Ultrasound-guided percutaneous liver biopsy in children. Five-year experience at a tertiary care center.

INTRODUCTION AND AIMS: Percutaneous liver biopsy with histopathologic analysis is a valuable tool for the diagnosis, prognosis, and treatment evaluation of liver diseases. Its ultrasound-guided performance is useful, making the procedure safer and reducing the risk for complications and hospital stay. Our aim was to describe the indications, histopathologic study, and complications associated with the performance of ultrasound-guided percutaneous liver biopsy in pediatric patients. MATERIAL AND METHODS: The study included 102 ultrasound-guided percutaneous liver biopsies performed on patients <16 years of age, within the time frame of January 2014 and December 2019. The information was obtained from electronic files and histopathologic studies and the data were analyzed through descriptive statistics. RESULTS: A total of 102 procedures were carried out on 99 patients. Mean patient age was 72 months and 58.8% of the patients were female. Over 65% of the indications for liver biopsy included autoimmune hepatitis (23.5%), elevated liver enzymes (21.5%), and chronic liver disease (20.5%). Four patients presented with immediate complications (3.9%), three of which were major (2.9%), concurring with that reported in the international literature. CONCLUSIONS: Our study corroborates the importance of ultrasound-guided liver biopsy in the diagnosis and follow-up of pediatric patients. The procedure also had a low complication rate of only 3.9%.

Ultrasound-guided percutaneous liver biopsy in children. Five-year experience at a tertiary care center. / Biopsia hepática percutánea guiada por ultrasonido en pediatría. Experiencia de 5 años en un centro de tercer nivel.

INTRODUCTION AND AIMS: Percutaneous liver biopsy with histopathologic analysis is a valuable tool for the diagnosis, prognosis, and treatment evaluation of liver diseases. Its ultrasound-guided performance is useful, making the procedure safer and reducing the risk for complications and hospital stay. Our aim was to describe the indications, histopathologic study, and complications associated with the performance of ultrasound-guided percutaneous liver biopsy in pediatric patients. MATERIAL AND METHODS: The study included 102 ultrasound-guided percutaneous liver biopsies performed on patients <16 years of age, within the time frame of January 2014 and December 2019. The information was obtained from electronic files and histopathologic studies and the data were analyzed through descriptive statistics. RESULTS: A total of 102 procedures were carried out on 99 patients. Mean patient age was 72 months and 58.8% of the patients were female. Over 65% of the indications for liver biopsy included autoimmune hepatitis (23.5%), elevated liver enzymes (21.5%), and chronic liver disease (20.5%). Four patients presented with immediate complications (3.9%), three of which were major (2.9%), concurring with that reported in the international literature. CONCLUSIONS: Our study corroborates the importance of ultrasound-guided liver biopsy in the diagnosis and follow-up of pediatric patients. The procedure also had a low complication rate of only 3.9%.

Health-related quality of life in patients with spinocerebellar ataxia. / Calidad de vida relacionada con la salud en pacientes con ataxias espinocerebelosas.

INTRODUCTION: The progressive deterioration of patients with spinocerebellar ataxia (SCA) has a major impact on their health-related quality of life (HRQOL). This study evaluates HRQOL in a sample of patients diagnosed with SCA and aims to estimate the predictive ability of a set of sociodemographic variables for the different dimensions of the General Health Questionnaire. METHODS: A total of 80 patients diagnosed with SCA were assessed using a sociodemographic questionnaire and the SF-36 General Health Questionnaire. The sociodemographic variables studied were sex, age, presence of a carer, employment status, and time elapsed from diagnosis of the disease. RESULTS: The 8 subscales of the SF-36 show positive and significant correlations to one another. Mean scores obtained on each SF-36 subscale differ between women and men, although this difference is significant only on the general health subscale, with men scoring higher than women. We found significant age differences on the vitality and social function subscales, with higher scores among younger patients (< 34 years). The variable 'presence of a carer' accounts for most of the total variance of the questionnaire. CONCLUSIONS: The SF-36 is a valid and useful instrument for evaluating HRQOL in patients diagnosed with SCA. Presence of a carer seems to be a determinant of self-perceived quality of life in these patients.

[Health-related quality of life in patients with amyotrophic lateral sclerosis]. / Calidad de vida relacionada con la salud en pacientes con esclerosis lateral amiotrófica.

