Question 1 of 10

    Nephrocalcinosis (Nephrocalcinoses)

    Nephrocalcinosis refers to abnormal deposition of calcium, oxalate crystals and phosphate on the kidney. Accumulation of these compounds can impair normal kidney function and lead to chronic kidney disease. Causes include any metabolic or intrinsic factors and illnesses interfering with normal calcium metabolism. Diagnosis is confirmed through radiographic and laboratory tests and treatment depends on the underlying cause.

    Nephrocalcinosis stems from this process: biochemical.

    Presentation

    The initial stages of nephrocalcinosis are asymptomatic, and patients may not report any complaints. When the disease is established, however, symptoms may appear, including polyuria, nocturia, hematuria, dysuria, and urinary incontinence [10]. Other symptoms, such as back pain or flank pain, either unilateral or bilateral, may appear, as well as nausea, vomiting, generalized edema and altered mental status (drowsiness, lethargy or altered consciousness) [11]. Other symptoms may accompany this condition, depending on the cause, and are related to elevated levels of serum calcium.

    urogenital
    Polyuria
    • Clinical features of hypercalcemic nephropathy : Relative vasopressin resistance with decreased renal concentrating ability and increased free water diuresis, manifesting as polyuria and polydipsia, Renal glycosuria, reduced glucose tubular maximum, aminoaciduria[omicsonline.org]
    • These include renal colic , polyuria and polydipsia : [4] Renal colic is usually caused by pre-existing nephrolithiasis , as may occur in patients with chronic hypercalciuria . [4] Less commonly, it can result from calcified bodies moving into the calyceal[en.wikipedia.org]
    • Some nephocalcinosis symptoms that might occur are: dysuria nocturia polyuria flank pain hematuria urine retention swelling , edema kidney infection frequent urination urine incontinence Most of these symptoms can be caused by other kidney problems too[healthblurbs.com]
    • Symptoms associated with hypercalcemia include polydipsia (excessive thirst), polyuria (excessive urination), nausea, and vomiting.[news-medical.net]
    • Urinary tract infection Passage of urinary stones Macroscopic nephrocalcinosis should not be considered synonymous with urinary stones because it signifies a metabolic derangement and has broader implications Episodes of urinary tract infections may occur Polyuria[learningradiology.com]
    Nocturia
    • Some nephocalcinosis symptoms that might occur are: dysuria nocturia polyuria flank pain hematuria urine retention swelling , edema kidney infection frequent urination urine incontinence Most of these symptoms can be caused by other kidney problems too[healthblurbs.com]
    • […] colic , polyuria and polydipsia : [4] Renal colic is usually caused by pre-existing nephrolithiasis , as may occur in patients with chronic hypercalciuria . [4] Less commonly, it can result from calcified bodies moving into the calyceal system. [4] Nocturia[en.wikipedia.org]
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  • Entire body system
    Asymptomatic
    • Determined by the underlying etiology, though in many cases, the condition remains asymptomatic and is identified only as a radiologic abnormality.[omicsonline.org]
    • The vast majority of these patients are asymptomatic.[sjkdt.org]
    • This disease may be asymptomatic or may be characterized by repeated renal colics, calculous disease, hematuria or repeated urinary tract infections.[sonoworld.com]
    • Symptoms of Nephrocalcinosis The list of signs and symptoms mentioned in various sources for Nephrocalcinosis includes the 13 symptoms listed below: Symptoms similar to symptoms of nephritis Calcium deposits in the kidney Impaired kidney function Asymptomatic[rightdiagnosis.com]
    • Range of symptoms: asymptomatic to vague, nonspecific findings or symptoms related to underlying cause of hypercalcemia Descriptive types of NC based on radiographic location ( 2 ): Medullary type: most commonly related to metabolic disorders; the most[unboundmedicine.com]
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  • Workup

    In order to establish the diagnosis of nephrocalcinosis, and its underlying cause, a detailed workup is necessary, and includes:

    • Patient history and physical examination - before obtaining blood samples for testing, it is imperative to obtain a good patient history and perform a thorough physical examination. Vital information may be obtained from the patient, which can guide the physician towards the diagnosis.
    • Biochemical analysis - electrolyte levels including serum levels of calcium, phosphate, sodium, potassium and magnesium, as well as urine levels of calcium, sodium and uric acid, should be measured. Additionally, the hormonal status should be evaluated, primarily focusing on the thyroid and parathyroid glands (TSH, free T3, T4, PTH)
    • Radiography - An abdominal ultrasound (US) may be performed to obtain a view of the kidneys. An abdominal Computerized Tomography (CT) scan may be useful as well, to gain an insight into the surrounding structures.

