Surgical treatment of infantile achalasia: a case report and literature review.

Allgrove syndrome is a rare genetic disorder typically manifested by alacrima, achalasia, and adrenal insufficiency, and is one of the rare causes of achalasia in infants. While the gold standard for achalasia treatment in adults is an esophageal myotomy with fundoplication, a standard treatment for infantile achalasia remains undetermined due to the low number of reported cases and rarity of the disease in this age group. We report a 7.7 kg infant with achalasia secondary to Allgrove syndrome who was successfully treated by Heller myotomy and simultaneous Toupet fundoplication. This case represents one of the smallest patients to ever be treated for achalasia and highlights the role that primary surgical therapy may have for the infantile variant of the disease.

Treatment of Helicobacter pylori infection in children.

The majority of Helicobacter pylori (H. pylori) infections appear to be acquired during childhood. Despite this fact, the natural history of H. pylori infection in children, such as the mode of acquisition, the clinical symptoms and signs of infection and the appropriate treatment, is poorly understood. There is no consensus regarding which children with H. pylori infection deserve treatment nor is there agreement on the appropriate treatment regimen. This stems from the lack of controlled studies into H. pylori infection during childhood. For example, there have been no controlled studies to determine effective treatment of H. pylori infection in children. Although published guidelines for the treatment of childhood H. pylori infection do not currently exist, there is reasonable evidence to support treatment in children with gastric or duodenal ulcer, gastric MALT (mucosa-associated lymphoid tissue) lymphoma and atrophic gastritis. There is no strong evidence to recommend treatment of children with H. pylori infection and recurrent abdominal pain, asymptomatic infection, children in chronic care facilities and children who have a family member with H. pylori infection. Current evidence suggests that single and dual therapy regimens for H. pylori infection in children are not effective. Triple therapy , generally the combination of 2 antibiotics and a proton pump inhibitor, given two times daily for 2 weeks appears to offer the best current treatment.

Novel therapies for Helicobacter pylori infection.

BACKGROUND: Increasing antibiotic resistance has begun to impair our ability to cure Helicobacter pylori infection. AIM: To evaluate orally administered novel therapies for the treatment of H. pylori infection. METHODS: Healthy H. pylori infected volunteers received: (a) hyperimmune bovine colostral immune globulins, (b) an oligosaccharide containing an H. pylori adhesion target, Neu5Aca2-3Galb1-4Glc-(3'-sialyllactose), or (c) recombinant human lactoferrin. Outcome was assessed by urea breath test or histological assessment of the number of H. pylori present. RESULTS: None of the novel therapies appeared effective and no adverse events occurred. CONCLUSION: Although in vitro data appeared promising, in vivo results were disappointing. Higher doses, longer duration of therapy, adjunctive acid suppression, or a combination could possibly yield better results.

The role of upper gastrointestinal endoscopy in the diagnosis and treatment of caustic ingestion, esophageal strictures, and achalasia in children.

The practice of upper gastrointestinal endoscopy in children continues to evolve. Therapeutic endoscopic procedures are now routinely performed in children. Patient preparation, sedation, and instrumentation have improved, allowing therapeutic endoscopy to be performed for a wide variety of conditions. This article focuses on the role of endoscopy in the diagnosis and care of caustic ingestion, balloon dilation of esophageal strictures, and new developments in the treatment of achalasia in children.

Diagnostic yield of oesophagogastroduodenoscopy in children with abdominal pain.

BACKGROUND: Abdominal pain is the most common indication for oesophagogastroduodenoscopy (OGD) in children. However, existing studies examining the diagnostic outcomes of OGD in children with abdominal pain are limited. AIM: To examine the diagnostic yield of OGD with biopsy in the evaluation of abdominal pain and to describe the endoscopic and histological findings in patients undergoing OGD for abdominal pain of unclear aetiology. METHODS: We performed a retrospective cross-sectional cohort study in children under 18 years of age who had OGD for the primary indication of abdominal pain, at Texas Children's Hospital and Children's Hospital of The King's Daughters from 1 January 2002 to 30 June 2005. RESULTS: Overall, OGD was diagnostic in 454 (38.1%) of the 1191 procedures, including reflux oesophagitis (23%, n = 271), Helicobacter pylori infections (5%, n = 55), peptic ulcers (3%, n = 32), eosinophilic oesophagitis (2%, n = 25), celiac disease (1%, n = 9) and Crohn's disease (0.5%, n = 7). Male gender, older age, elevated C-reactive protein and vomiting were associated with increased diagnostic yield. CONCLUSIONS: Our findings suggest that OGD is valuable for the evaluation of chronic abdominal pain in children, with a diagnostic yield of 38%. The majority of alarm symptoms and routine laboratory tests are not significantly associated with diagnostic yield.

