Incomplete pancreatic deficiency in cystic fibrosis of the pancreas

Gibbs, Gordon E.; Bostick, Warren L.; Smith, Pearl M.

doi:10.1016/s0022-3476(50)80150-6

Cited by 30 publications

(7 citation statements)

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“…Twenty of these patients (mean age 10. (1) recognized that a small subset of patients with CF had sufficient pancreatic function to maintain normal digestion without the need for exogenous pancreatic enz yme supplements. We have coined the term "pancreatic sufficient " to describe CF patients with normal digestion, which serves to distinguish them from those with pancreatic insufficiency who have fat maldigestion due to pancreatic failure.…”

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Decline of Exocrine Pancreatic Function in Cystic Fibrosis Patients with Pancreatic Sufficiency

et al. 1992

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ABSTRACT. Patients with cystic fibrosis and pancreatic sufficiency were investigated for evidence of progressive pancreatic disease. From a cohort of 630 patients, 20 pancreatic-sufficient patients became pancreatic insufficient after an average duration of 5.6 y (range 0.6-20.6 y) from diagnosis. Among 54 patients documented to be pancreatic sufficient by direct pancreatic stimulation test, 47 remained pancreatic sufficient and seven developed pancreatic insufficiency. The patients who ultimately developed pancreatic insufficiency were younger and had greatly reduced outputs of enzyme, fluid, and electrolytes. Those who remained pancreatic sufficient showed enzyme secretion close to or within the non-cystic fibrosis control range. Twenty of these patients underwent a second pancreatic stimulation test after an average interval of 4 y (range 1.3-6.2 y). No significant alteration in enzyme, fluid, or electrolyte output was seen in the patients who remained pancreatic sufficient, but there was further reduction in enzyme and fluid output in the patients who developed pancreatic failure. In conclusion, the majority of pancreatic-sufficient patients with pancreatic enzyme secretion within the control range showed no deterioration of function over an extended time period. However, a small number of pancreatic-sufficient patients with reduced enzyme and fluid secretion are at risk of pancreatic failure. (Pediatr Res 32: 179-182, 1992) in CF neonates diagnosed by newborn screening reveal that 38% of CF infants are pancreatic sufficient at diagnosis (3), but 21% of these patients develop pan creatic insufficiency between 3 and 36 mo of age. This observation supports the previous suggestion that some pancreatic-sufficient patients will develop pancreatic insufficiency with advancing age. Also, in nonscreened populations , the variable age of diagnosis and modes of clinical presentation may be attributed to varying onset of signs and symptoms of pancreatic failure.There are few data concerning longitudinal changes of exocrine pancreatic function in patients with pancreatic sufficiency. Our current cross-sectional data suggest that pancreatic-sufficient patients are capable of secreting adequate amounts of pancreatic enzymes to ensure normal digestion, but it is important to emphasize that this subgroup of CF patients is characterized by a wide range of acina r capability, from mean values similar to control subjects to enzyme secretory rates as low as I to 2% of the mean normal rate (4). The wide variation of exocrine pancreatic function in pan creatic-sufficient patients together with the fact that these parti cular patients are prone to recurring attacks of acute pancreatitis (5) strongly suggest that ongoing pancreatic damage occurs . To evaluate this possibility, we have assessed pancreatic-sufficient patients for evidence of progressive pancreatic disease and have attempted to characterize those at risk of developing pancreatic failure. Abbreviations PATIENTS AND METHODS

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Decline of Exocrine Pancreatic Function in Cystic Fibrosis Patients with Pancreatic Sufficiency

et al. 1992

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“…The same occurred in the present series; a large number of glands had minimal lesions (110), a few noticeable lesions (23), all being symptomless, and a small number (11) with the full fibrocystic syndrome. Degrees of the fibrocystic syndrome do occur clinically (Gibbs, Bostick, and Smith, 1950). Farber (1944) considers that these acinal lesions themselves produce the grossly scarred and fibrotic gland.…”

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confidence: 99%

The Relationship of Cystic Lesions of the Acini of the Pancreas to Age, Cause of Death, and Fibrocystic Disease in Children

