Meta-analysis of randomized controlled trials comparing different techniques with primary closure for chronic pilonidal sinus.

BACKGROUND: There are different open healing and primary closure approaches for chronic pilonidal sinus (CPD) that differ in principles and extension. AIMS: To compare the results of different closure surgical techniques, we performed a meta-analysis of randomized controlled trials (RCT) comparing: (1) open wide excision versus open limited excision (sinusectomy) or unroofing (sinotomy); (2) midline closure (conventional and tension-free) versus off-midline; (3) advancing versus rotation flaps; and (4) sinusectomy/sinotomy versus primary closure. METHODS: Data extraction and risk of bias assessment were conducted independently by the authors using the Cochrane Collaboration's tool. Data were pooled using fixed and random-effects models. Primary outcomes were rate of healing, recurrence, wound infection and dehiscence. Twenty-five trials (2,949 patients) were included. RESULTS: Four trials compared limited versus radical open healing. Although recurrence rate did not differ, all other outcomes favored the limited approach. Ten studies compared midline versus off-midline primary closure; wound infection and dehiscence were significantly higher after midline closure. Six RCT compared Karydakis/Bascom versus Limberg. No difference was found in recurrence or wound complications rate. Six RCT compared sinusectomy/sinotomy versus primary closure. Recurrence rate was significantly lower after sinusectomy/sinotomy; no significant differences were found in other outcomes. CONCLUSION: Our meta-analysis suggest that some of the questions of which is the best surgical technique for CPD have now been answered: open radical excision and primary midline closure should be abandoned. Sinusotomy/sinectomy or en bloc resection with off midline primary closure are the preferred approaches.

[Standardization of the quantification of iron concentration in the liver by magnetic resonance imaging]. / Estandarización de la cuantificación de la concentración de hierro en el hígado por resonancia magnética.

OBJECTIVE: To calibrate 1.5 tesla magnetic resonance scanners for the quantification of the concentration of iron in the liver. MATERIAL AND METHODS: We analyzed twenty-eight 1.5 tesla magnetic resonance scanners using a phantom with four tubes containing different concentrations of iron (III) chloride and one tube without iron. The phantom represented two typical patients: one with moderate iron overload and one with high iron overload. We measured the signal intensity ratio between each iron-containing tube and the tube without iron; then we calculated the theoretical levels of iron concentration in each scanner according to the model for the two levels of overload. We compared the results of each scanner with those of the reference scanner in which the model and the phantom had been designed, and we calculated the percentage of difference between the two scanners. RESULTS: The mean difference in the ratios compared to the reference center was 11% (0.3-39). The mean concentration of iron was 71 µmol Fe/g for moderate overload and 193 µmol Fe/g for high overload. The mean difference was 6% (1.2- 7%) and 3.4% (0-16%). respectively. In two scanners, we applied a correction factor so that the difference was below 25% in all cases. CONCLUSION: We calibrated twenty-eight 1.5 tesla scanners for the concentration of iron in the liver and achieved variability less than 25%.

Cognitive/personality pattern and triplet expansion size in adult myotonic dystrophy type 1 (DM1): CTG repeats, cognition and personality in DM1.

BACKGROUND: Although central nervous system (CNS) involvement in adult myotonic dystrophy type 1 (DM1) was described long ago, the large number of variables affecting the cognitive and personality profile have made it difficult to determine the effect of DM1 on the brain. The aim of this study was to define the cognitive and personality patterns in adult DM1 patients, and to analyse the relationship between these clinical patterns and their association with the underlying molecular defect. METHOD: We examined 121 adult DM1 patients with confirmed molecular CTG repeat expansion and 54 control subjects using comprehensive neuropsychological tests and personality assessments with the Millon Clinical Multiaxial Inventory (MCMI)-II. We used a multiple linear regression model to assess the effect of each variable on cognition and personality adjusted to the remainders. RESULTS: Patients performed significantly worse than controls in tests measuring executive function (principally cognitive inflexibility) and visuoconstructive ability. In the personality profile, some paranoid and aggressive traits were predominant. Furthermore, there was a significant negative correlation between the CTG expansion size and many of the neuropsychological and personality measures. The molecular defect also correlated with patients' daytime somnolence. CONCLUSIONS: Besides muscular symptomatology, there is significant CTG-dependent involvement of the CNS in adult DM1 patients. Our data indicate that the cognitive impairment predominantly affects the fronto-parietal lobe.