    Laboratory

    Urine
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  • Serum
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  • Imaging

    X-ray
    Nephrolithiasis
    • Box 59-2 The basic evaluation of nephrolithiasis.[abdominalkey.com]
    • "Recent advances in managing and understanding nephrolithiasis/nephrocalcinosis" .[en.wikipedia.org]
    • Recent observation suggests that nephrocalcinosis and calcium nephrolithiasis are to be considered independent pathologies, and that nephrocalcinosis may cause calcium nephrolithiasis only in certain conditions 2 .[ramb.elsevier.es]
    • Both nephrocalcinosis and nephrolithiasis may occur.[mypacs.net]
    • Madore F, Stampfer MJ, Rimm EB, et al. : Nephrolithiasis and risk of hypertension.[f1000research.com]
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  • Treatment

    Treatment of nephrocalcinosis is targeted at the underlying cause. Rarely is elevated serum calcium treated solely separately, without identifying the cause. Treatment principles range from supportive and symptomatic therapy to surgical procedures.

    Correction of elevated calcium levels in the serum can be achieved by oral intake of phosphates (for mild imbalance), by intravenous infusion of saline combined with furosemide (severe imbalance), and administration of Ca-lowering drugs, such as bisphosphonates. As a last resort, hemodialysis is indicated if Ca levels are very high (> 18 mg/dl) and other options are not effective. Corticosteroids may be useful in reducing serum calcium levels, as they reduce vitamin D activity and slightly decrease intestinal calcium absorption. Correction of other electrolytes, including magnesium and potassium, is sometimes necessary, in order to restore normal serum values.

    Apart from correcting hypercalcemia with medication, treatment of the underlying cause is imperative in order to permanently resolve nephrocalcinosis. For the correction of possible hyperparathyroidism, drugs such as cinacalcet may be used. In more aggressive forms of hyperparathyroidism, surgical removal may be indicated. If the presence of tumors of the skeletal system is confirmed, appropriate therapeutic measures must be implemented, whether it is surgery, chemotherapy or radiation therapy.

    Prognosis

    The prognosis depends on the etiology of nephrocalcinosis. In general, the prognosis is good if the underlying cause is recognized early and treated properly, with minimal or no residual damage to the kidneys. However, if nephrocalcinosis, and its underlying cause goes unrecognized and untreated (or if there is no cure for the diagnosis), renal failure may develop, which then has limited options for recovery.

