1/156. Low-dose cyclosporin A therapy in children with refractory immune thrombocytopenic purpura.Although splenectomy is the most effective treatment for chronic idiopathic thrombocytopenic purpura (ITP), many post-splenectomy patients have recurrent thrombocytopenia refractory to multiple medical therapies. Three consecutive patients with relapsed ITP after splenectomy and who were refractory to multiple medical therapies were treated with low dose cyclosporin A (CsA). In all 3 patients, the platelet count increased dramatically within 1 month from the onset of CsA therapy. The only detectable toxicity was hypomagnesemia and mild hypertension in 1 patient. CsA may be efficacious in treating patients with chronic ITP, which is refractory to all medical and surgical therapies currently being used.- - - - - - - - - - ranking = 1keywords = chronic idiopathic, idiopathic (Clic here for more details about this article) |
2/156. 47,XXX in an adolescent with premature ovarian failure and autoimmune disease.BACKGROUND: Premature ovarian failure (POF) may be idiopathic or may be associated with genetic or autoimmune disorders. The 47,XXX karyotype has been associated with POF and other genitourinary anomalies. CASE: A 17-year-old woman with a history of immune thrombocytopenic purpura was referred to the adolescent medicine clinic for evaluation of oligomenorrhea with secondary amenorrhea. Evaluation revealed hypergonadotrophic premature ovarian failure, a positive antinuclear antibody, and the 47,XXX karyotype. She has since developed a positive anti-cardiolipin antibody but does not meet diagnostic criteria for systemic lupus erythematosis. CONCLUSION: The presence of known autoimmune disease in a woman with POF should not dissuade the clinician from evaluating for a potential genetic cause.- - - - - - - - - - ranking = 0.0028533209557922keywords = idiopathic (Clic here for more details about this article) |
3/156. Isolated splenic tuberculosis.The authors report two cases of isolated splenic tuberculosis treated since 1989 in Nizam's Institute of Medical Sciences. One case presented as pyrexia of unknown origin (PUO) and another with idiopathic thrombocytopenic purpura (ITP). Both were found to have splenic tuberculosis after splenectomy. Pre operative diagnosis could not be made in these cases. splenectomy followed by antituberculous chemotherapy cured the condition in both the cases. The authors underline the diagnostic difficulties, essentially related to the rarity of this condition inspite of the progress in modern imaging.- - - - - - - - - - ranking = 0.0028533209557922keywords = idiopathic (Clic here for more details about this article) |
4/156. Thrombocytopenic purpura in infectious mononucleosis-- A rare complication?We have presented an illustrative case of thrombocytopenic purpura complicating infectious mononucleosis. Steroid therapy appeared to be beneficial although spontaneous recovery cannot be excluded. The use of the Paul-Bunnell heterophil agglutination test is recommended for patients having idiopathic thrombocytopenic purpura to rule out subclinical infectious mononucleosis. It is suggested that this syndrome be treated with the expectancy of long-term remissions. steroids appear to be of benefit. Platelet recovery is usually complete in less than 60 days. splenectomy should not be considered until at least two months have passed. Chronic thrombocytopenia is an unlikely complication.- - - - - - - - - - ranking = 0.0028533209557922keywords = idiopathic (Clic here for more details about this article) |
5/156. Renal failure after anti-D globulin treatment of idiopathic thrombocytopenic purpura.Idiopathic thrombocytopenic purpura (ITP) is a disorder of rapid destruction of antibody-coated platelets. Anti-D immune globulin has been used for treatment of ITP in the united states since 1995. Initial studies identified no significant side effects of treatment. However, a recent report highlighted occasional episodes of intravascular hemolysis after anti-D immune globulin. We describe two children with ITP who developed acute renal failure (ARF) after treatment with anti-D immune globulin and also analyze ten additional cases of ARF reported to the manufacturer, Cangene Corporation, through postmarketing surveillance. All episodes of ARF were associated with intravascular hemolysis. Four patients required dialysis. Patient age ranged from 1 to 82 years, but those requiring dialysis were all under age 15 years. Several patients with ARF had preexisting creatinine elevation. Three of the patients with ARF had serologic evidence of acute Epstein-Barr virus (EBV) infection. Renal biopsy in one patient showed acute tubular necrosis, with findings consistent with pigment nephropathy. Anti-D immune globulin, used to treat ITP, may be associated with intravascular hemolysis and resultant ARF. Renal function should be monitored in patients with evidence of intravascular hemolysis. Children and adolescents may have increased risk of ARF requiring dialysis.- - - - - - - - - - ranking = 0.011413283823169keywords = idiopathic (Clic here for more details about this article) |
6/156. Thrombocytopenic purpura and cardiomyopathy in pregnancy reversed by combined plasma exchange and infusion.thrombocytopenia and hemolytic anemia have been seen with thrombotic thrombocytopenic purpura (TTP), hellp syndrome (hemolysis, elevated liver enzymes, and low platelets), and hemolytic uremic syndrome (HUS). Differentiating between TTP, HUS, and hellp syndrome is often difficult. Coexistence of TTP and HELLP is possible. Cardiomyopathy occurring in pregnancy can be idiopathic or associated with TTP. We describe a previously healthy woman who developed thrombocytopenia and hemolysis at 34 wk gestation. The patient underwent delivery after transfusion with platelets and RBCs. The suspicion of TTP was raised, and plasma exchange was begun by the third hospital day. On the seventh day of treatment, the patient developed shortness of breath, and an echocardiogram showed global hypokinesis with an ejection fraction of 25%. Plasma infusion, one unit q 4 h, was initiated in addition to the daily plasma exchange. The patient improved and her ejection fraction normalized. plasma exchange and infusion and corticosteroids were gradually tapered off. von willebrand factor (vWF) protease activity in the plasma upon transfer was completely deficient with the presence of inhibitor. This case illustrates that vWF protease assay and detection of inhibitor can be used for the diagnosis of TTP during pregnancy; and a severe cardiomyopathy in TTP can be reversed rapidly with combined plasma exchange and infusion.- - - - - - - - - - ranking = 0.0028533209557922keywords = idiopathic (Clic here for more details about this article) |
