Transient Pseudohypoaldosteronism With Hyponatremia-Hyperkalemia in Infant Urinary Tract Infection

Schoen, Edgar J.; Bhatia, Sangeeta; Ray, G. Thomas; Clapp, Wesley; To, Trinh T.

doi:10.1097/00005392-200202000-00063

Cited by 18 publications

(19 citation statements)

References 27 publications

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“…In the first patient, the electrolyte and hormonal disturbances persisted well beyond successful treatment of the urinary tract infection, and could be attributed mainly to the dysplastic kidney and not to vesicoureteral reflux. Infants with urinary tract infection associated with vesicoureteral reflux and secondary PHA had no electrolyte and hormonal disturbances after treatment of the underlying infection [15]. In a previous study of children with urinary tract infection and renal scarring, the hormonal imbalance also persisted after successful treatment of the infection [16].…”

Section: Discussionmentioning

confidence: 91%

Rare causes of acute hyperkalemia in the 1st week of life

et al. 2004

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We describe three neonates with hyperkalemia and renal salt wasting during the 1st week of life. Endocrinological evaluation led to the diagnosis of selective hypoaldosteronism (HA) in two neonates and secondary pseudohypoaldosteronism (PHA) in one. The infant with PHA developed a urinary tract infection, and radiological investigation demonstrated a small dysplastic left kidney with vesicoureteral reflux. The electrolyte and hormonal disturbances in this infant persisted throughout the first months of life. The two infants with selective HA improved rapidly after administration of fludrocortisone orally and the electrolytes and renin values returned to normal. Secondary PHA and selective HA should be considered in the differential diagnosis in salt-losing neonates during the first days of life. Renal ultrasonography, urine culture, and assays of aldosterone and plasma renin activity should be performed in any infant presenting with hyperkalemia and salt wasting after the exclusion of congenital adrenal hyperplasia.

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Section: Discussionmentioning

confidence: 91%

Rare causes of acute hyperkalemia in the 1st week of life

et al. 2004

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“…et al [13] reported on 17 or 11 patients, respectively. Other reports with more than single cases included six [6,17], three [16,23,27,31,32] or two [18,20,[33][34][35][36][37] patients. Our patients exhibited clinical and laboratory features characteristic of THPA1, in keeping with those described in many previous reports [6,13,17,19,26,31].…”

Section: Discussionmentioning

confidence: 99%

“…This sensitivity lessens as the infant undergoes transition to a higher level of salt intake, either due to a gene-environment interaction or by the development of the renal tubule, allowing improved efficiency of the reninangiotensin-aldosterone system after early infancy [2]. [17,23,31,47]; nonobstructive nonrefluxive megaureter [7,34]; neurogenic bladder, VUR associated with UVJO or renal dysplasia [13]; ectopic urethral orifice and contralateral renal hypodysplasia [25]; unilateral renal hypoplasia [32] UT obstruction increases the intrarenal synthesis of a number of cytokines, such as transforming growth factor beta-1 (TGF-β1), tumor necrosis factor alpha (TNF-α), interleukin (IL)-1, IL-6 and vasoactive compounds (angiotensin II, endothelin thromboxane A 2 and prostaglandins) [10]. TGF-β1, produced by infiltrated macrophages and renal tubule cells [10,51] has been show to induce inhibition of the action of aldosterone [52].…”

Section: Discussionmentioning

confidence: 99%

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Transient type 1 pseudo-hypoaldosteronism: report on an eight-patient series and literature review

et al. 2009

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Eight boys aged 2-12 weeks with urinary tract malformations (UTMs) exhibited features of transient type 1 pseudo-hypoaldosteronism (TPHA1) in the course of urinary tract infection (UTI). Hyponatremia (120.9+/-5.8 mmol/l), hyperkalemia (6.9+/-0.9 mmol/l), metabolic acidosis (plasma bicarbonate 11+/-1.4 mmol/l), and a rise in serum creatinine levels (145+/-101 micromol/l) were associated with high urinary sodium (Na) and low potassium (K) excretion. Tubular resistance to aldosterone was indicated by high plasma aldosterone concentrations (170.4+/-100.5 ng/dl), high levels of the plasma aldosterone to potassium ratio (25.2+/-15.6), and diminished urinary K/Na values (0.31+/-0.19). With appropriate therapy, serum electrolytes, creatinine, and acid-base balance normalized within 2 weeks. A Medline search revealed another 85 cases of TPHA1 reported to date. All of the 93 patients were less than 7 months of age and 90% were less than 3 months of age, 90.3% suffered from UTM, with associated UTI in 89% of them, 11% had UTMin the absence of UTI, and 9.7% showed isolated UTI. These findings indicate that early infancy is the main contributing factor for TPHA1 to occur and that UTI and UTMare additional factors, with at least one being required for its development.

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“…Our cases, we believe, support the view that aldosterone resistance may occur solely in the setting of a urinary tract infection, and that the association with urinary tract malformations is due to their high incidence in infants diagnosed with severe urinary tract infections. Four other reports in the literature [6, 13, 16, 17] suggest that severe urinary tract infection itself, and not the combination with an underlying malformation may cause aldosterone resistance. In the case of patient 4, earlier hydronephrosis associated with meningomyelocoele had resolved, but during her second admission she again presented with a urinary tract infection and evidence of pseudohypoaldosteronism, suggesting that the inciting factor was the presence of severe renal tubular inflammation rather than urinary tract obstruction.…”

Section: Discussionmentioning

confidence: 99%

Transient Pseudohypoaldosteronism due to Urinary Tract Infection in Infancy: A Report of 4 Cases

Nandagopal

Vaidyanathan

Kaplowitz

2009

International Journal of Pediatric Endocrinology

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Hyponatremia with hyperkalemia in infancy is an uncommon but life-threatening occurrence. In the first weeks of life, this scenario is often associated with aldosterone deficiency due to salt-wasting congenital adrenal hyperplasia. However, alternative diagnoses involving inadequate mineralocorticoid secretion or action must be considered, particularly for infants one month of age or older. We report four infants who presented with profound hyponatremia accompanied by urinary tract infection, ultimately leading to the diagnosis of transient pseudohypoaldosteronism. Our cases provide support for the idea that the renal tubular resistance to aldosterone is due to urinary tract infection itself rather than to underlying urinary tract anomalies typically found in these infants. Awareness of this condition is important so that serum aldosterone, urine sodium, and urine cultures may be obtained immediately in any infant presenting with hyponatremia and hyperkalemia in whom a diagnosis of congenital adrenal hyperplasia was not found. Adequate replacement with intravenous saline and antibiotic therapy is sufficient to correct sodium levels over 24–48 hours.

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Transient Pseudohypoaldosteronism With Hyponatremia-Hyperkalemia in Infant Urinary Tract Infection

Abstract: Because of future renal scarring, decreased renal function and possible hypertension, appropriate hormonal studies should be performed in infants with infant urinary tract infection who also have hyponatremia and hyperkalemia.

Cited by 18 publications

References 27 publications

Rare causes of acute hyperkalemia in the 1st week of life

Rare causes of acute hyperkalemia in the 1st week of life

Transient type 1 pseudo-hypoaldosteronism: report on an eight-patient series and literature review

Transient Pseudohypoaldosteronism due to Urinary Tract Infection in Infancy: A Report of 4 Cases

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