754 results match your criteria Hyporeninemic Hypoaldosteronism


Early Renin Recovery After Adrenalectomy in Aldosterone-Producing Adenomas: A Prospective Study.

Horm Metab Res 2022 Apr 12;54(4):224-231. Epub 2022 Apr 12.

Department of Internal Medicine, University of Sao Paulo, Ribeirao Preto, SP, Brazil.

The aim of the study was to clarify the relationship and the time of aldosterone and renin recoveries at immediate and long-term follow-up in aldosterone-producing adenoma (APA) patients who underwent adrenalectomy. Prospective and longitudinal protocol in a cohort of APA patients was followed in a single center. Among 43 patients with primary aldosteronism (PA), thirteen APA patients were enrolled in this study. Read More

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Ineffectiveness of Intermittent Hemodialysis in a Critically Ill COVID-19 Patient: A Case of Persistent Heparin-Induced Hyperkalemia.

Case Rep Nephrol 2022 4;2022:8613656. Epub 2022 Mar 4.

Intensive Care Unit, Centre Médical de Kinshasa, Kinshasa, Democratic Republic of the Congo.

Heparin is widely used in the intensive care unit despite the risk of bleeding it can cause. Although it is rarely reported, hyperkalemia is one of the side effects associated with heparin therapy (unfractionated or fractionated heparin). It would be secondary to hypoaldosteronism by blocking the biosynthesis of aldosterone in the adrenal gland and often appears in context of prolonged heparin therapy or inappropriate renin production in elderly, diabetic, and kidney insufficiency patients. Read More

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Pattern Recognition versus Pathogenesis: Electrolytes in a Patient with Adrenal Insufficiency.

Clin J Am Soc Nephrol 2022 04 3;17(4):585-587. Epub 2022 Mar 3.

Nephrology Division, Department of Medicine, Beth Israel Deaconess Medical Center, Boston, Massachusetts.

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Pseudo-hypoaldosteronism secondary to infantile urinary tract infections: role of ultrasound.

Ital J Pediatr 2022 Jan 24;48(1):14. Epub 2022 Jan 24.

Department of Clinical Sciences and Community Health, Università degli Studi di Milano, Milan, Italy.

Background: The biochemical hallmarks of transient pseudo-hypoaldosteronism associated with a pyelonephritis include hyponatremia, hyperkalemia, and acidosis. We tested if the kidney-urinary tract ultrasound helps in predicting the diagnosis of overt pseudo-hypoaldosteronism in infants with a pyelonephritis.

Cases Presentation: Between 2013 and 2020, we managed 71 previously healthy infants 4 weeks to 24 months of age with a pyelonephritis (42 males and 29 females) and made the biochemical diagnosis of pseudo-hypoaldosteronism in 17 (24%). Read More

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January 2022

Familial hyperkalemic hypertension: hyperkalemia not hypertension defines dominant KLHL3 disease and may permit earlier recognition and tailored therapy.

J Nephrol 2022 Jan 8. Epub 2022 Jan 8.

Division of Nephrology, Department of Internal Medicine, SE419 GH, University of Iowa Carver College of Medicine, 200 Hawkins Drive, Iowa City, IA, 52242, USA.

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January 2022

Excessive Weight Loss in a Neonate - Novel Mutation Causing Primary Hypoaldosteronism.

Indian Pediatr 2021 Dec;58(12):1186-1187

Division of Neonatology, Department of Pediatrics, Fernandez Foundation, Hyderabad, Telengana.

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December 2021

Case Report: Newborns With Pseudohypoaldosteronism Secondary to Excessive Gastrointestinal Losses Through High Output Stoma.

Front Pediatr 2021 17;9:773246. Epub 2021 Nov 17.

Division of Pediatric Surgery, Department of Pediatrics, Ditmanson Medical Foundation Chia-Yi Christian Hospital, Chiayi City, Taiwan.

Life-threatening electrolyte imbalance is not uncommon in preemies. Differential diagnosis is important for immediate treatment. The syndrome of pseudohypoaldosteronism (PHA) is characterized by increased aldosterone secretion associated with clinical signs of hypoaldosteronism reflecting mineralocorticoid resistance. Read More

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November 2021

An unusual presentation of primary adrenal insufficiency with new onset type 1 diabetes: case report and review of the literature.

