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At least 1,027 records · Page 57Linked to original sources
PubMed1978

[Effects of the main derivatives of vitmain D in 3 siblings with "pseudo-deficiency" rickets].

Three siblings, respectively 20, 16 and 12 years old, presented with hypocalcemic vitamin D resistant rickets (Prader's type). Their clinical history included several periods of spontaneous cessation of therapy, with severe relapses. Since 1973, treatment was strictly observed, allowing to test the therapeutic effects of 25 OH D3, 1-25 (OH) 2 D3 and 1-alpha (OH) D3. The clinical effects are reported as well as biochemical data. Among them, an inactive form of hyperparathyroidism is emphasized, which may resemble some cases of pseudohypoparathyroidism. Simultaneous resistance to exogenous PTE was also demonstraded.

Adolescent↗
PubMed1979

Calcium, phosphate, and vitamin D.

It is evident that there has been enormous progress within the last decade in our understanding of vitamin D metabolism and its interaction with calcium, phosphate, parathyroid hormone, and a number of other factors. As a result largely of therapeutic trials with various metabolites of vitamin D, we now know something of their probable involvement in the development of rickets and osteomalacia. We do not yet have a clear comprehension of exactly how these metabolites influence bone mineralization other than through their indirect effect on serum calcium and phosphate. Nonetheless it seems likely that at least one, and perhaps more, of the metabolites do exert a more direct effect. One must conclude that a deficiency of the 1alpha-hydroxylase is probably not the only defect in renal insufficiency, although it currently that there must well known. It is premature to go so far as to suggest that there must be another metabolite not being synthesized in the diseased or absent kidney.

25-Hydroxyvitamin D3 1-alpha-Hydroxylase↗
PubMedDate not supplied

[Structural properties and lipid-binding characteristics of plasma fibrinogen and gamma-globulins in vitamin D-deficient rickets].

Structure properties and lipid-binding ability of fibrinogen and gamma-globulins were studied in blood plasma of children with rickets. Content of cholesterol and its esters, developing complexes with fibrinogen, was found to increase simultaneously with a decrease in the phospholipids, containing in the protein fraction, under conditions of the disease. Surface charge of the proteins studied, especially of fibrinogen, was altered, thus demonstrating the distinct impairments of their structure and functions. Lipid environment appears to be of importance in functional activity of these proteins.

Amino Acids↗
PubMed1989

Adult hypophosphataemic osteomalacia with Fanconi syndrome presenting in a patient with neurofibromatosis.

A patient is described with neurofibromatosis, increasing walking problems and low back pain. Initially, osteoporosis or pressure caused by a neurofibroma was suspected. However, the progressively increasing alkaline phosphatase activity and hypophosphataemia found during laboratory investigations led to metabolic studies which revealed proximal tubular dysfunction. A bone biopsy showed severe osteomalacia. A diagnosis of hypophosphataemic osteomalacia was made based on the Fanconi-syndrome. The association between hypophosphataemic osteomalacia and neurofibromatosis may be akin to the relationship between this type of osteomalacia and mesenchymal tumours, which has been noticed several times in the literature. The underlying mechanism is unknown.

Fanconi Syndrome↗
PubMed1989

[Diagnostic significance of calcitonin analysis in children with rickets and rickets-like diseases administered calcium test].

The secretory activity of C-cells of the thyroid was investigated in 25 children with rickets and rickets-like diseases. There were 13 children with grades II and III rickets, 6 with vitamin D-dependent rickets, and 6 with Debre-de Toni-Fanconi disease. Five normal children constituted a control group. The basal concentration of calcitonin did not differ from the control parameter in all patients' groups. An increased release of the hormone to the blood was noticed in response to calcium stimulation. The rise of blood calcitonin was determined by the disease pattern, which made it possible to use the test with a one-minute intravenous calcium injection for differential diagnosis purposes. The maximal changes were discovered in children suffering from vitamin D-dependent rickets.

Calcitonin↗
PubMed1989

Micromorphologic features of dentin in vitamin D-resistant rickets: correlation with clinical grading of severity.

This study was a histological analysis of 20 primary teeth from 5 patients with 3 clinical grades of vitamin D-resistant rickets (VDRR). The results showed that the degree of globular dentin formation in the histological sections may be graded into Grades I-III in increasing order of severity. In Grade I, the amount of globular dentin was less than 50% of the total dentin thickness, and the interglobular spaces were small. By contrast, in Grade III severity, globular dentin extended throughout the entire thickness of dentin, and the interglobular spaces were large. In Grade II severity, the amount of globular dentin was more than half but did not involve the entire dentin thickness. These histologic grades of severity correlated directly with the clinical grades of the patients. In addition, the study found that lack of medical treatment in affected mothers might lead to globular dentin formation in the fetus in-utero. Conversely, adequate phosphate supplementation in a hypophosphatemic mother might prevent the formation of globular dentin in the fetus.

Adolescent↗