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Biomedical subjects

L Bott

Publications and source records attributed to L Bott.

12 recordsLinked to original sources
PubMed2006

Comparison of resting energy expenditure in bronchopulmonary dysplasia to predicted equation.

OBJECTIVE: Children with bronchopulmonary dysplasia (BPD) often suffer from growth failure because of disturbances in energy balance with an increase of resting energy expenditure (REE). Evaluation of REE is a useful tool for nutritional management. Indirect calorimetry is an elective method for measuring REE, but it is time consuming and requires rigorous procedure. The objective of this study was to test accuracy of prediction equation to evaluate REE in BPD children. PATIENTS AND METHODS: Fifty-two children aged 4-10 years with BPD (30 boys and 22 girls) and 30 healthy lean children (20 boys and 10 girls) were enrolled. In this study, indirect calorimetry was compared to four prediction equations (Schoffield-W, Schoffield-HW, Harris-Benedict and Food and Agriculture Organization equation) using Bland-Altman pair wise comparison. RESULTS: The Harris-Benedict equation was the best equation to predict REE in children with BPD, and Schoffield-W was the best in healthy children. For the children with chronic lung disease of prematurity the Harris-Benedict equation showed the lowest mean predicted REE-REE measured by indirect calorimetry difference (difference = 15 kcal/day; limits of agreement -266 and 236 kcal/day; 95% confidence interval for the bias -207 to 177 kcal/day), and graphically, the best agreement. For the group of healthy children, it was the Schofield-W equation (-2.9 kcal/day; limits of agreement -275 and 269 kcal/day; 95% confidence interval for the bias -171 to 165 kcal/day), and graphically, the best agreement. CONCLUSION: Differences in prediction equation are minimal compared to calorimetry. Prediction equation could be useful in the management of children with BPD.

Basal Metabolism↗
PubMed2006

Body composition in children with bronchopulmonary dysplasia predicted from bioelectric impedance and anthropometric variables: comparison with a reference dual X-ray absorptiometry.

UNLABELLED: Since children with bronchopulmonary dysplasia often suffer from malnutrition and growth failure, evaluation of body composition is a very important tool to nutritional support. The aim of this study was to compare assessment of fat-mass (FM) and fat-free mass (FFM), evaluated by bio-impedancemetry and anthropometry compared to dual-X-ray-absorptiometry (DXA) in children with bronchopulmonary dysplasia. PATIENTS: Seventy-one children, aged 4-8 years, with bronchopulmonary dysplasia were enrolled. METHODS: FM and FFM measured using anthropometry and bio-impedancemetry were compared to FM and FFM obtained by DXA using the Bland-Altman method. RESULTS: Both bio-impedancemetry and anthropometry gave good agreement with DXA to evaluate FM and FFM. Anthropometry method, in general, slightly under-estimated FM (mean difference: -0.02 kg, standard deviation: 0.99) and FFM (mean difference: -0.70 kg+/-1.72). Bio-impedancemetry method overestimated FM (mean difference: 0.34 kg+/-2.06) and underestimated FFM (mean difference: -1.24 kg+/-3.32). CONCLUSION: In children with bronchopulmonary dysplasia aged, 4-8 years, both anthropometry and bio-impedancemetry cannot be used to precisely evaluate body composition.

Absorptiometry, Photon↗
PubMed2006

[Ataxia-telangiectasia: a review].

Ataxia-telangiectasia (AT) is an autosomal recessive inherited disease caused by mutational inactivation of the ATM gene. It is a multisystemic disease, characterized by progressive neurological dysfunction, especially in the cerebellum, oculo-cutaneous telangiectasia, immunodeficiency, recurrent sino-pulmonary infections and high incidence of neoplasms. The responsible gene, ATM, encodes a large protein that belongs to a family of protein kinases with a phosphatidylinositol 3-kinase (Pi3K) domain. ATM is a key regulator of cell cycle checkpoints that causes DNA repair or apoptosis. Several studies report ATM function in target cells (such as neurons, fibroblast, endothelium, germ cells, lymphocytes). The pleiotropic phenotypes of AT reflect the multifaceted activities of ATM protein. In nucleus (lymphocytes, fibroblasts, germ cells) ATM is involved in regulation of cell-cycle checkpoints; in cytoplasm ATM regulates redox state (neurons).

