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At least 631 records · Page 35Linked to original sources
PubMed1976

Hemoglobin-oxygen affinity in hypophosphatemic rickets.

In 14 patients with simple X-linked hypophosphatemic rickets, 5 were below the third percentile in height and 9 were between the third and twenty-fifth percentile. Although the mean serum inorganic phosphorus level was only 2.01 +/- 0.65 (normal range for all age groups is 3.8 to 6.0 mg/100 ml), both the mean values for red cell 2,3-diphosphoglycerate (2,3-DPG) and adenosine triphosphate (ATP) were normal at 4.78 +/- 1.23 and 1.02 +/- 0.17 mumol/ml of red blood cells respectively. Moreover, the mean P50 value was normal at 26.4 +/- 0.9 mmHg. These normal oxygen transport data make unlikely any proposal that short stature seen in these patients is secondary to chronic tissue hypoxia. They also indicate that the intra-erythrocytic organic phosphate levels are maintained at normal levels despite profound chronic hypophosphatemia.

Adenosine Triphosphate↗
PubMed1976

Defective neutrophil motility in hypovitaminosis D rickets.

The chemotactic activity and random motility of neutrophils, was studied in 38 patients with hypovitaminosis D rickets, and compared with 29 healthy controls of matched age. The chemotactic activity derived from the activated rickets serum as well as the amounts of the complement components C4, C3 and C5 was normal, but the cell motility was clearly defective (p less than 0.001). A possible relationship between defective neutrophil movement and the recurrent infections seen in these patients is suggested. The possible mechanisms responsible for the defect could be the alteration in Ca/P metabolism or a defective action of the vitamin D on the neutrophils.

Cell Movement↗
PubMed1983

A case of hypophosphataemic vitamin D-resistant rickets treated with initial massive doses of 1 alpha-hydroxy-vitamin D3 alone.

A 1 5/12-year-old girl with hypophosphataemic vitamin D-resistant rickets was treated with initial massive doses of 1 alpha-hydroxy-vitamin D3 (1 alpha-OH-D3) alone. A dramatic improvement of bone lesions and growth rate and normalization of the characteristic biochemical defects were noted within four months after the start of massive 1 alpha-OH-D3 therapy. The results of this study suggest that early therapy with massive doses of 1 alpha-OH-D3 alone improves the radiological findings and restores to normal the biochemical defects and the growth rate as early as possible. This regimen may improve motor strength and endurance and obviate the need for corrective orthopaedic procedures.

Bone and Bones↗
PubMed1984

Premature cranial synostosis in X-linked hypophosphatemic rickets: possible precipitation by 1-alpha-OH-cholecalciferol intoxication.

A child suffering from X-linked hypophosphatemic rickets developed vitamin D intoxication under treatment with 1-alpha-OH-cholecalciferol (1(OH)D3) and phosphorus. Beside the usual findings in this condition he showed precocious synostosis of the skull with signs of raised intracranial pressure. In view of earlier reports of coincidence of craniostenosis and X-linked hypophosphatemic rickets, we conclude that the possibility exists that intoxication with 1(OH)D3 has been the precipitating factor. In addition we found hypersensitivity to 1(OH)D3 2 months after cessation of treatment, and normal levels of calcitriol (1,25(OH)2D3) at the same time.

Cranial Sutures↗
PubMed1984

Height, sitting height and leg length in patients with hypophosphataemic rickets.

Height, sitting height (SH) and subischeal leg length (SLL) were determined in 5 boys and 11 girls with hypophosphataemic (vitamin D-resistant) rickets, aged 4-14 years. Their average height was -2.05 +/- 1.22 SDS, and SLL (average value: -2.59 +/- 1.18 SDS) was more reduced than SH (average value: -0.91 +/- 1.37 SDS). SLL and SH were abnormally low in 11 and 3 of the patients respectively. The difference between SLL and SH was abnormally low in only 4 of the children, indicating a mild degree of disproportion. None of the patients had relatively long legs. There was no relation between height and the degree of disproportion, a finding which tallied with the relatively mild degree of disproportion. This indicated that the normal interindividual differences in proportion were more important than a preferential effect of rickets on leg length.