J Pediatr Endocrinol Metab 2022 Apr 24;35(4):531-534. Epub 2021 Nov 24.

Pediatric Endocrinology, University of Minnesota Masonic Children's Hospital, Minneapolis, MN, USA.

Objectives: To describe an atypical presentation of primary adrenal insufficiency in conjunction with new onset type 1 diabetes.

Case Presentation: Here, we describe a case of new-onset type 1 diabetes (T1D) presenting simultaneously with an unusual presentation of primary adrenal insufficiency in a previously healthy 16-year-old. He was admitted for a typical presentation of diabetic ketoacidosis, but with extreme hyponatremia. Read More

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Persistent hypoaldosteronism post-adrenalectomy for primary aldosteronism - a role for pre-operative spironolactone?

Endocrinol Diabetes Metab Case Rep 2021 Oct 1;2021. Epub 2021 Oct 1.

Department of Endocrinology, Austin Health, Heidelberg, Victoria, Australia.

Summary: Primary aldosteronism is one of the most common (affecting up to 10%) yet treatable causes of hypertension in our community, notable due to an associated elevated risk of atrial fibrillation, stroke and myocardial infarction compared to essential hypertension. Guidelines have focussed on improving case detection due to significant underdiagnosis in the community. While our case experienced significant delay in diagnosis, we highlight a state of protracted, persistent post-operative hypoaldosteronism which manifested with severe hyponatraemia and hyperkalaemia, necessitating long-term mineralocorticoid replacement. Read More

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October 2021

Nephrogenic Syndrome of Inappropriate Antidiuresis Mimicking Hyporeninemic Hypoaldosteronism: Case Report of Two Infants.

J Clin Res Pediatr Endocrinol 2021 Oct 14. Epub 2021 Oct 14.

Ondokuz Mayıs University Medical Faculty, Department of Pediatric Endocrinology, Samsun, Turkey.

Nephrogenic syndrome of inappropriate antidiuresis (NSIAD) is an X-linked disease caused by activating mutations in the arginine vasopressin (AVP) receptor-2 () gene. Affected patients excrete concentrated urine despite very low levels of AVP, and consequently develop euvolemic hyponatremia. Due to its low frequency, patients may be misdiagnosed and treated incorrectly. Read More

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October 2021

Impaired aldosterone response to ACTH without hypoaldosteronism: An unrecognized secretory pattern in search of clinical implications.

Clin Endocrinol (Oxf) 2022 04 29;96(4):513-520. Epub 2021 Sep 29.

Institute of Endocrinology, Metabolism and Hypertension, Tel Aviv-Sourasky Medical Center, Tel Aviv, Israel.

Context: Aldosterone has been recently characterized as a 'stress hormone'. Stress per se elicits a sizable rise in aldosterone secretion, which could be replicated by the administration of a low dose (0.03-1 μg, IV) of adrenocorticotropic hormone (ACTH). Read More

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[Low molecular weight heparin-induced hyperkalemia and hyponatremia. Report of one case].

Authors:
Gonzalo Eymin

Rev Med Chil 2021 Feb;149(2):291-294

Departamento de Medicina Interna, Facultad de Medicina, Pontificia Universidad Católica de Chile, Santiago, Chile.

Low molecular weight heparin-induced hyperkalemia is not an uncommon side effect. The development of hyponatremia is well described although it is less common. We report a 72-year-old woman with lumbar metastases who developed hyponatremia and hyperkalemia on the tenth day of hospitalization. Read More

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February 2021

Catch-up Growth and Discontinuation of Fludrocortisone Treatment in Aldosterone Synthase Deficiency.

J Clin Endocrinol Metab 2022 01;107(1):e106-e117

Department of Pediatric Endocrinology and Diabetes, Marmara University, School of Medicine, Istanbul, Turkey.

Background: Aldosterone synthase deficiency (ASD) caused by mutations in the CYP11B2 gene is characterized by isolated mineralocorticoid deficiency. Data are scarce regarding clinical and biochemical outcomes of the disease in the follow-up.