[Forced spirometry in healthy preschool children]. / Espirometría forzada en preescolares sanos bajo las recomendaciones de la ATS/ERS: estudio CANDELA.

INTRODUCTION: Reference values for spirometry in healthy preschool children have not yet been obtained in accordance with American Thoracic Society (ATS) and the European Respiratory Society (ERS) guidelines. The objective was to establish reference values for spirometry in healthy preschoolers under the ATS/ERS 2007 statement. MATERIAL AND METHOD: Children of at least 2 and under 7 years of age were tested in 9 pediatric pulmonary function laboratories. The technicians were trained to apply a standardized protocol to perform spirometry. RESULTS: Valid spirometry results were obtained in 455 (81.54%) out of 558 children: 242 boys (53.2%) and 213 girls (46.8%). Ages were as follows: 31 at least 2 and under 3 years old; 96, at least 3 and under 4; 108, at least 4 and under 5; 122, at least 5 and under 6 years, and 98, at least 6 and under 7 years. Formulas were used to calculate the reference values for all the spirometry variables in preschoolers. CONCLUSIONS: Spirometry is feasible in the majority of preschool children under the new guidelines. The availability of the reference values presented is an important step, both for the care of preschoolers and for further research on pulmonary function.

Minimizing creatine kinase variability in rats for neuromuscular research purposes.

Rat serum or plasma creatine kinase (CK) activity is widely used to evaluate myopathic processes, to test the myotoxicity of different drugs, or to analyse the benefits of emerging gene therapies in some neuromuscular disorders. However, great variability is found in this determination. The aim of this study has been to control some factors of variation in order to reduce variability and increase the reproducibility of analytical data. 8-10-week-old Wistar-Han rats were used. The study consisted of four sequential phases. Phase I aimed to analyse the effect of ether and isoflurane as anaesthetic drugs. The objective of Phase II was to evaluate bleeding rats via retro-orbital sinus vs. tail vein. Phases III and IV were designed as two separate, repeated measure experiments on two factors: habituation to laboratory handling procedures in Phase III and gender in Phase IV. The repeated factor was the storage temperature of blood sample prior to centrifugation. Ether did not significantly increased the CK value. Using isoflurane, getting rats accustomed to laboratory handling procedures and whole blood refrigeration prior to centrifugation and serum separation resulted in statistically significant reduction in CK value and variability. Male rats showed significantly higher values than female rats. In the light of our findings, CK value and variability in rats may be minimized by choosing tail vein as site of bleeding, getting rats accustomed to laboratory handling procedures and maintaining whole blood refrigerated until centrifugation and serum separation.

Indirect costs associated with glioblastoma: Experience at one hospital. / Costes indirectos asociados al glioblastoma. Experiencia en un centro.

