Recovery from adenovirus pneumonia in a severe combined immunodeficiency patient treated with intravenous ribavirin.
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Adenoviridae Infections↗
Biomedical subjects
N M Wulffraat
Publications and source records attributed to N M Wulffraat.
Endocrine autoimmune diseases.
A considerable proportion of endocrine disorders (Graves' disease, primary hypothyroidism, type 1 diabetes, Addison's disease, and some forms of ovarian failure) are due to aberrant reactions of the immune system, viz. exaggerated reactions towards self-structures (autoantigens). Autoreactive T-cells are mainly responsible for the elicitation of destructive inflammatory responses in the target endocrine glands. Autoantibodies play a minor role in such reactions, but are useful in clinical practice as markers of the disease. A special type of autoantibody is formed by those autoantibodies that have endocrine functional activity, viz. autoantibodies capable of stimulatory or blocking the hormone synthesis and/or the growth of endocrine cells. These autoantibodies interfere with receptors, e.g. the TSH receptor or the IGF-I receptor. Antibodies stimulating the TSH receptor are responsible for the hyperthyroidism of Graves' disease.
Autoantibodies↗
[Erroneous diagnosis 'neonatal hypoglycemia' due to incorrect preservation of blood samples].
The high frequency of neonatal hypoglycaemia found in the St. Catherine's Hospital, Eindhoven [corrected], (8.2% of all newborn infants had values of 1.8 mmol/l glucose or less) promoted us to test the effects of the different conditions of blood preservation before measuring in a consecutive series of 18 newborn infants at risk for hypoglycaemia. When blood was preserved in microtest tubes coated with sodium fluoride (NaF) and measured after 1 hour at room temperature, the glucose level decreased by 7 to 36%. Storage on melting ice for one hour prevented this decline completely. In our hospital sodium fluoride coated microtest tubes are used. They were found to contain various concentrations of NaF, ranging from 0.85 to 2.65 mg/ml. In a 2nd experiment with cord blood from 8 patients, we proved that a concentration of 16 mg/ml sodium fluoride was necessary to inhibit glycolysis and to prevent a fall of plasma glucose. The rapid determination of blood glucose values, i.e. within 10 minutes after the blood sample is taken, decreased, as expected, the frequency of hypoglycaemia in the newborn at risk.
Blood Glucose↗
Familial Cushing's syndrome due to primary pigmented nodular adrenocortical disease. Reinvestigation 50 years later.
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Adolescent↗
Autoimmune involvement in Cushing syndrome due to primary adrenocortical nodular dysplasia.
Cushing syndrome due to primary adrenocortical nodular dysplasia was diagnosed in two patients, aged 3 years 9 months and 9.5 years. Subsequently, adrenalectomy was performed and followed by steroid replacement. In both cases, the adrenals were normal or only slightly enlarged and showed adrenocortical nodular dysplasia histologically. Small lymphocytic infiltrates consisting of T-cells and class II MHC positive macrophages were present in adrenal specimens of both the patients. Samples of protein A sepharose purified serum immunoglobulins from both children stimulated adrenocortical DNA synthesis and cortisol production in cultured guinea-pig adrenal segments in vitro in a dose dependent fashion. Adrenal stimulating immunoglobulins were also demonstrated in serum specimens of both patients' mothers. However, none of them had overt signs of adrenal disease. Our data support the view that autoimmune mechanisms may be involved in primary adrenocortical nodular dysplasia.
Adrenal Cortex↗
Familial Cushing's syndrome due to nodular adrenocortical dysplasia is an inherited disease of immunological origin.
Circulating immunoglobulins may be involved in the pathogenesis of familial Cushing's syndrome due to nodular adrenocortical dysplasia. The disease may also be part of a complex including cutaneous and cardiac myxomas, spotty pigmentation of the skin, and other signs of endocrine overactivity. In the present study of two sisters treated by complete adrenalectomy for Cushing's syndrome due to nodular adrenocortical dysplasia, and of their first-degree relatives, no evidence of associated disorders was present. However, the serum of both girls and of their mother contained immunoglobulins capable of stimulating adrenal DNA synthesis and cortisol production in vitro. The study supports the theory that familial Cushing's syndrome due to adrenocortical dysplasia is an inherited disease of immunological origin.
Adrenal Cortex↗
Immunoglobulins of patients with idiopathic Addison's disease block the in vitro action of adrenocorticotropin.
The presence of serum immunoglobulins (Ig) blocking ACTH-induced adrenal DNA synthesis and/or cortisol production was studied in 25 patients with idiopathic Addison's disease. For this purpose guinea pig adrenal segments kept in organ culture were exposed to ACTH and graded concentrations of patient IgG. After a 5-h culture period the cortisol present in the culture fluid was measured by RIA, and DNA synthesis in the adrenal cells was measured using Feulgen densitometry on frozen sections of the cultured adrenal segments. Addition of ACTH alone in concentrations of 0.1-10 pmol/L to the culture system stimulated in vitro cortisol secretion; the maximal stimulation was 63 +/- 35% (+/- SD; n = 5) at a concentration 0.1 pmol/L. ACTH also increased (in concentrations of 1 fmol/L to 1 pmol/L) the percentage of fasciculata cells in S-phase from 0-4% (nonstimulated) to 5-12%. IgG preparations from all but 2 of the 25 patients with idiopathic Addison's disease blocked these in vitro ACTH-induced adrenal responses in a dose-dependent fashion. IgG from 2 patients with tuberculous adrenalitis, 1 patient with secondary adrenal insufficiency, and 7 normal subjects had no blocking activity. Among 5 non-Addisonian autoimmune endocrinopathy patients who had adrenal cytoplasmic autoantibodies, 4 had no ACTH-blocking IgGs. Two of 9 patients with miscellaneous adrenal disorders (Cushing's disease, pigmented adrenal micronodular dysplasia, and adrenal nodules) had ACTH-blocking activity. These results demonstrate the existence of IgGs blocking the in vitro effects of ACTH and suggest their involvement in the pathogenesis of idiopathic Addison's disease.
