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PubMed1998

Clinical phenotype of desmosterolosis.

We describe a child with lethal multiple malformations and generalised accumulation of desmosterol. The infant had macrocephaly, a hypoplastic nasal bridge, thick alveolar ridges, gingival nodules, cleft palate, total anomalous pulmonary venous drainage, ambiguous genitalia, short limbs, and generalised osteosclerosis. Gas chromatography-mass spectrometry demonstrated an abnormal accumulation of desmosterol in kidney, liver. and brain. Higher than normal levels of the same sterol were detected in plasma samples obtained from both parents. The biochemical phenotype in this infant is highly suggestive of a novel inborn error of cholesterol biosynthesis caused by an autosomal recessive deficiency of 3betahydroxysterol-delta24-reductase. A phenotypic overlap of this case with Raine syndrome was noted; however, desmosterol accumulation was not found on postmortem tissue samples from a previously reported case of this disorder.

Abnormalities, Multiple↗

PubMed1997

Radiographic features predictive of radiographic progression of hip osteoarthritis.

OBJECTIVES: To evaluate potential radiographic predictors of hip osteoarthritis progression. PATIENTS AND METHODS: A prospective, longitudinal two-year study was conducted in patients meeting American College of Rheumatology criteria for hip osteoarthritis. Hip osteoarthritis progression was defined as a greater than 0.5-mm decrease in joint space width measured using a magnifying glass marked at intervals of 0.1 mm, at the site of maximum joint space narrowing, by a single investigator who was blinded to the chronological order of the radiographs. Radiographic parameters determined at study entry were as follows: presence of osteophytes, osteosclerosis, and subchondral cysts; femoral head migration (superolateral, superomedial, concentric); and severity (joint space width in mm, Kellgren and Lawrence grade, subjective evaluation of joint space narrowing). RESULTS: In the 463 study patients, joint space width decreased from 2.2 +/- 0.8 at baseline to 1.7 +/- 1.0 mm after two years (P < 0.0001). Radiographic progression was seen in 148 patients (32%). Radiologic parameters predictive of disease progression in the multivariate analyses were as follows: CONCLUSION: Our data suggest that a number of baseline radiological features including distribution of joint space loss, subchondral bone production, and severity of joint space loss are predictive of progression of hip osteoarthritis.

Aged↗

PubMed1997

[Idiopathic myelofibrosis: initial features, evolutive patterns and survival in a series of 106 patients].

BACKGROUND: Idiopathic myelofibrosis (IM) is an infrequent myeloproliferative disorder with few large series published in the medical literature. Information on the characteristics of more recently diagnosed patients with IM is scarce. PATIENTS AND METHODS: The initial features, evolutive patterns and survival from 106 patients diagnosed with IM in a single institution between 1975 and 1996 were analyzed. RESULTS: Median age of the series was 64 years (range: 17-89); there were 61 males and 55 females. One third of the patients were asymptomatic at IM diagnosis. The most common presenting symptoms were related to hypermetabolism, anemia and splenomegaly. A palpable spleen was noted in 85% of patients, and 66% had hepatomegaly. Anemia was the most frequent hematologic abnormality (50% of cases). Bone marrow biopsy showed cellular phase IM in 50 patients, IM without osteosclerosis in 39, and osteosclerotic IM in the remaining 17. Evolution into acute leukemia was seen in 14 patients (actuarial probability at 7 years: 20%, 95% CI: 0-40%), 8 patients developed portal hypertension, 5 liver failure without portal hypertension and 14 heart failure. With 62 patients having died, the series' median survival was 59 months (95% CI: 41-75). CONCLUSION: IM is usually diagnosed in the old age. From the histologic point of view, a predominance of the cellular phase is observed at disease presentation. In recent years an increased proportion of patients asymptomatic at diagnosis is observed.

Adolescent↗

PubMed1976

Serum calcium fractions in sheep treated with Solanum malacoxylon.

Sheep treated with a single dose of an extract of the dried leaves of Solanum malacoxylon (SM) at the rate of 0-2 g of leaves per kg liveweight produced a pronounced hypercalcaemia (49 per cent, P less than 0-005) after 24 h, which persisted for at least six days. The ultra-filtrable fraction of the serum calcium rose to approximately the same extent as the protein-bound calcium. This finding is consistent with osteosclerosis and parathyroid atrophy found to occur in cases of experimental SM intoxication. At the dose level given serum inorganic phosphorus was not significantly increased and packed cell volume, serum proteins and ceruloplasmin concentrations remained constant.

