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T Papo

Publications and source records attributed to T Papo.

At least 37 records · Page 2Linked to original sources
PubMed2001

[Fainting after drinking a glass of whiskey-soda...].

BACKGROUND: Alcohol intolerance is a rare syndrome that usually involves pain, pruritus, or vasomotor phenomena consecutive to moderate alcohol intake. This syndrome may unmask various malignancies, especially Hodgkin's disease or non-Hodgkin lymphoma, and carcinoma of the cervix. CASE REPORT: A patient who became unconscious after drinking a glass of whiskey-cola was explored for alcohol intolerance. High-level hypereosinophilia was discovered. The diagnosis of idiopathic hypereosinophilia was retained after extensive evaluation. DISCUSSION: Idiopathic hypereosinophilia may be a premalignancy expression of a lymphoproliferative disorder. In our patient, who also had alcohol intolerance known to be associated with later diagnosis of malignancy, lymphocyte phenotype studies revealed an abnormal population. However, rearrangement studies of T-cell and B-cell receptors, as well as bone marrow cytology and histology did not enable identification of a lymphoproliferative disorder. This patient will require close follow-up due to the risk of developing a lymphoproliferative disorder.

Abdominal Pain↗
PubMed2001

Dermatologic manifestations of relapsing polychondritis. A study of 200 cases at a single center.

Dermatologic manifestations of relapsing polychondritis (RP) have been relatively poorly studied compared to other manifestations. In this study we describe dermatologic manifestations in a large series of patients with RP and the corresponding pathologic findings. In this retrospective, single-center review of 200 patients diagnosed with RP according to Michet's criteria, we analyzed separately those suffering from associated diseases with potential dermatologic involvement or chronic dermatitis. Skin or mucosal biopsies taken from 59 patients were examined without knowledge of the clinical data. Among the 200 patients with RP, 73 had chronic dermatitis or associated diseases with potential dermatologic involvement, especially hematologic disorders (n = 24) and connective tissue diseases (n = 22). Among the other 127 patients, 45 (35.4%) had dermatologic manifestations: aphthosis (n = 21; oral in 14 and complex in 7), nodules on the limbs (n = 19), purpura (n = 13), papules (n = 10), sterile pustules (n = 9), superficial phlebitis (n = 8), livedo reticularis (n = 7), ulcerations on the limbs (n = 6), and distal necrosis (n = 4). Dermatologic manifestations were the presenting feature of RP in 15 cases (12%), and appeared concomitantly (n = 23) or not (n = 22) with attacks of chondritis. Histologic findings included vasculitis (n = 19, leukocytoclastic in 17 and lymphocytic in 2), neutrophil infiltrates (n = 6), thrombosis of skin vessels (n = 4), septal panniculitis (n = 3), and minor changes (n = 2). Patients with and without dermatologic manifestations did not differ with regard to male/female ratio; age at RP onset; frequency of auricular, nasal, or tracheobronchial chondritis; or frequency of rheumatologic, ocular, audiovestibular, renal, arterial, or venous involvement. The frequency of dermatologic manifestations (91% versus 35.4%; p < 0.0001), sex ratio (18 male/4 female versus 44 male/83 female, p < 0.0001), and age at first chondritis (63.3 +/- 14 yr versus 41.4 +/- 17 yr; p < 0.0002) were significantly higher in the 22 patients with myelodysplastic syndrome than in the 127 patients without any associated disease. In conclusion, although dermatologic manifestations occur frequently in patients with RP, especially in association with myelodysplasia, they are nonspecific and sometimes resemble those observed in Behçet disease or inflammatory bowel diseases. Their presence in the elderly warrants repeated blood cell counts to detect a smouldering myelodysplasia.

Adolescent↗
PubMed2000

A novel missense mutation (C30S) in the gene encoding tumor necrosis factor receptor 1 linked to autosomal-dominant recurrent fever with localized myositis in a French family.

