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PubMed2003

A novel mutation in the anion exchanger 1 gene is associated with familial distal renal tubular acidosis and nephrocalcinosis.

OBJECTIVE: The anion exchanger gene (AE1) or band 3 encodes a chloride-bicarbonate (Cl(-)/HCO(3)(-)) exchanger expressed in the erythrocyte and in the renal alpha-intercalated cells involved in urine acidification. The purpose of the present study was to screen for mutations in the AE1 gene in 2 brothers (10 and 15 years of age) with familial distal renal tubular acidosis (dRTA), nephrocalcinosis, and failure to thrive. METHODS: AE1 mutations were screened by single-strand conformation polymorphism, cloning, and sequencing. RESULTS: A complete form of dRTA was confirmed in the 2 affected brothers and an incomplete form in their father. All 3 were heterozygous for a novel 20-bp deletion in exon 20 of the AE1 gene. This deletion resulted in 1 mutation in codon 888 (Ala-888-->Leu) followed by a premature termination codon at position 889, truncating the protein by 23 amino acids. As band 3 deficiency might lead to spherocytic hemolytic anemia or ovalocytosis, erythrocyte abnormalities were also investigated, but no morphologic changes in erythrocyte membrane were found and the osmotic fragility test was normal. CONCLUSIONS: A novel mutation in the AE1 gene was identified in association with autosomal dominant dRTA. We suggest that RTA be considered a diagnostic possibility in all children with failure to thrive and nephrocalcinosis.

Acidosis, Renal Tubular↗

PubMed1995

Ultrastructural study of tooth enamel with amelogenesis imperfecta in AI-nephrocalcinosis syndrome.

This paper describes the ultrastructure of the affected enamel and the clinical features in two siblings with the syndrome of nephrocalcinosis and amelogenesis imperfecta. Nephrocalcinosis was diagnosed by intravenous pyelography, and confirmed by ultrasonography and CT scan. Amelogenesis imperfecta AI was diagnosed clinically and histologically. Light microscopy showed that the affected enamel surfaces were rough and the enamel was hypoplastic and mainly positively birefringent. Scanning electron microscopy revealed a rough and extensively cracked enamel surface covered with oval shaped blister-like protrusions. TEM showed porous enamel consisting of loosely packed and randomly oriented thin ribbon-like crystals with little or no prismatic structure. Observations showed that hypoplasia together with hypocalcification and/or hypomaturation defects were present in the same tooth, indicating the possibility of an abnormality in interstitial matrix, leading to dystrophic calcification in the kidney and abnormal tooth enamel formation, or alternatively an involvement of two separate but closely linked genes.

Adolescent↗

PubMed2000

Microscopic nephrocalcinosis and hypercalciuria in nephrotic syndrome.

Focal calcification is an occasional tubular abnormality seen in minimal-change nephrotic syndrome. Nephrocalcinosis was also reported in premature infants as a consequence of hypercalciuria resulting from long-term furosemide therapy. We describe 4 nephrotic children (3 minimal change, 1 diffuse proliferative glomerulonephritis) with transient hypercalciuria and intraluminal calcifications in renal histopathological specimens without radiologic evidence of renal calcification. These children were resistant to corticosteroid therapy and were receiving furosemide therapy along with albumin for management of oedema. Two of the children also had urinary infection. We were concerned that children with nephrotic syndrome are at risk for nephrocalcinosis, and urinary calcium and pH should be monitored carefully during prolonged furosemide use, especially in children with nephrotic syndrome with reduced initial responsiveness to corticosteroid therapy. HUM PATHOL 31:1363:1367.

Calcium↗

PubMed1992

Hyperoxaluria, nephrolithiasis, nephrocalcinosis and renal failure after massive resection of the small intestine: report of a case.

Hyperoxaluria is frequently seen in patients with inflammatory bowel disease, or after resection of the ileum. It is assumed to be responsible for the development of nephrolithiasis, nephrocalcinosis (oxalate nephrosis) and progressive renal impairment in these patients. Steatorrhea may aggravate the severity of hyperoxaluria. A 60-year-old male underwent massive resection of the jejunum and ileum 10 years prior to admission, due to strangulation of the small bowel, with occlusion of the superior mesenteric artery. He remained well except for steatorrhea which developed two-and-a-half years prior to admission, when microhematuria, proteinuria and oxaluria developed progressively. Since that time, the nephrolithiasis, nephrocalcinosis and renal failure have continued to worsen despite therapy with oxalate restriction and oxalate-binding agents. A renal biopsy, performed late in the clinical course, showed severe changes in the renal parenchyma. The decline in renal function proved irreversible. The unusual metabolic consequences of massive resection of the small intestine and their mechanisms are discussed.