Objective: Assessment of the growth and steroid profiles of patients with ASD at the time of diagnosis and after discontinuation of treatment. Read More

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January 2022

Aldosterone signaling defect in young infants: single-center report and review.

BMC Endocr Disord 2021 Jul 9;21(1):149. Epub 2021 Jul 9.

Department of Pediatrics, The First affiliated Hospital, Sun Yat-Sen University, No. 58, Zhongshan II Rd, Guangzhou, 510080, P. R. China.

Background: Aldosterone (Ald) is a crucial factor in maintaining electrolyte and water homeostasis. Defect in either its synthesis or function causes salt wasting (SW) manifestation. This disease group is rare, while most reported cases are sporadic. Read More

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A rare case of persistent hyperkalaemia.

Ann Clin Biochem 2021 11 4;58(6):661-665. Epub 2021 Jul 4.

Department of Biochemistry & Immunology, University Hospital of Wales, Wales, UK.

Hyperkalaemia is a common biochemical finding that can allude to preanalytical or truly pathological causes. Here, we present a case of a 41-year-old female patient who has regularly presented with incidences of isolated hyperkalaemia since 2012, with otherwise normal renal function and no other associated symptoms. Investigations into the patient's family history revealed similar biochemical findings in her brother and eldest son. Read More

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November 2021

Liddle Syndrome due to a Novel c.1713 Deletion in the Epithelial Sodium Channel β-Subunit in a Normotensive Adolescent.

AACE Clin Case Rep 2021 Jan-Feb;7(1):65-68. Epub 2020 Dec 28.

College of Medicine, California Northstate University, Elk Grove, California.

Objective: Liddle syndrome (LS) is a rare autosomal dominant condition secondary to a gain-of-function mutation affecting the epithelial sodium channels (ENaCs) in the distal nephron. It presents with early-onset hypertension, hypokalemia, and metabolic alkalosis in the face of hyporeninemia and hypoaldosteronism. We report a novel mutation affecting the ENaCs in a normotensive adolescent with LS. Read More

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December 2020

Quality of Life in Men With Congenital Adrenal Hyperplasia Due to 21-Hydroxylase Deficiency.

Front Endocrinol (Lausanne) 2021 19;12:626646. Epub 2021 Mar 19.

Department of Pediatrics, Amalia Children's Hospital, Radboud University Medical Center, Nijmegen, Netherlands.

Congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency (21OHD) is a disorder of adrenal steroid biosynthesis, leading to hypocortisolism, hypoaldosteronism, and hyperandrogenism. Impaired quality of life (QoL) has been demonstrated in women with CAH, but data on men with CAH are scarce. We hypothesized that disease severity and poor treatment control are inversely associated with QoL. Read More

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December 2021

Cotrimoxazole-induced hyperkalaemia in a patient with known hypoaldosteronism.

BMJ Case Rep 2021 Mar 4;14(3). Epub 2021 Mar 4.

Department of Diabetes and Endocrinology, Mater Dei Hospital, Msida, Malta.

A70-year-old man, with established hypoadrenalism due to a previous bilateral adrenalectomy, was admitted with recurrent episodes of postural dizziness and presyncope. He had been discharged from hospital 3 weeks earlier on a 1-month course of cotrimoxazole following a diagnosis of prostatitis. His electrolytes on admission showed new onset hyponatraemia and hyperkalaemia. Read More

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tabolic Acidosis, yperkalemia, enal nresponsiveness to Aldosterone Syndrome: Response to Treatment with Low-Potassium Diet.

Saudi J Kidney Dis Transpl 2020 Sep-Oct;31(5):1134-1139

Department of Nephrology and Hypertension, Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, Glickman Urological and Kidney Institute, Cleveland Clinic, Cleveland, Ohio, USA.

Gordon syndrome involves hyperkalemia, acidosis, and severe hypertension (HTN) with hypercalciuria, low renin and aldosterone levels. It is commonly observed in children and adolescents. Such patients respond successfully to sodium restriction and thiazide diuretics. Read More

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October 2021

Type IV RTA in Chronic Adrenal Insufficiency and Concomitant Lisinopril Treatment.