INTRODUCTION: Glioblastoma is the most common primary brain tumour. Despite advances in treatment, its prognosis remains dismal, with a mean survival time of about 14 months. Many articles have addressed direct costs, those associated with the diagnosis and treatment of the disease. Indirect costs, those associated with loss of productivity due to the disease, have seldom been described. MATERIAL AND METHOD: We conducted a retrospective study in patients diagnosed with glioblastoma at Hospital Universitario Donostia between January 1, 2010 and December 31, 2013. We collected demographics, data regarding the treatment received, and survival times. We calculated the indirect costs with the human capital approach, adjusting the mean salaries of comparable individuals by sex and age and obtaining mortality data for the general population from the Spanish National Statistics Institute. Past salaries were updated to 2015 euros according to the annual inflation rate and we applied a discount of 3.5% compounded yearly to future salaries. RESULTS: We reviewed the records of 99 patients: 46 women (mean age 63.53) and 53 men (mean age 59.94); 29 patients underwent a biopsy and the remaining 70 underwent excisional surgery. Mean survival was 18.092 months for the whole series. The total indirect cost for the series was €11 080 762 (2015). Mean indirect cost per patient was €111 926 (2015). DISCUSSION: Although glioblastoma is a relatively uncommon type of tumour, accounting for only 4% of all cancers, its poor prognosis and potential sequelae generate disproportionately large morbidity and mortality rates which translate to high indirect costs. Clinicians should be aware of the societal impact of glioblastoma and indirect costs should be taken into account when cost effectiveness studies are performed to better illustrate the overall consequences of this disease.

Apolipoprotein E epsilon4 allele in familial and sporadic Parkinson's disease.

Parkinson's disease (PD) is the second most common age-related neurodegenerative disease after Alzheimer's disease (AD). Common risk factors for both diseases have been explored to study potential etiologic interactions between these two neurodegenerative disorders. The APOE epsilon4 allele, previously associated with AD, has also been associated with risk of PD and with the presence of some clinical features in PD patients. However, the role of APOE epsilon4 allele in risk of PD remains unclear. We studied the distribution of APOE alleles in 276 unrelated familial and sporadic PD patients and in 212 controls. Patients and controls were classified by ethnicity. No genetic heterogeneity between Basques and people from other regions of Spain was found. No significant differences in APOE allele distribution between PD patients and controls were found; however, lower epsilon4 allele frequency was observed when the sporadic PD group was analyzed separately. By contrast, an increase in epsilon4 allele frequency was found in familial PD patients with cognitive decline. We conclude that the APOE epsilon4 allele may be associated with the risk of developing PD in isolated cases and that it is linked to the presence of cognitive decline in familial PD in our sample.

Effect of vitamin D and calcium supplementation on bone turnover in institutionalized adults with Down's Syndrome.

OBJECTIVE: To assess the status of vitamin D and the effects of calcium and vitamin D3 supplementation on the bone metabolism in a group of adults with Down's syndrome (DS). DESIGN: Randomized, parallel, controlled and open clinical trial. SETTING: Institution for mentally handicapped: Fundación Uliazpi, Diputación Foral de Guipúzcoa, San Sebastián, Spain. SUBJECTS: A total of 23 persons with DS, residents at the Uliazpi Foundation were recruited and all completed the study. INTERVENTION: In all, 12 participants were randomly allocated to receive 1 g of calcium and 800 IU of vitamin D once daily for 1 year while 11 were assigned to the control group, receiving no supplementation. RESULTS: We found no differences between groups regarding serum calcium and phosphorous levels. The remaining parameters showed differences between the two groups consistent with a beneficial effect of the intervention: serum levels of parathyroid hormone, osteocalcin and crosslaps diminished while serum 25 OH vitamin D3 level increased. CONCLUSIONS: The results obtained allow to include people with DS as a risk group with regards to vitamin D deficit, which that can be corrected with vitamin D and calcium supplementation, and giving rise to an improvement of the biochemical markers related to the phospho-calcium metabolism and bone remodelling.

LGMD2A: genotype-phenotype correlations based on a large mutational survey on the calpain 3 gene.

