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C August

Publications and source records attributed to C August.

66 records · Page 4Linked to original sources

Persistent cytomegalovirus infection: association with profound immunodeficiency and treatment with interferon.

Cytomegalovirus (CMV) was repeatedly isolated from urine and saliva of a 20-month-old male child with recurrent episodes of pneumonia, high fever, rash, lymphadenopathy, oral ulceration, and neutropenia. Immunologic evaluation revealed decreased serum IgG and IgA, increased IgM, depressed T- and B-lymphocyte functions, and decreased natural killer (NK) activity for herpes simplex-type I virus-infected targets. NK activity was augmented following exposure of the patient's lymphocytes to interferon (IF) in vitro. The child was treated with interferon (four courses, dosage varying from 2 million U/day to 1 million U three times/week for periods of 10, 28, 80, and 67 days, respectively, interspersed over 9 months) and hyperimmune plasma infusions every 3 weeks. Toward the end of interferon therapy oral Levamisole was started and a feeding gastrostomy was inserted to provide nutritional support. Clinical recovery was associated with reversal of immunologic abnormalities except for the hypogammaglobulinemia. Aggressive antiviral therapy (e.g., with IF) followed by immunostimulation (e.g., with Levamisole) may prove effective in controlling certain viral infections in immunodeficiency disorders.

Agammaglobulinemia↗

Antithoracic duct lymphocyte globulin therapy of severe aplastic anemia.

We performed a prospective randomized trial of antithoracic duct lymphocyte globulin (ATDLG), HLA-haploidentical marrow, and androgen (regimen ABA) versus androgen alone (concurrent STANDARD care controls) in 42 newly diagnosed individuals with severe aplastic anemia. ABA patients also were matched with patients from our preceding study (historical STANDARD care controls). Supportive care and pretreatment patient characteristics were the same in all groups. By life table analysis, 76% of patients receiving ABA are alive at 2 yr compared to 31% of the concurrent control group (p less than 0.002 versus ABA) and 19% of the historical controls (p less than 0.0001 versus ABA) given STANDARD care. ABA patients had greater hematologic improvement than either control group (p less than 0.001). However, improvement with ABA was often incomplete. Toxicity of ATDLG was considerable but manageable. Further studies to determine the mechanism of action and active component(s) of ABA are indicated.

Androgens↗

Gynecologic manifestations of chronic graft-versus-host disease.

Gynecologic manifestations of the clinicopathologic syndrome known as graft-versus-host disease seen in 5 patients treated with allogeneic bone transplantation are presented. The clinical symptoms and significant problems found in association with graft-versus-host disease may include extensive sclerosing vaginitis and stricture formation. Treatment and possible preventive therapy are discussed.

Adolescent↗

Epstein-Barr virus-related serology in marrow transplant recipients.

Serial sera from 50 marrow transplant recipients were examined for their spectra and titers of antibodies to EBV-specific antigens. Immediately before or after transplant, blood products passively transferred antibodies to EB viral capsid antigen (VCA) and EBV nuclear antigen (EBNA). In most recipients, passively-transferred antibodies were replaced by endogenous antibodies regardless of whether donor or recipient had EBV antibodies before transplantation. Commencement or resumption of endogenous EBV antibody production was not associated with signs of infectious mononucleosis or heterophil antibody responses. Antibodies to VCA rose to abnormally high titers, followed successively by antibody to early antigens (EA), and disproportionately low levels of anti-EBNA. Unusually high anti-VCA and anti-EA levels persisted when tests of immune function returned to normal. Antibodies to other herpes group viruses showed no consistent changes. We conclude that (1) EBV does not cause significant clinical problems in marrow transplant recipients; (2) persistent EBV infection can become established or reestablished in the presence of antibodies to EBV; (3) marrow transplant recipients show the same exaggerated immune response to EBV as other immunodeficient patients; and (4) the pattern of EBV-specific antibodies may be a more sensitive measure of defective cell-mediated immunity than most conventional tests of immune function.

Adolescent↗

[Modification of efferent vagal effects on the isolated atrium by increased extracellular K+ concentrations].

Isolated vagal-innervated rabbit atria are electrically driven. Alterations of action potential, contraction, and electrotropic and inotropic vagal effects are investigated during variations of the external potassium concentration. Action potential area and contraction amplitude decrease by increasing external potassium concentration. If the potassium concentration is higher than 11 mM, the action potential disappears. At 24 mM potassium concentration the contraction amplitude of the driven atrium is reduced to 2%. Adrenaline (2.10(-5) g/ml) causes a restitution of the action potential and the contraction. With increasing potassium concentration the inotropic and the electrotropic vagal effectivity increases also. The vagal effects at the adrenaline restituted action potentials and contractions (15 mM potassium, 2.10(-5) g/ml adrenaline) are also higher than in normal solutions. The relations of electromechanical coupling are altered by potassium variation at the same coupling curve. With increasing potassium concentration the reproducibility of the vagal effects decreases.

