Cyclic vomiting syndrome in adults.

Cyclic vomiting syndrome (CVS) was initially described in children but can occur in all age groups. Cyclic vomiting syndrome is increasingly recognized in adults. However, the lack of awareness of CVS in adults has led to small numbers of diagnosed patients and a paucity of published data on the causes, diagnosis and management of CVS in adults. This article is a state-of-knowledge overview on CVS in adults and is intended to provide a framework for management and further investigations into CVS in adults.

Patient web-resource interest and internet readiness in pediatric inflammatory bowel disease.

OBJECTIVE: The internet has been touted as a cost-effective method of providing valuable patient education and support resources. However, little is known of the level of internet interest in or access to web-based resources of most chronically ill populations generally, and pediatric populations in particular. Web-based patient resources could be especially applicable to the pediatric inflammatory bowel disease (IBD) population given the potential appeal of an anonymous forum for this group. METHODS: Tertiary-care clinic patients aged 8-18 years and diagnosed with IBD were surveyed by mail. chi and/or analysis of variance were used to compare demographic data from nonresponders and responders and for subgroup analyses. RESULTS: Of 162 eligible patients, 63 (38.9%) completed the survey. Responders did not differ significantly from nonresponders by age, sex, or distribution of disease. Overall, 98.4% of those surveyed had internet access, with 61.9% having access at school. Ninety-five percent had used the internet, while 91% expressed interest in visiting a pediatric IBD website for general IBD information (83%), for support (73%), to pose questions (78%) or read (73%) or respond (60%) to questions, or to chat with others (46%). Despite expressed interest, far fewer had ever used the internet as an IBD information (52.4%) or support (9.5%) resource. Similarly, although 15.8% anticipated visiting a pediatric IBD site weekly, only 1.5% had done so in the week of the survey. CONCLUSIONS: Reported levels of interest in web-based patient resources are high among pediatric IBD patients, yet use may be infrequent.

e-Health? Clinical information network interest and impediments in a community paediatric setting.

BACKGROUND: Increased interest in implementing evidence-based medicine in paediatric practice has led to the development of a variety of electronic clinical and decision-making support tools. Electronic medical records and information resources have the potential to improve both the clinical and cost effectiveness of paediatric community practice at the point of care. Barriers to the successful implementation of clinical information Intranet resources include physician attitudes, as well as system and practitioner capabilities. OBJECTIVE: To examine Ontario's community care paediatricians' electronic information resource needs and interest in accessing the proposed Central West Ontario Pediatric Information Network (CWPIN). DESIGN: Cross-sectional, interviewer-facilitated, structured survey. POPULATION STUDIED: Paediatricians providing community care in the Central West Ontario Health Region. MAIN RESULTS: Three-quarters of regional community paediatricians responded to the survey. Of those surveyed, 98% expressed interest in gaining access to the CWPIN. Computer literacy, suggested by home computer and Internet use rates (88% and 81%, respectively), did not differ significantly by age or sex. Other factors that may affect network use, such as workplace computer use and allied personnel role assignment, differed by sex, indicating potentially greater CWPIN use among male practitioners. CONCLUSIONS: Physicians reported an overwhelming interest in gaining access to and using the CWPIN. Disparities in current workplace but not home-based computer use by sex indicate that workplace role allocation, rather than computer literacy, may explain sex differences in CWPIN use rates. Attitudinal and computer proficiency issues did not appear to be obstacles to implementing the clinical information Intranet resource in the region.

Evaluation of neurocardiac signals in pediatric patients with cyclic vomiting syndrome through power spectral analysis of heart rate variability.

OBJECTIVE: To investigate autonomic regulation of neurocardiac signals in pediatric patients with cyclic vomiting syndrome (CVS). METHODS: Fourteen patients with CVS, ages 3 to 16 years, were screened to eliminate any underlying cause for their symptoms, although 11 of the 14 patients had a history of migraine in the immediate family. Analysis of autonomic regulation was accomplished through power spectral analysis of the beat-to-beat heart rate variability signal. Data from affected patients were compared with data from 38 control subjects, ages 5 to 16 years, by a one-way analysis of variance. A measure of sympathovagal balance was obtained by computing a ratio of power in the low-frequency band (0. 02-0.15 Hz) to the power in the high-frequency band (0.15-0.4 Hz). RESULTS: Pediatric patients with CVS have an elevated sympathetic modulation of the sinus node as represented by the low frequency/high frequency ratio (1.45 +/- 0.42 in patients vs 0.89 +/- 0.29 in healthy control subjects, P <.001). CONCLUSIONS: The patients with CVS we studied have an autonomic imbalance with enhanced sympathetic and diminished parasympathetic vagal modulation of the heart.