    Complications

    Interstitial Nephritis
    • Nine patients underwent a renal biopsy, and all had interstitial nephritis [ 7 ].[academic.oup.com]
    • nephritis Polycystic kidney disease Papillary necrosis Medullary sponge kidney Coagulopathy Miscellaneous (loin pain hematuria syndrome, avm's, chemical cystitis) Basic Evaluation of Stone Formers (1st stone) Stone history Medical history Diseases leading[quizlet.com]
    • nephritis Polycystic kidney disease Papillary necrosis Medullary sponge kidney Coagulopathy: bleeding disorders, anticoagulation therapy Miscellaneous: loin pain–hematuria syndrome, arteriovenous malformation, chemical cystitis, caruncle, factitious[abdominalkey.com]
    Nephrocalcinosis
    • A high urine pH can lead to Nephrocalcinosis.[en.wikipedia.org]
    • : Nephrocalcinosis Neonatal kidney and nephrocalcinosis: Nephrocalcinosis with increased echogenicity in the medullary portion of both kidneys Neonatal kidney and nephrocalcinosis: Nephrocalcinosis in a patient with Wolf Hirschorn syndrome[ultrasoundcases.info]
    • The most frequent form of nephrocalcinosis is medullary nephrocalcinosis.[news-medical.net]
    Acute Pyelonephritis
    • Clinical features of microscopic nephrocalcinosis include Reduced concentration capacity, Increased blood urea nitrogen (BUN), Prolongation of nephron transit time in the distal tubule, Acute pyelonephritis or calculous ureteral obstruction with renal[omicsonline.org]
    • pyelonephritis Renal colic Blood in urine more information...»[rightdiagnosis.com]
    • Causes of Hematuria Nephrolithiasis Infection: cystitis, prostatitis, urethritis, acute pyelonephritis, tuberculosis, schistosomiasis Malignancy: renal cell carcinoma, transitional cell carcinoma, prostatic carcinoma, Wilms tumor Trauma Glomerular disease[abdominalkey.com]
    Hypercalcemia
    • […] and hypercalciuria) Milk-alkali syndrome Hypervitaminosis D Multiple myeloma Hypercalciuria without hypercalcemia [ edit ] These conditions can cause nephrocalcinosis in association with hypercalciuria without hypercalcemia: Distal renal tubular acidosis[en.wikipedia.org]
    • Humoral hypercalcemia is the most common mechanism.[kidneypathology.com]
    • Nephrocalcinosis is related more to the duration than to the intensity of hypercalcemia.[emedicine.medscape.com]
    • These include the several causes of hypercalcemia and hypercalciuria.[mypacs.net]
    • More closely related to the duration of hypercalcemia rather than the severity.[slideshare.net]
    Nephrolithiasis
    • Box 59-2 The basic evaluation of nephrolithiasis.[abdominalkey.com]
    • "Recent advances in managing and understanding nephrolithiasis/nephrocalcinosis" .[en.wikipedia.org]
    • Recent observation suggests that nephrocalcinosis and calcium nephrolithiasis are to be considered independent pathologies, and that nephrocalcinosis may cause calcium nephrolithiasis only in certain conditions 2 .[ramb.elsevier.es]
    • Both nephrocalcinosis and nephrolithiasis may occur.[mypacs.net]
    • Madore F, Stampfer MJ, Rimm EB, et al. : Nephrolithiasis and risk of hypertension.[f1000research.com]
    Sarcoidosis
    • Distal RTA, Sarcoidosis and Milk Alkali syndrome are established causes for Nephrocalcinosis.[medicoapps.org]
    • Gouty Arthritis Dactylitis nefrocalcinozele osteofit Sarcoidosis Dactylitis Nephrocalcinosis osteofit Joints It tends to present as an inflammatory arthritis (often oligoarticular initially) with periarticular soft-tissue swelling, tenosynovitis, dactylitis[symptoma.com]
    • Medullary Nephrocalcinosis Hyperparathyroidism Renal tubular acidosis Medullary sponge kidney - favored if unilateral also Hypercalcemia / hypercaliuria Sarcoidosis Bony metastases Paget's disease Hypervitaminosis D Milk-alkali syndrome Williams syndrome[radipedia.com]
    • Causes include: malignancy (commonest cause) multiple myeloma hyperparathyroidism sarcoidosis vitamin D intoxication Investigations: blood tests - hyercalcaemia, increased creatinine urine tests - haematuria, proteinuria, pyuria, hypercalciuria abdominal[gpnotebook.co.uk]
    • Toxoplasmosis Hyperglobulinemia nefrocalcinozele osteofit Diabetes Mellitus Hyperglobulinemia nefrocalcinozele osteofit Infectious Mononucleosis Hyperglobulinemia nefrocalcinozele osteofit Subcutaneous Abscess Hyperglobulinemia nefrocalcinozele osteofit Sarcoidosis[symptoma.com]
    Medullary Sponge Kidney
    • It is most commonly seen as an incidental finding with medullary sponge kidney on an abdominal x-ray.[en.wikipedia.org]
    • Medullary sponge kidney is a developmental anomaly characterized by cystic dilatation of the collecting tubules in one or more renal medullary pyramids.[sonoworld.com]
    • […] hemihypertrophy of the body may exist Nephrocalcinosis associated with distal RTA and medullary sponge kidney usually is gross and renal function is relatively well preserved Renal papillary necrosis associated with phenacetin-induced analgesic nephropathy[learningradiology.com]
    • The disorder may be symmetric or, in anatomic disorders such as medullary sponge kidney, involve only a single kidney.[medicinenet.com]
    • Differential Dx Medullary sponge kidney and nephrolithiasis.[sites.google.com]
    Wilson Disease
    • disease; hyperglobulinemias ; and drug toxicity from amphotericin, outdated tetracycline, triamterene and acetazolamide.[symptoma.com]
    • Disease processes that cause secondary RTA by impairing hydrogen ion excretion by the distal tubule include Fanconi syndrome, Wilson disease, and amphotericin B toxicity.[radiologykey.com]
    • These include hypercalcemia; Wilson disease; hyperglobulinemias; and drug toxicity from amphotericin, outdated tetracycline, triamterene and acetazolamide.[mypacs.net]
