Estudio WAKE de vida real en pacientes con narcolepsia con cataplejía tratados con pitolisant no respondedores a tratamientos previos / Real-life WAKE study in narcolepsy patients with cataplexy treated with pitolisant and unresponsive to previous treatments

Introducción: La narcolepsia de tipo 1 es una enfermedad incapacitante que requiere tratamiento continuo, que no siempre es eficaz. El pitolisant es un nuevo fármaco con un mecanismo de acción diferente que ofrece una nueva opción de tratamiento. El objetivo del estudio fue analizar la efectividad y la seguridad del pitolisant en pacientes con narcolepsia de tipo 1 que no hubieran respondido o tolerado previamente los tratamientos habituales. Pacientes y métodos: Estudio observacional descriptivo multicéntrico de vida real que incluyó a pacientes diagnosticados de narcolepsia de tipo 1 no respondedores a tratamientos previos que iniciaron tratamiento con pitolisant. El estudio evaluó tres momentos: el inicio del tratamiento, la estabilización del tratamiento con pitolisant y los tres meses posteriores. Resultados: En 32 pacientes incluidos (media de edad, 44 años; 37,5% de mujeres), la media de la escala de somnolencia de Epworth se redujo de 17,1 a 13,5; un 47,8% de los pacientes mejoró subjetivamente de su cataplejía; un 65% de los pacientes mejoró su impresión clínica global a criterio médico y a criterio del paciente; y se redujo la media de medicamentos consumidos de 2,0 a 1,4. El efecto adverso más frecuente fue el insomnio, en un 43,8% de los pacientes. De los 32 pacientes, 23 mantuvieron el tratamiento durante los tres meses de seguimiento. Conclusiones: En pacientes con narcolepsia de tipo 1 que no responden a o no toleran los tratamientos disponibles, el pitolisant puede mejorar su situación clínica y reducir su consumo de medicamentos. Son necesarios estudios de mayor nivel de evidencia para confirmar estos resultados.(AU)

INTRODUCTION: Type 1 narcolepsy is a disabling disease that requires continuous treatment, which is not always effective. Pitolisant is a new drug with a different mechanism of action that offers a new treatment option. The objective of the study was to analyse the effectiveness and safety of pitolisant in patients with type 1 narcolepsy that did not respond to or tolerate previous standard treatments. PATIENTS AND METHODS: Real-life multicentre descriptive observational study that included patients diagnosed with type 1 narcolepsy who did not respond to or tolerate previous treatments and started treatment with pitolisant. The study evaluated three different moments: the start of treatment, the stabilization of treatment with pitolisant and the three months after. RESULTS: In 32 patients included (mean age, 44 years; 37.5% women) the mean of the Epworth Sleepiness Scale was reduced from 17.1 to 13.5; 47.8% of the patients improved from their cataplexy; 65% of the patients improved their clinical global impression at the physician’s and at the patient’s discretion and the mean number of medications consumed was reduced from 2.0 to 1.4. The most frequent adverse effect was insomnia in 43.8% of patients. Of the 32 patients, 23 continued with the treatment during the 3-month follow-up period. CONCLUSIONS: In patients with type I narcolepsy who do not respond to or do not tolerate the available treatments, pitolisant can improve their clinical situation and reduce their medication consumption. Studies with a higher level of evidence are needed to confirm these results.(AU)

[Real-life WAKE study in narcolepsy patients with cataplexy treated with pitolisant and unresponsive to previous treatments]. / Estudio WAKE de vida real en pacientes con narcolepsia con cataplejía tratados con pitolisant no respondedores a tratamientos previos.

