Newborn with pseudohypoaldosteronism and miliaria rubra

Akçakuş, Mustafa; Köklü, Esad; Poyrazoğlu, Hakan; Kurtoğlu, Selim

doi:10.1111/j.1365-4632.2006.02936.x

Cited by 14 publications

(14 citation statements)

References 8 publications

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“…Akcakus et al [3] reported a patient with AR-PHA1 and cutaneous eruptions that were similar to those of our patient. They reported that the cutaneous eruptions might have been occurred due to obstruction of eccrine sweat glands through inflammation caused by excessive sodium excretion during hyponatremic crises (sweat sodium concentration, 138 mEq/l).…”

Section: Discussionsupporting

confidence: 86%

“…Sodium chloride is lost via the kidneys, resulting in a reduced sodium level and an elevated potassium level in the serum, and leading to life-threatening disturbances of water and acid-base concentrations. The excessive sodium loss has been considered to be caused by an unresponsiveness of the renal tubules to endogenous and exogenous mineralocorticoids [1][2][3].…”

Section: Discussionmentioning

confidence: 99%

“…The autosomal recessive form of PHA1 (AR-PHA1) is characterized by multiple organ resistance to aldosterone, whereas the autosomal dominant form of PHA1 (AD-PHA1) is associated with isolated renal resistance to aldosterone and resistance to mineralocorticoids. Unlike AD-PHA1, AR-PHA1 is also characterized by increased sodium concentration in other body fluids (saliva, sweat, colonic, and pulmonary) in addition to the urine [1][2][3][4].…”

Section: Introductionmentioning

confidence: 99%

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Miliaria rubra and thrombocytosis in pseudohypoaldosteronism: Case report

et al. 2011

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Pseudohypoaldosteronism type 1 (PHA1) is a disease involving a state of renal tubular unresponsiveness to the action of aldosterone and characterized by excessive salt loss in the urine, hyperkalemia, and metabolic acidosis. In kidney, PHA1 may occur primarily by mutations in the subunits of the sodium channel or in the mineralocorticoid receptors, and secondarily by several renal disorders. Miliaria rubra and thrombocytosis are reported in a 6-month-old girl with PHA1. In patients with PHA1, miliaria rubra-like cutaneous eruptions are suggested to occur due to obstruction of eccrine sweat glands through inflammation caused by excessive sodium excretion in sweat during hyponatremic crises. The presence of thrombocytosis in patients with PHA1 has not been previously reported. A hypothesis is proposed suggesting that sympathetic activation which provides vascular tonus during sodium excretion in sweat and salt-depletion crisis may play a role in the development of eruptions and thrombocytosis in patients with PHA1.

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Section: Discussionsupporting

confidence: 86%

Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

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Miliaria rubra and thrombocytosis in pseudohypoaldosteronism: Case report

et al. 2011

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“…The condition usually occurs at the elasticized openings of the diaper and at the superolateral abdomen, where the diaper tapes attach to the front panel of the diaper. 65,66 Scabies Frequent in certain socioeconomic areas. Scabies can involve the diaper region in infants.…”

Section: Prevention Of Diaper Dermatitismentioning

confidence: 99%

Diagnosis and management of diaper dermatitis in infants with emphasis on skin microbiota in the diaper area

et al. 2017

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Diaper dermatitis is the most common inflammatory skin eruption within the diaper area in infants and toddlers. Although it rarely causes problems for prolonged periods of time, it causes considerable distress for both infants and parents. Diapered skin is exposed to friction, excessive hydration, varying pH, and is in constant contact with urine and feces, both of which are highly irritant to the skin. The three most common types of diaper dermatitis include chaffing dermatitis, irritant contact dermatitis, and diaper candidiasis. However, a variety of other skin conditions may manifest in the diaper area. Candida albicans and Staphylococcus aureus are most frequently isolated from the affected area and are thought to have a predominant role in diaper dermatitis. The review includes an overview of the epidemiology, etiology, and common skin microbiota in the diaper area. State-of-the-art strategies for diagnosis, management, and prevention of diaper dermatitis are also included. Appropriate skin care can help in the prevention of diaper dermatitis and can aid the reparation of the skin. This review discusses common causes, skin microbiota within the diapered area, differential diagnosis, and finally possible prevention and treatment recommendations. Recent findings have proven that the key to efficient eradication of irritant diaper dermatitis lies in its prevention.

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“…PHA is a rare heterogeneous syndrome of mineralocorticoid resistance classified as type 1, type 2 and type 3 [7,8]. PHA1 is a life-threatening salt-losing disease in neonates and infants.…”

Section: Discussionmentioning

confidence: 99%

Hook Effect: A Pitfall Leading to Misdiagnosis of Hypoaldosteronism in an Infant with Pseudohypoaldosteronism

Akın¹,

Kurtoğlu²,

Kendirci³

et al. 2010

Horm Res Paediatr

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We report herein the case of a premature infant who presented with failure to thrive, hyponatremia, hyperkalemia and metabolic acidosis. Initial serum hormone profiling suggested isolated hypoaldosteronism (aldosterone: 0.01 pg/ml, normal range: 50–900 pg/ml). A gas chromatography-mass spectrometry spot urinary steroid profile showed grossly elevated levels of 18-hydroxy-tetrahydro-11-dehydrocorticosterone (18-hydroxy-THA: 5,893 µg/l; normal upper limit 36 µg/l) and tetrahydroaldosterone (TH-Aldo: 5,749 µg/l; normal upper limit 36 µg/l) which are aldosterone precursor metabolite and aldosterone metabolite, respectively. Thus, aldosterone synthase deficiency was excluded and pseudohypoaldosteronism (PHA) was suggested. A repeated test after dilution of the serum revealed a very high level of aldosterone (6,490 pg/ml), confirming the diagnosis of PHA in this case.

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Newborn with pseudohypoaldosteronism and miliaria rubra

Cited by 14 publications

References 8 publications

Miliaria rubra and thrombocytosis in pseudohypoaldosteronism: Case report

Miliaria rubra and thrombocytosis in pseudohypoaldosteronism: Case report

Diagnosis and management of diaper dermatitis in infants with emphasis on skin microbiota in the diaper area

Hook Effect: A Pitfall Leading to Misdiagnosis of Hypoaldosteronism in an Infant with Pseudohypoaldosteronism

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