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PubMed1988

[Biologico-mechanical behavior of bone in osteomalacia and in phosphate diabetes in adulthood].

The main orthopedic complications in osteomalacia, skeletal deformities, Looser's transformation zones and fractures, are described. The limited value of a Looser's transformation zone as a radiographic diagnostic criterion for osteomalacia is mentioned, because such zones can also occur in other bone diseases with abnormal remodeling. This paper also outlines the differential diagnosis of a Looser zone against a stress fracture. In osteomalacia and phosphate diabetes the biological and mechanical properties of bone are different. In adult phosphate diabetes radiological examination is more likely to reveal osteosclerosis than a radio-opaque bone. For this reason we investigated the bone healing process in five adult patients with osteomalacia and in six with phosphate diabetes. In spite of the small number of patients we may assume that bone healing after surgery is delayed in phosphate diabetes compared with osteomalacia, in which a normal bone healing tendency is present.

Adult↗
PubMed1988

Effect of phosphorus supplementation on bone formation induced by osteosarcoma-derived bone-inducing substance in X-linked hypophosphatemic mice.

In our previous report, we demonstrated normal cartilage and bone matrix formation and a defect of bone mineralization in hypophosphatemic (Hyp) mice using an ectopic bone formation system. That system consisted of an osteogenic sarcoma-derived bone-inducing substance. In this report, we describe the effect of phosphorus supplementation on abnormal bone mineralization. The osteogenic sarcoma-derived bone-inducing substance was implanted in Hyp mice or control mice. The Hyp mice were divided into two groups after implantation. One group was fed a normal laboratory chow, while the other was fed a high-phosphorus diet for 4 weeks of the experimental period. Normal control mice were fed the normal laboratory chow. The mean serum phosphorus level in the high-phosphorus diet group was normal at 2, 3 and 4 weeks after implantation. Using the method of 85Sr incorporation, the high-phosphorus diet group showed marked improvement in bone mineralization at 2 and 4 weeks after implantation, but incomplete improvement at 3 weeks. On the other hand, histological study of the high-phosphorus diet group at 4 weeks after implantation still showed a meaningful amount of the osteoid matrix formation compared to the control. These findings suggest that the abnormal bone mineralization in Hyp mice was mainly due to their abnormally low serum phosphorus level. However, still other abnormalities might exist and these might be responsible for the incomplete improvement in bone mineralization.

Animals↗
PubMed1988

Ovariectomy abolishes the normalization of femoral mineral content in 40-week-old female X-linked hypophosphatemic mice.

All young adult X-linked hypophosphatemic (Hyp) mice exhibit low bone mineral content. However, between the ages of 13 and 40 weeks, the female Hyp mice have an increase in bone mineral content per centimeter length to the level of the normal mice. Older Hyp male mice do not normalize in mineral content/length ratio. To explore the mechanisms for this, gonadectomy was performed in 3- to 4-week-old normal and Hyp mice to determine whether the increase in mineral content was related to gonadal secretions. Sham-operated and gonadectomized mice of both sexes and genotypes were sacrificed at 7, 13, and 40 weeks of age. Gonadectomy had little effect on the development of low mineral content in young adult Hyp mice. However, gonadectomy blocked the increase in femoral mineral content and mineral content/length ratio in Hyp female mice at 40 weeks of age. Gonadectomy blocked the increase in femoral mineral content in Hyp females while femoral length was increased somewhat. Femoral midshaft diameter was significantly smaller in Hyp female mice, which suggested a decrease in periosteal bone growth due to gonadectomy between 13 and 40 weeks of age. In general, plasma phosphate and calcium values could not be used to explain bone mineral increases since no consistent changes were found. In conclusion, the increase in bone mineral content which occurs in older Hyp female mice is dependent upon the presence of the ovary.

Absorption↗
PubMed1988

Primary cultures of renal epithelial cells from X-linked hypophosphatemic (Hyp) mice express defects in phosphate transport and vitamin D metabolism.

Mutation in a gene (symbol Hyp) on the X chromosome causes hypophosphatemia in the mouse. The murine phenotype is a counterpart of X-linked hypophosphatemia in man. Both exhibit impaired renal reabsorption of phosphate in vivo. In vitro studies in the Hyp mouse have shown decreased Na+-dependent phosphate transport at the brush border membrane and abnormal mitochondrial vitamin D metabolism. To determine whether the mutant renal phenotype is intrinsic to the kidney or dependent upon putative extrinsic humoral factor(s) for its expression, we established primary cultures of renal epithelial cells from normal and Hyp male mouse kidneys. The cells are derived from proximal tubule. Initial uptake rates of phosphate and alpha-methyl-D-glucopyranoside (alpha-MG), a metabolically inert analogue of D-glucose, were measured simultaneously in confluent monolayers exhibiting epithelial polarity and tight junctions. The mean phosphate/alpha-MG uptake ratio in Hyp cultures was 82% of that in normal cells (P less than 0.01, n = 96). Moreover, the production of 24,25-dihydroxyvitamin D3 was significantly elevated in confluent cultures of Hyp cells relative to normal cells. These results imply that the Hyp gene is expressed in situ in renal epithelium and suggest that humoral factors are not necessary for the mutant renal phenotype in X-linked hypophosphatemia of mouse and man.

Animals↗
PubMed1987

Severe hypophosphatemia associated with diabetes mellitus in six dogs and one cat.

Severe hypophosphatemia was found in 6 diabetic dogs and in one diabetic cat. The cat suffered from hemolysis, and one dog had seizures, both apparently as a result of the severe hypophosphatemia. Clinical signs were not determined solely by the serum concentration of phosphorus, as seen in 5 other patients that did not have signs of disease despite similar serum phosphorus concentrations.

Animals↗
PubMed1987

[Phosphate diabetes and pregnancy].

An account is given on pregnancy, delivery and childbed of a 26 year old phosphate diabetic. Nothing but regular control of the required laboratory data allows a variable substitution of necessary medications as presupposition for an undisturbed course of pregnancy and development of the fetus.

Adult↗
PubMed1985

Hypophosphatemic rickets, hyperparathyroidism, and hyperthyroidism with hypercalcemia.

We describe a case of hypophosphatemic rickets in which hyperparathyroidism and hypercalcemia developed and were possibly secondary to co-existent hyperthyroidism. During the period of hypercalcemia with high immunoreactive parathyroid hormone levels the serum inorganic phosphorus level became, and remained, normal. This supports the concept that renal tubular phosphate absorption may be sensitive to calcium.

Adult↗
PubMed1985

Changing patterns of femoral and skeletal mineralization during growth in juvenile X-linked hypophosphatemic mice.

Femoral and skeletal mineralization were studied in normal and X-linked hypophosphatemic (Hyp) mice from birth to 13 weeks of age. Although the Hyp mice were continuously hypophosphatemic, their body weight, trunk length, femoral ash weight and total body calcium content were not significantly affected through 3 weeks of age. Tail length and femoral length were depressed by less than 10%. Between 3 and 7 weeks of age the normal mice continued their rapid growth, but there was little further lengthening of the femur or tail in the Hyp mice; body weight and trunk length fell behind the normals; and there was no further accumulation of femoral ash weight or total body calcium and little increase in total body phosphate. After 7 weeks of age the Hyp mice resumed accumulation of femoral and total body mineral.

Aging↗