Case Rep Endocrinol 2020 19;2020:8897112. Epub 2020 Oct 19.

Department of Medicine, University of Pittsburgh Medical Center, 200 Lothrop Street, Suite N715, Pittsburgh, PA 15213, USA.

Type IV renal tubular acidosis (RTA) is the only RTA characterized by hyperkalemia, and it is caused by a true aldosterone deficiency or renal tubular aldosterone hyporesponsiveness. It is frequent among hospitalized patients as it is related to type 2 diabetes mellitus (T2DM) and common medications such as ACE-inhibitors (ACE-is) and trimethoprim-sulfamethoxazole (TMP-SMX). Drug-induced RTA commonly manifests in patients with predisposing conditions such as mild renal insufficiency and certain pharmacological therapies. Read More

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October 2020

Molecular Analysis of the CYP11B2 Gene in 62 Patients with Hypoaldosteronism Due to Aldosterone Synthase Deficiency.

J Clin Endocrinol Metab 2021 01;106(1):e182-e191

Division of Endocrinology, Diabetes and Metabolism, Center for Rare Pediatric Endocrine Diseases, First Department of Pediatrics, Medical School, National and Kapodistrian University of Athens, "Agia Sophia" Children's Hospital, Athens, Greece.

Context: Isolated congenital hypoaldosteronism presents in early infancy with symptoms including vomiting, severe dehydration, salt wasting, and failure to thrive. The main causes of this rare autosomal recessive disorder is pathogenic variants of the CYP11B2 gene leading to aldosterone synthase deficiency.

Objective: To investigate the presence of CYP11B2 pathogenic variants in a cohort of patients with a clinical, biochemical, and hormonal profile suggestive of aldosterone synthase deficiency. Read More

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January 2021

COVID-19 and late-onset hypertension with hyporeninaemic hypoaldosteronism.

Int J Clin Pract 2021 01;75(1):e13773

Wexham Park Hospital, Frimley Health NHS Foundation Trust, Slough, UK.

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January 2021

Ibuprofen-Induced Distal Renal Tubular Acidosis and Hyporeninemic Hypoaldosteronism: Enough NSAID.

Mayo Clin Proc 2020 10;95(10):2296-2299

Wexham Park Hospital, Frimley Health NHS Foundation Trust, Slough, UK; University of Cyprus Medical School, Nicosia, Cyprus.

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October 2020

Type 4 Hyperkalemic Renal Tubular Acidosis After Coronary Artery Bypass Grafting.

J Cardiothorac Vasc Anesth 2021 09 16;35(9):2772-2774. Epub 2020 Aug 16.

Division of Cardiothoracic Surgery, Department of Surgery, University of Kentucky College of Medicine, Lexington, KY.

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September 2021

Aldosterone synthase deficiency type II: an unusual presentation of the first Greek case reported with confirmed genetic analysis.

Endocr Regul 2020 Jul;54(3):227-229

Second Department of Paediatrics, Children's Hospital 'P. & A. Kyriakou, National and Kapodistrian University of Athens, Athens, Greece.

Objective: Aldosterone synthase deficiency (ASD) is a rare, autosomal recessive inherited disease with an overall clinical phenotype of failure to thrive, vomiting, severe dehydration, hyperkalemia, and hyponatremia. Mutations in the CYP11B2 gene encoding aldosterone synthase are responsible for the occurrence of ASD. Defects in CYP11B2 gene have only been reported in a limited number of cases worldwide. Read More

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Aldosterone deficiency with a hormone profile mimicking pseudohypoaldosteronism.

J Pediatr Endocrinol Metab 2020 Nov;33(11):1501-1505

Departments of Pediatrics & Endocrinology, Hospital Infantil Universitario Niño Jesús, Instituto de Investigación La Princesa, Madrid, Spain.

