Síndrome de pseudo-Bartter como presentación inicial de fibrosis quística

Las manifestaciones clínicas de la fibrosis quística comprometen diferentes órganos; siendo los sistemas respiratorio y gastrointestinal los más frecuentemente afectados. Puede presentarse como un desequilibrio ácido-base y electrolítico conocido como síndrome de pseudo-Bartter que se define como un episodio de deshidratación con alcalosis metabólica hipoclorémica hiponatrémica e hipocalémica en ausencia de alteración tubular renal. Se presenta el caso clínico de un lactante menor masculino de cuatro meses con dos hospitalizaciones previas por deshidratación moderada y desequilibrio hidroelectrolítico con hiponatremia. La tercera hospitalización fue el 27 de enero de 2017 por 20 días. En esta ocasión fue admitido por gastroenteritis aguda, con deshidratación moderada, desequilibrio hidroelectrolítico, y observación por un trastorno metabólico. Por presentar deshidratación con alcalosis metabólica hipoclorémica hiponatrémica e hipocalémica sin tubulopatía renal se diagnosticó síndrome de pseudo Bartter y se sospechó fibrosis quística que se corroboró con medición de electrolitos en sudor y análisis molecular de las mutaciones . Conclusión: Debe considerarse el diagnóstico de fibrosis quística en un niño, sobre todo menor de dos años, con deshidratación, alcalosis metabólica hiponatrémica hipoclorémica aunque no haya presentado síntomas respiratorios o gastrointestinales típicos de la enfermedad. El diagnóstico temprano es fundamental para mejorar el pronóstico y la sobrevida a largo plazo

The clinical manifestations of cystic fibrosis may involve multiple organs. Although the respiratory and gastrointestinal are the most commonly affected systems, it can present as an acid- base and electrolyte imbalance called pseudo -Bartter syndrome which is defined as an episode of dehidration with metbolic alkalosis with hypochloremia, hyponatremia and hypokalemia in the absence of renal tubular pathology. We report a case of a 4-month-old male infant with 2 previous episodes of moderate dehydration and hydroelectrolyte imbalance with hyponatremia. He was admitted on January 27 th 2017 for 20-days hospital stay. On his 3th hospitalization, he was admitted with acute gastroenteritis, moderate dehydration, hydroelectrolyte imbalance, and probable metabolic disorder. Due to the presence of metabolic hypochloremic alkalosis with hyponatremia and hypokalemia without renal tubulopathy, Pseudo Bartter Syndrome was diagnosed and cystic fibrosis was suspected and corroborated later with the measurement of sweat electrolytes and molecular analysis of mutations. Conclusion: The diagnosis of cystic fibrosis must be suspected in a child, especially those under 2 years old, with hyponatremic hypochloremic,hypokalemic metabolic alkalosis dehydration should be considered even in the absence of respiratory or gastrointestinal symptoms, which are typically present in this disease. An early diagnosis is essential to improve the prognosis and long term survivor

Validation of the COPD severity score for use in primary care: the NEREA study.

Spirometry is underused for the assessment of severity of chronic obstructive pulmonary disease (COPD) in primary care (PC). Therefore, simple assessment tools are required in this setting. The aim of the present study was to validate the COPD severity score (COPDSS) for use in PC. A multicentric study was carried out in stable COPD patients in PC. The concurrent validity of the COPDSS was evaluated by examining the association between COPDSS, COPD clinical indicators and the London Chest Activity of Daily Living (LCADL) scale, European quality of life (EuroQOL) questionnaires and Charlson comorbidity index. A total of 837 patients with COPD were analysed (males 84.3%; mean+/-sd age 68+/-11 yrs; forced expiratory volume in one second 54.6+/-17.7% of the predicted value). A strong correlation was found between COPDSS and dyspnoea level and a moderate correlation between COPDSS and exacerbation number. The COPDSS discriminated between patients with varying degrees of dyspnoea (area under receiver operating characteristic (ROC) curve 0.837), and according to number of exacerbations in the last year (area under ROC curve 0.773). Higher COPDSS scores were significantly associated with lower EuroQOL scores, lower EuroQOL visual analogue scale scores and higher LCADL scores. The present results indicate that the chronic obstructive pulmonary disease severity score is a useful and reliable tool for assessing the severity of chronic obstructive pulmonary disease in primary care.

