Apparent Absence of Cystic Fibrosis Sweat Factor on Ion-selective and Transport Properties of the Perfused Human Sweat Duct

Bijman, J.; Quinton, Paul M.

doi:10.1203/00006450-198412000-00014

Cited by 16 publications

(3 citation statements)

References 11 publications

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“…The present results may afford more insight into the mechanisms of the transport of HC0,-, lactate, and protons in the sweat gland. It is perhaps surprising, in view of the fact that CF sweat differs dramatically from normal in its concentration of Na, C1, and K as well as in the transepithelial PD associated with its production (1,3,19), that no significant differences were found in pH or in lactate and bicarbonate concentrations (Table 1). his finding, at once, suggests that the mechanism for handling these species is unaffected, or minimally affected, in CFand raises the question of what kind of mechanism might be unaffected in the face of such overt alterations in other electrolyte transport functions.…”

Section: Discussionmentioning

confidence: 92%

Lactate and Bicarbonate Uptake in the Sweat Duct of Cystic Fibrosis and Normal Subjects

Bijman

Quinton

1987

Pediatr Res

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ABSTRACT. The sweat of single sweat glands of healthy MATERIALS AND METHODS individuals and cystic fibrosis patients was analyzed for differences in bicarbonate, lactate, and pH. These values were monitored as a function of sweat rate simultaneouslyThe method of sweat collection from single glands in viva has with 1) the electrical potential difference at the duct orifice been described in detail (I). In this study the sweat from a single (with respect to the interstitial fluid) and 2) the concentra-sweat gland on the volar surface of the forearm of nine CF tions of sodium, potassium, and chloride in surface sweat. patients and nine control subjects was collected in serial samples Sweat in both groups contained about equal concentrations over a period of 40 min. All CF patients had a confirmed of lactate and bicarbonate at similar sweat rates. Similarly, diagnosis from an established CF center. No two patients were the pH of sweat secreted at similar rates in the two on the same therapeutic regimen. All patients received various populations was not significantly different. Acidification of supplemental vitamins. All but one patient received pancreatic sweat increased with decreasing sweat rate. In both popu-enzyme supplements. All but one patient received various antilations, lactate and bicarbonate may be absorbed passively biotic therapy, but no two patients were treated with the same in the distal sweat duct in their nonionic form and, thus, regimen of antibiotics. Two patients did not use bronchodilator not influenced by the increased electrical potential differ-agents. All subjects were adolescents or older. The mean age of ence in the cystic fibrosis sweat duct. The uptake of these CF patients was 26.6 & 2.7 (SE) yr and of normal subjects was ions may involve active proton secretion by sweat duct 25.2 & 1.9 yr. Only one subject, a CF patient, was under 18 yr cells. (Pediatr Res 21: 79-82, 1987) of age. Because of the difficulty in performing a large number of HC03-and lactate microassays, only one sweat gland was seAbbreviations lected from a collection of about seven glands from each subject. The selected gland was chosen on the basis of its showing a wide CF, cystic fibrosis range in sweat rates. That is, since we were interested largely in PD, electrical potential difference the concentration change as a function of rate, we selected glands which gave the greatest difference in rates and which secreted throughout the collection period (30-40 min). The analysis of Na, K, and C1 in the sweat and the PD measurement were CF is a genetically inherited disorder predominantly affecting performed as described previously (I). Microvolumetric methods Caucasians. Pancreatic deficiency, pulmonary disease, and ele-were used measure (5) and lactate (6). vated sweat sodium and chloride levels are the foremost maniThe pH the sweat was measured by microfluorescence festations that characterize the disease. Present evidence suggest the ~H-sensitive fluorescent dye Dextran-FITC (Sigma; 10 mg/ that the elevated sodium...