Background Aldosterone deficiency (hypoaldosteronism) or aldosterone resistance (pseudohypoaldosteronism) both result in defective aldosterone activity. Case presentation A 42-day-old man presented with failure to thrive, hyponatremia, high urine sodium output, severe hyperkalemia and high plasma renin activity and aldosterone levels. NR3C2, SCNN1A, B and G sequencing showed no variants. Read More

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November 2020

The First Reported Case of Hyperreninemic Hypoaldosteronism Due to Mucopolysaccharidosis Disorder.

Cureus 2020 Jun 7;12(6):e8487. Epub 2020 Jun 7.

Pediatrics, Rush University Children's Hospital, Chicago, USA.

Mucopolysaccharidoses (MPS) are rare genetic lysosomal storage disorders caused by a deficiency of enzymes that catalyze the breakdown of glycosaminoglycans. MPS-III, also known as Sanfilippo syndrome, is caused by a deficiency of one of four enzymes that catalyze heparan sulfate proteoglycan degradation. MPS-IIIA results from a deficiency of heparan sulfatase. Read More

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Voriconazole-induced Severe Hyperkalemia Precipitated by Multiple Drug Interactions.

Electrolyte Blood Press 2020 Jun 18;18(1):10-15. Epub 2020 Jun 18.

Department of Internal Medicine, Hanyang University Hospital, Hanyang University College of Medicine, Seoul, Korea.

Voriconazole, a triazole antifungal agent used to treat serious fungal infections, has a pharmacokinetic characteristic of undergoing hepatic metabolism by the cytochrome P450 system. Few cases of hyperkalemia have been reported, which presented only when the serum voriconazole level was exceptionally elevated by drug-drug interactions. Additionally, azole antifungals may interfere with the biosynthesis of adrenal steroids and therefore can predispose patients to aldosterone deficiency. Read More

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Clinical and Genetic Characteristics of Patients with Corticosterone Methyloxidase Deficiency Type 2: Novel Mutations in

J Clin Res Pediatr Endocrinol 2021 06 16;13(2):232-238. Epub 2020 Jun 16.

İstanbul University-Cerrahpaşa, Cerrahpaşa Faculty of Medicine, Department of Pediatric Endocrinology, İstanbul, Turkey

Corticosterone methyloxidase deficiency type 2 is an autosomal recessive disorder presenting with salt loss and failure to thrive in early childhood and is caused by inactivating mutations of the gene. Herein, we describe four Turkish patients from two families who had clinical and hormonal features compatible with corticosterone methyloxidase deficiency and all had inherited novel variants. All of the patients presented with vomiting, failure to thrive and severe dehydration, except one patient with only failure to thrive. Read More

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Mineralocorticoid Dysfunction during Critical Illness: A Review of the Evidence.

Anesthesiology 2020 08;133(2):439-457

From the School of Clinical Medicine, Faculty of Health Sciences, University of the Witwatersrand, Johannesburg, South Africa (G.D.N., C.F.) the Department of Anaesthesia and Perioperative Medicine, Royal Brisbane and Women's Hospital, Queensland, Australia (G.D.N.) the Faculty of Medicine, University of Queensland, Brisbane, Queensland, Australia (G.D.N., J.C., J.L.) The George Institute, Sydney, New South Wales, Australia (J.C.) The Wesley Hospital, Brisbane, Queensland, Australia (J.C.) Intensive Care Services, Royal Brisbane and Women's Hospital, Herston, Brisbane, Queensland, Australia (J.C., J.L.) Nimes University Hospital, University of Montpellier, Nimes, France (J.L.) the Institute for Cellular and Molecular Medicine, Department of Immunology, Faculty of Health Sciences, University of Pretoria, Pretoria, South Africa (R.A.).

The recent demonstration of the significant reduction in mortality in patients with septic shock treated with adjunctive glucocorticoids combined with fludrocortisone and the effectiveness of angiotensin II in treating vasodilatory shock have renewed interest in the role of the mineralocorticoid axis in critical illness. Glucocorticoids have variable interactions at the mineralocorticoid receptor. Similarly, mineralocorticoid receptor-aldosterone interactions differ from mineralocorticoid receptor-glucocorticoid interactions and predicate receptor-ligand interactions that differ with respect to cellular effects. Read More

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