The Diagnosis and Management of Lipodystrophy Syndromes: A Multi-Society Practice Guideline.

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Term Occurence Count Dictionary
hypertriglyceridemia 11 endocrinologydiseases
obesity 1 endocrinologydiseases
Cushing's syndrome 1 endocrinologydiseases
acromegaly 1 endocrinologydiseases
congenital generalized lipodystrophy 2 endocrinologydiseases
hypoglycemia 2 endocrinologydiseases
hypogonadism 1 endocrinologydiseases
lipomatosis 1 endocrinologydiseases
metformin 2 endocrinologydiseasesdrugs
pioglitazone 1 endocrinologydiseasesdrugs
Familial partial lipodystrophy 1 endocrinologydiseases
Insulin 4 endocrinologydiseasesdrugs
diabetes mellitus 1 endocrinologydiseases
multiple symmetric lipomatosis 1 endocrinologydiseases
thyrotoxicosis 1 endocrinologydiseases
gigantism 1 endocrinologydiseases
hyperglycemia 1 endocrinologydiseases
lipodystrophy 85 endocrinologydiseases
Werner syndrome 1 endocrinologydiseases
hyperlipidemia 1 endocrinologydiseases

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Select Drug Character Offset Drug Term Instance
Insulin 6262 resulting in ectopic lipid storage in the liver, muscle, and other organs and causing insulin resistance. Insulin resistance leads to diabetes, hypertriglyceridemia, polycystic ovarian syndrome (PCOS), and nonalcoholic
Insulin 6692 HDL-cholesterol, acute pancreatitis, eruptive xanthomas)AGL, CGL, FPLD[4], [5], [7], [9], [13], [21], [30] Insulin resistance/diabetes, acanthosis nigricans (and diabetes complications)AGL, CGL, FPLD[4], [5], [7], [9],
Insulin 29822 comorbiditiesDiabetesMetformin is a first-line agent for diabetes and insulin resistance. (Class IIa, Level C) Insulin is effective for hyperglycemia. In some patients, concentrated preparations and high-doses may be required.
Insulin 30485 [61]). In patients with high insulin requirements, concentrated insulins should be considered ([62]). Insulin glargine and degludec kinetics may be altered when injected in lipodystrophic areas because their long
metformin 2302 partial lipodystrophy. Other treatments not specific for lipodystrophy may be helpful as well (eg, metformin for diabetes, and statins or fibrates for hyperlipidemia). Oral estrogens are contraindicated.PrecisMultiple
metformin 30161 with caution in generalized lipodystrophy. (Class IIb, Level B)Among the oral hypoglycemic agents, metformin is used most frequently. In patients with partial lipodystrophy, thiazolidinediones improved HbA1c,
pioglitazone 32822 treatments ([72]), and among pharmacological treatments, vitamin E (in children and adults) ([73], [74]) and pioglitazone (in adults) ([73], [75]) have shown the most consistent benefit for liver histopathology. However, these
Select Disease Character Offset Disease Term Instance
Cushing's syndrome 15942 lipodystrophies can be confused with mutations of the insulin receptor or acromegaly/gigantism, and FPLD with Cushing's syndrome , truncal obesity, and multiple symmetric lipomatosis.Establishing the subtype of lipodystrophyPattern
Familial partial lipodystrophy 9025 Metabolic complications are frequent and may be severe. Cardiomyopathy or rhythm disturbances may occur. Familial partial lipodystrophy FPLD is a group of usually autosomal dominant disorders characterized by loss of fat affecting the limbs,
Werner syndrome 21959 children who present with idiopathic AGL or atypical CGL. Specific progeroid syndromes (eg, Bloom and Werner syndrome s) are associated with increased malignancy risk (Supplemental Table 2).Treatment of Lipodystrophy SyndromesCurrent
acromegaly 15906 of body fat. Generalized lipodystrophies can be confused with mutations of the insulin receptor or acromegaly /gigantism, and FPLD with Cushing's syndrome, truncal obesity, and multiple symmetric lipomatosis.Establishing
congenital generalized lipodystrophy 2982 affecting the entire body (generalized) or only regions (partial). This yields four major categories: congenital generalized lipodystrophy (CGL), familial partial lipodystrophy (FPLD), acquired generalized lipodystrophy (AGL), and acquired
congenital generalized lipodystrophy 3545 four main subtypes of lipodystrophy syndromes. A, Lateral view of a 33-year-old Hispanic female with congenital generalized lipodystrophy (also known as Berardinelli-Seip congenital lipodystrophy), type 1 due to homozygous c.589–2A>G; p.(Val197Glufs*32)
diabetes mellitus 15449 include conditions presenting with severe weight loss (malnutrition, anorexia nervosa, uncontrolled diabetes mellitus , thyrotoxicosis, adrenocortical insufficiency, cancer cachexia, HIV-associated wasting, chronic infections).
