Early use of teduglutide in paediatric patients with intestinal failure is associated with a greater response rate: a multicenter study.

Teduglutide is a glucagon-like-peptide-2 analogue that reduces the need for parenteral support in patients with short bowel syndrome (SBS). Nevertheless, data about long-term therapy with teduglutide in children are still scarce. Our objective was to describe the real-life experience with teduglutide in children with SBS over the last 5 years in Spain. This was a national multicentre and prospective study of paediatric patients with intestinal failure (IF) treated with teduglutide for at least 3 months. The data included demographic characteristics, medical background, anthropometric data, laboratory assessments, adverse events, and parenteral nutrition (PN) requirements. Treatment response was defined as a > 20% reduction in the PN requirement. The data were collected from the Research Electronic Data Capture (REDCap) database. Thirty-one patients from seven centres were included; the median age at the beginning of the treatment was 2.3 (interquartile range (IQR) 1.4-4.4) years; and 65% of the patients were males. The most frequent cause of IF was SBS (94%). The most common cause of SBS was necrotizing enterocolitis (35%). The median residual bowel length was 29 (IQR 12-40) cm. The median duration of teduglutide therapy was 19 (IQR 12-36) months, with 23 patients (74%) treated for > 1 year and 9 treated for > 3 years. The response to treatment was analysed in 30 patients. Twenty-four patients (80%) had a reduction in their weekly PN energy > 20% and 23 patients (77%) had a reduction in their weekly PN volume > 20%. Among the responders, 9 patients (29%) were weaned off PN, with a median treatment duration of 6 (IQR 4.5-22) months. The only statistically significant finding demonstrated an association between a > 20% reduction in the weekly PN volume and a younger age at the start of treatment (p = 0.028).   Conclusions: Teduglutide seems to be an effective and safe treatment for paediatric patients with IF. Some patients require a prolonged duration of treatment to achieve enteral autonomy. Starting treatment with teduglutide at a young age is associated with a higher response rate. What is Known: •  Glucagon-like peptide-2 (GLP-2) plays a crucial role in the regulation of intestinal adaptation in short bowel syndrome (SBS). Teduglutide is a GLP-2 analog that reduces the need for parenteral support in patients with SBS. • Data about long-term therapy with teduglutide in children in real life are still scarce. What is New: • Most pediatric patients with SBS respond in a satisfactory manner to teduglutide treatment. The occurrence of long-term adverse effects is exceptional. • Starting treatment with the drug at a young age is associated with a greater response rate.

Continuous glucose monitoring in insulin pump treated children.

INTRODUCTION: Insulin pump therapy aims to improve glycemic control and decrease the risk of hypoglycemia in type 1 diabetes. OBJECTIVE: To evaluate interstitial glucose levels and the frequency, duration and symptoms of hypo- and hyperglycemia through the use of a continuous glucose monitoring system (GGMS) in children and adolescents with insulin pump-treated type 1 diabetes, and to determine whether this monitoring method is well tolerated by these patients. PATIENTS AND METHOD: Thirteen patients (4 boys) with insulin pump-treated type 1 diabetes mellitus were monitored. Age was 10.6±3.5 (range, 3.2-13.6) years, diabetes duration was 5.0±3.2 years, pump therapy duration was 12.0±4.6 months, insulin dose was 0.99 ± 0.19 U/kg/day, and last hemoglobin A1c level was 7.1% ± 0.8%. The Minimed CGMS was used for 72 hours. RESULTS: A 3-year-old preschool child did not tolerate the CGMS. Interstitial glucose concentration was 187±40 mg/dl. Hypoglycemia (below 70mg/dl) accounted for 3.6% ± 5.6% of total time, while hyperglycemia (above 180 mg/dl) occurred 47.3% ± 17.4% of the time. No asymptomatic hypoglycemia episodes were detected. CONCLUSIONS: Insulin pump-treated children and adolescents showed an irregular interstitial glucose level and did not achieve normoglycemia. In our patients, adrenergic symptoms of hypoglycemia were preserved and the CGMS was generally well tolerated.

