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Biomedical subjects

H Baart de la Faille

Publications and source records attributed to H Baart de la Faille.

At least 19 recordsLinked to original sources

Salivary gland swelling following naproxen therapy.

We report allergic sialadenitis as a novel side-effect of naproxen, a non-steroidal anti-inflammatory drug (NSAID). Bilateral swelling of the major salivary glands and a rash occurred a few days after the patient had taken the drug. High-dose systemic corticosteroid therapy was required to control the disorder. Because the salivary gland swelling was not initially recognized as an adverse drug reaction, further doses of naproxen were given on two occasions, with similar results.

Anti-Inflammatory Agents, Non-Steroidal↗

Lupus therapy.

Explore the source record for details and available documents.

Adrenal Cortex Hormones↗

Accumulation of iron in erythroblasts of patients with erythropoietic protoporphyria.

We have studied the iron metabolism in nine patients with erythropoietic protoporphyria (EPP) and three patients with sideroblastic anaemia (SA). All, except one EPP patient were iron deficient. The SA patients had a secondary haemochromatosis. The bone marrow aspirates of patients with SA and also three patients with EPP had a high incidence of ring sideroblasts. Ultrastructural examination of the bone marrow consistently showed finely dispersed electron-dense deposits localized in mitochondria of erythroblasts in all patients with EPP and SA. Mitochondrial electron energy-loss spectroscopy (EELS) indicated identical iron compounds in erythroblasts of all EPP and SA patients. These findings indicate that the mitochondrial iron utilization is disturbed in EPP and SA. The observation of mitochondrial iron deposition in erythroblasts in EPP and SA suggests that this failure is not of pathognomonic value for diagnosis of SA, but is apparently the result of an inefficient haem synthesis, in EPP due to a defective ferrochelatase. The mitochondrial iron deposition does not depend on the iron status (iron overload or iron deficiency) of the EPP patient.

Adult↗

Increased number of immunoreactive nerve fibers in atopic dermatitis.

The presence of immunologic markers for neurofilaments, neuropeptides of sensory nerve fibers (Calcitonin gene-related peptide and substance P), for noradrenergic innervation (neuropeptide Y and Tyrosine hydroxylase), and Neuron-specific protein 9.5 was evaluated in frozen tissue sections from normal skin (n = 34) and from skin biopsies manifesting urticaria (n = 6), leukocytoclastic vasculitis (n = 4), systemic lupus erythematosus (n = 23), and atopic dermatitis (n = 40, of which 16 were from lesions induced by epicutaneous atopic allergen patch tests). In some normal skin specimens immunoreactive nerve fibers expressing Neuron-specific protein 9.5 were observed in the epidermis, dermis, and around blood vessels. For the other markers, immunolabeling was mainly observed in the dermis around blood vessels. Neurofilaments, which are scarce in normal skin epidermis, were present in higher density in the epidermis of affected skin in all disease conditions. Biopsies from urticaria and systemic lupus erythematosus showed a decrease in density of fibers immunolabeled for neuropeptides substance P and Calcitonin gene-related peptide and for Neuropeptide Y. In biopsies from skin with atopic dermatitis, an increased density of fibers was observed for all markers except Neuropeptide Y and Tyrosine hydroxylase. In this group, biopsies from positive atopic allergen patch tests showed an enhanced density of fibers labeled by antibody to Neuron-specific protein 9.5 and a lower density in labeling for Tyrosine hydroxylase. The data indicate a potential role of innervation and neuropeptides in dermatoses like atopic dermatitis.

Adolescent↗

Early involvement of hepatic parenchymal cells in erythrohepatic protoporphyria? An ultrastructural study of patients with and without overt liver disease and the effect of chenodeoxycholic acid treatment.

Liver biopsy specimens obtained from two groups of erythrohepatic protoporphyria patients were studied histopathologically and ultrastructurally. Group 1 comprised seven erythrohepatic protoporphyria patients with a normal liver histology; from two patients liver biopsy specimens were available before and after 1 yr of chenodeoxycholic acid treatment. Group 2 consisted of four patients with a history of liver disease and liver histopathology; three patients were observed before and after 1 yr of chenodeoxycholic acid treatment. Liver specimens of nine kidney transplant donors served as controls. Unlike the morphology at the light microscopic level, the ultrastructure of hepatic parenchymal cells was affected in both groups of erythrohepatic protoporphyria patients. In both groups the nuclei, endoplasmic reticulum, lateral plasma membranes and bile canaliculi were altered. Collagen fibers were frequently present. In addition, in group 2 bile thrombi and intracytoplasmic protoporphyrin crystals were observed. After chenodeoxycholic acid administration, the latter feature had diminished. It is concluded that (a) in erythrohepatic protoporphyria ultrastructural changes are present in the hepatic parenchymal cells even in early stages of the disease. Changes in bile canalicular ultrastructure suggest a defective hepatic excretory function, probably caused by the toxic effect of protoporphyrin. (b) Chenodeoxycholic acid administration causes no distinct improvement of the ultrastructure of organelles in the hepatic parenchymal cell or the bile canalicular system but may decrease crystalline protoporphyrin deposition in the liver.

Adolescent↗

Binding of antibodies to nonhistone nucleoproteins on keratinocytes in suspensions.