Conscious sedation for endoscopy in the pediatric patient.

This article defines conscious sedation and ASA patient classification, reviews the pharmacologic and behavioral issues in children, and describes the essentials for successful sedation. It provides only guidelines as each patient requires individual attention. Clearly, conscious sedation works well in children for most endoscopic procedures and with continued refinement will become the method of choice.

Gastroenterologic endoscopy in children: past, present, and future.

Perhaps it was the innate human fear of the dark, or the recognition that health was linked in some way to diet. Maybe it was the Hippocratic description of dyspepsia. Whatever the reason, the human desire to peer inside the body was a driving force in the development of endoscopy. The field of gastroenterology began with the Phillip Bozzini's crude, candle-powered lichtleiter in 1805 and blossomed with the introduction of flexible gastrointestinal endoscopy by Basil Hirschowitz in the late 1950s. Pediatric gastroenterology began early 1970s. Fueled by the application of gastrointestinal endoscopy to childhood digestive disease, pediatric gastroenterology has emerged as one of the most diverse medical-surgical practices in modern medicine. Pediatric endoscopists are alerted to prolapse gastropathy, a more accurate description of an old and possibly common cause of upper gastrointestinal bleeding in children. Pediatric endoscopic retrograde cholangiopancreatography continues to evolve, with increasing use in the diagnosis of infant cholestasis and endoscopic treatment of pancreatitis. These developments suggest a need for advanced training in endoscopy for pediatric gastroenterologists. Trends in gastrointestinal endoscopy are moving toward more therapeutic procedures and less diagnostic endoscopy. Therapeutic endoscopy, for example, may soon include antireflux operations. Computer-assisted virtual endoscopy and the wireless pill videoendoscope may replace diagnostic endoscopy eventually. The purpose of this review is to explore the origins of pediatric endoscopy, discuss current innovations, and look at the future of our discipline.

Colon cleanout preparations in children and adolescents.

To determine the effectiveness and associated side effects of a variety of bowel cleansing preparations before colonoscopy, 52 children, ages 4 months to 18 years, were evaluated. Data gathered included type of cleanout preparation used, adequacy of the preparations, and side effects. Twenty-two combinations of laxatives, lavages, enemas, and rectal suppositories were used. Side effects were reported in 90% of patients. In 79% of the patients the colon was clean or clean with some liquid feces, allowing a complete examination of the colon. Formed feces were present in 21% of the subjects, and cleanout preparations were so inadequate in 9% of the subjects that the procedure had to be rescheduled. The most effective preparations were X-Prep liquid+Adult Fleet enema, GoLYTELY, and GoLYTELY+Adult Fleet enema, all of which allowed a complete examination of the colon. Pediatric Fleet ready-to-use enema was the least effective when used alone. X-Prep liquid+Adult Fleet enema caused the highest number of side effects (18%), while Pediatric Fleet ready-to-use enema alone caused the least (4%). The most effective cleanout preparation with the fewest side effects was GoLYTELY, used alone or in combination with enemas. These results suggest that many typical cleanout preparations yield inadequate results, wasting time or money, and argue in favor of the use of more effective preparations with fewer side effects, such as GoLYTELY.

Serologic and mucosal immune response to rotavirus infection in the rabbit model.

