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Biomedical subjects

G Delling

Publications and source records attributed to G Delling.

At least 217 records · Page 12Linked to original sources

[Renal osteodystrophy in children. Therapy with 1,25-dihydroxy-cholechalciferol (author's transl)].

Growth arrest and renal osteodystrophy are major problems in renal insufficiency of children. The present report describes our experiences in managing renal osteodystrophy in 14 dialyzed children using 1,25-DHCC for 12 months. Values in plasma of Ca, P, Mg, alkaline phosphatase, iPTH, 25-OH-D, and 1,25-DHCC were determined regulary. Skeletal X-rays and analysis of iliac crest biopsies were obtained in each child. In treatment with 1,25-DHCC episodes of severe but reversible hypercalcemia occurred. Alkaline phosphatase and iPTH normalized completely. Radiographic examinations revealed marked improvement. Histological signs of fibro-osteoclasia and resorptive defects disappeared but there was no recovery of osteomalacia. A reduction of osteoblast population and of bone transformation was obvious. 1,25-DHCC failed to normalize growth in uremic children. In short, neither vitamin D nor 1,25-DHCC can guarantee complete recovery of renal osteodystrophy and growth arrest in uremic children.

Adolescent↗

[Influence of vitamin D therapy on renal osteodystrophy in children (author's transl)].

Growth arrest and renal osteodystrophy is a major problem in renal insufficiency of children. The present report describes our experiences in managing renal osteodystrophy by using vitamin D3 for 24 months. Values in plasma of Ca, Mg, alkaline phosphatase, iPTH, 25-OH-D were determined regularly. Skeletal X-rays and analysis of iliac crest bone biopsies were obtained in each child. In treatment with vitamin D3 no hypercalcemia was seen despite high serum levels of 25-OH-D. Plasma-Ca, alkaline phosphatase, and iPTH normalized nearly. Radiographic abnormalities improved. Bone biopsies showed improvement in signs of secondary hyperparathyroidism and ostitis fibrosa, whereas osteomalacia remained unchanged. Osteoblast population showed a small reduction. No real increment in body growth was seen.

Alkaline Phosphatase↗

The action of 1,25 (OH)2D3 on turnover kinetic, remodelling surfaces and structure of trabecular bone in chronic renal failure.

Fibroosteoclasia as well as osteoidosis are reduced by 1,25(OH)2D3 treatment if secondary hyperparathyroidism preexists. Fibrosteoclasia completely disappeared after 6 months therapy in some cases. 1,25(OH)2D3 has no or only a very slight effect on the disturbed mineralization in type II of renal bone disorder (osteoidosis only, no signs of secondary hyperparathyroidism). The appositional rate of the osteoblasts increases under 1,25(OH)2D3 treatment if serum PTH values are raised. 1,25(OH)2D3 seemed to have, in the chosen dosage of this study, a self-limiting effect by reducing the bone-forming cells, i.e. the osteoblasts. This state already represents an overtreatment of the underlying bone disease.

Adult↗

[Treatment of renal osteopathy with 5,6-trans-25-hydroxycholecalciferol (author's transl)].

The influence of 5,6-trans-25-hydroxycholecalciferol on renal osteopathy was investigated in a total of 132 patients in 26 dialysis centres. Various doses were used, the average being 4000-6000 IU/day. In 32 patients a daily dose of 6000-9000 IU was used. The average individual duration of treatment was 276 days with a maximum of 910 days. Histologically there was an improvement in the renal osteopathy in 55.9% of evaluable cases (n = 34) and in 25.3% there was no deterioration. Radiographically these results were found in 21% and in 70.5% of evaluable cases (n = 105). Serum calcium increased in 46.6% of cases (n = 131), remained the same in 32.8% and decreased in 20.6%. The changes in alkaline phosphatase were similar : it dropped in 42.1% of patients, remained the same in 28.1% and rose in 29.8%. Immunoreactive parathormone which was invariably raised at the beginning of treatment (n = 36), fell in 25.0%, remained the same in 44.4% and rose further in 30.6%. The clinical symptoms of renal osteopathy which had been present in 57 patients improved in 51.0%, remained the same in 46.0% and deteriorated in 3.0%. Signs of intolerance and side effects were rare. Severe hypercalcaemia did not occur.

Adolescent↗

Shwachman's syndrome and leukaemia.