7/156. Severe thrombocytopenic purpura due to brucellosis.A case of severe thrombocytopenic purpura as the sole manifestation of brucellosis in an 11-y-old boy is presented. Clinical examination was negative and laboratory tests revealed anemia, leukopenia and severe thrombocytopenia. The initial diagnosis was idiopathic thrombocytopenic purpura and intravenous gamma globulin was given. A prompt hematological response was observed. However, on the fifth day after admission, blood culture yielded brucella which could not be serotyped. The boy was subsequently treated with intravenous gentamicin, oral doxycycline and rifampicin and was discharged in good health. brucellosis has occasionally been associated with mild hematological abnormalities such as anemia and leukopenia. thrombocytopenia is rare and only in very rare cases it is severe enough to cause bleeding. Prompt recognition of this complication of brucellosis and aggressive therapy are essential, especially if there is a family history of brucellosis or if there is suspicion of exposure to infected food products.- - - - - - - - - - ranking = 0.0028533209557922keywords = idiopathic (Clic here for more details about this article) |
8/156. Endogenous pneumococcal endophthalmitis followed by pneumococcal-induced uveitis.BACKGROUND: We describe the case of a fulminant bilateral endophthalmitis occurring in a patient, who had 9 years earlier a splenectomy for an idiopathic thrombocytopenic purpura. history AND SIGNS: A 40-year-old woman, back from a trip to morocco, presented with high fever, rapid decrease in visual acuity and loss of consciousness. Medical examination revealed a pneumococcal meningitis and bilateral endophthalmitis. THERAPY AND OUTCOME: endophthalmitis was treated with local and intravitreal antibiotics injections (vancomycin and amikacin). Repeated parabulbar betamethasone injections were performed. Intravenous (iv) methylprednisolone pulses were followed by oral steroid therapy while systemic antibiotics were given (ceftriaxone and vancomycin). In spite of this therapy, fundus examination was impossible because the anterior chamber was filled with fibrin. A cataract developed with severe vitritis and we could observe a progressive narrowing of the anterior chamber. The patient underwent a bilateral vitrectomy and lensectomy. The retina had no lesion. No bacteria were found in the vitreous culture. Evolution was characterized by an increased ocular pressure due to anterior synechiae. visual acuity remained under 1/10. The severe ocular inflammation could be the result of a mixed mechanism including an infectious followed by a severe immune response against bacterial components. CONCLUSIONS: This case report is rare. To our knowledge, only 3 similar cases have been reported in the literature.- - - - - - - - - - ranking = 0.0028533209557922keywords = idiopathic (Clic here for more details about this article) |
9/156. Subconjunctival hemorrhage: the first presenting clinical feature of idiopathic thrombocytopenic purpura.BACKGROUND: Subconjunctival hemorrhage as the first presenting clinical feature of idiopathic thrombocytopenic purpura, to the best of our knowledge, has not been reported earlier. CASE: A 60-year-old woman presented with an isolated finding of subconjunctival hemorrhage. She later developed hemorrhage from retinal vessels and had a single episode of hematuria. OBSERVATIONS: Her blood cell count showed an extremely low platelet count. Her medical history and clinical examination for any other systemic or ophthalmic pathology were negative. Even after treating the patient with blood and platelet transfusion and maintaining her on high doses of systemic steroids, she did not respond well. CONCLUSION: The appearance of spontaneous subconjunctival hemorrhage in a patient should be thoroughly investigated as it can be an initial sign of a grave systemic disorder.- - - - - - - - - - ranking = 0.014266604778961keywords = idiopathic (Clic here for more details about this article) |
10/156. bone marrow examination before steroids in thrombocytopenic purpura or arthritis.Corticosteroids were used to treat two children with presumed idiopathic thrombocytopenic purpura and one with juvenile rheumatoid arthritis without examination of the bone marrow. Of the two with presumed idiopathic thrombocytopenic purpura, one had Fanconi's anaemia and the other may have had aplastic anaemia. The third child had acute lymphoblastic leukaemia. The diagnosis of Fanconi's anaemia was delayed. A diagnostic and therapeutic dilemma was caused in the second case. In the third, delayed diagnosis and, perhaps, compromised outlook resulted. These three cases re-emphasize the well aired caveats about the diagnosis of idiopathic thrombocytopenic purpura and juvenile rheumatoid arthritis and provide further support for the arguments of those who believe that if corticosteroids are to be used to treat such children, their bone marrow should be examined first.- - - - - - - - - - ranking = 0.0085599628673766keywords = idiopathic (Clic here for more details about this article) |
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