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PubMed2004

Effect of the calcimimetic NPS R-467 on furosemide-induced nephrocalcinosis in the young rat.

BACKGROUND: Furosemide induces nephrocalcinosis in both humans and animals. We showed previously that parathyroidectomy protected against the development of furosemide-induced nephrocalcinosis in young rats, indicating a possible role for parathyroid hormone (PTH) in its pathogenesis. Calcimimetic agents such as NPS R-467 are potent and selective agonists at the calcium-sensing receptor in parathyroid glands and inhibit PTH secretion. METHODS: To determine whether NPS R-467 could, like parathyroidectomy, prevent furosemide-induced nephrocalcinosis, we studied 35 6-week-old male Sprague-Dawley rats, divided into five groups. Group A served as control, group B received intraperitoneally furosemide (40 mg/kg), groups C, D, and E received furosemide and NPS R-467 intraperitoneally at doses of 10, 20, and 40 micromol/kg, respectively, daily for 8 days. During the last 3 days, animals were placed in metabolic cages for measurement of urine output, food, and water intake. Blood and kidneys were collected on day 8, 60 to 90 minutes after the last doses. Kidney calcium content was measured and nephrocalcinosis scoring (0 to 5) was assessed histologically. RESULTS: Furosemide increased urine output and fluid intake, and decreased body weight gain similarly in all groups. Serum PTH levels (mean +/- SD) were significantly higher in furosemide-treated control animals (276 +/- 226 pg/mL vs. 64 +/- 21 pg/mL); NPS R-467 induced a dose-dependent decrease in PTH levels (52 +/- 51 pg/mL, 18 +/- 7 pg/mL, and 13 +/- 3 pg/mL in groups C, D, and E, respectively). Plasma Ca(2+) was slightly, but significantly lower in all three NPS R-467 treated groups (5.1 +/- 0.4 mg/dL, 4.8 +/- 0.3 mg/dL, and 4.5 +/- 0.3 mg/dL in groups C, D, and E, respectively) compared to 5.7 +/- 0.1 mg/dL and 5.5 +/- 0.2 mg/dL in groups A and B, respectively. Furosemide treatment induced a substantial increase in kidney calcium content (1819 +/- 664 microg/g dry weight vs. 126 +/- 26 microg/g dry weight) and nephrocalcinosis scoring (5.0 +/- 0.0 vs. 0.0 +/- 0.0). Treatment with NPS R-467 ameliorated the furosemide-induced increase in kidney calcium content (673 +/- 312 microg/g, 361 +/- 188 microg/g, and 563 +/- 291 microg/g) and nephrocalcinosis scoring (2.2 +/- 1.2, 0.7 +/- 0.8, and 1.0 +/- 1.2) in groups C, D, and E, respectively. CONCLUSION: The calcimimetic agent NPS R-467 prevents the development of hyperparathyroidism and attenuates nephrocalcinosis in the furosemide-treated young rat.

Aniline Compounds↗

PubMed1989

Familial distal renal tubular acidosis with neurosensory deafness: early nephrocalcinosis.

Nephrocalcinosis was observed in 3 children of one family with distal renal tubular acidosis (dRTA). At presentation, all 3 patients had failure to thrive, rickets, hyperchloremic metabolic acidosis, hypokalemia, hypophosphatemia and hypercalciuria. At a later age, sensorineural hearing impairment was detected. Nephrocalcinosis was diagnosed in the index case at the age of 5 years, when a plain abdominal roentgenogram was first made; in the younger brother and sister, nephrocalcinosis was detected earlier at the age of 4 months and 5 weeks, respectively. All 3 patients required large doses of alkali (7.5-9.5 mEq/kg body weight/day) during infancy and early childhood to correct the acidosis and to prevent progression of the nephrocalcinosis. Contrary to the current notion that in children with dRTA, nephrocalcinosis is observed only after the age of 3 years, it appears that in some instances nephrocalcinosis may develop in early infancy. The occurrence of nephrocalcinosis at a very young age may be a manifestation of a severe genetically transmitted variant of dRTA and emphasizes the need for early diagnosis and optimal treatment of these patients from the first days of life.

