Improving the quality of care in the molecular era for children and adolescents with medulloblastoma.

PURPOSE: Elevated mortality and morbidity rates persist in pediatric patients with medulloblastoma. We present a clinical audit of a real-world cohort of patients in search for pragmatic measures to improve their management and outcome. METHODS/PATIENTS: All pediatric patients with medulloblastoma treated between 2003 and 2016 at a Spanish reference center were reviewed. In the absence of internationally accepted quality indicators (QIs) for pediatric CNS tumors, diagnostic, therapeutic, survival, and time QIs were defined and assessed. RESULTS: Fifty-eight patients were included, 24% were younger children (< 3 years), 36% high risk (anaplastic, metastasis, or surgical residue > 1.5 cm2), and 40% standard risk. Five-year OS was 59.2% (95% CI 47-75); 5-year PFS 36.4% (95% CI 25-53). Five main areas of quality assurance were identified: diagnosis, global strategy, frontline treatment modalities, outcomes, and long-term and end-of-life care. A set of 34 QIs was developed and applied. Lack of central pathology review, delay in the incorporation of novel molecular markers, and absence of a neurocognitive and quality-of-life evaluation program were some of the audit findings. CONCLUSIONS: This real-world research study resulted in the development of a pragmatic set of QIs, aimed to improve clinical audits and quality of care given to children and adolescents with medulloblastoma. We hope that our findings will serve as a reference to further develop a quality assurance system with specific QIs for pediatric CNS tumors in the future and that this will ultimately improve the survival and quality of life of these patients.

Use of genitofemoral and ilioinguinal and iliohypogastric nerve block during orchiectomy in a post-lung transplant patient. A case report. / Uso de bloqueo genitofemoral en combinación con bloqueo ilioinguinal e iliohipogástrico para orquiectomía en paciente postrasplantado pulmonar. A propósito de un caso.

The case is presented of a post-lung transplant patient, ASA III, proposed for orchiectomy due to testicular cancer. A combination of iliohypogastric (ILH), ilioinguinal (ILI) and genitofemoral (GF) nerve block together with sedation was used as anaesthetic technique. The inguinal area received sensory innervation mainly from ILI, ILH and GF nerves. The genital branch of the GF nerve supplies innervation to skin of the anterosuperior portion of the scrotum. When performing the echo-guided block of GF nerve, it is necessary to identify the spermatic cord, and administer the local anaesthetic on the inside and periphery of the cord. Peripheral nerve blocks are a valid option for complex patients. Its main advantage is the anaesthesia and analgesia level that it provides without the haemodynamic instability associated with general or neuraxial anaesthesia. GF nerve block provides hemi-scrotal anaesthesia, allowing manipulation and intervention in the inguinal-scrotal area, complementing the anaesthesia provided by ILI and ILH nerve blocks.

Brote de queratoconjuntivitis epidémica en un Servicio de Oftalmología / A nosocomial outbreak of epidemic keratoconjunctivitis

We describe a nosocomial outbreak of keratoconjunctivitis at the Ophthalmology Clinic in Hospital Carlos Van Buren, that affected 150 patients, during August and September of 2006. This outbreak was concomitant with a community outbreak produced by the same agent in the city of Valparaiso that affected 681 other patients. Although adenovirus was not isolated in the nosocomial cases, positive cultures were obtained from community cases, so the diagnosis was based on clinical and epidemiological criteria. The infection rate within the Clinic patients was 5.2 percent considering the population that attended the Clinic in this period. The evaluation of patient care practices showed that common risk factors among affected cases were measurement of ocular tension with a tonometer in the Ophthalmology Clinic (without disinfection of the tonometer between patients), contamination of work surfaces (equipment, furniture), and poor compliance of hand hygiene. Control measures adopted were cleaning, disinfection of tonometer, equipment and work surfaces, and reinforcement of hand hygiene measures. With these measures, it was possible to control the nosocomial outbreak, despite the continued outpatient care of community-acquired cases.