Humans↗

PubMed1992

Nephrocalcinosis caused by hyperparathyroidism in progression of renal failure: treatment with calcitriol.

Nephrocalcinosis is accepted to contribute to the progression of renal failure. We have reviewed evidence that nephrocalcinosis is caused directly by the excess parathyroid hormone produced in renal disease. Evidence that hyperparathyroidism in uremic patients results from calcitriol deficiency and the mechanisms by which this comes about have been discussed. We have shown that renal secondary hyperparathyroidism can be eliminated or substantially reduced without increasing blood calcium using a low-dosage regimen of calcitriol. Decreasing PTH concentrations to or near normal alleviates this hormone's toxicity to many organs, including the kidneys. Potential benefits for the uremic patient include an increase in the quality and length of life. Calcitriol treatment provides a powerful means to reduce PTH concentration in uremic patients that may not be achieved with other methods. Further prospective clinical studies of uremic dogs and cats are warranted to document preservation of renal function and histology during calcitriol treatments.

Animals↗

PubMed2004

Effects of simultaneous increases in dietary phosphorus and magnesium concentrations on nephrocalcinosis and kidney function in female rats.

The effects of simultaneous increases in dietary phosphorus (P) and magnesium (Mg) concentrations while maintaining a constant P:Mg ratio on nephrocalcinosis and kidney function in female rats was investigated. Female Wistar rats were fed a control diet (3.12 g P, 0.51 g Mg per kg diet) or a diet having either 3 times the control P and Mg concentrations (3-fold diet; 9.25 g P and 1.42 g Mg per kg diet) or 5 times the control concentrations (5-fold diet; 14.97 g P and 2.37 g Mg per kg diet) for 21 d. The three experimental diets all had same P:Mg molar ratios (control diet; 4.81, 3-fold diet; 5.11, 5-fold diet; 4.96). The 3-fold diet had no significant influence on kidney calcium (Ca), Mg or P concentrations. However, kidney Ca, Mg and P concentrations were significantly higher in rats fed the 5-fold diet than in rats fed the control or 3-fold diets. No significant differences in creatinine clearance were observed among the three groups. Urinary albumin and beta 2-microglobulin excretion were higher in rats fed the 5-fold diet than in rats fed the control or 3-fold diets, while the 3-fold diet had no significant influence on the urinary albumin and beta 2-microglobulin excretion. These results suggest that absolute concentrations of dietary P and Mg are important factors with regard to the development of nephrocalcinosis and diminished kidney function.

Albumins↗

PubMed2004

Nephrocalcinosis in a 2-month-old girl suffering from a mild variant of idiopathic infantile hypercalcemia.

Authors present a rare case of a mild variant of idiopathic infantile hypercalcemia (IIH), in a girl in whom nephrocalcinosis was diagnosed in the 8th week of life. Supplementation of vitamin D3 (400 i.u. per day) has been applied since the age of 3 weeks. The disease was recognised when exploring pyuria and lack of weight gain. Hypercalcemia was corrected by diet reduced in calcium, free of vitamin D3 and with high fluid intake. The first abdominal ultrasound after delivery was performed after 5 week vitamin D3 supplementation. It seems that if abdominal ultrasound scan had been done earlier it could have drawn our attention to the developing nephrocalcinosis and allowed us to start treatment more promptly.

Female↗

PubMed1992

Nephrocalcinosis in the rat: a literature review.

Nephrocalcinosis is a common disorder in female rats. Various etiological factors are involved in the pathogenesis, e.g. sex, age, genetical and dietary factors. Dietary phosphorus concentration appears to be of crucial importance in the induction of nephrocalcinosis. The pathological changes in calcinotic kidney tissue are described. Possible mechanisms underlying nephrocalcinogenesis are discussed. Phosphorus concentration within the proximal tubule may be the major determinant of nephrocalcinogenesis.

Age Factors↗

PubMed2006

[The diagnosis of familial hypomagnesemia with hypercalciuria and nephrocalcinosis in a girl with acute lymphoblastic leukemia--case report].