gigantism 15917 fat. Generalized lipodystrophies can be confused with mutations of the insulin receptor or acromegaly/ gigantism , and FPLD with Cushing's syndrome, truncal obesity, and multiple symmetric lipomatosis.Establishing
hyperglycemia 29847 first-line agent for diabetes and insulin resistance. (Class IIa, Level C)Insulin is effective for hyperglycemia . In some patients, concentrated preparations and high-doses may be required. (Class IIa, Level C)Thiazolidinediones
hyperlipidemia 2354 specific for lipodystrophy may be helpful as well (eg, metformin for diabetes, and statins or fibrates for hyperlipidemia ). Oral estrogens are contraindicated.PrecisMultiple worldwide endocrine societies developed practice
hypertriglyceridemia 698 deficient body fat associated with potentially serious metabolic complications, including diabetes, hypertriglyceridemia , and steatohepatitis. Due to their rarity, most clinicians are not familiar with their diagnosis and
hypertriglyceridemia 6300 liver, muscle, and other organs and causing insulin resistance. Insulin resistance leads to diabetes, hypertriglyceridemia , polycystic ovarian syndrome (PCOS), and nonalcoholic fatty liver disease (NAFLD) ([1]).Table 2.Major
hypertriglyceridemia 14445 U/d ≥2 U/kg/d Requiring U-500 insulin Severe hypertriglyceridemia ≥500 mg/dL with or without therapy ≥250 mg/dL despite
hypertriglyceridemia 14661 despite diet and medical therapy History of acute pancreatitis secondary to hypertriglyceridemia Non-alcoholic steatohepatitis in a non-obese individual Early-onset cardiomyopathy PCOSOther
hypertriglyceridemia 15687 difficult is differentiating lipodystrophy from uncontrolled diabetes because both may have extreme hypertriglyceridemia . However, restoring glycemic control in patients with nonlipodystrophic diabetes leads to a regain of
hypertriglyceridemia 27275 LDL- and total cholesterol but did not change HDL-cholesterol ([47], [48]). Acute pancreatitis due to hypertriglyceridemia has occurred in patients who acutely discontinued or reduced metreleptin ([47]).Metreleptin reduced
hypertriglyceridemia 28332 partial lipodystrophy is less robust than in generalized lipodystrophy. In one study, metreleptin reduced hypertriglyceridemia and improved glycemia in severely hypoleptinemic patients with partial lipodystrophy and severe metabolic
hypertriglyceridemia 31918 long-chain omega-3-fatty acids from fish oils have wide clinical use to avoid acute complications of severe hypertriglyceridemia ([46]) but have not been formally studied. Plasmapheresis has been used in extreme hypertriglyceridemia,
hypertriglyceridemia 32022 hypertriglyceridemia ([46]) but have not been formally studied. Plasmapheresis has been used in extreme hypertriglyceridemia , but must be repeated frequently ([69]). Additional lipid-lowering drugs have not been studied in patients
hypertriglyceridemia 34251 IIa, Level C)Oral estrogens are contraindicated in lipodystrophy syndromes due to the risk of severe hypertriglyceridemia and acute pancreatitis. Transdermal estrogens may be safer due to lesser hepatic exposure ([83]). There
hypertriglyceridemia 35211 difficult, with attendant fetal risks. Furthermore, metreleptin withdrawal has been associated with rebound hypertriglyceridemia ([41]), placing patients at risk for pancreatitis, endangering both mother and fetus.ConclusionsLipodystrophy
hypoglycemia 26781 as early as the first week ([42]) and lowered HbA1c by 2% after 1 year ([46]). To reduce the risk of hypoglycemia , frequent glucose monitoring is recommended. Providers should consider reducing insulin doses by approximately
hypoglycemia 29087 metreleptinApproximately 30% of patients experience side effects ([47]). The most clinically important are hypoglycemia (in patients receiving concomitant insulin) and infrequent injection-site reactions (erythema, urticaria).In
hypogonadism 20172 performed annually in children. (Class IIa, Level C)Early adrenarche, true precocious puberty, or central hypogonadism may occur in children with generalized lipodystrophy. Oligo/amenorrhea, decreased fertility, and PCOS
lipodystrophy 899 their diagnosis and management. This practice guideline summarizes the diagnosis and management of lipodystrophy syndromes not associated with HIV or injectable drugs.Participants:Seventeen participants were nominated
lipodystrophy 1857 diagnosed by clinical phenotype, supplemented by genetic testing in certain forms. Patients with most lipodystrophy syndromes should be screened for diabetes, dyslipidemia, and liver, kidney, and heart disease annually.