INTRODUCTION: Progressive deterioration in patients with amyotrophic lateral sclerosis (ALS) has a major impact on their health-related quality of life (HRQOL). The objectives of this study are to evaluate HRQOL in a sample of patients diagnosed with ALS and estimate the predictive capability of a set of sociodemographic variables for the different scales covered by a general health survey. METHODS: A total of 63 patients diagnosed with ALS were assessed using a sociodemographic questionnaire and the SF-36 general health survey. The sociodemographic variables studied were sex, age, presence of a caregiver, employment status, and time from diagnosis of disease. RESULTS: The SF-36 survey shows positive correlations between the different scales composing it, which proves its reliability. The mean scores obtained for each of the SF-36 scales were higher in men than in women, although the only statistically significant difference was for the Physical Role scale. The lowest age range (less than 56 years) presented the highest mean scores for most of these dimensions. Most of the variance in the test is explained by the variable 'presence of caregiver'. CONCLUSIONS: The SF-36 health survey has been confirmed as a valid and useful tool for evaluating HRQOL in ALS patients, and it discriminates between patients in different states of health according to their level of dependency.

[Nitrogen and protein content analysis of human milk, diurnality vs nocturnality]. / Análisis del contenido en nitrógeno y proteínas de leche materna, día vs noche.

Breast milk is changing with the progression of lactation and during a 24-h period. To determine the effect of diurnality or nocturnality on total nitrogen and protein content of the breast milk. We collected human milk samples from health mothers living throughout Community of Extremadura (Spain) from January 2008 to December 2008 with less than two months of lactation. We divided the samples in three groups: calostral group (1-5 days postpartum), transitional group (6-15 days postpartum) and mature group (> 15 days postpartum). All samples were stored in a freezer at -80 ºC. We considered as day period between 08:00-20:00 h and night period 20:00-08:00 h. Analysis of the human milk samples was based on the Kjeldahl method. Protein contents were calculated from total nitrogen x 6,25. The statistical analysis of the data was descriptive (mean ± standard deviation) and inferential (T-Student test). No differences (P > 0,05) were found to exist among the contents of individual human milk samples. The mean contents of each component were as follows: Total nitrogen of calostral, transitional and mature group was 0,30 ± 0,06 g/dL (night period), 0,29 ± 0,05 g/dL (day period); 0,26 ± 0,04 g/dL (night period), 0,25 ± 0,04 g/dL (day period); 0,22 ± 0,05 g/dL (night period), 0,20 ± 0,04 g/dL (day period) respectively, in this mature group with a statistical variation (P < 0,05). Protein content of calostral, transitional and mature group was 1,88 ± 0,4 g/dL (night period), 1,81 ± 0,3 g/dL (day period); 1,62 ± 0,3 g/dL (night period), 1,59 ± 0,3 g/dL (day period); 1,35 ± 0,3 g/dL (night period), 1,26 ± 0,3 g/dL (day period) respectively, in this mature group with a statistical variation (P < 0,05). Although we observed differences in the nitrogen and protein content during the individual stages of lactation, it is just in the population of mature lactating women, where the components analyzed varied significantly between day and night.

Effects of oral administration of L-methionine on activity/rest rhythm.

We describe a chronobiological study of the effects of the oral administration of the essential amino acid L-methionine to common quail ( Coturnix coturnix ). This amino acid is a precursor of the neurotransmitter acetylcholine which is responsible for controlling and maintaining wakefulness through the ventrolateral pre-optic area of the hypothalamus and controlling the REM sleep in the nucleus reticularis pontinus oralis (NRPO). The quail model was chosen as these birds are monophasic and active by day, as are humans. The animals were kept under a constant 12h:12h light/dark cycle, fed ad libitum and housed in separate cages equipped for activity recording. Methionine was administered daily (1 h before lights off) for 1 week (chronic treatment), with the birds divided into 4 groups: a capsule with 15 mg of L-methionine (Met15 treatment group); a capsule with 30 mg of L-methionine (Met30 treatment group); a capsule with methylcellulose as excipient (control group); no capsule (basal group). In addition, we compared the first day of treatment (acute experiment) with the basal and control results. Actimetry (DAS24) was used to quantify the activity data, and the sleep/wake rhythm was analyzed using the Ritme software package. The statistical analysis of the activity data was descriptive (+/- SD) and inferential (Tukey test). The data showed increased (p<0.05) mean diurnal activity pulses in the Met30 group versus the other groups in both the acute and the chronic experiments. No changes were found in nocturnal activity. The chronobiological analysis showed a significant increase in the MESOR parameter of the Met30 group in both chronic and acute experiments versus the other groups. The acrophase showed no significant changes, in all groups being at around 13:45 h. In conclusion, the oral administration of L-methionine increased diurnal activity; probably due to the stimulating neuromodulatory action of acetylcholine.

