PubMed Health⌕ Search

Biomedical subjects

J C Nossent

Publications and source records attributed to J C Nossent.

At least 19 recordsLinked to original sources
PubMed2004

Anti-dsDNA antibodies and disease classification in antinuclear antibody positive patients: the role of analytical diversity.

BACKGROUND: The presence of "anti-DNA antibodies in abnormal titres" is a well established criterion for SLE classification, but there is no agreement on the performance of this test. OBJECTIVE: To study the correlation between clinical findings and five different solid and solution phase anti-DNA antibody assays. METHODS: 158 consecutively collected ANA positive sera were studied in a double blind fashion. Anti-DNA antibodies were determined by different solid phase assays (ssDNA-, dsDNA- specific ELISA, EliA anti-dsDNA assay, Crithidia luciliae assay), and by an experimental solution phase anti-DNA assay using biotinylated pUC18 plasmid, human, calf thymus, and E coli DNA. Antibody affinity was determined by surface plasmon resonance. Clinical data were obtained independently of the laboratory analyses and later related to the anti-dsDNA findings. RESULTS: Anti-dsDNA antibodies were most frequently detected by ELISA, but were not specific for SLE as they were present in up to 30% of other disease groups. Those detected by the Crithidia luciliae assay were predictive for SLE, while antibodies binding in solution phase ELISA using the pUC18 correlated strongly with the Crithidia luciliae assay. Surface plasmon resonance analysis showed that antibody binding to pUC18 was not due to higher relative affinity for dsDNA in general, but apparently to specificity for that plasmid DNA. Serum samples from three patients with lupus nephritis were positive in both pUC18 solution phase and Crithidia luciliae assays. CONCLUSIONS: Assay principle selection is decisive for the detection of clinically significant anti-DNA antibodies. Revision of the anti-DNA antibody criterion in the SLE classification may be needed.

Animals↗
PubMed1999

Fibrosing alveolitis predating microscopic polyangiitis.

A 65 year old male was diagnosed with "cryptogenic fibrosing alveolitis (CFA)" and treated successfully with Prednisone. In the year following Prednisone-tapering he presented with livedo reticularis, segmental pauci-immune glomerulonephritis and necrotizing vasculitis of the peripheral nerves, increased pulmonary fibrosis, and the presence of p-ANCA antibodies. Aggressive immunosuppressive treatment of this microscopic polyangiitis (MPA) was successful and also resulted in stabilization of the pulmonary fibrosis. This case illustrates that MPA may present itself monosymptomatic as CFA.

Aged↗
PubMed1998

Systemic lupus erythematosus VII: frequency and impact of secondary Sjøgren's syndrome.

BACKGROUND: The prognosis of patients with systemic lupus erythematosus (SLE) largely depends on the severity of cumulative organ damage during the course of the disease. While Sjøgren's syndrome (SS) predominantly affects exocrine glands, a considerable number of patients develop visceral organ damage. Thus, the occurrence of a secondary SS (2(o)SS) in SLE patients, may result in more extensive organ damage and thereby adversely affect prognosis. PATIENTS/METHODS: 138 patients meeting the 1982 American College of Rheumatology (ACR) classification criteria for SLE were prospectively studied over a mean period of ninety months. 2(o)SS was diagnosed according to the 1993 European Study Group criteria and complication rates and prognosis were compared between patients with and without SS. RESULTS: 27 patients (19%) developed SS after a mean period of 48 months. There was a gradual increase in SS prevalence over time after SLE-onset. 2(o)SS patients were older (mean age 41 vs 35 years, P = 0.03), had less renal disease (19% vs 38%, P = 0.04), more thrombocytopenia (26% vs 9%, P = 0.05) and similar serological profiles (including anti-SSa) as patients without SS. Overall mortality was lower in patients with SS (4% vs 13.5%, P = 0.01), while lifetable analysis showed improved survival estimates for 2 SS patients with borderline statistical significance (P = 0.06). CONCLUSIONS: 2(o)SS develops in about one-fifth of SLE patients in a time-dependent fashion: these patients are older, have less renal involvement and their prognosis is at least as good as for those remaining free of SS.

Adolescent↗
PubMed1998

SLICC/ACR Damage Index in Afro-Caribbean patients with systemic lupus erythematosus: changes in and relationship to disease activity, corticosteroid therapy, and prognosis.

OBJECTIVE: To apply the recently described Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (SLICC/ACR-DI) in a well defined cohort of patients with systemic lupus erythematosus (SLE) and to study its association with disease activity, corticosteroid therapy, and prognosis. METHODS: We conducted a record review of 90 patients with SLE followed at a single center for a mean period of 6 years with periodic evaluations of SLE Disease Activity Index (SLEDAI), cumulative damage according to SLICC/ACR-DI, and therapy. Overall disease activity during the disease course was calculated as weighted averages of SLEDAI (WAS). RESULTS: Mean SLICC/ACR-DI was 0.6 six months after diagnosis and increased to 2.4 at last assessment. Thirteen patients (14%) remained free of accumulated damage at last visit. Index scores showed significant correlations with WAS scores and the number of disease exacerbations (SLEDAI > 10), but not with age, mean daily, or cumulative corticosteroid dosage. High WAS scores were independently associated with poorer survival, but SLICC/ACR-DI scores were not. CONCLUSION: SLICC/ACR-DI scores correlate with overall disease activity, but not with length or intensity of corticosteroid therapy. While easily applicable, its prognostic value is subordinate to that of persistent disease activity.

