Biomedical subjects
Publications and source records attributed to R Blanco.
Small-vessel vasculitis may be a paraneoplastic syndrome. It may be associated with haematologic malignancies and less frequently with solid tumors. We describe a patient with myelodysplastic syndrome presenting as Henoch-Schönlein Purpura. To our knowledge this association has not previously reported.
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PURPOSE: To evaluate the clinical features and outcome of patients with isolated hypersensitivity vasculitis (HV). PATIENTS AND METHODS: Retrospective study of patients with cutaneous vasculitis followed up at a University Hospital from 1975 to 1994. Patients with vasculitis secondary to collagen vascular diseases, neoplasia, or major infections were excluded. Patients were classified as HV according to the differential criteria proposed by Michel et al (J Rheumatol. 1992;19:721-728). RESULTS: Ninety-five patients were classified as HV. The mean age was 42.7 +/- 21.7 years, with similar disease frequency in both sexes. In 43 patients, the precipitating event was drug therapy, either alone or as a treatment for a coexistent infection, usually an upper respiratory tract infection. The most frequent clinical manifestation was palpable purpura followed by joint symptoms. Systemic involvement was infrequent: 7 patients had nephropathy, manifested almost exclusively by microhematuria, and 5 patients had gastrointestinal symptoms. In 54 subjects the vasculitis did not require treatment; 26 patients were treated with NSAIDs, and 14 required corticosteroids (associated to immunosuppressive agents in 2 of them). After a mean follow-up of 15.5 +/- 28.9 months (median 6), only 2 patients had slight renal impairment, whereas the remaining had a complete recovery. CONCLUSION: Hypersensitivity vasculitis is usually a benign syndrome, often secondary to drugs or infections, or both. Its main clinical manifestations are skin and joint symptoms. The systemic involvement is scarce and its prognosis is excellent.
OBJECTIVE: To identify in polymyalgia rheumatica the best set of predictors for a positive temporal artery biopsy and to define predictive models with either a high or low probability of giant cell arteritis (GCA). PATIENTS AND METHODS: Retrospective study of 227 patients, 137 with polymyalgia rheumatica unassociated with arteritis (group A) and 90 with polymyalgia associated with biopsy-proven giant cell arteritis (group B or training set). Data on demographic features, clinical and laboratory abnormalities were collected. Risk factors for arteritis were estimated by nonlinear logistic regressions. Simple predictive models were constructed with those predictors more related to arteritis by multivariable analysis. These models were then tested in group B and in 89 cases of arteritis without polymyalgia rheumatica (group C or test set). RESULTS: The best predictors of arteritis were a new headache odds ratio (OR) 13.6 (95% confidence interval [CI] 4.7 to 39.3); age at onset < 70 years OR 0.11 (CI 0.04 to 0.35); abnormal temporal arteries OR 4.2 (CI 1.3 to 13.7); raised liver enzymes OR 2.9 (CI 1.1 to 7.8), and jaw claudication OR 4.8 (CI 1.0 to 22.7). Amaurosis was only observed in patients with arteritis. Three subsets had a very high risk of arteritis: (1) Patients with recent headache, abnormal arteries, and > or = 70 years at disease onset: sensitivity 44%, positive predictive value (PPV) 93%, likelihood ratio (LR) 20.3; (2) patients with a new headache, jaw claudication, and abnormal arteries: sensitivity 34.4%, PPV 96.9%, LR 47.2; and (3) those, that in addition to the last 3 features, were > or = 70 years of age at disease onset: sensitivity 26.7%, PPV 100%. We could also identify a subset with a very low risk of arteritis constituted by patients < 70 years, without headache, and with clinically normal temporal arteries: sensitivity 1.1%, PPV 1.7%, LR 0.03. In group C or the test set, these four predictive models correctly identified 57.3%, 29.2%, 23.6, and 3.4% of patients, respectively. CONCLUSIONS: In polymyalgia rheumatica it is feasible to identify subsets with a very high likelihood of GCA. Although in some of these subsets the diagnosis of arteritis is almost certain, we suggest that even then it should be confirmed by temporal artery biopsy. By contrast, in those patients with polymyalgia < 70 years and without cranial features of giant cell arteritis, the risk of vasculitis is so low that the biopsy could be initially avoided and the patient treated with low-dose corticosteroids.
