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Current Diagnosis, Treatment and Clinical Challenges in the Management of Lipodystrophy Syndromes in Children and Young People

ABSTRACT: Lipodystrophy is a heterogeneous group of disorders characterized by lack of body fat in characteristic patterns, which can be genetic or acquired. Lipodystrophy is associated with insulin resistance that can develop in childhood and adolescence, and usually leads to severe metabolic complications. Diabetes mellitus, hypertriglyceridemia, and hepatic steatosis ordinarily develop in these patients, and most girls suffer from menstrual abnormalities. Severe complications develop at a relatively young age, which include episodes of acute pancreatitis, renal failure, cirrhosis, and complex cardiovascular diseases, and all of these are associated with serious morbidity. Treatment of lipodystrophy consists of medical nutritional therapy, exercise, and the use of anti-hyperglycemic and lipid-lowering agents. New treatment modalities, such as metreleptin replacement, promise much in the treatment of metabolic abnormalities secondary to lipodystrophy. Current challenges in the management of lipodystrophy in children and adolescents include, but are not limited to: (1) establishing specialized centers with experience in providing care for lipodystrophy presenting in childhood and adolescence; (2) optimizing algorithms that can provide some guidance for the use of standard and novel therapies to ensure adequate metabolic control and to prevent complications; (3) educating patients and their parents about lipodystrophy management; (4) improving patient adherence to chronic therapies; (5) reducing barriers to access to novel treatments; and (5) improving the quality of life of these patients and their families.

SUBMITTER: Ozen S

PROVIDER: S-EPMC7127888 | biostudies-literature | 2020 Mar

REPOSITORIES: biostudies-literature

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Current Diagnosis, Treatment and Clinical Challenges in the Management of Lipodystrophy Syndromes in Children and Young People

Özen Samim S Akıncı Barış B Oral Elif A. EA

Journal of clinical research in pediatric endocrinology 20190822 1

Lipodystrophy is a heterogeneous group of disorders characterized by lack of body fat in characteristic patterns, which can be genetic or acquired. Lipodystrophy is associated with insulin resistance that can develop in childhood and adolescence, and usually leads to severe metabolic complications. Diabetes mellitus, hypertriglyceridemia, and hepatic steatosis ordinarily develop in these patients, and most girls suffer from menstrual abnormalities. Severe complications develop at a relatively yo ...[more]

PMID: 31434462

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Project description:ObjectiveThe objective of this study is to summarise the contemporary evidence regarding the prevalence, diagnosis, and management of osteitis pubis (OP) specially from urological point of view, while proposing an algorithm for the best management based on the current evidence.MethodsWe performed a literature search using the PubMed database for the term 'osteitis pubis' until December 2020. We assessed pre-clinical and clinical studies regarding the aetiology, pathophysiology, and management of OP. Case reports and case series were evaluated by study quality and patient outcomes to determine a potential clinical management algorithm.ResultsOsteitis pubis is a chronic painful condition of the symphysis pubis joint and its surrounding structures. Still, there is a paucity of data outlining the management plan and the possible triggers. The aetiology seems to be multifactorial with different proposals trying to explain the pathophysiology and correlate the findings to the outcome. The diagnosis is usually based on high suspicion index and clinical experience. The infective variant of the disease is aggressive and requires strict and active management. Universal consensus is still lacking regarding a formal algorithm of management of the condition, especially due to multiple specialities involved in the decision-making process. Conservative management remains the cornerstone; nevertheless, surgical interventions may be needed in special settings. Hence, a multi-disciplinary approach is of pivotal value in fashioning the plan for each case. The prognosis is usually satisfactory; however, a longstanding debilitating disease form is not uncommon.ConclusionOP remains a rare condition with real challenges in its diagnosis. The current management is focused on conservative management; however, surgical intervention is still needed in some difficult scenarios. Continued research into the triggers of OP, multidisciplinary approach, and standardised clinical pathways can improve the quality of care for patients suffering from this condition.

Dataset Information

Current Diagnosis, Treatment and Clinical Challenges in the Management of Lipodystrophy Syndromes in Children and Young People

Publications

Current Diagnosis, Treatment and Clinical Challenges in the Management of Lipodystrophy Syndromes in Children and Young People

Similar Datasets

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