Adrenal Cortex Hormones↗
PubMed1996

[Low incidence of non-Hodgkin lymphoma but high seroprevalence of HTLV-I in Curaçao].

OBJECTIVE: To determine whether the low incidence of non-Hodgkin's lymphoma (NHL) in Curaçao has changed in comparison with the increase in incidence in many western countries, and to investigate the role of the HTLV-I infection that is endemic in the Caribbean area. DESIGN: Retrospective. SETTING: Curaçao, Netherlands Antilles. METHOD: Retrospective file analysis in the only hospital in Curaçao. RESULTS: During the period 1987-1992, 31 patients had a histologically confirmed diagnosis of NHL resulting in an annual incidence rate of 4.9/100,000 adults. There was a strong age-related increase in NHL incidence rate (0.5 for patients < 30 years to 17.8 for patients > or = 70 years), with a male to female ratio of I. (In the western world the incidence is 12-14, in the seventies it was 4.5 in Curaçao.) Seven of 12 patients (58%) tested were seropositive for HTLV-I. Median survival was 6 months, despite conventional therapy. CONCLUSION: While HTLV-I infection can often be demonstrated in NHL patients in Curaçao, NHL incidence has remained low over the past 25 years.

Adolescent↗
PubMed1993

Incidence and course of symptomatic deep venous thrombosis of the lower extremities in a black Caribbean population.

During a 5-year period 131 patients with symptomatic deep venous thrombosis of the lower extremities (DVT) were identified in a black Caribbean population. Eighty-one patients (61%) had objective evidence (ascending venography), while in 39% the diagnosis was based on clinical findings only. The overall annual incidence rate for definite DVT was 11 per 100,000 person years; there was a steep increase with age in both sexes. Proximal DVT was present in 69% of patients. Swelling (92%), pain on palpation (89%) and tenderness (87%) were the most frequent symptoms, while immobilization (43%) and varicosities (42%) were the most frequent risk factors; DVT was rare during pregnancy (1 in 15,000 deliveries). Seventeen patients (21%) developed pulmonary embolism and five patients (6.2%) died during the hospital stay (four of fatal pulmonary embolism, one due to toxic epidermolysis after venography). We conclude, that symptomatic DVT of the lower extremities has a low incidence in this black Caribbean population, but is nonetheless associated with considerable morbidity and mortality due to pulmonary embolism.

Adult↗
PubMed1993

[AIDS on Curaçao: the first 6 years].

We present our experience with AIDS in Curaçao in the first 6 years after the introduction of a serologic HIV screening method. 71 cases of full-blown AIDS were diagnosed in the period 1-1-1986 to 12-31-1991 giving a mean incidence of 81 cases per million per year. Non-homosexual transmission was the predominant mode of spread of HIV with a low male-to-female ratio of 1.3:1. Kaposi's sarcoma, non-Hodgkin lymphoma and hairy leukoplakia were not found. Survival probability was 48% and 30% at 1 and 2 year after diagnosis respectively, with better survival for female patients. In view of the still rising HIV prevalence rate, the peak in AIDS incidence in Curaçao can be expected between 1993 and 1998 so that the AIDS epidemic will become a tremendous social and economic burden in the Dutch Antilles in the years to come.

AIDS-Related Opportunistic Infections↗
PubMed1993

Cerebral arteriovenous malformations in The Netherlands Antilles. High prevalence of hereditary hemorrhagic telangiectasia-related single and multiple cerebral arteriovenous malformations.

Seventeen patients with symptomatic cerebral arteriovenous malformations (AVMs) were diagnosed between 1980 and 1990 in the Leeward Islands of the Netherlands Antilles. Five patients had multiple AVMs. The annual incidence of symptomatic AVMs was 1.1/100,000. The mean age of presentation was 35 years. In 6 patients cerebral AVMs were associated with hereditary hemorrhagic telangiectasia (HHT); 4 of these patients had multiple AVMs. We conclude that HHT is frequently encountered in Netherlands Antillians with symptomatic and multiple cerebral AVMs.

Adolescent↗
PubMed1993

Course and prognostic value of Systemic Lupus Erythematosus Disease Activity Index in black Caribbean patients.

