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At least 1,513 records · Page 84Linked to original sources
PubMed1996

Craniodiaphyseal dysplasia; another cause of difficult intubation.

A nine-year-old boy with craniodiaphyseal dysplasia (CDD) presented for mandibular reduction. Patients with CDD present problems to the anaesthetist, specifically difficulties with airway management and tracheal intubation. This child was managed using laryngeal mask airway (LMA) guided fibreoptic intubation. Spontaneous respiration was maintained throughout intubation, following which ventilation was controlled and anaesthesia was provided using nitrous oxide, isoflurane and fentanyl. The perioperative management is described.

Anesthesia, Inhalation↗
PubMed1995

Rothmund-Thomson syndrome in fraternal twins.

Fraternal twins of Malay descent had the Rothmund-Thomson syndrome. This is a rare, autosomal recessive disorder characterized by photosensitivity, poikiloderma, short stature, skeletal defects, and juvenile cataracts. This is the first case report of the syndrome from southeast Asia.

Adolescent↗
PubMed1983

Hereditary factor VII deficiency in a Chinese family.

Hereditary deficiency of factor VII is demonstrated in a Chinese family. The proposita was a 32-year-old female with bleeding diathesis consisting of spontaneous ecchymosis, menorrhagia and recurrent haemarthrosis. The prothrombin time was prolonged and the prothrombin and proconvertin test was 10% of normal. The activated partial thromboplastin time and the Stypven-cephalin clotting time were normal. The prolonged prothrombin time could be corrected by the addition of normal serum, but not by adsorbed normal or coumadin plasma. The factor VII level was 3.6% of normal. One of her brothers had bleeding symptoms and died at age 25, suggesting that factor VII deficiency might have been present. 13 of her family members had partial deficiency of factor VII with plasma levels ranging from 24 to 50%. These results suggest an autosomal recessive inheritance with a homozygous state occurring in the proposita and possibly in her brother, and a heterozygous state occurring in 13 of her family members. Our study marks an extensive survey of factor VII deficiency in an Oriental family.

Adult↗
PubMed1984

'Incomplete' pyroglobulin-gamma disease in a patient with osteosclerotic myeloma.

A 50-year-old female, heterozygous for beta-thalassaemia was found to have a lytic lesion surrounded by osteosclerotic tissue in the 1st lumbar vertebra. Aspiration of the lesion showed 100% atypical plasma cells. The bone marrow contained 17% myeloma cells. Despite normal electrophoresis and immunoelectrophoresis of serum and urine, 'rouleaux' formation was pronounced. Treatment of the serum sample with 2-mercaptoethanol and heat (56 degrees C) disclosed an uncommon pyroglobulin. Analysis of the ammonium sulphate precipitate of the serum by sodium-dodecyl-sulphate polyacrylamide gel electrophoresis revealed a 43 kD component with higher anodic mobility than normal gamma chains. Ultrafiltration column chromatography of the serum revealed a narrow spike of approximately 4 S that contained gamma heavy chain antigenic determinants in addition to normal 7 S IgG.

Electrophoresis, Polyacrylamide Gel↗
PubMed1987

Urinary hydroxyproline excretion in the myelofibrosis-osteomyelosclerosis syndrome and related diseases.

The total urinary hydroxyproline excretion was assessed in 47 patients with chronic myeloproliferative disorders. Urinary hydroxyproline excretion was normal in 16 patients with idiopathic myelofibrosis and in 5 out of 6 patients with acute myelofibrosis. In patients with osteomyelosclerosis (n = 8) values for urinary hydroxyproline excretion were higher (median 202, range 54-652) than those in idiopathic myelofibrosis (median 139, range 84-216). This difference was not significant (p greater than 0.1). Elevated values for urinary hydroxyproline excretion were found in 10 patients (1 AMF patient, 3 OMS patients and 6 patients with CML in the accelerated phase of the disease). All but 1 of these patients had been treated, or were being treated, with cytotoxic agents at the time of investigation. These findings are compatible with impaired degradation of bone marrow collagen which, together with enhanced collagen synthesis from bone marrow fibroblasts, accounts for progressive accumulation of connective tissue in the bone marrow. This process appears to be influenced by cytotoxic treatment as reflected in increased urinary hydroxyproline excretion in those patients receiving cytotoxic agents.

