Aramchol in patients with nonalcoholic steatohepatitis: a randomized, double-blind, placebo-controlled phase 2b trial.

Nonalcoholic steatohepatitis (NASH), a chronic liver disease without an approved therapy, is associated with lipotoxicity and insulin resistance and is a major cause of cirrhosis and hepatocellular carcinoma. Aramchol, a partial inhibitor of hepatic stearoyl-CoA desaturase (SCD1) improved steatohepatitis and fibrosis in rodents and reduced steatosis in an early clinical trial. ARREST, a 52-week, double-blind, placebo-controlled, phase 2b trial randomized 247 patients with NASH (n = 101, n = 98 and n = 48 in the Aramchol 400 mg, 600 mg and placebo arms, respectively; NCT02279524 ). The primary end point was a decrease in hepatic triglycerides by magnetic resonance spectroscopy at 52 weeks with a dose of 600 mg of Aramchol. Key secondary end points included liver histology and alanine aminotransferase (ALT). Aramchol 600 mg produced a placebo-corrected decrease in liver triglycerides without meeting the prespecified significance (-3.1, 95% confidence interval (CI) -6.4 to 0.2, P = 0.066), precluding further formal statistical analysis. NASH resolution without worsening fibrosis was achieved in 16.7% (13 out of 78) of Aramchol 600 mg versus 5% (2 out of 40) of the placebo arm (odds ratio (OR) = 4.74, 95% CI = 0.99 to 22.7) and fibrosis improvement by ≥1 stage without worsening NASH in 29.5% versus 17.5% (OR = 1.88, 95% CI = 0.7 to 5.0), respectively. The placebo-corrected decrease in ALT for 600 mg was -29.1 IU l-1 (95% CI = -41.6 to -16.5). Early termination due to adverse events (AEs) was <5%, and Aramchol 600 and 400 mg were safe, well tolerated and without imbalance in serious or severe AEs between arms. Although the primary end point of a reduction in liver fat did not meet the prespecified significance level with Aramchol 600 mg, the observed safety and changes in liver histology and enzymes provide a rationale for SCD1 modulation as a promising therapy for NASH and fibrosis and are being evaluated in an ongoing phase 3 program.

Creating a model to predict time intervals from induction of labor to induction of anesthesia and delivery to coordinate workload.

BACKGROUND: Induction of labor continues to become more common. We analyzed induction of labor and timing of obstetric and anesthesia work to create a model to predict the induction-anesthesia interval and the induction-delivery interval in order to co-ordinate workload to occur when staff are most available. METHODS: Patients who underwent induction of labor at a single medical center were identified and multivariable linear regression was used to model anesthesia and delivery times. Data were collected on date of birth, race/ethnicity, body mass index, gestational age, gravidity, parity, indication for labor induction, number of prior deliveries, time of induction, induction agent, cervical dilation, effacement, and fetal station on admission, date and time of anesthesia administration, date and time of delivery, and delivery type. RESULTS: A total of 1746 women met inclusion criteria. Associations which significantly influenced time from induction of labor to anesthesia and delivery included maternal age (anesthesia P <0.001, delivery P =0.002), body mass index (both P <0.001), prior vaginal delivery (both P <0.001), gestational age (anesthesia P <0.001, delivery P <0.018), simplified Bishop score (both P <0.001), and first induction agent (both P <0.001). Induction of labor of nulliparous women at 02:00 h and parous women at 04:00 or 05:00 h had the highest estimated probability of the mother having her first anesthesia encounter and delivering during optimally staffed hours when our institution's specialty personnel are most available. CONCLUSIONS: Time to obstetric and anesthesia tasks can be estimated to optimize induction of labor start times, and shift anesthesia and delivery workload to hours when staff are most available.

Helicobacter pylori eradication in Mexico with a levofloxacin-based scheme versus standard triple therapy: Results from an open-label, randomized, noninferiority phase iiib trial. / Erradicación de Helicobacter pylori en México con un esquema basado en levofloxacina versus la triple terapia estándar: resultados de un estudio clínico de fase iiib, abierto, aleatorizado, de no inferioridad.