Acidosis, Renal Tubular↗

PubMed1997

Salt supplementation, growth, and nephrocalcinosis in the furosemide-treated weanling rat.

OBJECTIVE: Furosemide treatment in the human neonate is associated with sodium depletion, growth retardation, hypercalciuria and nephrocalcinosis. Dietary sodium intake is known to directly influence urinary calcium excretion. The objectives of this study were to create a rat model of furosemide-induced nephrocalcinosis and to test the effects of dietary sodium supplementation on growth, electrolyte balance, calciuria, and renal calcifications. METHODS: Initially, 18 weanling Sprague-Dawley rats were randomly divided into three groups. Groups A (control) and B were fed a basal diet. Group C was fed a sodium-enriched diet. Groups B and C received furosemide (40 mg/kg) intraperitoneally daily for 28 days. At the end of the study, serum, urine, and kidney samples were obtained for biochemical and histologic analyses. The three groups were then compared for differences in growth, electrolyte homeostasis, calcium excretion and nephrocalcinosis. Subsequently an additional 15 rats were studied to confirm our findings regarding urinary calcium excretion and kidney calcifications. RESULTS: Treatment with furosemide without sodium supplementation (group B) resulted in decreased weight gain compared with group A (137.5 +/- 12.9 vs 154.0 +/- 10.6 g; p < 0.05), hypokalemia (3.7 +/- 0.1 vs. 4.4 +/- 0.4 mEq/l; p < 0.05), and nephrocalcinosis (187.1 +/- 155 vs. 18.8 +/- 6.9 micrograms Ca/g dry kidney; p < 0.05). Sodium supplementation (group C) normalized weight gain and corrected electrolyte abnormalities without increasing calciuria or nephrocalcinosis. CONCLUSIONS: We conclude that in this animal model, chronic furosemide treatment results in growth failure and development of nephrocalcinosis. Sodium supplementation protects against the deleterious effects of furosemide on weight gain and electrolyte homeostasis with no adverse effect on nephrocalcinosis.

Animals↗

PubMed1997

Nephrocalcinosis.

The routine use of ultrasonography has resulted in an explosion in the number of conditions reported to be associated with nephrocalcinosis. It has also been increasingly recognized that urolithiasis and nephrocalcinosis can coexist in the same patient. The two conditions most commonly associated with nephrocalcinosis in childhood are the use of furosemide in infancy and the treatment of patients with hypophosphatemic rickets with phosphate and vitamin D preparations. Although originally thought to be related to hypercalciuria, more recent studies in humans and research animals indicate a multifactorial etiology for furosemide-related nephrocalcinosis. In patients with hypophosphatemic rickets, it seems that the dose of phosphate and in particular the development of secondary hyperparathyroidism play a central role in the development of nephrocalcinosis. Among the entities recently reported to be associated with nephrocalcinosis are some that characteristically include Fanconi's syndrome. These findings dispute the previous teaching of lack of renal calcifications in this syndrome, which involves proximal renal tubular acidosis. The diagnosis of nephrocalcinosis requires a metabolic work-up to identify the offending factor. When identified, appropriate intervention should be instituted.

Furosemide↗

PubMed1999

Observer reliability in grading nephrocalcinosis on ultrasound examinations in children.

BACKGROUND: Nephrocalcinosis is often associated with a variety of hypercalcemic conditions. Diagnostic ultrasound is often used for assessing nephrocalcinosis in children, but its reliability has not been proven. OBJECTIVE: To determine the reliability of expert interpretation of sonographic films with a grading scale of severity for nephrocalcinosis. MATERIALS AND METHODS: Fifty-eight ultrasonographic films of 30 children with Williams syndrome and other conditions know to be associated with nephrocalcinosis were assessed. We used a blinded randomized design to assess intra- and interobserver reliability. RESULTS: Grades I, II, and III nephrocalcinosis were noted in 13 %, 19 %, and 27 % of the examinations, respectively. The weighted kappa coefficient was 0.80 (standard error 0.12; 95 % confidence interval 0.68-0.92) for intraobserver agreement and 0.76 (standard error 0.13; 95 % confidence interval 0.63 to 0.89) for interobserver agreement. Reliability in assessing change from one examination to the next, with independently graded films, was fair with an unweighted kappa coefficient of 0.68 (95 % confidence interval 0.38-0.96) and 0.51 (95 % confidence interval 0.21-0.80) for intra- and interobserver reliability, respectively. CONCLUSION: The severity of nephrocalcinosis can be reliably interpreted with an ultrasonography grading scale.

