Erythrocyte transmembrane Na and K fluxes in pseudohypoaldosteronism

Cugini, P; Natoli, G.; Gerlini, G; Palma, L. Di; Rota, Rossella; D’Onofrio, Mara; Verna, Roberto

doi:10.1016/0885-4505(92)90071-6

Cited by 4 publications

(6 citation statements)

References 17 publications

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“…The increase in red blood cell enzyme values correlated with gradual clinical improvement, reduction in salt intake, and normalisation of plasma renin activity. Furthermore, Cugini et al 33 found low Na + K + ATPase activity and reduced Na + K + transmembrane flux in the red blood cells of a child with PHA. It is therefore tempting to assume that the increase in red blood cell Na + K + ATPase activity in term babies occurs in the renal tubules as well.…”

Section: Discussionmentioning

confidence: 99%

Sodium potassium adenosine triphosphatase activity in preterm and term infants and its possible role in sodium homeostasis during maturation

Bistritzer

Berkovitch

Rappoport

et al. 1999

Archives of Disease in Childhood - Fetal and Neonatal Edition

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Section: Discussionmentioning

confidence: 99%

Sodium potassium adenosine triphosphatase activity in preterm and term infants and its possible role in sodium homeostasis during maturation

Bistritzer

Berkovitch

Rappoport

et al. 1999

Archives of Disease in Childhood - Fetal and Neonatal Edition

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“…Previously, reduced red blood cell (RBC) Na + ,K + ‐ATPase activity was found in the isolated renal form of PHA (Cugini et al ., 1992; Bistritzer et al ., 1994).…”

mentioning

confidence: 99%

Erythrocyte Na⁺,K⁺‐ATPase and nasal potential in pseudohypoaldosteronism*

Bistritzer

Kerem

Berkovitch

et al. 2002

Clinical Endocrinology

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“…No immunologic methods for the determination of the enzyme were performed. Therefore, the reduced activity found by us and by others (16,22) may not necessarily reflect a true reduction in the number of enzyme units. A circulating endogenous inhibitor reducing its activity could also be considered, but were it so, we have to assume its persistence into adulthood.…”

Section: Discussionmentioning

confidence: 50%

“…The very low Na+,K+-ATPase activity observed by us and by others (16,22) may either result from a relative aldosterone unresponsiveness causing a reduction in transmembraneal electrolyte fluxes or reflect a primary defect in the activity ofthe enzyme. The normal Na+,K+-ATPase activity found in the child with HA despite very low levels of plasma aldosterone favors the idea of a primary defect in Na+,K+-ATPase in children with PHA.…”

Section: Discussionmentioning

confidence: 59%

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Reduced Na+, K+-ATPase Activity in Patients with Pseudohypoaldosteronism

et al. 1994

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ABSTRACT. Pseudohypoaldosteronism is a hereditary wasting syndrome varies widely with age and is particularly salt-wasting syndrome usually seen in infancy with weight severe in infancy. Spontaneous improvement in sodium conserloss, dehydration, and failure to thrive. The patho-vation occurs as the individuals become older. Aldosterone dephysiologic origin of pseudohypoaldosteronism is un-ficiency in the face of overproduction of zona glomerulosa 18-known. The defect could be related to the unresponsiveness hydroxycorticosterone (10) was found, an abnormality for which of target organs to mineralocorticoids resulting in hypo-the term type 2 corticosterone methyl-oxidase defect was coined natremia, hyperkalemia, and markedly elevated plasma (1 1). Treatment with mineralocorticoids and high-sodium diet aldosterone and renin levels. Red blood cell Na+,K+-ATP reduces the secretion of the aldosterone precursor, restores elecase activity was measured in a pair of twins with pseudo-trolyte balance, and normalizes the growth rate (3). hypoaldosteronism, in an unrelated child with hypoaldosLittle information has been added in regard to the pathoteronism, and in an age-matched group of 50 healthy physiologic expression of PHA since its original description by infants and young children. The enzyme was assayed by a Cheek and Peny (12) in 1958. It is believed that patients with method that couples A T P hydrolysis with NADH oxida-PHA represent a blunted target organ responsiveness to aldostertion. Plasma renin and aldosterone levels were measured one either because of a reduced number in mineralocorticoid by RIA. Red blood cell Na+,K+-ATPase activity in the (type 1) receptors (13) or a low receptor affinity to aldosterone twins with pseudohypoaldosteronism was very low a t the (14). A defect in sodium reabsorption along the proximal tubule time of diagnosis (3 wk). In both twins a time-related and the loop of Henle has also been suggested (15). Low Na+,K+-gradual increase in enzyme activity was observed during ATPase activity in both proximal and distal tubular segments in the 1st mo of life, reaching control values between 6 and 8 a child with PHA has been demonstrated previously (16). No mo of age. This increase was associated with both a reduc-further data substantiating these findings have been published. tion in salt requirement and clinical improvement. Plasma In the present study we investigated RBC Na+,K+-ATPase activrenin activity and aldosterone levels were very high a t the ity in a pair of twins with PHA and in an infant with HA to time of diagnosis. Plasma renin activity reverted gradually clarify the pathophysiologic expression of PHA. to normal values, whereas aldosterone levels remained high throughout the follow-up period. The child with hypoaldosteronism had normal Na+,K+-ATPase activity a t diag- The twins were born at 35 wk of gestation weighing 2.3 and 2.2 RBC, red blood cell kg, respectively. At the age of 3 wk, they were hospitalized HA, hypoaldosteronism because of vomiting, lethargy, and loss of app...

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Erythrocyte transmembrane Na and K fluxes in pseudohypoaldosteronism

Cited by 4 publications

References 17 publications

Sodium potassium adenosine triphosphatase activity in preterm and term infants and its possible role in sodium homeostasis during maturation

Sodium potassium adenosine triphosphatase activity in preterm and term infants and its possible role in sodium homeostasis during maturation

Erythrocyte Na⁺,K⁺‐ATPase and nasal potential in pseudohypoaldosteronism*

Reduced Na+, K+-ATPase Activity in Patients with Pseudohypoaldosteronism

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Erythrocyte transmembrane Na and K fluxes in pseudohypoaldosteronism

Cited by 4 publications

References 17 publications

Sodium potassium adenosine triphosphatase activity in preterm and term infants and its possible role in sodium homeostasis during maturation

Sodium potassium adenosine triphosphatase activity in preterm and term infants and its possible role in sodium homeostasis during maturation

Erythrocyte Na+,K+‐ATPase and nasal potential in pseudohypoaldosteronism*

Reduced Na+, K+-ATPase Activity in Patients with Pseudohypoaldosteronism

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Erythrocyte Na⁺,K⁺‐ATPase and nasal potential in pseudohypoaldosteronism*