María Asunción Martínez-Brocca

A national survey on the efficacy and safety of continuous subcutaneous insulin infusion in patients with type 1 diabetes in Spain

AIMS To assess safety and benefits of continuous subcutaneous insulin infusion (CSII) therapy in a cohort of type 1 diabetes patients in Spain. METHODS A web-based national registry was created by the Working Group of the Spanish Diabetes Association. All patients on CSII being followed at selected referral centers were included. A cross-sectional analysis was performed. RESULTS A total of 1275 patients were included. Data completion for patients on CSII was 67 ± 32%. Indications for treatment were suboptimal glycemic control (32%), high glucose variability (24%), preconception care (14%) and hypoglycemia (11%). In the patients on CSII for ≥1 year (n = 843, mean CSII duration of 5 years), HbA1c decreased by 5 mmol/mol (0.5%) in the whole population and by 8 mmol/mol (0.7%) in subjects with suboptimal glycemic control as CSII indication. Percentage of patients achieving HbA1c ≤ 53 mmol/mol (7%) increased from 20% before CSII to 34% at the end of follow-up. Severe hypoglycemia decreased from 29% to 5%. The rate of discontinuation was 9.5%. HbA1c was lower in patients using bolus advisor and temporary basal rates. CONCLUSIONS CSII was associated with a sustained improvement in glycemic control and a reduction in severe hypoglycemia. The use of advanced CSII settings was related to better glycemic control.

Implementación del protocolo hospitalario de insulinización subcutánea para pacientes no críticos en hospitales andaluces de tercer nivel

INTRODUCTION In 2009, the Andalusian Society of Endocrinology and Nutrition designed a protocol for subcutaneous insulin treatment in hospitalized non-critically ill patients (HIP). OBJECTIVE To analyze implementation of HIP at tertiary care hospitals from the Andalusian Public Health System. METHOD A descriptive, multicenter study conducted in 8 tertiary care hospitals on a random sample of non-critically ill patients with diabetes/hyperglycemia (n=306) hospitalized for ≥48 hours in 5 non-surgical (SM) and 2 surgical (SQ) departments. Type 1 and other specific types of diabetes, pregnancy and nutritional support were exclusion criteria. RESULTS 288 patients were included for analysis (62.5% males; 70.3±10.3 years; 71.5% SM, 28.5% SQ). A scheduled subcutaneous insulin regimen based on basal-bolus-correction protocol was started in 55.9% (95%CI: 50.5-61.2%) of patients, 63.1% SM vs. 37.8% SQ (P<.05). Alternatives to insulin regimen based on basal-bolus-correction included sliding scale insulin (43.7%), diet (31.3%), oral antidiabetic drugs (17.2%), premixed insulin (1.6%), and others (6.2%). For patients previously on oral antidiabetic drugs, in-hospital insulin dose was 0.32±0.1 IU/kg/day. In patients previously on insulin, in-hospital insulin dose was increased by 17% [-13-53], and in those on insulin plus oral antidiabetic drugs, in-hospital insulin dose was increased by 26.4% [-6-100]. Supplemental insulin doses used for<40 IU/day and 40-80 IU/day were 72.2% and 56.7% respectively. HbA1c was measured in 23.6% of patients (95CI%: 18.8-28.8); 27.7% SM vs. 13.3% SQ (P<.05). CONCLUSIONS Strategies are needed to improve implementation of the inpatient subcutaneous insulin protocol, particularly in surgical departments. Sliding scale insulin is still the most common alternative to insulin regimen based on basal-bolus-correction scheduled insulin. Metabolic control assessment during hospitalization should be encouraged.

Alteraciones endocrinológicas y psicológicas en la polisomía 48.XXXY

14 year-old male patient was referred to the endocrinology utpatient clinic for hypogonadism. He had an unremarkble family and personal history. He was born in China, and ad lived in Spain with his biological family since the age of hree. This boy was referred by the urology outpatient clinic ue to delayed genital development possibly due to hypergnadotropic hypogonadism with a total testosterone level of .7 nmol/L (reference range: 9.9--27.8), FSH 18.3 IU/L, and H 13.3 IU/L (1--25). His family reported poor academic performance, diffiulties in relations with other students, and progressive ehavioral changes (attention deficit, irritability, impulse ontrol problems) in the previous two years. Physical examination found a weight of 50.5 kg, a height f 169 cm (97th percentile of height and 50th percentile f weight for Chinese adolescents of similar chronological ge),1 and an arm span of 173 cm. He had a longilinear ody habitus, pectus excavatum, kyphoscoliotic pattern, rominent elbows, and increased abdominal fat. Low hair mplantation, abnormal ears, hypertelorism, and epicanthus ere also found. As regards secondary sexual characterisics, he had no pubic and axillary hair and showed infantile enitalia (2-mL testes, 3-cm penis). The rest of the examiation was unremarkable (Fig. 1). Based on these findings, laboratory tests were requested, ncluding a hormone profile, X-rays of the left hand, and aryotype. The patient was also referred to the infanile mental health unit (USMI). Results of supplemental ests were as follows: total testosterone, 5.5 nmol/L; sex ormone-binding globulin (SHBG), 29.9 nmol/L (NV 10--80); H, 29.6 IU/L; FSH, 37.3 IU/L; 17-beta-estradiol, 24 pmol/L 20--1800); TSH, 3.93 U/mL (0.4--4); total cholesterol, 06 mg/dL (150--200); and triglycerides, 58 mg/dL (70--170). due to Klinefelter-like syndrome secondary to aneuploidy 48,XXXY was therefore made. Treatment was started with testosterone cypionate 50 mg every 4 weeks by the intramuscular route, with three-monthly dose titration based on total testosterone and gonadotropin (FSH and LH) levels. Drug treatment was well tolerated and had no influence on behavior. Based on recommendations by the USMI, the patient entered a specific support and follow-up program at school. Klinefelter syndrome encompasses a group of disorders characterized by the presence of at least one X chromosome additional to the normal male karyotype, 46,XY. The classical form is a karyotype 47,XXY, but there are other much more uncommon variants, such as those caused by aneuploidies 48,XXYY, 49,XXXXY, and 48,XXXY.2 The 47,XXY is the most common chromosome abnormality in humans, with an incidence of one case per 650 males born. The incidence of the 48,XXXY variant is very low and is estimated at approximately 1:50,000 males born.3 While patients with these polysomies share common clinical characteristics, there are differential traits between the different forms reported. This syndrome is traditionally characterized by tall height, narrow shoulders, gynecomastia, decreased testicular size and penis length, facial dysmorphism, hypergonadotropic hypogonadism, and mental retardation, among other changes. These traits vary depending on the underlying chromosome abnormality and specifically on the number of surplus X chromosomes. Thus, patients with polysomy 48,XXXY have higher mean heights as compared to those with polysomy 47,XXY (190 cm versus 179--188 cm) and are more likely to have congenital malformations such as radioulnar synostoses or clinodactyly.3,4 Facial and body dysmorphism (hypertelorism, epicanthus, narrow lid opening, low hairline implantation, flat feet, joint hyperextensibility and hyperlaxity) are also more common in polysomy 48,XXXY. Decreased testicular volume (less than 3 mL and usually lower than 1.5 mL) is a constant trait, and is also more likely in patients with polysomy 48,XXXY.3