Bone mineral metabolism in pediatric inflammatory bowel disease.

The development of reliable techniques to measure bone densitometry and evolving effective drug treatment have kindled great interest in the diagnosis and treatment of osteoporosis in adults with inflammatory bowel disease. A number of studies have examined the prevalence of abnormal bone mineral metabolism in children and adolescents. Studies, conducted over the past decade, indicate a greater likelihood of clinically significant problems in Crohn's disease than in ulcerative colitis. Corticosteroids have been proven to impair bone mineral status. It is increasingly clear that inflammation and other factors play a bigger role than malabsorbtion of minerals or vitamin D in most patients. As the use of the bisphonate class of drugs is limited in pediatric patients, there is a need to emphasize the role of diet and exercise in children and teenagers, particularly in those affected by inflammatory bowel disease.

Patients' diets and preferences in a pediatric population with inflammatory bowel disease.

PURPOSE: To determine the dietary practices of the pediatric inflammatory bowel disease population at the Children's Hospital of the Hamilton Health Sciences Corporation and the reported effectiveness of those diets. PATIENTS AND METHODS: A questionnaire mailed to 153 pediatric patients was returned by 125 patients (76 Crohn's disease [CD] and 49 ulcerative colitis [UC] patients)--an 82% response rate. RESULTS: The median age of respondents was 13 years, and 62% were male. Ninety per cent and 71% of CD and UC patients, respectively, had changed their diets since diagnosis. Caloric supplements (eg, BOOST [Mead Johnson Nutritionals]), sole source nutrition, low fibre and lactose-free diets were used by more than 15% of CD patients, whereas lactose-free, nonspicy, low acid, additive-free, caloric supplement and low fibre diets were used by more than 15% of UC patients. A diet supplement was more commonly used in CD patients (P < 0.05) and an additive-free diet in UC patients. Corn and corn products, nuts, milk and bran were avoided by more than 20% of CD and UC patients; however, more CD than UC patients avoided corn and corn products. In addition, UC patients (more than 20%) also avoided tomato, other dairy (nonfluid milk-based products and foods containing milk products), chocolate, cheese, wheat, tomato sauces and fruit juice. A benefit was reported for 103 of 141 reported diets, with the most commonly alleviated symptoms being abdominal pain, diarrhea and flatulence. CONCLUSION: Many children with inflammatory bowel disease have altered their diets to manage their disease and have attributed symptomatic relief to these diets.

Community paediatrics in Ontario: 1996 paediatric physician resource study.

Physician resource planning is a basic underpinning of the management of the health care system. Accurate data on physician numbers and distribution are essential to the process of planning. This paper presents the results of a study commissioned by the Paediatric Executive of the Ontario Medical Association to provide an updated profile of the number and distribution of paediatricians in Ontario in 1995/96.

Glycogen storage disease in adults.

OBJECTIVE: To identify complications amenable to prevention in adults with glycogen storage disease (GSD) types Ia, Ib, and III and to determine the effect of the disease on social factors. DESIGN: Case series and clinical review. SETTING: Referral medical centers in the United States and Canada. PATIENTS: All patients with GSD-Ia (37 patients), GSD-Ib (5 patients), and GSD-III (9 patients) who were 18 years of age or older. MEASUREMENTS: Ultrasound or radiographic studies identified liver adenomas, nephrocalcinosis, or kidney stones. Radiographic studies identified osteopenia. Reports of the clinical examination, serum chemistry results, and social data were obtained. RESULTS: For patients with GSD-Ia, problems included short stature (90%), hepatomegaly (100%), hepatic adenomas (75%), anemia (81%), proteinuria or microalbuminuria (67%), kidney calcifications (65%), osteopenia or fractures or both (27%), increased alkaline phosphatase (61%) and gamma-glutamyltransferase (93%) activities, and increased serum cholesterol (76%) and triglyceride (100%) levels. Hyperuricemia was frequent (89%). Patients with GSD-Ib had severe recurrent bacterial infections and gingivitis. In patients with GSD-III, 67% (6 of 9) had increased creatinine kinase activity. Four of these patients had myopathy and cardiomyopathy. CONCLUSIONS: For GSD-Ia, hyperuricemia and pyelonephritis should be treated to prevent nephrocalcinosis and additional renal damage. For GSD-Ib, granulocyte-colony-stimulating factor may prevent bacterial infections. For GSD-III, more data are required to determine whether the myopathy and cardiomyopathy can be prevented. Most of the patients with GSD-I and GSD-III had 12 or more years of education and were either currently in school or employed.

Longitudinal assessment of growth, mineral metabolism, and bone mass in pediatric Crohn's disease.

