Course of life into adulthood of patients with biliary atresia: the achievement of developmental milestones in a nationwide cohort

PurposeTo investigate the course of life of young adults diagnosed with biliary atresia (BA) in infancy by comparing patients who did and did not underwent transplantation with an age-matched Dutch reference group.MethodsAll patients from the Dutch BA registry, aged >18 years, were invited to complete the course of life questionnaire.ResultsForty patients participated (response = 74%). Twenty-five had not undergone transplantation; 15 had undergone orthotopic liver transplantation. One significant between-group difference was found, namely in substance use and gambling. BA patients who underwent transplantation reported less use than the reference group (p = .01, moderate effect size). Additional moderate effect sizes were found for differences in psychosexual and social development and antisocial behavior. Patients who underwent transplantation had lower scores than one or both other groups.ConclusionsDevelopment of BA survivors who did not undergo transplantation seems not delayed, whereas that of transplanted patients does seem somewhat delayed. However, patients who underwent transplantation display less risk behavior. Larger samples are necessary to confirm these findings.     

Growth retardation as a result of a presumption of multiple food hypersensitivity

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.     

Halitosis in two children caused by a foreign body in the nose

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.     

A critical appraisal of current management practices for infant regurgitation – recommendations of a working party

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: (1A) parental reassurance; (1B) milk-thick ening agents; (2) prokinetics; (3) positional therapy as an adjuvant therapy; (4A) H2-blockers; (4B) proton pump inhibitors; (5) surgery. Received: 26 July 1996 / Accepted: 22 November 1996     

Noninvasive hemodynamic assessment of vasospasm in patients with primary Raynaud's phenomenon

In order to assess vasospastic ischemic disease objectively 41 patients with primary Raynaud's phenomenon and 21 normal volunteers were investigated noninvasively by digital systolic blood pressure and digital skin temperature measurements before and after instant cold provocation. Much care had been taken to be certain that all patients had primary Raynaud's phenomenon. The results of digital pressure measurements appeared to be affected by interindividual variations in systemic systolic blood pressure. By introducing a digit-to-brachial systolic blood pressure index (DBI), such variations could be eliminated. Instant cold provocation did not change DBI significantly. No pressure drop due to a closing phenomenon could be observed. Both digital skin temperature and DBI differed significantly between healthy males and females. In the patient group males and females did not show significant differences. The results in healthy females were hardly different from the results in patients. Healthy males could be well discriminated from patients with both techniques. Sex differentiation appeared to be essential for the objective assessment of primary Raynaud's phenomenon.     

Incomplete intestinal absorption of fructose.

Intestinal D-fructose absorption in 31 children was investigated using measurements of breath hydrogen. Twenty five children had no abdominal symptoms and six had functional bowel disorders. After ingestion of fructose (2 g/kg bodyweight), 22 children (71%) showed a breath hydrogen increase of more than 10 ppm over basal values, indicating incomplete absorption: the increase averaged 53 ppm, range 12 to 250 ppm. Four of these children experienced abdominal symptoms. Three of the six children with bowel disorders showed incomplete absorption. Seven children were tested again with an equal amount of glucose, and in three of them also of galactose, added to the fructose. The mean maximum breath hydrogen increases were 5 and 10 ppm, respectively, compared with 103 ppm after fructose alone. In one boy several tests were performed with various sugars; fructose was the only sugar incompletely absorbed, and the effect of glucose on fructose absorption was shown to be dependent on the amount added. It is concluded that children have a limited absorptive capacity for fructose. We speculate that the enhancing effect of glucose and galactose on fructose absorption may be due to activation of the fructose carrier. Apple juice in particular contains fructose in excess of glucose and could lead to abdominal symptoms in susceptible children.     

The relation between gastro-oesophageal reflux, sleeping-position and sudden infant death and its impact on positional therapy

 Many infants do regurgitate. The recommend-ed 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°) 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 recom-mended 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 diag-nostic possibilities (infection, etc.) are considered rejected. Received: 16 January 1996 / Accepted: 24 May 1996