Long-term use of continuous subcutaneous insulin infusion compared with multiple daily injections of glargine in pediatric patients.

BACKGROUND: Studies comparing continuous subcutaneous insulin infusion (CSII) and glargine in pediatric patients are scarce and cover only 1 year follow up. Although short-term results are favorable for CSII, there is no clear evidence whether this therapy leads to a sustained improvement of glycemic control. OBJECTIVE: To evaluate long-term effects and safety of CSII versus multiple daily injections (MDI) of glargine over 2 years of treatment in children and adolescents. PATIENTS AND METHODS: 32 patients (aged 12.5 +/- 2.4 years; 19 females) with intensively treated type 1 diabetes mellitus were eligible. They were divided into two groups, virtually identical at study entry: eight on CSII and 24 on MDI of glargine. Variables at 0, 6, 12, 18 and 24 months were compared between groups by two-sided Mann-Whitney and Fisher tests. RESULTS: No significant differences were found between groups (CSII vs MDI) throughout the study period. At 24 months hemoglobin A1c was 7.70 +/- 0.64% vs 7.54 +/- 0.74% (p = 0.8); body mass index SDS was 0.33 +/- 0.74 vs 0.40 +/- 1.01 (p = 0.9); total daily insulin requirements were 0.95 +/- 0.10 vs 1.05 +/- 0.18 U/kg (p = 0.4), incidence of severe hypoglycemia was 0.00 +/- 0.00 vs 0.04 +/- 0.14 episodes/patient/year (p = 0.8); and incidence of ketoacidosis was 0.20 +/- 0.27 vs 0.04 +/- 0.14 episodes/patient/year (p = 0.2). CONCLUSION: CSII and MDI with glargine are equally effective and safe in pediatric patients at 2-year follow-up.

[Age of onset of puberty and menarche in type 1 diabetic girls]. / Edad del inicio puberal y de la menarquia en niñas con diabetes tipo 1.

INTRODUCTION: Delayed pubertal maturation has been reported in girls with type 1 diabetes. OBJECTIVES: To report the age of onset of puberty and menarche in girls with type 1 diabetes diagnosed before puberty. To investigate clinical factors affecting the occurrence of puberty and menarche in this population. PATIENTS AND METHODS: A retrospective study of 38 girls with type 1 diabetes, all of them on intensive insulin therapy since diagnosis and followed up at our hospital until menarche. Age of onset of puberty and age of menarche were collected as dependent variables, and time since onset of diabetes, glycosylated hemoglobin levels, daily insulin requirements, and body mass index standard deviation score were collected as independent variables. Variables are expressed as mean ± standard deviation. Multivariate linear regression models tested the associations between dependent and independent variables. Statistical analysis was performed using SPSS software. RESULTS: Thirty-eight girls were enrolled. Age of onset of puberty was 10.4 ± 1.1 years and age of menarche, 12.6 ± 1.0 years. Time since diabetes onset influenced age at onset of puberty (ß = +0.12; p=0.047). A negative association was found between body mass index standard deviation score and age at menarche (ß=-0.39; p=0.014). CONCLUSION: Diabetes duration and body mass index were correlated with age of onset of puberty and age of menarche in girls with type 1 diabetes.

Hemorragía sub-aracnoidea primaria y sustancias inmunoreactivas endogena similar a la digoxina / Subarachnoid hemorrage and endogenous immunoreactiva substance similar to digoxin