Child↗

PubMed2006

Neonatal nephrocalcinosis: long term follow up.

AIMS: To assess the spontaneous resolution of neonatal nephrocalcinosis and its long term effects on renal function. METHODS: Fourteen very low birthweight preterm babies with nephrocalcinosis were followed up at 5-7 years of age; 14 controls were matched for sex, gestation, and birth weight. Height, weight, blood pressure, and renal symptomatology were recorded, and a renal ultrasound scan was performed. Early morning urine osmolality and creatinine ratios of albumin, phosphate, calcium, oxalate and beta microglobulin were determined. Urea and electrolytes in the study group were determined, and glomerular filtration rate (GFR) and TmP/GFR (tubular reabsorption of phosphate per GFR) were calculated. Statistical analysis was performed on a group basis using the Mann-Whitney confidence interval. RESULTS: Mean age was 6.9 years (range 5.81-7.68). An early morning urine osmolality >700 mOsm/kg was achieved in all cases. In two cases and four controls, the calcium/creatinine ratio was >0.7 mmol/mmol. In all cases, the GFR was normal (median 132.6 ml/min/1.73 m(2) (range 104.1-173.1)). Median TmP/GFR was 1.22 mmol/l (0.73-1.61), with two having levels below the normal range. These did not have persisting nephrocalcinosis. Nephrocalcinosis was found in three of the 12 cases scanned and one control. There were no significant differences in urine biochemistry. CONCLUSIONS: Resolution of nephrocalcinosis occurred in 75% of cases. No evidence was found to suggest that nephrocalcinosis is associated with renal dysfunction in the long term. There was evidence of hypercalciuria in the cases and controls, suggesting that prematurity may be a risk factor.

Birth Weight↗

PubMed2001

Nephrocalcinosis in preterm babies.

OBJECTIVES: To determine prospectively the incidence and cause of nephrocalcinosis in preterm infants. STUDY DESIGN: Inborn babies of gestation less than 32 weeks or birth weight less than 1500 g were eligible to be entered into a prospective observational study. Two renal ultrasound scans were performed, the first at 1 month postnatal age and the second at term or discharge. Data were collected on gestation, birth weight, sex, race, family history of renal calculi, oliguria on first day, respiratory support (ventilation, steroid, and oxygen dependency), and use of nephrotoxic drugs (gentamicin, vancomycin, and frusemide). Intake of fluid, calcium, and phosphate and plasma urea, creatinine, calcium, and phosphate were recorded for the first 6 weeks of life. Random urinary calcium/creatinine, oxalate/creatinine, and urate/creatinine ratios and tubular absorption of phosphate were measured once at term. RESULTS: A total of 101 preterm infants were studied. Twenty three (23%) had abnormal ultrasound scans. Sixteen (16%) had nephrocalcinosis. On univariate analysis, gestational age, male sex, duration of ventilation, oxygen dependency, duration and frequency of gentamicin treatment, toxic gentamicin/vancomycin levels, and postnatal dexamethasone were significantly associated with nephrocalcinosis. In addition, babies with nephrocalcinosis had a lower intake of fluid, calcium, and phosphate, longer duration of total parenteral nutrition, and higher urinary oxalate/creatinine and urate/creatinine ratios than infants who did not have the condition. There was also a significant association with plasma urea and creatinine but not with plasma calcium or phosphate or urinary calcium. Multivariate analysis showed that the strongest predictors of nephrocalcinosis were duration of ventilation, toxic gentamicin/vancomycin levels, low fluid intake, and male sex. CONCLUSION: 16% of babies born at less than 32 weeks gestation developed nephrocalcinosis. The multifactorial origin, in particular, the association with extreme prematurity and severity of respiratory disease, is confirmed. In addition, an association with male sex, frequency and duration of gentamicin use, and high urinary oxalate and urate excretion is shown.

