Inhibition of Net Sodium Transport in Single Sweat Glands by Sweat of Patients with Cystic Fibrosis of the Pancreas

Kaiser, D.; Drack, E; Rossi, E

doi:10.1203/00006450-197104000-00004

Cited by 33 publications

(12 citation statements)

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“…Kaiser er a/. (10) have demonstrated a factor in the sweat of children with C F P which causes inhibition of net sodium transport in the sweat gland. It thus seems probable that there is a factor present in several body fluids of children with C F P which inhibits active Na transport.…”

Section: Methodsmentioning

confidence: 99%

“…The presumed decrease in the rate of sodium reabsorption in the salivary ducts of children with C F P represents only one aspect of a widespread disturbance in active membrane cation transport in children with this disease (9,10,17). We have reported a factor in the plasma of children with C F P which inhibits a ouabain-sensitive, sodium-potassium-activated component of ATPase from normal erythrocyte membranes (4).…”

Section: Speculationmentioning

confidence: 99%

“…Mangos et a[. (14) have shown that retrograde perfusion of the rat parotid duct with C F P saliva resulted in a decrease in the rate of sodium reabsorption in the parotid duct, but were unable to demonstrate an effect of C F P saliva on (Na, K)-ATPase fro'm various tissues.The presumed decrease in the rate of sodium reabsorption in the salivary ducts of children with C F P represents only one aspect of a widespread disturbance in active membrane cation transport in children with this disease (9,10,17). We have reported a factor in the plasma of children with C F P which inhibits a ouabain-sensitive, sodium-potassium-activated component of ATPase from normal erythrocyte membranes (4).…”

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confidence: 99%

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Inhibition of Ouabain-sensitive ATPase by the Saliva of Patients with Cystic Fibrosis of the Pancreas

Cole

Sella

1975

Pediatr Res

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Extractobtained by asking each child to rinse his mouth with water, and A study has been made of the effect of saliva from children with cystic fibrosis of the pancreas (CFPI on various components of the ATP hydrolyzing enzyme system. The ouabain-sensitive ATPase activity of erythrocyte membranes prepared from intact erythrocytes preincubated with CFP saliva was 35 4 nmol P,/mg dry wt membrane suspension/hr, compared with 48 i 7 nmol P,/mg dry wt membrane suspension/hr when the erythrocytes were preincubated with control saliva. A calcium-activated component of ATPase was decreased from 202 30 nmol P,/mg dry wt membrane suspension/hr in erythrocytes preincubated with control saliva, to 151 17 nmol P,/mg dry wt membrane suspension/hr when the incubation was carried out with CFP saliva.In a second series of experiments, ultrafiltered saliva was added directly to an ATPase assay. The saliva from children with CFP brought about a mean decrease in ouabain-sensitive ATPase of 16% compared with control saliva. SpeculationInhibition of active cation transport at various sites in the body is one of the hallmarks of CFP. Electrolyte abnormalities in the salita of patients with CFP may result from the action of an ATPase inhibitory factor in the saliva which inhibits various components of ATPase in the ducts of the salivary glands. Such a factor could be similar or identical with the ciliotoxic factor identified by other investigators in the serum and saliva of patients with CFP.Children with C F P have been demonstrated to have a higher than normal concentration of sodium in mixed saliva (19). The reasons for this elevation of salivary sodium have not been defined. Mangos et a[. (14) have shown that retrograde perfusion of the rat parotid duct with C F P saliva resulted in a decrease in the rate of sodium reabsorption in the parotid duct, but were unable to demonstrate an effect of C F P saliva on (Na, K)-ATPase fro'm various tissues.The presumed decrease in the rate of sodium reabsorption in the salivary ducts of children with C F P represents only one aspect of a widespread disturbance in active membrane cation transport in children with this disease (9,10,17). We have reported a factor in the plasma of children with C F P which inhibits a ouabain-sensitive, sodium-potassium-activated component of ATPase from normal erythrocyte membranes (4). The action of a similar factor in the saliva of children with C F P could have been obscured in previous studies by the relatively high concentration of phosphorous normally present in the saliva. In this paper, we report the results of two series of experiments designed to study the effect of C F P saliva on an ATPase assay system. METHODSThe patients with C F P used in this study were attending the outpatient clinic of the Montreal Children's Hospital. They ranged in age from 5 to 15 years, were about equally divided as to sex, and had Shwachman scores ranging from 33 91. Mixed saliva was then giving him a biece of laboratory parafilm to chew. N o effort was made to record or con...

