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Biomedical subjects

Ajay Kaul

Publications and source records attributed to Ajay Kaul.

12 recordsLinked to original sources

Eosinophilic esophagitis in infants and toddlers.

Feeding refusal is often described in conjunction with the diagnosis of eosinophilic esophagitis (EE) in pediatric patients; however, there are little data regarding the specific clinical manifestations and effective management of this condition in very young children. The aim of this study was to evaluate the presentation of EE in infants and toddlers referred to the Interdisciplinary Feeding Team Clinic of a tertiary referral center and to document responses to treatment. Database matching was performed (from January 2000 to June 2003) to identify infants and toddlers diagnosed with EE who had been referred to the Interdisciplinary Feeding Team Clinic. Endoscopic features required for a diagnosis of EE included esophageal mucosal furrowing, erythema, exudates, or decreased vascular markings. Histologic features of EE were more than 24 eosinophils per high-power field (HPF), thickening of basal cell layer, and papillary (rete peg) lengthening or elongation. All study patients were treated with a combination of proton pump inhibitors (PPI) and fluticasone (swallowed). In addition, elemental diet was instituted in those documented to have a food allergy. Treatment success was defined by an improved oral intake, adequate weight gain, and improved endoscopic and histologic findings at 3-6-month followup. A total of 15 subjects [mean age = 19.9 months (SD = 9.7 months)] who fulfilled the entry criteria during the study period were identified. All 15 children had documented endoscopic improvement and 14/15 children had histologic resolution of EE after therapy. In 13 of the 15 children, this translated to clinical improvement as well.

Child, Preschool↗

The Hlx homeobox transcription factor is required early in enteric nervous system development.

BACKGROUND: Development of the enteric nervous system (ENS) requires interactions between migrating neural crest cells and the nascent gastrointestinal tract that are dependent upon genes expressed by both cell compartments. Hlx, a homeobox transcription factor gene that is expressed in mouse intestinal and hepatic mesenchyme, is required for normal embryonic growth of intestine and liver, and the Hlx-/- genotype is embryonic lethal. We hypothesized that Hlx is required for ENS development. RESULTS: Enteric neurons were identified in Hlx+/+ and Hlx-/- mouse embryos by immunostaining of embryo sections for the neural markers PGP9.5 and Phox2b, or by staining for beta-galactosidase in whole-mount embryos containing the dopamine beta-hydroxylase-nLacZ transgene. In Hlx+/+ embryos, neural crest cells/enteric neurons have moved from the stomach into the intestine by E10.5. By contrast, neural crest cells/enteric neurons remain largely restricted to the lateral stomach mesenchyme of Hlx-/- embryos, with only a few scattered neural crest cells/enteric neurons in the intestine between E10.5-16.5. CONCLUSION: The Hlx homeobox transcription factor is required for early aspects of ENS development.

Animals↗

An enteric-coated high-buffered pancrelipase reduces steatorrhea in patients with cystic fibrosis: a prospective, randomized study.

OBJECTIVE: Enteric-coated (EC) high-buffered (2.5 mEq [2.5 mmol] bicarbonate per capsule) pancrelipase microsphere enzymes were compared to EC-nonbuffered pancreatic enzymes for efficacy in reducing steatorrhea in patients with cystic fibrosis. DESIGN: Prospective, randomized, controlled trial using a crossover design with each subject as his/her own control. SUBJECTS/SETTING: Eighteen subjects with cystic fibrosis, who had pancreatic insufficiency and required large enzyme doses, were studied over two consecutive 7-day treatment periods. INTERVENTION: Each 7-day period consisted of 3 days at home followed by 4 days in a general clinical research center for careful control of diets, enzyme lipase doses (given at approximately 50% of the subject's usual lipase dose), and carmine red-labeled stool collections for 72-hour fecal fat balance studies. MAIN OUTCOME MEASURE: Fecal fat excretion. STATISTICAL ANALYSES PERFORMED: Differences in fat excretion, when each subject received EC-high-buffered pancrelipase vs EC-nonbuffered enzymes, were compared using linear modeling. RESULTS: Mean fat excretion decreased significantly in each subject during periods when given EC-high-buffered pancrelipase compared with periods when given EC-nonbuffered enzymes (fat excretion 18.2% vs 24.9% or fat absorption 81.8% vs 75.1%, respectively; P=0.01). Thirteen of 18 subjects (72%) excreted less fat when receiving EC-high-buffered pancrelipase whereas 10 (56%) decreased fat excretion by more than 5%, and five subjects did not respond. CONCLUSIONS: EC-high-buffered pancrelipase decreased fat excretion, symbolizing improved fat absorption, when compared with EC-nonbuffered pancreatic enzymes given at equivalent, reduced (approximately 50% of usual) lipase doses in nourished subjects with cystic fibrosis and mild pulmonary disease.

Adolescent↗

Down syndrome and complete tracheal rings.