We examined the humoral immune response to rotavirus infection in specific pathogen-free rabbits inoculated and challenged orally with rabbit Ala rotavirus (7.5 x 10(5) to 1 x 10(7) PFU). The humoral immune response in both serologic and mucosal samples was monitored by using total antibody enzyme-linked immunosorbent assays (ELISAs), isotype-specific ELISAs, and plaque reduction neutralization assays. Following a primary infection, all rabbits shed virus and serologic and mucosal antibody responses were initially detected by 1 week postinoculation. Intestinal immunoglobulin M was detected by 3 days postinoculation, and secretory immunoglobulin A was detected by 6 days postinoculation. Following challenge, rabbits were protected (no detectable virus shedding) from infection. An anamnestic immune response was observed only with mucosal neutralizing antibodies, and all serologic and mucosal immune responses persisted at high levels until at least 175 days postchallenge (204 days postinoculation). Detection of neutralization responses was influenced by the virus strain used in the neutralization assay; all inoculated rabbits developed detectable serum and intestinal neutralizing antibodies against the infecting (Ala) virus strain. Neutralization activity in both serum and mucosal samples was generally, but not exclusively, homotypic (VP7 serotype 3) after both primary and challenge inoculations with Ala virus. Heterotypic serum neutralization activity was observed with serotype 8 (9 of 12 rabbits) and 9 (12 of 12 rabbits) viruses and may be based on reactivity with the outer capsid protein VP4 or on a shared epitope in the C region of VP7. Comparisons of heterologous (serotype 3) and heterotypic neutralizing responses in mucosal and serologic samples revealed that 43% (21 of 49) of the responses were discordant. In 19 of 49 (39%) of these cases, a heterotypic serologic response was seen in the absence of a heterotypic mucosal response, but in 2 of 49 (4%) instances, a heterotypic mucosal response was seen in the absence of a concomitant serologic response. These results provide insight into factors which may affect detection of heterotypic responses.

Oxygen desaturation and cardiac arrhythmias in children during esophagogastroduodenoscopy using conscious sedation.

To determine whether oxygen desaturation and cardiac arrhythmias occur in children during esophagogastroduodenoscopy with the use of conscious sedation, we prospectively studied 34 consecutive patients between the ages of 2 months and 18 years. Patients with pulmonary, cardiac, and neurologic disorders were defined as high risk and those without were defined as normal. All patients received intravenous sedation with meperidine, diazepam, or midazolam, used alone or in combination. Pulse oximetry, respiratory rate, and lead II electrocardiogram were recorded throughout all episodes of desaturation and tachycardia. Oxygen desaturation to less than 90% occurred in 68% of normal patients and in 58% of high-risk patients during esophagogastroduodenoscopy. Seventy-five percent of the high-risk patients and 82% of the normal patients had an arrhythmia during esophagogastroduodenoscopy usually associated with oxygen desaturation. Sinus tachycardia was the most common arrhythmia, although other arrhythmias were identified. Despite the frequency of oxygen desaturation and cardiac arrhythmias, no adverse outcome was observed in any patient. Most episodes of oxygen desaturation and cardiac arrhythmia resolved spontaneously. Subdivision of patients into high-risk groups by age, sex, weight, or diameter of endoscope used did not allow prediction of oxygen desaturation or cardiac arrhythmia. Our data suggest that conscious sedation in children undergoing esophagogastroduodenoscopy is safe and free of significant adverse clinical problems. However, conscious sedation during esophagogastroduodenoscopy continues to have certain inherent risks. Therefore we strongly advocate the routine use of continuous cardiac rhythm and pulse oximetry monitoring of all children during esophagogastroduodenoscopy performed with the use of conscious sedation.

Extraesophageal associations of gastroesophageal reflux disease in children without neurologic defects.