The course and prognostic value of disease activity measured by the validated Systemic Lupus Erythematosus Disease Activity Index (SLEDAI) was investigated in 68 newly diagnosed black Caribbean cases. A high percentage of patients had clinical renal involvement (78%). Disease activity at onset was mild to moderate (SLEDAI < or = 10) in 36% of patients; about half never reached a higher SLEDAI score, whereas the other half advanced to higher disease activity (SLEDAI > 10). SLEDAI scores decreased significantly over time from diagnosis. Within 3 months after disease onset, 54% of patients reached their maximum SLEDAI scores. There were no differences in clinical features or survival between these patients and those with later (mean, 35 months) maximum disease activity, although the latter had more frequent disease flares. Overall survival was poor (91% and 56% at 1 and 5 years, respectively). High persistent disease activity (weighted average of SLEDAI scores > 10) was independently associated with decreased survival, whereas a high initial SLEDAI, a high maximum SLEDAI, and an increase number of flares were not. The main cause of death was infection, which often was associated with active disease (mean SLEDAI at death, 16 +/- 8.9). SLEDAI was a practical and reliable way of evaluating disease activity but was of limited prognostic value.

Adolescent↗
PubMed1993

Clinical renal involvement in Afro-Caribbean lupus patients.

This study reports the severity of clinical lupus nephritis (LN), defined as the sustained presence of proteinuria and/or cellular casts, in a group of 68 newly diagnosed patients with systemic lupus erythematosus (SLE) in Curaçao. Fifty-four patients (78%) developed clinical signs of LN, of whom 31 (54%) had clinical LN at the time of SLE diagnosis. The probability of developing clinical LN reached 80% in the first 3 years after SLE diagnosis and hardly increased later. No clinical or serological differences existed at the time of SLE diagnosis or at onset of LN between patients with early-or late-onset LN. Survival in patients without LN was 100% at 5 years, while for patients with clinical LN these rates at 1 and 5 years were 91% and 59%, respectively (P = 0.0001); male LN patients had a worse prognosis than females (P = 0.012), while time of LN onset did not influence survival. Six patients (11%) developed end-stage renal failure; all were female, five had early LN and one had late-onset LN (P = 0.17). Renal survival was 97% and 80% at 1 and 5 years, with decreased rates for patients with nephrotic-range proteinuria (P = 0.02). Hypertension was present in 13% of LN patients, but had no influence on patient or renal survival. Thus, clinical LN was a frequent complication, which carried a poor prognosis in these Afro-Caribbean lupus patients.

Adult↗
PubMed1993

Multiple myeloma in the Afro-Caribbean population of Curaçao.

To determine the incidence and course of multiple myeloma (MM) in the Afro-Caribbean population of Curaçao, we studied all MM patients discharged from the only hospital on the island during an 11-year period starting in 1980. As 50 patients fulfilled the diagnostic criteria for MM proposed by Durie, the average annual incidence (AI) of MM was estimated at 3.1/100,000 person years; AI was similar in males and females, but showed a steep increase with age in both sexes; 10% of all MM patients were < 40 years of age. At diagnosis 68% of patients were in Stage III, in 26% serum creatinine levels were > 20 mg/l, 36% had hypercalcaemia, and 50% had multiple bone lesions. Median survival was 20.5 months; Stage III myeloma and bone marrow plasma cell percentage > 50 were independent risk factors for poor survival. Infections were the immediate cause of death in 54% of the non-survivors. We conclude that the incidence rate of MM in the Afro-Caribbean population of Curaçao is one of the lowest reported in black populations; however, the presentation and course of MM follow the pattern seen in most other countries.

Adult↗
PubMed1993

Panniculitis as the first manifestation of systemic lupus erythematosus: description of two cases.

Two patients are described in whom panniculitis proved to be the first symptom of the disease systemic lupus erythematosus (SLE). Histological examination of a painless solitary cutaneous lesion in the otherwise asymptomatic first patient showed a nonspecific nodular panniculitis; 1 yr later a definite diagnosis of SLE was made. In the second patient panniculitis had the clinical appearance of erythema nodosum and was one of several presenting symptoms of SLE. These patients were part of a group of 68 newly diagnosed patients with SLE, indicating a 3% prevalence of panniculitis in SLE. These cases illustrate that SLE should always be considered as an underlying disease in young female patients presenting with nodular panniculitis, which may mimic the clinical picture of erythema nodosum.

Adult↗
PubMed1992

[Cerebrovascular accidents at a young age in Rendu-Osler-Weber disease; a survey in the Netherlands Antilles].

Thirty-two patients (17 men and 15 women) are presented in whom the diagnosis Rendu-Osler-Weber disease was established between 1980 and 1990 during hospitalisation in the Leeward Islands of the Netherlands Antilles. Among this group there were eight families, each of which contained two patients. Estimated point-prevalence rate in 1991 was 19.4/100,000, which is ten times higher than has been reported so far. Epistaxis, gastrointestinal blood loss and cerebrovascular accidents (CVA) were the main presenting symptoms. Mean age at diagnosis was 53.2 years. Arteriovenous fistulae in the lung developed in 11 patients (34%). Eleven patients experienced a total of 12 CVAs at a mean age of 48 years; four of these patients had an ischaemic CVA in the presence of pulmonary fistulae, while three patients had a haemorrhagic stroke in the presence of a cerebral arteriovenous malformation. Thus, cerebrovascular accidents at an early age are common complications in Rendu-Osler-Weber disease and warrant further investigation of this disease among inhabitants of the Leeward Islands of the Netherlands Antilles.

Adult↗