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Biomedical subjects

S Boonpucknavig

Publications and source records attributed to S Boonpucknavig.

At least 19 recordsLinked to original sources

Cushing's syndrome caused by Ewing's sarcoma secreting corticotropin releasing factor-like peptide.

Procedures were carried out in a 12-year-old girl to relate Ewing's sarcoma of the left tibia with Cushing's syndrome. Computed tomography revealed a normal pituitary and hypothalamus but bilateral adrenal hyperplasia without focal enlargement, thus readily excluding hypothalamic-pituitary-adrenal tumor. Negative results from a high-dose dexamethasone suppression test do not support pituitary-dependent Cushing's disease. Ewing's sarcoma was diagnosed on tibial biopsy. The regression of the physical and biochemical findings of Cushing's syndrome subsequent to amputation of the left lower leg strongly suggests ectopic Cushing's syndrome caused by Ewing's sarcoma. Immunohistochemical studies of the resected bone were negative for corticotropin but positive for corticotropin releasing factor-like peptide. We conclude that this is the first reported case of ectopic Cushing's syndrome in a child that is caused by Ewing's sarcoma secreting corticotropin releasing factor-like peptide.

Adrenocorticotropic Hormone

Wilms' tumor, male pseudohermaphroditism and glomerulonephritis: the Drash syndrome. First case report in Thailand and review the literatures.

The Drash syndrome, the first case in Thailand is reported. The patient had male pseudohermaphrodite, Wilms' tumor of the left kidney and mesangial proliferative glomerulonephritis. Metastasis of Wilms' tumor was noted in the liver, omentum, pelvic peritoneum and vertebral body. Chemotherapy (actinomycin D, vincristine and dexamethasone) and local irradiation were given. The patient developed pneumonia, diarrhea and ended up with Steven-Johnson syndrome from Cotrimoxazole hypersensitivity.

Disorders of Sex Development

Hematological and coagulation studies in malaria.

One hundred and twenty-six patients with malaria (30 cases of P. vivax and 96 cases of P. falciparum) were studied for evidence of hematological coagulation and fibrinolysis abnormalities. Anemia associated with malaria was observed only in P. falciparum infections and there was no correlation between the degree of anemia and the percentage of parasitemia. Decreased hematocrit levels were found to be statistically significant in P. vivax infected patients (P greater than 0.05). Thrombocytopenia was observed in both P. vivax and P. falciparum malaria patients (P less than 0.001) and correlated with the degree of parasitemia (r = 0.974). Plasmin activity was normal in P. vivax malarial patients but it was significantly increased in patients with a P. falciparum of more than 5 per cent parasitemia. Coagulation profiles showed normal PT, aPTT, and TT in P. vivax infected patients while prolonged PT and aPTT were observed in P. falciparum infection which correlated with the degree of parasitemia (r = 0.0992). Coagulation factors V, VII, and IX were the most sensitive parameters in the expression of coagulation defects and most coagulation abnormalities were due to liver involvement. However, 2 of 20 complicated cases of P. falciparum showed evidence of disseminated intravascular coagulation (DIC).

Anemia

Immunological and immunopathological studies of patients with burn scar.

Sera obtained from 5 cases of burn scars (patients group 1) and cases of burn scars with squamous cell carcinoma (patients group 2) of the skin are analysed for anticollagen antibodies. Anticollagen type VI was demonstrable in the serum of one out of 5 cases in group 1 and 4 out of 6 cases in group 2. Collagen type VI was demonstrable in the scar without carcinoma of one patient whose serum contained anticollagen type VI. It is suggested that collagen type VI in the scar and their immunobiologic reaction may be related to the regulation of tumor development in scar tissue of the skin.

Antibodies, Anti-Idiotypic

Heterogeneity of antibody response to glomerular basement membrane antigen in patients with different forms of glomerulonephritis.

Antibodies to collagenous and noncollagenous components of glomerular basement membrane (GBM) have been detected by immunoblotting in some sera from patients with various kinds of glomerulonephritis. A half proportion of patients with rapidly progressive glomerulonephritis (RPGN), chronic focal glomerulonephritis (CFGN), idiopathic membranous glomerulonephritis (MGN). IgA nephropathy and lupus nephritis (LE-GN) had IgG antibodies to heterogenous components in acid insoluble fraction of pepsin digested GBM. This acid insoluble fraction represented a complex of collagen and noncollagenous proteins of GBM. Following digestion of acid insoluble fraction with bacterial collagenase, the triple helical collagenous components of GBM were destroyed and released most likely of noncollagenous proteins. Antibodies to this noncollagenous proteins were found in only some patients with chronic glomerulonephritis (17.6%) and lupus nephritis (21.4%). Upon reaction with human placenta derived type IV collagen, different frequencies of antibody response were found in patients of different groups. However, all these reactive sera showed a similar immunoblotting pattern. The relationship between antibody response to antigenic components from human GBM or human placenta and pathogenesis of renal disease is unclear. However, the occurrence of spontaneous autoantibody response to some exposed GBM self antigens may mediate further renal destruction resulting in chronic ongoing stage of the disease.