BACKGROUND & AIMS: The potential association between gastroesophageal reflux disease (GERD) and extraesophageal manifestations remains unknown in children without neurological defects. We conducted a large case-control study to examine the association between GERD and several upper and lower respiratory disorders in these children. METHODS: We identified all patients between 2 and 18 years of age with GERD who were seen at Texas Children's Hospital between 1996 and 2000. Patients seen during the same time period without GERD were randomly selected as controls (4:1 ratio). Patients with mental retardation, cerebral palsy, or congenital esophageal anomalies were excluded. We compared the presence of several predefined upper and lower respiratory disorders in cases and controls. RESULTS: We identified 1980 patients with GERD and 7920 controls without GERD. Cases and controls were without neurological deficits or congenital esophageal anomalies. Cases were older than controls (9.2 years +/- 4.6 vs. 8.6 +/- 4.9, P < 0.0001), and were more likely to be female (51.2% vs. 47.2%, P = 0.0028) and white (60.2% vs. 41.2%, P < 0.0001). Compared with controls in univariate analyses, cases with GERD had more sinusitis (4.2% vs. 1.4%, P < 0.0001), laryngitis (0.7% vs. 0.2%), asthma (13.2% vs. 6.8%, P < 0.0001), pneumonia (6.3% vs. 2.3%, P < 0.0001), and bronchiectasis (1.0% vs. 0.1%, P < 0.0001). However, otitis media was less common in cases than controls (2.1% vs. 4.6%, P < 0.0001). After adjusting for differences in age, gender, and ethnicity in the regression analyses, GERD remained a significant risk factor for sinusitis (adjusted odds ratio [OR], 2.3; 95% confidence intervals [CI], 1.7-3.2; P < 0.0001), laryngitis (OR, 2.6; CI, 1.2-5.6; P = 0.0228), asthma (OR, 1.9; CI, 1.6-2.3; P < 0.0001), pneumonia (OR, 2.3; CI, 1.8-2.9; P < 0.0001), and bronchiectasis (OR, 2.3; CI, 1.1-4.6; P = 0.0193). CONCLUSIONS: GERD in children without neurological defects is associated with a several-fold increase in the risk of sinusitis, laryngitis, asthma, pneumonia, and bronchiectasis. Further studies are needed to examine whether a cause-effect relationship exists between GERD and these disorders in children.

Molecular biology and immunology of rotavirus infections.

Rotaviruses were first recognized about 15 years ago in association with diarrhea in children and animals. Since then, rotaviruses have been determined to be the most important viral agent that causes clinically significant diarrhea in children and a need for an effective vaccination program has been recognized. This article reviews the progress which has been made in understanding the molecular biology of rotaviruses and summarizes information on the immune responses to rotavirus infections obtained in a new animal model in rabbits. This model is useful to systematically evaluate active protective immunity following infection of seronegative animals.

Efficacy of ursodeoxycholic acid in the treatment of primary sclerosing cholangitis in children.

BACKGROUND: Ursodeoxycholic acid (UDCA) has been shown to be beneficial in reducing disease activity in adult patients with primary sclerosing cholangitis (PSC). However, there has been little published regarding PSC in children and no studies investigating the efficacy of UDCA as a treatment for PSC. METHODS: This retrospective study included 10 children who were found to have the diagnosis of PSC during the past 15 years at the Texas Children's Hospital and Herman Hospital, both in Houston, Texas. The male:female ratio was 8:2, the median age of onset was 12 years (range, 1-17 years), and eight patients had coexistent inflammatory bowel disease (IBD; six ulcerative colitis, one Crohn's disease, one unspecified). At the time of diagnosis, five patients were asymptomatic, all of whom had IBD with elevated liver enzymes and three of whom had hepatomegaly. Nine patients were treated with UDCA. The one patient who did not receive UDCA was lost to follow-up soon after diagnosis. The mean dose of UDCA was 17 mg/kg with the doses ranging from 9 to 37 mg/kg. RESULTS: There were no side effects from the medication recorded for any of the patients. These patients showed a significant reduction in alkaline phosphatase, alanine aminotransferase, aspartate aminotransferase, and gamma-glutamyl transferase at 1, 3, 6, 15, and 20 months after treatment. CONCLUSIONS: This study demonstrates that children with PSC treated with UDCA have significant improvements in liver biochemical indices. However, the long-term effect of UDCA on clinical outcome is unknown.

New treatment for rectal impaction in children: an efficacy, comfort, and safety trial of the pulsed-irrigation enhanced-evacuation procedure.

