Congenital hyperinsulinism and surgical outcome in a single tertiary center in Brazil.

OBJECTIVE: Congenital hyperinsulinism (CHI) is a heterogeneous genetic disease characterized by increased insulin secretion and causes persistent hypoglycemia in neonates and infants due to dysregulation of insulin secretion by pancreatic ß cells. Babies with severe hypoglycemia and for whom medical treatment has been ineffective usually require surgical treatment with near-total pancreatectomy. To evaluate the clinical and surgical aspects affecting survival outcomes in babies diagnosed with CHI in a single tertiary care center. METHODS: Retrospective Cohort study involving a single university tertiary center for the treatment of CHI. The authors study the demographics, clinical, laboratory, and surgical outcomes of this casuistic. RESULTS: 61 % were female, 39 % male, Birth weight: 3576 g (±313); Age of onset of symptoms: from the 2nd hour of life to 28 days; Time between diagnosis and surgery ranged between 10 and 60 days; Medical clinical treatment, all patients received glucose solution with a continuous glucose infusion and diazoxide. 81 % of the patients used corticosteroids, 77 %. thiazide, 72 % octreotide, 27 % nifedipine; Neurological sequelae during development and growth: 54 % had some degree of delay in neuropsychomotor development, 27 % obesity. Surgery was performed open in 6 and 12 minimally invasive surgery (MIS). HISTOPATHOLOGY: 2 focal and 16 diffuse, Length of stay (days) was lower in MIS (p < 0.05). Survival was 100 %. CONCLUSIONS: CHI is a rare and difficult-to-manage tumor that must be performed in a multidisciplinary and tertiary center. Most surgical results are good and the laparoscopic approach to disease has been the best choice for patients.

Carbohydrate counting and insulin analogs to treat type 1 diabetes mellitus. How to improve metabolic control?

OBJECTIVES: Clinical-laboratory comparison of a population of children and adolescents with DM1 followed at a Brazilian outpatient university clinic, at two different periods (2014 and 2020), regarding changes made both to the insulin therapy scheme and to the nutritional approach to carbohydrate counting. METHODS: The data of patients with DM1 aged 0-19 years enrolled in the service in 2014 and 2020 were collected. Student's t-test was performed to compare the means of HbA1c and the variables of interest. RESULTS: NPH + regular insulin was predominantly used in 2014 (49.1%), while in 2020, the predominance shifted to insulin analogs (48.4%). Pump use tripled from 1.3% in 2014 to 4.4% in 2020, and the percentage of patients performing carbohydrate counting reduced from 28.3% to 17.8%. Regarding HbA1c, the 2014 group of patients had a mean of 9.8%, while the 2020 group had a mean of 9.6% (p = 0.49). CONCLUSION: The change in treatments between 2014 and 2020 did not result in a significant improvement in HbA1c levels. However, it was identified the importance of carbohydrate counting and the use of insulin analogs to improve metabolic control in this population at both times.

Teleconsultation for pediatric patients with type 1 diabetes mellitus during the COVID-19 pandemic: experience of a university hospital in Brazil.

OBJECTIVE: Evaluate the impact of teleconsultation on glycemic control and prevention of acute complications related to diabetes mellitus in children and adolescents treated in a reference hospital during the COVID-19 pandemic in 2020. METHOD: Descriptive study of data from pediatric diabetic patients who received teleconsultation between April and September 2020. RESULTS: During this period, 143 diabetic patients were evaluated, with a median of 3.4 teleconsultations per patient in the studied period, requiring adjustment of insulin doses in 84.6% of cases. The hospital admission rate was 8,4% due to diabetic decompensation. The metabolic control (HbA1c) became worse in 46% of the sample and improved in 37%. CONCLUSION: The teleconsultation promoted health care for patients with diabetes mellitus during the COVID-19 pandemic but was not able to guarantee adequate glycemic control.

Insulin pump therapy in type 1 diabetes mellitus.

OBJECTIVES: To review the current experience with insulin pump therapy in children and adolescents in order to guide pediatricians regarding indications and complications. SOURCES OF DATA: Systematic review of articles published in the literature referring to the use of insulin pump therapy, indications, complications and response to treatment. All articles published between 1995 and 2005 and appearing in the MEDLINE and LILACS databases were reviewed. The keywords were: insulin pump, type 1 diabetes mellitus and diabetes mellitus. The articles covering the subject of interest and referring to children and adolescents were selected. SUMMARY OF THE FINDINGS: Insulin pump therapy is not required for all patients with type 1 diabetes, since intensive treatments produce very similar results in terms of glycated hemoglobin and control of complications over the medium and long terms. However, the pump allows for greater comfort for patients, with less rigid meal schedules and better quality of life. The first requirement for patients intending to use the pump is getting used to having a device attached to the body and following strict glucose control; otherwise, pump therapy is not advantageous. Complications are rare due to the technologies currently available. The cost, however, is greater than with conventional treatments. CONCLUSION: The development of infusion pumps and glucose monitors, including continuous monitoring systems, will lead to "intelligent pumps," so that a true "artificial pancreas" will be available, which can even be implanted in the patient, allowing non-diabetic persons to lead a normal life.

Is immunity in diabetic patients influencing the susceptibility to infections? Immunoglobulins, complement and phagocytic function in children and adolescents with type 1 diabetes mellitus.

OBJECTIVE: Previous reports suggest an increased susceptibility of diabetes patients to infections, but little information is available on possible underlying immunologic dysfunctions. The aim of this study was to evaluate humoral factors in pediatric patients with type 1 diabetes mellitus. METHODS: There were 66 diabetic patients (39 males:27 females; 5-17 yr) classified into two groups according to levels of glycohemoglobin (limit 9%): Group C - controlled (n = 33) and Group UC - uncontrolled (n = 33). We evaluated five patients in C and six in UC who reported previous infections. Immunologic analysis included measurement of plasma concentrations of immunoglobulins (Ig), C3, and C4 levels (turbidimetry); functional hemolytic assays for complement evaluation (CPH for classical and APH for alternative pathways), quantification of C4 isotypes C4A and C4B (ELISA), phagocytosis assays, measurement of bactericidal activity against Staphylococcus aureus, as well as tests of fungicidal capacity for Candida albicans. RESULTS: The UC Group had higher mean age, received higher insulin doses, and had higher concentrations of glycohemoglobin than the C Group. No significant differences in duration of the disease or nutritional conditions were detected between the groups. Lower IgA values in C (10/33) and lower IgG levels in UC (23/33) were detected, and there were inverse relationship with HbA1c values. Analysis of CPH, APH, C3, and C4 showed normal levels in both groups and no statistical correlation with the HbA1c. However, 9/33 children of the UC Group had decreased C3 values. C4B levels were below the normal range in 8/20 and correlated with higher HbA1c. Both phagocytic assays for S. aureus and Candida albicans were within normal limits. CONCLUSIONS: Low IgG concentrations and to some degree reduction in C4B levels were related to impaired metabolic control. No strong link between the immunological alterations was found in diabetic patients and the occurrence of infections.