We present here the clinical, molecular and biochemical findings from 238 limb-girdle muscular dystrophy type 2A (LGMD2A) patients, representing approximately 50% (238 out of 484) of the suspected calpainopathy cases referred for the molecular study of the calpain 3 (CAPN3) gene. The mean age at onset of LGMD2A patients was approximately 14 years, and the first symptoms occurred between 6 and 18 years of age in 71% of patients. The mean age at which the patients became wheelchair bound was 32.2 years, with 84% requiring the use of a wheelchair between the age of 21 and 40 years. There was no correlation between the age at onset and the time at which the patient became wheelchair bound, nor between the sex of the patient and the risk of becoming wheelchair bound. Of the cases where the CAPN3 gene was not affected, approximately 20% were diagnosed as LGMD2I muscular dystrophy, while facioscapulohumeral muscular dystrophy (FSHD) was uncommon in this sample. We identified 105 different mutations in the CAPN3 gene of which 50 have not been described previously. These were distributed throughout the coding region of the gene, although some exons remained free of mutations. The most frequent mutation was 2362AG-->TCATCT (exon 22), which was present in 30.7% of the chromosomes analysed (146 chromosomes). Other recurrent mutations described were N50S, 550DeltaA, G222R, IVS6-1G-->A, A483D, IVS17+1G-->T, 2069-2070DeltaAC, R748Q and R748X, each of which was found in >5 chromosomes. The type of mutation in the CAPN3 gene does not appear to be a risk factor for becoming dependent on a wheelchair at a determined age. However, in the cases with two null mutations, there were significantly fewer patients that were able to walk than in the group of patients with at least one missense mutation. Despite the fact that the results of phenotyping and western blot might be biased due to multiple referral centres, producing a diagnosis on the basis of the classical phenotype is neither sufficiently sensitive (86.7%) nor specific (69.3%), although western blot proved to be even less sensitive (52.5%) yet more specific (87.8%). In this case LGMD2I was a relevant cause of false-positive diagnoses. Considering both the clinical phenotype and the biochemical information together, the probability of correctly diagnosing a calpainopathy is very high (90.8%). However, if one of the analyses is lacking, the probability varies from 78.3 to 73.7% depending on the information available. When both tests are negative, the probability that the sample comes from a patient with LGMD2A was 12.2%.

Serum carboxy-terminal propeptide of procollagen type I is a marker of myocardial fibrosis in hypertensive heart disease.

BACKGROUND: This study was designed to investigate whether the serum concentration of the carboxy-terminal propeptide of procollagen type I (PIP), a marker of collagen type I synthesis, is related to myocardial fibrosis in hypertensive patients. METHODS AND RESULTS: The study was performed in 26 patients with essential hypertension in which ischemic cardiomyopathy was excluded after a complete medical workup. Right septal endomyocardial biopsies were performed in hypertensive patients to quantify collagen content. Collagen volume fraction (CVF) was determined on picrosirius red-stained sections with an automated image analysis system. The serum concentration of PIP was measured by specific radioimmunoassay. Compared with normotensives, both serum PIP and CVF were increased (P<0.001) in hypertensives. A direct correlation was found between CVF and serum PIP (r=0.471, P<0.02) in all hypertensives. Histological analysis revealed the presence of 2 subgroups of patients: 8 with severe fibrosis and 18 with nonsevere fibrosis. Serum PIP was higher (P<0.05) in patients with severe fibrosis than in patients with nonsevere fibrosis. Using receiver operating characteristic curves, we observed that a cutoff of 127 microg/L for PIP provided 78% specificity and 75% sensitivity for predicting severe fibrosis with a relative risk of 4.80 (95% CI, 1.19 to 19.30). CONCLUSIONS: These results show a strong correlation between myocardial collagen content and the serum concentration of PIP in essential hypertension. Although preliminary, these findings suggest that the determination of PIP may be an easy and reliable method for the screening and diagnosis of severe myocardial fibrosis associated with arterial hypertension.

Skin to calcaneus distance in the neonate.