Se describe un brote nosocomial de queratoconjun-tivitis epidémica en la Policlínica de Oftalmología del Hospital Carlos Van Burén, que afectó a 150 pacientes, durante los meses de agosto y septiembre de 2006. Este brote se presentó en el contexto de un brote en la ciudad de Valparaíso que afectó a otros 681 pacientes. Aun cuando no se logró aislar adenovirus (ADV) en los casos nosocomiales, se aisló ADV en pacientes del brote comunitario, por lo que el diagnóstico de los casos nosocomiales se basó en criterios clínicos y epidemiológicos. La tasa de ataque del brote nosocomial fue de 5,2 por ciento considerando la población consultante del período. La evaluación de prácticas de atención directa mostró que los factores de riesgo de los casos serían la medición de la tensión ocular mediante tonómetro en la Policlínica de Oftalmología (sin desinfección del tonómetro entre paciente y paciente), contaminación de superficies de trabajo (equipos, mobiliario) y bajo cumplimiento de la higienización de manos. Las medidas de control fueron limpieza, desinfección del tonómetro, equipos y superficies de trabajo, junto a reforzamiento de lavado de manos. Con estas medidas se logró controlar el brote intrahospitalario a pesar de mantenerse las consultas provenientes del brote en la comunidad.

[A nosocomial outbreak of epidemic keratoconjunctivitis]. / Brote de queratoconjuntivitis epidémica en un Servicio de Oftalmología.

We describe a nosocomial outbreak of keratoconjunctivitis at the Ophthalmology Clinic in Hospital Carlos Van Buren, that affected 150 patients, during August and September of 2006. This outbreak was concomitant with a community outbreak produced by the same agent in the city of Valparaiso that affected 681 other patients. Although adenovirus was not isolated in the nosocomial cases, positive cultures were obtained from community cases, so the diagnosis was based on clinical and epidemiological criteria. The infection rate within the Clinic patients was 5.2% considering the population that attended the Clinic in this period. The evaluation of patient care practices showed that common risk factors among affected cases were measurement of ocular tension with a tonometer in the Ophthalmology Clinic (without disinfection of the tonometer between patients), contamination of work surfaces (equipment, furniture), and poor compliance of hand hygiene. Control measures adopted were cleaning, disinfection of tonometer, equipment and work surfaces, and reinforcement of hand hygiene measures. With these measures, it was possible to control the nosocomial outbreak, despite the continued outpatient care of community-acquired cases.

[Cranial lesions due to forceps delivery]. / Lesiones craneales secundarias a parto asistido con forceps.

Deliveries with forceps or vacuum-extraction increase the incidence of perinatal craneoencephalic lesions, for which reason cesarean sections are performed more frequently. We report 3 cases of cranial lesions due to forceps deliveries, 2 with depressed skull fractures and 1 with a depressed fracture and an associated epidural hematoma. Diagnosis is made on clinical and radiological founds with CT scan or MRI. Treatment is surgical and consists of elevation of the depressed fracture and evacuation of the hematoma. The correct use of forceps is very important to avoid this kind of lesions in the newborn, especially in cases of difficult delivery.

[Childhood spinal tumours]. / Tumores espinales infantiles

We report a large series of 48 childhood spine tumors diagnosed and treated at our Hospital between 1986 and 2006. Spinal tumors in children are a rare and heterogeneous condition that frequently are diagnosed late because of their uncharacteristic clinical picture. Symptoms are usually limited to diffuse back pain or spinal deformities, prior to leg paresis or sphincter dysfunction. Diagnosis is usually made with MRI or CT. Treatment is surgical in most cases. The prognosis is variable due to the diverse histological findings and it may require the use of complementary treatments as chemotherapy or radiation.

[Brain-stem tumors in children]. / Tumores de tronco cerebral en la población pediátrica.

We report a large series of brain-stem tumors seen during 18 years of at our hospital. We diagnosed and treated a total of 42 patients between 1988 and 2006; 36 of them were operated with partial resection in most cases. Brain-stem tumors constitute a rare condition with very bad prognosis. A surgical complete resection of the mass is not possible in most cases, so the principal surgical objective is reduction and decompression. The best prognosis is seen in patients with low grade tumors with minimal neurologic deficit. Most of these tumors cause death in a short period, usually one year or less.

The levonorgestrel intrauterine system (Mirena) for treatment of idiopathic menorrhagia. Assessment of quality of life and satisfaction.