Emery¹

1951

Archives of Disease in Childhood

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Dilated plugged acini in the pancreas are one of the fundamental histological features in 'fibrocystic disease. ' Andersen (1938), however, in her original article on 'Fibrocystic Disease in the Pancreas in Relation to Celiac Syndrome' pointed out that cystic acini occur in a large number of children, and she was careful to confine her diagnosis of fibrocystic disease to cases where more than 90 % of the acini of the gland were affected. Following Andersen's work there has been an increased interest in the presence of cystic changes in the acini of the pancreas, and lesions have been described in a number of apparently quite different conditions. Baggenstoss (1947, 1948a and b) described a high incidence of plugged acini in chronic nephritis, hypertension, uraemia, ulcerative colitis, and intestinal obstruction; Mallory (1947) in severe infections and in all of 24 cases of typhus; found the lesion in malnutrition in South Africa; Wallace and Ashworth (1942) and Andrew (1944) in old age; and Hartz (1948) in patients dying after starvation.Relatively little interest appears to have been taken in the significance of these plugged acini in children not showing the full fibrocystic syndrome. An attempt has been made here to determine the incidence of plugged and cystic acini in the pancreas of children at necropsy, and to relate these lesions to the clinical disease and age of the children.Material and Methods Blocks were taken from the head and tail of the pancreas of children coming to necropsy at the Sheffield Children's Hospital during the years 1947-50. Sections were stained with haemotoxylin and eosin and also with Masson's trichrome. With the latter stain the colloids in dilated acini frequently take on a different colour from that of the surrounding cells, making the plugged acini much more easily identifiable than in a haemotoxylin-eosin preparation. All glands were excluded which showed gross post-mortem autolysis, or had been affected by extensive lesions such as tumour infiltration or haemorrhage. The number of glands remaining and analysed from 400 necropsies was 310.The clinical features recorded were the age of the child, duration of illness, cause of death, presence or absence of staphylococcal infection, and the major disease causing death.The glands were divided into four groups: (1) showing fibrocystic disease in which the majority of acini were affected and corresponding to the lesions described by Andersen (1938); (2) in which plugged acini were readily identified on the sections and were a prominent feature of the pancreas, although affecting less than 90% of the acini; (3) cases in which plugged acini were found in small numbers affecting less than 1 % of the acini; (4) no lesions.For this survey a pancreas was reported as negative if plugged acini were not found in at least two complete cross sections taken from different parts of the gland. ResultsOf the 310 glands examined, fully developed fibrocystic disease was present in 11 (3. 50%) cases; large numbers of plugged acini were found in...

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“…There is heterogeneity in mode of presentation, organ involvement type of complications, disease severity and life expectancy (Boat et al 1989). About 15% of patients with CF have sufficient pancreatic function to prevent steatorrhea (Gibbs et al 1950, Corey et al 1989.…”

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Molecular and clinical analyses of cystic fibrosis in the South of Spain

et al. 1994

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We report molecular and clinical analyses in 71 unrelated patients with cystic fibrosis (CF) from Andalusia (South of Spain). Direct mutation analysis of six mutations of the CFTR gene (ΔF508, G542X, R1162X, N1303K, W1182X and 1949del84) was performed. The proportion of CF chromosomes with the above‐mentioned mutations was 58.5%. Haplotype analysis was performed with the marker/enzyme pairs XV2C/TaqI and KM19/PstI. A particular haplotype has been found associated with each of the studied mutations, while the pooled data for the unknown mutations are not associated with any particular haplotype. This lack of association indicates that there will not be a single predominant mutation amongst the other CF chromosomes. To assess the relationship between genotype and phenotype in these patients, we correlated the pancreatic status and the occurrence of chronic Pseudomona aeruginosa infection with the observed genotype. Pancreatic insufficiency was present in all patients in whom the analyzed mutations were found to be homozygous or compound heterozygous. We also found a higher rate of Pseudomonas colonization in the group of patients in whom the genotype was homozygous or compound heterozygous for the analysed mutations when compared with the group of patients with a different genotype, but the difference was not statistically significant.

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Incomplete pancreatic deficiency in cystic fibrosis of the pancreas

Cited by 30 publications

References 6 publications

Decline of Exocrine Pancreatic Function in Cystic Fibrosis Patients with Pancreatic Sufficiency

Decline of Exocrine Pancreatic Function in Cystic Fibrosis Patients with Pancreatic Sufficiency

The Relationship of Cystic Lesions of the Acini of the Pancreas to Age, Cause of Death, and Fibrocystic Disease in Children

Molecular and clinical analyses of cystic fibrosis in the South of Spain

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