BACKGROUND: Current recommendations for obtaining blood from neonates advise avoidance of the midline area of the heel and are based on postmortem studies. OBJECTIVE: Because of the potential pain and tissue damage from repeated heel pricking in the same area, to investigate using ultrasonography whether the distance from skin to calcaneus is less at the midline than at the borders. METHODS: One hundred consecutive healthy preterm and 105 consecutive healthy term neonates were studied 48-72 hours after delivery. The skin to perichondrium distance (SPD) was measured on two occasions by ultrasound at the external, midline, and internal areas of the heel. FINDINGS: Mean SPD was 0.2 mm less at the midline than at the other sites. The proportion of measurements <3 mm at any of the three sites was the same. Depth was <3 mm in less than 3% of the term and approximately 20% of the preterm infants. The SPD correlated only with gestational age. Of children <33 weeks gestational age, 38% had an SPD <3 mm compared with 8% of older preterm infants. The proportions of preterm infants of > or = 33 weeks gestation and term infants with an SPD <3 mm were similar (8% v 3%). INTERPRETATION: With the use of automated lancets of 2.2 mm length or less, the whole heel plantar surface is safe for obtaining blood in term and preterm infants of > or = 33 weeks gestation. This means that soft tissue damage and pain from repeated pricking in the same area can be reduced.

Prevalence of myotonic dystrophy in Guipúzcoa (Basque Country, Spain).

Prevalence figures for inherited neuromuscular disorders are important both for health care planning purposes and for evaluating the need for DNA diagnostic services for eugenic approaches. We screened for the prevalence of myotonic dystrophy (MyD) through extensive inquiry of neurologic and primary health services of Guipúzcoa (Basque Country, northern Spain) between 1989 and 1991. Typical adult-onset and neonatal cases and relatives at risk; suffering from a partial syndrome, were included. In the latter, molecular typing was performed with DNA probes close to the MyD gene to demonstrate the MyD gene carrier status. The high prevalence detected (26.5 cases per 100,000 population) could be explained by methodological factors, but intrinsic factors, such as a possible founder genetic effect or the quick growth of the Guipúzcoa population since the last century may contribute to one of the highest MyD prevalence in the world. In the future, the methodological basis for epidemiologic surveys of MyD must combine molecular technology with more-extensive family inquiries.

Antibodies to hepatitis C virus: a cross-sectional study in patients attending a trauma unit or admitted to hospital for elective surgery.

OBJECTIVE: To assess the prevalence of hepatitis C virus (HCV) antibodies in patients attending a trauma unit or admitted to hospital for elective surgery, and to identify risk factors for acquiring HCV infection. DESIGN: A cross-sectional study. SETTING: The emergency room of a Department of Orthopaedic Surgery and a hospital for elective (non-digestive) surgery. PATIENTS: A study population of 1824 subjects (mean age 40.1 +/- 18.1 years) and sera from a control group of 4703 blood donors giving their first donation. INTERVENTIONS: Serum samples were screened for HCV antibodies by second-generation enzyme-linked immunosorbent assays and recombinant immunoblot assays. MAIN OUTCOME MEASURES: HCV serostatus and the relationship between anti-HCV-positivity and age, intravenous drug use, tattoos, blood transfusion, a history of hepatitis and exposure to a contact who had had hepatitis. RESULTS: The overall prevalence of anti-HCV antibodies was 2.85% in the study population and 0.5% among blood donors (chi 2 = 64.5; P < 0.0001). Anti-HCV-positivity was more common in men (3.8 versus 1.9%; P < 0.05) and in subjects with history of hepatitis (13.5 versus 2.0%; P < 0.001), exposure to a contact who had had hepatitis (12.5 versus 1.9%; P < 0.001), blood transfusion (7.8 versus 2.3%; P < 0.001), tattoos (36 versus 2.4%; P < 0.001) and intravenous drug use (85.7 versus 1.9%; P < 0.001). However, one third of subjects had no risk factors for HCV infection. The logistic regression analysis confirmed a statistically significant association (P < 0.05) between anti-HCV-positivity and intravenous drug use, tattoos, blood transfusion, a history of hepatitis and exposure to a contact who had had hepatitis. CONCLUSIONS: These results show almost a five-fold higher prevalence of anti-HCV among patients attending a trauma unit or admitted to hospital for elective surgery than among blood donors.

Strategies for increasing women participation in community breast cancer screening.