Familial hypomagnesemia with hypercalciuria and nephrocalcinosis (FHHNC) is a rare autosomal recessive tubulopathy resulting from mutation in the gene encoding paracelin 1. The main symptoms of FHHNC include excessive urinary calcium and magnesium excretion, nephrocalcinosis, and chronic renal failure. We present 16-year old girl in whom symptoms of FHHNC were accidentally recognized during therapy of acute lymphoblastic leukemia. In our patient, some symptoms of FHHNC were initially taken for the adverse effects of cytostatic therapy that delayed an adequate diagnosis. To the best of our knowledge, this is the first report of FHHNC associated with acute lymphoblastic leukemia. However, in our opinion this association is accidental.

Adolescent↗

PubMed1991

Nephrocalcinosis and idiopathic hypercalciuria.

In a sample of 28 children diagnosed as having idiopathic hypercalciuria, ultrasound studies showed nephrocalcinosis in 6 cases, the rest having normal sonographs. On biochemical comparison, the two groups were statistically distinguishable by means of three parameters. The group with nephrocalcinosis showed higher values of calciuria and calciuria versus magnesuria ratio (p less than 0.05), as well as lower maximum urinary osmolality (p less than 0.01) than the group with normal sonographs. A negative correlation was found between the calciuria values and the values obtained from the concentration test (p less than 0.05). At present, the factors favoring the deposit of calcium salts in children with idiopathic hypercalciuria are not yet fully known.

Calcium↗

PubMed1991

The influence of dietary lactose on phosphorus-induced nephrocalcinosis in female rats.

The effect of dietary lactose, when compared with glucose, on phosphorus-induced nephrocalcinosis was studied in young, female rats. Nephrocalcinogenic diets containing either 0.4 or 0.6% (w/w) phosphorus were used, and lactose was added up to concentrations of 30%. The diets were fed for 28 days. The 0.4 and 0.6% phosphorus diets, when compared with a diet containing 0.2% phosphorus, caused mild and severe kidney calcification, respectively; kidney calcification was demonstrated chemically by the analysis of kidney calcium, and histologically by staining kidney slides for calcium deposits. Dietary lactose caused calciuria, decreased urinary pH and increased cecum weights. The addition of lactose to the diet partly counteracted nephrocalcinogenesis induced by diets containing 0.4% phosphorus, but it did not influence the severity of nephrocalcinosis seen in rats fed diets containing 0.6% phosphorus. It is suggested that high amounts of lactose in the diet have only weak anti-nephrocalcinogenic activity.

Animals↗

PubMed1991

Dietary fructose produces greater nephrocalcinosis in female than in male magnesium-deficient rats.

The synergistic interaction of fructose and magnesium (Mg) deficiency on kidney calcification was compared in male and female rats. Male and female weanling rats were divided into four dietary groups: fructose or starch, with or without Mg. Rats were fed their respective diets for 9 weeks, and 24 h urine was collected to measure urinary output, pH, Mg, calcium (Ca), and oxalic acid. Rats were fasted overnight. After decapitation, blood was collected immediately, and kidneys were removed to determine their Mg and Ca content. Dietary fructose significantly increased kidney Ca in female rats fed deficient or adequate Mg diet and in male rats fed Mg-deficient diet only; the greatest kidney calcification occurred in female rats fed Mg-deficient diet (P less than 0.0001). Even in starch groups female rats fed the Mg-deficient diet showed some kidney Ca accumulation. The synergistic interaction of fructose and magnesium deficiency on nephrocalcinosis was significantly greater in female than in male rats. Low urinary output, optimal pH 6.8 for calcium phosphate precipitation, hypercalcaemia, hypercalciuria, hypomagnesuria, and low ratio of urinary Mg to Ca may independently or multifactorially contribute to nephrocalcinosis. The possible mechanism of this interaction is discussed.

Analysis of Variance↗

PubMed1971

Relationship between the ingredients of semipurified diets and nutritional nephrocalcinosis of rats.

Nephrocalcinosis occurred in rats fed semipurified diets containing the levels of essential nutrients recommended for the rat by the Committee on Animal Nutrition of the National Research Council. The renal lesions were less severe in males than in females when weanling rats were fed the diet for 8 weeks. A study of the dietary ingredients indicated that there was an interaction between the mineral and nonmineral components which modulated the severity of the disease. It was shown that nephrocalcinosis did not result from a lack of trace minerals or vitamins, and the disease was prevented in one instance by reducing the dietary levels of calcium and phosphorus. Before final conclusions can be made concerning the etiology of this disease, additional information is required about the effects of mineral and nonmineral interactions on calcium metabolism and renal function.

Animal Nutritional Physiological Phenomena↗

PubMedDate not supplied

Acetazolamide-induced nephrocalcinosis.

