Long term follow-up and efficacy of continuous subcutaneous insulin infusion in the treatment of children with type 1 diabetes mellitus in Qingdao / 中华实用儿科临床杂志

Objective:To study the effect of continuous subcutaneous insulin infusion (CSII) in the long-term treatment of type 1 diabetes mellitus (T1DM) children, to analyze the factors influencing the efficacy, and to provide theoretical guidance for the application of CSII in long-term therapy and follow-up management.Method:A total of 60 T1DM children who were under 18 years old, lived in Qingdao for a long time, had CSII for more than 6 months, and visited the Outpatient Department of Endocrinology of Qingdao Women and Children′s Hospital for a long term from January 2019 to June 2019 were followed up with questionnaires to understand their general condition and treatment-related data.Result:After the CSII therapy, the hemoglobin A1c (HbA1c) of T1DM children significantly decreased from (9.58±2.08)% to (7.12±1.11)% ( t=7.315, P<0.05), the daily insulin dosage per unit weight significantly declined from 0.91(0.86, 0.94) IU to 0.80 (0.66, 0.88) IU ( Z=-5.514, P<0.05), and the frequency of both hypoglycemia and ketoacidosis was significantly reduced.Diet control, daily exercise time, the visit frequency and the self-monitoring frequency of blood glucose affected the curative effect of CSII therapy (all P<0.05). Conclusion:CSII therapy can effectively control the blood glucose of children with T1DM in Qingdao area, significantly lower HbA1c, and reduce the insulin dosage and the incidence of hypoglycemia and ketoacidosis.Good diet control, regular exercise, regular follow-up and a high frequency of blood glucose monitoring are associated with better glycemic control.

Vomiting, weakness and glyceroluria after eating dessert / 中华实用儿科临床杂志

During 2 years,a 6-year-old girl was hospitalized for 2 times with recurrent onset of episodes of vomiting,weakness and fever after eating dessert at the Department of Neurology & Endocrine Pediatrics,the Affiliated Hospital of Qingdao University.The arterial blood gas analysis revealed severe hypoglycemia,lacticacidemia and metabolic acidosis,the urine ketone body was positive.After intravenous infusion of glucose,bicarbonate and antibiotics,there was a dramatic clinical improvement in a short time.Physical examination showed tachypnea and mild hepatomegaly,and she had normal physical and mental development.The laboratory findings revealed transient hyperuricacidemia.Urine organic acids analysis repeatedly showed an elevation of lactic acid,ketone and glycerol.Glyceroluria was a very distinctive trait.The literatures in PubMed was searched with glyceroluria as keyword.Three related diseases were identified:FBPase deficiency,glycerol kinase (GK) deficiency and complex GK deficiency.Further reading of related literatures to understand the characteristics of diseases and laboratory tests,the clinical diagnosis of GK deficiency and complex GK deficiency was excluded.The mutation analysis of FBPase gene (FBP1) was performed by Sanger sequencing and a novel compound heterozygous mutations of c.355G ＞A and c.960delG was discovered.Full analysis of disease-related traits and targeted gene testing is one of the effective methods for accurate diagnosis and treatment of inherited metabolic disorders.

Advances in familial glucocorticoid deficiency / 国际儿科学杂志

Familial glucocorticoid deficiency (FGD) known as one of primary congenital adrenal hypoplasia diseases,is a rare autosomal recessive disorder.FGD is characterised by isolated glucocorticoid deficiency,therefore the patients exhibit low serum cortisol and high plasma adrenocorticotropic hormone levels.The patients typically present with hypoglycemia,recurrent infections,hyperpigrnentation and tall stature.Much research on the pathogenic genes and molecular biology mechanisms have been performed,and presently some pathogenic genes have been discovered.In order to enhance the clinicians'understanding of familial glucocorticoid deficiency,this review focuses on the pathogenic genes,pathogenesis,diagnosis and treatments of the disease.

Quantitative analysis for individual baseline drift for immunoglobulin and complement within 24 h / 中国免疫学杂志

Objective:To analyze quantitatively individual baseline drift of immunoglobulin and complement within 24 h.Methods:Blood samples were drawn from adult volunteers on day 1 and day 2 at 7.00 am.Immunoglobulin and complement including IgG,IgA,IgM and C3,C4 were measured with automatic immunobiochemistry analyzer.The rate of changes between two times of measured value,baseline drift,could be calculated.Results:The individual baseline drifts of measured values were from 6.12% to 7.97% (mean=7.22%) in 5 immunological parameters.There were significations between two groups within 5 immunological parameters (P