lipodystrophy 2042 kidney, and heart disease annually. Diet is essential for the management of metabolic complications of lipodystrophy . Metreleptin therapy is effective for metabolic complications in hypoleptinemic patients with generalized
lipodystrophy 2162 Metreleptin therapy is effective for metabolic complications in hypoleptinemic patients with generalized lipodystrophy and selected patients with partial lipodystrophy. Other treatments not specific for lipodystrophy may
lipodystrophy 2211 complications in hypoleptinemic patients with generalized lipodystrophy and selected patients with partial lipodystrophy . Other treatments not specific for lipodystrophy may be helpful as well (eg, metformin for diabetes,
lipodystrophy 2260 generalized lipodystrophy and selected patients with partial lipodystrophy. Other treatments not specific for lipodystrophy may be helpful as well (eg, metformin for diabetes, and statins or fibrates for hyperlipidemia). Oral
lipodystrophy 2513 contraindicated.PrecisMultiple worldwide endocrine societies developed practice guidelines for diagnosis and management of lipodystrophy syndromes based on current evidence.The lipodystrophy syndromes are a heterogeneous group of rare disorders
lipodystrophy 2567 practice guidelines for diagnosis and management of lipodystrophy syndromes based on current evidence.The lipodystrophy syndromes are a heterogeneous group of rare disorders that have in common selective deficiency of adipose
lipodystrophy 3005 (generalized) or only regions (partial). This yields four major categories: congenital generalized lipodystrophy (CGL), familial partial lipodystrophy (FPLD), acquired generalized lipodystrophy (AGL), and acquired
lipodystrophy 3043 (partial). This yields four major categories: congenital generalized lipodystrophy (CGL), familial partial lipodystrophy (FPLD), acquired generalized lipodystrophy (AGL), and acquired partial lipodystrophy (APL) (Figure 1).
lipodystrophy 3086 congenital generalized lipodystrophy (CGL), familial partial lipodystrophy (FPLD), acquired generalized lipodystrophy (AGL), and acquired partial lipodystrophy (APL) (Figure 1). Additional subtypes include progeroid disorders,
lipodystrophy 3128 familial partial lipodystrophy (FPLD), acquired generalized lipodystrophy (AGL), and acquired partial lipodystrophy (APL) (Figure 1). Additional subtypes include progeroid disorders, autoinflammatory disorders, and others
lipodystrophy 3300 disorders, autoinflammatory disorders, and others (Table 1). This practice guideline will not discuss lipodystrophy in HIV infected patients or localized lipodystrophy (eg, from injectable drugs).Figure 1.Physical appearance
lipodystrophy 3352 (Table 1). This practice guideline will not discuss lipodystrophy in HIV infected patients or localized lipodystrophy (eg, from injectable drugs).Figure 1.Physical appearance of patients with the four main subtypes of
lipodystrophy 3466 (eg, from injectable drugs).Figure 1.Physical appearance of patients with the four main subtypes of lipodystrophy syndromes. A, Lateral view of a 33-year-old Hispanic female with congenital generalized lipodystrophy
lipodystrophy 3568 lipodystrophy syndromes. A, Lateral view of a 33-year-old Hispanic female with congenital generalized lipodystrophy (also known as Berardinelli-Seip congenital lipodystrophy), type 1 due to homozygous c.589–2A>G; p.(Val197Glufs*32)
lipodystrophy 3626 Hispanic female with congenital generalized lipodystrophy (also known as Berardinelli-Seip congenital lipodystrophy ), type 1 due to homozygous c.589–2A>G; p.(Val197Glufs*32) mutation in the AGPAT2 gene. The patient