INTRODUCTION AND AIMS: Helicobacter pylori (H. pylori) infection remains the leading cause of several gastroduodenal diseases. Despite the fact that multiple antibiotic regimens have been used to change its associated morbidity and mortality, the prevalence of this bacterial infection continues to be disproportionately high worldwide, mainly due to antibiotic resistance. To assess the noninferiority efficacy and safety of 210-day triple regimens on H. pylori eradication, we evaluated clarithromycin 500mg, lansoprazole 30mg, and amoxicillin 1g, all bid (standard triple therapy or CLA, Group 1) vs. pantoprazole 80mg, levofloxacin 500mg and azithromycin 500mg, all od (PLA, Group 2). Both regimens were compared in treatment-naïve patients. MATERIALS AND METHODS: An open label phase IIIb randomized and noninferiority trial comparing CLA vs. PLA was carried out for a 10-day period, within the time frame of June 2012 and March 2014. Eradication was verified with 13C-urea breath testing. Gastric biopsies were tested for fluorescence in situ hybridization (FISH)-clarithromycin resistance prior to any antibiotic administration. Efficacy and safety results were analyzed according to the noninferiority methodological approach. RESULTS: From the 227 H. pylori positive subjects that were randomized, 194 were finally analyzed as per-protocol. The group 2 eradication rate was 63% and was noninferior to the group 1 eradication rate of 58.5% (upper limit 95% CI: 0.11608; below the noninferiority margin: 0.1200). FISH clarithromycin-resistance was found in 28.2% of the cases. Adverse events, all minor and self-limited, were significantly higher in group 1 than in group 2 (86 vs. 65.4%; p=0.001). CONCLUSIONS: First-line H. pylori eradication with pantoprazole/levofloxacin/azithromycin combination therapy is as effective as the standard triple therapy, with better tolerability and easier dosing. Clarithromycin resistance should be considered when selecting antibiotics in Helicobacter pylori eradication treatments. ClinicalTrials.gov identifier NCT02726269.

Adherence to Anaphylaxis Guidelines: Real-World Data From the Emergency Department of a Tertiary Hospital.

BACKGROUND AND OBJECTIVE: Few studies have evaluated adherence to anaphylaxis guidelines in emergency departments (EDs). Objective: The objective of this study was to evaluate adherence to anaphylaxis guidelines in the ED of a tertiary hospital. METHODS: Medical records of patients attended in the ED of University Hospital of Salamanca, Spain were reviewed. Those patients fulfilling the anaphylaxis criteria proposed by the NIAID/FAAN were selected. RESULTS: During a 1-year period, we identified 89 patients (74 adults and 15 children). The anaphylactic reaction was moderate in 65% of adults, severe in 34%, and very severe in 1%. In children, all reactions were moderate. Fewer than half of the patients (42%) received adrenaline in the ED; this was administered intramuscularly in only 19% of cases. As for the severity of the reaction, 65% of patients with moderate reactions and 42% with severe reactions were not treated with adrenaline. At discharge from the ED, an adrenaline auto-injector was recommended to only 5.6% of patients. Fifty-two percent of patients received a documented allergy referral (57% adults vs 27% children, P=.047), 29% instructions about avoidance of triggers (31% adults vs 20% children, NS), and 51% written instructions for recognition of anaphylaxis warning signs (41% adults vs 100% children, P<.001). CONCLUSIONS: The results of the study show a large discrepancy between recommendations in guidelines and management of anaphylaxis in the ED. Additional training efforts are needed to improve the treatment of patients with anaphylactic reactions.

[Ivermectin as an adjunct in the treatment of refractory epilepsy]. / Ivermectina como coadyuvante en la epilepsia refractaria.

INTRODUCTION: Ivermectin, a 22'23 dihydro derivative of avermectins beta-1a, is a highly effective veterinary and human anti parasitic, used to treat endoparasites of difficult control such as filariasis and onchocerciasis, with a median plasma life of at least of 16 hours. The recommended therapeutic doses range from 0.05 to 0.40 mg/kg, without undesirable effects or risk to human life. It went from being a great success in animal health to its application in humans, where it has had great impact. Studies in basic sciences have shown that ivermectin has anticonvulsive effects in different epileptic animal models, where five different mechanisms of action have been described. PATIENTS AND METHODS: Descriptive, prospective observational study, performed between 2013 and 2015, with 32 refractory epileptic patients, who received ivermectin as an a dose of adjunctive treatment of 10 mg/day three or seven times a week, controlled every three months, followed by 12-24 months, without withdrawal of anticonvulsant medications that they received previously. RESULTS: Progressively, patients entered into crisis control, at the end of the programmed follow-up period, the total percentage of crisis reduction was 97%, of which 57% did not return to crisis from the beginning of treatment, all patients being free of crisis according to International League Against Epilepsy criteria. CONCLUSION: Ivermectin has been useful as an adjuvant, achieving a significant decrease in seizures in this group of drug resistant patients.

TITLE: Ivermectina como coadyuvante en la epilepsia refractaria.Introduccion. La ivermectina es un 22'23 dihidroderivado de las avermectinas beta-1a, un antiparasitario de uso veterinario y humano de gran eficacia, utilizado para tratar endoparasitos de dificil control, como la filariasis y la oncocercosis. Tiene una vida media plasmatica de al menos 16 horas. Las dosis terapeuticas recomendadas varian entre 0,05 y 0,40 mg/kg, sin efectos indeseados ni riesgo para la vida humana. Paso de ser un gran exito en sanidad animal a su aplicacion en seres humanos, donde ha tenido gran impacto. Estudios en ciencias basicas han demostrado que la ivermectina tiene efectos anticonvulsionantes en diferentes modelos animales epilepticos, donde se han descritos cinco diferentes mecanismos de accion. Pacientes y metodos. Estudio descriptivo, observacional prospectivo, realizado entre 2013 y 2015, con 32 pacientes epilepticos refractarios, quienes recibieron ivermectina como tratamiento coadyuvante en dosis de 10 mg/dia, tres o siete veces por semana, controlados cada tres meses, seguidos durante 12-24 meses, sin retirada de los medicamentos anticonvulsionantes que recibian previamente. Resultados. Progresivamente, los pacientes entraron en control de las crisis. Al final del periodo de seguimiento programado, el porcentaje total de reduccion de las crisis fue del 97%. El 57% de estos pacientes no volvio a presentar crisis desde el inicio del tratamiento, y todos los pacientes se encontraron libres de crisis segun los criterios de la Liga Internacional contra la Epilepsia. Conclusion. La ivermectina ha sido util como coadyuvante y ha logrado una significativa disminucion de crisis en este grupo de pacientes farmacorresistentes.