Action Potentials↗

Radiation-induced atypia. A review.

A review of radiation-induced atypia is presented. The radiation changes are divided into acute, intermediate, and late. The presence of cancer cells following irradiation is divided into persistent carcinoma, early recurrent carcinoma, and late recurrent carcinoma. The features of each group are described, supplemented by illustrations depicting the changes. The cytologic changes of radiation are discussed by correlating the light microscopic features with the electron microscopic findings in 8 cases that were studied ultrastructurally.

Adult↗

[Arthrogryposis, renal tubular dysfunction, cholestasis (ARC) syndrome: case report and review of the literature].

The ARC-syndrome is a rare disease with the obligatory symptoms arthrogryposis, renal tubular dysfunction and cholestasis. Optional further symptoms like ichthyosis, diarrhea, central nervous system defects and recurrent infections have been reported. The ARC-syndrome was first reported by Lutz-Richner and Landolt in 1973. The pathophysiology is still unknown, an autosomal recessive inheritance is postulated. Patients rarely exceed an age of six month. We report a boy of consanguineous Turkish parents who suffered from congenital deformities of the lower extremities, a metabolic acidosis and failure to thrive. In the sequel he developed a renal Fanconi syndrome and cholestasis. Histology of liver and muscle biopsy specimen showed the typical findings of the disease with giant cell hepatitis and neurogenous muscle atrophy. His condition could be stabilized and he increased in weight by substituting fluid, electrolytes, buffer and parenteral nutrition. Total enteral nutrition of the 280 ml/kg/d he required failed even by nasogastric tube and percutaneous endoscopic gastrostomy. Additional fluid substitution by central venous catheter remained necessary. At the age of 7 month he died.

Abnormalities, Multiple↗

Orthopaedic manifestations of chronic graft-versus-host disease.

Chronic graft-versus-host disease (GVHD) is a well-recognized complication of allogeneic bone marrow transplantation (BMT). Musculoskeletal manifestations include joint contractures, polymyositis, polyserositis, and fasciitis. We present 14 patients with orthopaedic complications of chronic GVHD. Long-term conservative management of joint contractures with physical therapy and orthotics was generally successful in restoring patients' premorbid functional status. Surgical release of joint contractures yielded poor results and rendered the affected joints unresponsive to further conservative treatment. Surgical intervention in the treatment of joint contractures resulting from chronic GVHD does not appear qualitatively to improve functional status in patients affected with this disease process.

Adolescent↗

Simultaneous bilateral central catheters: a safe technique.

Optimal central catheter care includes restriction usage for blood sampling and blood product administration on enhance continued sterility, but our experience with 25 children receiving bone marrow transplants after cytoreduction challenges this concept. Prior to transplantation, bilateral percutaneous subclavian vein silastic catheters were inserted without incident, one utilized for continuous nutritional support in caloric quantity to assure body weight maintenance, and the contralateral catheter utilized for daily venous sampling plus administration of medications including blood products. Patients subsequently entered a protective environment and bi-weekly surveillance cultures were monitored. Nutritional therapy was given for 876 days through 53 catheters. One patient developed culture-proven sepsis, an organism first cultured from the skin. The patient complication rate of 4% and the per diem rate of 0.11% in this immunocompromised population compares favorably to the 10.5 and 0.32% incidence we previously reported for 200 children with unilateral catheters. These data demonstrate that bilateral central catheters can be safely utilized in children for nutrition and sampling.

Adolescent↗

Renal parenchymal malakoplakia: ultrastructural findings in different stages of morphogenesis.

Light microscopic and ultrastructural findings in five cases of renal parenchymal malakoplakia detected in renal biopsy specimens (four cases) or observed at autopsy (one case) are reported. The spectrum of ultrastructural changes ranging from lamellar and microvesicular phagolysosomal inclusions arranged in a biphasic pattern to fully developed Michaelis-Gutmann bodies is described. In three of the biopsy cases the lesions appeared to represent early stages of malakoplakia lacking classic Michaelis-Gutmann bodies. Especially in this phase of disease, ultrastructural investigation can distinguish between this condition and other histiocytic interstitial renal processes. The findings show that focal cytoplasmic degeneration and autophagolysosomal processes observed in macrophages could precede the disturbance in the process of bacterial breakdown that is responsible for the peculiar granuloma-like inflammatory histiocytic reaction.

Adult↗