OBJECTIVE: To characterize both phenotypic (clinical features and magnetic resonance imaging [MRI] findings) and genotypic aspects of autosomal-dominant recurrent fever, also known as tumor necrosis factor receptor (TNFR)-associated periodic syndrome (TRAPS), in a French family and to investigate the role of the mutated 55-kd tumor necrosis factor alpha (TNFalpha) receptor (TNFR1) in the pathogenesis of the disease. METHODS: The coding region of TNFR1 was sequenced in 2 individuals with TRAPS (the propositus and her grandfather) and in 3 clinically unaffected relatives. Expression of soluble TNFR1 (sTNFR1) was investigated in 3 of the family members carrying a C30S mutation in TNFR1, and was compared with the levels of soluble TNFR2 (sTNFR2) by enzyme-linked immunosorbent assay. The membrane TNFR1 expression was then compared with membrane TNFR2 levels at the surface of peripheral blood mononuclear cells by flow cytometric analysis. The clinical heterogeneity in this French family was investigated by searching polymorphic variants in the TNFalpha promoter by DNA sequencing. RESULTS: Both the disease course and the clinical presentation in the propositus were highly indicative of TRAPS. MRI study of the segmental inflammatory process in the limbs showed abnormal signals in the muscle and subcutaneous tissue without involvement of adjacent joints or fascia. A novel missense mutation, C30S, in the first extracellular N-terminal cysteine-rich domain (CRD1) of TNFR1 was characterized in the propositus, her affected grandfather, and her clinically unaffected father. Expression of membrane TNFR1 at the surface of monocytes and polymorphonuclear leukocytes, as well as the levels of sTNFR1 in serum when the disease was not active were not modified in the 3 individuals carrying the TNFR1 C30S mutation. In contrast, during attacks, sTNFR1 levels remained abnormally low, as compared with the levels in unrelated patients with active adult-onset systemic Still's disease. The clinical heterogeneity could not be explained by a polymorphic variant in the TNFalpha promoter. CONCLUSION: TRAPS is a distinct clinical and radiologic disease entity that is responsible for recurrent fever and migratory cellulitis-like processes with localized myositis. We have identified a novel TNFR1 mutation, C30S, that is located in the CRD1 domain in a French family affected by the disease. This mutation seems to affect the level of sTNFR1, which did not increase in the propositus during inflammatory attacks.

Adolescent↗
PubMed2000

Systemic vasculitis with bilateral perirenal haemorrhage in chronic myelomonocytic leukaemia.

The cases of two patients with chronic myelomonocytic leukaemia associated with periarteritis nodosa-like, antineutrophil cytoplasmic antibody negative, systemic vasculitis, are reported. A 61 year old man was admitted with fever, diffuse myalgia, and abdominal pain. Blood and bone marrow examination showed chronic myelomonocytic leukaemia. Vasculitis of the gall bladder was responsible for acalculous cholecystitis. A massive spontaneous bilateral perirenal haemorrhage occurred. A 73 year old woman with chronic myelomonocytic leukaemia had been followed up for one year when unexplained fever occurred. Two months after the onset of fever, sudden abdominal pain was ascribed to spontaneous bilateral renal haematoma related to bilateral renal arterial aneurysms. Neuromuscular biopsy showed non-necrotising periarteriolar inflammation. To our knowledge, systemic vasculitis has never been reported in chronic myelomonocytic leukaemia. In our two cases a non-random association is suggested because (a) chronic myelomonocytic leukaemia is a rare myelodysplastic syndrome, (b) spontaneous bilateral perirenal haematoma is not a usual feature of periarteritis nodosa.

Aged↗
PubMed1999

[Polyvalent intravenous immunoglobulins in systemic lupus].

PURPOSE: To evaluate intravenous immunoglobulins (IVIG) treatment, which is immunomodulatory but not immunosuppressive, in SLE. MAIN ISSUES: IVIG indications in SLE could be categorized as already validated as for chronic polyradiculoneuropathy or thrombocytopenia; failure of classical treatment in threatening active disease; undetermined manifestation as reactive hemophagocytic syndrome which could be both disease-specific or iatrogenic, infection-related. To date, no published study has firmly established the efficacy of IVIG in SLE. CONCLUSION: IVIG therapy in SLE should be evaluated in prospective trials.

Humans↗
PubMed1999

Renal involvement in systemic lupus erythematosus. A study of 180 patients from a single center.