Impact of Moderate Heat, Carvacrol, and Thymol Treatments on the Viability, Injury, and Stress Response of Listeria monocytogenes.

The microbial safety and stability of minimally processed foods are based on the application of combined preservative factors. Since microorganisms are able to develop adaptive networks to survive under conditions of stress, food safety may be affected, and therefore understanding of stress adaptive mechanisms plays a key role in designing safe food processing conditions. In the present study, the viability and the sublethal injury of Listeria monocytogenes exposed to moderate heat (55 °C) and/or essential oil compounds (carvacrol and thymol, 0.3 mM) treatments were studied. Synergistic effects were obtained when combining mild heat (55 °C) with one or both essential oil compounds, leading to inactivation kinetics values three to four times lower than when using heat alone. All the treatments applied caused some injury in the population. The injury levels ranged from around 20% of the surviving population under the mildest conditions to more than 99.99% under the most stringent conditions. Protein extracts of cells exposed to these treatments were analysed by two-dimensional gel electrophoresis. The results obtained revealed that stressed cells exhibited differential protein expression to control cells. The proteins upregulated under these stressing conditions were implicated, among other functions, in stress response, metabolism, and protein refolding.

GIDEON (Global Investigation of therapeutic DEcisions in hepatocellular carcinoma and Of its treatment with sorafeNib): second interim analysis.

BACKGROUND: GIDEON (Global Investigation of therapeutic DEcisions in hepatocellular carcinoma [HCC] and Of its treatment with sorafeNib) is a global, prospective, non-interventional study undertaken to evaluate the safety of sorafenib in patients with unresectable HCC in real-life practice, including Child-Pugh B patients who were excluded from clinical trials. METHODS: Patients with unresectable HCC, for whom the decision to treat with sorafenib, based on the approved label and prescribing guidelines, had been taken by their physician, were eligible for inclusion. Demographic data and disease/medical history were recorded at entry. Sorafenib dosing and adverse events (AEs) were collected at follow-up visits. The second interim analysis was undertaken when ~1500 treated patients were followed up for ≥ 4 months. RESULTS: Of the 1571 patients evaluable for safety, 61% had Child-Pugh A status and 23% Child-Pugh B. The majority of patients (74%) received the approved 800 mg initial sorafenib dose, regardless of Child-Pugh status; however, median duration of therapy was shorter in Child-Pugh B patients. The majority of drug-related AEs were grade 1 or 2, and the most commonly reported were consistent with previous reports. The incidence and nature of drug-related AEs were broadly similar across Child-Pugh, Barcelona Clinic Liver Cancer (BCLC) and initial dosing subgroups, and consistent with the overall population. CONCLUSIONS: Consistent with the first interim analysis, overall safety profile and dosing strategy are similar across Child-Pugh subgroups. Safety findings also appear comparable irrespective of initial sorafenib dose or BCLC stage. Final analyses in > 3000 patients are ongoing.

Dos nuevas mutaciones en el gen que codifica la alfa-glucosidasa ácida en un adolescente con enfermedad de Pompe de inicio tardío / Two new mutations in the gene that codes for acid alpha-glucosidase in an adolescent with late-onset Pompe disease

Introducción. La glucogenosis tipo II o enfermedad de Pompe es una enfermedad lisosomal con un patrón de herencia autosómico recesivo. La enfermedad de Pompe de inicio tardío es una miopatía metabólica progresiva causada por una disminución de la actividad de la enzima alfa-glucosidasa ácida (GAA), lo que origina una disminución de la degradación y posterior acúmulo del glucógeno dentro de los lisosomas y el citoplasma celular. Caso clínico. Adolescente venezolano, de 16 años, diagnosticado de glucogenosis tipo II o enfermedad de Pompe, de comienzo tardío, basado en la clínica del paciente y los hallazgos bioquímicos. La atrofia muscular de los miembros superiores e inferiores era evidente y presentaba maniobra de Gowers positiva. Los niveles séricos de creatincinasa eran elevados. Su capacidad funcional respiratoria estaba disminuida. La cuantificación de la actividad enzimática de la GAA en papel de filtro no mostraba una disminución significativa de la actividad. El análisis genético molecular reveló la existencia de dos mutaciones en condición homocigótica en el gen GAA, c.547-67C>G y c.547-39T>G, ambas en el exón 2 del cromosoma 17. De acuerdo con la base de datos del genoma humano y la revisión emprendida, los cambios detectados en este paciente representan nuevas mutaciones en el gen GAA. Esta afirmación está en concordancia con la clínica y cambios bioquímicos encontrados en el paciente. Conclusión. Es obligatorio el estudio genético molecular en un paciente en el que se sospecha la enfermedad (AU)