Tracheal stenosis caused by complete tracheal rings has previously not been associated with Down syndrome. We identified 7 children with Down syndrome and complete tracheal rings. We found the clinical presentation and airway anatomy to be variable, advocating a heightened level of suspicion when evaluating a child with Down syndrome.

Child, Preschool↗

An intact intermediate filament network is required for collateral sprouting of small diameter nerve fibers.

Expression of the intermediate filament (IF) protein peripherin is initiated during development at the time of axonal extension and increases during regeneration of nerve fibers. To test whether the IF network is essential for neuron process outgrowth in the mature organism in vivo, we disrupted endogenous peripherin IF in small-sized dorsal root ganglion (DRG) neurons in transgenic mice via expression of a mutant peripherin transgene under control of peripherin gene regulatory sequences. Anatomical and functional analyses showed that these neurons send peripheral and central axonal projections to correct targets, express correct neuropeptides, and mediate acute pain responses normally. However, disruption of IF significantly impaired the ability of uninjured small-sized DRG neurons to sprout collateral axons into adjacent denervated skin, indicating a critical role for intact IF in plasticity, specifically in compensatory nociceptive nerve sprouting.

Animals↗

A multicomponent behavioral program for oral aversion in children dependent on gastrostomy feedings.

OBJECTIVES: Describe outcomes in nine children with Nissen fundoplication and feeding gastrostomy treated in a multicomponent feeding program. The importance of comprehensive evaluation, appetite regulation, and family-focused intervention within a behavioral feeding program are discussed. METHODS: Prospective clinical intervention with dependent measures evaluated before treatment, after treatment, and at follow-up. RESULTS: Nine children (4 girls; mean age, 3.1 +/- 1.2 years; range, 1.8-5.5 years) and their mothers were admitted for intensive treatment (mean duration, 11.4 +/- 1.7 days; range, 5-16 days). At discharge, 4 of 9 (44%) children were weaned completely from gastrostomy feedings. The mean oral intake of all patients increased 50% from pretreatment to posttreatment assessment. At a mean of 3.1 +/- 0.5 months (range, 2.4-3.6 months) after treatment, six of nine children were weaned completely from gastrostomy feedings. The percent of daily nutritional needs consumed orally increased from a pretreatment mean of 14.6% +/- 21.2% (range, 0%-67%) to a posttreatment mean of 63.4% +/- 18.3% (range, 34%-85%) and a follow-up mean of 88.1% +/- 25.1% (range, 30%-100%). The mean percent ideal body weight for height was not compromised during intensive treatment. CONCLUSIONS: Short-term intensive biobehavioral treatment was successful in improving oral intake and weaning from gastrostomy tube feeding in children with Nissen fundoplication and feeding gastrostomy.

Behavior Therapy↗

Pediatric gastroesophageal reflux disease--current perspectives.

PURPOSE OF REVIEW: Although heartburn and regurgitation are common manifestations of gastroesophageal reflux disease (GERD), otolaryngologic or respiratory symptoms may be the only indication of GERD. This review focuses on the recent developments in the pathophysiology, diagnosis, and treatment of GERD and their implications in pediatrics. RECENT FINDINGS: Newer diagnostic modalities include intraesophageal impedance and Bravo wireless pH monitoring. Impedance technology measures both acid and non-acid reflux, whereas the Bravo capsule allows prolonged pH monitoring under more physiologic conditions. In managing children with GERD, there is increasing evidence that they need higher dosages of acid suppressive therapy to achieve clinical response. Alternative therapeutic options are also currently being explored, including pharmacotherapy that targets the primary mechanism for GERD. Endoluminal therapy for GERD in adults as an alternative to surgery has been an exciting development. At present, two of these procedures, Stretta (using radiofrequency energy) and Endocinch (gastroplication), have been approved by the Food and Drug Administration for use in adults. SUMMARY: Impedance technology has increased our understanding of acid and nonacid reflux; however, normal values for children are not yet established. There are also limitations to the applicability of the new endoluminal therapies in children. Further research is needed before these developments can be recommended for use in pediatric practice.

Adolescent↗

Gastrointestinal microflora studies in late-onset autism.

Some cases of late-onset (regressive) autism may involve abnormal flora because oral vancomycin, which is poorly absorbed, may lead to significant improvement in these children. Fecal flora of children with regressive autism was compared with that of control children, and clostridial counts were higher. The number of clostridial species found in the stools of children with autism was greater than in the stools of control children. Children with autism had 9 species of Clostridium not found in controls, whereas controls yielded only 3 species not found in children with autism. In all, there were 25 different clostridial species found. In gastric and duodenal specimens, the most striking finding was total absence of non-spore-forming anaerobes and microaerophilic bacteria from control children and significant numbers of such bacteria from children with autism. These studies demonstrate significant alterations in the upper and lower intestinal flora of children with late-onset autism and may provide insights into the nature of this disorder.

Age of Onset↗