OBJECTIVES: To evaluate patient satisfaction and improvement in quality of life (QoL) among women with idiopathic menorrhagia treated with the levonorgestrel intrauterine system (LNG-IUS). STUDY DESIGN: Prospective-observational study with one-year follow-up in which 225 women were enrolled who had a LNG-IUS inserted for control of idiopathic menorrhagia. Bleeding, tolerability, user satisfaction, and health-related QoL (by means of the SF-36 QoL questionnaire) were assessed. RESULTS: There was a statistically significant reduction in the amount of bleeding, an increase of haemoglobin and ferritin levels, and an improved QoL score. A high degree of satisfaction was reported by over 98% of patients. CONCLUSIONS: The LNG-IUS is an effective and well-tolerated treatment modality in idiopathic menorrhagia. The QoL of women treated with the LNG-IUS is markedly improved, causing high levels of patient satisfaction. This IUS can be regarded as a first-choice therapy in idiopathic menorrhagia.

Assessment of cervical involvement in endometrial carcinoma by hysteroscopy and directed biopsy.

The objective of this study was to assess the diagnostic validity of hysteroscopy and directed biopsy for assessing cervical involvement in endometrial carcinoma. We conducted a study of 240 hysteroscopies and 44 cervical biopsies performed on 240 patients with endometrial carcinoma between 2000 and 2005. Hysteroscopy results suggested cervical involvement in 55 cases, of which only 31 were confirmed (a subtotal hysterectomy was performed in two cases, and the resection margins were examined for tumor invasion). In the 31 patients with confirmed cervical involvement, 19 of the hysteroscopy-directed biopsies were positive, 3 were negative, and 3 provided insufficient material. Six patients did not undergo biopsy. In the 24 patients with hysteroscopy false positive, 10 directed biopsies were positive and 9 were negative. Five patients did not undergo biopsy. Hysteroscopy suggested an absence of cervical involvement in 185 patients. Diagnosis was confirmed in 177 cases, and there were eight false negatives. In our case, office hysteroscopy proved to be a valid means of ruling out cervical involvement in endometrial carcinoma. However, it was not a valid means of detecting tumor involvement, even when combined with directed biopsy.

Stability studies of tablets containing 5 mg of policosanol.

The stability studies of tablets containing 5 mg of policosanol, a new cholesterol lowering drug, were conducted to predict an expiration date and to search the appearance of putative degradation products. All quality parameters such as colour, moisture content, hardness, disintegration, policosanol content and microbiological limits of the tablets were assessed. The effect of extreme treatments such as acid and basic hydrolysis, oxidative and photolytic degradation as well as thermal degradation, on the policosanol content was studied. In addition, studies under extreme conditions of storage [(40 +/- 2) degree C and (75 +/- 5)% R.H.] as well as 37, 45, 55 and 60 degrees C combined with 50, 75 and 92% R.H.) and under ambient conditions of storage for climatic zones II and IV were performed. These studies demonstrate that these tablets are a stable pharmaceutical formulation, without significant changes in their quality criteria at the stressed conditions used, so that policosanol content remains unchanged during the entire studies. The chromatographic profile of the samples after 9 months of thermal degradation shows chromatographic peaks that corresponds to the palmitate and stearate esters of octacosanoyl, triacontanoyl and hexacosanoyl, being the only degradation products observed on these studies.

Uso de la prostaglandina E1 para evidenciar la incurvación peneana en el tratamiento quirúrgico del niño hipospádico / Used of the prostanglandin E1 for evidence the curved penis in the treatment quirúrgic of the children hypospádico

El hipospadias es una causa frecuente de consulta externa. Un niño portador de esta malformación constituye motivo de angustia para sus padres pues involucra el área genital. Se plantea estudiar el efecto y consecuencias de la inyección de Prostaglandina E1 para evidenciar la incurvación peneana durante la corrección quirúrgica de esta entidad. Se dan recomendaciones para mejorar la técnica, promover su uso, y utilizarla como alternativa eficaz y con menos efectos colaterales que la prueba de Gittes. Se revisaron las historias clínicas de 50 pacientes que ingresaron al Hospital Ortopédico Infantil, con diagnóstico de hipospadias, de 1 a 12 años de edad, en quienes se usó prostaglandina. El evaluando tipo de hipospadias y curvatura, la dosis indicada, tiempo y duración de la erección, grado de rigidez, turgencia peneana y efectos colaterales. 50 pacientes fueron intervenidos. La mayoría fueron hipospadias distales. En 38 casos (76 por ciento) se evidenció incurvación peneana de mayor o menor grado. 12 pacientes (24 por ciento) sin incurvación. La dosis utilizada fluctuó entre 2,5 a 10 microgramos del medicamento. La erección, se produjo en general a los 4 minutos, con una duración promedio de 60 minutos. Se utilizaron varias técnicas de uretroplastia y en los casos severos de cuerda se uso la técnica de Nesbit para la ortoplastia peneana. En los trabajos de Perovic y Kogan se utilizan dosis mayores de PGE1, destacándose las diferencias al usar el test de Gittes. Se observa las diferentes técnicas utilizadas en uretroplastia. La evaluación preoperatoria de la incurvación peneana en pacientes hipospádicos, usando la prostaglandina. El intracavernosa, permite mejorar el resultado de la ortoplastia peneana, utilizando dosis adecuadas de acuerdo a la edad, con menos efectos colaterales que la prueba de inyección con solución salina