INTRODUCTION: Type 1 narcolepsy is a disabling disease that requires continuous treatment, which is not always effective. Pitolisant is a new drug with a different mechanism of action that offers a new treatment option. The objective of the study was to analyse the effectiveness and safety of pitolisant in patients with type 1 narcolepsy that did not respond to or tolerate previous standard treatments. PATIENTS AND METHODS: Real-life multicentre descriptive observational study that included patients diagnosed with type 1 narcolepsy who did not respond to or tolerate previous treatments and started treatment with pitolisant. The study evaluated three different moments: the start of treatment, the stabilization of treatment with pitolisant and the three months after. RESULTS: In 32 patients included (mean age, 44 years; 37.5% women) the mean of the Epworth Sleepiness Scale was reduced from 17.1 to 13.5; 47.8% of the patients improved from their cataplexy; 65% of the patients improved their clinical global impression at the physician's and at the patient's discretion and the mean number of medications consumed was reduced from 2.0 to 1.4. The most frequent adverse effect was insomnia in 43.8% of patients. Of the 32 patients, 23 continued with the treatment during the 3-month follow-up period. CONCLUSIONS: In patients with type I narcolepsy who do not respond to or do not tolerate the available treatments, pitolisant can improve their clinical situation and reduce their medication consumption. Studies with a higher level of evidence are needed to confirm these results.

TITLE: Estudio WAKE de vida real en pacientes con narcolepsia con cataplejía tratados con pitolisant no respondedores a tratamientos previos.Introducción. La narcolepsia de tipo 1 es una enfermedad incapacitante que requiere tratamiento continuo, que no siempre es eficaz. El pitolisant es un nuevo fármaco con un mecanismo de acción diferente que ofrece una nueva opción de tratamiento. El objetivo del estudio fue analizar la efectividad y la seguridad del pitolisant en pacientes con narcolepsia de tipo 1 que no hubieran respondido o tolerado previamente los tratamientos habituales. Pacientes y métodos. Estudio observacional descriptivo multicéntrico de vida real que incluyó a pacientes diagnosticados de narcolepsia de tipo 1 no respondedores a tratamientos previos que iniciaron tratamiento con pitolisant. El estudio evaluó tres momentos: el inicio del tratamiento, la estabilización del tratamiento con pitolisant y los tres meses posteriores. Resultados. En 32 pacientes incluidos (media de edad, 44 años; 37,5% de mujeres), la media de la escala de somnolencia de Epworth se redujo de 17,1 a 13,5; un 47,8% de los pacientes mejoró subjetivamente de su cataplejía; un 65% de los pacientes mejoró su impresión clínica global a criterio médico y a criterio del paciente; y se redujo la media de medicamentos consumidos de 2,0 a 1,4. El efecto adverso más frecuente fue el insomnio, en un 43,8% de los pacientes. De los 32 pacientes, 23 mantuvieron el tratamiento durante los tres meses de seguimiento. Conclusiones. En pacientes con narcolepsia de tipo 1 que no responden a o no toleran los tratamientos disponibles, el pitolisant puede mejorar su situación clínica y reducir su consumo de medicamentos. Son necesarios estudios de mayor nivel de evidencia para confirmar estos resultados.

Evaluación de las reincidencias de las urgencias ORL en un Hospital de tercer nivel. Oportunidades de mejora / Assessment of the relapse rate of ENT emergencies in a third level hospital: opportunities for improvent

El objetivo principal del estudio es un análisis descriptivo general de las visitas y de la calidad asistencial ORL en el Servicio de Urgencias Hospitalarias (SUH) durante el mes de septiembre de 2005. Para la valoración de las visitas se analizó la actividad según los diagnósticos emitidos en SUH (CIE-9), según variables temporales y según gravedad. Para la valoración de la calidad asistencial se han evaluado el índice de reincidencias (4,3%) y el porcentaje de fugas (3,6%). Los diagnósticos más frecuentes fueron otitis externa (10%), amigdalitis aguda (7,4%) y epitaxis (5,9%). El 50% de las visitas se consideraron de tipo ambulatorio de acuerdo a la gravedad (AU)

A describe study is made of the visits and nursing quality of the ENT Service in the Hospital Casualty Department of the Hospital Clínic during the month of September 2005. The visits are analysed according to the diagnosis given, time variables and the severity. The conclusions indicate that the most frequently diagnosed given, time variables and the severity. The conclusions indicate that the most frequently diagnosed pathologies in this Service were otitis externa, acute tonsillitis and nose bleeds. 50% of the visits are considered as outpatient´s department type according to the severity (AU)

