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Biomedical subjects

C Vermylen

Publications and source records attributed to C Vermylen.

At least 73 records · Page 4Linked to original sources

Parotid gland abnormality found in children seropositive for the human immunodeficiency virus (HIV).

Out of our series of 24 children seropositive for the Human Immunodeficiency Virus (HIV), parotid gland enlargement was noted in 4 children with AIDS-related complex (ARC) presenting also a Lymphocytic Interstitial Pneumonitis (LIP) on their chest radiographs. The ultrasound (US) aspect of the parotid gland suggests acinar enlargement (suggesting the presence of lymphocytic infiltration). The aspect displayed in the parotid mirrors the process developing in other areas (lungs, liver, spleen, lymph-nodes), i.e. a syndrome of lymphocytic (CD8) proliferation present at the stage of ARC.

Child, Preschool↗

Progressive tubulointerstitial nephropathy with hepatic involvement in an infant.

We describe an infant with renal and liver disease who died at the age of 10 months from chronic renal failure. Hepatosplenomegaly was present along with biochemical findings of cholestasis and cytolysis. The diagnosis of tubulointerstitial nephropathy with cortical microcysts associated with hepatic fibrosis was based upon laboratory, radiological, and histological data. This rarely described disorder is characterized by an early clinical onset and unfavourable progression to end-stage renal failure before the age of 2.

Humans↗

Treatment of immune thrombocytopaenic purpura in childhood. A review of 146 patients.

Between 1969 and 1988, 146 children with immune thrombocytopaenic purpura (ITP) were seen in the outpatient clinic. The diagnosis was based upon an isolated thrombocytopaenia, without disseminated intravascular coagulation, splenomegaly or systemic disease and a normal bone marrow. Patients who required treatment (116/146) received either steroids (105/116) or IV gammaglobulins (IV-Ig) (11/116) as initial therapy. The long term outcome was similar in both groups. IV-Ig had the advantage to give a rapid increase in the platelet count without major side effects and to be very useful in chronic ITP either as maintenance therapy or as preparation for splenectomy.

Adolescent↗

Combined CO2-laser and alfa recombinant interferon treatment in five children with juvenile laryngeal papillomatosis.

Juvenile laryngeal papillomatosis is a rare and benign tumoral disease of childhood characterized by numerous relapses despite complete resection. The ENT treatment of choice is to vaporize the papillomas with a CO2 laser. Since the discovery of a viral etiology (Human Papilloma Virus), resection has been followed by medical attempts to control the disease by using various antiviral treatments. Among the latter, alfa interferon has proved effective during the first six months of treatment. In this article, we report on five cases of refractory juvenile laryngeal papillomatosis treated by excision (CO2 laser in four children, surgical resections in one child) and alpha-r IFN 1.5 x 10(5) U/kg daily. With this strategy, three of the five children are currently disease-free for periods ranging from 22 to 68 months. This series includes one remarkable observation of one child who responded only to double doses of alpha-r IFN, after initial failure at conventional doses. This therapeutic scheme reduced the frequency of relapses in a fourth child. In only one child the treatment did fail to modify the natural course of the disease. Side effects were tolerable and included anorexia (one case), palmar erythema (one case), a flu-like syndrome (two cases) and mild transient transaminase rise (three cases) not precluding further treatment. CO2-laser caused one laryngeal oedema and synechia of the anterior commisure of the vocal laryngeal cords in one other case.

Carbon Dioxide↗

Familial essential thrombocythemia.

We report three siblings (2 F, 1 M) aged 9, 12, and 15 years with a thrombocytosis, above 650 X 10(9)/l for at least 18 months and a leukocyte alkaline phosphatase score below 37. The diagnosis of essential thrombocythemia was based upon the exclusion of a reactive thrombocytosis or another myeloproliferative disease. Cytogenetic examination of marrow cells was normal.

Adolescent↗

Cyclosporine A in five children with severe aplastic anemia.

Five children with aplastic anemia (AA) were treated with Cyclosporine A (CyA) after failure or poor response to other immunosuppressive agents. A complete remission was achieved in 3 patients, a good partial response in 1, and a poor partial response in another. In 1 patient in whom a complete remission was obtained, a relapse occurred 4 months after CyA was stopped. The treatment was resumed and a complete remission was again observed. Four patients were still on treatment, and side effects were mild. CyA appeared to be an effective treatment in AA.

Administration, Oral↗

Glomerular and urinary heparan sulphate in congenital nephrotic syndrome.

