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2.1
Hyporeninemic Hypoaldosteronism
Hyporeninemia Hypoaldosteronism

Presentation

We describe an 11-month-old boy who presented clinically with hyperkalemic renal tubular acidosis due to hyporeninemic hypoaldosteronism. Persistent hyperchloremic acidosis and mild azotemia were present. [ncbi.nlm.nih.gov]

We report the case of a 10-year-old boy with BOR syndrome who presented with hyperkalemic hyperchloremic metabolic acidosis due to hyporeninemic hypoaldosteronism. [sjkdt.org]

Entire Body System

  • Amyloidosis

    Hyporeninemic hypoaldosteronism from secondary amyloidosis. [pubfacts.com]

    (see Amyloidosis, [[Amyloidosis]]) Obstructive Uropathy Post-Acute Tubular Necrosis (ATN) (see Acute Kidney Injury, [[Acute Kidney Injury]]) Sickle Cell Disease (see Sickle Cell Disease, [[Sickle Cell Disease]]) Systemic Lupus Erythematosus (SLE) (see [mdnxs.com]

    A specific subset of AA amyloidosis which is secondary to dialysis is beta-2 microglobulin amyloidosis. [renalfellow.blogspot.com]

    Hypoaldosteronism Hyporeninemic hypoaldosteronism Adrenal insufficiency Adrenocorticotropic hormone deficiency Primary hyporeninemia Primary renal tubular defects Sickle cell disease Systemic lupus erythematosus Obstructive uropathy Hereditary tubular defects Amyloidosis [aafp.org]

  • Intravenous Administration

    Twenty children with chronic renal failure never had hyperkalemia, and both renin and aldosterone were normally stimulated by intravenous administration of furosemide, whereas three patients had moderate hyperkalemia (serum potassium concentration between [ncbi.nlm.nih.gov]

    Rapid correction is possible with oral potassium; the fastest results are likely best achieved by combining oral (e.g., 20 to 40 mmol) and intravenous administration. 22 When intravenous potassium is used, standard administration is 20 to 40 mmol of potassium [aafp.org]

Cardiovascular

  • Orthostatic Hypotension

    Disease relevance of Hypoaldosteronism Plasma renin activity (PRA) and aldosterone are decreased in diabetic with nephropathy and hypertension, in those with neuropathy including orthostatic hypotension, and in those with hypoaldosteronism [1]. [wikigenes.org]

  • Tachycardia

    Arrhythmias associated with hypokalemia include sinus bradycardia, ventricular tachycardia or fibrillation, and torsade de pointes. 19 Although the risk of ECG changes and arrhythmias increases as serum potassium concentration decreases, these findings [aafp.org]

Musculoskeletal

  • Muscle Weakness

    Liddle's syndrome was diagnosed in a 72-year-old man who presented clinically with hypertension and muscle weakness. [ncbi.nlm.nih.gov]

    Overview Hyporeninemic hypoaldosteronism: A rare condition where low levels of renin result in insufficient aldosterone being produced Symptoms - Hyporeninemic hypoaldosteronism Hyperkalemia Heart arrhythmia Muscle weakness Low plasma rennin activity [checkorphan.org]

    Except for arrhythmia and muscle weakness in severe cases, hyperkalemia produces no physical signs. Mild acidosis may be present, but associated physical signs (eg, Kussmaul respiration) usually are absent. [emedicine.medscape.com]

    Symptoms include Weight loss Muscle weakness Fatigue that gets worse over time Low blood pressure Patchy or dark skin Lab tests can confirm that you have Addison disease. If you don't treat it, it can be fatal. [icdlist.com]

    Upon further questioning, the patient's mother states he has episodes of constipation and muscle weakness. Physical examination is notable for growth failure. [medbullets.com]

Neurologic

  • Paresthesia

    HISTORY AND PHYSICAL EXAMINATION Severe hyperkalemia (more than 6.5 mEq per L [6.5 mmol per L]) can cause muscle weakness, ascending paralysis, heart palpitations, and paresthesias. [aafp.org]

Urogenital

  • Kidney Failure

    Some of the potential causes of Hypoaldosteronism are: Defects in the adrenal gland Side-effects of medications used for controlling blood pressure (certain NSAIDs, ACE inhibitors and diuretics) Kidney failure Acute kidney ailments Presence of chronic [hxbenefit.com]

    RENAL FAILURE CHRONIC RENAL FAILURE (CRF) Treatment Factors aggravating or producing kidney failure (eg, Na and water depletion, nephrotoxins, heart failure, infection, hypercalcemia, obstruction) must be treated specifically. [163.178.103.176]