A 12-week placebo-controlled study of rupatadine 10 mg once daily compared with cetirizine 10 mg once daily, in the treatment of persistent allergic rhinitis.

BACKGROUND: With the current increasing incidence of allergies worldwide, new treatments showing efficacy and long term safety are needed for chronic conditions such as persistent allergic rhinitis (PER). New generation H1-antihistamines have demonstrated anti-allergic properties, which could possibly enhance their effectiveness in long-term periods of treatment. OBJECTIVE: To investigate the efficacy of rupatadine, in controlling symptoms of PER over a 12-week period. METHODS: A randomized, double blind, parallel-group, placebo-controlled study was carried out in patients aged older than 12 years with PER. Main inclusion criteria were: instantaneous total symptom score (i6TSS) >or=45, nasal obstruction score or=2 as moderate during the first visit. The primary efficacy endpoint was the 12-week average change from baseline of the patients' i6TSS. RESULTS: In all, 736 patients were selected. Of them, 543 (73.8%) were randomized in three different groups: placebo (n = 185), cetirizine (n = 175) and rupatadine (n = 183). Rupatadine (P = 0.008) but not cetirizine (P = 0.07) statistically reduced the baseline i6TSS vs placebo (47.8%, 44.7% and 38.8%, respectively), after 12 weeks. Onset of action was observed at the first 24 h for both treatments (rupatadine vs placebo, P = 0.013; cetirizine vs placebo, P = 0.015). Furthermore, instantaneous total nasal symptoms score (iTNSS) (including nasal blockage) mean change from baseline showed a significant reduction with rupatadine 10 mg in comparison with placebo, along all treatment duration of 12 weeks. Study treatments were well tolerated. CONCLUSION: Rupatadine significantly relieves symptoms of PER, providing a rapid onset of action and maintains its effects over a long period of 12-weeks.

Rupatadine in the treatment of chronic idiopathic urticaria: a double-blind, randomized, placebo-controlled multicentre study.

BACKGROUND: Chronic urticaria is one of the most common and disturbing cutaneous condition. The treatment of chronic idiopathic urticaria (CIU) is still a challenge. Antihistamines are recommended as first-line treatment. Rupatadine is a new potent nonsedative anti-H1. OBJECTIVE: To study rupatadine efficacy and safety for moderate to severe CIU treatment. METHODS: This randomized, double-blind, placebo-controlled, parallel-group, multicentre, study was designed to assess primarily mean pruritus score (MPS) reduction with rupatadine, 10 and 20 mg, administered once daily for 4 weeks. Three hundred and thirty-three patients with active episodes of moderate-to-severe CIU were included. RESULTS: A 57.5% (P < 0.005) and 63.3% (P = 0.0001) significative MPS reduction from baseline, was observed at week 4 with 10 and 20 mg rupatadine, respectively, compared with placebo (44.9%). Both doses of rupatadine were not significantly different at any time point, with respect to their effects on pruritus severity, number of wheals and total symptoms scores. Rupatadine 10 mg had an overall better adverse event profile. CONCLUSION: Rupatadine 10 mg is a fast, long-acting, efficacious and safe treatment option for the management of patients with moderate-to-severe CIU.

Comportamiento de la vancomicina con las nuevas técnicas de diálisis / Behaviour of vancomycin with the new techniques in haemodialysis