Adolescent↗
PubMed1993

Growth in children with X-linked hypophosphataemic rickets.

Currently, X-linked hypophosphataemic rickets (XLHR) is most commonly treated with a combination of phosphate and vitamin D, but there is conflicting evidence about the effects of this treatment on linear growth. In all, 25 patients with XLHR (current age range, 4.1-22.1 years; median, 8.2 years) were studied to determine whether there was any improvement in height SDS during treatment. The duration of therapy was 2.9-15.0 years (median, 5.7 years). Measurements before the age of 2 years or after the onset of puberty were excluded to remove the effects of measurement difficulties in small infants and of variation in pubertal timing. The growth of these patients was compared with a similar group of untreated historical controls. Patients treated with calcitriol and phosphate for at least 2 years before the onset of puberty (n = 22) had a significantly better mean height SDS than the historical controls (-1.23 compared with -2.05 for the historical controls; p = 0.02). Among patients treated with calcitriol and phosphate for at least 2 years (n = 21), the change in height SDS had a positive correlation with the duration of therapy (r = 0.51; p = 0.02). The growth of children with XLHR treated with combination therapy was thus significantly better than that of historical controls.

Adolescent↗
PubMed1997

Comparison of total alkaline phosphatase and three assays for bone-specific alkaline phosphatase in childhood and adolescence.

We compared serum levels of total alkaline phosphatase (TAP) and bone-specific alkaline phosphatase (BAP) as determined by three different assays (lectin affinity electrophoresis, immunoradiometric assay, enzyme-linked immunosorbent assay) in subjects aged 5-20 years suffering from X-linked hypophosphatemic rickets (n = 14), chronic renal failure (n = 10) and chronic cholestatic liver disease (n = 16). Results were compared to controls of the same age and were expressed as standard deviation scores (SDS). TAP correlated significantly with BAP (r > 0.9 for each assay; p < 0.001) in controls. In children with cholestatic diseases, TAP (median SDS + 2.0) was elevated, but BAP, as measured by the electrophoretic assay, was within the reference range for most patients (median SDS: -0.4; p = 0.003 for the difference between the median SDS of TAP and BAP). In contrast, results for BAP as determined by the two immunoassays were not significantly different from TAP in any of the three patient groups (p > 0.05 in each group for both assays). In this study, the two immunoassays did not have a detectable advantage over lectin affinity electrophoresis in the determination of BAP.

Adolescent↗
PubMed2005

Vitamin D-dependent rickets type II in a cat.

A cat with clinical signs Indicating rickets was diagnosed as having a defect of vitamin D receptors. Clinical signs had been seen from four months of age. Treatment with calcium supplementation and various forms of vitamin D did not alter plasma calcium levels or reverse skeletal lesions of lateral antebrachial bowing, lumbar spinal lordosis and costochondral beading. Analgesics were effective for relieving skeletal pain during the bone growth phase and were withdrawn when the animal reached skeletal maturity. Therapy for hip osteoarthritis was given from five years of age until the cat was euthanased at nine years of age as a result of refractory hip pain.

Analgesics↗
PubMed1971

Serum parathyroid hormone in X-linked hypophosphatemia.

Serum immunoreactive parathyroid hormone(IPTH) is normal in patients with X-linked hypophosphatemic rickets who are not treated with phosphate salts. Phosphate raises IPTH in these patients. Endogenous IPTH does not influence the existing defect in tubular reabsorption of phosphate in male patients.

Calcium↗
PubMed1971

Strontium induced rickets: metabolic basis.

Dietary strontium inhibits both the synthesis of 1,25-dihydroxycholecalciferol and intestinal calcium absorption in vitamin D(3)-repleted chicks. 1,25-Dihydroxycholecalciferol restores calcium absorption to normal, while 25-hydroxycholecalciferol is without effect in the strontium-fed chick. It is suggested that strontium induces rickets by blocking the biosynthesis of 1,25-dihydroxycholecalciferol, the metabolically active form of vitamin D in the intestine.

Animals↗