BACKGROUND: Debate as to whether surgery in severe necrotizing pancreatitis (SNP) should be done early or late has been present ever since the disease was described. There are no prospective, randomized studies addressing this specific issue. METHODS: Patients with SNP, documented clinically, with Ranson's criteria, and dynamic pancreatography (DP) findings were randomly allocated in two groups for treatment. Group A included early necrosectomy (within 48 to 72 hours of onset) and group B, late necrosectomy (at least 12 days after onset). Both groups continued with open packing and staged necrosectomies. Cultures were obtained at each laparotomy and necrosis was verified histologically in all instances. RESULTS: During a 36-month study period, 150 patients with unequivocal acute pancreatitis were admitted for treatment. Forty-one with SNP initially entered the study; there were 5 drop outs. Patients in group A (25) and group B (11) had no difference in distribution by gender or mean age, etiology, mean Ranson's signs (4 versus 3.8), DP findings, rate of infected necrosis, or necrosectomies required per patient. Although the mortality rate (58% versus 27%) did not reach statistical significance, the odds ratio for mortality was 3.4 times higher in group A, which made us finish the study. CONCLUSION: This prospective, randomized study from a single institution clearly demonstrates that early intensive conservative treatment with late necrosectomy for selected cases is the current rationale approach for SNP.
The eruption of the deciduous teeth in Down's individuals is reportedly delayed, but the extent of delay in comparison to normal children has been little studied. The eruption characteristics of the deciduous teeth in a sample of Chilean individuals with Down's syndrome were compared with those of the normal Chilean population. The sample consisted of 255 Down's individuals (all with trisomy 21), 127 males and 128 females. Boys with Down's syndrome showed significantly delayed eruption in six teeth: in the maxilla the right central incisor and right and left lateral incisors, and in the mandible the right central incisor and right and left canines. Girls with Down's syndrome showed significant delays in the eruption of 11 teeth: in the maxilla the right and left lateral incisors, right and left canines and first left molar, and in the mandible the left central incisor, right and left lateral incisors and canines and second right molar. The chronological sequence of eruption in Down's children was not completely different from that of normal individuals. With a few exceptions no significant departures from Gaussian distribution were found in the age of eruption among both normal and Down's individuals. The variance was significantly larger in cases of Down's syndrome.
A case of autoimmune disease-associated lymphadenopathy (ADAL) with histological, immunophenotypic, Epstein-Barr virus (EBV) in situ hybridization, and genotypic analyses is presented. The patient had a well-documented history of systemic lupus erythematosus (SLE) and was found at autopsy to have massive lymphadenopathy, thymic enlargement, pulmonary nodules, and polyclonal serum dysproteinemia. Histological examination revealed a polymorphous lymphoid infiltrate containing many plasma cells, rare immunoblasts, and a pronounced arborizing vasculature. No foci of necrosis were found and there was no evidence of lymphocyte depletion. The plasma cells were immunophenotypically polyclonal and no EBV mRNA (EBER-1) or gene rearrangements were identified. The unusual gross features, which resembled a malignant lymphoproliferative process, as well as the unusual histological features make this case a notable addition to the spectrum of atypical lymphoproliferative disorders associated with an autoimmune disorder. We conclude that although reminiscent of angioimmunoblastic lymphadenopathy with dysproteinemia (AILD), this case lacks the diagnostic features of AILD, and is, perhaps, best classified as an autoimmune disease-associated lymphadenopathy (ADAL).