[Innovations and new techniques: tracheostomy cannulas]. / Innovaciones y técnicas nuevas. Cánulas de traqueostomía.

In recent years, therapy by means of a tracheostomy has increased considerably due to the use of new trachea opening procedures and the development of the newest generation of tubes employed in tracheostomy. Percutaneous tracheostomy has become the preferred choice technique for patients in critical condition since it is a simple procedure, almost bloodless, and furthermore, this procedure can be carried out on a patient in a bed in a normal ward. The translaryngeal tracheostomy is one of the newest innovative techniques performed from inside the trachea; due to its complex nature, it is not commonly used yet although it promises to be an alternative procedure when confronting various counter-indications found in the percutaneous technique. In addition to the development of these techniques, substantial design modifications in the tubes used in tracheostomy have been produced; their objectives include improving a patient's comfortableness, reducing complications and easing a nurse's work.

Sutura meniscal artroscópica con agujas: técnica fuera-dentro / Arthroscopic meniscal suture by two needles: qutside in technique

Se presenta una cohorte descriptiva (observacional, prospectiva y longitudinal) de 8 pacientes con lesión meniscal, que se trataron con reparación artroscópica con 2 agujas, mediante nylon 000. La lesión fue de menos de 3 meses de evolución y como mínimo de 10 mm de longitud, en el cuerno anterior, en zona roja-roja o roja-blanca. Cuando hubo lesión ligamentosa se hizo la reconstrucción respectiva. Se colocó un vendaje de Jones por 2 semanas y se difirió la carga de peso hasta después de 6 semanas. Después de un seguimiento de 7 a 14 meses, se encontró desprendimiento de la sutura en dos casos, confirmados por artroscopía, en quienes se resecó el fragmento suelto y se remodeló el fragmento parietal. El resultado general fue bueno en 4 casos (50 por ciento), regular en 2 (25 por ciento) y malo en 2 (25 por ciento).

Mutation analysis in patients with Wilson disease: identification of 4 novel mutations. Mutation in brief no. 250. Online.

In order to obtain novel mutations in the recently discovered Wilson disease gene, we screened 5 unrelated German individuals for mutations in the 21 exons and their flanking intronic sequences. We detected 9 mutations affecting the Wilson disease gene. Four of those, designated 802-808delTGTAAGT, 2008-2013delTATATG, Cys985Thr, and Ile1148Thr have not yet been reported. One patient had a homozygous mutation whereas the remaining four subjects were compound heterozygous. Therefore these data confirm, that mutations causing Wilson disease are frequently found in affected subjects and they are very heterogenous.

[Prospective study with auditory evoked potentials of the brain stem in children at risk]. / Estudio prospectivo con potenciales evocados auditivos de tronco cerebral en niños de riesgo.

OBJECTIVE: The aim of this study was to evaluate methods of hypoacusis screening. PATIENTS AND METHODS: The early detection of audition problems is vital for quick rehabilitation. For this reason, resting on the criteria of the Comisión Española para la Detección Precoz de la Hipoacusia (Spanish Commission for the Early Detection of Hypoacusis), we have carried out a prospective study, from January to May 1998, evaluating patients at risk of suffering from hypoacusis. The study included 151 patients with ages between birth and 14 years. Medical records and brainstem auditory evoked responses (BAER) were carried out. RESULTS: The most common reason for requesting a consultation for the 151 patients included in our study was the suspicion of hypoacusis. Seventy-one (47%) presented pathological BAER, 37 of them were bilateral. In most cases the loss of audition was of cochlear origin, with 11 patients having a serious deafness, 4 with bilateral affection (3 suspicious of hypoacusis and 1 of hyperbilirubinemia) and 7 unilateral deafness. CONCLUSIONS: BAER is a good screening method for children at risk. It is an innocuous, objective and specific test that does not require the patient's collaboration. The level of positives is high (47%).

