The main points of diagnosis and treatment of "Fulminant Type 1 Diabetes"

Author: Sun Jingxia, People's Hospital of Guangxi Zhuang Autonomous Region This article is published with the authorization of the author, please do not reprint without permission
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Today, I would like to share with you a rare case in clinical practice - fulminant type 1 diabetes
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What is the difference between the diagnosis and treatment of fulminant type 1 diabetes and ordinary type 1 diabetes? Through the case and related literature, let's review and learn together
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 The main complaints of case sharing: dry mouth, polydipsia, and polyuria for 9 months, and numbness in the limbs for more than 2 months
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History of present illness: The patient had sudden dry mouth, polydipsia, polyuria, no polyphagia, no weight loss without obvious incentives before September.
After drug treatment (specific unknown), drowsiness, unconsciousness, no smell of rotten apple, ignoring There was no nausea, vomiting, abdominal distension, diarrhea, palpitations, chest tightness, or chest pain.
Then, he went to the emergency department of another hospital to see "glycated hemoglobin 7.
5%, glucose 55.
93 mmol/L, blood gas PH 7.
34, lactic acid 0.
7 mmol/L", Considering "diabetic ketoacidosis", the symptoms improved after treatment (specifically unknown), and after discharge from the hospital, "Novo Ruite Charge 16U-16U-16U + Changxiulin 26U/before going to bed" was treated
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On June 1, 2021, he was seen in our hospital.
Fasting blood sugar: 9.
65mmol/L↑, 2-hour postprandial blood sugar: 13.
78mmol/L↑, β-hydroxybutyric acid: 0.
40mmol/L↑, PH 7.
4, fasting C-peptide , insulin and 2 hours postprandial C-peptide, low insulin, positive diabetic autoantibodies (GAD 17.
86U/ml), high lipase, diagnosed with fulminant type 1 diabetes, type 1 diabetic ketosis, and admitted to the hospital for intensive insulin pump therapy , was discharged to Humalog 6/14/2U + Lantus 33U/bedtime hypoglycemic therapy, and gradually changed the hypoglycemic regimen to Novoray 6/6/6U + insulin glargine 26U after discharge
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He developed numbness in his limbs more than 2 months ago, and complained that the fasting blood sugar and postprandial blood sugar fluctuated within 10mmol/L recently.
He is now admitted to our department for further treatment
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Since the onset of the disease, the patient has been in poor spirits, poor appetite, poor sleep, normal bowel movements, and has lost about 8kg in weight in the past September
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Past History: Surgery for appendicitis 20 years ago
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Personal History: None special
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Marital History: Married, healthy spouse, 2 children and 1 daughter, healthy children
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Family History: There is a family history of diabetes, the eldest sister had diabetes
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Physical examination: T: 36.
4℃, P: 82 times/min, R: 20 times/min, BP: 123/77mmHg, weight 76.
4kg, height 179.
5cm, BMI 23.
71kg/m^2
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There was no abnormality in the heart, lungs and abdomen, and no abnormality was found in the nervous system examination.
The lower extremities were not swollen, and the pulse of the dorsal artery of the foot was normal
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Auxiliary examination Hematuria and stool routine: biochemistry, liver function: blood lipids: cardiac function enzymes: pancreas two items: glycosylated hemoglobin: 12.
0% ↑ lactic acid, β-hydroxybutyric acid: pancreatic islet function: insulin antibodies and diabetes autoantibodies: diabetic nephropathy screening: Doppler blood flow map of limbs: the ABI index of the right lower extremity was 1.
08 for the posterior tibial artery and 1.
15 for the dorsal foot artery, and the ABI index for the left lower limb was 1.
08 for the posterior tibial artery and 1.
04 for the dorsal artery of the foot, indicating normal blood supply to both lower extremities; vibration sensory threshold check- Foot sensory nerves: shallow sensation is normal; fundus photography: normal fundus; vascular ultrasound: no abnormality in carotid artery and lower extremity artery; diagnosis 1.
Fulminant type 1 diabetes (main diagnosis); 2.
1 type diabetic nephropathy (G1A2 stage); 3.
Type 1 diabetic peripheral neuropathy; 4.
Hyperlipidemia
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Diagnosis is based on 1.
The patient is middle-aged male, with acute onset of chronic disease and repeated disease; 2.
The onset is dry mouth, polydipsia, and polyuria, and the glycosylated hemoglobin (7.
5%) and blood sugar (55.
93 mmol/L) are elevated in the initial course of the disease.
Glycated hemoglobin does not match the degree of blood glucose elevation; fasting C-peptide/insulin and C-peptide/insulin 2 hours postprandial decreased; rapid progression to "diabetic ketoacidosis", positive diabetes autoantibodies (GAD 17.
86U/ml), past She has received intensive insulin therapy and is currently taking NovoRapid 6/6/6U + insulin glargine 26U/bedtime hypoglycemic therapy; 3.
The patient's previous creatinine: 88.
26umol/L, eGFR 95.
26, urine albumin: creatinine: 92.