The clinical and morphological characteristics of Shwachman's syndrome (exocrine pancreatic insufficiency, pancytopenia, skeletal changes) were observed in a boy who, at the age of 8 years, developed a juvenile form of chronic myeloic leukemia which did not respond to cytostatic treatment. Autopsy revealed a striking lipomatous atrophy of the pancreas, defects in the ossification zones of the bones and marked dwarfism. In addition there was leukaemic infiltration of the pancreas, the spleen, the liver and the lymph nodes. The association of Shwachman's syndrome with leukaemia is a rare, but remarkable complication of this entity because of its relationship to the preceeding pancytopenia. Thorough follow-up of the haematological status of patients with Shwachman's syndrome is recommended.

Autopsy↗

Histomorphometric analysis of bone changes in surgically proven primary hyperparathyroidism and nephrolithiasis--the importance of bone biopsy in diagnosis.

The morphologic changes in trabecular bone were studied in 60 patients with surgically proven hyperparathyroidism and in 69 patients with nephrolithiasis. The hyperparathyroid bone lesions showed substantial variation in their extent. Four, typical stages were defined. The structure of trabecular bone remained intact in most cases. Bone turnover is significantly higher in the patients with primary hyperparathyroidism. Fifty percent of all patients with nephrolithiasis had bone changes similar to those found in the surgically proven hyperparathyroidism group. In 50% of so-called asymptomatic cases of hyperparathyroidism, the iliac crest biopsy is a useful supplement to clinical and hormonal data in deciding whether to operate on the parathyroid glands. In about 45% of cases, however, no definite diagnoses is possible. The determination of serum parathyroid hormone in primary hyperparathyroidism has a greater importance for diagnostic purposes than morphologic investigation of the bone biopsy.

Adolescent↗

Renal bone disorders in children: therapy with vitamin D3 or 1,25-dihydroxycholecalciferol.

Twelve children with chronic renal failure (CRF) and sixteen children receiving regular dialysis therapy (RDT) were treated with between 10,000 and 50,000 IU of vitamin D daily. This was associated with an increase in serum calcium levels and reduction in PTH levels. In the children with CRF, secondary hyperparathyroidism was improved with treatment but its development was not completely prevented nor was healing complete. In the patients receiving RDT, treatment with vitamin D improved the changes associated with secondary hyperparathyroidism in 50% of cases but these features sometimes reappeared despite continuing treatment. Hypercalcaemia or metastatic calcification was not seen. Subsequently, 1,25(OH)2D3 was administered to 14 children receiving RDT. This was associated with the return of serum calcium levels to normal, inhibition of PTH synthesis and an improvement in intestinal calcium absorption. Fibro-osteoclasia was cured and there was improvement in actual bone resorption. There was also improvement in osteoidosis in those children who showed disturbances of mineralisation. Calcification in the limbus area of the eyes may occur and hypercalcaemia was seen commonly. Treatment with 1,25(OH)2D3 should only be offered to children with severe renal bone disease. Neither vitamin D3 nor 1,25(OH)2D3 can guarantee complete recovery of osteodystrophy and of growth arrest in uraemic children.

Adolescent↗

[Reaction of articular cartilage to subchondral defect filling with autologous cancelous bone, Kiel's bone chips and bone cement].

Equally sized subchondral osseous defects were produced in 30 adult rabbits. The defects were filled with autologous cancellous bone, bone cement, or macerated heterologous cancellous bone. Twelve weeks later those filled with autologous cancellous bone showed consolidation with preservation of the subchondral border line. There were no degenerative changes of the joint's cartilage. Filling with bone cement or heterologous cancellous bone, on the other hand, led to destruction of the joint's cartilage. The defects treated with heterologous cancellous bone had not reached osseous consolidation at that time. These results suggest that effective treatment of subchondral osseous defects can only be achieved by filling with autologous cancellous bone.

Animals↗

[A rare case of so-called idiopathic osteolysis associated with a lymphangioma of the fibula (author's transl)].

In a 70-year old female patient, a so-called idiopathic massive osteolysis with destruction of the proximal right femur within six months, was observed. Histologically, the following conditions were found at the time of resection: a stroma rich in fibres, with the inclusion of blood vessels, as well as an osteoclastic absorption which was still clearly active at the margins, besides marginally defined processes of bone formation. A good functional result without progressing osteolysis at the femur was achieved via a total endoprosthetic replacement of the right hip joint. Within the following three years, polycystic intramedullar osteolysis developed from a slight loosening of the spongiosa in the right head of the fibula. In contrast to the changes which had occurred at the femur, this was a clear case of a rare intraosseous lymphangioma.