    • Arch Dis Child 69:299–303 PubMed Google Scholar Hoppe B, Neuhaus T, Superti A, Leumann E (1993b) Hypercalciuria and nephrocalcinosis, a feature of Wilson’s disease.[link.springer.com]
    • […] antigen D2 Bartter syndrome type 5 XLR CASR Calcium‐sensing receptor (CasR) Hypercalciuria with hypocalcemia AD G6PC Glucose‐6‐phosphatase Glycogen storage disease type 1a AR FAH Fumarylacetoacetase Tyrosinemia type 1 AR ATP7B Copper‐transporting ATP‐ase Wilson[intechopen.com]
    Williams Syndrome
    • Medullary Nephrocalcinosis Hyperparathyroidism Renal tubular acidosis Medullary sponge kidney - favored if unilateral also Hypercalcemia / hypercaliuria Sarcoidosis Bony metastases Paget's disease Hypervitaminosis D Milk-alkali syndrome Williams syndrome[radipedia.com]
    • syndrome Congenital hypothyroidism Nephrocalcinosis Treatment/Prevention Increase fluid to 2L/day Low protein diet Low salt diet Potassium Citrate if pH[quizlet.com]
    • N Engl J Med 327:1141–1152 PubMed Google Scholar Cote G, Jéquier S, Kaplan P (1989) Increased renal medullary echogenicity in patients with Williams syndrome.[link.springer.com]
    Renal Tubular Acidosis
    • Renal tubular acidosis: the clinical entity.[ramb.elsevier.es]
    • General Prevention Prevention of preterm birth Commonly Associated Conditions Hypercalcemia Hypercalciuria Renal tubular acidosis Hyperoxaluria Urinary stasis Hyperparathyroidism Treatment of hypoparathyroidism Medullary sponge kidney Osteoporosis --[unboundmedicine.com]
    • However, it may be severe enough to cause (as well as be caused by) renal tubular acidosis or even end stage renal failure , due to disruption of the renal tissue by the deposited calcium.[en.wikipedia.org]
    • Found At: Elsevier Version: Author's manuscript Abstract: Purpose Nephrocalcinosis is commonly present in primary hyperparathyroidism, distal renal tubular acidosis and medullary sponge kidney disease.[scholarworks.iupui.edu]
    Milk-Alkali Syndrome
    • Distal RTA, Sarcoidosis and Milk Alkali syndrome are established causes for Nephrocalcinosis.[medicoapps.org]
    • Medullary Nephrocalcinosis Hyperparathyroidism Renal tubular acidosis Medullary sponge kidney - favored if unilateral also Hypercalcemia / hypercaliuria Sarcoidosis Bony metastases Paget's disease Hypervitaminosis D Milk-alkali syndrome Williams syndrome[radipedia.com]
    • Pathology Causes include 1-2 : medulla hyperparathyroidism medullary sponge kidney renal tubular acidosis (type 1) hypervitaminosis D milk-alkali syndrome sarcoidosis other pathological hypercalcemic or hypercalciuric states Cushing syndrome multiple[radiopaedia.org]
    • alkali syndrome, oral sodium phosphate as bowel preparation ( Hum Pathol 2004;35:675 ), sarcoidosis, vitamin D intoxication Treatment Reduce urinary saturation of calcium by increasing fluid intake, crystallization inhibitors, disease-specific medication[pathologyoutlines.com]
    • This has been termed the milk-alkali syndrome .[radiologykey.com]
    Hypoparathyroidism
    • A few potential conditions in this class include hyperparathyroidism (this is when the parathyroid gland is producing too much parathyroid hormone, which increases calcium levels in the blood), and conversely hypoparathyroidism can also cause this condition[zocdoc.com]
    • General Prevention Prevention of preterm birth Commonly Associated Conditions Hypercalcemia Hypercalciuria Renal tubular acidosis Hyperoxaluria Urinary stasis Hyperparathyroidism Treatment of hypoparathyroidism Medullary sponge kidney Osteoporosis --[unboundmedicine.com]
    • […] incidence Contributing mechanisms are hypercalcemia, acidosis, and reduced excretion of citrate in the presence of increased urinary pH Renal function is fairly well maintained Other causes of nephrocalcinosis are Hypervitaminosis D due to treatment of hypoparathyroidism[learningradiology.com]
    • Other causes of nephrocalcinosis are hypervitaminosis-D states [ 11 ] resulting from excessive treatment of hypoparathyroidism, self-administration of vitamins, and the presence of a granulomatous disease, such as sarcoidosis. [12] In granulomatous disorders[emedicine.medscape.com]
    • Ultrasound: more sensitive than conventional radiography and can be superior to CT scan in some clinical scenarios (eg, nephrocalcinosis associated with hypoparathyroidism). [ 5 ] CT scan: more effective in detecting calcification and can be used to differentiate[patient.info]
    Isosthenuria
    • The patient also suffered from mild nephrogenic diabetes insipidus with isosthenuria and 3 to 4 L of urine per day.[pubmedcentralcanada.ca]
    Hypervitaminosis D
    • Medullary Nephrocalcinosis Hyperparathyroidism Renal tubular acidosis Medullary sponge kidney - favored if unilateral also Hypercalcemia / hypercaliuria Sarcoidosis Bony metastases Paget's disease Hypervitaminosis D Milk-alkali syndrome Williams syndrome[radipedia.com]
    • Pathology Causes include 1-2 : medulla hyperparathyroidism medullary sponge kidney renal tubular acidosis (type 1) hypervitaminosis D milk-alkali syndrome sarcoidosis other pathological hypercalcemic or hypercalciuric states Cushing syndrome multiple[radiopaedia.org]
    • Hypervitaminosis D Excessive vitamin D ingestion (hypervitaminosis D) facilitates increased absorption of calcium from the gut and may cause nephrocalcinosis.[mypacs.net]
    • A few other rare causes of medullary nephrocalcinosis include sarcoidosis, hypervitaminosis D (too much vitamin D ingestion), renal tubular acidosis (a group of kidney disorders that alter the absorption and secretion of certain compounds in the kidney[zocdoc.com]
    • D due to treatment of hypoparathyroidism or self-administration of vitamins Milk-alkali syndrome due to ingestion of milk or alkali for ulcer dyspepsia Sarcoidosis due to increased conversion of 25-hydroxycholecalciferol to 1,25-dihydroxycholecalciferol[learningradiology.com]