Animals↗

PubMed1998

Unclassified sclerosing bone dysplasia with osteopathia striata, cranial sclerosis, metaphyseal undermodeling, and bone fragility.

Sclerosing bone dysplasias are diagnosed on the basis of a characteristic pattern of osteosclerosis and clinical manifestations; in many of them, cause and pathogenesis are still unknown. A 33-year-old man had five fractures of the humerus, tibiae, and femur as a result of mild traumatic incidents that occurred between the ages of 18 and 33 years as well as a remnant of rib fractures without apparent trauma on radiographs. His height was 158 cm (-2.2 SD). Radiographic evaluation showed cranial sclerosis, longitudinal striations in the metaphyses of the femur and tibia, fan-like striation in the ilium, metaphyseal widening in the femur and tibia, and sclerosis of the ribs. The blood chemistry findings, including serum calcium, phosphorus, and alkaline phosphatase, were normal. Biopsy from the ilium showed thick trabeculae composed of woven bone. The coexistence of osteopathia striata, cranial sclerosis, metaphyseal undermodeling, and bone fragility has not been recognized previously. Our case appears to represent a new form of sclerosing bone dysplasia.

Abnormalities, Multiple↗

PubMed1998

[Schnitzler's syndrome].

The authors describe the development of disease in a patient with monoclonal immunoglobulin IgM, urticarial morphoeae and intensive pain in the region of the pelvic bones due to osteolysis and osteosclerosis. The combination of these symptoms corresponds with the so-called Schnitzler syndrome which is analyzed in detail in the discussion. The urticarial manifestations diminish only temporarily during corticoid treatment, other drugs have no effect. 2-chlorodeoxyadenosine, the most effective drug in treatment of m. Waldenström, suppressed the skin manifestations temporarily. However, the concentration of monoclonal IgM did not decline even after two cycles and therefore the authors did not proceed with it during subsequent cycles. The severity of urticaria called for a permanent daily dose of Prednisone. The second symptom, bone pain, was however influenced by the administration of pamidronate (Aredia), using an initial dose of 120 mg per month. Now the patient takes a maintenance dose of 60 mg/month. The authors describe the case to draw attention to a rare cause of urticaria and the possibility to treat bone pain with bisphosphonates.

Humans↗

PubMed1976

Mechanism of the increased splenic erythropoiesis in mice treated with estradiol benzoate.

Pharmacological doses of estrogens induce osteosclerosis of the bone marrow, and depress colony-forming units (CFU's) and platelet and leukocyte counts in mice. A compensatoryincreasts in mice. A compensatory increase in splenic erythropoiesis prevents a fall in the hematocrit. The mechanism of this compensation was investigated as follows, BDF1 female mice were injected subcutaneously thrice weekly for 6 weeks wiht 50 mugg of estradiol benzoate (EB) or sesame oil (SO). Subsequently the hematocrit, red cell mass (RCM), and 4 hour per cent of 59Fe uptake into the femurs and spleens were determined in groups of five mice for 4 consecutive days. The RCM and hematocrit were not significantly different in the two groups. The per cent of 59Fe uptake into the femurs of EB-treated mice was less than 30 per cent of that in SO-treated mice and the per cent of 59Fe uptake into the spleens of EB mice was more than two times that in SO mice. To ascertain whether the increase in plenic erythropoiesis resulted from an increase in the number of splenic erythropoietin-responsive cells (ERC), the 4 hours per cent 59Fe uptake into the spleen was determined in continuously hypertransfused EB and SO mice injected with erythropoietin (Ep). Whereas hypertransfuction depressed the splenic per cent of 59Fe uptake in EB and SO mice equally, injection of Ep increased the per cent of 59Fe uptake into the spleens of of EB mice to greater than two times that of SO mice. Next, the plasma Ep level of mice injected with EB or SO for 2, 4, or 6 weeks was determined after exposure of the animals to hypoxia. Ep titers were greater than three times higher in EB mice than in SO mice, We conclude that at least two mechanisms act to cause the compensatory increase in splenic erythropoiesis after marrow suppression by EB: (1) the Ep levels rise and (2) the splenic ERC population increases. The latter is probably not due to the increased plasma Ep level because it also occurs in mice whose Ep production is suppressed by plethora.

Animals↗

PubMed1961