Antibody binding on the cell surface of epidermal cells, recently established on cultured neonatal foreskin cells, is supposed to play a role in the pathogenesis of in vivo antinuclear antibodies (ANA) of the skin. To study this phenomenon in suspensions of adult human keratinocytes, a cell system more closely related to the in vivo situation, we investigated the binding capacity of nine sera with various antibody profiles against nuclear components, as well as a murine monoclonal Sm-antibody. It was found that sera containing antibodies against nonhistone nucleoproteins bound to the cell surface of keratinocytes, whereas monospecific anti-dsDNA sera and the murine anti-Sm serum did not. This binding was found in both basal and suprabasal keratinocytes. The percentage of cells showing antibody binding was not significantly enhanced by preirradiation with ultraviolet light, as was found in a previously study. The cell surface binding is probably an antigen-antibody binding and not the result of cross-reactivity. Such cell surface binding may be important for the formation of in vivo ANA in the skin.

Antibodies↗

In vivo antinuclear antibody of the skin: diagnostic significance and association with selective antinuclear antibodies.

Immunofluorescence microscopy of the skin has disclosed antibodies bound to epidermal cell nuclei in several connective tissue disorders. To establish the diagnostic potential of this phenomenon the results of immunofluorescence microscopy of biopsy specimens from 1651 subjects with various diseases and from 315 patients with systemic connective tissue disorders and related diseases were reviewed. It was found that the predictive value of the phenomenon for the presence of a systemic connective tissue disorder was, in general, 88%. Except for the homogeneous and thready patterns, which seldom appear, but are specific for SLE, in vivo antinuclear antibody (ANA) does not discriminate better between the various disorders than do serum antibodies. The presence of in vivo ANA in the skin was related to serum antibodies against non-histone nucleoproteins, but not to anti-dsDNA antibodies. Combined with the finding that antibodies against non-histone nucleoproteins can bind on the surface of human keratinocytes, this suggests that ANA of the skin occurs in vivo.

Antibodies, Antinuclear↗

Management of nonstaphylococcal toxic epidermal necrolysis: follow-up study of 16 case histories.

In this study the clinical and laboratory data of 14 patients who experienced 16 attacks of nonstaphylococcal toxic epidermal necrolysis (TEN) are presented. All patients were treated in the Department of Dermatology of the University Hospital of Utrecht. Only 1 patient died. Attention is focused on a successful management consisting of reversed barrier nursing, painstakingly executed skin care, timely use of antibiotics and a therapy consisting of high dosages of corticosteroids. Thorough management in centers experienced in the treatment of TEN is essential for good treatment results. Further investigations concerning the use of corticosteroids in treating TEN are needed.

Adrenal Cortex Hormones↗

Comparison of narrow-band UV-B phototherapy and PUVA photochemotherapy in the treatment of psoriasis.

The therapeutic effectiveness of a new fluorescent lamp, Philips TL-01, which emits a narrow peak around 311-312 nm, was compared with the currently used PUVA photochemotherapy consisting of oral 8-MOP followed 2 h later by UV-A from fluorescent lamps Philips TL-09. Comparisons of therapeutic efficacy were performed in 10 patients with widespread, symmetrically distributed psoriasis lesions. They received treatment with PUVA on one half of the body and with TL-01 light on the other half; both treatments were given twice a week. It is concluded that on the average phototherapy with narrow-band UV-B is an effective as PUVA; it is certainly more convenient and probably less carcinogenic.

Adult↗

Immunohistopathology of light-induced skin lesions in lupus erythematosus.

Recently we have been able to induce pathological skin reactions with UVB, UVA and visible light in patients with lupus erythematosus (LE). The pathological skin reactions had the appearance of spontaneously developed LE lesions. In the present study, using patients with polymorphic light eruption as controls, we subsequently investigated what types of immunohistochemical abnormalities were found in these lesions. It was shown that in the induced skin lesions, phenotypically similar inflammatory cells were found as in spontaneously evolved lesions. Granular deposits of immunoreactants, as found in most spontaneously evolved LE lesions, occurred in 12 out of 16 LE patients 7-10 days after onset of the artificial irradiation. The dermal infiltrates in light-induced LE lesions differed mainly from those in polymorphic light eruption, by the amounts of CD1+ cells (Langerhans' cells). In polymorphic light eruption, the relatively large amount of these cells suggests an active migration of antigen-presenting cells, a mechanism apparently not operative in LE. Our results underline the importance of the pathogenic action of light in LE.

Antibodies, Monoclonal↗

Light-induced skin lesions in lupus erythematosus: photobiological studies.

To investigate the light sensitivity to various wavelength regions in lupus erythematosus (LE), phototests were performed in 24 LE patients with clinical photosensitivity (7 had systemic LE, 9 discoid LE, and 8 subacute cutaneous LE). Skin areas (measuring 40-60 cm2) were irradiated daily, maximally six times. With all three light sources used (emitting UVB, UVA, and visible light respectively) abnormal papular or papulosquamous reactions could be induced. In four of the 20 patients reacting abnormally, lesions occurred 10 or more days after cessation of the phototests; this indicates that the problem of photosensitivity in LE may be greater than appreciated so far.

Adolescent↗

[Canthaxanthin retinopathy].

The authors examined 32 patients with light-dermatosis or vitiligo who were being treated with orally administered Canthaxanthin and betacarotene. Eight had crystalline retinopathy with typical "gold dust" particles in the inner retinal layers, forming a band-shaped zone around the macula and, in more severe cases, also around the optic disk. No deterioration of visual function was found in any of these patients. Crystal deposition was correlated significantly to the total Canthaxanthin dosage but not to the duration of treatment.

Canthaxanthin↗