Introduction. Glycogen storage disease type II, or Pompe disease, is a lysosomal disease with an autosomal recessive pattern of inheritance. Late-onset Pompe disease is a progressive metabolic myopathy caused by decreased activity of the enzyme acid alpha-glucosidase (GAA), which gives rise to reduced degradation and later accumulation of glycogen in the lysosomes and cell cytoplasm. Case report. A 16-year-old Venezuelan male, diagnosed with late-onset glycogen storage disease type II, or Pompe disease, based on the patient’s clinical picture and the biochemical findings. The patient presented unmistakable signs of muscular atrophy in the upper and lower limbs, as well as positive Gowers’ sign. Levels of creatinkinase in serum were high. His functional respiratory capacity was diminished. The quantification of the enzymatic activity of acid alpha-glucosidase on filter paper did not show any significant decrease in activity. A molecular genetic analysis revealed the existence of two homozygotic mutations in the gene GAA, c.547-67C>G and c.547-39T>G, both on exon 2 of chromosome 17. According to the human genome database and the review that was undertaken, the changes detected in this patient represent new mutations in the acid alpha-glucosidase gene, GAA. This claim is in agreement with the clinical features and biochemical changes found in the patient. Conclusion. A molecular genetic study is mandatory in patients suspected of having this disease (AU)

First interim analysis of the GIDEON (Global Investigation of therapeutic decisions in hepatocellular carcinoma and of its treatment with sorafeNib) non-interventional study.

AIMS: Global Investigation of therapeutic DEcisions in hepatocellular carcinoma and Of its treatment with sorafeNib (GIDEON), a global, non-interventional, surveillance study, aims to evaluate the safety of sorafenib in all patients with unresectable hepatocellular carcinoma (uHCC) under real-life practice conditions, particularly Child-Pugh B patients, who were not well represented in clinical trials. METHODS: Treatment decisions are determined by each physician according to local prescribing guidelines and clinical practice. Patients with uHCC who are candidates for systemic therapy, and for whom a decision has been made to treat with sorafenib, are eligible for inclusion. Demographic data and medical and disease history are recorded at entry. Sorafenib dosing and adverse events (AEs) are collected throughout the study. RESULTS: From January 2009 to April 2011, >3000 patients from 39 countries were enrolled. The prespecified first interim analysis was conducted when the initial approximately 500 treated patients had been followed up for ≥4 months; 479 were valid for safety evaluation. Preplanned subgroup analyses indicate differences in patient characteristics, disease aetiology and previous treatments by region. Variation in sorafenib dosing by specialty are also observed; Child-Pugh status did not appear to influence the starting dose of sorafenib. The type and incidence of AEs was consistent with findings from previous clinical studies. AE profiles were comparable between Child-Pugh subgroups. DISCUSSION: The GIDEON study is generating a large, robust database from a broad population of patients with uHCC. First interim analyses have shown global and regional differences in patient characteristics, disease aetiology and practice patterns. Subsequent planned analyses will allow further evaluation of early trends.

[Thyroid dysfunction in children with chronic renal failure]. / Alteraciones de la función tiroidea en niños con insuficiencia renal crónica.

OBJECTIVES: To determine the frequency and type of thyroid dysfunction in children with chronic renal failure (CRF) in peritoneal dialysis (PD) or hemodialysis (HD); and to establish the accuracy of the presence of goiter to identify patients with CRF and thyroid dysfunction. PATIENTS AND METHODS: This is a cross-sectional study performed in a tertiary pediatric medical care center. CRF patients younger than 17 years old, with more than three months in PD or HD were included. All patients were assessed regarding their growth and sexual development; thyroid dysfunction was evaluated by serum concentration of thyrotropin (TSH), thyroxine (T4L) and triiodothyronine (T3T). RESULTS: 50 patients were included, 25 were male, and mean age was 12 years old. There were 14 (28%) patients with thyroid dysfunction; nine had subclinical hypothyroidism, three patients had euthyroid sick syndrome and two primary hypothyroidism. Thirteen patients had goiter: seven had thyroid dysfunction and in six patients the thyroid function was normal. The sensitivity of goiter to detect thyroid dysfunction was 50% and the specificity was 83.3%. The two patients with the greatest delay in their growth were hypothyroid. CONCLUSIONS: Given that the high frequency of thyroid dysfunction in children with CRF, these patients need a systematic screening, in order to improve their quality of care.

