aetiology of fat necrosis is thought to result from local trauma and tissue injury caused by both physical and chemical insults, stimulating an inflammatory reaction within the adipose tissue (3) . Aseptic saponification of fat by lipases was proposed to be
P Hanson, M Pandit, V Menon, S Roberts, and T M Barber
Athanasios Fountas, Zoe Giotaki, Evangelia Dounousi, George Liapis, Alexandra Bargiota, Agathocles Tsatsoulis, and Stelios Tigas
Background Lipodystrophy is a rare group of clinically heterogeneous disorders characterized by the complete or partial loss of subcutaneous adipose tissue ( 1 , 2 ). This condition is often accompanied by metabolic complications such as
Aysenur Ozderya, Sule Temizkan, Kadriye Aydin Tezcan, Feyza Yener Ozturk, and Yuksel Altuntas
supraclavicular area ( Fig. 2 ). Biopsies from these areas were concordant with lipomatous tissue ( Fig. 3 ). An abdominal CT examination revealed an enlarged liver (cranio-caudal axis: 24 cm) and spleen. After a differential diagnosis of the causes of the
Baris Akinci, Rasimcan Meral, Diana Rus, Rita Hench, Adam H Neidert, Frank DiPaola, Maria Westerhoff, Simeon I Taylor, and Elif A Oral
Background Lipodystrophy syndromes are a heterogeneous cluster of diseases characterized by loss of subcutaneous adipose tissue that leads to severe insulin resistance and dyslipidemia. Metreleptin therapy is now an approved treatment for
Betty Korljan Jelaska, Sanja Baršić Ostojić, Nina Berović, and Višnja Kokić
Glycogen storage disease (GSD) type I is characterized by impaired production of glucose from glycogenolysis and gluconeogenesis resulting in severe hypoglycaemia and increased production of lactic acid, triglyceride and uric acid. The most common type, glycogenosis type Ia, demands a balanced, sufficient carbohydrate intake to preserve normal 24-h glycaemia. Insufficient intake of carbohydrates can cause hypoglycaemia, as the missing glucose-6-phosphatase enzyme cannot free the glucose stored as liver glycogen and nor is gluconeogenesis possible. The principle means of handling this disorder is to avoid starving by taking regular meals during the day and night. Such a dietary regimen could lead to obesity. Herein, we present the case of an adult patient with glycogenosis type Ia suffering from hyperuricaemia, dyslipidaemia and arterial hypertension. The accumulation of these cardiovascular risk factors could lead to the early onset of atherosclerosis, which should be postponed by contemporary methods of surveillance and treatment.
- Continuous subcutaneous glucose monitoring may be of value in every adult patient with GSD type I to evaluate the actual prevalence of eventual hypoglycaemic and hyperglycaemic episodes.
- Good dietary management minimizes the metabolic abnormalities of the disease and decreases the risk of long-term complications.
- Treatment of obesity in patients with GSD reduces the risk of earlier atherosclerosis and cardiovascular disease.
Albert S Kim, Rashida Hakeem, Azaliya Abdullah, Amanda J Hooper, Michel C Tchan, Thushari I Alahakoon, and Christian M Girgis
A 19-year-old female presented at 25-weeks gestation with pancreatitis. She was found to have significant hypertriglyceridaemia in context of an unconfirmed history of familial hypertriglyceridaemia. This was initially managed with fasting and insulin infusion and she was commenced on conventional interventions to lower triglycerides, including a fat-restricted diet, heparin, marine oil and gemfibrozil. Despite these measures, the triglyceride levels continued to increase as she progressed through the pregnancy, and it was postulated that she had an underlying lipoprotein lipase defect. Therefore, a multidisciplinary decision was made to commence therapeutic plasma exchange to prevent further episodes of pancreatitis. She underwent a total of 13 sessions of plasma exchange, and labour was induced at 37-weeks gestation in which a healthy female infant was delivered. There was a rapid and significant reduction in triglycerides in the 48 h post-delivery. Subsequent genetic testing of hypertriglyceridaemia genes revealed a missense mutation of the LPL gene. Fenofibrate and rosuvastatin was commenced to manage her hypertriglyceridaemia postpartum and the importance of preconception counselling for future pregnancies was discussed. Hormonal changes in pregnancy lead to an overall increase in plasma lipids to ensure adequate nutrient delivery to the fetus. These physiological changes become problematic, where a genetic abnormality in lipid metabolism exists and severe complications such as pancreatitis can arise. Available therapies for gestational hypertriglyceridaemia rely on augmentation of LPL activity. Where there is an underlying LPL defect, these therapies are ineffective and removal of triglyceride-rich lipoproteins via plasma exchange should be considered.
- Hormonal changes in pregnancy, mediated by progesterone,oestrogen and human placental lactogen, lead to a two- to three-fold increase in serum triglyceride levels.
