Anales Espanoles De Pediatria最新文献

Aim: To analyze the psychological sequelae in long-term survivors of childhood cancer and to establish the relationship between the changes produced in both cognitive (intelligent quotient) and emotional factors (anxiety and depression) and diagnostic and therapeutic variables as well as in sensorial sequelae (visual and auditory).

Methods: One hundred and thirty eight survivors were evaluated. Of these 73 had had acute leukemia and 65 had had solid tumour (nephroblastoma or sympathetic nervous system tumour) diagnosed before the age of 15 years. Elapsed time since diagnosis was at least 10 years and duration of therapy was more than two years. Demographic and social data of the survivors, their parents and siblings, diagnosis, number of relapses and treatment given were obtained from medical records and from individual interviews at the time of assessment. Cognitive and emotional dimensions and the tools used to measure them were intelligent quotient (Wechsler Intelligence Scale for children and Wechsler Adult Intelligence Scale), anxiety features and state (State-Trait Anxiety Inventory for children and adults) and depressive symptoms (Minnesota Multiphasic Personality Inventory Depression subscale). Ophthalmologic assessment included visual acuity, measurement of intraocular pressure and ophthalmoscopic examination. Audiologic evaluation included tonal audiometry for frequencies from 125 to 8,000 Hz.

Results: Total, verbal and performance intelligent quotients were 102, 106 and 105 respectively for the whole sample. Five percent of survivors scored under 70 (mental deficiency), and 6.5 % over 129 (gifted). The scores of solid tumour survivors were higher than those of leukemia survivors who were cranially irradiated at dosages >= 24 Gy (108 vs 98; p = 0.03), and were similar to those of leukemia survivors irradiated at lower dosages (102) or who had not been irradiated (109). Intelligent quotient correlated positively with age at diagnosis and negatively with cumulated intrathecal methotrexate and cranial irradiation dosages. Survivors of acute leukemia who relapsed scored 14 points less than those who had not relapsed. The most affected cognitive areas were comprehension, arithmetic ability, attention, visual and verbal memory, causative reasoning and visual-motor coordination. No relationship was found between sensory sequelae and cognitive capacities, probably due to the mildness of the sequelae. The prevalence of depression was greater in cancer survivors than in the general population but that of anxiety was lower.

Conclusions: Intelligent quotient was within normal limits. Lower scores were related to cranial radiotherapy, age at diagnosis and relapses. Emotionally, the survivors coped successfully with cancer, depressive symptoms being more prevalent than in the general population and anxiety almost negligible.

Aim: To describe the rate of hospitalization for bronchiolitis among newborn infants in our environment, to evaluate the influence of prematurity and other perinatal conditions on hospitalization for bronchiolitis, and to compare our data with those of the Impact-RSV Study Group.

Materials and methods: Descriptive studying based on analysis of the perinatal data of all neonates and patients hospitalized for bronchiolitis during a 3-year period.

Results: Among 12,895 newborn infants, 455 (3.52 %) required hospitalization for bronchiolitis. The hospitalization rate for bronchiolitis was 3.18 % among term infants, 8.6 % among preterm infants, 9.8 % among infants with congenital heart disease and 21.1 % among preterm infants who required mechanical ventilation during the neonatal period. Intensive care unit admission was associated with postnatal age under 6 weeks (OR: 1.68; 95 % CI: 1.04-8.19; p 5 0.04) and prematurity (OR: 2,67; 95 % CI: 1.01-7.56; p 5 0.006). The hospitalization rate for bronchiolitis was 40 % lower in our neonatal population than in that of the Impact-RSV Study Group. When infants with bronchopulmonary dysplasia, congenital heart disease and mechanical ventilation during the neonatal period were excluded, the hospitalization rate for respiratory syncytial virus (RSV) was not significantly different between infants born at < weeks' gestation and those born at < 32 weeks' gestation and those born at 32-35 weeks' gestation (3.4% vs 4.3%; p>0.10).