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Section: Methodsmentioning

confidence: 99%

Section: Speculationmentioning

confidence: 99%

mentioning

confidence: 99%

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Inhibition of Ouabain-sensitive ATPase by the Saliva of Patients with Cystic Fibrosis of the Pancreas

Cole

Sella

1975

Pediatr Res

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“…Studies by Kaiser e t al. (5) also show that sweat from CF patients causes inhibition of sodium reabsorption when it is perfused into normal human sweat gland ducts. A specific sodium reabsorption inhibitory factor (5,(10)(11)(12) may be the cause of the elevated sodium concentrations found in these secretions; however, such a factor has not been identified.…”

Section: Speculationmentioning

confidence: 99%

Standardized Assay for the Sodium Reabsorption Inhibitory Effect and Studies of Its Salivary Gland Distribution in Patients with Cystic Fibrosis

et al. 1974

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ExtractThe retrograde perfusion assay of Mangos for the sodium reabsorption inhibitory effect in sweat and mixed saliva from patients with cystic fibrosis (CF) was modified by (I) using the second parotid gland of the rat as a control, (2) carefully controlling the quantity of saliva perfused into the rat parotid gland so that the ratio of milliliters perfused to gram dry gland weight fell between 1.8 and 2.8, and (3) reporting the inhibitory effect as percentage of inhibition of the rate of sodium reabsorption (RNa) in the perfused gland calculated from the difference between the RNals in the two glands. Using this modified assay we were able to confirm that significant differences exist in the percentage of inhibition of RNa caused by CF and normal mixed saliva (47.0 f 24.8 and 18.0 -1. 8.3, mean f SD). In addition, similar significant differences in the percentage ' of inhibition of R~~ by submandibular gland secretions (39.7 f 13.5 and 16.0 f 11.8). sublinrrual dand secretions (39.5 f 6.0 and 19.7 + 14.3), and'submu~osal gland secretions (34.0 f 11.7 and 21.7 f 12.7) between CF and normal subjects were observed. However, parotid gland secretions from CF subiects showed no increased inhibitory effect (12.5 + 7.9 and" 12.5 f 6.6). These findings demonstrate, that the sodium reabsorption inhibitory effect is produced by the secretions from some but not all salivary glands of patients with CF. SpeculationThese results indicate that the substance or substances responsible for the sodium reabsorption inhibitory effect of CF secretions is produced by cells present in submandibular, sublingual, and submucosal glands of patients with CF. Because mucus-producing cells are abundant in submandibular, sublingual, and submucosal glands, but are sparse in parotid glands (1) we suggest, as have Johansen e t al. (4), that the inhibitory effect is in some way related to the mucous secretory product of the cystic fibrosis glands.Cystic fibrosis is an inherited disease involving the exocrine glands. The most consistent finding in this disease is that of elevated concentrations of chloride and sodium in sweat (2) and secretions of the minor salivary glands (14). The pathophysiology of this alteration has not been clearly defined. Mangos e t al. (12) and Mangos and McSherry (10) have shown that retrograde perfused CF sweat and saliva inhibit sodium reabsorption in the ducts of the rat parotid glands. Studies by Kaiser e t al. (5) also show that sweat from CF patients causes inhibition of sodium reabsorption when it is perfused into normal human sweat gland ducts. A specific sodium reabsorption inhibitory factor (5, 10-12) may be the cause of the elevated sodium concentrations found in these secretions; however, such a factor has not been identified.The sodium reabsorption inhibitory effect in rat parotid gland ducts has not been reproduced by other laboratories. Using a modification of the Mangos technique we now have been able to demonstrate a sodium reabsorption inhibitory effect in mixed saliva of patients with CF. Further wor...

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“…There is, however, increasing evidence that abnormalities of water and electrolyte transport may underlie the dysfunction of exocrine glands in this disorder, and CF "factor(s)" have been implicated as being primary in pathogenesis (10)(11)(12). Such factor(s) have been reported in sweat, saliva, and serum (2,5,7,15,16,18,22) using a variety of in vitro techniques.…”

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Short Communication: Cystic Fibrosis “Factor(s)” Present Also in Sera of Shwachman's Pancreatic Insufficiency

et al. 1981

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Summarvprotracted diarrhoea of undetermined cause) aged 6 months to 7Using an in vivo closed-loop technique in rat jejunum, we have confirmed previous in vitro studies that cystic fibrosis (CF) serum inhibits water, sodium, and glucose absorption and transmural potential difference. CF heterozygote serum or sera from children with diarrhoeal disorders not associated with exocrine pancreatic insufficiency had no effect on transport. Sera from patients with Shwachman's syndrome and exocrine pancreatic insufficiency had identical effects to CF serum. These results challenge the specificity of the CF "factor." Speculation PANCREATIC-RELATED FACTORSThe pancreas may modulate small intestinal absorption of water, electrolytes, and glucose via an humoral pathway. In exocrine pancreatic insufficiency, there could. be a deficiency of a normal serum factor which stimulates absorption or, alternatively, a factor which inhibits absorption could be present.

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Inhibition of Net Sodium Transport in Single Sweat Glands by Sweat of Patients with Cystic Fibrosis of the Pancreas

Abstract: cystic fibrosis of the sodium pancreas sodium transport humoral transducibility sweat potassium

Cited by 33 publications

References 3 publications

Inhibition of Ouabain-sensitive ATPase by the Saliva of Patients with Cystic Fibrosis of the Pancreas

Inhibition of Ouabain-sensitive ATPase by the Saliva of Patients with Cystic Fibrosis of the Pancreas

Standardized Assay for the Sodium Reabsorption Inhibitory Effect and Studies of Its Salivary Gland Distribution in Patients with Cystic Fibrosis

Short Communication: Cystic Fibrosis “Factor(s)” Present Also in Sera of Shwachman's Pancreatic Insufficiency

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