Bone Marrow↗
PubMed1982

Panosteitis in a cocker spaniel dog.

A 12-month-old, desexed female Cocker Spaniel had intermittent bilateral foreleg lameness which was due to panosteitis of the radius and ulna. The disease was in the active 'middle' state. Endosteal and periosteal new bone formation was demonstrated by histological examination and accounted for the diffuse osteosclerotic appearance of the radial and ulnar diaphyses seen on radiographs.

Animals↗
PubMed1993

A half-century of community water fluoridation in the United States: review and commentary.

The nearly 50-year history of community water fluoridation is reviewed with the major emphasis on the benefits and safety of fluoridation. Other aspects of water fluoridation also described include the apparent reduction in measurable fluoridation benefits because of the abundance of other fluoride sources, the diffusion of fluoridation effects into fluoride-deficient communities, preeruptive and posteruptive effects, technical and cost aspects, sociopolitical and legal issues that affect the successful fluoridation of communities, and alternatives to community water fluoridation. The majority of studies have evaluated the effectiveness of water fluoridation on the permanent teeth of children, while there are fewer studies on deciduous teeth and in adults; the relationship between fluoride ingestion and bone health needs further clarification; the sociopolitical issues of fluoridation need to be better understood.

Cost-Benefit Analysis↗
PubMed1982

Type II collagen autoimmunity in otosclerosis and Meniere's Disease.

Antibodies to collagen types I, II, and IV were measured in patients with otosclerosis and patients with Meniere's disease. Levels of antibodies to type II collagen were significantly higher in these patients than in control subjects, while no differences were found among levels of antibodies to collagen type I or type IV. These observations suggest a possible role for type II collagen autoimmunity in the etiology of otosclerosis and Meniere's disease.

Autoantibodies↗
PubMed1990

Paradoxical bone mineralisation in the twin to twin transfusion syndrome.

We report twin preterm infants with the twin to twin transfusion syndrome, exhibiting grossly different bone densities on chest radiographs. Photonabsorptiometry showed the polycythaemic twin was osteopenic and the anaemic twin osteosclerotic; bone mineral contents were 0.028 g/cm and 0.074 g/cm respectively (normal mean (SD) 0.041 (0.006) g/cm. We speculate that alterations in macrophage derived osteoclastic activity contribute to these previously unreported findings.

Absorptiometry, Photon↗
PubMed1992

Scintigraphic assessment of osteoarthritis of the knee joint.

Clinical, radiographic, and scintigraphic abnormalities of the knee joint have been correlated in a cross sectional study of 100 patients with osteoarthritis. The group comprised 73 women and 27 men with a mean age of 65.7 years. One hundred and ninety one of the 200 knees had clinical (175) or radiographic (185) evidence of osteoarthritis, or both (161). Scintigraphic images of the knees were obtained 4-5 minutes (early phase) and 2.5-3.5 hours (late phase) after intravenous injection of 600 mBq of technetium-99m diphosphonate. Abnormal images were recorded in 162 knees (81%), and six different patterns were detected. Generalised isotope retention around the knee (early or late phase) was less common than focal areas of uptake around the joint margin (early or late phase) or in the patella or subchondral bone (late phase). Some knees with abnormal scans were normal on radiography (n = 7), or vice versa (n = 21). Different scan patterns correlated with different clinical and radiographic features: the generalised pattern correlated with pain (odds ratio (OR) = 45.1) and osteophytes (OR = 48.3); joint line retention correlated with subchondral bone sclerosis on radiography (OR = 62.1); and subchondral bone retention correlated with more severe radiographic changes. It is concluded that different patterns of scintigraphic abnormality reflect various aspects of the disease process of osteoarthritis.

Aged↗