Aged↗

Morphology of osteosarcoma: new qualitative and quantitative investigations.

Results on seven cases of osteosarcoma are reported, based on new morphologic methods and quantitative procedures. Tumor tissue was embedded without prior decalcification in plastic and sectioned. Imprint cytology preparations were produced from fresh tumor tissue, and cell nuclei were measured with an electronic image analysing computer system. The loss of differentiation seen in osteosarcomas differs among osteoblasts, osteocytes, and osteoclasts. The differentiation of osteoclasts, namely their resorptive characteristics, disappears relatively early. Tumor osteocytes show loss of differentiation in their osteocyte processes. The new formation of tumor bone tissue remains in the near normal range of volume density when nuclear polymorphy is limited. The formation of ground substance and mineralization are apparently closely couplet to one another, since in our cases mostly ordered osteoid seams were observed. The capacity for mineralization of bone tissue is lost with marked polymorphy. The significance of these results for diagnostic statements and therapeutic consequences will be further discussed in long term studies.

Adolescent↗

[The coincidence of Paget's disease and an immunoblastic sarcoma (author's transl)].

Malignant change is to be expected in about 1% of patients with Paget's disease. Usually these are osteosarcomas. Fibrosarcomas, benign and malignant giant cell tumours and chondrosarcomas are less common. The present case concerns the development of an immunoblastic sarcoma in the humerus, which was affected by Paget's disease. Only one other similar case is recorded in the literature. Other myelogenous tumours, such as plasmocytomas, are very rare in association with Paget's disease. Consequently there is no evidence for a causal connection between Paget's disease to these tumours, whereas sarcomas arising from bine tissue may be regarded as a form of malignant degeneration of Paget's disease.

Bone Marrow Diseases↗

Ultrastructural study of tumor cell differentiation in osteosarcoma of jaw bones.

Tw osteosarcomas of jaw bones have been studied by electron microscopy. The objectives were to examine the specific cell types in relation to functions and ultrastructural features, and to examine matrices produced by tumor cells. The osteosarcoma cells were subdivided into four cell types: anaplastic, chondroblastic, osteoblastic, and osteocytic--giant cells were not considered in the present investigation. Compared to normal bone cells, no specific sign of malignancy was found. However, tumor cells seem to lose functional abilities, i.e. a modification of matrix. Consequently, tumor matrix has altered organic and inorganic components with impairment of collagen maturation and matrix mineralization. The alteration in both processes may be related to a diminished production of proteoglycans. The cytogenic hypothesis of a tumor stem cell may be supported by the identification of anaplastic osteosarcoma cells resembling immature reticulum cells. One may speculate on transformation of this cell type as a genetically predetermined osteoprogenitor cell of malignant potential.

Adult↗

Kinetics of PTH metabolism and PTH fragments in chronic renal failure.

Distribution and metabolic degradation of bPTH was studied in man after infusion of 400 U bPTH and blood sampling up to 2h. Disappearance rates of intact 1-84 bPTH, carboxyl- and amino-regional peptides were calculated for healthy subjects (n = 12); patients suffering from moderate (GFR 15--30 ml/min, n = 4) and advanced (GFR less than or equal to 10 ml/min, n = 36) chronic renal failure; and bilateral nephrectomized patients (n = 3). Two components with a rapid and a slow disappearance rate can be separated ("distribution", "metabolism"). Half-lives are found to be in the range known from animal experiments with marked differences between intact 1-84 PTH and peptide fragments. Influence of impaired renal function on metabolic turnover rates of PTH is given.

Alkaline Phosphatase↗

1,25-dihydroxycholecalciferol in hypophosphataemic osteomalacia presenting in adults.

The effects of exogenous 1,25-dihydroxycholecalciferol (1,25(OH)2D3) in two adults with late-onset hypophosphataemic osteomalacia were studied. In the presence of elevated 25-hydroxyvitamin D levels 1,25OH)2D3 improved intestinal 47Ca absorption and increased urinary calcium, phosphate and hydroxyproline excretion. Hypophosphataemia, parathyroid hormone and bone mineralization were not significantly affected. The rise of the 1,25-dihyroxyvitamin D concentrations was dose dependent. The results indicate htat the impared renal phosphate conservation--the primary defect in this disorder--is not corrected by exogneous 1,25-dihydroxycholecalciferol.

Adult↗