Alteraciones de la función tiroidea en niños con insuficiencia renal crónica / No disponible

Objetivos: Determinar la frecuencia y tipo de alteraciones de la función tiroidea en niños con insuficiencia renal crónica (IRC) en programa de diálisis peritoneal (DP) o hemodiálisis (HD), así como establecer la utilidad de bocio como marcador clínico para identificar pacientes con IRC que cursan con alteraciones de la función tiroidea. Pacientes y métodos: Estudio transversal y descriptivo, realizado en un hospital pediátrico de tercer nivel de atención. Se incluyeron pacientes menores de 17 años, con IRC y con más de tres meses en DP o HD. En cada paciente se evaluó su crecimiento y desarrollo, así como la presencia de bocio. Las alteraciones tiroideas se detectaron mediante la cuantificación de los niveles séricos de tirotropina (TSH), tiroxina (T4L) y triyodotironina (T3T). Resultados: Se incluyeron 50 pacientes, 25 del sexo masculino, con edad promedio de 3 años. Hubo 14 (28%) pacientes con alteración en la función tiroidea, nueve con hipotiroidismo subclínico, tres con síndrome de enfermo eutiroideo y dos con hipotiroidismo primario. En 13 pacientes se detectó bocio, siete con disfunción tiroidea y seis con función normal. La sensibilidad del bocio para la detección de alteraciones tiroideas fue del 50%, y la especificidad del 83.3%. Dos de los pacientes con hipotiroidismo presentaron la mayor afectación en su crecimiento. Conclusiones: Debido a la alta frecuencia de alteraciones tiroideas en niños con IRC, es necesaria su valoración de manera sistemática, a fin de mejorar la calidad de su atención (AU)

Objectives: To determine the frequency and type of thyroid dysfunction in children with chronic renal failure (CRF) in peritoneal dialysis (PD) or hemodialysis (HD); and to establish the accuracy of the presence of goiter to identify patients with CRF and thyroid dysfunction. Patients and methods: This is a crosssectional study performed in a tertiary pediatric medical care center. CRF patients younger than 17 years old, with more than three months in PD or HD were included. All patients were assessed regarding their growth and sexual development; thyroid dysfunction was evaluated by serum concentration of thyrotropin (TSH), thyroxine (T4L) and triiodothyronine (T3T). Results: 50 patients were included, 25 were male, and mean age was 13 years old. There were 14 (28%) patients with thyroid dysfunction; nine had subclinical hypothyroidism, three patients had euthyroid sick syndrome and two primary hypothyroidism. Thirteen patients had goiter: seven had thyroid dysfunction and in six patients the thyroid function was normal. The sensitivity of goiter to detect thyroid dysfunction was 50% and the specificity was 83.3%. The two patients with the greatest delay in their growth were hypothyroid. Conclusions: Given the high frequency of thyroid dysfunction in children with CRF, these patients need a systematic screening, in order to improve their quality of care (AU)

[Landau-Kleffner syndrome: an analysis of 10 cases in Venezuela]. / Síndrome de Landau-Kleffner: análisis de 10 casos en Venezuela.

INTRODUCTION: Landau-Kleffner syndrome is characterised by acquired aphasia and encephalographic alterations that may or may not be accompanied by epileptic seizures. AIM. To analyse the clinical and encephalographic features and response to treatment of 10 patients with Landau-Kleffner syndrome. PATIENTS AND METHODS: We reviewed the patient records, encephalograms and treatment administered to patients catalogued as having Landau-Kleffner syndrome. RESULTS: The mean age of the patients was 44 months. Of these cases, 60% presented epilepsy when the diagnosis was established and 70% were found to have epileptic status during slow-wave sleep in the encephalographic study. Results showed that 40% corresponded to variants of Landau-Kleffner syndrome. No cause of the disease could be established in any of the patients. In the neuroimaging study, only one patient displayed abnormalities in the magnetic resonance imaging of the brain. All the patients received adrenocorticotropic hormone (ACTH)-based treatment, at a dose of 1 IU/kg/day for one month, administered together with antiepileptic drugs such as valproic acid and clobazam. Convulsive seizures and epileptic status during slow-wave sleep disappeared in all the patients. In the patients without epileptic status, epileptic activity became less frequent, although it did not completely disappear. Aphasia improved considerably, which meant that all the patients were able to enroll in normal schools. CONCLUSIONS: We believe that early diagnosis, together with suitable and timely management of aphasic patients with encephalographic alterations that allow ACTH to be used at low doses, make it possible to offer an early education so as to provide maximum recovery from the disease.

Síndrome de Landau-Kleffner: análisis de 10 casos en Venezuela / Landau-Kleffner syndrome: an analysis of 10 cases in venezuela