    Etiology

    Disorders that interfere with calcium metabolism and lead to renal calcification are numerous [3] [4]:

    Because of the nature of the disease, nephrocalcinosis usually occurs bilaterally.

    It is important to distinguish nephrocalcinosis from nephrolithiasis (formation of kidney stones). The majority of Ca oxalate stones develop from the abnormally deposited Ca in the interstitium, which makes nephrocalcinosis one of the initial steps in the development of kidney stones.

    Causes

    Wilson Disease
    • disease; hyperglobulinemias ; and drug toxicity from amphotericin, outdated tetracycline, triamterene and acetazolamide.[symptoma.com]
    • Disease processes that cause secondary RTA by impairing hydrogen ion excretion by the distal tubule include Fanconi syndrome, Wilson disease, and amphotericin B toxicity.[radiologykey.com]
    • These include hypercalcemia; Wilson disease; hyperglobulinemias; and drug toxicity from amphotericin, outdated tetracycline, triamterene and acetazolamide.[mypacs.net]
    • Arch Dis Child 69:299–303 PubMed Google Scholar Hoppe B, Neuhaus T, Superti A, Leumann E (1993b) Hypercalciuria and nephrocalcinosis, a feature of Wilson’s disease.[link.springer.com]
    • […] antigen D2 Bartter syndrome type 5 XLR CASR Calcium‐sensing receptor (CasR) Hypercalciuria with hypocalcemia AD G6PC Glucose‐6‐phosphatase Glycogen storage disease type 1a AR FAH Fumarylacetoacetase Tyrosinemia type 1 AR ATP7B Copper‐transporting ATP‐ase Wilson[intechopen.com]

    Epidemiology

    Nephrocalcinosis is found in the majority of patients with kidney stones and in up to 100% of patients with calcium phosphate stones [6]. Given the fact that 1/1000 adults in the USA is hospitalized annually because of renal calculi [7], and that up to 12% of men and 5% of women develop a urinary calculus by age 70, it can be concluded that nephrocalcinosis is common among patients with kidney stones. This disorder can be observed in any individual regardless of age and gender, but adults, as well as those who have predisposing conditions which lead to hypercalcemia and hypercalciuria, are patients who most frequently develop the condition.

