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Biomedical subjects

M Bueno

Publications and source records attributed to M Bueno.

At least 19 recordsLinked to original sources
PubMed1999

Epidermal naevus syndrome and hypophosphataemic rickets: description of a patient with central nervous system anomalies and review of the literature.

The epidermal naevus syndrome (ENS) is a rare dermatological condition consisting of congenital epidermal nevi associated with anomalies in the central nervous system, bones, eyes, hear or genito-urinary system. We report a new case of ENS associated with hypophosphataemic rickets. The girl was born with a mixed-type epidermal naevus and skeletal anomalies. Hypophosphataemic rickets was diagnosed at the age of 2.5 years. At 14 years of age. MRI of the head demonstrated right brain hypotrophy, a left temporal arachnoid cyst and asymmetric lateral ventricles. We reviewed the literature and found 13 reported cases of ENS associated with hypophosphataemic rickets. Conclusion We report a further patient with epidermal naevus syndrome and hypophosphataemic rickets, followed from birth to the age of 15 years, who had structural central nervous system anomalies with normal intellectual functioning. A comprehensive neurological work up is recommended in patients with epidermal naevus syndrome.

Adolescent↗
PubMed1999

Improving performance through change: an academic medical center's experience.

In order to perform at peak efficiency and effectiveness, health care organizations must be change-adept. Increased scrutiny by consumers, payers, and regulatory agencies is but one of the drivers necessitating change. This article discusses methodologies for improving performance and promoting continued success in an academic medical center.

Academic Medical Centers↗
PubMed1998

Skinfold thickness measurements are better predictors of body fat percentage than body mass index in male Spanish children and adolescents.

OBJECTIVE: To develop equations, from some simple anthropometric measurements, for the prediction of body density from underwater weighing in male spanish children and adolescents. SUBJECTS: One hundred and seventy-five males, aged 7.0-16.9 y, participated in this study, they were recruited from primary and secondary schools. MEASUREMENTS: Body weight and height and skinfold thicknesses by anthropometry, body density by underwater weighing. RESULTS: Correlations between body density and body mass index (BMI) were high until 14.0-16.9y. Correlations between body density and log sigma 4 skinfolds were higher than those with BMI at all ages. Log sigma 4 skinfolds explained between 61% (14.0-16.9 y) and 68% (11.0-13.9 y) of the body density variance. Regression equations for body density from BMI and triceps skinfold thickness explained between 51% (14.0-16.9y) and 68% (7.0-10.9 y) of the body density variance. CONCLUSIONS: The best estimators of body density in the children and adolescents studied were log sigma 4 skinfolds and a combination of BMI and triceps skinfold.

Adipose Tissue↗
PubMed1998

Reference values of gastric intramucosal pH in children.

To determine the reference values of gastric intramucosal pH (pHi) by tonometry in paediatric patients, we studied 17 children (nine males, eight females) with no systemic or gastrointestinal disease, aged six months to 12 years undergoing minor reconstructive surgery. Following anaesthetic induction a sigmoid tonometry catheter was inserted (Tonometrics, Inc.) into the stomach of the patients under direct vision. All children were normoventilated and were haemodynamically stable. After an equilibration period of 30 min, gastric pHi was calculated by applying the Henderson-Hasselbalch equation on the PCO2 obtained with the tonometer and the bicarbonate from the arterial blood gas analysis. The mean gastric pHi in our patients was 7.35 +/- 0.06 (SD). The normal pHi in the general population is estimated to be 7.31-7.40, with a confidence interval of 99%. No correlation was found between pHi and arterial pH, bicarbonate or base excess. Under conditions of normal ventilation and haemodynamic stability, healthy children during general anaesthesia have gastric intramucosal values similar to those of adults.

Blood Chemical Analysis↗
PubMed1998

Fat distribution in obese and nonobese children and adolescents.

BACKGROUND: It is not known whether fat deposition in obese children and adolescents occurs in the body's central or peripheral regions. METHODS: A representative sample of children was drawn from the schools of Zaragoza, Spain. The population selected comprised 1728 children ranging in ages from 6 to 14.9 years. Of the original sample, 368 children (21.29%) were excluded because of chronic diseases or refusal to participate. The waist-to-hip circumference ratio and the triceps-to-subscapular skin-fold thickness ratio were calculated and served as indexes of body fat distribution. The children were divided into tertiles according to standard deviation score of body mass index. RESULTS: Both in boys and in girls, waist-to-hip circumference ratio was higher in tertile 3 than in the other two tertiles, and triceps-to-subscapular skinfold thickness ratio was lower in tertile 3 than in the other two tertiles. Waist-to-hip circumference ratio was higher and triceps-to-subscapular skinfold thickness ratio was lower in obese than in nonobese children and adolescents of both sexes. CONCLUSIONS: Increased adiposity may be accompanied by an increase of fat depot in the abdominal region. In obese children and adolescents, fat deposition seems to occur in the body's central regions.

Adipose Tissue↗
PubMed1998

Severe malformations in males from families with osteopathia striata with cranial sclerosis.