Introducción. El síndrome de Landau-Kleffner se caracteriza por una afasia adquirida y alteraciones encefalográficas que se pueden acompañar o no de crisis epilépticas. Objetivo. Analizar las características clínicas y encefalográficas y la repuesta al tratamiento de 10 pacientes con síndrome de Landau-Kleffner. Pacientes y métodos. Revisamos las historias clínicas, los encefalogramas y el tratamiento administrado a los pacientes catalogados con síndrome de Landau-Kleffner. Resultados. La edad promedio de los pacientes fue de 44 meses. El 60% de los casos presentaba epilepsia en el momento de realizar el diagnóstico, y el 70%, estado epiléptico durante el sueño lento en el estudio encefalográfico. El 40% correspondía a variantes del síndrome de Landau-Kleffner. En ningún paciente se pudo establecer una causa de la enfermedad. En el estudio neurorradiológico, sólo un paciente mostró anormalidades en la resonancia magnética cerebral. Todos los pacientes recibieron tratamiento a base de hormona adrenocorticotropa (ACTH), en dosis de 1 UI/kg/día, durante un mes, administrada junto con fármacos antiepilépticos, como el ácido valproico y el clobazam. Las crisis convulsivas y el estado epiléptico durante el sueño lento desaparecieron en todos los pacientes. En los pacientes sin estado epiléptico, la actividad epiléptica se hizo menos frecuente, sin llegar a desaparecer. La afasia mejoró notablemente, lo que permitió a todos los pacientes incorporarse a escuelas normales. Conclusiones. Consideramos que el diagnóstico temprano y el manejo adecuado y oportuno de un paciente afásico con alteraciones encefalográficas permitiendo el uso de ACTH en dosis bajas, brindan la posibilidad de una educación temprana para proporcionar una recuperación máxima de la enfermedad

Introduction. Landau-Kleffner syndrome is characterised by acquired aphasia and encephalographic alterations that may or may not be accompanied by epileptic seizures. Aim. To analyse the clinical and encephalographic features and response to treatment of 10 patients with Landau-Kleffner syndrome. Patients and methods. We reviewed the patient records, encephalograms and treatment administered to patients catalogued as having Landau-Kleffner syndrome. Results. The mean age of the patients was 44 months. Of these cases, 60% presented epilepsy when the diagnosis was established and 70% were found to have epileptic status during slow-wave sleep in the encephalographic study. Results showed that 40% corresponded to variants of Landau-Kleffner syndrome. No cause of the disease could be established in any of the patients. In the neuroimaging study, only one patient displayed abnormalities in the magnetic resonance imaging of the brain. All the patients received adrenocorticotropic hormone (ACTH)-based treatment, at a dose of 1 IU/kg/day for one month, administered together with antiepileptic drugs such as valproic acid and clobazam. Convulsive seizures and epileptic status during slow-wave sleep disappeared in all the patients. In the patients without epileptic status, epileptic activity became less frequent, although it did not completely disappear. Aphasia improved considerably, which meant that all the patients were able to enrol in normal schools. Conclusions. We believe that early diagnosis, together with suitable and timely management of aphasic patients with encephalographic alterations that allow ACTH to be used at low doses, make it possible to offer an early education so as to provide maximum recovery from the disease

Amuchina 10% solution, safe antiseptic for preventing infections of exit-site of Tenckhoff catheters, in the pediatric population of a dialysis program.

Although, decreasing in incidence with the disconnection systems, the first complication is still peritonitis in patients with chronic renal failure and the second is infection of Tenckhoff catheter exit-site. All efforts made to diminish the frequency of exit-site infection lower the possibility of peritonitis. The pediatric population is well-known to have a major risk of infectious complications, and making easy and safe the care of the exit-site will prevent the peritonitis that follows. The aim of the study was to evaluate the efficacy of the Amuchina 10% solution vs. pH neutral soap in children with chronic renal failure, on preventing exit-site infection. There were 60 patients who were assigned randomly to one of two groups. One group used Amuchina 10% solution for the daily cleaning of the exit-site, and the other used pH neutral soap, with 14 months of follow-up. Before the study they have to be free of infection for at least 30 days. All were taught by the same nurse how to clean their exit-site. Groups were almost identical in years, sex, and time on dialysis. We had nine infections in the soap group and none in the Amuchina 10% solution group, with an OR: 17 (p = 0.004). From these nine infections, the bacteria isolated were: 4 (13%) were caused by Pseudomona aeruginosa, 1 (3.3%) by Staphylococcus aureus, coagulase-positive staphylococci in 2 (6.6%) and Serratia marcensens in 1 (3.3%). In conclusion, Amuchina 10% solution is effective in preventing infection on the exit-site, without any secondary topical reaction.

[Alternating hemiplegia of childhood treated as epilepsy. Two new cases]. / Hemiplejía alternante de la infancia tratada como epilepsia. Dos nuevos casos.

INTRODUCTION: Alternating hemiplegia (AH) of childhood is a rare disease that gives rise to transient attacks of hemiplegia that may affect either side of the body indistinctly, or even both sides at the same time. Onset occurs before the age of 18 months and in some cases there are neonatal symptoms such as abnormal eye movements, especially nystagmus, and dystonic or tonic seizures. Attacks of hemiplegia, which disappear during sleep, begin before the age of one year. These symptoms can initially be taken for an epileptic disorder. CASE REPORTS: We describe the study of two male patients whose clinical symptoms appeared at the age of 6 months, with tonic seizures, upward deviation of gaze, without loss of consciousness, which occurred with a recurrence rate of between one and two attacks a week or several times a day. They were initially treated with antiepileptic drugs (AED), although the complementary tests, including electroencephalogram (EEG), computerised axial tomography (TAC), magnetic resonance (MR) scans and metabolic tests, were all normal. CONCLUSIONS: Since, to date, no specific test has been confirmed for the disease, diagnosis is essentially clinical and by exclusion. Treatment is symptomatic using flunarizine. In one of our cases, administration of a single 10 mg/day dose this drug at night lowered the frequency of the attacks of hemiplegia. The possibility of treating a patient who is a carrier of an AH with AED makes early knowledge and identification of this disease necessary in order to be able to improve the patient's prognosis.

