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.

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.

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.

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.

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.

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.

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.

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.

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.

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.

Ectopic ACTH production by a thymic carcinoid tumour.

An ACTH-producing thymic carcinoid tumour was diagnosed in a 10-year-old girl, 8 years after bilateral adrenalectomy for Cushing's syndrome. The peptides produced by the tumour were characterised thoroughly. High circulating levels of beta-endorphin and other peptides may have contributed to mood and behaviour disturbances.