Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0020500 (hyperoxaluria)
 912 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

4 patients with nephrocalcinosis were treated with disodium ethane 1-hydroxy-1, 1-diphosphonate (EHDP) for a period of 13 months. No clinical side-effects were observed and growth proceeded normally. Radiographic changes of osteitis fibrosa cystica developed in 1 child and bone biopsy in 2 children showed defective osteoid mineralisation. It is suggested that EHDP prevented further crystal deposition in 3 children but did not prevent non-calcium stone formation in the 4th child. In view of this and the development of histological and radiographic evidence of osteomalacia and/or secondary hyperparathyroidism in these patients the value of EHDP remains dubious. On the other hand its use may be justified when rapidly increasing calcification is expected, as for example in hyperoxaluria.
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PMID:Diphosphonate therapy in nephrocalcinosis. 82 72

Bone biopsy specimens from four patients with hyperoxaluria who underwent hemodialysis were studied. Calcium oxalate crystals are laid down in marrow spaces and sometimes inside the bone matrix and uncalcified osteoid tissue. They are clearly visible by polarizing microscope and are stained grayish-brown by Pizzolato's method. Most are surrounded by basophilic, amorphous material. By electron microscope they appear as elongated, empty spaces and after hydrogen peroxide treatment appear as fragmented, slightly electron-dense, needle-like structures. In marrow spaces, oxalate crystals aggregate in round clusters surrounded by a granulomatous reaction. This, however, cannot remove the oxalate crystals. Bone histology shows advanced renal osteodystrophy, ie, severe osteomalacia and hyperparathyroidism. The granulomatous reaction induced by the oxalate crystals probably contributes to and worsens the changes from hyperparathyroidism.
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PMID:Bone oxalosis and renal osteodystrophy. 689 47

Intestinal bypass was a popular surgical procedure for morbid obesity resulting, on average, in a 50 kg weight loss. We describe a 66-year-old woman who underwent the procedure 12 years earlier and subsequently presented with recurrent episodes of erythema nodosum-like lesions. Further investigations revealed hyperoxaluria, renal failure, deficiency of fat-soluble vitamins (causing night blindness, osteomalacia and easy bruising) and anaemia. Antibiotics led to only temporary remission and, as with 24-30% of similar cases, she underwent surgical reversal to prevent the complications from worsening.
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PMID:Intestinal bypass syndrome presenting as erythema nodosum. 1511 6

A 58-year-old woman with a surgical history of jejunoileal bypass in 1980 for weight reduction sought medical attention with multiple complaints. The patient had not been taking any nutritional supplements since her bypass surgery, 26 years previously. She was found to have osteomalacia, chronic diarrhea, secondary hyperparathyroidism, and hyperoxaluria with a frequent history of nephrolithiasis. Because of her severe osteodystrophy and metabolic complications, reversal of her jejunoileal bypass was recommended. Reversal of the jejunoileal bypass with a sleeve gastrectomy was performed. Laparotomy revealed brown discoloration of the entire alimentary limb with atrophy of the bypassed intestinal limb. Histologic examination of the resected small bowel demonstrated brown pigment deposits within smooth muscle cells of the bowel wall. The pigment stained positive with Fontana-Masson most likely representing lipofuscin. We report a case of brown bowel syndrome complicating jejunoileal bypass, the first case reported in the literature to the best of our knowledge.
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PMID:Brown bowel syndrome secondary to jejunoileal bypass: the first case report. 1950 85