BACKGROUND: Strategies for reducing breast cancer mortality in western countries have focused on screening, at least for women aged 50 to 69 years. One of the requirements of any community screening program is to achieve a high participation rate, which is related to methods of invitation. Therefore, it was decided to systematically review the scientific evidence on the different strategies aimed at improving women's participation in breast cancer screening programs and activities. OBJECTIVES: To assess the effectiveness of different strategies for increasing the participation rate of women invited to community (population-based) breast cancer screening activities or mammography programs. SEARCH STRATEGY: MEDLINE (1966-2000), CENTRAL (2000), and EMBASE (1998-1999) searches for 1966 to 1999 were supplemented by reports and letters to the European Screening Breast Cancer Programs (Euref Network). SELECTION CRITERIA: Both published and unpublished trials were eligible for inclusion, provided the women had been invited to a community breast screening activity or program and had been randomised to an intervention group or a control group with no active intervention. DATA COLLECTION AND ANALYSIS: We identified 151 articles, which were reviewed independently by two people. The discrepancies were resolved by a third reviewer in order to reach consensus. Thirty-four studies were excluded because they lacked a control group; 58 of the other 117 articles were considered as opportunistic and not community-based; 59 articles, which reported 70 community-based randomised controlled trials or clinical controlled trials, were accepted. In 24 of these, the control group had not been exposed to any active intervention, but 8 of the 24 had to be excluded because the denominator for estimating attendance was unknown. At the end, 16 studies constituted the material for this review, although two studies were further excluded because their groups were not comparable at baseline. Data from all but one study were based on or converted to an intention-to-treat analysis. Attendance in response to the mammogram invitation was the main outcome measure. MAIN RESULTS: The evidence favoured five active strategies for inviting women into community breast cancer screening services: letter of invitation (OR 1.66, 95% CI 1.43 to 1.92), mailed educational material (OR 2.81, 95% CI 1.96 to 4.02), letter of invitation plus phone call (OR 2.53, 95% CI 2.02 to 3.18), phone call (OR 1.94, 95% CI 1.70 to 2.23), and training activities plus direct reminders for the women (OR 2.46, 95% CI 1.72 to 3.50). Home visits did not prove to be effective (OR 1.06, 95 % CI 0.80 to 1.40) and letters of invitation to multiple examinations plus educational material favoured the control group (OR 0.62, 95 % CI 0.32 to 1.20). REVIEWER'S CONCLUSIONS: Most active recruitment strategies for breast cancer screening programs examined in this review were more effective than no intervention. Combinations of effective interventions can have an important effect. Some costly strategies, as a home visit and a letter of invitation to multiple screening examinations plus educational material, were not effective. Further reviews comparing the effective interventions and studies that include cost-effectiveness, women's satisfaction and equity issues are needed.

Characteristics of continuous esophageal pH-metering in infants with gastroesophageal reflux and apparent life-threatening events.

Episodes of apnea during the first year of life have been classified with the term "Apparent Life-Threatening Events" (ALTE). Gastroesophageal reflux (GER) has been accepted to be one of the factors which can favor ALTE. The aim of this work is to study the continuous 24 h gastroesophageal pH-metering (fundamentally the Reflux Index [RI] and the Area Under Curve [AUC] parameters in three different periods of time: total pH-metering, pH-metering excluding the first two post-prandial hours, and pH-metering during sleep time, for 24 infants, 14 with ALTE and 10 without it). Between these two groups (with and without ALTE) there were no significant differences in the total pH-metering concerning the RI (2 +/- 1.2 vs 5.6 +/- 7.5 p > 0.05) and concerning the AUC (24.9 +/- 14.4 vs 67.4 +/- 84 p > 0.05), but in the analysis of the sleep period, the RI (p < 0.05) and the AUC (p < 0.01) both showed significant differences in the group of infants without ALTE. These results confirm that pH-metering study during sleep is the procedure of choice for recognizing infants with ALTE.

A simple severity index for pressure ulcers.

