Clinical characteristics of fulminant Type 1 diabetes mellitus / 中南大学学报(医学版)

OBJECTIVES@#To compare the differences in clinical characteristics between Type 1 diabetes mellitus (T1DM) and fulminant Type 1 diabetes mellitus (FT1DM), and to reduce the missed diagnosis, misdiagnosis, and mistreatment of FT1DM by medical staff.@*METHODS@#A total of 101 hospitalized patients with T1DM (including 8 cases of FT1DM) were enrolled in this study from Changsha Central Hospital between June 2012 and December 2018. Clinical characteristics of the 8 FT1DM patients were collected and compared with all T1DM patients.@*RESULTS@#All FT1DM patients were adult with the average age of (30.25±5.28) years old, accompanied by severe diabetic ketoacidosis (DKA) occurred within 1 week after onset. Moreover, pancreatic beta cells in these patients were destroyed and the islet-related antibodies were negative, while the serum pancreatic enzyme levels were increased. Compared with classic T1DM patients, the plasma glucose levels in FT1DM patients were much higher [(41.89±12.54) mmol/L vs (22.57±9.74) mmol/L], but glycosylated hemoglobin (HbA1c) and fasting C peptide levels were significantly lower [(6.08±0.41)% vs (10.87±2.46%)%, @*CONCLUSIONS@#The onset time of FT1DM patients is very urgent via driving DKA. These patients have higher blood glucose concentration than classic T1DM patients, accompanied by electrolyte disturbances, impaired renal function, partially impaired liver function, as well as gastrointestinal symptoms and elevated trypsin. Most FTDM patients are adolescents and adults with no gender difference, especially pregnant women who are at high risk. Lifelong insulin dependence in FT1DM patients should be paid more attention in clinical treatment.

Classic Type of Kaposi's Sarcoma Showing Spontaneous Regression / 대한피부과학회지

Self-regression of Kaposi's sarcoma is rare with the exception of the iatrogenic immunosuppressive type. No cases of classic type Kaposi's sarcoma showing spontaneous regression have been reported in the Korean dermatologic literature. We report a case of classic type of Kaposi's sarcoma in a 69-year-old man which followed a clinical course of spontaneous regression. He presented with multiple asymptomatic beefy red papules and nodules scattered on both cheeks and upper extremities during a state of increase in the number of skin lesions at the initial visit. He had no history of immunosuppressive treatment and the laboratory findings were unremarkable. An anti-HIV antibody test was negative. A histopathologic examination revealed typical findings of Kaposi's sarcoma and positive staining with CD31 and D2-40. HHV-8 DNA was detected in a biopsy specimen. Without any specific treatment, the skin lesions healed without leaving any complications after 15 months, and did not show any evidence of recurrence during the ensuing 5 years.

A Case of Maple Syrup Urine Disease

Maple syrup urine disease (MSUD) is an autosomal recessive disease caused by a deficiency in subunits of the branched-chain alpha-ketoacid dehydrogenase complex (BCKDH). The disease is characterized by the accumulation of the branched-chain amino acids leucine, isoleucine, valine, alloisoleucine, and their corresponding alpha-ketoacid in blood and urine. MSUD is a heterogenous disorder, and classic, intermittent, intermediate and thiamine-responsive phenotypes have been identified. We experienced a case of Maple syrup urine disease (classic type) in a female neonate, who suffered from lethargy, poor feeding, apnea, alternating periods of hypertonicity and flaccidity, generalized convulsions, and a peculiar burned sugar smell from the body and urine. She died of respiratory failure 22 days after the birth. The brief review of the literature was made.