Charts of 180 patients (147 women, 33 men) with systemic lupus erythematosus (SLE) complicated by renal involvement were retrospectively analyzed from a series of 436 patients. Mean age at renal disease onset was 27 years. Thirty-six percent of the patients had renal involvement after diagnosis of lupus, for 30.7% of that group it was more than 5 years later. Renal involvement occurred more frequently in young male patients of non-French non-white origin. Patients with renal involvement suffered more commonly from malar rash, psychosis, myocarditis, pericarditis, lymphadenopathy, and hypertension. Anemia, low serum complement, and raised anti-dsDNA antibodies were more frequent. According to the 1982 World Health Organization classification, histologic examination of initial renal biopsy specimen in 158 patients showed normal kidney in 1.5% of cases, mesangial in 22%, focal proliferative in 22%, diffuse proliferative in 27%, membranous in 20%, chronic sclerosing glomerulonephritis in 1%, and other forms of nephritis in 6.5%. Distribution of initial glomerulonephritis patterns was similar whether renal involvement occurred before or after the diagnosis of lupus. Transformation from 1 histologic pattern to another was observed in more than half of the analyzable patients (those who underwent at least 2 renal biopsies). Nephritis evolved toward end-stage renal disease in 14 patients despite the combined use of steroids and cyclophosphamide in 12. Initial elevated serum creatinine levels, initial hypertension, non-French non-white origin, and proliferative lesions on the initial renal biopsy were indicators of poor renal outcome. Twenty-four patients died after a mean follow-up of 109 months from SLE diagnosis. Among our 436 patients, the 10-year survival rate was not significantly affected by the presence or absence of renal involvement at diagnosis (89% and 92%, respectively).

Adolescent↗
PubMed1999

Sneddon syndrome with or without antiphospholipid antibodies. A comparative study in 46 patients.

Sneddon syndrome is characterized by the association of livedo reticularis and cerebral ischemic arterial events (stroke or transient ischemic attack). Reported prevalence of antiphospholipid antibodies is highly variable. We conducted this study to compare the clinical and pathologic features of patients with Sneddon syndrome according to the presence or absence of antiphospholipid antibodies. Forty-six consecutive patients with Sneddon syndrome were analyzed. All were examined by the same dermatologist who classified the livedo of the trunk according to the regularity of the fishnet reticular pattern and according to the thickness of the fishnet reticular pattern (> or = 10 mm = large; < 10 mm = fine). Skin biopsies were systematically performed, from both the center and the violaceous netlike pattern in 38 patients. Antiphospholipid antibodies-positive Sneddon syndrome was defined by the presence of lupus anticoagulant or abnormal titers of anticardiolipin antibodies on repeated determinations. Group I consisted of 27 antiphospholipid antibodies-negative patients and Group II, of 19 antiphospholipid antibodies-positive patients. All patients except I in Group II had irregular livedo reticularis. Large livedo racemosa was more frequently observed in Group I (89%) than in Group II (21%, p < 0.001). On skin biopsy, arteriolar obstruction was detected in only 8 patients (4 in each group). The following parameters were not statistically different between the 2 groups: gender, mean age at detection of livedo, mean age at first clinical cerebral event, hypertension, Raynaud phenomenon, patients with extracerebral and extracutaneous arterial or arteriolar thrombosis or stenosis, patients with venous thrombosis, and women with 2 fetal losses or more. In contrast, seizures (11% in Group I versus 37% in Group II, p < 0.05), mitral regurgitation on echocardiogram (19% versus 53%, p = 0.02), and thrombocytopenia < 150,000/muL (0% versus 42%, p < 0.005) were more frequently observed in Group II. The number of events per year of follow-up was lower with antiplatelet therapy (0.08 versus 0.5) in Group I, but was not different with anticoagulation (0.056 versus 0.06). Antiphospholipid antibodies-negative and -positive patients with Sneddon syndrome belong to close but different subsets of Sneddon syndrome.

Adolescent↗
PubMed1999

Acute posterior multifocal placoid pigment epitheliopathy associated with Wegener's granulomatosis.