Addison Disease↗
Immunoglobulins of patients with Cushing's syndrome due to pigmented adrenocortical micronodular dysplasia stimulate in vitro steroidogenesis.
The putative stimulation of adrenal steroid production by immunoglobulins (Igs) of five patients with pigmented adrenocortical micronodular dysplasia and clinical Cushing's syndrome was investigated. Ascorbate depletion, a process linked to steroid production, was measured by a cyto-chemical bioassay employing guinea pig adrenal explants in organ culture and exposed to IgG from the patients and normal subjects. We also measured cortisol production by these segments during a 5-h culture period using a RIA. For positive reference values we studied the effects of ACTH-(1-39), ACTH-(1-24), ACTH-(11-24), and ACTH-(18-39) on in vitro ascorbate depletion and cortisol production. Both ACTH-(1-39) and ACTH-(1-24) depleted ascorbate and stimulated cortisol production in adrenal cells. The dose-response kinetics of the peptides were bell-shaped; maximal responses were reached in both instances at 1 fmol/L to 10 pmol/L. In all tests, stimulation of in vitro cortisol production was paralleled by ascorbate depletion. ACTH-(18-39) also stimulated ascorbate depletion and cortisol production, but at one concentration only (100 fmol/L), and TSH and LH had no effect. Protein-A-Sepharose-purified IgG preparations of the five patients stimulated ascorbate depletion and/or cortisol production in a dose-dependent fashion; however, the responses occurred over a narrow concentration range (15-150 micrograms IgG/mL culture fluid). These observations support the hypothesis that the hypercortisolism of the syndrome of pigmented adrenocortical micronodular dysplasia is due to circulating Igs that stimulate adrenal steroidogenesis.
Adolescent↗
Effects of ACTH and ACTH fragments on DNA synthesis in guinea-pig adrenal segments kept in organ culture.
Stimulation of adrenal DNA synthesis by ACTH and its fragments ACTH (Synacthen) and ACTH was investigated. Synthesis of DNA was measured as the increase in the percentage of cells in S-phase (Feulgen densitometry) in guinea-pig adrenal explants kept in organ culture and exposed to the peptides for 5 h at 37 degrees C. ACTH and its C-terminal fragment ACTH (corticotrophin-like intermediate lobe peptide) were found to be potent stimulators of in-vitro adrenal DNA synthesis. The dose-response kinetics were biphasic and optimal responsiveness was reached in both instances at 1 fmol/1-10 pmol/1 (this biological effect of ACTH has hitherto not been described). The N-terminal fragment ACTH gave only minimal responses. Thyrotrophin and LH, tested as controls, did not induce adrenal DNA synthesis. Epidermal growth factor was a potent stimulator of adrenal DNA synthesis in vitro. Our data suggest a trophic action of the C-terminal part of the corticotrophic molecule. Clear trophic effects were also found for the N-terminal part of the pro-opiomelanocortin molecule N-POC (optimum 0.1 nmol/l) and N-POC(51-62) (optimum 0.1 pmol/l). The latter observations support earlier concepts that this part of the pro-opiomelanocortin molecule has a stimulatory effect on adrenal DNA synthesis.
Adrenal Cortex↗
Frequency and coordination of ciliary beat after cryopreservation of respiratory epithelium.
The effect of cryopreservation on human nasal mucosal biopsies was evaluated by determining the frequency and coordination of the ciliary beat. Samples were cryopreserved in a medium containing 80% Gey's balanced salt solution, 10% dimethyl sulfoxide, and 10% fetal calf serum. After thawing, the samples were put in a solution of 90% Gey's balanced salt solution and 10% fetal calf serum. Video recordings of the samples before and after cryopreservation were compared using a semiquantitative method. All the frequencies and coordination patterns seen before cryopreservation could be found in the sample after cryopreservation. It is concluded that ciliated epithelial biopsies can be stored in liquid nitrogen with the maintenance of ciliary beat frequency. In the recorded ciliated cells the ciliary beat coordination was slightly reduced; a lack of coordination was present in 20% of cells after cryopreservation as compared to 10% before cryopreservation.
Adult↗
Bone marrow mitotic index: a methodological study.
The mitotic index (MI) of bone marrow specimens is assessed in 3 different ways: method I determines the number of mitotic figures per 1,000 nucleated cells; method II counts the number of mitoses seen per 1,000 nucleated cells belonging to the proliferative pool, and method III is the same as method I, but excludes all lymphoid cells. 30 Giemsa-stained bone marrow smears from 6 children were screened by 3 independent investigators. MI of method II is found to be approximately twice as high as the MI of method I. The results of method III indicate that the size of the lymphoid population introduces a bias, which renders method I less reliable. It is concluded that method II is the method of choice for a reliable assessment of bone marrow MI.
Bone Marrow Cells↗