Aminoglycosides↗

PubMed1993

Hyperoxaluria is not a cause of nephrocalcinosis in phosphate-treated patients with hereditary hypophosphatemic rickets.

The treatment of X-linked hypophosphatemia (XLH) consists of phosphate and vitamin D3 derivatives. Transient hypercalciuria and hypercalcemia are well-known signs of vitamin D intoxication. Despite urinary calcium excretion control, the danger of nephrocalcinosis in treated patients has been emphasized. It has recently been suggested that hyperoxaluria might be a causative factor of nephrocalcinosis other than calcium in phosphate-treated XLH patients. We measured urinary oxalate and phosphate excretion in 12 patients with the syndrome of hereditary hypophosphatemic rickets with hypercalciuria (HHRH) receiving only oral phosphates and in 5 XLH patients receiving both oral phosphates and vitamin D. No correlation was found between the dosage of phosphate supplements or urinary phosphate excretion and urinary oxalate excretion, in either group of patients. Nephrocalcinosis, presenting as hyperechogenicity of the medullary pyramids, was found in 2 of the 5 XLH patients and only in 2 HHRH patients who had been treated with excessive doses of vitamin D2 and calcium, prior to the true diagnosis being established. We conclude: (1) hyperoxaluria is not a cause of nephrocalcinosis in phosphate-treated patients with hereditary hypophosphatemic rickets; (2) prolonged phosphate treatment alone does not induce nephrocalcinosis in HHRH patients, and (3) we believe that in XLH patients, nephrocalcinosis is essentially due to vitamin D overdosage at some stage, or noncompliance in phosphate intake, leading to repeated undetected hypercalciuric periods.

Adolescent↗

PubMed1991

Phosphorus-induced nephrocalcinosis in female rats: a study on regression and clinical abnormalities.

The question addressed was whether preestablished phosphorus (P)-induced nephrocalcinosis would regress after dietary P restriction. Female rats were fed purified diets containing either 0.2% (w/w) P (low P) or 0.6% P (high P). After 29 days, the high-P diet had caused massive nephrocalcinosis as demonstrated chemically (by the analysis of calcium in kidney) and histologically (by inspection of kidney sections stained for calcium phosphate deposits). Switching rats from the high P to the low P diet did not result in a decrease in the degree of nephrocalcinosis within 91 days. Thus, P-induced nephrocalcinosis may not regress upon subsequent P restriction. Rats that had been fed either the 0.2 or 0.6% P diet for 56 days were examined clinically with respect to 14 selected variables. None of the variables discriminated between rats with or without nephrocalcinosis. This might imply that P-induced nephrocalcinosis in female rats does not cause significant discomfort.

Animals↗

PubMed1999

Comparison of various phosphate salts as the dietary phosphorus source on nephrocalcinosis and kidney function in rats.

