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Biomedical subjects

W Kuis

Publications and source records attributed to W Kuis.

At least 109 records · Page 6Linked to original sources
PubMed1989

Idiopathic chondrolysis of the hip. A distinct clinical entity?

We report 4 cases of idiopathic chondrolysis of the hip in children, a rare disease. In several aspects, there was a close resemblance to classical pauciarticular juvenile chronic arthritis. Hence, we question the clinical entity denoted as idiopathic chondrolysis of the hip.

Adolescent↗
PubMed1988

Treatment of two cases of cryptococcal meningitis with fluconazole.

Two patients with cryptococcal meningitis were treated with the investigational triazole drug fluconazole (UK-49,858). Cerebrospinal fluid (CSF) levels of fluconazole were between 3.0 and 5.4 mg/l 2 h after an oral dose of 50 mg daily in the first patient and between 7.9 and 9.0 mg/l after an oral dose of 100 mg daily in the second patient. These levels were in the same range as plasma levels. The first patient, a 46-year-old renal transplant patient, was both clinically and microbiologically cured after 28 weeks of therapy (follow-up 14 months). In the second patient, a 15-year-old girl with chronic mucocutaneous candidiasis, fluconazole led to clinical cure of the meningitis, but failed to eradicate cryptococci from the CSF. These cases illustrate that fluconazole is useful for the treatment of cryptococcal meningitis, especially when prolonged treatment is indicated as in patients with immunodeficiencies.

Adolescent↗
PubMed1988

[Disorders in humoral defense: clinical aspects, diagnosis and therapy].

Clinical and immunological findings of 5 patients with distinct defects in either humoral immunity or in the complement system are described. The syndromes presented comprise examples of type I dysimmunoglobulinaemia, X-linked agammaglobulinaemia (XLA), familial deficiency of complement factor C1q and a patient with a selective deficiency in the synthesis of antibodies against pneumococcal polysaccharides. The patients with a defect in humoral immunity all showed recurrent bacterial infections of the respiratory tract. The XLA-patient developed a dermatomyositis-like syndrome and ECHO-virus encephalitis. Prior to the development of a SLE-like syndrome the two siblings with C1q deficiency showed recurrent respiratory tract infections, most probably on basis of a defect in the clearance of immune complexes.

Adolescent↗
PubMed1987

Phenotypical and functional analysis of B lymphocytes of two siblings with combined immunodeficiency and defective expression of major histocompatibility complex (MHC) class II antigens on mononuclear cells.

Phenotypic and functional analysis of B lymphocytes in two siblings with combined immunodeficiency associated with defective expression of class I and class II major histocompatibility complex (MHC) antigens on mononuclear cells is described. The results of the analysis of the membrane phenotype of the B cells performed at the age of 1 and 5 years, respectively, by the use of monoclonal antibodies against class I (HLA-A, -B, -C) and class II (HLA-DR, -DP, -DQ) MHC antigens showed a decreased expression of class I antigens and a complete lack of class II antigens. Class I antigen expression consistently remained of the same magnitude during follow-up. Class II antigen expression remarkably had been positive early in life on B cells and activated T cells, whereas monocytes were negative for class II from birth onward. B lymphocytes of both patients responded in vitro to polyclonal activation with Staphylococcus aureus Cowan I staphylococci (SAC) with the production of IgM-type immunoglobulins only. This neonatal type of response was in agreement with the membrane immunoglobulin phenotype of the B cells since a high sIgM/sIgD ratio characteristic of neonatal B cells was present. However, the expression of the FMC7 antigen on B cells of both patients was comparable to that on B cells of normal adults. We hypothesized that the lack of MHC antigen expression may impose a resting state on the lymphocytes in these patients due to ineffective cellular interactions. In this view the high sIgM/sIgD ratio reflects the activation state of the B cells rather than the maturational state of the cells.(ABSTRACT TRUNCATED AT 250 WORDS)

B-Lymphocytes↗
PubMed1987

A new case of purine nucleoside phosphorylase deficiency: enzymologic, clinical, and immunologic characteristics.

Deficiency of purine nucleoside phosphorylase (PNP) was detected in a 3-yr-old boy who was admitted for investigation of a behavior disorder and spastic diplegia. The urinary excretion of purines, analyzed by high-performance liquid chromatography, showed the presence of large amounts of (deoxy)inosine and (deoxy)guanosine and low uric acid levels. Analysis of the (deoxy)nucleotide pools of erythrocytes showed elevated levels of deoxyguanine nucleotides and NAD and decreased guanine nucleotides. PNP activity in red blood cells was 0.1-0.5% of normal on two occasions and undetectable on four later measurements. Furthermore no immunoreactive material could be detected in his red cell lysate using an anti-PNP antiserum. PNP activities in the red cells of the patient's parents were 35 and 50% of normal. The presence of (minor) residual PNP activity in the patient enabled the investigation of some enzyme properties after partial purification. No abnormalities could be detected in substrate affinity for inosine, heat stability, and electrophoretic properties. In the heterozygous parents no signs of a mutant enzyme could be found. The molecular specific activities of the parental enzymes were also normal, indicating that no immunoreactive material attributable to inactive-mutant enzyme subunits was present. A striking feature of the patient is the prevailing neurologic abnormalities presumably caused by the metabolic disorder. A severe lymphopenia exists; however, clinical symptoms of an immune deficiency did not become apparent until the age of 4 yr.

