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Biomedical subjects

G B Theil

Publications and source records attributed to G B Theil.

18 recordsLinked to original sources

Nonexertional heatstroke. Physiologic management and cooling in 14 patients.

Fourteen patients with nonexertional heatstroke were evaluated in a general hospital during the summer of 1980. They were managed according to a prospectively devised protocol designed to effect heat dissipation primarily via convection and evaporation rather than by conduction. The time from entry into the emergency room to the first recorded rectal temperature of less than 103 degrees F (39.4 degrees C) ranged from 34 to 89 minutes (median, 60 minutes). Only one patient died; none had residual neurologic deficits. The use of these methods can result in a low incidence of permanent neurologic impairment and a low fatality rate.

Adult↗

Schönlein-Henoch syndrome in adults.

Nine adult patients, aged from 16 to 70 years, (mean 42 years), with Schönlein-Henoch syndrome were studied during the acute stage of their illness. Each presented with the typical purpuric skin rash, and renal disease was found in every case. Microscopic haematuria and proteinuria were consistently found and red cell casts were seen in urine from five patients. Kidney biopsy performed on nine patients showed focal glomerulonephritis in seven cases and diffuse proliferative nephritis in two. The finding of mesangial deposits of immunoglobulin A (IgA) in seven of eight patients is in agreement with recent reports suggesting that the syndrome is an immune complex disorder. The diffuse proliferative lesion was accompanied by loss of renal function, but there were no fatalities in this series. Corticosteroids administered during the acute stage appear to ameliorate the symptoms, especially the arthralgia and abdominal pain, and may prevent progression of the renal disease in some cases.

Adolescent↗

A new familial disorder of combined lower motor neuron degeneration and skeletal disorganization.

A new familial disorder, inherited as an autosomal dominant trait and characterized by dual features of neurologic degeneration and skeletal disorganization, is reported. The neurologic disease is a degenerative process of lower motor neurons and develops in middle age with progressive muscle weakness and ends in respiratory failure and premature death. Clinical examination, electromyography, and muscle biopsy with histochemical stains are diagnostic. The skeletal disorganization resembles Paget's disease of bone. It is characterized by polyostotic radiographic abnormalities, elevated serum alkaline phosphatase of bone origin, abnormal radioisotopic bone scan, elevated hydroxyprolinuria, and bone histology.

Age Factors↗

Acute myelofibrosis and malignant hypercalcemia.

A 33 year old man presented with symptoms of one week's duration; he had a serum calcium of 22.5 mg/dl and a markedly hypercellular bone marrow. Despite therapy with saline diuresis, furosemide mithramycin, total parathyroidectomy and corticosteroids, symptomatic hypercalcemia was poorly controlled. Inappropriate serum parathyroid hormone (PTH) levels were found before and after parathyroidectomy whereas assays of the peripheral blood for osteoclast-activating factor and prostaglandin E (PGE2) were negative. An elevated leukocyte alkaline phosphate level, the inability to aspirate marrow, the marked generalized hyperplasia of all hematopoietic marrow elements, the focal accumulations of blastic cells and increasing reticulin fiber formation led to the diagnosis of acute myelofibrosis. A single course of cytosine arabinoside and thioguanine therapy was followed by profound hyperphosphatemia, hypocalcemia and death. The rarity of hypercalcemia with myeloproliferative disorders is documented by a review of the world literature, and the possible mechanism for hypercalcemia in this patient is discussed.

Acute Disease↗

Effect of mithramycin on hydroxyproline metabolism in Paget's disease.

Hydroxyproline was measured in the plasma and urine of six patients with symptomatic osseous Paget's disease before and after parenteral mithramycin treatment. The plasma profile data included free, peptide, and protein hydroxyproline values. Corresponding urine samples were analyzed for total hydroxyproline content and, in some instances, for free hydroxyproline. Plasma values of free and peptide hydroxyproline fell promptly toward the range of normal within 24 hr after the first intravenous injection of mithramycin. Protein hydroxyproline in the plasma was slightly but significantly lower than in the 15 normal control subjects, and this value did not change appreciably over the 4-day period during which the patients were treated. The urine excretion rate of total hydroxyproline decreased precipitously. Our results support the hypothesis that mithramycin rapidly reduces the accelerated rate of "collagenolytic" activity of osteocytes and osteoclasts in this disorder, presumably by an inhibiting effect on these bone-modeling cells. The major degradative lesion, as reflected in the plasma and urine, is characterized by the production of non-TCA-precipitable peptides containing hydroxyproline, which rapidly fell to normal levels after the administration of mithramycin.

Humans↗

Mithramycin effects on calcium, phosphorus and parathyroid hormone in osseous Paget's disease.

Six patients with symptomatic osseous Paget's disease were treated with a four-day course of mithramycin (25 mug/kg/day). The effects of treatment on the plasma calcium, phosphorus, parathyroid hormone, and the urinary fractional clearance of calcium and phosphorus were studied. Mithramycin produced significant hypocalcemia and hypophosphatemia, and also significantly and simultaneously increased the plasma parathyroid hormone concentration. Despite the increase in parathyroid hormone, the hypocalemia persisted because of the probable blocking effect which mithramycin exerted on the osteoclasts. The physiological response of the kidney to the elevated plasma parathyroid hormone appeared to be well preserved, as evidenced by the decreased fractional excretion of calcium and the increased fractional excretion of phosphorus in the urine; however, the fall in the clearance of calcium could be the result of the decreased filtered load.

Aged↗