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At least 649 records · Page 36Linked to original sources
PubMed1980

Chloramphenicol optic neuropathy.

Three patients with cystic fibrosis of the pancreas initially were seen because of optic neuropathy caused by a toxic reaction to chloramphenicol. The ocular symptoms were characterized by loss of visual acuity, central scotomas, red-green dyschromatopsia, and fundus changes. An increase in the latency of the occipital evoked potentials paralleled the visual impairment. A transient photopic decline in the red-light-elicited electroretinogram and the occurrence of Uhthoff's sign in the acute stage of the disease were observed. All visual impairment recovered partially or totally after the withdrawal of therapy with the drug.

Adolescent↗
PubMed1977

Renin-like (angiotensinogenase) activity in sweat of patients with cystic fibrosis and controls.

Using bioassay method (rat blood pressure technique) as well as the radioimmunoassay, renin-like activity (RLA) was measured in eccrine sweat of patients with cystic fibrosis of the pancreas (CF) and of controls. Sweat-formation was induced by pilocarpine-iontophoresis or by local injection of carbamylcholine (Doryl). RLA-values between O (not measurable) and 460 ng/ml.h were measured. With increasing sweat flow-rate a tendency to lower RLA-values was detected. No significant difference was observed between CF and controls. From the observation that RLA of sweat is up to 30 times higher than that of plasma, it is concluded that RLA is probably released not from plasma but from the sweat glands themselves, where it is stored or synthesized.

Adolescent↗
PubMed1974

Neutrophil granulocyte function in patients with pulmonary infection.

Neutrophil granulocyte function was evaluated in patients with pulmonary disease to determine if a change in function occurred during active pulmonary infections. Twenty-five patients with cystic fibrosis of the pancreas were studied. Ten patients were asymptomatic and 15 patients had symptoms and signs of active pulmonary infection. Leukotaxis, random migration, and nitroblue tetrazolium dye reduction by neutrophils were compared in the asymptomatic and symptomatic patients. The leukotactic activity of circulating neutrophils was markedly increased in all of the patients with active pulmonary infection and spontaneous nitroblue tetrazolium dye reduction by neutrophils was increased in 13 of 15 patients. Bacterial infection confined to the respiratory tract is capable of stimulating circulating neutrophils making them more responsive to chemotactic factors as well as increasing nitroblue tetrazolium reduction.

Case-Control Studies↗
PubMed1986

Fecal chymotrypsin levels in children with pancreatic insufficiency.

The fecal chymotrypsin (FC) levels in samples collected over 24 h were determined by a new commercial colorimetric method from Boehringer Mannheim in 82 children suffering from various pancreatic disorders. The patients were divided into 4 groups, in accordance with the following etiologies: cystic fibrosis of the pancreas (CFP), chronic severe hepatic disorders (CSH), primary malabsorption syndrome (PMS) and malnutrition due to nondigestive causes (M). The control group comprised 48 children of similar ages. The 24th FC levels as U/g (mean +/- SD) were: 34 +/- 6 in the control group, 2 +/- 2 in the CFP group, 15 +/- 6 in the M group, 19 +/- 9 in the CSH group and 43 +/- 13 in the PMS group. The differences between the CFP patients and all the other groups were statistically significant. These results indicate that the FC levels may be suitable as a diagnostic indication of CFP and capable of differentiating between this disorder and other causes of pancreatic insufficiency.

Adolescent↗
PubMed1975

Dietary supplement and nutrition in children with cystic fibrosis.

Assessment of nutritional status of patients with cystic fibrosis of the pancreas (CFP) showed that poor growth was associated with low concentrations of albumin, urea nitrogen, and cholesterol in serum and with elevated white blood cell (WBC) counts. Patients with CFP maintained weight approximately 1 standard deviation below the mean until 8 years, after which there was a progressive decline in growth rate compared to normal. A complete dietary supplement consisting of a beef serum hydrolysate, a glucose polymer, and medium-chain triglycerides was given to 15 patients for a year. Patients who received the diet showed significant gains in weight, significant increase in clinical score, significant increase in serum albumin level, and a significant drop in the WBC count compared to control patients who did not receive the supplement.

Adolescent↗
PubMed1994

[Cystic fibrosis revealed by dehydration with hypochloronatremic alkalosis in 3 infants and a neonate].