Attention Deficit Disorder with Hyperactivity↗
PubMed1986

Purine nucleoside phosphorylase (PNP) deficiency leading to accumulation of lymphocytes in S-phase.

Freshly isolated mononuclear cells of a patient with purine nucleoside phosphorylase (PNP) deficiency contained 7-11% cells in S-phase. During treatment with deoxycytidine and tetrahydrouridine these cells disappeared from peripheral circulation, indicating that the in vivo accumulation of S-phase cells is caused by a shortage in deoxycytidine triphosphate. In vitro it was not possible to cause a blockade in S-phase by culturing normal or PNP-deficient lymphocytes in the presence of deoxyguanosine.

Deoxycytosine Nucleotides↗
PubMed1985

Heterogeneity of immune defects in three children with a chronic active Epstein-Barr virus infection.

Three children, all girls, showed long-lasting clinical and serologic evidence of chronic active Epstein-Barr virus (EBV) infection. Extremely high serum titers of IgG- and IgA-type VCA antibodies and EA antibodies were present, whereas EBNA antibody titers were in the range of those found in seropositive individuals. All three patients repeatedly showed the presence of nonspecific pokeweed mitogen (PWM)-activatable suppressor cells in the peripheral blood. The analysis of EBV-specific cytotoxic T cells showed that one patient exhibited normal cytotoxicity, whereas a second patient demonstrated no EBV-specific cytotoxicity together with unusually high levels of virus-infected B cells in the blood and lymph node. The third patient repeatedly showed refractoriness of the circulating B cells to EBV infection, probably on the basis of some developmental defect. It was concluded that each patient has his or her own peculiar defect in the virus-host balance, indicating that heterogeneity may underlie the syndrome of chronic active EBV infection in humans.

Adolescent↗
PubMed1983

Antigen-specific plaque-forming cell response of human cord blood lymphocytes after in vitro stimulation by T cell-dependent antigens.

Mononuclear cells isolated from human cord blood (CBL) of full-term neonates were stimulated in vitro with a dose range of T cell-dependent antigens, i.e. ovalbumin or sheep erythrocytes, and tested for the capacity to mount an antigen-specific plaque-forming cell (PFC) response. Both of the antigens used induced in CBL a PFC response with the same kinetics of PFC formation and of the same magnitude as found in cultures of adult peripheral blood lymphocytes (PBL). However, optimal PFC responses in CBL were obtained at a hundredfold lower concentration of the antigens compared with the optimal antigen doses for the induction of a PFC response in adult PBL. This phenomenon was further investigated with respect to the antigen dose dependency of the activation of neonatal B cells and neonatal regulatory T cells. The induction of a PFC response in CBL at antigen concentrations that were suboptimal for adult PBL showed a correlation with the particular antigen dose requirements for the activation of B cells and T helper cells in neonates. Furthermore, the findings suggest that the decrease of the PFC response in CBL stimulated with supraoptimal doses of antigen was not caused by the induction of unresponsiveness at the B cell level or by interference of pregnancy-associated substances with the PFC response, but was rather the result of the activation of antigen-specific T suppressor cells. Neonatal T suppressor cells were activated at antigen concentrations that generated T helper activity in the adult. Thus, although neonatal B cells possess the intrinsic capacity to mature into antigen-specific PFC, the conditions for effective activation of neonatal T cells regulating the B cell response differ from those for the activation of adult regulatory T cells.

Antigens↗
PubMed1983

Comparison of internal diameter of great arteries in congenital heart disease. A cross-sectional echocardiographic study.

Cross-sectional echocardiographic studies were used to measure and compare the internal diameter of both aortic root and pulmonary artery in 104 patients with normally related great arteries. Six groups of patients were assessed: normal, with an intracardiac shunt, with tetralogy of Fallot, with pulmonary stenosis, with aortic stenosis, and with atresia of the right atrioventricular valve orifice. In addition, a postmortem study was carried out in normal heart specimens and in specimens with an atrioventricular septal defect. It appeared that the echocardiographically studied ratio between the aortic and pulmonary artery diameter varied considerably. In normal subjects the pulmonary artery diameter tends to be slightly larger than the aortic root diameter: this observation was confirmed by the postmortem data. In other instances the pulmonary artery diameter appeared to be smaller than the aortic root diameter, for example patients with tetralogy of Fallot and those with tricuspid atresia, whereas the reverse was noted in patients with an intracardiac shunt. This two dimensional study has shown that functional adaptations of the calibre of the great arteries can be measured and identified in patients with congenital heart disease.

Adolescent↗