In children with inflammatory bowel disease, controversy continues about the use of long-term alternate day prednisone therapy (ADP) to suppress disease activity and to encourage appetite and growth. One possible side effect of both disease process and prednisone therapy is risk of development of osteoporosis. To evaluate this risk factor, growth, biochemical indices of mineral and vitamin D status, and bone mass were measured in nine adolescents with Crohn's disease (CD) who were treated with ADP (0.3 mg/kg > 3 months per year) compared with eight adolescents treated with minimal ADP exposure (< 3 months per year). Single photon densitometry was used to measure bone mineral mass at the 1/3 distal radius three times over 2 years. Mean age of the 17 CD boys was 13.9 +/- 2.1 years at baseline. CD patients had lower bone BMC/BW mineral content/bone width (BMC/BW) compared with age- and height-matched normal boys at all times. The difference was less when compared to height-matched normal values as CD patients were shorter than healthy reference boys. Plasma 1,25-dihydroxyvitamin D, alkaline phosphatase, and parathyroid hormone significantly increased with treatment of disease but there were no differences between treatment groups. CD patients treated with ADP had similar heights and weights at baseline and demonstrated similar linear growth over 2 years (9.1 cm/2 years) to CD patients without ADP (10.3 cm/2 years). In both groups, BMC/BW increased significantly from year 1 to year 2, but absolute values for bone mass did not differ between the groups.

The single-subject randomized trial. A useful clinical tool for assessing therapeutic efficacy in pediatric practice.

The purpose of a single-subject randomized trial is to assess objectively the efficacy of a specific therapeutic intervention in an individual patient. Treatment is randomly alternated with placebo over a number of study periods. Specific outcome measures are recorded blindly and later compared via paired statistical analysis. Single-subject trials have long been successfully performed in adults, but rarely in children. We present single-subject trials of two pediatric patients done to assess the effect of cisapride on symptoms arising from gastroesophageal reflux. In the first patient, the drug affected neither vomiting nor gagging, although stool frequency increased. Since the symptoms of concern were unaffected, cisapride was discontinued. In the second patient, use of cisapride led to a significant decrease in vomiting and wheezing; the drug was therefore incorporated into the therapeutic regimen. Single-subject randomized trials are inexpensive and simple and can be used by the family physician, pediatrician, or pediatric surgeon in daily practice. They permit the rational use of effective therapy and the abandonment of ineffective measures.

Oral and intravenous rehydration of children.

We compared oral rehydration therapy (ORT) with rapid intravenous rehydration (IV) in 42 young children with mild to moderate dehydration due to diarrhea. Overall, treatment was successful for 82% of the ORT patients and for 78% of the IV patients. Many physicians in hospitals are unfamiliar with the use of ORT for treating dehydration.

Home esophageal self-dilatation in children.

Home esophageal self-dilatation permits greater independence and more frequent treatments than intermittent hospital procedures for patients with esophageal strictures. The article describes an innovative program for teaching esophageal self-dilatation to preadolescent children, using a form of creative visualization. The child focuses on a 2-minute mental image (e.g., video game, a popular song, or swimming a race) from his/her own experience while passing a Maloney bougie. A nasal breathing technique is used, and active encouragement is provided. Topical lidocaine and oral diazepam can be used initially to assist relaxation and are stopped when the child gains confidence with the technique. The authors taught esophageal self-dilatation to three children aged 12 to 13 years with esophageal strictures. All three learned the technique within 1 week and continued it at home for between 4 and 6 months. Two of the children were taught in hospital by a nurse, and the third was taught as an outpatient by a peer who had already mastered the technique. Home esophageal self-dilatation can be successfully taught to children in the preadolescent age group. The teaching program is simple and effective and can be easily applied to other tasks such as pain control, nasogastric tube feeding, and self-injection.

Olsalazine versus sulfasalazine in mild to moderate childhood ulcerative colitis: results of the Pediatric Gastroenterology Collaborative Research Group Clinical Trial.

The safety and efficacy of olsalazine sodium was compared to sulfasalazine over 3 months in a multicenter, randomized, double-blind study of 56 children with mild to moderate ulcerative colitis. Twenty-eight children received 30 mg/kg/day of olsalazine (maximum, 2 g/day) and 28 received 60 mg/kg/day of sulfasalazine (maximum, 4 g/day). Side effects were frequent in both groups. Eleven of 28 patients (39%) on olsalazine reported headache, nausea, vomiting, rash, pruritus, increased diarrhea, and/or fever. Thirteen of 28 on sulfasalazine (46%) reported similar side effects and/or neutropenia, and four patients had the drug stopped because of an adverse reaction. After 3 months, 11 of 28 (39%) on olsalazine were asymptomatic or clinically improved, compared to 22 of 28 (79%) on sulfasalazine (p = 0.006). In addition, 10 of 28 patients on olsalazine versus one on sulfasalazine required prednisone because of lack of response or worsening of colitis (p = 0.005). The dose of olsalazine used in this clinical trial was thought to be equivalent to a standard dose of sulfasalazine, but fewer patients on olsalazine improved and a greater number had progression of symptoms when compared to sulfasalazine. Although side effects were slightly less frequent for olsalazine, the number of patients was too small to detect a clinically significant difference.