BACKGROUND--Acute dehydration with hypochloronatremic metabolic alkalosis is a classical complication of cystic fibrosis of the pancreas. Its progressive development as a revealing manifestation of the disease is rare as is its appearance in newborns. Case n. 1.--A 13 month-old girl was admitted because of status epilepticus. She was severely dehydrated (20% weight loss) but had normal diuresis. Investigations showed metabolic alkalosis, hypochloronatremia and hypokalemia. All the manifestations disappeared within 5 days with treatment but three sweat tests were abnormal. Case n. 2.--A 7 month-old girl was admitted because she suffered from progressive loss of weight (10%); she was dehydrated and had metabolic alkalosis plus hypochloronatremia, but her diuresis was normal. Two sweat tests were abnormal. Case n. 3.--A 4.5 month-old boy was admitted because he suffered from severe (12%) weight loss. His diuresis was normal despite dehydration; metabolic alkalosis and hypochloronatremia were found. Two subsequent sweat tests were abnormal. Case n. 4.--A 3 day-old girl was admitted suffering from meconium ileus. Two initial mechanism analysis for protein were abnormal. She had a cardiorespiratory arrest on the 13th day of life, when she had lost 14% of her birth weight; investigations showed metabolic alkalosis, hyponatremia (83 mEq/l), hypochloremia (45 mEq/l); kalemia was 5.9 mEq/l. Peritoneal dialysis was needed to correct hydroelectrolytic changes. A sweat test performed on the 26th day of life was also abnormal. Investigations performed during the periods of dehydration, and repeated later, showed transient functional kidney failure. None of the four patients had any respiratory or gastrointestinal clinical manifestations of cystic fibrosis. CONCLUSION--Metabolic alkalosis with hypochloronatremia plus progressive, severe dehydration in infants whose diuresis is paradoxically normal must be followed by examination for cystic fibrosis.

Alkalosis↗
PubMedDate not supplied

[The meconium ileus equivalent in mucoviscidosis].

Meconium ileus equivalent (MIE) complicating cystic fibrosis of the pancreas (CF) increases in frequently with increasing age of patients. In the present paper the course of 11 children and adolescents with MIE diagnosed and treated at the University Dept. of Paediatrics in Zürich during the last 15 years, i.e. 9% of 120 CF patients, is analyzed. 9 were successfully managed by medical treatment alone, 1 three months old infant was treated surgically with no later relapse and a 26 years old patient with a chronic deleterious course leading to opiate dependency could be relieved only by ileostomy. 5 patients experienced only 1 episode of MIE, 2- two to three and in 4 a chronic refractory course with multiple episodes was probably due to an inadequate medical management. The evaluation of the events in our patients and of the available data from the literature allows the following conclusions: MIE is a preventable condition in CF patients; a rigorous medical treatment of exocrine pancreatic insufficiency supplemented with mucolytic agents orally can usually prevent MIE and relieve an established MIE; surgical treatments is indicated only in desperate situations.

Adolescent↗
PubMed1988

[Indications of enteral nutrition in pancreatic disorders].

In the treatment of disorders of the pancreas, artificial nutrition must satisfy nutritional requirements while avoiding stimulation of exocrine pancreatic juice observed during oral feeding. Although total parenteral nutrition (PN) induces pancreatic hyposecretion or weak pancreatic stimulation, enteral nutrition (EN) whether elementary or semi-elementary type stimulates pancreatic secretion and the release of CCK, with weaker stimulation in case of intrajejunal feeding. In acute pancreatitis, semi-elemental EN by jejunal feeding has successfully been used in the treatment of moderately serious cases, once the acute phase of the disease has been passed. Although PN remains the best indication for the treatment of pancreatic fistula, several studies have reported the closure of pancreatic fistulas during elementary enteral feeding administered by jejunal route. In the treatment of chronic pancreatitis, EN especially provides nutritional support for very undernourished patients, most often in the preoperative context. Finally, in children suffering from cystic fibrosis of the pancreas, prolonged sessions of EN provide marked improvement in the nutritional and respiratory status of these patients.

Animals↗
PubMed1979

Evidence against a specific effect of serum from patients with cystic fibrosis on sodium-dependent glucose transport in the rat jejunum.

