[Abdominal pain and vomiting: a 4-year old boy with relaxation of diaphragm].
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Biomedical subjects
Publications and source records attributed to C M Kneepkens.
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The [13C]octanoic acid breath test was used for the measurement of differences in gastric emptying in preterm infants for the evaluation of pharmacological therapy. In order to perform a good intra-individual comparison of the gastric emptying in preterm infants under non-standardisable test conditions, we adjusted t1/2 for variations in non-recovered label (=label retention) and introduced an "effective half 13CO2 breath excretion time" t1/2eff = t1/2/m expressed as min per percentage of the cumulative dose recovered. In a pilot study, we investigated the action of the gastrointestinal prokinetic drug cisapride on gastric emptying in seven premature infants, of whom four suffered from gastric stasis and three had constipation. The postnatal age and weight at the start of treatment ranged from 15 to 64 days and from 815 to 1635 g, respectively. All infants received the standard formula for premature infants (Nenatal, Nutricia). Cisapride was administered orally 0.2 mg/kg, four times daily. The changes in gastrointestinal motility were studied using the total bowel transit time of carmine red. After 7 days of treatment in all children, the gastric emptying coefficient and the half 13CO2 breath excretion time adjusted for label retention were improved (n=7, the gastric emptying coefficient range before treatment was 1.69-3.34 (mean 2.59 +/- 0.80) and after treatment it was 2.79-3.76 (mean 3.28 +/- 0.30); the half 13CO2 breath excretion time adjusted for label retention range before treatment was 3.0-14.7 min/% dose (mean 7.0 +/- 5.0) and after treatment 2.6-4.0 min/% dose (mean 3.1 +/- 0.6). The total bowel transit time was only slightly improved in two patients (n=7, mean total bowel transit time before: 23.7 h compared to mean total bowel transit time after 7 days of treatment: 35.5 h). Side effects during cisapride treatment were not seen. We conclude that in premature infants cisapride is effective in shortening gastric emptying time and reducing gastric stasis; the therapeutic role in constipation has to be further investigated.
Two sisters, aged 5 and 6 years, were admitted to the hospital because of growth retardation accompanying supposed multiple food hypersensitivity. The older girl had asthma. Her symptoms had given cause for several non-regular practitioners to diagnose her and subsequently her sister as having 'food hypersensitivity'. The diet they were put on was further restricted by their parents when the symptoms did not subside. The diet was shown to be very monotonous and the energy supply was only half of the recommended daily allowances. Stepwise normalization of the diet was achieved and both girls showed catch-up growth in the following years. Food hypersensitivity has a considerably higher incidence when self reported than when diagnosed according to established criteria (elimination, provocation, re-elimination). Dietary changes in children should always take place under the supervision of a dietician, who can ensure that the diet is nutritionally adequate.
In two boys aged 4.5 and 1.5 years with halitosis a nasal foreign body was found. After removal the foetor disappeared. Little is known about the epidemiology of halitosis in children. Apart from specific odours with certain systemic disorders, local pathology such as chronic sinusitis, upper and lower respiratory tract infections and to a lesser degree gastrointestinal disorders may be the cause of the offensive smell. As in adults, bad breath in children is usually related to poor oral hygiene or disease of the oral cavity. The first-line treatment is proper oral hygiene and if necessary dental sanitization. In resistant cases further evaluation should be aimed at disclosing the causative pathology, in which case in children the possibility of a nasal foreign body should also be considered.
UNLABELLED: We describe a 6-year-old boy admitted with lethargy and somnolence. Laboratory tests showed hyperammonaemia (arterial level 186 micromol/l) and slightly elevated prothrombin time. The patient was treated with sodium benzoate, lactulose and a protein-restricted diet. This resulted in an insufficient decrease in blood ammonia levels. Metabolic investigations were unrevealing apart from a slightly elevated urinary glutamine concentration. Liver tissue showed steatosis and mildly decreased activity of N-acetylglutamate synthase suggesting partial deficiency. Treatment with N-carbamyl glutamate did not affect serum ammonia levels. Colour Doppler sonography and MR angiography demonstrated a patent ductus venosus. After surgical ligation of the ductus venosus, serum ammonia levels returned to normal and mental and motor performance improved markedly. CONCLUSION: In late onset hyperammonaemia, partial N-acetylglutamate synthase deficiency and portocaval shunt should be ruled out.
The prokinetic drug cisapride is widely used for treating gastro-oesophageal reflux in infants and children. As in the Netherlands it is not officially registered for use in these age groups, prescribing doctors have problems obtaining adequate information on proper use and side effects. In the treatment of pediatric gastro-oesophageal reflux with cisapride the major recommendations include not to exceed the maximum dose of 0.2 mg/kg four times daily, to avoid comedication with imidazoles and macrolides and to check the QTc interval in patients with known risk factors for QTc prolongation.