Osteopathia striata with cranial sclerosis (OS-CS) is a bone dysplasia characterized by longitudinal striations of the long bones and sclerosis of the craniofacial bones. Affected patients show macrocephaly, ocular hypertelorism, frontal bossing, broad nasal bridge and abnormalities of the palate. Anomalies such as hearing loss, congenital heart defect, vertebral anomalies and mental impairment have also been reported. Pedigree analysis has suggested an autosomal dominant inheritance, but a recent report of a family with significantly more affected males than females suggested the possibility of X-linked inheritance. Here we describe a new family with OS-CS (the twelfth in the literature) with four affected individuals (two males and two females) spanning three generations. The affected male in the third generation was stillborn with multiple congenital anomalies, whereas the other three affected individuals had mild features. This family may represent another example of X-linked OS-CS where the mutated gene(s) is more severe in males.

Abnormalities, Multiple↗
PubMed1998

Lymphocyte T subset counts in children with hypercholesterolemia receiving dietary therapy.

BACKGROUND: In children with hypercholesterolemia, dietary therapy is indicated; however, we do not know if a low-fat diet can modify some organic functions, i.e. immune function. METHODS: 42 children with hypercholesterolemia received a low-fat, low-cholesterol diet during 6 months. At baseline and after the treatment period, we determined a lipoprotein profile and some immune characteristics: immunoglobulins G, A and M; complement components (C3, C4 and factor B), and blood lymphocyte subsets (CD3, CD4 and CD8). RESULTS: Total cholesterol serum concentrations showed a significant reduction after 6 months of dietary therapy (p = 0.008). After 6 months on a low-fat diet, lymphocyte T subset counts (CD3, CD4 and CD8) showed significant decreases (p < 0.01 to p < 0.003), but lymphocyte counts were always within normal ranges. There was also a significant correlation between changes in some lymphocyte T subset counts (CD3 and CD8) and changes in triglyceride serum concentrations (p < 0.05). CONCLUSIONS: A low-fat, low-cholesterol diet diminished CD3, CD4 and CD8 lymphocyte subset counts that are elevated in children with hypercholesterolemia. Dietary therapy, with emphasis on the intake of n-3 fatty acids, could be useful in the modulation of the immune response at the atheromatous plaque level.

Adolescent↗
PubMed1997

Pro-ACT II: integrating utilization management, discharge planning, and nursing case management into the outcomes manager role.

Building on redesign efforts that created case management, clinical care technicians, support service hosts, and pharmacy technician roles, this redesign focused on integrating case management, utilization management, and discharge planning functions into a new outcomes manager role. The authors describe the process of developing and implementing the new role and outline specific actions that eliminated redundancy and inefficiency. Results of the evaluation of the project are reviewed, including full-time equivalent and salary savings and employee and physician satisfaction improvements.

Case Management↗
PubMed1997

Indices of body fat distribution in Spanish children aged 4.0 to 14.9 years.

BACKGROUND: Reference values for the main indices of body fat distribution in children are not available. METHODS: The study population comprised 1638 Caucasian children and adolescents: 848 boys and 790 girls, with ages ranging from 4.0 to 14.9 years, and living in the central part of Spain (Provincia de Zaragoza). The waist-to-hip circumference ratio and the triceps-to-subscapular skinfold thickness ratio were measured. RESULTS: In boys, mean waist-to-hip circumference ratio ranges from 0.834, at 13.5 years, to 0.896 at 4.5 years. In girls, waist-to-hip circumference ratio ranges from 0.756, at 14.5 years, to 0.877, at 4.5 years. We observed a decrease of waist-to-hip circumference ratio with age, especially in girls. In boys, triceps-to-subscapular skinfold thickness ratio ranges from 1.360, at age 14.5, to 1.704, at age 5.5. In girls, triceps-to-subscapular skinfold thickness ratio ranges from 1.468, at age 13.5, to 1.727, at age 9.5. We observed a decrease with age only in boys. CONCLUSIONS: We present reference values for the main indices of body fat distribution, which could be useful in clinical practice. However, research is needed that will compare these indices with data on body fat distribution obtained by a gold standard method, such as computed tomography or magnetic resonance imaging.

Adolescent↗
PubMed1996

Relationship between immunoinflammatory proteins containing sialic acid and low-density lipoprotein serum concentrations.

The aim of this study was to investigate the relationship between sialoglycoproteins and the lipoprotein profile in a group of children with different levels of low-density lipoprotein cholesterol. We have studied 177 children of 132 families who were sent to our Pediatric Lipid Research Clinic because of serum cholesterol concentrations higher than 5.17 mmol/l. At the time of diagnosis, we analyzed the serum lipoprotein profile and the sialoglycoproteins: alpha 1-antitrypsin, acid alpha 1-glycoprotein, haptoglobin, alpha 2-macroglobulin, transferrin, IgA, IgG, IgM, complement C3 component and ceruloplasmin. At 7.0 to 10.9 years, alpha 1-glycoprotein serum concentrations were higher in the high risk group than in the moderate risk group (P < 0.05). At 2.0 to 6.9 years, IgA and IgM serum concentrations were higher in the moderate risk group than in the low risk group (P < 0.05 and P < 0.01, respectively), and IgG and IgM serum concentrations were also higher in the high risk group than in the low risk group (P < 0.05). Our results seems to reflect a general reaction to injury or inflammation which could be associated with the atherosclerotic process.

Adolescent↗