Se determinaron niveles de digoxina por un método de inmunoensayo fluorométrico enzimático en plasma de 15 pacientes con hemorragia subaracnoidea primera (HSA) por ruptura de aneurisma intracraneal, en 15 controles sanos (Control I) y 15 controles con afecciones neuroquirúrgicas distintas a la HSA (Control II). Ninguno de los 45 pacientes había recibido digoxina. En 11 de los 15 pacientes con HSA, el método determinó la presencia en plasma de substancia inmunoreactiva endógena similar a la digoxina (SIESD) en un rango de 0,13 a 0,42 ng/ml (promedio: 0,28 ng/ml) días antes del tiempo promedio (8 días) de aparición de vasoespasmo clínico. Los mayores niveles se observaron en pacientes que se complicaron posteriormente con trastornos del sensorial y disfunción neurológica. De los 15 controles sanos solo dos mostraron niveles de SIESD de 0,11 y 0,17 ng/ml. De los 15 controles II solo tres (prolactinosa, meningismos múltiples y malformación arteriovenosa cerebelosa) presentaron valores de 0,10 y 0,21 ng/ml. La diferencia de los valores de SIESD entre los pacientes con HSA y cada uno de los grupos controles fue estadísticamente significativo (p < 0,001). La SIESD se encuentra con frecuencia en pacientes con HSA y pudiera ser utilizado como factor predictivo de vasoespasmo clínico

Antibioticoterapia profilactica en neurocirugía: estudio prospectivo y controlado, en doscientas craneotomías / Prophylactic antibiotictherapy in neurosurgery: prospective study and controlated in 200 craniotomy

La utilidad de la Oxacilina como antibiótico profiláctico en neurocirugía fue estudiado en forma prospectiva, en el Servicio de Neurocirugía del Hospital Vargas de Caracas. Este grupo de estudio fue conformado por 100 casos de craneotomías limpias realizadas entre enero de 1989 y junio de 1990, presentándose un 2 por ciento de infecciones postoperatorias. Este resultado se comparó con el grupo control, constituido por 100 casos de craneotomías limpias, realizadas antes del inicio de este estudio, sin profilaxia con antibióticos preoperatorio, los cuales presentaron un 13 por ciento de infecciones postoperatorias. Esta diferencia en el porcentaje de infecciones entre los dos grupos fue estadísticamente significativa (p<0,05), demostrando la eficacia de la profilaxia antibiocoterápica preoperatoria en la prevención de infecciones postoperatorias.

Manejo estereotáctico en lesiones de la región pineal / stereotactic management in pineal region lesions

En la actualidad con las técnicas estereotácticas para los tumores de la región pineal, se obtiene un diagnóstico histológico definitivo con mínimo riesgo. Se presenta una revisión de 13 pacientes ingresados al servicio de neurocirugía del Hospital de Caracas con diagnóstico clínico e imagenológico del tumor de la región pineal, se practicó biopsia estereotáctica de la lesión en 11 de ellos y en 9 se determinaron marcadores tumorales (ß -gonadotrofina coriónica humana y µ -fetoproteína), obteniéndose diagnóstico definitivo en 10 pacientes, lo que permitió planificar el tratamiento adicional en 9 casos (4 con cirugía abierta más radioterapia y los otros 5 con radioterapia sola ). Se realizó la aspiración del contenido quístico en tres lesiones, de los cuales en uno con tumor quístico se procedió a instilar P intratumoral. Se presentaron complicaciones transitorias en tres casos (trastornos de la conciencia y ataxia), y una muerte debido a hemorragia intratumoral en el postoperatorio inmediato. Se concluye que este procedimiento permite tanto el diagnóstico definitivo, al obtener muestras directas de la lesión, permitiendo la planificación del tratamiento adecuado; así como la realización de procedimientos terapéuticos de aspiración de lesiones quísticas, instilación intratumoral de radionucleótidos y la ventriculostomía

Guía para el manejo de pacientes con traumatismo raquimedular / Guide for the management of patients with spinal trauma

Se presenta una guía práctica para el manejo de los pacientes con trauma raquimedular agudo utilizada en el Departamento de Neurocirugía del "Hospital Vargas de Caracas". Estamos conscientes de que algunas de estas pautas terapéuticas para este tipo particular de trauma están en discusión y son frecuentemente origen de controversia