Intestinal permeability compared in pediatric and adult patients with inflammatory bowel disease.

Increased urinary excretion of 51Cr-EDTA after oral administration has been demonstrated in adults with active inflammatory bowel disease (IBD). Pediatric patients with Crohn's disease (CD) and ulcerative colitis (UC) were compared to pediatric and adult controls and adult patients with IBD using this technique. Seventy-five pediatric IBD patients, 51 (mean age 13.8 y) diagnosed with active CD and 24 (mean age 11.9 y) with active UC, were examined. These were compared to 26 pediatric controls with recurrent abdominal pain or chronic non-specific diarrhea. Further comparison was made to 80 adult controls (mean 32.0 y), 63 adults with active CD and 31 adults with active UC. After an overnight fast, 925 kBq of 51Cr-EDTA was given orally and urine collected for 24 h. Excretion of the probe by the pediatric controls and adult controls was 1.5%/24 h and 1.3%/24 h (median), respectively. Of the pediatric patients, 45/51 (88.2%) with active CD (median 3.9%/24 h) and 16/24 (66.7%) with active UC (median 4.8%/24 h) showed increased excretion. Pediatric patients with active IBD demonstrated increased probe excretion comparable to levels of adult patients. In the pediatric population, accuracy of the first test was 83.0%. Thus, urinary excretion of 51Cr-EDTA is a useful non-invasive test in the investigation of pediatric patients with gastrointestinal symptoms.

Late anastomotic ulceration after ileocolic resection in childhood [corrected].

Anastomotic ulcers are a rare late complication of ileocolic resection in children. The authors describe the cases of two patients who presented 10 and 8 years, respectively, after ileocolic resection. Both had suffered from iron-deficiency anemia caused by an anastomotic ulcer. In one patient the anemia improved after resection of the ulcer; in the second patient cholestyramine and a lactose-free diet resolved the problems of anemia and diarrhea. Anastomotic ulceration should be suspected in any child who has undergone an ileocolic anastomosis and subsequently presents with persistent iron-deficiency anemia and occult blood in the stool.

False diagnosis of intestinal obstruction in a fetus with congenital chloride diarrhea.

Intestinal obstruction is often diagnosed prenatally by ultrasound, providing an opportunity for prenatal counseling, genetic investigation, and planned delivery at a perinatal center. We describe a patient with typical features of fetal bowel obstruction, who was found at birth to have congenital chloride diarrhea. A 25-year-old white woman had marked polyhydramnios; multiple dilated, fluid-filled loops of intestine were seen in the fetal abdomen on prenatal ultrasound. However, postnatally, there was no evidence of bowel obstruction. The infant girl passed large amounts of watery stools, but tolerated feeds well. A rectal biopsy showed normal ganglion cells. On the fourth day of life her serum sodium and chloride were markedly decreased, and stool chloride levels were diagnostic of congenital chloride diarrhea. She was placed on sodium chloride and potassium chloride supplements, and her serum electrolytes normalized. Congenital chloride diarrhea is a rare, inherited condition caused by an abnormality of intestinal electrolyte transport. This case illustrates that it may present prenatally with a picture similar to that seen with intestinal obstruction.

Heterotopic gastric mucosa in the gallbladder: a cause of chronic abdominal pain in a child.

A 12-year-old boy presented with a 7-year history of intermittent colicky epigastric pain. Serial abdominal ultrasound studies showed a contracted gallbladder, and single and double dose oral cholecystograms demonstrated nonfunction. All other investigation results were normal. At cholecystectomy, there was a fibrotic stricture at the junction of the gallbladder neck and cystic duct. The small, thin-walled gallbladder contained white bile. Histologically, the gallbladder was lined with normal mucous secreting columnar epithelium. The area of stricture showed diffuse fibrosis with islands of heterotopic gastric mucosa containing all gastric mucosal cell types. One year postoperatively, there have been no further episodes of abdominal pain. This is the seventh reported case of heterotopic gastric mucosa in the gallbladder of a child causing symptoms necessitating cholecystectomy. We recommend that a child with abdominal pain and a nonfunctioning gallbladder be considered for cholecystectomy even in the absence of cholelithiasis.