Antibody Formation

Rapidly progressive glomerulonephritis in Thai children.

Sixteen children with extensive crescentic glomerulonephritis and rapid renal deterioration were selected from 476 patients with glomerulopathy for study. The patients (1-14 yr, M:F = 5:11) presented with edema, oligoanuria, hypertension, gross hematuria and uremic symptoms in 81, 62, 62, 56 and 50 per cent, respectively. The mean Scr was 804 (+/- 436) micromole/L and BUN 38 (+/- 13.4) mmole/L. Anemia was found in 100 per cent, hematuria in 100 per cent, heavy proteinuria 75 per cent, hypoalbuminemia 40 per cent, hypercholesterolemia 38 per cent and low C3 40 per cent. The underlying causes of RPGN included idiopathic 9, PSAGN 6 and LE 1. Eight patients recovered with normal or slightly elevated Scr while the diseases progressed to ESRD in 8 patients. Idiopathic RPGN and extensive (greater than 80%) crescentic glomerulonephritis correlated with a poor prognosis.

Adolescent

Development of immune-complex glomerulonephritis and amyloidosis in Syrian golden hamsters infected with Opisthorchis viverrini.

Renal disease associated with Opisthorchis viverrini infection was investigated in Syrian golden hamsters. On the fourth week after infection with 100 viable metacercariae; anti-tegumental membrane antibodies were detected in the sera by immunofluorescence antibody technic and by enzyme-linked immunosorbent assay. Six weeks after infection tegumental and anti-tegumental membrane immune-complex and amyloid fibrils were found in the glomeruli. Amyloid was characterized to be AA protein. Acute proliferative glomerulonephritis associated with the brightest immune-complex deposits developed in week 8 after infection. Intensity of immune-complexes in all glomeruli were reduce gradually thereafter and replaced by amyloid. Progressive obsolescence of the glomeruli, tubular atrophy, interstitial inflammation and fibrosis associated with massive proteinuria and deterioration of renal function appeared in week 10 after infection toward the end of the experiment in week 38 after infection.

Amyloidosis

Immunofluorescent staining of platelet suspensions and detection of antiplatelet antibody in patients with idiopathic thrombocytopenic purpura.

Direct immunofluorescent staining of 31 specimens of platelets obtained from 13 cases of idiopathic thrombocytopenic purpura (ITP) revealed positive staining on the surface of platelets for both immunoglobulins (Igs) and human B1C globulin in 9 specimens, for only Igs in 1 specimen and for human B1C alone in 5 specimens. The pattern of the positive immunofluorescent staining was granular. Indirect immunofluorescent staining of normal platelets in serum obtained from patients with ITP was positive for antiplatelet antibody in 9 out of 31 specimens. This suggests that platelets in patients with ITP may be damaged by an antiplatelet autoantibody acting directly on the platelet surface and/or by antigen antibody complexes binding via Fc IgG receptors on the surface of the platelets.

Adolescent

The pattern and nature of the lymphocyte population response in dengue hemorrhagic fever.

Eighty-eight specimens consisting of lymphocytes separated from peripheral blood samples from 76 patients with dengue hemorrhagic fever (DHF) were studied. The results revealed a significant increase in numbers of atypical lymphocytes and B lymphocytes, and a decrease in T lymphocytes by comparison with normal controls. Anti-T lymphocyte antibody was also detected in the serum of all patients with DHF. The greatest increments and decrements of the above were noted on the day of shock of subsidence of fever.

Adolescent

Immunofluorescence study of skin rash in patients with dengue hemorrhagic fever.

Fifty-three skin biopsy specimens obtained from the cutaneous rashes of patients who had dengue hemorrhagic fever (DHF) were studied by immunofluorescence technique. Six specimens showed deposits of IgM, beta 1 C-globulin, dengue antigen, and fibrinogen during the first week of fever. Some but not all of these components (IgM, beta 1 C, dengue antigen) were demonstrated in 29 specimens. Twenty-three of them yielded negative results. Granular deposits of IgM and beta 1 C appeared in the blood vessel walls of dermal papillae. Dengue antigen was seen in mononuclear cells that were closely infiltrated around the blood vessel wall in dermal papillae. Fibrinogen was located within or about the blood vessels. The findings suggest that the cutaneous rashes occurring in DHF are caused by an immunopathologic process.

Antigens, Viral

Circulating immune complexes in serum from patients with dengue haemorrhagic fever.

Circulating immune complexes were detectable in 80% of serum from patients with dengue haemorrhagic fever. The immune complexes were detected for the first time on day two after the onset of the fever. The amount of complexes reached the maximum value on day 4 or 5 after onset, or when the patients developed shock or subsidence of fever, after which the complexes decreased in number. The number of complexes also correlated well with the clinical grading (severity) of the disease, i.e. the maximum amount was shown in grade III. These complexes may play a part in the pathogenesis of this disease.