    Sex distribution
    Age distribution

    Pathophysiology

    The physiological mechanism of calcium and phosphate homeostasis is quite complex, but under normal circumstances, the kidneys play a vital role in the regulation of serum calcium levels. The tubular system has a role of absorbing and secreting calcium, phosphate and oxalate, among many other substances, under the influence of parathyroid hormone (PTH), vitamin D and calcitonin.

    Normally, tiny depositions of calcium are formed in the kidney intersititum, but they break loose and travel along with urine. However, when hypercalcemia occurs, it poses a threat to the kidneys and the tubular system. Among other mechanisms which regulate serum calcium concentrations, the kidneys compensate by increasing the excretion of Ca ions, resulting in hypercalciuria. However, increased levels of Ca in urine lead to its accumulation along the renal system and calcification of the renal interstitium can frequently occur, leading to nephrocalcinosis. 

    Elevated serum Ca has several effects on the kidneys. Renal vasoconstriction is induced by elevated serum calcium, which reduces the glomerular filtration rate (GFR) and overall kidney function. In addition, hypercalcemia interferes with effects of vassopressin, or antidiuretic hormone (ADH), presumably through calcium-sensing receptors or through medullary synthesis of prostaglandins [8]. All of these factors contribute to an overall decrease of kidney function, which may lead to chronic kidney disease and even renal failure.

    Because of the straining of the kidneys in the attempt to regulate serum levels of Ca, other metabolic disturbances may occur. In the case of hyperparathyroidism, hydrogen ion excretion is reduced, leading to tubular acidosis. Also, sodium and magnesium excretion is increased, leading to hyponatremia and hypomagnesemia [9], which initiates other forms of compensation for sodium loss, mainly through ADH.

    All of these alterations have a negative effect on the kidneys and the tubular system. If nephrocalcinosis is not resolved, it leads to a chronic state and numerous consequences may arise, the most detrimental being life-threatening and irreversible kidney failure.

    Prevention

    Prevention of nephrocalcinosis primarily focuses on identifying the signs and symptoms of the disease in its early stages, although the disease may initially have an asymptomatic course. However, regular check-ups should include serum electrolyte levels, especially for patients with nephrolithiasis and other diseases of the kidney, because progressed stages of kidney failure are practically impossible to treat. Identifying the cause early is the key to preventing nephrocalcinosis from causing chronic kidney disease.

    Summary

    Nephrocalcinosis is a disease characterized by abnormal deposition of calcium (mainly in the forms of calcium-phosphate and calcium-oxalate) in the kidney interstitium, which can result in impaired kidney function [1]. It is caused by numerous conditions which interfere with the normal metabolism of calcium, including autoimmune, metabolic and congenital diseases, but also infections, certain drugs and chronic diseases of the kidney. Under physiological conditions, calcium deposits grow in the interstitium of the kidney and spontaneously break loose, excreted with urine. Conditions that lead to higher concentrations of calcium and other salts, result in calcifications of the surrounding structures and decreased functional capacity of the kidney, thus rendering patients prone to nephrolithiasis and, potentially, kidney failure [2]. Workup involves the detection of the underlying cause of abnormal calcium deposition and includes radiographic, biochemical and microbiological investigations. Treatment is directed at the underlying cause and prognosis depends on the cause of this condition. Nephrocalcinosis may lead to chronic kidney disease and renal failure if not treated early.

    Patient Information

    Nephrocalcinosis refers to the abnormal accumulation of calcium and its salts in the kidneys. Normally, the kidneys constantly excrete some amounts of calcium, which sometimes "sticks" onto the system of the kidney and under normal circumstances breaks loose and travels into the urinary bladder, where it is then excreted. However, when calcium levels are higher in the blood (and consequently in urine) than usual, larger amounts of calcium can be deposited and can impair normal kidney function.