Studies using cationic probes have suggested that a reduction in glomerular anionic sites, composed principally of the glycosaminoglycan heparan sulphate, is responsible for the abnormal glomerular permeability in the congenital nephrotic syndrome (CNS). We therefore analysed the glycosaminoglycan content of the glomerular basement membrane (GBM) from an infant who died of CNS and from an infant who died of unrelated causes. We also measured the urinary excretion of glycosaminoglycans in children with nephrotic syndrome, both congenital and acquired, and in healthy children. Heparan sulphate constituted 59% of the glycosaminoglycan content of the GBM in the normal infant, the other principal glycosaminoglycan being chondroitin sulphate. In the GBM from the infant with CNS the heparan sulphate was greatly reduced, constituting only 3% of total glycosaminoglycans. The urinary excretion of heparan sulphate was significantly increased in CNS (expressed both in relation to creatinine and to chondroitin sulphate) compared with normal children and to those with acquired nephrotic syndrome. Diminished GBM content of heparan sulphate may be responsible for the abnormal glomerular permeability in CNS and may be a consequence of defective incorporation of heparan sulphate into the GBM with subsequent loss into the urine.

Basement Membrane↗

Yersinia enterocolitica in donor blood: a case report and review.

Routine sterility control of a unit of leukocyte-depleted erythrocyte concentrate yielded growth of Yersinia enterocolitica serotype O:3. Plasma of the donor showed a high titer of agglutinins against the homologous organism. Although the donor was apparently well at the time of donation, he had a history of protracted terminal ileitis treated by surgery. The recipient of the contaminated blood was taking broad-spectrum antibiotics and did not experience any adverse effect. Fourteen other cases of transfusion-associated yersiniosis have been reported.

Adult↗

Itraconazole versus ketoconazole for the prophylaxis of fungal infection in neutropenic children: results of two consecutive nonrandomized studies.

Two consecutive nonrandomized studies were conducted in children with prolonged granulocytopenia to evaluate the prophylactic antifungal activity of ketoconazole and the new triazole itraconazole. The conditions were equivalent in both studies. The incidence of colonization was 10% in the ketoconazole group and 19% in the itraconazole group (this difference is not significant). For suspected and proven infections, the incidence was 5% for ketoconazole and 10% for itraconazole, but the incidences were too low for statistical comparison. Although the colonization rate was higher for itraconazole, there was no sign of Aspergillus, whereas in the ketoconazole group an autopsy, proven aspergillosis has been reported.

Adolescent↗

[Deletion of the long arm of chromosome 18, primary hypothyroidism, Biermer's anemia and IgM hypogammaglobulinemia].

We report the case of an adolescent girl who presents with the 18q-syndrome, primary hypothyroidism, pernicious anemia and IgM hypogammaglobulinemia. Her karyotype was performed during infancy because of malformations and showed deletion of the long arm of chromosome 18. The patient had been treated with levothyroxine (Elthyrone) since age 13 when primary hypothyroidism was documented. A close hematological follow-up was then undertaken due to the presence of anti-parietal cell antibodies. A megaloblastic anemia of sudden offset led to the diagnosis of pernicious anemia by age 16, which was confirmed by a positive Shilling's test. Recently, the patient was found to have antimicrosome antibodies and moderate IgM hypogammaglobulinemia.

Adolescent↗

Bone marrow transplantation in five children with sickle cell anaemia.

Five children with severe sickle cell anaemia underwent an HLA compatible allogeneic bone marrow transplantation. In four children the engraftment was rapid and sustained. The fifth child rejected the bone marrow graft and required a second bone marrow transplantation 62 days after the first one. The outcome was then uneventful. In all cases there was complete cessation of vaso-occlusive episodes and haemolysis. The haemoglobin electrophoretic pattern became similar to that of the donor (AA or AS) and cytogenetic studies in three patients confirmed the donor origin of bone marrow cells.

Anemia, Sickle Cell↗

Inhibition of lipoprotein lipase by plasma from children with the steroid responsive nephrotic syndrome.

The study was designed to investigate the hyperlipidaemia associated with the steroid responsive nephrotic syndrome in children and in particular to examine the mechanism for the delayed clearance of the circulating triglyceride-rich lipoproteins. The possibility that plasma from patients with steroid responsive nephrotic syndrome may contain an inhibitor of lipoprotein lipase activity was studied by examining the effect of the addition of plasma from patients, on normal postheparin lipoprotein lipase activity. Plasma from children with steroid responsive nephrotic syndrome significantly inhibited lipoprotein lipase activity (p less than 0.001), whereas that from patients with familial hypercholesterolaemia and normal children had no significant effect. The inhibition of lipoprotein lipase activity by plasma from patients with steroid responsive nephrotic syndrome correlated significantly with their increased plasma cholesterol and reduced plasma albumin concentrations (p less than 0.001 and less than 0.02, respectively), but there was no significant correlation with plasma triglyceride concentrations. Thus, the degree of inhibition probably reflected the severity of the condition at the time of study. Neither the cholesterol, albumin nor triglyceride concentrations appeared to directly influence the lipoprotein lipase activity of postheparin plasma.

Adolescent↗