Adolescent↗
PubMed2004

Congenital idiopathic intestinal pseudo-obstruction and hydrocephalus with stenosis of the aqueduct of sylvius.

We present the first report of an association between hydrocephalus with stenosis of the aqueduct of Sylvius (HSAS) and a specific form of congenital idiopathic intestinal pseudo-obstruction (CIIP) in an infant. Diagnosis of HSAS was suspected during the neonatal period because of a severely dilated ventricular system associated with bilateral adducted thumbs, and was confirmed by demonstration of a mutation in the gene encoding L1 cell adhesion molecule (L1CAM). L1CAM mutations cause a variable clinical spectrum. This gene is located at Xq28 and encodes a transmembrane glycoprotein involved in neurite outgrowth and neuronal migration. Hirschprung disease has been reported to involve an L1CAM mutation that manifests as a quantitative defect in the migration of neural crest cells in distal segments of the gut. We report an association that suggests that alterations of L1CAM may cause another type of intestinal pseudo-obstruction distension with a qualitative defect in differentiated Cajal's cells in the anterior part of the gut. This observation suggests that L1CAM has a role in the developmental regulation of multiple systems. Further clinical descriptions of gastroenterological and neuropathological data are required to extend our understanding of the mechanisms underlying L1CAM functions.

Cerebral Aqueduct↗
PubMed2004

[Nutrition and bronchopulmonary dysplasia].

Bronchopulmonary dysplasia remains a frequent complication of extreme prematurity. In preterm neonates catch-up and pulmonary alveolar growth occur during the first two years of life. However 10 to 25% of preterm infants with bronchopulmonary dysplasia are under-nourished after two years of age, and 30 to 60% of them also suffer from persistent airway obstruction, hyperinflation and bronchial hyperreactivity. Recommendations on nutritional requirements in this population are not yet clearly defined, but an adequate nutritional status in prenatal and early postnatal period can have long-term consequences on brain and lung development. There are a few randomised trial of nutrition for preterm infants with bronchopulmonary dysplasia after discharge. Caloric and protein requirements in this population are probably higher than in full-term infants. Moreover there are potential benefits in using specific nutrients: supplementation with long chain polyunsaturated fatty acids could decrease lung inflammation injuries, glutamine is the main source of energy of pneumocyte, vitamin A is essential for lung development, inositol is necessary for surfactant synthesis, vitamin E and selenium have anti-oxidant effects. Controlled nutritional trial are needed with a long term follow-up in late childhood in order to test their effects on growth and pulmonary status.

Bronchopulmonary Dysplasia↗
PubMed2003

[Perforated gastric ulcer in the child: a rare complication, a case report].

UNLABELLED: Perforated gastric ulcer is unusual in children. We report a case in a girl with an unexpected evolution. CASE REPORT: A 13-year-old girl was admitted for abdominal pain. She had no particular personal history but her father had a perforated ulcer. On admission she was not painful, her abdomen was soft on palpation. The white blood cell count was 1.7 x 10(3)/mm3. A right pneumoperitoneum was seen on an abdominal X-ray film. Because of her good general status and the normalization of the abdominal X ray film six hours later, no surgical exploration was performed. On the fourth day, a gastrointestinal endoscopy showed an anterior gastric ulcer which was perforated. Biopsies did not isolate H. pylori. The patient was given a treatment with amoxicillin-metronidazole (7 d) and oméprazole (7 weeks). An endoscopic control, one month later, showed a total healing of the gastric ulcer. CONCLUSION: Peptic ulcerations and their complications are underdiagnosed in childhood. This could lead to delay in diagnosis or inappropriate treatment specially in case of perforation.

Adolescent↗
PubMed2001

Contamination of gastrostomy feeding systems in children in a home-based enteral nutrition program.

