandible),while later in childhood,acanthosis nigricans黑棘皮症 can develop and become widespread.Hyperinsulinemia and hypertriglyceridemia高胰岛素血症和高甘油三酯血症 can occur at an early age,with ketosis-resistant diabetes mellitus酮症性糖尿病 usually developing later in adolescence.Con
6、genital Generalized Lipodystrophy8 Hepatomegaly from severe hepatic steatosis is common and can progress to steatohepatitis脂肪性肝炎,cirrhosis,and liver failure.females with CGL may have hirsutism,clitoromegaly,irregular menstrual periods,polycystic ovaries,and/or infertility.There are at least three ki
7、nds of disease-causing genes,clinically divided into three subtypes:CGL1,CGL2,CGL3.95%of CGL for the first two subtypes,and mutations associated基因突变 AGPAT2 and BSCL2.Congenital Generalized Lipodystrophy Congenital Generalized Lipodystrophy Congenital generalized lipodystrophy in A,a 6-month-old infa
8、nt with promi-nent muscularity and veins,B,a 16-year-old girl with acanthosis nigricans and umbilical prominence,C,a 15-year-old boy with umbilical prominence and otherwise normal appearing muscular habitus.910 AGL(获得性全身脂肪营养不良),or Lawrence syndrome(劳伦斯综合征),in contrast to CGL,patients with AGL are bo
9、rn with normal fat distribution but lose fat in a generalized fashion,typically starting in childhood or adolescence(rarely beginning after 30 years of age)acquired generalized lipodystrophy 11 Progressive fat loss usually occurs over a period of months to years,or as rapid as a few weeks for some p
10、atients,and affects large areas of the body,especially the face and extremities(including the palms and soles).Intra-abdominal fat loss is vari-able,and there may be sparing of bone marrow骨髓 and retroorbital眶后的 fat.acquired generalized lipodystrophy 12 In some patients,the onset of AGL is heralded b
11、y the development of subcutaneous inflammatory nodules皮下炎性结节(panniculitis);AGL occurs in approximately 3 times as many women as men.In a case series of 79 patients,AGL was classified into panniculitis-associated脂膜炎(25%of cases),autoimmune自身免疫(25%),and idiopathic types特发性(50%)on the basis of clinical
12、 findings.Autoimmune diseases,especially juvenile dermatomyositis儿童皮肌炎 and autoimmune hepatitis,occur commonly with AGL,suggesting that AGL could represent an autoimmune disease itself,but the inciting factors(autoantigens or effector mechanisms)remain to be elucidated.acquired generalized lipodystr
13、ophy 13 acquired generalized lipodystrophy Acquired generalized lipodystrophy(AGL)in A,a 19-year-old woman,B,a 9-year-old girl with juvenile dermatomyositis.Some common features among the 2 cases include lack of body fat and acanthosis nigricans,as well as abdominal protuberance.14 For both CGL and
14、AGL,the presentation of diabetes in association with clinical evidence of insulin resistance胰岛素抵抗(e.g.,high triglyceride levels)in a nonobese pediatric patient should serve as key distinguishing features from type 1 diabetes.it is important to recognize that patients with all forms of generalized li
15、podystrophy can develop ketoacidosis酮症酸中毒,especially under severe metabolic stress.generalized lipodystrophy 15 FPL(家族性部分脂肪营养不良)is predominantly inherited in an autosomal dominant 常染色体显性遗传fashion.Patients with FPL usually have normal body fat distribution during infancy and early childhood,but,begin
16、ning around or after puberty青春期,typically develop variable and progressive loss of subcutaneous fat in the arms and legs resulting in a peripheral muscular appearance with variable loss of fat in the anterior abdomen and chest.Familial Partial Lipodystrophy16 Many patients(especially women)have fat
17、accumulation in the face,neck,and intra-abdominal region,which may lead to a Cushingoid 类库欣appearance.Familial Partial Lipodystrophy17 Diabetes is more common and more severe in women than in men,particularly among multiparous women with excessive intra-abdominal fat deposition.Most affected women a
18、re able to reproduce normally,although some may develop hirsutism多毛 and menstrual irregularities.Familial Partial Lipodystrophy18 Hypertriglyceridemia is a common finding in FPL and can be severe,potentially leading to acute pancreatitis急性胰腺炎,while hepatic steatosis and acanthosis nigricans 肝脂肪变性和黑棘
19、皮病may be less clinically impressive than that occurring in patients with generalized forms of lipodystrophy.Finally,some patients with FPL may develop myopathy肌病,cardiomyopathy心肌病,and/or conduction system abnormalities传导系统障碍。Familial Partial Lipodystrophy19 Familial Partial LipodystrophyFamilial par
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