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Acquired Generalized Lipodystrophy

GCL


Presentation

  • Recent case reports revealed that lymphoma is present in some patients but its prevalence is not known at this time.[en.wikipedia.org]
  • We propose that AGL be added to the list of possible diseases associated with CVI and, owing to the similar clinical presentation with type 1 diabetes mellitus, be included in the differential diagnosis of this condition, which is present in 1.5% of patients[ncbi.nlm.nih.gov]
  • Progression towards partial lipoatrophy, focal or generalized, has been reported in patients with dermatomyositis, amongst whom this could be a late relapse, and it is more common that the antibody anti-p155 is present.[orpha.net]
Accelerated Growth
  • During childhood, those living with CGL have large appetites, accelerated growth in height, and advanced bone age.[psychneuro.wordpress.com]
  • During childhood, affected individuals are described as being voracious eaters and may experience accelerated growth. Affected individuals may also experience fatigue.[rarediseases.org]
  • Hepatosplenomegaly, umbilical prominence or hernia, acanthosis nigricans, voracious appetite and accelerated growth can occur. Female patients develop hirsutism, may have clitoromegaly, oligomenorrhea and polycystic ovaries.[intechopen.com]
Coarse Facial Features
  • She had coarse facial features with generalized loss of subcutaneous fat and prominent muscularity. Remarkable acanthosis nigricans was present over the neck, axilla, and umbilicus.[ncbi.nlm.nih.gov]
Increased Energy
  • Increased fat cell size may represent the failure of the adipose tissue mass to expand and thus to accommodate an increased energy influx.[scienceopen.com]
Overeating
  • We present the case of a 78-year-old woman who experienced almost complete loss of subcutaneous adipose tissue over 6 years.[ncbi.nlm.nih.gov]
  • Visualize more of the conditions you see in practice with over 3500 illustrations, of which over 1,400 are new: 1,039 clinical images, 398 pathology slides, and 152 schematics.[books.google.com]
Increased Appetite
  • Hypermetabolism is an important feature and is associated with increased appetite; there is ingestion of large amounts of calories and profuse sweating. There may be hyperthermia.[ijdvl.com]
Absence of Subcutaneous Fat
  • […] of subcutaneous fat and the presence of muscle hypertrophy, hyperlipemia, diabetes mellitus, and hepatosplenomegaly with cirrhosis.[epathologies.com]
  • Case Reports Case 1 A 13-month-old female child, born out of nonconsanguineous marriage, was brought to our department with complaints of abnormal facial features, almost complete generalized absence of subcutaneous fat, muscular hypertrophy, generalized[ijpd.in]
  • The physical appearance of patients with familial and acquired PL may be more difficult to discern compared with generalized lipodystrophy (GL) where patients have a stark absence of subcutaneous fat (Figure 1) [ 2 ].[omicsonline.org]
  • Therefore, novel CGL genes may be identified in the future. 23 The most striking clinical manifestations of CGL are abnormal appearance due to the absence of subcutaneous fat in the abdomen and thorax, which becomes evident within the first 2 years of[dovepress.com]
Chronic Urticaria
  • Case 3 is an 11-year-old boy with active autoimmune hepatitis and chronic urticaria, whose hyperphagia has resolved and his liver enzymes and hepatosplenomegaly have improved.[ncbi.nlm.nih.gov]
  • AGL case 3 (AGL-3) AGL-3 is an 11-year-old boy with AGL and autoimmune hepatitis, chronic urticaria, and IgA deficiency.[academic.oup.com]

Workup

  • "Localized Lipodystrophy Workup." Medscape. Updated: Dec 16, 2014. Handelsman et al. "The clinical approach to the detection of lipodystrophy- an AACE consensus statement." Endocr Pract. 2013 Jan-Feb; 19(1): 107–116. Lipodystrophy United.[medicinenet.com]
  • Despite extensive workup, neutropenia of unknown etiology persisted. At the age of 11, she had hepatosplenomegaly and the possibility of portal hypertension was considered.[karger.com]
  • Clinical Testing and Workup Although the diagnosis of lipodystrophy is primarily clinical, a variety of tests can be used to aid in the diagnosis and/or rule out other conditions.[rarediseases.org]
LDL Decreased
  • Metabolic syndrome In the general population, MS is described as an association of increased upper-body fat, particularly in the visceral area, with IR responsible for and revealed by metabolic alterations, including increased LDL, decreased HDL, increased[intechopen.com]