Conclusions: Neonatal mechanical ventilation, bronchopulmonary dysplasia and congenital heart disease are more closely associated with hospitalization for RSV(1) bronchiolitis and mean length of stay than with gestational age.

Aim: Retrospective study of the outcome of cord blood transplantation (CBT) in children in Spain.

Methods: Twenty-eight patients (mean age 6.5 years; mean weight 25 kg) received a CBT between July 1994 and May 1998 in several centres of the Spanish Pediatric Bone Marrow Transplant Group. In 2 patients the donor was an identical human leukocyte antigen (HLA)-sibling and in two the donor was a mismatched family donor. In 24 patients the donor was unrelated, and 21 of these received an HLA-mismatched CBT. Twenty-one patients (75 %) received a CBT for leukemia mainly in advanced phase. Seven patients were transplanted for genetic disease. Of these, five had congenital immunodeficiency. The conditioning treatment included total body irradiation in ten patients and combined chemotherapy in the remaining patients. In all patients graft-versus-host disease (GVHD) prophylaxis was performed with cyclosporine, and corticosteroids or methotrexate were added in patients with HLA-mismatched donors. The mean number of nucleated cells infused was 53.4 x 106/kg.

Results: Graft failure was observed in nine patients. Eighteen patients (64.3%) developed grade IIIV acute GVHD. Eight patients (28.6%) developed severe GVHD. Actuarial event free survival (EFS) of all the patients was 34.4 +/- 9% at 3 years, with a mean followup of 16.6 months. EFS was more favorable in patients with genetic disease (71>=6 17%) and in those with an HLA (A, B and DR) identical donor (6/6) (66>=6 19%).

Conclusions: The most favorable results were obtained in patients with genetic diseases. We observed an inverse correlation between EFS and patients with HLA identical donors. The high incidence of severe acute GVHD could have been related to a lack of accuracy in the HLA typography of some patients.

Aim: In recent years, the high frequency of atypical cases of celiac disease (CD) and of forms of this disease with minor symptoms has prompted the search for analytical markers that may support indications for intestinal biopsy. The commonest tests are those for serum class IgG and IgA antigliadin antibodies (IgG-AGA, IgA-AGA) and IgA antiendomysial antibodies (IgA-EmA).

Methods: We report our 10 year experience of studying AGA in 1,075 serum samples from patients with CD and IgA-EmA in 534 samples. The serological markers were compared with 152 intestinal biopsies performed simultaneously with the other tests.

Results: In patients with severe intestinal atrophy the sensitivity of IgA-AGA (91%) and IgA-EmA (94%) was high. IgA-EmA and the latter showed the highest positive (88%) and negative (97%) predictive values. In all patients, IgA-EmA positivity coincided with alterations in the biopsy. Determination of IgA-EmA was also the most efficient marker for monitoring the gluten free diet phase. However, in patients in whom minimal histological changes were found in the intestinal mucosa, none of the markers was sufficiently accurate.

Results: IgA- EmA antibodies are the most accurate serological marker of CD. In view of these results and the estimated prevalence of the disease, protocols for the use of serological markers are proposed for the differential diagnosis of malabsorption symptoms, for use in patients at low and high risk of CD and for the followup of those with a diagnosis of CD.

Abnormal glucose tolerance and diabetes mellitus are a common complication of cystic fibrosis (CF). Cystic fibrosis related diabetes (CFRD) is due to decreased insulin secretion, secondary to pancreatic insufficiency. Patients with CFRD have increased morbidity and mortality and are subject to the same microvascular complications as patients with other types of diabetes. Prompt diagnosis and aggressive management of CFRD is important.A consensus conference on CFRD was held in Madrid in May 2000to define the current standards for the diagnosis and management of this disease in Spain.

