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G A Bruyn

Publications and source records attributed to G A Bruyn.

41 records · Page 3Linked to original sources

Cranial nerve palsy as the presenting feature of secondary plasma cell leukemia.

A patient with IgA lambda multiple myeloma (MM) developed plasma cell leukemia (PCL), presenting as oculomotor nerve palsy. The cerebrospinal fluid (CSF) contained plasma cells, which double stained with fluoresceinated anti-IgA and anti-lambda antisera. The palsy was most probably due to meningeal myelomatosis. The neurologic disorder appeared to be refractory to the therapy used, although plasma cells disappeared from the peripheral blood. Secondary plasma cell leukemia is a rare complication of MM, usually occurring in the terminal stage of the disease. Those patients may be eligible for central nervous system (CNS) prophylaxis, as is commonly performed in patients with other types of leukemia.

Bone Marrow↗

Chronic intestinal pseudoobstruction as a possible sequel to encephalitis.

A patient is reported who presented with a typical intestinal pseudoobstruction syndrome. Before this illness, the patient had suffered from measles encephalitis at the age of 15 months. A postencephalitic syndrome was present which included severe mental retardation, parkinsonism, and epilepsy. There were no relatives with a similar disease. Clinically, there was a frank recurrent pseudoobstruction syndrome with occasional diarrhea. Radiographic barium studies showed delayed transit, hypomotility of the intestines, and gross distension of the esophagus, stomach, small intestine, and colon. Histological examination of the gastrointestinal tract revealed a normal appearance of the mucosa; however, there was a hypertrophic muscle layer with abnormalities of the plexuses. Both the submucosal and the myenteric plexuses were reduced in number and size. They showed a decreased number of ganglion cells, proliferation of Schwann cells and infiltration by lymphocytes. The abnormalities were most strikingly present in the esophagus and the small intestine. The intestinal neuropathy resulting in clinical pseudoobstruction is proposed to be part of the generalised neurological pathology as a late sequel to the measles encephalitis.

Adult↗

Pneumococcal bacteremia in adults over a 10-year period at University Hospital, Leiden.

The medical records on all cases of pneumococcal bacteremia in adults at the University Hospital, Leiden, over a 10-year period (1976-1986) were retrospectively reviewed. In this series of 147 episodes (an annual incidence of 0.8 episodes/10,000 adults), overall mortality was 25.9%. Factors significantly related to a higher mortality rate were shock, respiratory insufficiency, preexisting renal failure, and rapidly fatal underlying disease. Several laboratory abnormalities-such as a low percentage of band forms, an elevated level of serum lactate dehydrogenase, and hyperbilirubinemia-were significantly related to a poor outcome. Multilobar pneumonia and meningitis were both associated with high mortality, although not to a statistically significant degree. Discriminant analysis showed the presence of shock as the most powerful predictive factor of death. Surprisingly, prior splenectomy did not correlate with higher mortality. Treatment with beta-lactam antibiotics favorably influenced the outcome of illness in patients with ultimately fatal and nonfatal underlying disease, while the use of these agents in patients with rapidly fatal underlying disease did not correlate significantly with a good prognosis.

Adolescent↗

Risk assessment for osteoporosis by quantitative ultrasound of the heel in ankylosing spondylitis.

OBJECTIVE: The aim of this cross-sectional cohort study is to assess the potential of quantitative ultrasound (QUS) of the calcaneus in pre-screening for vertebral/non-vertebral fractures, and in discriminating osteoporotic from normal bone density in patients with ankylosing spondylitis (AS); a second objective is to determine the prevalence of osteoporosis using dual-energy X-ray absorptiometry (DEXA) in this patient group. RESULTS: Included are 50 consecutive AS patients with no history of osteoporosis: mean (SD) age 52 (12) yrs, range 26-75 yr; female/male ratio 15/35. The mean (SD) DEXA T score in the lumbar spine (AP view) was -0.82 (1.73), mean (SD) DEXA T score in femoral neck -1.46 (1.12). The mean (SD) calcaneal QUS T score was -0.73 (0.95). In our population of AS patients the prevalence of femoral neck osteoporosis according to the WHO definition (DEXA T < -2.5) was 20%. Osteoporosis criteria were met at the femoral neck in 10 (20%) patients, and 7 of them (70%) were correctly diagnosed using QUS, with T < -1.0 as cut-off value; normal bone density at the femoral neck was found in 15 AS patients (30%), yet in 2 of them the calcaneal QUS T was < -1.0. In AS the 20% pretest probability of having femoral neck osteoporosis increased using calcaneal QUS, with a cut-off level T < -1.0 (70% sensitivity, 68% specificity), and then rose to 35% as the predictive value of a positive test, yielding a net result of QUS testing of +15%. The predictive value of a negative QUS test result was 90%, which makes QUS applicable to exclude severe osteoporosis. Vertebral and/or non-vertebral fractures occurred in 12 out of 50 AS patients (24%); 5 of them (10%) were associated with osteoporosis as defined by WHO criteria measured via DEXA. CONCLUSION: The performance of QUS is similar to DEXA in finding patients with osteoporosis-associated fractures: the sensitivity of QUS T < -1.0 in finding the fracture is 80%, and the sensitivity of femoral neck DEXA T < -2.5 in finding fractured patients is 60%. We conclude that both osteoporosis and fractures are common sequelae in AS. Calcaneal QUS offers a promising approach to screen for osteoporosis, and may be applied to exclude osteoporosis-associated high fracture risk in AS.

Adult↗