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The objective was to assess the efficacy of therapy with danazol in refractory immune thrombocytopenia associated with different rheumatic diseases. Patients with severe immune thrombocytopenia (platelet counts < 40 x 10(9)/l) with a bone marrow biopsy showing megakaryocytes in normal or increased number and normal morphology were included if they fulfilled at least one of the following criteria: (a) thrombocytopenia refractory to prednisone (> or = 1 mg/kg/day during > or = 4 weeks); (b) patients requiring an unacceptably high dose of prednisone for > 2 months (prednisone dose > or = 20 mg/day); (c) no response to at least another drug besides corticosteroids. Other causes of thrombocytopenia were excluded. They were treated with danazol (100-200 mg q.i.d.) and followed for at least 12 months. Four patients diagnosed with systemic lupus erythematosus, two with rheumatoid arthritis and one with primary antiphospholipid syndrome met the inclusion criteria. All of them achieved acceptable platelet counts within the first 4 weeks of danazol therapy that allowed the prednisone dosage to be tapered. No important side-effects related to danazol therapy were observed. Danazol therapy seems to be a useful and well-tolerated treatment for refractory immune thrombocytopenia associated with different rheumatic diseases.
Intraperitoneal administration of methylazoxymethanol (MAM) acetate (0.05 microl/g of body weight) in male Sprague-Dawley rats aged 3 days produced cell death in the external granule layer of the cerebellum which peaked at 48 hours (h) and was followed by removal of cellular debris at 72 h. Dying cells had the morphological features of apoptosis and were stained with the method of in situ labeling of nuclear DNA fragmentation. Strong c-Jun immunoreactivity was observed in apoptotic cells during the whole process of MAM-induced apoptosis. No differences of c-Fos immunoreactivity were observed between control and MAM-treated rats throughout the period studied. Western blotting of cerebellar homogenates in control rats disclosed two bands which reacted with both c-Jun antibodies, one located at p39 that corresponds to the molecular weight of c-Jun, and the other at about p62. MAM-treated rats showed a robust band at p62, together with a thinner band located immediately above it, which was accompanied by a reduction of the p39 band. The specificity of the immunoreaction was tested by incubating the antibodies with the appropriate control peptides. No difference between control and MAM-treatad rats was observed in Western blots processed with antibodies to c-Fos during this study. These results show that MAM-induced apoptosis in the external granule cell layer of the rat is associated with strong c-Jun expression, which is restricted to apoptotic cells, and with the formation of high-molecular-weight c-Jun complexes. Taken together, the present observations suggest that c-Jun may participate in the genetic cascade of events leading to apoptotic cell death in the developing cerebellum.
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A retrospective study of 112 patients with septic arthritis (SA) caused by pyogenic organisms and fungi attended at the same Medical Service from 1975 to 1994. This SA was more common among male patients and the age at onset of disease was 42 +/- 23.2 years. Predisposing factors were identified in 53 patients, the more common being connective tissue diseases and parenteral drug abuse (PDA). The main portal of entry was bacteremia from a distant infective source. The most common causative agents were Grampositive cocci (particularly S. aureus) and the more uncommon organisms were recovered from patients with underlying diseases. The diagnostic delay was 14.1 +/- 28.1 days. Single joint SA occurred in 88% of patients. Large joints were mainly involved. On admission, 25% of patients had no fever and only 54% of them had leukocytosis; therefore, the lack of these findings in a given patient with acute arthritis should not rule out the possibility of SA. Initial therapy consisted of daily arthrocentesis and i.v. antibiotics. The mean duration of antibiotic therapy was 47 +/- 23 days; the duration of the i.v. route was 23 +/- 14 days. An eighty percent of patients recovered without functional sequelae. The 31 patients who required surgical drainage had a longer diagnostic delay and a worse evolution, thus stressing the necessity of an early diagnosis. In the last decade (1986-1994) an increase in some predisposing factors (iatrogenic SA and PDA), a shorter diagnostic delay, a more prolonged antibiotic therapy, a smaller proportion of surgical drainage and an ultimate evolution identical to that in the previous decade were observed.