    Chronic kidney disease, diabetes, heart failure, and liver disease all increase the risk of hyperkalemia. [aafp.org]

Workup

If the patient is presenting for the first time, order a complete workup for the underlying renal disease. [emedicine.medscape.com]

Primary Aldosteronism Workup. Medscape. Available online at http://emedicine.medscape.com/article/127080-workup#aw2aab6b5b9. Accessed Jan. 28, 2012. Bartter syndrome. MedlinePlus Medical Encyclopedia. [labtestsonline.it]

[…] more practical approach is calculation of the urine potassium-to-creatinine ratio from a spot urine specimen; a ratio greater than 1.5 mEq per mmol (13 mEq per g) is indicative of renal potassium wasting. 18 If no cause is identified with the initial workup [aafp.org]

X-Ray

  • Nephrolithiasis

    It has been shown to precipitate and result in nephrolithiasis in some patients, and therefore these individuals are instructed to increase their water intake. [renalfellow.blogspot.com]

Serum

  • Thrombocytosis

    Other causes included overzealous potassium replacement, spironolactone therapy, hemolysis, acute renal failure, acidosis, thrombocytosis, and Addison's disease. [ncbi.nlm.nih.gov]

    Diagnosis First, exclude pseudohyperkalemia, which is seen with difficult venipunctures and in thrombocytosis. Repeat the serum potassium determination to confirm, with a better venipuncture if possible. [emedicine.medscape.com]

    For patients with extreme leukocytosis or thrombocytosis, potassium levels should be measured in plasma samples that have been promptly separated from the cellular components since extreme elevations in either leukocytes or platelets results in leakage [rrnursingschool.biz]

    […] potassium Certain forms of pica Pseudohyperkalemia Hemolysis Tourniquet use Fist clenching Blood sample cooling Intravenous fluids with potassium Cell hyperplasia Significant leukocytosis (> 75,000 cells per mm 3 [75.0 × 10 9 per L]) Erythrocytosis Thrombocytosis [aafp.org]

  • Abnormal Renal Function

    Despite only mildly abnormal renal function, serum K+ was elevated to 6.2 meq/L, and plasma renin activity (0.12 ng/mL h) and aldosterone (4.4 ng/dL) failed to respond to the combined stimuli of furosemide and posture. [ncbi.nlm.nih.gov]

    Despite only mildly abnormal renal function, serum K + was elevated to 6.2 meq/L, and plasma renin activity (0.12 ng/mL h) and aldosterone (4.4 ng/ dL) failed to respond to the combined stimuli of furosemide and posture. [annals.org]

Treatment

Treatment led to improvement of impairment of water diuresis due to hypersecretion of ADH. Hyporeninemic hypoaldosteronism persisted after treatment. [ncbi.nlm.nih.gov]

This article reviews some of the pathophysiologic aspects, the clinical picture, and the treatment strategies of hyporeninemic hypoaldosteronism from the standpoint of clinical presentation, evaluation, and treatment. [emedicine.medscape.com]

Conclusion: When persistent hyperkalemia is detected, mineralocorticoid replacement treatment, with a low-potassium and high-sodium diet should be initiated. [endocrine-abstracts.org]

We performed the hypertonic saline infusion test before and after the treatment. [jstage.jst.go.jp]

Prognosis

Prognosis - Hyporeninemic hypoaldosteronism Not supplied. Treatment - Hyporeninemic hypoaldosteronism Not supplied. Resources - Hyporeninemic hypoaldosteronism Not supplied. [checkorphan.org]

Overview Historical Perspective Classification Pathophysiology Causes Differentiating Hypoaldosteronism from other Diseases Epidemiology and Demographics Risk Factors Screening Natural History, Complications and Prognosis Diagnosis History and Symptoms [wikidoc.org]

Hyperaldosteronism Prognosis With early diagnosis and cure, the prognosis for Primary Hyperaldosteronism is found to be quite good. In case of Secondary Hyperaldosteronism, the outcome depends on the underlying cause of the disease. [hxbenefit.com]

Prognosis. Medscape. Available online at http://emedicine.medscape.com/article/238670-overview#aw2aab6b2b5aa. Accessed February 2014. Bartter syndrome: Overview. Office of Rare Diseases Research. [labtestsonline.it]