    This condition can occur as a result of various causes, including hyperparathyroidism, renal tuberculosis, chronic kidney disease, tumors of the skeletal system, but also due to inherited conditions, such as Alport syndrome and any other condition that disturbs the normal metabolism and distribution of calcium in the body.

    Nephrocalcinosis usually occurs in adults, but also in younger patients who have familial conditions which impair the metabolism of calcium. Symptoms may be absent in the initial stages of the disease, but later on, symptoms such as increased urine volume, urinary incontinence, frequent and painful urination, a need to urinate at night and blood in the urine may be observed and reported by patients. Additional symptoms such as back pain or flank pain may also be reported.

    The diagnosis of nephrocalcinosis is achieved through patient history and physical examination and evaluation of serum electrolyte levels, mainly calcium, phosphate, other electrolytes and uric acid, to confirm the presence of elevated calcium in the blood, as well as other electrolyte disturbances. Assessment of renal function, as well as hormone levels, especially of the thyroid and parathyroid glands, may be useful in determining the cause. The prognosis of this disorder depends on a timely diagnosis and its early identification, since it may lead to chronic kidney disease and cause potentially debilitating and life-threatening kidney failure, if left untreated.

    Treatment of nephrocalcinosis depends on the underlying cause, since calcium-correcting and adjunctive therapy may only temporarily resolve the symptoms. Prevention is the key, because when it is diagnosed early, minimal or no damage to the kidneys may occur, while latter stages of the disease may cause severe complaints and greatly impair the quality of life.

    Other symptoms

    Hyperparathyroidism
    • Read more about the symptoms of hyperparathyroidism .[blog.parathyroid.com]
    • "Primary hyperparathyroidism: is there an increased prevalence of renal stone disease?"[en.wikipedia.org]
    • General Prevention Prevention of preterm birth Commonly Associated Conditions Hypercalcemia Hypercalciuria Renal tubular acidosis Hyperoxaluria Urinary stasis Hyperparathyroidism Treatment of hypoparathyroidism Medullary sponge kidney Osteoporosis --[unboundmedicine.com]
    • Hyperparathyroidism for example, when severe.[kidneystones.uchicago.edu]
    • Causes of medullary nephrocalcinosis Hyperparathyroidism Hyperparathyroidism results in renal lithiasis in 20% of patients, with radiographically detectable nephrocalcinosis in as many as 11% of patients.[mypacs.net]
    Glycogen Storage Disease
    • II 10 Glycogen Storage Disease Type III 5 Glycogen Storage Disease Type IV 4 Glycogen Storage Disease Type IXa1 3 Glycogen Storage Disease Type IXc 3 Glycogen Storage Disease Type IXd 3 Glycogen Storage Disease Type V 6 Glycogen Storage Disease Type[preventiongenetics.com]
    • Glycogen storage disease 1 .[patient.info]
    • Radiology 153:105–107 PubMed Google Scholar Restaino I, Kaplan BS, Stanley C, Baker L (1993) Nephrolithiasis, hypocitraturia, and a distal renal tubular acidification defect in type I glycogen storage disease.[link.springer.com]
    • storage disease type 1a AR FAH Fumarylacetoacetase Tyrosinemia type 1 AR ATP7B Copper‐transporting ATP‐ase Wilson disease AR CLCN5 Chloride channel 5 (ClC‐5) Dent disease 1 XLR OCRL Phosphatidylinositol 4,5‐bisphosphate 5‐ phosphatase (OCRL1) Dent disease[intechopen.com]
    Hypophosphatasia
    • […] transplant, distal renal tubular acidosis, primary hyperparathyroidism, inherited disorders of the kidney, hyperoxaluria, loop diuretics in neonates, vitamin D and A toxicity, FAM20A mutations – enamel renal syndrome -, claudins, hypomagnesemic states, and hypophosphatasia[kidneystones.uchicago.edu]
    • 4 Adolescent Nephronophthisis 3 Adrenal Hyperplasia, Congenital, Due To 21-Hydroxylase Deficiency 1 Adrenal Insufficiency, Congenital, With 46,XY Sex Reversal, Partial Or Complete 4 Adrenocortical Carcinoma, Hereditary 1 Adrenoleukodystrophy 4 Adult Hypophosphatasia[preventiongenetics.com]
    Polycystic Liver Disease
    • Liver Disease 2 1 Polydactyly Preaxial Type 4 3 Polydactyly, Postaxial, Type A1 3 Polydactyly, Preaxial II 1 Polyglandular Autoimmune Syndrome, Type 1 6 Polyglucosan body disease, adult form 2 Polyglucosan Body Myopathy 1 with or without Immunodeficiency[preventiongenetics.com]