OBJECTIVE: Progressive increases in the incidence rate of giant cell arteritis (GCA) have been observed in different geographic areas. The incidence of GCA in Lugo, Northwestern Spain, was previously considered low. Our aim was to analyze trends in incidence and clinical features of GCA in Lugo. METHODS: Retrospective study of biopsy proven GCA diagnosed from January 1, 1986 through December 31, 1995. The average annual incidence rate of GCA for population age > or = 50 years was analyzed at 5 year intervals from 1986 to 1995, inclusive. A comparative study of clinical features and laboratory findings of GCA in patients diagnosed 1991-1995 with those diagnosed 1986-1990 was performed. RESULTS: Forty-one and 52 Lugo residents were diagnosed with GCA in the 1986-1990 and 1991-1995 time periods, respectively. For each period the average annual incidence rate for population age > or = 50 years was 8.26 and 10.49/10(5), respectively. A lower frequency of classic features of GCA such as constitutional symptoms (67.3 vs 95.1%) and polymyalgia rheumatica (30.8 vs 51.2%) was observed in patients diagnosed 1991-1995. Other typical findings were less common than in the 1986-1990 period, namely, headache (82.7 vs 87.8%), abnormal examination of temporal artery (61.5 vs 70.7%), jaw claudication (36.5 vs 43.9%), and amaurosis fugax (9.6 vs 14.6%). There was a longer delay to diagnosis 1991-1995 than 1986-1990 (12.7 +/- 12.1 wks vs 8.9 +/- 6.2). Also, at the time of diagnosis, anemia, thrombocytosis, and elevated alkaline phosphatase were less frequently observed in the period 1991-1995. CONCLUSION: In recent years, we observed a progressive increase in the incidence of GCA in our area. Such an increase correlates with lower frequency of classic manifestations of GCA.
OBJECTIVE: To assess the longterm survival of patients with giant cell arteritis (GCA) in a well defined area in Northwestern Spain. METHODS: A followup study of consecutive biopsy proven patients with GCA diagnosed in Lugo, Spain January 1, 1982-March 31, 1996 was performed. Patients were followed from time of diagnosis until either their death or October 1, 1996. Time and cause of death were reviewed. Statistical methods included standardized mortality ratio (SMR), and Kaplan-Meier product-limit survival analysis. Cox proportional hazard models were used to identify clinical features and laboratory findings associated with survival. RESULTS: By October 1, 1996, full information about 109 biopsy proven patients with GCA (59 men/50 women) was available. The mean age +/- SD at the time of diagnosis was 73.9 +/- 7.3 years for women and 74.1 +/- 5.8 for men (p = NS). After a median followup of 54 months, 22 patients (20.2%) had died. Three died within the first month after diagnosis due to either vascular complications related to GCA or therapy complications. Apart from a history of severe underlying diseases (comorbid condition unrelated to GCA), neither sex nor any clinical features of GCA were significantly associated with an increase in mortality. As in the general population of the same age in Lugo, the majority of deaths were due to cardiovascular and cerebrovascular complications. SMR was 0.80 (95% CI 0.47-1.13). One, 2, 5, and 10 year survival rates were 95, 91, 81, and 62%, respectively. Hazard function was 1.8% at Day 30 after diagnosis and remained low until the end of the first year of treatment. Thereafter, mortality increased slightly. As this function was constant, we applied an exponential model. The estimated risk of death with this model was 5.3% per year. CONCLUSION: Longterm mortality of GCA in our area is low. However, it may be possible to further lower the mortality rate through early diagnosis and careful followup.
This study is focused to learn about the cellular localization of transcription factors binding to the cAMP response element-CREBs-in the brain of normal rats and in animals subjected to excitotoxic cell damage. For this purpose, CREB-1 and CREB-2 immunoreactivity is examined in the developing and adult rat brain under physiological conditions, and following systemic kainic acid (KA) injection at convulsant doses in the adult, as a validated experimental model of excitotoxic injury. CREB-1 immunoreactivity is constitutively expressed in periventricular glia and Bergmann glia, and appears in reactive astrocytes following KA-induced excitotoxic cell damage. In contrast, CREB-2 is constitutively expressed in all neurons of the cerebrum, cerebellum and brain stem in the developing and adult brain. CREB-2 immunoreactivity is not increased following KA excitotoxic cell damage. These results demonstrate that CREB-1 and CREB-2 in the brain of the rat are localized in separate cellular compartments and that their expression is differentially regulated in pathologic states.