The aim of this study was to design and test a pressure ulcer severity score based on assessment by experienced clinicians. Fifty pressure ulcers were each evaluated independently by seven experienced clinicians, using a scale that ranged from 0 (mildest damage) to 10 (most severe). Ulcer characteristics were analysed to determine which factors related to the assessments. A second set of 50 ulcers was used to test the validity of the resulting scoring system. Inter- and intra-observer reliability were tested in a further 10 pressure ulcers. A multivariate linear model was produced, based on the following three variables: colour (CO-matched-matched) in the wound base; depth (DE) of the ulcer; and mean diameter (D) of the wound ('CODED'). The coefficients for each variable were rounded to yield points in an additive equation, the CODED score. This score had a high correlation (r = 0.92, P < 0.001) with the second set of evaluations, used to test the score performance. The CODED score is a simple and valid tool to quantify the progress of a pressure ulcer. It has a high correlation with the severity assessment of experienced clinicians and is reliable when used by non-experienced observers.

[Severe primary vesicoureteral reflux in infants. A follow-up of 203 cases]. / El reflujo vesicoureteral primario severo en el primer año de la vida. Revisión de 203 casos.

OBJECTIVE: The aim of our study is to analyze the clinic characteristics and evolution of the primary reflux in infants. METHODS: We studied retrospectively 203 infants in our hospital, diagnosed of severe primary renal reflux. Renal ecography and cyclic mictional cystography were practiced in all cases. DMSA was carried out in 181 patients. RESULTS: Renal reflux was unilateral in the 23% of the patients, and bilateral in the remaining cases; 72% of the renal reflux were grade IV and 28% grade V. The renal injuries affected to male infants and reflux grade V. The renal injury was focal (27%), global (44%) and atrophic (29%). The 79% of the patients had conservative treatment, while 21% had surgical treatment. 100% infants with surgical treatment and 94.2% infants with conservative treatment were recovered (Test of Kaplan-Meier). The 27% of patients developed one or several urinary infections, but progression of old renal injuries or formation of new ones, were exceptional (3 cases): While the time the study lasted none of the patients developed chronic renal failure nor arterial hypertension. CONCLUSIONS: 1) The fetal severe primary reflux of the patients was characterized by the following features: to be bilateral reflux, to affect mainly to male infants and to be associated in 33% of cases with a severe renal injury of congenital origin (renal displasia) most of them unilateral. 2) The natural evolution of the reflux goes to spontaneous recovery, so treatment must be conservative. 3) Some patients underwent urinary infections, but progression or formation of new renal injuries were inusual. None of the patients had terminal renal failure nor hypertension and 4) Risk patients would be male infants with bilateral injuries although these are infrequent.

[Clinical manifestations of myotonic dystrophy: epidemiologic survey]. / Manifestaciones clínicas de la distrofia miotónica: encuesta epidemiológica.

BACKGROUND: The presence of a local aggregation of cases of myotonic dystrophy (MD) allows the evaluation of clinical symptoms of the disease in a sample in which the influence of a possible genetic heterogeneity is decreased. METHODS: The degree of global neuromuscular handicap and the incidence and severity of four of the most characteristic symptoms (cataracts, myotonia, muscular weakness and neuropsycologic disturbances) were studied in 183 patients with MD (146 typical adult forms, 19 neonatal, and 18 partial syndromes) in relation with the age of onset of the symptomatology or length of disease. RESULTS: Only 8.3% of the patients (excluding the neonatal forms) were severely handicapped, and the degree of neuromuscular handicap depended fundamentally on the age of onset of the disease. Cataracts and myotonia were present in 87 and 89% of the patients, respectively. Almost all the patients above the age of 40 presented cataracts. No clinical or subclinical evidence of neuromuscular involvement was present in 11% of the patients with MD. These patients principally corresponded to the group in whom the disease initiated over the age of 50. CONCLUSIONS: The age of onset of the symptomatology appears to be the determining factor to establish both the global prognosis of neuromuscular incapacity of patients with myotonic dystrophy and the explanation of the chronology of the appearance of the most characteristic symptoms of the disease. The presence of carriers without neuromuscular symptomatology is of note, this fact reinforcing the need to incorporate DNA examination in the evaluation of asymptomatic relatives or with exclusive ocular symptomatology.