25mg/ gCr↑, the diagnosis of diabetic nephropathy (G1A2 stage); 4.
The patient's sensory threshold: the patient's superficial sensation (small fiber neuropathy) is impaired, the diagnosis of diabetic peripheral neuropathy; ; No obvious target organ damage was found in the heart, blood vessels, nerves, and fundus
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Treatment plan ➤ Hypoglycemic: Lantus 18U at 7:00 am + Humalog 6U/6U/6U subcutaneous injection of voglibose tablets (200ug) before three meals, orally three times a day ➤Lipid regulation: Atorvastatin calcium tablets ( 20mg) orally every night ➤ Nutritional nerves: Epalrestat tablets (50mg) Oral mecobalamin tablets (0.
5mg) three times a day Summary: 1.
The patient is a middle-aged male, with a slender body, acute exacerbation of chronic disease, The first onset was DKA.
At that time, the glycosylated hemoglobin was 7.
5% and the glucose was 55.
93 mmol/L.
Fulminant type 1 diabetes was considered.
After admission, the hypoglycemic plan was adjusted to insulin glargine injection (Lantase) (12iu) at 7:00 am + lispro Insulin refills (Hummalog) (7U before breakfast, 6U before lunch, 6U before dinner), the patient's blood sugar is still acceptable
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2.
The patient is currently diagnosed with fulminant type 1 diabetes mellitus, pancreas binomial, muscle enzymes, cardiac function enzymes, and transaminase are generally normal, and no obvious target organ damage is found in the heart, blood vessels, nerves, and fundus
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3.
According to the 2019 WHO diabetes diagnostic criteria, fulminant type 1 diabetes is classified as idiopathic type 1 diabetes
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Compared with classic type 1 diabetes, the disease has its unique clinical features: rapid onset, critical condition, and pancreatic islet failure
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4.
The treatment principle of fulminant type 1 diabetes is the same as that of type 1 diabetes; insulin treatment is required, and the amount of insulin is large and the blood sugar is brittle.
It is necessary to pay close attention to blood sugar and avoid hypoglycemia
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5.
Patients with fulminant type 1 diabetes may suffer from multiple organ damage such as liver, kidney, heart, muscle, etc.
due to the rapid loss of pancreatic β-cell function and related metabolic disorders.
Regular review of biochemistry, electrolytes, creatine kinase,
etc.

Microvascular complications of type 1 diabetes appear earlier than those of classic type 1 diabetes, and may be accompanied by elevated pancreatic enzymes, but there are no changes in pancreatic imaging, which should be paid close attention
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Knowledge sharing Fulminant type 1 diabetes mellitus (FT1DM) is an acute onset of T1DM, first proposed by Japanese scholars
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According to the 2019 WHO diabetes classification criteria, it is classified as idiopathic type 1 diabetes; the reports are mainly seen in East Asian populations: accounting for about 20% of acute T1DM in Japan, about 7% of acute T1DM in Korea, and rare in people of European descent
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 1.
The main clinical features of FT1DM include: ➤ sudden onset; ➤ very short duration of hyperglycemia symptoms (usually less than 1 week); ➤ almost no C-peptide secretion at diagnosis; ➤ ketoacidosis at diagnosis; ➤ most islets Negative for related autoantibodies; ➤ Elevated serum pancreatin levels; ➤ Frequent flu-like symptoms and gastrointestinal symptoms before disease onset
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 2.
Clinical diagnosis can be made if the following criteria are met: 1.
It is found that the blood sugar rises for 1 week and then progresses to ketoacidosis; 2.
The blood sugar at the initial diagnosis is ≥ 16.
0 mmol/l, HbA1c < 8.
5%; 3.
Fasting serum C-peptide < 0.
10 nmol/L (0.
3ng/mL), the peak serum C-peptide after glucagon stimulation or after eating is less than 0.
17nmol/L (0.
5 ng/mL)
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Other manifestations of the disease: ➤ Islet antibodies are often negative; ➤ 98% of patients have elevated blood amylase, and no abnormal pancreatic ultrasound; ➤ 70% have flu-like symptoms and gastrointestinal prodromal symptoms; ➤ Often during pregnancy or childbirth 3.
The treatment principle of FT1DM is the same as that of type 1 diabetes.
If there is ketoacidosis, intensive insulin therapy should be given after the ketoacidosis has been corrected.
Sudden loss of function, combined with abnormal pancreatic exocrine secretion and functional damage of other organs, the dynamic changes of blood biochemical indicators should be closely monitored; ➤ It is difficult to control blood sugar in FT1DM, and the risk of diabetic microvascular complications is higher than that of autoimmune type 1 diabetes
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Therefore, it is necessary to do a good job in diabetes education for FT1DM clinically, try to control blood sugar to meet the standard, and delay the occurrence of complications
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 References: [1] Nat Clin Pract Endocrinol Metab.
2007; 3: 36–45.
NEJM.
2000; 342: 301–7.
[2] WHO2019: Classification of diabetes [3] J Diabetes Investig.
2012; 3(6) :536-9[4] Diabetologia.
2007;50(3):531-7