Sera from patients with cystic fibrosis of the pancreas (CF) and normal human sera were assayed for the ability to inhibit sodium-dependent glucose transport in rat brush-border membrane vesicles. Fresh CF and age- and sex-matched control sera were both inhibitory when compared to physiologic saline. The inhibition by CF serum was 44 +/- 13% (mean +/- SD) at a final serum concentration of 6.7%, 67 +/- 34% at 10% serum, and 68 +/- 28% at 20% serum. The ratio of the inhibition of CF sera compared to that of control sera was 1.00, 0.78, and 0.93 at 6.7, 10, and 20% serum concentrations, respectively. Although a slightly greater inhibition by CF serum was observed at a concentration of 10%, this is probably not significant because no difference could be detected at a concentration of 20% serum. Glucose transport in the presence of serum was sensitive to phlorizin indicating that the residual glucose transport was proceeding by the sodium-dependent glucose transport system. These findings suggest that CF serum does not specifically inhibit the sodium-dependent glucose transport system. The intravesicular space accessible to glucose was reduced in the presence of CF or control serum. Fresh CF serum was 1.4 times more effective than fresh control serum (P less than 0.01). The presence of substantial vesicle-shrinking activity in control serum indicates that this activity cannot be considered specific for CF.

Animals↗
PubMed1988

[Mucoviscidosis: a genetic disease of adulthood].

Mucoviscidosis (cystic fibrosis of the pancreas) is the most frequent lethal genetic disorder in the white race. It is an autosomal recessive transmission. In spite of its recent localisation on the 7th chromosome, the fundamental mechanism responsible for the symptoms remains unknown and it is still a syndrome more than a disease. It presents as a generalised seromucous exocrinopathy. The serous glands are functionally abnormal and have secretions too rich in chloride which allows for the sweat test (the only test of diagnostic value). The accumulation of abnormal mucous secretions is responsible for the clinical manifestations: intestinal troubles, bronchial and pulmonary disease, progressive pancreatic insufficiency and biliary cirrhosis. Mucoviscidosis is not longer a disease exclusively of children. The treatment which is solely symptomatic has profoundly changed the prognosis. Half of the children afflicted become adults. This fact and the better condition of diagnosis explain the appearance of a new symptomatology sometimes with a late presentation. On the basis of the genetic and prognostic implications one should consider the diagnosis at any age even if the general state is well conserved. The diagnosis rests on the association of suggestive symptoms and a disturbed sweat test. In the adult the presenting signs are always respiratory, more rarely digestive, and sometimes sterility. These associations are very suggestive. The current evolution requires a specific clinical management of new adult cases who, even if not in a very good clinical state, maintains a vital hope for several years with symptomatic treatment (and the hope perhaps that the discovery of the fundamental defect will lead to more specific therapy).

Adult↗
PubMed1956

Coeliac disease.

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Celiac Disease↗
PubMed1987

The relationship of plasma aldosterone-binding globulin to blood pressure regulation in young adults with cystic fibrosis.

Findings of increased secretion rate and decreased metabolic clearance rate (MCR) of aldosterone in patients with cystic fibrosis of the pancreas (CF) and our own evidence on the association of increased aldosterone-binding globulin (ABG)-binding and decreased MCR in essential hypertension (EH) inspired us to investigate the plasma aldosterone, with the inclusion of protein-binding variables, in CF patients. (1) The percentage of plasma aldosterone specifically bound to ABG was measured in 55 young adults with CF in addition to total plasma aldosterone, total plasma corticosteroids and for comparison of corticosteroid-binding globulin (CBG)-binding capacity. (2) The percentage of ABG-bound plasma aldosterone was found to vary with the seasonal change in temperature and the hepatic function of CF patients. Many of the CF patients, particularly during spring, summer and fall, had elevated plasma ABG-bound aldosterone which would be expected to result in low MCR. This binding was less elevated during cooler weather, suggesting that ABG-bound aldosterone is participating in the adaptation to warmer weather by probably increasing extrarenal sodium retention, thereby preventing a fall of blood pressure (BP) to pathologically low levels. A significant correlation was consequently found between the ABG capacity and the ambient temperature. (3) CF patients with low liver function had significantly lower protein binding of aldosterone and only slightly lower CGB capacity, presumably due to disturbed protein synthesis by the liver. (4) In some patients, elevated total plasma aldosterone and total corticosteroids were found, probably as a result of an adaptation to excessive sweat losses of sodium and the consequent contraction of intravascular volume. (5) Our findings also demonstrated a positive correlation between plasma ABG-bound aldosterone and both systolic and diastolic BP.

Adrenal Cortex Hormones↗