The effects of various phosphate salts as the dietary phosphorus sources on the development of nephrocalcinosis and kidney function were examined in rats fed diets containing monophosphate salts (sodium dihydrogenphosphate, NaH2PO4, or potassium dihydrogenphosphate, KH2PO4) or polyphosphate salts (sodium tripolyphosphate, Na5P3O10, or potassium tripolyphosphate, K5P3O10), at levels representing normal phosphorus (normal phosphorus diet) or high phosphorus (high phosphorus diet) contents for 21 d. High phosphorus diet-feeding increased the kidney calcium and phosphorus concentrations. Kidney calcium and phosphorus concentrations were higher in rats fed the high phosphorus diet containing Na5P3O10 or K5P3O10 than in rats fed the high phosphorus diet containing NaH2PO4 or KH2PO4. Nephrocalcinosis was observed in all rats fed a high phosphorus diet, and the degree of nephrocalcinosis was more severe in rats fed Na5P3O10 or K5P3O10 than in rats fed NaH2PO4 or KH2PO4. In rats fed the high phosphorus diet, creatinine clearance was higher in rats fed Na5P3O10 or K5P3O10 than in rats fed NaH2PO4 or KH2PO4. In rats fed Na5P3O10 or K5P3O10, urinary albumin excretion and N-acetyl-beta-D-glucosaminidase (NAG) activity in the urine were increased in rats fed the high phosphorus diet. These were higher in rats fed the high phosphorus diet containing Na5P3O10 than in rats fed the high phosphorus diet containing NaH2PO4 or KH2PO4. This study observed that the development of nephrocalcinosis and kidney function in rats fed the high phosphorus diet was influenced by the difference in monophosphate or polyphosphate salts provided as the dietary phosphorus source, while the effects of sodium and potassium salts were not evident. We suggest that the development of nephrocalcinosis and kidney function in rats fed a high phosphorus diet was altered depending on the form of phosphate salts provided as the dietary source of phosphorus. Additionally, the development of nephrocalcinosis and diminished kidney function in rats fed the high phosphorus diet was more severe for polyphosphate salts as compared to monophosphate salts.

Acetylglucosaminidase↗

PubMed1975

The development of nephrocalcinosis in the rat following injections of neutral sodium phosphate.

The development of nephrocalcinosis in the rat following intraperitoneal injections of various concentrations of neutral sodium phosphate (pH 7-4) was studied using histology, histochemistry, electron microscopy and quantitative techniques. Daily injections of 0-5 M phosphate consistently produced nephrocalcinosis after 6 days or more. Calcium deposits were at first confined to the basement membranes of proximal tubules; but a longer course of injections, up to 10 days, resulted in additional basement membrane calcification in the outer cortes, and outer medulla, together with intra-luminal casts, often calcified, in the outer medulla and papilla. Calcification was not found in other organs such as liver, lung, heart or aorta. Results from quantitative estimations of total kidney calcium and phosphorus suggested that it was the calcium content which was important to the initiation of nephrocalcinosis. Ultrastructural changes, suggestive of degeneration or alteration in function, were found in mitochondria of proximal tubules in experimental animals before the onset of histologically evident nephrocalcinosis. Later changes, especially to the basal part of proximal tubular cells and their basal laminae, were thought to be consequent upon the mitochondrial changes. It is suggested that the initial renal damage was caused both directly, by a toxic effect of the phosphate load on the kidney and, indirectly, by stimulation of the parathyroid glands as a result of the hypocalcaemia and hyperphosphataemia which followed an injection of phosphate. Daily doses of 1 M phosphate for 3 days produced a type of nephrocalcinosis which was more typical of that reported by previous investigators, who used high doses of phosphate. Twice daily injections of 0-25 M phosphate for 6 days did not induce nephrocalcinosis, whereas 0-375 M phosphate given twice daily for 6 days produced only minimal calcium deposits compared with animals given 0-5 M phosphate once daily for the same period. This may have important clinical implications, since phosphate has been used to control hypercalcaemia of various etiologies.

Alkaline Phosphatase↗

PubMed2004

Nephrocalcinosis in very low birth weight infants: a single center experience.