[Hospital admissions for varicella complications in children aged less than 15 years old]. / Hospitalizaciones por complicaciones de la varicela en niños menores de 15 años.

BACKGROUND: The complications of varicella are one of the arguments in favor of universal vaccination programs in children. OBJECTIVE: To describe the complications of varicella requiring hospital admission in a well-defined population (Gipuzkoa, Spain) and to compare the incidence of hospitalization with that reported in other series. MATERIAL AND METHODS: Observational, retrospective, multicenter study of admissions for varicella. The medical histories codified as varicella (minimum data set, CIE-0, codes 952.0-052.9) from 1 January 1993 to 31 December 2002 were reviewed. Calculation of hospitalization rates was based on emergency department visits and population data. The pediatric population of Gipuzkoa seeking medical attention at one of the four Basque Country Health Service hospitals in the area: Hondarribia, Mendaro, San Sebastian and Zumarraga. The mean coverage in Gipuzkoa is 54,999 children aged less than 15 years/year. All the children aged 0-15 years old admitted for more than 24 h with a discharge diagnosis of varicella complications. The variables studied are: age, gender, personal history, varicella immunization, immune status, fever, chest X-ray, complementary investigations, length of hospital stay, treatment, discharge diagnosis, clinical course, complications and sequelae at discharge. RESULTS: Seventy-one children were hospitalized. None had been vaccinated against the varicella-zoster virus. Eighty percent were aged less than 5 years and three were immunocompromised. Fifty-six percent had bacterial superinfection and invasive forms were found in seven patients. The mean length of admission was 6.5 days +/- 5.1. No deaths or sequelae were reported. CONCLUSIONS: The annual incidence rate of admissions longer than 24 hours due to varicella complications was 12.9 cases per 100,000 children aged less than 15 years, representing 0.31% of all annual admissions in this age group.

[Duodenogastric reflux: values in normal children and in children with gastroesophageal reflux]. / Reflujo duodenogástrico: valores en niños normales y con reflujo gastroesofágico.

Duodenogastric reflux (DGR) is a natural event, occurring occasionally and whose pathological significance is not well known. The accuracy of 24-hour gastric pH-metry for the DGR diagnosis has been tested in adults but not in children. For this purpose we measured the area under curve (AUC) at pH 4, 6 and 7 and the percentage of total time above pH 4 (%pH4), 6 (%pH6) and 7 (%pH7), excluding the 2-hours post-prandial period in 88 children suspected of having gastroesophageal reflux (GER). Forty were considered normal whereas 40 had acid GER and 8 with GER. In the control group %pH4 was 9.68 +/- 14.1, %pH6 3.4 +/- 8.7 and %pH7 1.0 +/- 2.4. The values for AUC were 101 +/- 112.8, 16.5 +/- 22.3 and 3.3 +/- 6.1 pH unit/min, respectively. Comparable results were found in the acid GER group. On the other hand alkaline refluxes had higher figures for all parameters: %pH4 22.1 +/- 13.9, %pH6 12 +/- 13 y %pH7 7 +/- 12, AUC at pH 4,406.5 +/- 410, 136.1 +/- 194 at pH6 and 48.2 +/- 85.1 at pH7 (p < 0.05). Because of the large dispersion of values in the control group we selected the 95 percentile, as the upper limit of normal values instead of the mean +/- SD. Therefore the upper limit were 27 for %pH4, 9 for %pH6 and 3.8 for %pH7. The AUC, 316, 64 and 16 pH/min, respectively. These results prove that DGR in children is a very common event and confirm that DGR definitely contributes to alkaline GER.