[…] filtration rate may also contribute to the development of hyperkalemia Intrinsic Renal Disease Acute Glomerulonephritis with Volume Expansion (see Acute Glomerulonephritis, [[Acute Glomerulonephritis]]) Treatment: responds to mineralocorticoid replacement Prognosis [mdnxs.com]

Etiology

Our results appear most consistent with the concept that the primary etiological factor in the HH syndrome is impairment of renal activation of renin. [ncbi.nlm.nih.gov]

(See "Etiology and treatment of hypoaldosteronism (type 4 RTA)", section on Congenital adrenal hyperplasia and isolated hypoaldosteronism, which reviews the biosynthetic defects in this disorder). [enotes.tripod.com]

Etiology Decreased Aldosterone Synthesis Inherited Disorders Congenital Isolated Hypoaldosteronism 21 Hydroxylase Deficiency Other Defects Pseudohypoaldosteronism Type 2 (Gordon’s Syndrome) Physiology : defects in WKNK1 or WNK4 kinases Clinical Familial [mdnxs.com]

[…] as with ongoing diuretic therapy or hyperaldosteronism, 20 mmol per day is usually sufficient. 15 Causes of Hyperkalemia Hyperkalemia is caused by excess potassium intake, impaired potassium excretion, or transcellular shifts ( Table 2 ). 8, 24 The etiology [aafp.org]

Epidemiology

[…] collaborate analysis of diabetic criteria in Asia DECODA研究 Diabetes epidemiology collaborate analysis of diabetic criteria in Europe DECODE研究 Diabetes epidemiology research international DERI研究 Diabetes prevention program DPP試験 Diabetes prevention trial [jds.or.jp]

Overview Historical Perspective Classification Pathophysiology Causes Differentiating Hypoaldosteronism from other Diseases Epidemiology and Demographics Risk Factors Screening Natural History, Complications and Prognosis Diagnosis History and Symptoms [wikidoc.org]

C CHU Besançon, serv. médecine interne-gériatrie Source Médecine et hygiène. 1984, Vol 42, Num 1564, pp 1664-1669 ; ref : 26 ref CODEN MEHGAB ISSN 0025-6749 Scientific domain Hygiene and public health, epidemiology, occupational medicine; General medicine [pascal-francis.inist.fr]

[…] typically within 1-2 wks) leads to resolution of hyperkalemia Chronic Kidney Disease (CKD) (see Chronic Kidney Disease, [[Chronic Kidney Disease]]): with chronic interstitial nephritis Diabetic Nephropathy (see Diabetes Mellitus, [[Diabetes Mellitus]]) Epidemiology [mdnxs.com]

Pathophysiology

This edition has been thoroughly revised by world-renowned contributors to reflect recent developments in renal pathophysiology. [books.google.com]

The total has now risen to more than 100, including 7 reported in this issue. 2 The first breakthrough in unraveling the pathophysiologic nature of. .. Gordon H. Williams, M.D. Brigham and Women's Hospital, Boston, MA 02115 [nejm.org]

This article reviews some of the pathophysiologic aspects, the clinical picture, and the treatment strategies of hyporeninemic hypoaldosteronism from the standpoint of clinical presentation, evaluation, and treatment. [emedicine.medscape.com]

Prevention

Urinary ammonium excretion correlated inversely with serum potassium concentration and did not decrease on discontinuation of therapy if hyperkalemia was prevented from recurring. [ncbi.nlm.nih.gov]

Rapidly find the answers you need with separate sections on diseases and disorders, differential diagnosis, clinical algorithms, laboratory results, and clinical preventive services, plus an at-a-glance format that uses cross-references, outlines, bullets [books.google.com]

| Secondary Prevention | Cost-Effectiveness of Therapy | Future or Investigational Therapies Case Studies Case #1 Adrenal gland Pseudohypoaldosteronism Hyperaldosteronism Addison's disease References v t e Endocrine pathology : endocrine diseases ( E00 [wikidoc.org]

[…] program DPP試験 Diabetes prevention trial-1 DPT-1試験 Diabetes prevention trial-Type 1 DPT-1試験 Diabetic nephropathy remission and regression team trial in Japan DNETT-Japan Diabetic retinopathy candesartan trials DIRECT試験 Duloxetine デュロキセチン Dupuytren contracture [jds.or.jp]

Prevention - Hyporeninemic hypoaldosteronism Not supplied. Diagnosis - Hyporeninemic hypoaldosteronism Not supplied. Prognosis - Hyporeninemic hypoaldosteronism Not supplied. Treatment - Hyporeninemic hypoaldosteronism Not supplied. [checkorphan.org]

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