Adolescent

Immunopathological studies of Plasmodium berghei infected mice: (effect of carbon particles).

An attempt was made to block the role of the macrophages in the immune response by saturating them with carbon particles. The experiment was performed on Swiss albino female mice injected intraperitoneally with 10(5) P. berghei berghei. These mice were injected with carbon particles of 20 mgs on the day before the inoculation and again 10 mgs on day 4 and day 9 after the inoculation. The degree of parasitaemia was slightly higher throughout the experiment in the infected mice treated with carbon than in those untreated. By direct immunofluorescent method the free floating form of malaria antigen was detected in the infected organs (liver, kidney and spleen) on day 3 of infection onwards. The antigen was more intense in the carbon treated mice than in the untreated ones. The granular form of the antigen in the walls of glomerular capillaries of the kidney, in the Kupffer cells of the liver and in the reticulo-endothelial cells of the spleen, the malarial antibody and the immune complex (malaria antigen, antibody and mouse beta 1 C globulin) in the kidney were detected later on (day 11).

Animals

Demonstration of dengue antibody complexes on the surface of platelets from patients with dengue hemorrhagic fever.

By the direct immunofluorescent technic, dengue antigen, human immunoglobulins, and beta 1C globulin were detectable on the surfaces of platelet suspensions from 48% of patients with dengue hemorrhagic fever. The percentages of positive-staining platelets were not related to the severity of thrombocytopenia, which was marked on the day after the patient developed shock or subsidence of fever. It is suggested that an immunologic mechanism is one of the factors associated with the thrombocytopenia caused by increased platelet destruction.

Adolescent

Plasmodium berghei--infected mice. Focal glomerulonephritis in hyperimmune state.

Twenty-one male Swiss albino mice were injected intraperitoneally with 2 x 10(5) Plasmodium berghei berghei-infected cells. Twenty-four mice were used as a noninfected control group. On the tenth day after infection, three mice were killed and the remainder were treated with chloroquine phosphate (0.025% solution) given orally. The dosage was subsequently adjusted to keep the parasitemia below 5%. Eighteen of the controls received the same chloroquine dosage. Diffuse proliferative glomerulonephritis was seen from day 10 of infection up to two months after treatment. Granular deposits of immune complex were seen in all glomeruli. At the end of third month and thereafter, all experimental mice were in a hyperimmune state, and the kidneys showed the characteristic of focal glomerulonephritis resembling some types of human focal glomerulonephritis.

Animals

The defined antigen substrate sphere system with direct immunohistoperoxidase for detection of soluble dengue antigen in sera of patients with dengue hemorrhagic fever.

The defined antigen substrate sphere system is a simple method for detecting antigen or antibody in the circulation. The technic is based on the coupling of antigen or antibody with Sepharose 4B beads that have been activated by cyanogen bromide. In this study the activated beads were exposed to dengue antigen in the serum from a patient with dengue hemorrhagic fever and then stained with antidengue antibody conjugated with horseradish peroxidase. The positive reaction showed brown beads by light microscopy, whereas the negative reaction gave colorless beads. The authors examined 134 specimens from 91 cases. The results were positive in 53.85%. The dengue antigen appeared in the sera on the day before shock or subsidence of fever. The percentages of sera containing soluble dengue antigen were greatest on the day of shock or subsidence of fever (33.33%) and on the fifth day of fever (28.07%). The highest titers of soluble dengue antigen (1:40 to 1:80) appeared in the sera of patients who had Grade III disease on the day of shock. The dengue antigen appeared most often in sera that had high titers of dengue antibody. It is postulated that this detected dengue antigen may be a part of soluble immune complexes formed during the hyperimmune stage of the immune response, and plays a significant role in the pathogenesis of dengue hemorrhagic fever and shock syndrome.

Antibodies, Viral

Immunofluorescence skin test for lupus erythematosus. A correlative study between frequency and patterns of skin test results, glomerular pathologic abnormalities, and immunologic findings.

We undertook a correlative study between the frequency and patterns of immunoglobulin deposits at the dermoepidermal junction of clinically normal skin and the morphologic features (histopathologic and immunopathologic) of the glomerular lesions and various immunologic measurements in 38 patients with systemic lupus erythematosus. Thirty-five (92.1%) showed a positive immunoglobulin band at the skin. A statistically significant (P less than .001) correlation was observed between the pattern of deposit and the duration of disease. The stippled pattern was found to be more common in those cases of less than one year's duration, while the thready pattern was more common in those cases lasting longer than one year. There seemed to be no significant relationship between either the frequency (P greater than .100) and pattern (P greater than 0.25) of the immunoglobulin band and the glomerular pathologic or other immunologic measurements.

Complement C3