Taquipnea neurógena ligada al sueño: una etiopatogenia no aclarada

La taquipnea neurógena ligada al sueño (TNLS) es un raro trastorno sobre el que existen muy pocas referencias en la literatura, y con una etiopatogenia no aclarada. Se define como un incremento sostenido de la frecuencia respiratoria durante el sueño, que se mantiene durante el mismo y revierte con el despertar. Algunos pacientes presentan excesiva somnolencia diurna como síntoma de consulta, y en otros es completamente asintomática. Se ha asociado a trastornos respiratorios (ronquido, apneas) y con diferentes patologías del SNC. Se presentan en este trabajo tres pacientes con TNLS estudiados en nuestra unidad, que presentaron características clínicas y polisomnográficas similares a las descritas. Con este motivo se realiza una discusión sobre las posibles causas de este trastorno, con especial énfasis en el papel de la formación reticular (AU)

Primary polyarteritis nodosa presenting as acute symmetric quadriplegia.

We report a case of peripheral neuropathy presenting as acute symmetric areflexic quadriplegia in the setting of a well-defined clinical, histopathologic, and angiographic diagnosis of classic polyarteritis nodosa. While it is usually easy to recognize the typical clinical presentation of necrotizing angiopathy-induced peripheral neuropathy as a mononeuritis multiplex or a distal polyneuropathy in association with a collagen vascular disease, clinicians must be equally sensitive to a number of more challenging possibilities. Acute quadriplegia similar to that seen in Guillain-Barré syndrome can be secondary to primary classic polyarteritis nodosa and the former may be the chief or even the sole manifestation of the latter.

[Cat-scratch disease. Presentation of 2 cases in a family with serologic follow-up and discussion of diagnostic methods]. / Enfermedad por arañazo de gato. Presentación de dos casos familiares con seguimiento serológico y discusión de métodos diagnósticos.

Two cases are reported of cat scratch disease which we consider worth describing both because of their familial presentation (the involved patients were brothers) and the serologic follow-up performed after diagnosis, which showed significant increases in IgG antibody titers to Bartonella henselae in both patients, one on day 35 and the other on day 45 after diagnosis. Cat scratch disease is a rarely diagnosed condition in our environment and it is easily misdiagnosed with other regional lymphadenitis; hence, a serologic test revealing a seroconversion or a significant increase in serologic titers will be very useful, as well as the epidemiologic antecedent of cat exposure.

[Behçet's disease with an onset prior to the appearance of chronic myeloid leukemia]. / Enfermedad de Behçet de inicio previo a la aparición de leucemia mieloide crónica.

The case of a Ph-positive female patient with chronic myeloid leukemia (CML) is reported. The patient presented a cutaneous-mucous picture prior to the appearance of the hemopathy consisting of genitals ulcers, buccal aphthae and nodular cutaneous lesions the study of which demonstrated panniculitis. The lesions improved with the administration of low doses of prednisone and colchicine. The CML evolved to a blastic crisis of a monocytic phenotype at 14 months of diagnosis leading to death of the patient. The cutaneous-mucous picture was catalogued as Becçet disease (BD) according to the criteria of the International Study Group for Behçet Disease. Given the lack of serologic tests or pathognomonic histologic lesions the difficulty in the diagnosis of BD is commented upon and the differential diagnosis of this disease, particularly with respect to the Sweet syndrome, is discussed.

Prosthetic valve endocarditis caused by Corynebacterium pilosum.

A case of prosthetic valve endocarditis caused by Corynebacterium pilosum in a 79-year-old woman developed eighty years after aortic valve replacement with bovine pericardium bioprosthesis is described. In spite of the antibiotic therapy she presented an unfavourable course that led to her death.

[IgM myeloma: a report of 2 cases]. / Mieloma IgM: presentación de dos casos.