lipodystrophy 3993 (enlarged mandible, hands, and feet). B, Lateral view of a 26-year-old female with familial partial lipodystrophy of the Dunnigan variety due to heterozygous c.575A>T; p.(Asp192Val) mutation in the LMNA gene. She had
lipodystrophy 4289 sc fat in the face and chin. C, Anterior view of an 8-year-old German boy with acquired generalized lipodystrophy . He had severe generalized loss of sc fat with marked acanthosis nigricans in the neck, axillae, and
lipodystrophy 4484 neck, axillae, and groin. D, Anterior view of a 45-year-old Caucasian female with acquired partial lipodystrophy (Barraquer-Simons syndrome). She had marked loss of sc fat from the face, neck, upper extremities, and
lipodystrophy 4615 syndrome). She had marked loss of sc fat from the face, neck, upper extremities, and chest but had lipodystrophy on localized regions on the anterior thighs. She had increased sc fat deposition in the lower extremities.Table
lipodystrophy 6101 comorbidities (Table 2) that depend on the subtype, extent of fat loss, age, and gender. Many complications of lipodystrophy are secondary to deficient adipose mass, resulting in ectopic lipid storage in the liver, muscle, and
lipodystrophy 7559 disease; FSGS, focal segmental glomerulosclerosis. Many of these features are also found in other forms of lipodystrophy , including progeroid disorders.Major causes of mortality include heart disease (cardiomyopathy, heart
lipodystrophy 7909 carcinoma) ([6], [7]), kidney failure ([6]), acute pancreatitis ([7]), and sepsis.Due to the rarity of lipodystrophy syndromes, many clinicians are unfamiliar with their diagnosis and management. In December 2015, an
lipodystrophy 8433 in the Supplemental Data.Overview of Lipodystrophy SyndromesThis section reviews major categories of lipodystrophy . Details on individual subtypes are in Supplemental Table 2.Congenital generalized lipodystrophy (Berardinelli-Seip
lipodystrophy 8530 of lipodystrophy. Details on individual subtypes are in Supplemental Table 2.Congenital generalized lipodystrophy (Berardinelli-Seip syndrome)CGL is an autosomal recessive disorder characterized by near-complete lack
lipodystrophy 9042 complications are frequent and may be severe. Cardiomyopathy or rhythm disturbances may occur.Familial partial lipodystrophy FPLD is a group of usually autosomal dominant disorders characterized by loss of fat affecting the limbs,
lipodystrophy 9596 increased risk of coronary heart disease ([15]) and occasionally early cardiomyopathy.Acquired generalized lipodystrophy (Lawrence syndrome)AGL is more common in females (females:males, 3:1) and appears usually before adolescence
lipodystrophy 10042 frequent and may be severe. AGL is often associated with autoimmune diseases ([4], [16]).Acquired partial lipodystrophy (Barraquer-Simons syndrome)APL is more frequent in females (females:males, 4:1) and usually begins in
lipodystrophy 10680 nephritic factor. Metabolic complications are uncommon ([17]).Diagnosis of LipodystrophyDiagnosis of lipodystrophy is based on history, physical examination, body composition, and metabolic status. (Class I, Level B)There
lipodystrophy 10880 (Class I, Level B)There are no defined serum leptin levels that establish or rule out the diagnosis of lipodystrophy . (Class IIa, Level C)Confirmatory genetic testing is helpful in suspected familial lipodystrophies.
lipodystrophy 11173 members. (Class IIa, Level C)Serum complement levels and autoantibodies may support diagnosis of acquired lipodystrophy syndromes. (Class IIa, Level B)Firm diagnostic criteria for lipodystrophy have not been established.