Con el uso de técnicas de diálisis de alta convección surge la necesidad de plantearsela idoneidad de los protocolos habituales de administración de algunos fármacos,como la vancomicina.Objetivos: Confirmar si la pauta habitual de vancomicina es eficaz en pacientes atratamiento con biofiltración libre de acetato (AFB) y hemodiafiltración en línea(On-line). Proponer una pauta alternativa de administración.Materiales y métodos: Trece pacientes a tratamiento con AFB u On-line. Diez utilizabanfiltros de polisulfona y 3 de AN69. Primera parte: a 6 pacientes se les administró1 g iv de vancomicina en la última hora de diálisis. Segunda parte: a 7 pacientesse les administró una dosis de ataque de 30 mg/kg iv durante las dos últimas horas dediálisis, con un refuerzo de 500 mg post-diálisis. Se hizo un seguimiento de los nivelessanguíneos del antibiótico durante la semana siguiente a la administración.Resultados: En la primera fase se observó un descenso del 41% de los niveles séricosde vancomicina durante la diálisis, condicionando niveles subterapéuticos enel 83% de los pacientes hasta el final del estudio. Durante la segunda fase se consiguiómantener niveles terapéuticos y no tóxicos durante todo el estudio. Se confirmóla existencia de un rebote post-diálisis del 21%. Con la técnica de On-line se conseguíaun mayor aclaramiento de vancomicina que con AFB (176 vs 135 ml/min). Encontramosuna fuerte correlación entre el descenso del antibiótico y el volumen ultrafiltradocon la técnica de On-line.Conclusiones: La pauta habitual de vancomicina puede resultar insuficiente enpacientes a tratamiento con On-line y AFB. Podría ser adecuada una pauta basadaen una dosis de ataque de 30 mg/kg y un refuerzo de 500 mg al final de cada diálisis.Posiblemente el aclaramiento de este antibiótico con la técnica de On-line seproduzca por transporte convectivo

When using high convection dialysis techniques it arouses the necessity of consideringthe suitability of the regular protocols when administrating drugs, such as vancomycin. Objectives: To confirm if the usual guideline of vancomycin is efficient in patientsundergoing treatments with acetate free biofiltration (AFB) and haemodiafiltrationon-line (on-line). To propose an alternative guideline of administration.Material and methods: 13 patients treated with AFB or On-line. 10 of them usedfilters of polysulfone and 3 of them of AN69. First part: 6 patients were administered1 g iv during the last hour of dialysis. Second part: 7 patients were given a loadingdose of 30 mg/kg iv with a reinforcement of 500 mg post-dialysis. The blood levelsof the antibiotic were monitorized during the week following the administration.Outcomes: During the first phase it was noticed a decrease of 41% in the serumlevel of vancomycin during dialysis, conditioning subtherapeutic levels in the 83% ofthe patients until the end of the study. As for the second phase, therapeutic non-toxiclevels were maintained during the whole study. The existence of a post-dialysis reboundof the 21% was confirmed. A bigger clearance of vancomycin was obtainedwith the On-line technique rather than with AFB (176 vs 135 ml/min). We find astrong correlation between the decrease of the antibiotic and the volume ultrafiltratedwith the On-line technique.Conclusions: The usual guideline of vancomycin may not be enough with the newconvective dialysis techniques. A guideline based on a loading dose of 30 mg/kg anda reinforcement of 500 mg at the end of each dialysis could be adequate. The antibioticclearance with the On-line technique is probably made by convective transport

[Behaviour of vancomycin with the new techniques in haemodialysis]. / Comportamiento de la vancomicina con tas nuevas técnicas de diálisis.

UNLABELLED: When using high convection dialysis techniques it arouses the necessity of considering the suitability of the regular protocols when administrating drugs, such as vancomycin. OBJECTIVES: To confirm if the usual guideline of vancomycin is efficient in patients undergoing treatments with acetate free biofiltration (AFB) and haemodiafiltration on-line (on-line). To propose an alternative guideline of administration. MATERIAL AND METHODS: 13 patients treated with AFB or On-line. 10 of them used filters of polysulfone and 3 of them of AN69. First part: 6 patients were administered 1 g iv during the last hour of dialysis. Second part: 7 patients were given a loading dose of 30 mg/kg iv with a reinforcement of 500 mg post-dialysis. The blood levels of the antibiotic were monitorized during the week following the administration. OUTCOMES: During the first phase it was noticed a decrease of 41% in the serum level of vancomycin during dialysis, conditioning subtherapeutic levels in the 83% of the patients until the end of the study. As for the second phase, therapeutic non-toxic levels were maintained during the whole study. The existence of a post-dialysis rebound of the 21 % was confirmed. A bigger clearance of vancomycin was obtained with the On-line technique rather than with AFB (176 vs 135 ml/min). We find a strong correlation between the decrease of the antibiotic and the volume ultrafiltrated with the On-line technique. CONCLUSIONS: The usual guideline of vancomycin may not be enough with the new convective dialysis techniques. A guideline based on a loading dose of 30 mg/kg and a reinforcement of 500 mg at the end of each dialysis could be adequate. The antibiotic clearance with the On-line technique is probably made by convective transport.