Induction of tyrosine hydroxylase gene expression in human foetal cerebral cortex.

Human foetal cerebral cortex (9-14 weeks gestational age) was dissected out and cultured in microwell plates. It was then treated with brain-derived neurotrophic factor (BDNF, 50 ng/ml), dopamine (10 mM) or their combination. After 5 weeks of this treatment tyrosine hydroxylase (TH)-immunopositive neurones were detected at a level of 0.73% of total neurones present. This represented 300-500 TH + neurones per microwell. None were seen in untreated cultures. This correlates with induction of the entire dopaminergic phenotype in foetal rat cerebral cortex (E1214) by the same co-treatment applied for a much shorter time period (7 days), which implies that the complete dopaminergic phenotype is also induced in cultured human foetal tissue over a longer period, reflecting the 5-fold longer neuronal gestational period.

[Tracheostomy cannulas]. / Cánulas de traqueostomia.

Tracheostomic clyster pipes are mechanisms which allow one to artificially maintain the permeability of the air passageway under those circumstances when it is not possible to breathe by the conventional way. Nowadays, there is a wide variety of clyster pipes available to medical professionals. Each of these has its own traits and precise applications. This article describes those clyster pipes most frequently used in hospitals and outpatient clinics; furthermore, their use, care and therapeutic applications are mentioned.

Sequential computerized tomography changes and related final outcome in severe head injury patients.

The authors analysed the serial computerized tomography (CT) findings in a large series of severely head injured patients in order to assess the variability in gross intracranial pathology through the acute posttraumatic period and determine the most common patterns of CT change. A second aim was to compare the prognostic significance of the different CT diagnostic categories used in the study (Traumatic Coma Data Bank CT pathological classification) when gleaned either from the initial (postadmission) or the control CT scans, and determine the extent to which having a second CT scan provides more prognostic information than only one scan. 92 patients (13.3% of the total population) died soon after injury. Of the 587 who survived long enough to have at least one control CT scan 23.6% developed new diffuse brain swelling, and 20.9% new focal mass lesions most of which had to be evacuated. The relative risk for requiring a delayed operation as related to the diagnostic category established by using the initial CT scans was by decreasing order: diffuse injury IV (30.7%), diffuse injury III (30.5%), non evacuated mass (20%), evacuated mass (20.2%), diffuse injury II (12.1%), and diffuse injury I (8.6%). Overall, 51.2% of the patients developed significant CT changes (for worse or better) occurring either spontaneously or following surgery, and their final outcomes were more closely related to the control than to the initial CT diagnoses. In fact, the final outcome was more accurately predicted by using the control CT scans (81.2% of the cases) than by using the initial CT scans (71.5% of the cases only). Since the majority of relevant CT changes developed within 48 hours after injury a pathological categorization made by using an early control CT scan seems to be most useful for prognostic purposes. Prognosis associated with the CT pathological categories used in the study was similar independently of the moment of the acute posttraumatic period at which diagnoses were made.

Idiopathic normal pressure hydrocephalus: analysis of factors related to cerebrospinal fluid dynamics determining functional prognosis.

This investigation has been undertaken to analyze the findings with both the cerebrospinal fluid (CSF) pressure (Pcsf) and CSF pulse pressure (PP) in order to predict the outcome of patients with the syndrome of idiopathic normal pressure hydrocephalus (NPH). Accordingly, a prospective clinical study was planned in which two groups of patients with NPH, having analogous prevalence of several matched clinical and radiological parameters, were separated on the basis of their positive or negative response to shunting. Both the resting Pcsf and CSF PP profiles were compared in these two groups, and between them and normal controls. CSF PP amplitude and CSF PP latency correlated directly in conditions associated with either normal or high compliance (controls and patients with Alzheimer-like disorders), whereas this correlation was inverse in states of low compliance (NPH). On the other hand, shunt-responders showed a resting Pcsf significantly higher than both non-responders and controls. The following conclusions were obtained: 1) CSF PP is a high-amplitude and relative low-latency wave in NPH when compared with controls: 2) CSF PP amplitude and latency correlate directly in normal subjects and in those with primary cerebral atrophy; 3) a non-reversible stage of NPH could be conceived in contradistinction to the reversible one, in both of which an inverse correlation between the amplitude and the latency takes place, the main difference between them being the resting Pcsf, which is significantly lower in the former than in the latter, depending on the degree of atrophic changes developed.