lipodystrophy 11247 support diagnosis of acquired lipodystrophy syndromes. (Class IIa, Level B)Firm diagnostic criteria for lipodystrophy have not been established. Figure 2 shows a suggested diagnostic approach.Figure 2.Diagnostic approach
lipodystrophy 11367 not been established. Figure 2 shows a suggested diagnostic approach.Figure 2.Diagnostic approach to lipodystrophy syndromes. Lipodystrophy should be suspected in patients with regional or generalized lack of adipose
lipodystrophy 13013 shoulders, arms, and upper trunk, and most patients have low serum C3 levels.Establishing the presence of lipodystrophy Lipodystrophy should be suspected in patients with regional or generalized lack of adipose tissue outside
lipodystrophy 13395 (Supplemental Table 3) ([18]). Recognizing the loss of sc fat is particularly challenging in partial lipodystrophy and especially in men, in whom low body fat overlaps with normal variation and metabolic manifestations
lipodystrophy 13516 especially in men, in whom low body fat overlaps with normal variation and metabolic manifestations of lipodystrophy are less severe. In both genetic and acquired lipodystrophies, the loss of fat may be gradual, delaying
lipodystrophy 13719 gradual, delaying diagnosis.Physical, historical, and comorbid features that increase the suspicion of lipodystrophy ([18]) are shown in Table 3.Table 3.Clinical Features That Increase the Suspicion of LipodystrophyEssential
lipodystrophy 15143 Ref. [18].Because serum leptin assays are not standardized and leptin concentrations in patients with lipodystrophy (especially partial forms) overlap the general population, leptin levels do not help in diagnosis but
lipodystrophy 15616 cancer cachexia, HIV-associated wasting, chronic infections). Especially difficult is differentiating lipodystrophy from uncontrolled diabetes because both may have extreme hypertriglyceridemia. However, restoring glycemic
lipodystrophy 16042 Cushing's syndrome, truncal obesity, and multiple symmetric lipomatosis.Establishing the subtype of lipodystrophy Pattern of fat lossAlthough the pattern of body fat loss in patients with a particular subtype of genetic
lipodystrophy 16161 lipodystrophyPattern of fat lossAlthough the pattern of body fat loss in patients with a particular subtype of genetic lipodystrophy is quite characteristic, heterogeneity occurs in the onset, severity, and pattern of fat loss, even
lipodystrophy 16328 onset, severity, and pattern of fat loss, even within families.Distinguishing genetic from acquired lipodystrophy Pedigree analysis can suggest genetic vs acquired lipodystrophy. Review of photographs from infancy may
lipodystrophy 16391 families.Distinguishing genetic from acquired lipodystrophyPedigree analysis can suggest genetic vs acquired lipodystrophy . Review of photographs from infancy may distinguish CGL from AGL because infants typically show absent
lipodystrophy 16723 months of life ([4]). Patients with AGL lack family history but can be confused with any type of genetic lipodystrophy , especially de novo mutations.The presence of autoimmune diseases (myositis, type 1 diabetes, autoimmune
lipodystrophy 16937 autoimmune hepatitis, and others) ([4], [10], [16], [17], [19], [20]) increases the suspicion of acquired lipodystrophy . In APL, low serum C3, C3 nephritic factor, proteinuria, or biopsy-proven MPGN support the diagnosis.Genetic
lipodystrophy 17295 The website www.genetests.org lists clinical and research laboratories conducting genetic testing for lipodystrophy syndromes. Because there is strong evidence for additional loci for genetic lipodystrophies, negative
lipodystrophy 17778 premarital counseling with genetic testing to detect carrier status can be considered.Clinical diagnosis of lipodystrophy may be difficult in men ([21]), and some genotypes are associated with mild lipodystrophy phenotypes
lipodystrophy 17868 diagnosis of lipodystrophy may be difficult in men ([21]), and some genotypes are associated with mild lipodystrophy phenotypes ([22], [23]). Genetic screening of family members may help identify individuals with subtle
lipodystrophy 18450 clinical judgment should guide follow-up screening. Screening for comorbidities specific to individual lipodystrophy subtypes is not extensively discussed here.Diabetes mellitusDiabetes screening should be performed annually.