OBJECTIVES: To determine the incidence and risk factors of nephrocalcinosis in very low birth weight infants. MATERIAL AND METHOD: Medical records of inborn infants with gestational age less than 32 weeks or birth weight less than 1,250 grams were collected and analyzed. All infants were born at King Chulalongkorn Memorial Hospital in the year 2003. At least one renal ultrasonographic scan was performed on every infant as a routine screening before discharge. Data on family history of renal stone, gestational age, birthweight, infant's illness,fluid intake during the first 6 weeks of life, duration of respiratory support, medications, serum calcium, phosphate and alkaline phosphatase level, duration of parenteral nutrition, length of hospitalization, ultrasonographic findings and related renal morbidity were collected and compared between the groups of infants with and without nephrocalcinosis. RESULTS: Thirty six infants were included in the present study. Fourteen had abnormal ultrasound scans compatible with nephrocalcinosis giving an overall incidence of 38.9%. Factors associated with nephrocalcinosis included severity of respiratory illness, PDA, oxygen dependency, furosemide therapy, and fluid restriction. Urinary tract infection was the renal morbidity found in 3 infants (21.4%). Nephrocalcinosis was resolved in one out of 7 infants who had repeated renal ultrasound scan at about 2 months after the first scan. CONCLUSION: Very low birth weight, preterm infants have a risk of developing nephrocalcinosis especially those with severe respiratory illness and prolonged use of furosemide. Infants at risk should be screened with renal ultrasonography prior to discharge from the hospital.

Comorbidity↗

PubMed1980

Nephrocalcinosis in radiographs, computed tomography, sonography and histology.

In 12 patients with nephrocalcinosis, radiographs were compared with computed tomography, sonography and histology. Computed tomography detects nephrocalcinosis at a very early stage of the disease, gives a better picture of the density and extent of nephrocalcinosis and may detect other findings (e. g. cysts). Radiographs may show many more details than computed tomography; they seem to be more suitable for observing the course of the disease and have a lower radiation dose. Sonography has not been helpful in the diagnosis of nephrocalcinosis. Histology correlates only moderately with the degree of nephrocalcinosis demonstrated in radiographs and computed tomography.

Adolescent↗

PubMed1984

Influence of diet on the development of nephrocalcinosis in the rat.

A histological examination of the effect of a purified diet containing 20% alpha protein (an alkali-treated soyprotein) on the development of nephrocalcinosis induced by intraperitoneal injections of 0.5 neutral (pH 7.4) sodium phosphate was carried out in female weanling rats. Animals that were fed a standard commercial laboratory diet and given daily injections of phosphate for six or ten days developed a form of nephrocalcinosis that consisted mainly of intraluminal (intratubular) calcification at the junction of the outer and inner stripes of the outer medulla and in the inner stripe of the outer medulla. By contrast, rats that were fed the alpha protein diet and given injections of phosphate for six or ten days developed a form of nephrocalcinosis that was characterized primarily by a type of tubular basement membrane calcification at the junction of the inner stripe of the outer medulla and the inner medulla. The differences in nephrocalcinosis between the two dietary groups and the fact that an alpha protein diet by itself can cause renal calcification, leads to the suggestion that some component(s) or factor(s) in the alpha protein diet strongly influence(s) the development of nephrocalcinosis induced by injected neutral sodium phosphate.

Animals↗

PubMed2006

Continuous infusion of oxalate by minipumps induces calcium oxalate nephrocalcinosis.

It is hypothesized that oxalate plays an active role in calcium oxalate (CaOx) nephrocalcinosis and oxalate driven nephrolithiasis by interacting with the kidney. We developed an adjustable, nonprecursor, continuous infusion model of hyperoxaluria and CaOx nephrocalcinosis to investigate this hypothesis. Minipumps containing PBS or KOx (60-360 micromol/day; n = 5-7/dose) were implanted subcutaneously in male Sprague-Dawley rats on D0 and D6. Rats were killed on D13. Oxalate excretion and CaOx crystalluria were monitored by 20+4 h urine collections. Localization and content of intrarenal crystals were determined on frozen sections using polarization and microFTIR. Oxalate excretion was significantly elevated in all KOx rats (P < or = 0.005). CaOx crystalluria was most persistent in the 240-360 micromol/day KOx rats, but even 60 micromol/day KOx rats showed sporadic crystalluria. One hundred percent of KOx rats had CaOx nephrocalcinosis as confirmed by microFTIR. Most crystals were localized to the lumens of the corticomedullary collecting ducts. A few crystals are localized just under the papillar urothelium. The minipump model is the first model of hyperoxaluria to provide continuous infusion of oxalate. It permits control of the levels of hyperoxaluria, crystalluria and CaOx nephrocalcinosis. The level of sustained hyperoxaluria and CaOx nephrocalcinosis induced by treatment with 360 micromol/day KOx for 13D models the conditions frequently observed in jejunoileal bypass patients. Adjustments in the length of treatment and level of hyperoxaluria may allow this model to also be used to study the oxalate driven CaOx-nephrolithiasis common in patients with hyperoxaluria due to other causes.

