Colonic wall thickening is related to age and not dose of high strength pancreatin microspheres in children with cystic fibrosis.

OBJECTIVE: Colonic fibrosis causing stricture is a recently described complication in cystic fibrosis (CF). Studies have suggested that ultrasound evidence of bowel thickening predicts this complication and that it is prevalent among children receiving large doses of high-strength pancreatin preparations. We performed ultrasound studies on our patients to look for evidence of bowel wall thickening or early stricture. METHOD: Detailed colonic ultrasounds were carried out in 33 children with CF including 25 who had been receiving high-strength pancreatin (Creon 25,000) continuously for 3 years at the time of study. RESULTS: Median lipase intake was 19 330 U/kg/day (range 0-59 880 U/kg/day) and median protease intake was 387 U/kg/day (range 0-1170 U/kg/day). The combined thickness of mucosa, sub-mucosa and muscle layers was measured in ascending, transverse and descending colon using a 7.5 MHz transducer. Measurements were also made in nine healthy controls. There was no relationship between enzyme dosage and colon thickness but simple regression identified a significant relationship (P < 0.001) between age and maximum colon thickness in all three areas. The colon of CF children was up to 50% thicker than in controls. CONCLUSIONS: Thickening of the order described elsewhere did not occur among any of the children studied. The results suggest that the most important factor determining the thickness of the CF colon is age.

Diagnosis of cystic fibrosis: Indian perspective.

Cystic fibrosis (CF) is one of the common life limiting inherited diseases in Caucasian population. Recent reports suggest that the diagnosis of cystic fibrosis in Indian children is missed or delayed due to low index of suspicion. The diagnosis of cystic fibrosis is suspected by the typical clinical features and should be confirmed by doing sweat chloride estimation. If sweat test is not available, ancillary tests including blood electrolyte and acid base balance, airway microbiology, tests to identify pancreatic insufficiency and semen analysis for obstructive azoospermia in post pubertal boys should be carried out. Positive results of these tests make the suspicion very strong. A strongly suspected case should be treated as cystic fibrosis, but for giving a diagnosis of CF, sweat test should be done from the nearest centre where it is available. In the presence of typical clinical features with borderline sweat chloride values sweat test should be repeated 2-3 times and the child should be investigated for alternative diagnosis. In the absence of alternative diagnosis with consistently high or borderline sweat chloride values an attempt should be made to get tests for mutations.

A caecal diaphragm in cystic fibrosis.

A right iliac fossa mass may be a difficult diagnostic problem in a patient with cystic fibrosis. We present a patient with such a mass who was thought to have a non-obstructing intussusception on clinical and radiological grounds. However, at laparotomy she was found to have a pathology not previously described in cystic fibrosis. She had a partial diaphragm almost blocking the lumen to her appendix. The differential diagnosis of a right iliac fossa mass is considered and the cause of her pathology discussed.

Dietary fibre and the occurrence of gut symptoms in cystic fibrosis.

OBJECTIVE: To evaluate the effect of currently recommended energy rich cystic fibrosis diets on fibre intake and to investigate the relationship between fibre intake and the occurrence of gut symptoms. METHOD: Prospective completion of non-weighed five day food diaries by 28 children with cystic fibrosis and comparison of mean daily fibre intake with age matched controls who did not have cystic fibrosis. Prospective completion of similar diaries to a total of 68 children with cystic fibrosis and comparison of fibre and lipase intake with the occurrence of gut symptoms. RESULTS: Mean daily fibre intake in children with cystic fibrosis was 7.00 g compared with 14.65 g in controls (p < 0.001). Mean daily fibre intake in eight patients troubled with moderate or severe abdominal pain was 0.144 g/kg. This was significantly lower (p < 0.01) than mean values for 22 patients with occasional but mild symptoms (0.249 g/kg) and 38 with no gut symptoms (0.312 g/kg). There was a trend towards higher pancreatic enzyme doses (lipase/kg/day) in children with abdominal pain. CONCLUSIONS: Currently recommended cystic fibrosis diets have a low fibre content. A low residue diet might be an important factor in the pathogenesis of gastrointestinal symptoms.

Lung resection in cystic fibrosis patients with localised pulmonary disease.

The results of lobar resection to treat severe localised bronchiectasis in six children with cystic fibrosis are described. Sustained clinical improvements occurred in children undergoing this surgical approach to treatment. Detailed assessment and intensive preoperative and postoperative medical treatment are essential to a favourable outcome in carefully selected patients.

Faecal interleukin-8 and tumour necrosis factor-alpha concentrations in cystic fibrosis.

Interleukin-8 (IL-8) and tumour necrosis factor-alpha (TNF-alpha) concentrations were measured in faecal samples from nine patients with cystic fibrosis and nine healthy age matched controls. The patients were assessed with Shwachman score, apparent energy absorption, pancreatic enzyme dosage, simple spirometry, and presence of pseudomonal colonisation. Median (range) wet stool IL-8 and TNF-alpha concentrations in patients were 32,113 pg/g (21,656-178,128) and 3187 pg/g (368-17,611) respectively, compared with < 43.5 pg (IL-8)/g (< 22-4079) and 99 pg (TNF-alpha)/g (< 0.26-231) in controls. IL-8 concentration was negatively correlated with Shwachman score (r = -0.79) and pancreatic enzyme dosage (r = -0.77), but not with energy absorption. Seven patients were mature enough to cooperate with spirometry. Their IL-8 concentrations correlated with percentage predicted forced expiratory volume in one second (r = -0.78). IL-8 concentration was greater in four patients with, than five without, established pseudomonal colonisation: median difference 134,583 pg/g. TNF-alpha concentration was not correlated with measures of disease severity. Faecal IL-8 concentration might reflect the severity of pulmonary inflammation in cystic fibrosis and could provide an easily obtainable marker of disease activity.