Treatment

  • Management and treatment The treatment of the metabolic manifestations is a priori no different to the treatment of other forms of insulin resistance: physical exercise, insulin sensitizers such as metformin or pioglitazone, insulin (or preferably insulin[orpha.net]
  • Treatments are also similar and mainly supportive for symptomatic alleviation.[en.wikipedia.org]
  • Our observations suggest that risk or presence of autoimmune disease should not lead to withholding of metreleptin treatment from patients with AGL, but should prompt close clinical follow up in light of cautionary preclinical data.[ncbi.nlm.nih.gov]
  • […] the metreleptin treatment ( Figure 1 D).[academic.oup.com]

Prognosis

  • Prognosis The prognosis is not well known but is probably related to cardiovascular risk (linked to the insulin-resistance syndrome) and to the underlying cause of the disease.[orpha.net]
  • What is the prognosis and lifespan for a person with lipodystrophy? It is difficult to estimate the health prognosis for a given person with lipodystrophy because there are many different kinds of the disorder.[medicinenet.com]
  • The prognosis of the disease is unknown as of December, 2017. Many researches for the treatment of lipodystrophy focus on the safety and efficacy of leptin replacement therapy and the outlook is positive in many studies.[en.wikipedia.org]
  • Affected individuals should talk to their physician and medical team about their specific case, associated symptoms, and overall prognosis.[rarediseases.org]

Etiology

  • Etiology The cause of the disease remains unknown. There may be infectious triggering factors (there was a recent case of the panniculitis type that appeared after tuberculosis) or an autoimmune mechanism.[orpha.net]
  • There are three main etiologies of AGL suspected: autoimmune, panniculitis-associated, or idiopathic. After its onset, the disease progresses over a few days, weeks, months, or even in years.[en.wikipedia.org]
  • In most patients evidence exists of an autoimmune etiology.[endocrine-abstracts.org]
  • Types of Lipodystrophy Lipodystrophies are categorized on either etiology – whether the disease was acquired or congenital – or through the pattern of adipose tissue loss which may be generalized (affecting the whole body) or partial (only concentrated[gudhealth.com]

Epidemiology

  • Summary Epidemiology More than 100 cases have been described and the female to male ratio is 3:1.[orpha.net]
  • Descrizione: 1 online resource : illustrations (chiefly color) Contenuti: Hormones and hormone action -- Endocrine autoimmunity -- Evidence-based endocrinology and clinical epidemiology -- Hypothalamus and pituitary gland -- The posterior pituitary (neurohypophysis[worldcat.org]
  • Harmonizing the metabolic syndrome: a joint interim statement of the International Diabetes Federation Task Force on Epidemiology and Prevention; National Heart, Lung, and Blood Institute; American Heart Association; World Heart Federation; International[doi.org]
Sex distribution
Age distribution

Pathophysiology

  • Wyndham Wilson provided insight in the pathophysiology of lymphoma.[doi.org]
  • ; Insulin: Making Sense of Current Options; Nutrition in Diabetes; Bariatric Surgery: Pathophysiology and Outcomes; Future Therapies in Diabetes; Lipodystrophic Syndromes; and In-patient Diabetes Management in the 21st Century.[books.google.com]
  • The exact pathophysiologic mechanism is mostly unknown; however, each of three main origins, autoimmune, panniculitis, or idiopathic, may have different mechanisms of pathogenesis.Normally, adipose tissues contain adipocytes to store fat for energy during[en.wikipedia.org]
  • These two new paradigms provide a framework to advance our understanding of the pathophysiology of the insulin-resistance syndrome.[scienceopen.com]

Prevention

  • Although idiopathic AGL accounts for about 50% of all AGL, it can vary in its origin and its is unclear how it develops.No known preventive measurement has been reported.[en.wikipedia.org]
  • Management focuses on preventing and treating metabolic complications.[ncbi.nlm.nih.gov]
  • Early recognition and differentiation from other congenital generalized lipodystrophies help in the initiation of appropriate preventive and therapeutic measures such as lifestyle modification and pharmacotherapy that helps postpone the onset of metabolic[idoj.in]
  • You'll need to work closely with your doctor to prevent complications. For example, people with AGL are likely to have heart and liver problems. Diabetes can be hard to control.[webmd.com]
  • One hypothesis is that the adipocyte hormone leptin has a critical role in preventing the insulin resistance and hypertriglyceridemia of lipodystrophy.[doi.org]

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