[Influence of coronary anatomy on the anatomic repair of transposition of great arteries]. / Influencia de la anatomía coronaria en la corrección anatómica de la transposición de grandes arterias.

INTRODUCTION: The anatomic diversity of the coronary artery and the alignment of the aortic and pulmonary valves have special importance on the surgical anatomic correction of the transposition of the great arteries and double outlet of the right ventricle, because of their repercussions on surgical technique and the effect on the operative results. OBJECTIVE: To determine the coronary artery pattern; the aortic and pulmonary valve alignment, and their effects on the surgical results in 57 patients submitted to anatomic correction in our hospital. METHOD: We used the Yacoub and Radley-Smith patterns in order to classify the different coronary distributions. The relation between the coronary pattern and degree of alignment with surgical difficulty and the mortality rate was examined. The coronary pattern established by echocardiography was compared with the surgical findings in the last 16 patients. RESULTS: 31 patients belonged to group A, 3 to B, 2 to C, 19 to D and 2 to E. There was no difference in the extracorporeal circulation time among the groups, although the difficulty in the coronary transfer was not the same among them. Type E mortality rate was 50%, type B 33%, and type D 5.2%. There were no deaths in the other groups. Malalignment was found in the three patients who died. The coronary pattern found by echocardiography was confirmed in 13 patients whose images were quite clear. CONCLUSIONS: Coronary pattern and the malignment affect both surgical difficulty and mortality rate of patients undergoing anatomic correction. Bidimensional-echocardiography is useful to demonstrate the different coronary patterns in most cases.

[Ebstein's anomaly of the tricuspid valve. Apropos 35 cases]. / Anomalía de Ebstein de la válvula tricúspide. A propósito de 35 casos.

Ebstein's anomaly of the tricuspid valve is a relatively uncommon congenital heart defect which consists of an apical displacement of the valvar annulus with a deformity of the septal and posterior leaflets. We report 35 cases studied from 1969 to 1993 (45% males and 55% females). One patient presented a polymalformation syndrome and another had a case of Ebstein's anomaly amongst his siblings. We performed an echocardiography in 31 patients and a catheterization in 15. The mean follow-up was 7.6 years (range: 1 day to 16.1 years). The diagnosis was established during the neonatal period in 66.7%. We found other associated cardiac anomalies in 57.1%, with radiologic cardiomegaly in 80.7%. Upon analysis of the EKGs, the P wave was higher than 0.3 mV in 48.4% and 16.1% showed Wolff-Parkinson-White syndrome. Paroxysmal supraventricular tachycardia developed in 12.9%. Long term cardiotonic treatment was needed in 27.3%. In 24.2% cardiac surgery was performed, including one heart transplantation. The mortality rate was 27.3%. The severity of Ebstein's anomaly is extremely variable, not only in its anatomy, but also in the clinical presentation. The severe neonatal form, the associated cardiac defects and heart failure worsen the prognosis.

[Abnormal origin of the left coronary artery of the pulmonary artery trunk. Report of 2 cases]. / Origen anómalo de la arteria coronaria izquierda del tronco de la arteria pulmonar. Aportación de dos casos.

Two cases are presented with anomalous origin of the left coronary artery from the pulmonary artery. Both cases initiate clinical symptoms in the first three months of life, defined by angina and congestive heart failure. In both cases cardiac catheterization showed the existence of anomalous origin of the left coronary artery and underdeveloped collateral circulation. Reimplantation of the anomalous coronary to ascending aorta on the two patients. Has excellent results on the first patient, but not in the second one who died during surgery.

[Congenital generalized fibromatosis. Presentation of a case and review of the literature]. / Fibromatosis congénita generalizada. Presentación de un caso y revisión de la literatura.

We report a case of congenital generalized fibromatosis in a newborn who presented with a solitary subcutaneous tumour, developing subsequently a widespread generalization (soft tissues, bone and lung). The tumours were found to be fibromatosis pathological on study. A review of the literature related to the clinic aspects and to the prognosis is made.