To determine the efficacy, comfort, and safety of the pulsed-irrigation enhanced-evacuation (PIEE) procedure in children, we evaluated 27 procedures performed on 24 consecutive children, 16 boys and 8 girls, 4-15 years old (mean, 8.7), with chronic constipation, encopresis, and rectal impaction. Children with known heart, lung, and/or neurologic diseases were excluded. Temperature, pulse, respiratory rate, and blood pressure were obtained prior to, during, and immediately after the procedure. Abdominal radiographs were obtained before and after the procedure, and the degree of disimpaction was estimated by a single radiologist. Patients graded the discomfort of the procedure at the time of speculum insertion and every 15 min from 1 to 5: 1, painless; 2, mild discomfort; 3, moderate discomfort; 4, severe discomfort; 5, extremely painful. Serum sodium, potassium, chloride, and bicarbonate values were obtained before, immediately after, and 90 min after the procedure and compared by paired Student t analysis. A digital rectal examination confirmed rectal impaction prior to insertion of the speculum. One course of pulsed irrigations was performed, typically lasting 25-30 min. No significant changes in vital signs were found during or after the procedure. In 18 of 23 (78%) procedures, there was at least a 50% emptying of feces from the colon immediately after the procedure. Patients continued to empty their colon up to 12 h later; thus, the procedure effectively disimpacted all patients. In terms of comfort, patients graded the PIEE a 2.6 +/- 0.75 (range, 1-5). Speculum insertion was the most uncomfortable factor and received a 2.7. No child had any indication of water intoxication.(ABSTRACT TRUNCATED AT 250 WORDS)

Rotavirus disease, but not infection and development of intestinal histopathological lesions, is age restricted in rabbits.

The rabbit model of rotavirus infection has proved to be useful for assessing active immunity and protection after infection or vaccination with virus or virus-like particles. One limitation of the rabbit model is that after experimental infection of rabbits, clinical diarrhea is not routinely induced. Lack of diarrhea in the rabbit model has been proposed to be due to the fluid absorptive capability of the cecum or attenuation of virus strains through tissue culture adaptation. To test whether a wild-type lapine rotavirus strain BAP (BAPwt) isolated from diarrheic rabbits would cause disease on passage in rabbits, 1-, 2-, 10-, and 16-week-old rabbits were orally inoculated with BAPwt, its tissue culture-adapted counterpart strain (BAP-2), tissue culture-adapted lapine strain ALA, or PBS. Lapine rotavirus infection in 1-week-old, but not >/=2-week-old, rabbits resulted in the development of disease characterized by soft, wet, yellow-to-brownish-green partially formed-to-liquid stools observed only at the time of virus antigen shedding. The level and duration of virus shedding after infection were prolonged in 1-week-old rabbits compared with rabbits >/=2 weeks of age. Although diarrhea was not observed beyond the first 2 weeks of life, histopathological changes, including villus shortening and fusion, increased vacuolation of epithelial cells, and mononuclear infiltration of the lamina propria, were observed throughout the small intestine between 12 and 120 h after ALA infection in 1-week-old, 1- to 2-month-old, and 11-month-old rabbits. In 11-month-old rabbits, onset of intestinal damage appeared to be slightly delayed, was less severe, and was not observed in the duodenum. There were no differences in the immune responses to rotavirus infection in rabbits of different age groups (1 week to 5 years of age). All lapine rotavirus-inoculated rabbits seroconverted and were protected from virus challenge at 28 days postinoculation. Like in mice, rotavirus disease is age restricted in rabbits.

Extraintestinal rotavirus infections in children with immunodeficiency.

Some rotavirus strains, including vaccine candidates, have been demonstrated to cause hepatitis in immunodeficient and malnourished mice and to grow in human liver cells. To determine whether rotavirus spreads outside the intestine in naturally infected children, we examined tissues from four immunodeficient children affected with severe combined immunodeficiency disease, acquired immunodeficiency disease syndrome, or DiGeorge syndrome. Chronic rotavirus-related diarrhea, which persisted until death, had also developed in each child. Using indirect immunoperoxidase techniques, we identified rotavirus antigen in the liver and kidney with a hyperimmune guinea pig antiserum prepared to double-shelled rotavirus particles. Similar immunostaining with an antiserum to a rotavirus nonstructural protein (NS26) provided evidence of active virus replication. The observed reactivity was eliminated specifically when serial sections were immunostained with the same antiserum that had been absorbed with either double-shelled rotavirus particles or NS26. Immunostaining was not observed in the liver of children with other diseases (alpha 1-antitrypsin deficiency, inspissated bile syndrome, and acute rejection of a transplanted liver). These findings demonstrate that rotavirus infections in children can extend beyond the intestinal tract. Further studies are warranted to determine whether extraintestinal rotavirus replication occurs in children without severe immunodeficiency, such as malnourished children.