For the diagnosis of coeliac disease (CD) to be made in children and for the prescription of a gluten-free diet, clear histological changes in the small bowel biopsy sample have to be found. Serological testing for antibodies against endomysium and gliadin has only a preliminary significance. In children with strong clinical suspicion of CD, histological investigation of a small bowel biopsy should be performed independently of the results of serological screening (endomysium and gliadin antibodies). In children with less clear clinical suspicion of CD, serological screening may be used to select those who need investigation of small bowel histology. The number of small bowel biopsies that should be performed has been changed. In children < 2 years with clinical symptoms and small bowel villous atrophy, CD is confirmed when symptoms and atrophy significantly improve during gluten-free diet, while small bowel histology significantly deteriorates during gluten challenge. In children > 2 years with clinical symptoms combined with abnormal serology and villous atrophy, CD is confirmed when during gluten-free diet the symptoms disappear and serology improves. In case of family screening, diseases associated with CD or Down's syndrome, CD is confirmed when symptoms (if any), and previous abnormal serology and small bowel histology significantly improve during gluten-free diet.
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A girl was born after an uneventful pregnancy of 36 weeks. Prenatally, distended bowel loops had been seen on ultrasound. Multiple small-bowel atresia was diagnosed and treated surgically. In the course of the next eleven weeks, previously patent segments of small bowel became obstructed. In 4 separate operative sessions, several segments of jejunum and ileum were resected, leaving 23 cm of ileum with the ileocecal valve in place. On microscopic examination of all resected material, necrosis of the mucosa was found consistent with ischemia. The child survived and tolerated full enteral feeding at the age of 8.5 months. The origin of the progressive obliterating process remains unknown.
BACKGROUND: Cisapride is a gastrointestinal prokinetic agent that is used worldwide in the treatment of gastrointestinal motility-related disorders in premature infants, full-term infants, and children. Efficacy data suggest that it is the most effective commercially available prokinetic drug. METHODS: Because of recent concerns about safety, a critical and in-depth analysis of all reported adverse events was performed and resulted in the conclusions and recommendations that follow. RESULTS: Cisapride should only be administered to patients in whom the use of prokinetics is justified according to current medical knowledge. If cisapride is given to pediatric patients who can be considered healthy except for their gastrointestinal motility disorder, and the maximum dose does not exceed 0.8 mg/kg per day in 3 to 4 administrations of 0.2 mg/kg (not exceeding 40 mg/d), no special safety procedures regarding potential cardiac adverse events are recommended. However, if cisapride is prescribed for patients who are known to be or are suspected of being at increased risk for drug-associated increases in QTc interval, certain precautions are advisable. Such patients include those:(1) with a previous history of cardiac dysrhythmias, (2) receiving drugs known to inhibit the metabolism of cisapride and/or adversely affect ventricular repolarisation, (3) with immaturity and/or disease causing reduced cytochrome P450 3A4 activity, or (4) with electrolyte disturbances. In such patients, ECG monitoring to quantitate the QTc interval should be used before initiation of therapy and after 3 days of treatment to ascertain whether a cisapride-induced cardiac adverse effect is present. CONCLUSIONS: With rare exceptions, the total daily dose of cisapride should not exceed 0.8 mg/kg divided into 3 or 4 approximately equally spaced doses. If higher doses than this are given, the precautions above are advisable. In any patient in whom a prolonged QTc interval is found, the dose of cisapride should be reduced or the drug discontinued until the ECG normalizes. If the QTc interval returns to normal after withdrawal of cisapride, and the administration of cisapride is considered to be justified because of its efficacy and absence of alternative treatment options, cisapride can be restarted at half dose with control of the QTc interval. Unfortunately, at present, normal ranges of QTc interval in children are unknown. However, a critical analysis of the literature suggests that a duration of less than 450 milliseconds can be considered to be within the normal range and greater than 470 milliseconds as outside it.
UNLABELLED: Many infants do regurgitate. The recommended therapeutic approach starts with postural and dietary measures, followed by antacids and prokinetics. However, the recent findings regarding the increased risk for sudden infant death (SID) in the prone sleeping position challenge the current recommendations. Management of regurgitation should in the first place aim at reducing parental anxiety. Postural treatment favouring the prone-elevated (30 degrees) position is no longer recommended as a first line treatment of regurgitation, despite its efficacy, because of the unexplained association of SID with the flat prone sleeping position. Favouring the prone elevated position would result in an increased parental anxiousness. CONCLUSION: Positional treatment can only be recommended in children beyond the age of SID risk, or as an adjuvant therapy in cases resistant to reassurance, thickeners and prokinetics and in whom other diagnostic possibilities (infection, etc.) are considered rejected.