lipodystrophy 20224 adrenarche, true precocious puberty, or central hypogonadism may occur in children with generalized lipodystrophy . Oligo/amenorrhea, decreased fertility, and PCOS are common in women.Cardiac diseaseBlood pressure should
lipodystrophy 21640 not been established but would reasonably include annual skin and lymph node examination. Generalized lipodystrophy has been reported as a paraneoplastic manifestation of pilocytic astrocytoma in three children who regained
lipodystrophy 22143 2).Treatment of Lipodystrophy SyndromesCurrent therapies prevent or ameliorate the comorbidities of lipodystrophy syndromes. There is no cure for lipodystrophy and no treatment that can regrow adipose tissue.DietMost
lipodystrophy 22189 therapies prevent or ameliorate the comorbidities of lipodystrophy syndromes. There is no cure for lipodystrophy and no treatment that can regrow adipose tissue.DietMost patients should follow diets with balanced
lipodystrophy 22885 triglycerides in infants. (Class IIa, Level C)The cornerstone of therapy for metabolic complications of lipodystrophy is diet. Studies of specific diets in lipodystrophy are lacking, and recommendations rely on sparse
lipodystrophy 22937 cornerstone of therapy for metabolic complications of lipodystrophy is diet. Studies of specific diets in lipodystrophy are lacking, and recommendations rely on sparse literature and clinical experience.Patients with lipodystrophy,
lipodystrophy 23048 lipodystrophy are lacking, and recommendations rely on sparse literature and clinical experience.Patients with lipodystrophy , especially generalized forms, are typically hyperphagic due to leptin deficiency. Energy-restricted
lipodystrophy 24321 pancreatitis, bowel rest followed by a very-low-fat (<20 g) diet should be used.ExercisePatients with lipodystrophy should be encouraged to exercise in the absence of specific contraindications. (Class IIa, Level C)Patients
lipodystrophy 24460 exercise in the absence of specific contraindications. (Class IIa, Level C)Patients with subtypes of lipodystrophy predisposed to cardiomyopathy should undergo cardiac evaluation before initiating an exercise regimen.
lipodystrophy 24614 undergo cardiac evaluation before initiating an exercise regimen. (Class III, Level C)Individuals with lipodystrophy engaged in intense exercise have amelioration of metabolic complications. Most patients should be encouraged
lipodystrophy 24984 patients with severe hepatosplenomegaly and CGL patients with lytic bone lesions.MetreleptinIn generalized lipodystrophy , metreleptin (with diet) is a first-line treatment for metabolic and endocrine abnormalities (Class
lipodystrophy 25292 IIb, Level C)Metreleptin may be considered for hypoleptinemic (leptin <4 ng/mL) patients with partial lipodystrophy and severe metabolic derangements (HbA1c >8% and/or triglycerides >500 mg/dL). (Class IIb, Level B)Currently,
lipodystrophy 25508 B)Currently, metreleptin (recombinant human methionyl leptin) is the only drug approved specifically for lipodystrophy . It is approved in the United States as an adjunct to diet for treatment of metabolic complications
lipodystrophy 25651 States as an adjunct to diet for treatment of metabolic complications in patients with generalized lipodystrophy (http://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm387060.htm). In Japan, it is approved
lipodystrophy 25798 (http://www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/ucm387060.htm). In Japan, it is approved for both generalized and partial lipodystrophy (http://www.shionogi.co.jp/en/company/news/2013/pmrltj0000000ufd-att/e_130325.pdf). It is available
lipodystrophy 26337 change, with clinical and laboratory assessment performed every 3–6 months.Metreleptin in generalized lipodystrophy Metreleptin decreases hyperphagia ([41][42][45]), frequently leading to weight loss. Reduced food intake
lipodystrophy 28182 ovarian morphology ([45], [53], [55]). In males, metreleptin increased T ([45]).Metreleptin in partial lipodystrophy The response to metreleptin in partial lipodystrophy is less robust than in generalized lipodystrophy.
lipodystrophy 28234 metreleptin increased T ([45]).Metreleptin in partial lipodystrophyThe response to metreleptin in partial lipodystrophy is less robust than in generalized lipodystrophy. In one study, metreleptin reduced hypertriglyceridemia
lipodystrophy 28283 lipodystrophyThe response to metreleptin in partial lipodystrophy is less robust than in generalized lipodystrophy . In one study, metreleptin reduced hypertriglyceridemia and improved glycemia in severely hypoleptinemic
lipodystrophy 28424 reduced hypertriglyceridemia and improved glycemia in severely hypoleptinemic patients with partial lipodystrophy and severe metabolic derangements (baseline HbA1c >8%, triglycerides >500 mg/dL, leptin <4 ng/mL) ([46]).
