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

Giapros, Vasileios; Tsatsoulis, Agathoklis; Drougia, Ekaterini A.; Kollios, Konstantinos; Siomou, Ekaterini; Andronikou, Styliani

doi:10.1007/s00467-004-1500-6

Cited by 23 publications

(28 citation statements)

References 15 publications

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“…In the face of increased urinary salt loss, infants are not able to compensate by increasing salt intake as older children may be able to do. Our patients represent a slightly older group than those previously reported by some [3, 16, 17], though 3 of our 4 patients (including the first episode in the case of patient 4) presented at age 3.5 months or younger, in agreement with the report by Melzi et al [5], which suggests that infants 3 months of age or less seem to be at higher risk for this complication of pyelonephritis.…”

Section: Discussionsupporting

confidence: 92%

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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Section: Discussionsupporting

confidence: 92%

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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“…Secondary PHA may resemble congenital adrenal hyperplasia in neonates [12,26]. A differential diagnosis may be achieved by acute analysis of urine culture and renal ultrasonography.…”

Section: Discussionmentioning

confidence: 98%

“…A differential diagnosis may be achieved by acute analysis of urine culture and renal ultrasonography. [1], several clinical cases of children have been reported in the literature, mostly in association with urinary tract anatomical abnormalities, acute pyelonephritis, or the combination of both [2][3][4][5][6][7][8][9][10][11][12][13][14]. However, more pediatric specialists should become aware of this condition and its natural history.…”

Section: Discussionmentioning

confidence: 98%

“…Secondary PHA due to renal unresponsiveness to aldosterone produces a renal saltlosing state with hyponatremia, hyperkalemia, acidosis, and high levels of plasma renin activity and aldosterone. However, it has also been observed in patients with urinary tract obstruction, hydronephrosis, and vesicoureteral reflux without urinary tract infection [11][12][13][14]. A high intrarenal pressure in the renal tubules is considered to be the likely pathogenesis of this renal dysfunction, leading to tubular resistance to aldosterone [1,2,12,15].…”

Section: Introductionmentioning

confidence: 99%

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Electrolyte disturbances in acute pyelonephritis

et al. 2011

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The aim of this study was to determine whether renal unresponsiveness to aldosterone associated with hyperkalemia is present in infants with acute pyelonephritis in the absence of significant urinary tract anomalies and to describe the clinical characteristics of patients presenting an inadequate renal response to hyperkalemia. The patient cohort comprised 113 infants with acute pyelonephritis (APN), based on the criteria of a temperature >38°C and significant bacteriuria. Serum and urine electrolytes, creatinine, osmolality, and renal tubular function tests were performed at diagnosis. The findings were compared to those present in 75 children who had fever without significant bacteriuria. Hyperkalemia (>5.5 mmol/L) was observed in infants with an APN diagnosis, who exhibited a lower transtubular potassium concentration gradient (TTKG) and a higher fractional sodium excretion. We defined inadequate renal response to hyperkalemia as the combination of hyperkalemia and TTKG below the normal range established for the age of the subject. Infants presenting an inadequate response to hyperkalemia were younger and associated more frequently with an APN diagnosis. This alteration could be explained by the renal interstitial inflammation present in acute pyelonephritis and the immaturity of the renal tubular responsiveness to aldosterone due to infancy in the absence of urinary tract infection or obstruction.

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“…7,[9][10][11]15 Este trabajo además evidencia la heterogeneidad clínico-fenotípica del síndrome. Tres pacientes se presentaron con anomalías urológicas y en dos de ellos asociadas con síndromes malformativos: uno con cloaca posterior (un caso similar fue descripto previamente) 14 y otro con anomalía VACTERL (sin asociación previa descripta).…”

Section: Discussionunclassified

Seudohipoaldosteronismo de tipo 1: una emergencia hidroelectrolítica infrecuente. Comunicación de cuatro casos

Marín¹

2011

Arch Argent Pediat

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RESUMENEl seudohipoaldosteronismo de tipo 1 es un síndrome infrecuente de resistencia a la aldosterona que se manifiesta con pér-dida salina, hiponatremia, hiperpotasemia, acidosis metabólica hiperclorémica e hiperaldosteronismo hiperreninémico. El sín-drome puede ser genético; secundario a uropatías e infección urinaria entre otras causas o presentarse esporádicamente. La pérdida salina puede ser sistémica y grave o localizada a nivel renal, por lo general, con mejor pronóstico. El cuadro clínico se manifiesta predominantemente en los primeros siete meses de vida; un marcado retraso pondoestatural y vómitos recurrentes suelen ser los signos clínicos habituales, rara vez se presenta como una emergencia hidroelectrolítica en forma de shock hipovolémico, arritmias cardíacas hiperpotasémicas y crisis convulsiva por hiponatremia. Se presentan cuatro pacientes que debutaron como una emergencia hidroelectrolítica. Palabras clave: seudohipoaldosteronismo de tipo 1, hiponatremia, hiperpotasemia, aldosterona, infección urinaria. SUMMARYPseudohypoaldosteronism type 1 is a rare syndrome of resistance to aldosterone manifested by salt wasting, hyponatremia, hyperkalemia, hyperchloremic metabolic acidosis, and hiperreninemic hyperaldosteronism. The syndrome may be genetic, secondary to uropathies and urinary tract infection among other causes or it may occur sporadically. The salt wasting may be systemic and severe or localized to the kidney usually with better prognosis. The clinical picture is prevalent in the first seven months of life, failure to thrive and recurrent vomiting are usually the common clinical signs, an electrolyte emergency in the form of hypovolemic shock, hyperkalemic cardiac arrhythmias and hyponatremic seizures is rare. Four patients presenting with an electrolyte emergency are reported INTRODUCCIÓNLa aldosterona es un mineralocorticoide que actúa principalmente en el riñón y secundariamente en colon, pulmón y glándulas sudoríparas, salivales y lagrimales. Su acción requiere, a nivel celular, la existencia del receptor mineralocorticoide (RM) y de proteínas transportadoras de sodio denominadas canales epiteliales de sodio (CES), que actúan a nivel apical, donde favorece la reabsorción electrogénica de sodio y la eliminación de potasio hacia la orina.

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

Cited by 23 publications

References 15 publications

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

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

Electrolyte disturbances in acute pyelonephritis

Seudohipoaldosteronismo de tipo 1: una emergencia hidroelectrolítica infrecuente. Comunicación de cuatro casos

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