- Pharmacological intervention for management of gestational hypertriglyceridaemia rely on the augmentation of lipoprotein lipase (LPL) activity to enhance catabolism of triglyceride-rich lipoproteins.
- Genetic mutations affecting the LPL gene can lead to severe hypertriglyceridaemia.
- Therapeutic plasma exchange (TPE) is an effective intervention for the management of severe gestational hypertriglyceridaemia and should be considered in cases where there is an underlying LPL defect.
- Preconception counselling and discussion regarding contraception is of paramount importance in women with familial hypertriglyceridaemia.
Sarah W Y Poon, Karen K Y Leung, and Joanna Y L Tung
of Clinical Lipidology 2018 12 243 – 245 . ( https://doi.org/10.1016/j.jacl.2017.10.018 ) 10.1016/j.jacl.2017.10.018 6 Näsström B Olivecrona G Olivecrona T Stegmayr BG . Lipoprotein lipase during continuous heparin infusion: tissue
Soledad Bell, Gabriela Alejandra Sosa, Ana del Valle Jaen, and María Fabiana Russo Picasso
Background Thyroid lipomatosis is a rare disease hither to described in only 20 patients. It is characterized by diffuse infiltration of mature adipose tissue in the thyroid gland, resulting in its progressive enlargement and potentially to
Peter Novodvorsky, Emma Walkinshaw, Waliur Rahman, Valerie Gordon, Karen Towse, Sarah Mitchell, Dinesh Selvarajah, Priya Madhuvrata, and Alia Munir
Bariatric surgery is an effective therapy for obesity but is associated with long-term complications such as dumping syndromes and nutritional deficiencies. We report a case of a 26-year-old caucasian female, with history of morbid obesity and gestational diabetes (GDM), who became pregnant 4 months after Roux-en-Y bypass surgery. She developed GDM during subsequent pregnancy, which was initially managed with metformin and insulin. Nocturnal hypoglycaemia causing sleep disturbance and daytime somnolence occured at 19 weeks of pregnancy (19/40). Treatment with rapid-acting carbohydrates precipitated further hypoglycaemia. Laboratory investigations confirmed hypoglycaemia at 2.2 mmol/L with appropriately low insulin and C-peptide, intact HPA axis and negative IgG insulin antibodies. The patient was seen regularly by the bariatric dietetic team but concerns about compliance persisted. A FreeStyle Libre system was used from 21/40 enabling the patient a real-time feedback of changes in interstitial glucose following high or low GI index food intake. The patient declined a trial of acarbose but consented to an intraveneous dextrose infusion overnight resulting in improvement but not complete abolishment of nocturnal hypoglycaemia. Hypoglycaemias subsided at 34/40 and metformin and insulin had to be re-introduced due to high post-prandial blood glucose readings. An emergency C-section was indicated at 35 + 1/40 and a small-for-gestational-age female was delivered. There have been no further episodes of hypoglycaemia following delivery. This case illustrates challenges in the management of pregnancy following bariatric surgery. To our knowledge, this is the first use of FreeStyle Libre in dumping syndrome in pregnancy following bariatric surgery with troublesome nocturnal hypoglycaemia.
- Bariatric surgery represents the most effective treatment modality in cases of severe obesity. With increasing prevalence of obesity, more people are likely to undergo bariatric procedures, many of which are women of childbearing age.
- Fertility generally improves after bariatric surgery due to weight reduction, but pregnancy is not recommended for at least 12–24 months after surgery. If pregnancy occurs, there are currently little evidence-based guidelines available on how to manage complications such as dumping syndromes or gestational diabetes (GDM) in women with history of bariatric surgery.
- Diagnosis of GDM relies on the use of a 75 g oral glucose tolerance test (OGTT). The use of this test in pregnant women is not recommended due to its potential to precipitate dumping syndrome. Capillary glucose monitoring profiles or continuous glucose monitoring (CGM) is being currently discussed as alternative testing modalities.
- As the CGM technology becomes more available, including the recently introduced FreeStyle Libre Flash glucose monitoring system, more pregnant women, including those after bariatric surgery, will have access to this technology. We suggest urgent development of guidelines regarding the use of CGM and flash glucose monitoring tools in these circumstances and in the interim recommend careful consideration of their use on a case-to-case basis.
Yasufumi Seki, Satoshi Morimoto, Naohiro Yoshida, Kanako Bokuda, Nobukazu Sasaki, Midori Yatabe, Junichi Yatabe, Daisuke Watanabe, Satoru Morita, Keisuke Hata, Tomoko Yamamoto, Yoji Nagashima, and Atsuhiro Ichihara
expression might represent the alteration of its function ( 8 ). Further study is needed to solve these limitations and suggest this hypothesis. Another possible contributor to aldosterone excess might be increased adipose tissue caused by hypogonadism. In