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Section: Discussionmentioning

confidence: 92%

Lactate and Bicarbonate Uptake in the Sweat Duct of Cystic Fibrosis and Normal Subjects

Bijman

Quinton

1987

Pediatr Res

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“…The demonstration that the defect persisted and was clearly expressed in a completely in vitro system with all systemic fluids replaced by defined media provided a compelling lesson that the defect in transport was inherent in the affected cells and not induced by external "factors," as they were then called (112,121). Additionally, companion studies with isolated normal ducts perfused with secretions from CF patients gave convincing lessons that such factors did not cause the electrolyte transport defect in CF tissues (18).…”

Section: Reviewsmentioning

confidence: 99%

Cystic Fibrosis: Lessons from the Sweat Gland

2007

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Lessons from the sweat gland on cystic fibrosis (CF) began long before modern medicine became a science. In European folklore, the curse that "a child that taste salty when kissed will soon die" (Alonso y de los Ruyzes de Fonteca J. Diez Previlegios para Mugeres Prenadas. Henares, Spain, 1606) has been taken by many as a direct reference to cystic fibrosis [Busch R. Acta Univ Carol Med (Praha) 36: 13-15, 1990]. The high salt concentration in sweat from patients with CF is now accepted as almost pathognomonic with this fatal genetic disease, but the earliest descriptions of cystic fibrosis as a disease entity did not mention sweat or sweat glands (Andersen DH. Am J Dis Child 56: 344-399, 1938; Andersen DH, Hodges RG. Am J Dis Child 72: 62-80, 1946). Nonetheless, defective sweating soon became an inseparable, and major, component of the constellation of symptoms that diagnose "cystic fibrosis" (Davis PB. Am J Respir Crit Care Med 173: 475-482, 2006). The sweat gland has played a foremost role in diagnosing, defining pathophysiology, debunking misconceptions, and increasing our understanding of the effects of the disease on organs, tissues, cells, and molecules. The sweat gland has taught us much.

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“…The fl-adrenergic and CI-permeability defect was expressed in epithelial cells in the absence of cystic fibrosis serum or sweat (McPherson et al, 1985(McPherson et al, , 1986aSato and Sato. 1984;Bijman and Quinton, 1984;Welsh and Liedtke, 1986;Frizzeli et al, 1986;Cotton et al, 1987) and in cells maintained in culture for a few weeks (Yankaskas et al, 1985;Pedersen et al, 1986), suggesting an intrinsic cellular abnormality, rather than an abnormality resulting from influence of an abnormal circulating serum factor (McPherson et al, 1983;Dorin et al, 1987). The finding that the transepithelial potential difference of transplanted trachea was normal in a cystic fibrosis patient one year after heart-lung transplant (Alton et aI., 1987c) also supports this view.…”

Section: Fluid Secretion and Electrolyte Transport In Sweat And Respimentioning

confidence: 93%

Recent advances in cystic fibrosis

McPherson

1988

J of Inher Metab Disea

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Cystic fibrosis, one of the most common lethal inherited disorders in N. European and N. American populations, is characterized by the production of abnormally viscous mucous secretions in the lungs and digestive tract. The pathophysiological basis of the disease is unknown. However, during the last few years, rapid advances in molecular genetics and biochemical and physiological studies on cystic fibrosis epithelial cells have led to optimism that the cystic fibrosis defect will soon be identified. Current evidence suggests that the basic disturbance lies in altered regulation of protein secretion and electrolyte transport leading to an imbalance in composition of epithelial secretions in cystic fibrosis patients. Increasing knowledge of the mechanisms regulating production and secretion of mucins and movement of electrolytes across the cell membrane should lead to development of pharmacological manipulation(s) to correct the cellular abnormality. Ultimately, it is hoped that this will lead to the development of a rational treatment for cystic fibrosis patients.

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Apparent Absence of Cystic Fibrosis Sweat Factor on Ion-selective and Transport Properties of the Perfused Human Sweat Duct

Cited by 16 publications

References 11 publications

Lactate and Bicarbonate Uptake in the Sweat Duct of Cystic Fibrosis and Normal Subjects

Lactate and Bicarbonate Uptake in the Sweat Duct of Cystic Fibrosis and Normal Subjects

Cystic Fibrosis: Lessons from the Sweat Gland

Recent advances in cystic fibrosis

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