Regurgitation is a common manifestation in infants below the age of 1 year and a frequent reason of counselling of general practitioners and paediatricians. Current management starts with postural and dietary measures, followed by antacids and prokinetics. Recent issues such as an increased risk of sudden infant death in the prone sleeping position and persistent occult gastro-oesophageal reflux in a subset of infants receiving milk thickeners or thickened "anti-regurgitation formula" challenge the established approach. Therefore, the clinical practices for management of infant regurgitation have been critically evaluated with respect to their efficacy, safety and practical implications. The updated recommendations reached by the working party on the management of infant regurgitation contain five phases: (1 A) parental reassurance; (1 B) milk-thickening agents; (2) prokinetics; (3) positional therapy as an adjuvant therapy; (4 A) H2-blockers; (4 B) proton pump inhibitors; (5) surgery.
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Breath hydrogen (H2) studies have made clear that small intestinal absorption of fructose is limited, especially in toddlers. Malabsorption of fructose may be a cause of recurrent abdominal pain and chronic nonspecific diarrhea (toddler's diarrhea). Fructose absorption is facilitated by equimolar doses of glucose and, as we have found, amino acids (especially L-alanine); the mechanism underlying this effect remains unclear. To study fructose absorption in a more direct way, we combined breath H2 studies with breath 13CO2 studies. Gastric emptying was studied by using L-glycine-1-13C in 4 children from 12.1 to 16.0 years of age. After 25 gm of fructose and 27.5 gm of glucose, when given together, gastric emptying was significantly (p<0.05) slower than with either sugar alone. In a second series of experiments, 5 children from 12.0 to 15.9 years of age were tested with 25 gm of fructose, alone and with equimolar doses of glucose and L-alanine, and 4 younger children from 3.1 to 6.1 years of age were tested with 2 gm/kg (max 37.5 gm) fructose, alone or with an equimolar dose of L-alanine. All fructose solutions were enriched with 15 mg of D-fructose-13C-6. In all 9 children, fructose was malabsorbed as judged by breath H2 increases > or = 20 ppm, and the addition of glucose or L-alanine resulted in significantly lower breath H2 increases (p < or = 0.005 for glucose, p < or = 0.001 for alanine). In contrast, the addition of alanine or glucose did not change the pattern of breath 13CO2 excretion in the 5 older children, whereas in the 4 younger children (with relatively higher doses), L-alanine addition resulted in significantly lower increases in breath 13CO2. In the latter group, for each time point, breath H2 and 13CO2 concentrations after fructose were compared with those after fructose plus L-alanine; in 20 out of 24 points, both H2 and 13CO2 were higher after fructose. These results suggest that 13CO2 not only originated from the oxidation of absorbed substrate but also, at least in part, from colonic bacterial metabolism. For the detection of [correction of or] fructose malabsorption--as opposed to, for instance, lactose--the 13CO2 breath test seems to be of limited value.
Chronic nonspecific diarrhea, or toddler's diarrhea, is a frequently encountered disorder of defecation in otherwise healthy children. Although the precise pathophysiology remains to be elucidated, evidence suggests that toddler's diarrhea primarily is a gut motility disorder, modulated by dietary factors. Although the role of low-fat diets has since long been established, the liberal consumption of fruit juices and soft drinks is considered an equally important factor. Normalization of the child's diet, especially with regard to fat, fiber, fluids, and fruit juices, usually suffices to attain resolution of the diarrhea.
Regurgitation in infants is a common problem. Recent issues, such as the increased risk of sudden infant death in the prone sleeping position, the finding of persisting occult gastro-oesophageal reflux with feed thickeners, and the increasing awareness of the cost-benefit ratio of medications may challenge the currently recommended management approach. A round table was organized to elaborate on the impact of (i) the pro supine sleeping campaigns in relation to sudden infant death and (ii) advancement in medical treatment on therapeutic strategies in regurgitating infants. The participants were opinion leaders from Europe and North America (Belgium, Canada, France, UK, Italy, Switzerland and The Netherlands). The importance of parental reassurance is stressed. As a consequence of the supine sleeping campaigns aiming to decrease the incidence of sudden infant death syndrome, the "prone elevated sleeping position" is no longer advised as a first-line therapeutic approach, although it is still recommended in "complicated reflux". It is emphasized that milk thickeners are an adequate therapeutic tool for regurgitation, but not in reflux disease. According to the literature, the efficacy of (alginate )antacids, although very popular in some countries, is questionable. These recommendations will be of interest to first-line paediatricians, since about 40% of their patients, according to the literature, present because of regurgitation.
Chronic functional constipation (CFC) may be difficult to recognise and information regarding its long term prognosis is scarce. The records of 244 children with CFC, aged 0-18 years, were analysed for symptoms at presentation and results of treatment, and long term outcome was evaluated by means of a telephone interview in 137 patients discharged for more than one year. The patients presented with a great variety of symptoms, only 22% having infrequent defecation of increased consistency, another 22% having an obviously normal defecation pattern. The mean duration of treatment was 13 months. At the time of discharge, 69% of the patients still used laxatives. At a median of four years after discharge, 66% of the children were free of symptoms and without medication, 39% having experienced a recurrence. It is concluded that CFC may be difficult to recognise and can be alleviated by an intensive laxative regimen. Recurrence of symptoms is common, but the long term prognosis is good in most patients.