PURPOSE: To report acute posterior multifocal placoid pigment epitheliopathy (APMPPE) in the course of systemic Wegener's granulomatosis. METHODS: A complete ophthalmologic evaluation was undertaken, including fluorescein and indocyanine green angiography. RESULTS: Two patients diagnosed with Wegener's granulomatosis developed multiple white-yellow subretinal areas with smooth margins typical of APMPPE. Case 1 presented with bilateral choroidal involvement and a granulomatous anterior uveitis. In Case 2, placoid lesions were diagnosed 2 months after onset of systemic Wegener's granulomatosis. In both cases, fluorescein angiography showed an early hypofluorescence and a late staining of the lesions. Indocyanine green angiography revealed early and late hypofluorescence corresponding to the areas seen clinically. Wegener's granulomatosis was diagnosed histologically in both cases. After combined steroid and cyclophosphamide therapy, the lesions healed, and after a follow-up period of 1.5 and 4 years, respectively, funduscopic and angiographic examinations showed cicatricial lesions. CONCLUSION: Acute posterior multifocal placoid pigment epitheliopathy is a rare manifestation of Wegener's granulomatosis and should be considered a possible systemic involvement of the disease. A prompt, thorough investigation should be undertaken for an underlying systemic disorder.

Acute Disease↗
PubMed1999

The spectrum of reactive hemophagocytic syndrome in systemic lupus erythematosus.

We address the relationship between reactive hemophagocytic syndrome (RHS), systemic lupus erythematosus (SLE) activity, and treatment in 4 female patients with SLE. Febrile pancytopenia was related to cytologically proven RHS in all patients. Followup was 45+/-7 months from RHS onset. No causal infection could be identified. Outcome could be classified as: (1) RHS onset during a SLE flare and complete efficacy of high dose steroids; (2) death despite therapy for concomitant severe RHS and active SLE; (3) severe RHS in inactive SLE under immunosuppressants, with remission after steroid tapering and cyclophosphamide withdrawal. Three patients were treated with intravenous IgG. We conclude that (1) when SLE is active, RHS should be considered a specific manifestation and treated with steroids; (2) RHS occurring in otherwise inactive SLE might be related to iatrogenic immunosuppression; (3) intravenous IgG treatment might be indicated in both situations.

Acyclovir↗
PubMed1999

[Retinopathy in acute pancreatitis].

Acute pancreatitis may involve remote organ systems, including the eye. Funduscopy may reveal in these rare cases presence of a typical ischemic retinopathy, similar to that diagnosed after severe traumas, known as the Purtscher's retinopathy. Pathogenesis of retinopathy in alcoholic retinopathy remains controversial: occurrence of fat embolism or leucothrombosis is discussed in the literature. The outcome of ischemic retinopathy in acute alcoholic pancreatitis is variable. Although there is no specific available treatment, its diagnosis may allow appropriate diagnosis of the causal disease when the general signs are not present.

Acute Disease↗
PubMed1998

Acalculous ischemic gallbladder necrosis in the catastrophic antiphospholipid syndrome.

A 29-year-old woman was referred for abdominal pain. Results of tests for lupus anticoagulant and antibodies to phosphatidylserine and to beta2-glycoprotein I were positive, but the patient had no features of systemic lupus erythematosus (SLE). Abdominal ultrasonography showed a thickening of the gallbladder wall without cholelithiasis. A surgical procedure revealed necrotic areas of the gallbladder wall, and a cholecystectomy was performed. Histologic examination of the gallbladder showed multiple thrombi and no vasculitis. Despite full-dose heparin, the patient developed a catastrophic antiphospholipid syndrome (APS) and subsequently died. Among connective tissue disorders, acute acalculous cholecystitis has been reported in patients with polyarteritis nodosa and/or SLE. APS should be considered as a possible cause of acalculous cholecystitis.

Adult↗
PubMed1998

[An unknown cause of prolonged fever: apropos of 6 cases of chronic aortic dissection].

PURPOSE: Aortic arch dissection may be sometimes misdiagnosed due to the lack of mild to moderate chest pain. Definite diagnosis is often made while dissection has already occurred more than 15 days ago, being thereafter considered as chronic. Aortic dissection may then present as a prolonged febrile illness with fever and/or inflammation as main symptoms, with little or no pain. METHODS: We retrospectively reviewed cases of chronic aortic dissections seen in a department of internal medicine and a department of neurology between 1975 and 1992. RESULTS: We report six cases of patients presenting with aortic dissection and describe their outcome and treatments after the diagnosis was made based on either thoracic computerized tomography or trans-esophageal echocardiography evidence. Four patients had surgical aortic arch repair while one patient was treated with beta-blockers. CONCLUSION: Chronic aortic dissection has rarely been reported to cause fever or increased sedimentation rate. Treatment has to be discussed between medical and surgical teams involved in the therapeutical management of these unusual patients.

Aged↗