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    References

    1. Asplin JR, Mandel NS, Coe FL. Evidence of calcium phosphate supersaturation in the loop of Henle. Am J Physiol. 1996; 270(4 Pt 2):F604.
    2. Markowitz GS, Nasr SH, Klein P, et al. Renal failure due to acute nephrocalcinosis following oral sodium phosphate bowel cleansing. Hum Pathol. 2004; 35(6):675. 
    3. Schepens D, Verswijvel G, Kuypers D, et al; Images in Nephrology. Renal cortical nephrocalcinosis. Nephrol Dial Transplant. 2000; Jul 15(7):1080-2.
    4. Hoppe B, Langman CB. A United States survey on diagnosis, treatment, and outcome of primary hyperoxaluria. Pediatr Nephrol. 2003;Oct 18(10):986-91.
    5. Leumann E, Hoppe B. The primary hyperoxalurias. J Am Soc Nephrol. 2001; Sep; 12(9):1986-93.
    6. Sayer JA, Carr G, Simmons NL. Nephrocalcinosis: molecular insights into calcium precipitation within the kidney. Clin Sci (Lond). 2004; 106(6):549.
    7. Porter RS, Kaplan JL. The Merck Manual of Diagnosis and Therapy. 19th Edition, Whitehouse Station, NJ; 2011. Merck & CO., Inc;
    8. Sands JM, Naruse M, Baum M, et al. Apical extracellular calcium/polyvalent cation-sensing receptor regulates vasopressin-elicited water permeability in rat kidney inner medullary collecting duct. J Clin Invest. 1997;Mar 15; 99(6):1399-405.
    9. Jonsson KB, Zahradnik R, Larsson T, et al. Fibroblast growth factor 23 in oncogenic osteomalacia and X-linked hypophosphatemia. N Engl J Med. 2003;Apr 24; 348(17):1656-63.
    10. Ammenti A, Pelizzoni A, Cecconi M, et al; Nephrocalcinosis in children: a retrospective multi-centre study. Acta Paediatr. 2009; Oct 98(10):1628-31.
    11. Adams ND, Rowe JC. Nephrocalcinosis. Clin Perinatol. 1992; Mar 19(1):179-95.

    • Acute and chronic urinary infection: present concepts and controversies - SM Goldman - Urologic radiology, 1988 - Springer
    • Acetazolamide and sodium bicarbonate induced nephrocalcinosis and nephrolithiasis: Relationship to citrate and calcium excretion - AM Parfitt - Archives of internal medicine, 1969 - Am Med Assoc
    • Amelogenesis imperfecta and nephrocalcinosis syndrome: Case studies of clinical features and ultrastructure of tooth enamel in two siblings - RK Hall, P Phakey, J Palamara, DA McCredie - Oral Surgery, Oral , 1995 - Elsevier
    • Abnormal electrolyte and water metabolism in acute intermittent porphyria: the transient inappropriate secretion of antidiuretic hormone - ES Hellman, DP Tschudy, FC Bartter - The American journal of medicine, 1962 - Elsevier
    • Diagnostic examination of the child with urolithiasis or nephrocalcinosis - B Hoppe, MJ Kemper - Pediatric Nephrology, 2010 - Springer
    • Childhood extraordinary daytime urinary frequency—a case series and a systematic literature review - M Bergmann, T Corigliano, I Ataia, R Renella - Pediatric , 2009 - Springer
    • Consider sarcoidosis in patients with nephrocalcinosis, even if the chest roentgenogram is normal. - J Freitag, U Göbel, J Passfall, U Kettritz - Nephrology Dialysis , 1997 - ERA-EDTA
    • Familial hypomagnesemia with hypercalciuria and nephrocalcinosis - M Praga, J Vara, E Gonzalez-Parra, A Andres - Kidney , 1995 - nature.com
    • Atypical distal renal tubular acidosis confirmed by mutation analysis - S Weber, M Soergel, N Jeck, M Konrad - Pediatric Nephrology, 2000 - Springer

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