PMC:6636912 / 7970-12801 JSONTXT

{"project":"2_test","denotations":[{"id":"31232947-10868861-68672924","span":{"begin":102,"end":103},"obj":"10868861"},{"id":"31232947-17130187-68672925","span":{"begin":241,"end":242},"obj":"17130187"},{"id":"31232947-26071380-68672925","span":{"begin":241,"end":242},"obj":"26071380"},{"id":"31232947-29600506-68672925","span":{"begin":241,"end":242},"obj":"29600506"},{"id":"31232947-28668376-68672926","span":{"begin":919,"end":921},"obj":"28668376"},{"id":"31232947-30280274-68672927","span":{"begin":922,"end":924},"obj":"30280274"},{"id":"31232947-3543673-68672928","span":{"begin":1078,"end":1079},"obj":"3543673"},{"id":"31232947-26530207-68672929","span":{"begin":1125,"end":1126},"obj":"26530207"},{"id":"31232947-26071380-68672930","span":{"begin":1127,"end":1128},"obj":"26071380"},{"id":"31232947-26171144-68672931","span":{"begin":1129,"end":1131},"obj":"26171144"},{"id":"31232947-27657549-68672931","span":{"begin":1129,"end":1131},"obj":"27657549"},{"id":"31232947-22704172-68672931","span":{"begin":1129,"end":1131},"obj":"22704172"},{"id":"31232947-22611441-68672932","span":{"begin":1388,"end":1390},"obj":"22611441"},{"id":"31232947-20170930-68672933","span":{"begin":1654,"end":1656},"obj":"20170930"},{"id":"31232947-26372299-68672934","span":{"begin":1657,"end":1659},"obj":"26372299"},{"id":"31232947-30556656-68672935","span":{"begin":1876,"end":1878},"obj":"30556656"},{"id":"31232947-28668376-68672936","span":{"begin":2223,"end":2225},"obj":"28668376"},{"id":"31232947-26171144-68672937","span":{"begin":2710,"end":2712},"obj":"26171144"},{"id":"31232947-22704172-68672938","span":{"begin":2713,"end":2715},"obj":"22704172"},{"id":"31232947-28668376-68672939","span":{"begin":2942,"end":2944},"obj":"28668376"},{"id":"31232947-10868861-68672940","span":{"begin":3161,"end":3162},"obj":"10868861"},{"id":"31232947-26210987-68672941","span":{"begin":3447,"end":3449},"obj":"26210987"},{"id":"31232947-28668376-68672942","span":{"begin":3972,"end":3974},"obj":"28668376"},{"id":"31232947-26210987-68672943","span":{"begin":3975,"end":3977},"obj":"26210987"},{"id":"31232947-29490093-68672944","span":{"begin":4184,"end":4186},"obj":"29490093"}],"text":"3 Discussion\nThe distinction between type 1 and type 2 diabetes mellitus is usually straightforward.[7] However, as reported in this case, there may be an overlap in the presentations of the 2 disorders, which creates a diagnostic dilemma.[8–10] Questions raised as we review the clinic process of the young ketosis prone diabetes patients. How to classify type of diabetes for the young patient based on his initial presentation? What was the likely reason for his metabolic decompensation? The young patient presented with marked dehydration and uncontrolled hyperglycemia in the absence of any precipitating factor such as infection., along with diabetic acidosis and ketosis resulting from deficient insulin secretion, However, the phenotypic features of obesity, acanthosis nigricans, marked insulin resistance and strong family history of adult onset diabetes which are classically associated with classic T2DM.[11,12] All findings were consistent with atypical diabetes or ketosis-prone diabetes, which first systematically reported by Winters and his colleges in 1987.[5] KPDM is an emerging heterogeneous syndrome.[6,9,13–15]This syndrome of episodic diabetic ketoacidosis without immunologic markers of type 1 diabetes is characterized by insulin dependence at the time of presentation, but followed by absence of insulin requirements for years as observed in type 2 diabetes.[16] Because of the mixed features of type 1 and type 2 diabetes, this variant of diabetes has been referred to in the literature as diabetes type 1B, idiopathic type 1 diabetes, atypical diabetes, Flatbush diabetes, and more recently, ketosis-prone type 2 diabetes.[17,18]\nThe pathogenesis of this syndrome is unclear, even though the environmental factors, such as diet and physical activity, coupled with still largely unknown genetics factors clearly interact to produce the syndrome.[19] What causes the initial beta-cell insult leading to acute insulin deficiency? Glucose toxicity has been suggested as a contributing factor. Accumulating data suggest that severe glucotoxic blunting of an intracellular pathway leading to insulin secretion may contribute to the reversible beta cell dysfunction characteristic of KPDM patients.[20] One prospective study of patients presenting with DKA demonstrated a lower glucagon-mediated C-peptide response in obese patients with diabetic ketoacidosis compared with ketosis-resistant hyperglycemia patients, suggesting that there may be other causes and mechanisms involved. Prior studies by our group and other investigators indicate that most patients with ketosis-prone type 2 diabetes had a higher prevalence of a parental history of diabetes and were generally more obese.[13,15] Because of its association with obesity and hyperlipidemia as well as hyperglycemia, some investigators have examined whether high levels of free fatty acids or other lipids might be the trigger of unproved diabetic ketosis.[20] Is it possible that the ketosis-prone diabetes in Chinese patients represents an attack by disease sources factor such virus or bacteria? However, there is no evidence that those patients had an infectious illness.[7] Why are Asian more susceptible to glucose toxicity and lipotoxicity? Differences in the lifestyle, including the consumption of rice and wheat as staple food or gene-environment interactions may also have an impact on the incidence of KPDM and the findings call for further studies.[21]\nWhat is the best clinic treatment strategy and option? All patients with KPDM should be treated according to established principles of acute management of metabolic decompensation. Insulin replacement therapy is necessary at the acute state of insulin deficient and hyperglycemia crisis. Lifestyle changes, including optimal diet, weight loss, exercise and smoking cessation, are an important part of the treatment regimen to prevent obesity and improve insulin sensitivity, as well as to maintain good glycemic control.[20,21] Screening and treatment for microvascular and macrovascular complications of diabetes should be advised according to American Diabetes Association (ADA) recommendation and long term management guidelines.[22] In summary, the description is of a case of ketosis prone diabetes in an obese young Chinese teenager, who displayed uncommon clinic presentation and findings typical of both type 1 and type 2 diabetes. However, after initial insulinization near-normoglycaemic control and restoration of normotriglycemia rapidly lead to improvement in beta-cell function. The patient was able to come off insulin therapy without relapse of ketoacidosis as previous reported. His unprovoked DKA, negative auto-antibodies and partially preserved beta cell functional reserve after the acute of diabetic ketosis suggested that he has the phenotype of “A–β+” KPD."}