Hemiplejía alternante de la infancia tratada como epilepsia. Dos nuevos casos / Alternating hemiplegia of childhood treated as epilepsy. Two new cases

Introducción. La hemiplejía alternante (HA) de la infancia es una enfermedad poco frecuente. Provoca crisis de hemiplejía transitorias que pueden afectar indistintamente a uno u otro hemicuerpo, e incluso a ambos a un mismo tiempo. Se inicia antes de los 18 meses y, en algunos casos, se presentan síntomas neonatales, como movimientos anormales oculares, sobre todo nistagmo, y crisis distónicas o tónicas. Antes del año de edad se inician los ataques hemipléjicos, que desaparecen con el sueño. Estos síntomas pueden llevar a una confusión inicial con un trastorno epiléptico. Casos clínicos. La observación de dos pacientes varones cuyas manifestaciones clínicas aparecieron a los 6 meses de edad con crisis tónicas, con desviación hacia arriba de la mirada y sin pérdida de la conciencia, que se producían con una periodicidad recurrente variable de una a dos crisis por semana o varias al día. Se trataron inicialmente con fármacos antiepilépticos (FAE), aunque las pruebas complementarias, entre ellas el electroencefalograma (EEG), la tomografía axial computarizada (TAC), la resonancia magnética (RM), y las pruebas metabólicas eran normales. Conclusiones. Ya que no se ha demostrado hasta la actualidad un examen específico de la enfermedad, el diagnóstico es fundamentalmente clínico y por exclusión. El tratamiento es sintomático con flunaricina. En uno de nuestros casos, una dosis de 10 mg/día única nocturna produjo una disminución en la frecuencia y la intensidad de los ataques hemipléjicos. La posibilidad de tratar a un paciente portador de una HA con FAE hace necesario el conocimiento y la identificación precoz de esta enfermedad, con el fin de mejorar su pronóstico

Introduction. Alternating hemiplegia (AH) of childhood is a rare disease that gives rise to transient attacks of hemiplegia that may affect either side of the body indistinctly, or even both sides at the same time. Onset occurs before the age of 18 months and in some cases there are neonatal symptoms such as abnormal eye movements, especially nystagmus, and dystonic or tonic seizures. Attacks of hemiplegia, which disappear during sleep, begin before the age of one year. These symptoms can initially be taken for an epileptic disorder. Case reports. We describe the study of two male patients whose clinical symptoms appeared at the age of 6 months, with tonic seizures, upward deviation of gaze, without loss of consciousness, which occurred with a recurrence rate of between one and two attacks a week or several times a day. They were initially treated with antiepileptic drugs (AED), although the complementary tests, including electroencephalogram (EEG), computerised axial tomography (TAC), magnetic resonance (MR) scans and metabolic tests, were all normal. Conclusions. Since, to date, no specific test has been confirmed for the disease, diagnosis is essentially clinical and by exclusion. Treatment is symptomatic using flunarizine. In one of our cases, administration of a single 10 mg/day dose this drug at night lowered the frequency of the attacks of hemiplegia. The possibility of treating a patient who is a carrier of an AH with AED makes early knowledge and identification of this disease necessary in order to be able to improve the patient’s prognosis

Effect of different densities of the twospotted spider mite Tetranychus urticae on CO2 assimilation, transpiration, and stomatal behaviour in rose leaves.

The effect of population density of Tetranychus urticae Koch on CO2 assimilation, transpiration and stomatal behaviour in rose leaves and on the diameter and length of stems and flower buds was investigated under greenhouse conditions. The investigation was performed in order to gain more insight into integrated control systems in rose crops grown under greenhouse conditions. Physiological processes, such as photosynthesis and transpiration, as well as stomatal behaviour and chlorophyll content, were studied as they form part of the plant's nutrition mechanism and therefore affect the quantity and quality of the flowers. Information related to the effect of spider mite population density on bloom quality, diameter and length of stems and flower buds was also collected. The data indicate that increased mite density coincides with a decrease in the net photosynthetic rate, transpiration and chlorophyll content. Higher mite densities on leaves cause stomata to remain open for longer periods, which allows a greater loss of water. Spider mite densities of 10 and 50 mites per leaf cause a reduction in flower stem length of 17 and 26%, respectively, as compared to plants with no mites present.

[Methylmalonic aciduria associated with myoclonic convulsions, psychomotor retardation and hypsarrhythmia]. / Aciduria metilmalónica asociada a convulsiones mioclónicas, retraso psicomotor e hipsarritmia.

