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]

• Absence of Subcutaneous Fat

  Over the next 3 years, a complete generalized loss of subcutaneous fat developed that began focally in the lower extremities. [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]

  Overall, in CGL, blood leptin concentrations are low, consistent with near total absence of body fat. [dovepress.com]

"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

  Dyslipidemia A number of HIV-infected patients present dyslipidemia, increased cholesterol, low-density lipoprotein (LDL)-cholesterol and triglycerides together with decreased HDL. [intechopen.com]

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]

Treatment for HIV patients: There are several treatments described for HIV-associated lipodystrophy. [medicinenet.com]

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]

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]

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]

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]

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]

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]

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]

Wyndham Wilson provided insight in the pathophysiology of lymphoma. [doi.org]

In some patients, it is genetic, and in others it may be acquired for different pathophysiological, and in some cases unknown, reasons. Generalized lipodystrophy is characterized by a widespread loss of fat tissue under the skin. [raredr.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]

Management focuses on preventing and treating metabolic complications. [ncbi.nlm.nih.gov]

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]

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]

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]

Though diet modification does not really do much in treating the disease, it helps in prevention of the disease progression. [gudhealth.com]