BACKGROUND: There are few data concerning the risk of contamination of enteral feeding systems via gastrostomy in children, and none for conditions that pertain to home-based care. METHODS: To investigate the risk of contamination of enteral feeding systems during the home-based care of 20 children receiving gastrostomy tube feeding, five samples were taken for analysis: two samples before the enteral feeding period (gastrostomy, enteral feeding system) and three after this period (gastrostomy, distal giving set, liquid remained in container). Microorganisms were identified and counted. Different factors were studied to elucidate their role in bacterial colonization: acid suppressive therapy, gastrostomy tube or button, hanging feeding time, rate of enteral feeding, gastric pullulation and retrograde contamination, manipulation error, and use of open or closed enteral feeding systems. RESULTS: Overgrowth was defined as a microorganismal load exceeding 10(4) colony-forming units (cfu)/mL. Overgrowth was present in 85% of gastrostomy samples before enteral nutrition started. Most microorganisms belonged to gastric flora. Some bacteria had an environmental origin or derived from cutaneous flora. Forty-five percent of the lines showed overgrowth at the end of enteral nutrition period, mainly with the same microorganism found in the gastrostomy. Closed enteral bags remained sterile, even if manipulation error occurred. Duration, rate of enteral feeding, and acid suppression treatment were not risk factors for overgrowth. CONCLUSIONS: Retrograde contamination of gastrostomy feeding systems occurs frequently. The preferential use of closed enteral feeding systems is recommended for home-based enteral nutrition programs.

Adolescent↗
PubMed1996

Incidence of multiglandular disease in primary hyperparathyroidism determined by parathyroid hormone secretion.

BACKGROUND: Successful parathyroidectomy depends on recognition and excision of all hyperfunctioning parathyroid glands. Because histologic definition is limited, multiglandular disease (MGD) is usually determined grossly by means of estimation of gland size and the experience of the surgeon, resulting in frequency varying from 8% to 33%. Normalization of elevated intraoperative intact parathyroid hormone (iPTH) levels after excision of all hyperfunctioning glands is necessary for postoperative normocalcemia and indicates normal secretion of remaining parathyroids. Abnormal hormone secretion measured during operation has been used to define the extent of excision and the incidence of MGD. METHODS: One hundred ten consecutive parathyroidectomy patients with no previous neck surgery or history of multiple endocrine neoplasia had intraoperative iPTH assays performed before and after excision of any suspected abnormal parathyroid gland(s). A drop in iPTH level after gland excision predicted postoperative normal calcium levels. RESULTS: All patients except one had normalization of serum calcium levels (average follow-up, 15 months). One hundred five patients had only one hyperfunctioning gland removed, and all have remained normocalcemic. Five (5%) patients had more than one gland involved: four had two or more hyperfunctioning parathyroids and one patient, who had a large parathyroid cyst removed, remained hypercalcemic. CONCLUSIONS: By using a biochemical assay, instead of estimated size, to predict which parathyroid glands are hypersecreting, the incidence of MGD in primary hyperparathyroidism was found to be 5%.

Adolescent↗
PubMed1989

The phenomenology of schizophrenia occurring in childhood.

Thirty-five children, aged 4 to 13 (X = 9.54), meeting strict DSM-III criteria for schizophrenia, are described. The subjects were diagnosed using a new semistructured interview. All were in the normal range of intelligence (mean IQ = 94) and free of neurological disorders. Characteristic auditory hallucinations were present in 80% and delusions in 63% of the sample. The mean age of onset of psychotic symptoms was 6.9 years. Premorbid histories of attention deficit, conduct disturbance and/or developmental abnormalities were common. The nature and content of psychotic symptoms varied with developmental stage. The phenomenological presentation of the sample was similar to previous studies of young schizophrenic children.

Child↗
PubMed1987

Patterns of intellectual functioning in non-retarded autistic and schizophrenic children.

The WISC-R factor scores of non-retarded children meeting DSM III criteria for autism and schizophrenia were compared. The scores of the schizophrenic children on factor 3 were significantly lower than those of the autistic children, below the range of normal children, and significantly lower than the scores they obtained on the verbal comprehension and perceptual organization factors. The autistic children scored in the superior range on the block design subtest and did not show gross impairments in language function as indexed by scores on the verbal comprehension factor. The only subtest autistic children were impaired on was the comprehension subtest.

Attention↗