INTRODUCTION: Organic acidurias have long been known to cause neurological problems, such as convulsions, stupor, coma, and psychomotor and mental retardation. The organic acidurias include propionic aciduria, methylmalonic aciduria (MMA), isovaleric acidemia, lactic acidemia and glutaric acidemia type I. However, the association of MMA with electrical activity of the brain characterised by a hypsarrhythmic pattern, refractory convulsions and psychomotor retardation is very rare. CASE REPORTS: Two patients, one male and one female, were seen to have psychomotor retardation, erratic attacks of myoclonic convulsions, hypsarrhythmic encephalographic pattern and an increase in the urinary excretion of methylmalonic acid, as shown by gas chromatography and mass spectrometry, all of which supported a diagnosis of MMA in both cases. In one patient, the brain MRI with gadolinium showed lesions compatible with brain atrophy. Protein restrictions, the administration of vitamin B12 and l carnitine re established the normal neurological state and reduced the urinary excretion of methylmalonic acid in one of them. CONCLUSIONS: To the best of our knowledge these are the first cases of MMA that have been seen accompanied by hypsarrhythmia. The rareness of this clinical presentation with the characteristics described above make us suspect that we are dealing with a new clinical syndrome.

Aciduria metilmalónica asociada a convulsiones mioclónicas, retraso psicomotor e hipsarritmia / Methylmalonic aciduria associated with myoclonic convulsions, psychomotor retardation an hypsarrhythmia

Introducción. Desde hace muchos años se sabe que las acidurias orgánicas son causantes de problemas neurológicos,como convulsiones, estupor, coma y retraso psicomotor y mental. Dentro de las acidurias orgánicas se encuentran la aciduria propiónica, la aciduria metilmalónica (AMM), la acidemia isovalérica, la acidemia láctica y la acidemia glutárica tipo I. Sin embargo, la asociación de la AMM con una actividad eléctrica cerebral caracterizada por un patrón hipsarrítmico, convulsiones de difícil control terapeútico y retraso psicomotor, es muy rara. Casos clínicos. La observación de dos pacientes, uno masculino y otro femenino, con retraso psicomotor, crisis convulsivas mioclónicas erráticas, patrón encefalográfico hipsarrítmico, y la demostración por medio de la cromatografía de gases y espectrometría de masas de un aumento de la excreción urinaria de ácido metilmalónico, sustentan el diagnóstico de AMM en ambos casos. En un paciente, la RM cerebral con gadolinio mostraba lesiones compatibles con atrofia cerebral. La restricción proteica y la administración de vitamina B12 y L-carnitina devolvieron la normalidad neurológica y redujeron la excreción urinaria de ácido metilmalónico en uno de ellos. Conclusiones. Hasta donde sabemos, estos son los primeros casos de AMM que se acompañan de hipsarritmia. La rareza de esta presentación clínica con las características descritas nos hace sospechar que estamos ante un nuevo síndrome clínico (AU)

Introduction. Organic acidurias have long been known to cause neurological problems, such as convulsions, stupor, coma, and psychomotor and mental retardation. The organic acidurias include propionic aciduria, methylmalonic aciduria (MMA), isovaleric acidemia, lactic acidemia and glutaric acidemia type I. However, the association of MMA with electrical activity of the brain characterised by a hypsarrhythmic pattern, refractory convulsions and psychomotor retardation is very rare. Case reports. Two patients, one male and one female, were seen to have psychomotor retardation, erratic attacks of myoclonic convulsions, hypsarrhythmic encephalographic pattern and an increase in the urinary excretion of methylmalonic acid, as shown by gas chromatography and mass spectrometry, all of which supported a diagnosis of MMA in both cases. In one patient, the brain MRI with gadolinium showed lesions compatible with brain atrophy. Protein restrictions, the administration of vitamin B12 and L-carnitine re-established the normal neurological state and reduced the urinary excretion of methylmalonic acid in one of them. Conclusions. To the best of our knowledge these are the first cases of MMA that have been seen accompanied by hypsarrhythmia. The rareness of this clinical presentation with the characteristics described above make us suspect that we are dealing with a new clinical syndrome (AU)

Clasificación de las huellas labiales en una población de Bogotá / Lip prints classification in a population of Bogota city

El propósito de este estudio fue el de establecer una clasificación de huellas labiales en una muestra en la ciudad de Bogotá (Colombia), y compararlo con los resultados que se han encontrado en otros países con el fin de verificar diferencias o similitudes basadas en la clasificación de Renaud (1972) para determinar su aplicabilidad como método de identificación. Se tomaron 260 marcas de labios en hombres y mujeres entre 18 y 60 años de edad en diferentes zonas de Santafé de Bogotá. La muestra fue recogida por medio de una impresión de la huella labial desplazando un formato de cartulina entre los labios, posteriormente se hizo el revelado de la misma y quedando evidente el queilograma para realizar el análisis encontrando que en hombres y mujeres todos los queilogramas son diferentes, siendo la letra C la que se encuentra con mayor frecuencia y observando un nuevo tipo de surco labial al cual se le denominó K demostrando de ésta forma que puede servir como un método adicional, para la identificación de individuos en Colombia.