lipodystrophy 28884 patients with serum leptin <7 ng/mL ([57]). Metreleptin is only available to patients with partial lipodystrophy through clinical trials, compassionate use programs, and in Japan.Side effects of metreleptinApproximately
lipodystrophy 29492 serious adverse events occurring during metreleptin treatment are likely related to the underlying lipodystrophy syndrome, rather than metreleptin. These include T-cell lymphoma in patients with AGL ([35]), pancreatitis
lipodystrophy 30024 be required. (Class IIa, Level C)Thiazolidinediones may improve metabolic complications in partial lipodystrophy but should only be used with caution in generalized lipodystrophy. (Class IIb, Level B)Among the oral
lipodystrophy 30090 metabolic complications in partial lipodystrophy but should only be used with caution in generalized lipodystrophy . (Class IIb, Level B)Among the oral hypoglycemic agents, metformin is used most frequently. In patients
lipodystrophy 30221 Level B)Among the oral hypoglycemic agents, metformin is used most frequently. In patients with partial lipodystrophy , thiazolidinediones improved HbA1c, triglycerides, hepatic volume, and steatosis but may increase regional
lipodystrophy 30671 areas because their long duration of action requires sc fat ([63], [64]). Patients with generalized lipodystrophy may have to take insulin by im routes for the lack of sc fat. Many other hypoglycemic agents have been
lipodystrophy 30796 take insulin by im routes for the lack of sc fat. Many other hypoglycemic agents have been used in lipodystrophy , but their efficacy has not been studied.DyslipidemiaStatins should be used concomitantly with lifestyle
lipodystrophy 32155 repeated frequently ([69]). Additional lipid-lowering drugs have not been studied in patients with lipodystrophy .HypertensionAngiotensin-converting enzyme inhibitors or angiotensin receptor blockers are first-line
lipodystrophy 32665 with FPLD in a double-blind, placebo-controlled crossover study ([71]). In NAFLD not associated with lipodystrophy , diet and exercise are first-line treatments ([72]), and among pharmacological treatments, vitamin E
lipodystrophy 32990 benefit for liver histopathology. However, these treatments have not been studied in patients with lipodystrophy and are not approved for NAFLD.Cosmetic treatmentPatients should be assessed for distress related to
lipodystrophy 33105 and are not approved for NAFLD.Cosmetic treatmentPatients should be assessed for distress related to lipodystrophy and referred as necessary to mental health professionals and/or plastic surgeons. (Class IIa, Level
lipodystrophy 33257 health professionals and/or plastic surgeons. (Class IIa, Level C)Changes in physical appearance from lipodystrophy can cause psychological distress and physical discomfort (eg, from absent fat pads in feet or buttocks).
lipodystrophy 34201 needed, transdermal estrogen should be used. (Class IIa, Level C)Oral estrogens are contraindicated in lipodystrophy syndromes due to the risk of severe hypertriglyceridemia and acute pancreatitis. Transdermal estrogens
lipodystrophy 34630 care from an obstetrician experienced in managing diabetes and a physician experienced in managing lipodystrophy . (Class IIa, level C)Should a patient become pregnant while taking metreleptin, clinicians may consider
lipodystrophy 34981 pregnancy are unknown (FDA category C), and wishes to continue. (Class IIc, level C)In patients with lipodystrophy with extreme insulin resistance, worsening insulin resistance during pregnancy may make diabetes management
lipomatosis 16002 or acromegaly/gigantism, and FPLD with Cushing's syndrome, truncal obesity, and multiple symmetric lipomatosis .Establishing the subtype of lipodystrophyPattern of fat lossAlthough the pattern of body fat loss in
multiple symmetric lipomatosis 15983 the insulin receptor or acromegaly/gigantism, and FPLD with Cushing's syndrome, truncal obesity, and multiple symmetric lipomatosis .Establishing the subtype of lipodystrophyPattern of fat lossAlthough the pattern of body fat loss in
obesity 15970 mutations of the insulin receptor or acromegaly/gigantism, and FPLD with Cushing's syndrome, truncal obesity , and multiple symmetric lipomatosis.Establishing the subtype of lipodystrophyPattern of fat lossAlthough
thyrotoxicosis 15468 presenting with severe weight loss (malnutrition, anorexia nervosa, uncontrolled diabetes mellitus, thyrotoxicosis , adrenocortical insufficiency, cancer cachexia, HIV-associated wasting, chronic infections). Especially

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