The use of a dummy as a comforting object in childhood is widespread in civilized societies. The advisability of this practice, as well as the shape, material, or time of use of these objects, is controversial. Data on the possible beneficial effects of these objects are numerous, but there is also a long list of disorders which have been associated with their use. Their soothing effect is the most widely recognized beneficial effect and the possible relationship with a lower incidence of sudden infant death syndrome is the newest. The most important risks of this nonnutritive sucking habit are failure of breastfeeding, dental deformities, recurrent acute otitis media, and the risk of accidents. The development of latex allergy, tooth decay, oral ulcers and sleep disorders are other possible problems. The association with a lower intelligence quotient is disputed. Insufficient data are provided by a review of the pros and the cons of dummy use to encourage or discourage this habit. However, there are sufficient data on which to base firm recommendations such as not starting their use in the first days of life, restricting use after the age of 8 months and withdrawing these objects at the age of 1 year. Pediatricians should be aware of and recommend only dummies that meet safety requirements.

Aim: Polygraphy enables differentiation between primary snoring and obstructive sleep apnea syndrome (OSAS). A clinical score (Brouillette Index) has been proposed that could be useful in classifying children suspected of suffering from this disorder and in reducing the number of polysomnographic studies.

Patients and methods: We evaluated 192 consecutive children with adenoton-sillar hypertrophy and with no craniofacial abnormalities or other associated diseases referred to our pediatric respiratory clinic for suspected OSAS. The Brouillette Index (BI) was used to classify the patients into a) non-OSAS (BI < neg 1), b) uncertain OSAS (BI between neg 1 and 3.5 and c) OSAS (BI > 3.5). For the polygraphic diagnosis we analyzed two different criteria for differentiating between OSAS and primary snoring: a respiratory distress index (RDI) >= 3 or >= 5.

Results: The BI correctly classified only 23% of the patients with suspected OSAS. In the great majority of the children, polysomnographic monitoring was needed to differentiate between OSAS and primary snoring. In one group of children (10.9% or 6.4% depending on the diagnostic criteria), the BI would have led to incorrect classification.

Conclusions: The BI is not an efficient tool for discriminating between primary snoring and OSAS in an outpatient pediatric respiratory clinic.

Aim: To compare urinary gram staining and dipstick for the detection of urinary tract infection (UTI)in febrile infants.

Methods: Prospective study of 175 febrile infants aged 124 months. In all infants, a urine specimen was analyzed to detect UTI. The dipstick test was used to detect leukocytes and nitrites and samples were taken for gram staining and urine culture. Urine was obtained by urethral catheterization. Positive urine results were defined as 50.000 colony forming units per millimeter of urinary tract pathogen.

Results: The mean age was 9.8 months (SD: 6.64). Urine culture was positive in 87 patients (49.5%). Diagnosis of UTI was confirmed in 91 patients (51.9%), of whom 74 were admitted for clinically suspected pyelonephritis (81.3%). Gram stain had the highest specificity (98.9%) and pyuria the highest sensitivity (90.8%). Better results were obtained using the combination of dipstick and Gram stain with a sensitivity of 93.1%, specificity of 98.4%, positive predictive value of 98.5% and negative predictive value of 92.5%.

Conclusions: Urinary Gram stain appears to be more reliable than dipstick in detecting UTI in febrile infants but the results of both tests should be interpreted together.

Aim: To describe the implementation of a program for the early detection of deafness in newborn infants in a private hospital.

Methods: Between August 1, 1994 and July 31, 1999, 1,750 neonates from the nursery and neonatology unit of our hospital were studied using evoked acoustic emissions (EAE). Screening was performed after the first 24 hours of life and after parent consent had been obtained.

Results: The coverage (number of neonates screened using otoacoustic emissions compared with the total number of live births) increased from 6% in 1994 to 37.5% in 1999. Six mild moderate neurosensory hypoacusis were detected; overall results were 4% hypoacusis and 2.3% bilateral hypoacusis.

Conclusions: This study demonstrates that hardness of hearing can be detected at birth by means of EAE. Because of lack of awareness in the general population, universal screening is difficult. Consequently, governments and medical organizations should legislate for such screening programs and should make the general population aware of them.