Animals↗

PubMed2005

Case report of a rare syndrome associating amelogenesis imperfecta and nephrocalcinosis in a consanguineous family.

A rare syndrome associating amelogenesis imperfecta (AI) with nephrocalcinosis has been reported. The purpose of this study is to characterise the phenotype of a consanguineous family presenting amelogenesis imperfecta, delayed permanent teeth eruption and nephrocalcinosis. Six family members were examined. Ground sections of the case index deciduous teeth and biopsies of enlarged dental follicles were analysed. The patients's parents were first cousins. The case index had yellow discoloration and altered teeth shapes, retention of deciduous teeth, and delayed eruption. Panoramic radiographs revealed multiple enlarged pericoronal follicles in unerupted teeth and generalised intrapulpal calcifications. Renal ultrasound showed the presence of nephrocalcinosis. No other family members presented enamel defects or nephrocalcinosis. Histologically, the enamel appeared hypoplastic, and dental follicles indicated pericoronal hamartoma. The consanguineous marriage suggests an autosomal recessive mode of inheritance. Further studies are necessary to clarify the genetic defect behind this syndrome that associates AI, nephrocalcinosis and impaired tooth eruption.

Adolescent↗

PubMed1989

[Nephrocalcinosis in childhood. Sonographic findings and differential diagnosis].

Ultrasonography is the method of choice for the detection of medullary or cortical nephrocalcinosis in infancy and childhood. Compared with abdominal radiographs and computed tomography even smallest calcifications are detected more accurately. Using furosemide, ACTH, steroids or high doses of vitamin D, early forms of medullary nephrocalcinosis associated with a faint hyperechogenic rim at the margins of the renal pyramids can be diagnosed by ultrasound. Idiopathic hypercalciuria, Bartter's syndrome and renal tubular acidosis cause medullary nephrocalcinosis, whereas cortical nephrocalcinosis is the result of renal vein thrombosis; primary hyperoxaluria is associated with cortico-medullary calcifications. Due to the localisation of the nephrocalcinosis and the age distribution of the diseases, sonography merely enables us to narrow down differential diagnosis.

Aging↗

PubMed1988

Microscopic nephrocalcinosis in cystic fibrosis.

Airway, sweat-duct, and other epithelial cells in patients with cystic fibrosis display abnormal ion transport. To test whether the kidney, the organ most exquisitely adapted for ion transport, has a similar defect, we measured the levels of calcium excretion and searched for microscopic nephrocalcinosis in patients with cystic fibrosis. Thirty-eight specimens of kidney tissue were stained for calcium deposits, and 24-hour levels of urinary calcium excretion were measured in 14 patients and 15 control subjects. Microscopic nephrocalcinosis was observed in 35 of the 38 specimens (92 percent), and hypercalciuria (greater than 182 mg per gram of creatinine) in 5 of the 14 patients (36 percent). Notably, nephrocalcinosis was detected near the time of birth (in six patients under one year old, including two neonates and one stillborn infant), which supports the hypothesis that such renal calcium deposits reflect the genomic defect and are not due to longstanding pulmonary dysfunction, chronic infection, therapeutic agents, or disease progression. None of the patients with hypercalciuria or nephrocalcinosis had clinical evidence of renal dysfunction. The finding of microscopic nephrocalcinosis near the time of birth in patients with cystic fibrosis suggests a primary abnormality of calcium metabolism in the kidney. Studies of the pathophysiologic features of the kidney in cystic fibrosis may elucidate the molecular alterations observed in this disorder.

Adolescent↗