Nephrocalcinosis has been reported in patients treated with acetazolamide and other agents, such as furosemide or bicarbonate. We present a case of nephrocalcinosis induced solely by acetazolamide and diagnosed radiographically. Possible mechanisms of pathogenesis are reviewed.

Acetazolamide↗

PubMed2001

Effectiveness of a traditional Chinese medicine, Wulingsan, in suppressing the development of nephrocalcinosis induced by a high phosphorus diet in young rats.

The development of nephrocalcinosis in rats fed a high phosphorus diet, and the effectiveness of the Chinese traditional medicine, Wulingsan, and its components (Poria, Alismatis Rhizoma, Atractylodis Rhizoma, Cinnamomi Ramus, Polyporus) in suppressing the development of calcinosis were studied. The rats were fed a high phosphorus diet (1.5% P) supplemented with Wulingsan or its individual components (0.5 g/kg body weight) as separate experimental groups for a 2-week period. Upon histological observation by light microscopy and electron microscopy, signs of nephrocalcinosis were observed in almost all areas of the kidney. Calculi, consisting mainly of needle-shaped crystals of hydroxyapatite, were observed in the proximal tubules, in the collecting ductal lumina, and in the mitochondria of the proximal tubular cells and the interstitial cells. X-ray microanalysis revealed that the calculi were composed of hydroxyapatite (Ca and P). In the group fed the diet supplemented with Wulingsan, the severity of calcinosis in the corticomedullary junction was only slight. In all groups fed individual components of Wulingsan, the severity of calcinosis was almost the same as that in the group fed the high phosphorus diet (1.5% P). Wulingsan suppressed the development of calcinosis in rats fed the high phosphorus diet supplemented with this Chinese medicine, whereas its individual components alone had no effect. The process of calcinosis and the mechanism responsible for the activity of this Chinese medicine in the suppression of calcinosis are discussed.

Animals↗

PubMed1995

Hypercalcemia and nephrocalcinosis in patients with congenital lactase deficiency.

We describe 11 infants with congenital lactase deficiency, whose age at diagnosis varied from 6 to 88 days. At the time of admission, 7 of 10 infants had hypercalcemia. Five of the seven infants for whom renal ultrasonography was performed at the time of diagnosis had medullary nephrocalcinosis. Hypercalcemia ceased within a week of the start of a lactose-free diet. At the time of reevaluation, at the ages of 2 to 10 years, one of the patients still had hypercalciuria and nephrocalcinosis was still present in 3 of 11 patients. The mechanism of hypercalcemia is unclear but may be related to metabolic acidosis or may be promoted by the lactose effect (i.e., by nonhydrolyzed lactose that has a direct enhancing effect on calcium absorption in the ileum).

Acidosis↗

PubMedDate not supplied

Nephrocalcinosis in homozygous familial hypercholesterolemia: ultrasound and CT findings.

An association between homozygous familial hypercholesterolemia (FH) and nephrocalcinosis has not, to our knowledge, been previously reported. Evaluation in 10 cases of homozygous FH revealed evidence of nephrocalcinosis in 7 cases. Fine, uniform calcifications in the renal papillae were demonstrated by renal ultrasound or CT. Renal papillary necrosis was demonstrated by intravenous pyelogram in one case. No renal function impairment was noted in these seven patients. There was no evidence of hypercalcemia. These findings may have implications for the use of a potentially nephrotoxic drug for the treatment of this disease--in particular, when an immunosuppressive agent is required following liver transplantation.

Adolescent↗

PubMed1977

[Goodpasture's syndrome and nephrocalcinosis].

In comparison with literary data is reported on 3 young men with a Goodpasture syndrome. Immunohistologically in all cases a glomerulonephritis of the type of basal membrane antibodies was proved, in one patient also still 14 months after having reached the terminal stage of the renal insufficeincy. In every case the disease led to the dialytic treatment without the necessity of nephrectomies for commanding haemmorrhages of the lungs. The longest survival time was 3 years. -- In 1 patient within metastatic calcifications a pronounced nephrocalcinosis developed. Massive depositions of calcium were found in the basal membranes of the glomeruli and tubuli. It is discussed on the genesis of nephrocalcinosis, in which case a secondary hyperparathyrodism, an elevation of the calcium-phosphate-product, an alkalotic metabolic position as well as the dystrophic renal parenchyma of the rapidly progressing glomerulonephritis of Goodpasture's syndrome are taken into consideration.

Adolescent↗