The IgM myeloma is a rare type of multiple myeloma (MM) with some features which differentiate it from other immunologic types of myeloma and from Waldenström macroglobulinemia (WM). Two patients with IgM myeloma in whom the only clinical feature of the disease was the development of bone lesions and general deterioration are reported. In none of the cases an M component was detected in proteinogram. A bone marrow proliferation of plasma cells was discovered in both, which it was accompanied with dura mater infiltration in one of them. The type of secreted immunoglobulin was IgM lambda in the first case and kappa in the second. Both patients had a poor clinical course after the diagnosis and died due to infective complications. Emphasis is made on the need to differentiate this condition from WM due to their different prognosis and therapy.

[Thrombosis of the superior vena cava as an early manifestation of Behçet's disease. Presentation of 2 cases]. / Trombosis de vena cava superior como manifestación precoz de la enfermedad de Behçet. Presentación de dos casos.

The vascular involvement of Behçet's disease is nowadays considered as an important sign of the clinical evolution of these patients. It can affect both the arterial and the venous territories. Superficial thrombophlebitis is the most common vascular manifestation. Thrombosis of deep veins is relatively rare and it generally appears within the inferior vena cava territory as a late manifestation in the evolution of the disease. We describe two cases of Behçet's disease which presented, at an early stage, thrombosis of the superior vena cava as the more relevant manifestation of the disease. The evolution of both patients was satisfactory with the combination of cyclophosphamide and colchicine. The etiopathogenesis of this manifestation and its therapeutic possibilities are discussed.

[High-degree malignancy non-Hodgkin's lymphoma simulating a disseminated carcinoma. Presentation of 2 cases]. / Linfoma no hodgkiniano de alto grado de malignidad que simula un carcinoma diseminado. Presentación de dos casos.

Highly malignant non-Hodgkin lymphomas (HM-NHL) may sometimes develop clinical features simulating an epithelial carcinoma with metastatic dissemination. Conventional histopathological study may be insufficient to differentiate between both conditions. Two patients with HM-NHL are reported with a rapid general deterioration; one of them had osteolysis and hypercalcemia. In both cases a diffuse bone marrow infiltration by large sized cells with blastic appearance was found. The initial suspected diagnosis was occult epithelial neoplasia with metastatic dissemination. The morphological study with optic microscopy and the ultrastructural analysis did not establish the origin of these cells. The definitive diagnosis was obtained by immunohistochemical techniques. In both cases, the cells were positive for the CD 45 (common leukocyte antigen) monoclonal antibody (MoAb), and for several MoAbs of lymphoid B differentiation. In one of them, the B lymphoid lineage was confirmed by monoclonal reordering of the gene that synthetises the immunoglobulin heavy chain.

Acute renal failure complicating viral hepatitis in the absence of severe hepatic insufficiency.

Four patients (2 were HBsAg positive) with acute icteric viral hepatitis (VH) developed acute renal failure (ARF) in the course of their illness and in the absence of other complications. Their peak serum creatinine values (4.7-10, mean 7 mg/dl) were reached either before or simultaneously with their maximum serum aminotransferase values (1,390-2730, mean 2,032 mU/ml). Apart from VH no other factors responsible for precipitating ARF could be identified. In the HBsAg-negative patients, serological investigations for infectious mononucleosis, cytomegalovirus infection, and leptospirosis were negative. In 2 patients liver biopsy showed changes consistent with VH. Proteinuria was absent in all cases, making glomerulonephritis unlikely. The urinary sodium excretion was uniformly high (57-104, mean 78 mmol/l in random samples). Two patients required short courses of dialysis. All cases recovered completely with return of serum creatinine to normal values after a mean duration of 25 days. After a normal serum creatinine level had been achieved, 1 case was lost to follow-up, and the other 3 cases maintain normal renal and liver function tests 9 months (mean) after the initial episode. Otherwise uncomplicated VH is a potential cause of ARF, even in the absence of severe hepatic insufficiency. The mechanism of ARF in VH is unknown, but vasoconstriction phenomena induced by endotoxemia might contribute.