Pathogenesis of congenital alkalosis with diarrhea

Bieberdorf, Frederick A.; Görden, Phillip; Fordtran, John S.

doi:10.1172/jci107002

Cited by 95 publications

(35 citation statements)

References 18 publications

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“…The possible heterozygosity of the controls does not seem to leave the nature of the colonic defect in doubt; if they were heterozygotes the difference between CCD and the truly normal state could only be larger. The present studies of colonic function in CCD revealed a pattern similar to that found in previous studies of the ileum (14). Na+ transport was intact, but net absorption of Na+ was dependent on luminal presence of HC08-, in contrast to the controls.…”

Section: Discussionsupporting

confidence: 89%

“…secreting Cl-and absorbing HCOs. Bieberdorf, Gorden, and Fordtran (14) observed absence of active transfer of C1-; passive movement occurred according to the concentration gradient. Pearson et al (7) reported that no Na+ or Cl-movement took place in the ileum.…”

Section: Introductionmentioning

confidence: 92%

“…In eight perfusions of the controls, the mean flow rate was 0.9 (range 0.2-1.9) ml/min and the mean concentrations of Nat 108. 3 (18,19 HCO3-exchange in CCD (7,12,14) is the cause of the increased effluent volume.…”

Section: Ileal Effluentmentioning

confidence: 99%

See 2 more Smart Citations

Colonic electrolyte transport in health and in congenital chloride diarrhea.

Holmberg¹,

Perheentupa²,

Launiala³

1975

J. Clin. Invest.

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Section: Discussionsupporting

confidence: 89%

Section: Introductionmentioning

confidence: 92%

See 1 more Smart Citation

Colonic electrolyte transport in health and in congenital chloride diarrhea.

Holmberg¹,

Perheentupa²,

Launiala³

1975

J. Clin. Invest.

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“…Both influx and efflux of Cl -are practically absent or greatly reduced. 18 This abnormality is due to an impaired active C1 -/HCO 3 -exchange mechanism in the distal ileum and colon. The loss of Na + and K + usually accompanies that of Cl -.…”

Section: Discussionmentioning

confidence: 99%

Congenital Chloride Diarrhea: A Single Center Experience with Ten Patients

Al-Abbad

Nazer

Sanjad

et al. 1995

Annals of Saudi Medicine

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“…the intestinal CI-transport. Perfusion studies have shown that Plasma renin activity was measured with radioimmunoassay the active CI-/HC0; exchange of the ileum and the colon is (12) from samples taken in the supine position between 8 and 9 defective (3,17,30,36). This results in osmotic diarrhea with A M after an 8-hr fast.…”

Section: Speculationmentioning

confidence: 99%

Electrolyte Economy and Its Hormonal Regulation in Congenital Chloride Diarrhea

Holmberg

1978

Pediatr Res

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Summary SUBJECTSThe economy of CI-, K+, and Mg++, extracellular volume (ECV) and plasma volume, and the role of hyperreninemia and hyperaldosteronism were explored in 22 patients with congenital chloride diarrhea. Stool volume was in significant correlation with its CI-, Na+ and K+ content, the correlation being significantly better with CI-content than with the Na+ content. Low fecal CI-concentrations were seen in chronic hypochloremic contraction, but acute episodes did not cause reduction of fecal CI-concentration from the basal level of 140-150 mmoll liter. The adequate condition (defied as normal serum electrolyte concentrations and blood pH, and presence of CI-in urine) was associated with high total exchangeable C1-and ECV. This excess CI-and ECV roughly equalled the high daily fecal amount of CI-and volume. Reduced ECV was accompanied bv high renin activities and hyperaldosteronism, but in theadequate condition these were normal. Hvperaldosteronism caused a decrease in urinary Na+-K+ ratio-and, after the age of 2-6 months, in the fecal Na+-K+ ratio. Total exchangeable K+ was normal in the adequate condition. No Mg++ depletion was present, although the patients lack Mg++ substitution. The adequate condition could be maintained with an oral supplement of NaCI, KC1 and water.Twenty-two patients, aged 0.5-14.5 years (18), were studied. Their early long term substitution was with a 0.15-0.3 M water solution of KC1 only, the dose (45-204 mmol/m2/24 hr) being adjusted to maintain normal serum electrolyte concentrations. Several episodes of acute dehydration had occurred lasting up to a week during the first years of life, but the subsequent clinical condition of the patients had been good. They passed a constantly Cl--free urine and tended to be slightly alkalotic.Five years ago the therapy was changed to a 0.15-0.3 M NaCl + KC1 solution with the molar ratio of the salts individually varying from 3: 1 to 1: 1. The dose was 60-216 mmol/m2 daily and was adjusted to maintain chloriduria in addition to normal serum electrolyte concentrations and blood pH. Such a state will be referred to as the adequate condition. The recordings of low serum electrolyte concentrations and alkalosis in this study were obtained from observations during acute exacerbations of the diarrhea, due to respiratory and gastrointestinal infections, and from observations in our early patients. METHODS SpeculationSerum electrolyte concentrations and blood pH were measured according to standard methods. Urinary and fecal electroHyperreninemi% h~~eraldosteronism, and h~~o k a l e m i c a k a -lyte excretion was always measured from 24-hr collections. losis in congenital chloride diarrhea (CCD) are secondary to F~~ measurement of total exchangeable (-1-(TECI-), 0.3 hypochloremic ECV contraction and can be completely cor-p~i / k g of 36C1, NaCl solution ( > 3 mCilg ~1 -) was injected rected with adequate substitution. Mg++ depletion does not intravenously with 5 % glucose. Urines and stools were collected play a Part in CCD. The to h~~eraldosteron-...

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Pathogenesis of congenital alkalosis with diarrhea

Cited by 95 publications

References 18 publications

Colonic electrolyte transport in health and in congenital chloride diarrhea.

Colonic electrolyte transport in health and in congenital chloride diarrhea.

Congenital Chloride Diarrhea: A Single Center Experience with Ten Patients

Electrolyte Economy and Its Hormonal Regulation in Congenital Chloride Diarrhea

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