Is water out of vogue? A survey of the drinking habits of 2-7 year olds.

OBJECTIVE: To survey the drinking habits of young children with reference to the consumption of plain water, and to estimate the proportion of a child's recommended energy intake contributed by drinks. DESIGN: A prospective survey. SETTING: Health centres, mother and toddler groups, and infant schools in and around Southampton. SUBJECTS: 39 preschool and 66 infant schoolchildren. INTERVENTIONS: Parents kept a diary of all drinks consumed by the child over 48 hours. Parents were interviewed with a questionnaire about the drinking habits of their child. MAIN OUTCOME MEASURES: The type of drinks and volume of fluid consumed over 48 hours; the proportion of a child's recommended energy intake consumed through drinks. RESULTS: 72.5% of the preschool group and 50% of the infant school group never drank plain water. Squash was by far the most frequently consumed drink. 15% of the preschool group consumed just under 50% of their recommended daily energy intake in drinks. CONCLUSIONS: Young children consume large quantities of squash which constitutes a substantial energy supply. It is possible that they are conditioned at an early age to the sweet taste of drinks that may be no nutritional benefit to them.

Morbidity from excessive intake of high energy fluids: the 'squash drinking syndrome'.

OBJECTIVE: To identify children suffering morbidity from excessive intake of energy from fluids. DESIGN: Prospective enrolment of outpatients in a supervised reduction of energy rich fluid intake. SETTING: Outpatient paediatric clinic. SUBJECTS: Eight children (four boys, mean age 20.8 months, mean duration of symptoms seven months) who were referred with non-specific symptoms such as poor appetite, poor behaviour at mealtimes, poor weight gain, and loose stools. RESULTS: All children were able to reduce their intake of energy rich fluids, as prescribed. All children demonstrated an improvement in symptoms and an increase in weight. CONCLUSIONS: A careful dietary history, which includes documentation of fluid intake may identify children whose intake of high energy drinks may be excessive. The pathogenesis, symptoms, and response to treatment of these patients are consistent enough to be regarded as a distinct clinical entity: the 'squash drinking syndrome'.

Plasma lipid concentrations in children with cystic fibrosis: the value of a high-fat diet and pancreatic supplementation.

Impaired digestion of dietary fat is an almost universal feature of cystic fibrosis (CF) which results in low concentrations of essential fatty acids in plasma lipids. We have evaluated the effect of a high-lipid diet and pancreatic enzyme supplementation, using enteric-coated microsphere preparations, on plasma lipid concentrations in paediatric CF patients. Absorption of dietary lipid was comparable between control and CF subjects. This resulted in plasma cholesterol, triacylglycerol, total phosphatidylcholine and individual phosphatidylcholine molecular species concentrations in CF patients which were in the same range as those in controls. Normal values for these variables were also found in patients with clinically detectable liver disease. These results show that present dietary management of CF patients supports normal plasma lipid concentrations.

Vitamin therapy in cystic fibrosis--a review and rationale.

Vitamin supplements are routinely prescribed in cystic fibrosis, but published recommendations vary widely and there is little consistency in clinical practice. A review of the literature confirms that, while supplementation of the water-soluble vitamins (including B12 and folate) is unnecessary in uncomplicated cystic fibrosis, deficiency of the fat-soluble vitamins can lead to clinical problems. Supplements of these vitamins should be ensured for all patients with cystic fibrosis, while sparing them the unnecessary inconvenience of taking other vitamin supplements except where these are specifically indicated.

Factors influencing the presence of faecal lactobacilli in early infancy.

The faecal flora of 46 preterm infants and 52 born at full term was studied at 10 days of age; 46 born at full term and 37 preterm infants were also studied at 30 days. Viable counts of coliforms, lactobacilli, and bifidobacteria were made; gas liquid chromatography was used to identify the anaerobes. Lactobacilli, but not bifidobacteria, were found in high counts in the stools of most of the infants born at full term by 30 days of age. The mode of delivery, but not the method of feeding, had a significant influence on early colonisation. A selective deficiency of lactobacilli compared with coliform organisms was found in preterm infants. Previous treatment with antibiotics and being nursed in an incubator were also significantly associated with a lower rate of early colonisation with lactobacilli. Our findings indicate that lactobacilli may be an important part of the normal stool flora in early infancy, and that modern methods of neonatal care are associated with delayed or deficient colonisation.

Childhood handicap in Montserrat - abstract

Handicap in childhood is a worldwide phenomenon that is gaining an increasingly high priority as a health problem in developing countries. In Montserrat, prior to 1988, no attempt to define the prevalence, range or severity of handicap had been made. During February to April 1988, a programme of developmental screening in under-five-year-old children, with assessment of any identified problems, was introduced in Montserrat. As part of this programme, a register of handicapped children of all ages was compiled. Sixty children (2 per cent of approx. 3,000 children under 16 years old) with significant disability were identified. This was considerably more than local health workers expected. The main categories diagnosed were 12 with severe global disability, 21 with mental retardation, one with visual, hearing and speech problems only, 7 with physical disability and 3 with learning difficulties. Although all major handicapped children have probably been identified, these figures may still present an under-estimate; particularly of under-five-year-olds, who are not yet all screened, and school learning difficulties (surveys not yet completed). For realistic manpower and resource planning, it is essential that comprehensive handicap registers be compiled (AU)

Immunoreactive trypsin in Shwachman's syndrome.

We studied two infants with Shwachman's syndrome in whom the immunoreactive trypsin concentration was found to be abnormally low. Experience with several hundred assays for immunoreactive trypsin has not shown this low concentration. This finding is probably specific for pancreatic acinar deficiency at this age and strongly suggests Shwachman's syndrome.