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Yilmaz Sebnem - - 2006
Idiopathic thrombocytopenic purpura usually presents with minor bleeding such as petechia and purpura. Rarely, life-threatening events as intracranial and intra-abdominal bleedings can be seen. We would like to present a rare case diagnosed as idiopathic thrombocytopenic purpura, presenting with abdominal pain and paleness. In this 17-year-old female patient, extensive abdominal ...
Lazarczyk Maciej - - 2006
It has previously been reported that pemphigoid coexists with psoriasis more frequently than it could be predicted on the basis of random distribution in the general population. In this study we present three psoriatic patients who developed tense blisters, which most likely were not provoked by anti-psoriatic treatment. Diagnosis of ...
Anzai Yuki - - 2006
Generally, prognosis of hypertensive encephalopathy in childhood is favorable. We reported a 5-year-old girl who presented with a headache attack and EEG abnormalities after hypertensive encephalopathy due to purpura nephritis. The patient had suffered from hypertensive encephalopathy due to purpura nephritis, which soon ameliorated. Five months later, she developed attacks ...
Arnold Paul O - - 2006
Thrombotic thrombocytopenic purpura (TTP), a well-recognized syndrome with typical diagnostic features, has been implicated in rare causes of sudden death. Involvement of TTP on the myocardium and cardiac conduction system has been characterized, but the consequences related to these effects are infrequently reported. The outcome is almost always death, thus ...
Cheung Winson Y - - 2006
BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is a life-threatening syndrome characterized by platelet aggregation leading to occlusive microangiopathy. TTP has been rarely reported to simultaneously present with systemic lupus erythematosus (SLE). CASE: A 61-year-old male presenting with TTP was also concurrently diagnosed with SLE. The patient recovered only after combined treatments ...
Alajlan Abdulmajeed - - 2006
BACKGROUND: Linear IgA bullous dermatosis of childhood is a rare autoimmune bullous disease that mainly affects preschool-aged children. Dapsone is considered the first-line therapy with prompt response from most patients. However, it may be contraindicated in certain conditions such as glucose-6-phosphate dehydrogenase deficiency. OBJECTIVE: We sought to assess the efficacy ...
Rekhtman N - - 2006
We describe a case of fatal systemic amyloidosis presenting with mucocutaneous bullous lesions in a patient with IgA kappa monoclonal gammopathy. The amyloid plaques were composed of an unusual mixture of immunoglobulin kappa light chain and amyloid A proteins. Whereas oesophageal and oropharyngeal blisters are known to occur in several ...
Iosifidis Michael I - - 2006
Thrombotic thrombocytopenic purpura (TTP) or Moschowitz's syndrome is characterized by release of unusually large von Willebrand factor (ULvWF) multimers and a deficiency of vWF metalloprotease. It is a very rare condition, but it causes serious problems. The etiology is still unknown, although surgical stress has been associated with TTP, probably ...
Do─ču Figen - - 2006
Wiskott-Aldrich syndrome (WAS) is an X-linked disorder characterized by thrombocytopenia, eczema and immunodeficiency. Clinical features of the disease are highly varied; therefore, the diagnosis is sometimes difficult, especially in solitary cases or cases with milder forms of the disease. However, the identification of the WASP gene has made possible a ...
Lourenço Silvia V - - 2006
Mucous membrane pemphigoid is a chronic inflammatory, bullous subepithelial auto-immune disease, with predominant involvement of the mucosal surfaces. Oral mucous membrane pemphigoid occurring in childhood is extremely rare. We describe a new case of this entity occurring in a 4-year-old girl who presented with desquamative gingivitis. Diagnosis was based on ...
Balighi Kamran - - 2006
Several diseases may be confused with scabies. Atypical manifestations of scabies have previously been reported, including urticaria, contact dermatitis, and dermatitis herpetiformis. There are also reports of scabies mimicking bullous pemphigoid. Here we report a case of scabies in a patient with manifestation similar to bullous pemphigoid. Direct smear of ...
Gast Thomas - - 2006
Purpura fulminans is a rare complication of a coagulopathy or an infection. Haemophilus influenzae infection, which has decreased since the haemophilus influenzae type B vaccine was initiated, is an unusual initiating cause of purpura fulminans. This case is the first reported in the literature of an adult who developed purpura ...
Chen Yu-Chieh - - 2005
Thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS) are rare and closely related disorders characterized by microangiopathic hemolytic anemia, thrombocytopenia, and renal failure. Many risk factors have been reported including infection, cancer, pregnancy, a variety of drugs (e.g. anticancer drugs), and autoimmune diseases. The incidence of TTP-HUS is higher ...
Kalyoncu Mukaddes - - 2006
We report on a case of Henoch-Schönlein purpura (HSP) with pulmonary hemorrhage and severe renal involvement. The patient also had active carditis related to acute rheumatic fever. He died despite intensive treatment. Regarding this case, we discuss the pathogenesis and clinical findings of pulmonary hemorrhage and active carditis in HSP.
Gach J E - - 2005
Pemphigoid nodularis is a chronic and uncommon variant of bullous pemphigoid. The condition is characterized by the presence of prurigo nodularis-like lesions, possible history of blistering, and immunohistochemical findings of bullous pemphigoid. These patients are often unresponsive to conventional therapy with potent topical and systemic steroids and different immunosuppressive agents ...
Søreide Kjetil - - 2005
BACKGROUND/PURPOSE: The aim of this study was to present the current experience and evidence relating to surgical evaluation and management of nonrenal genitourinary presentation in children with Henoch-Schönlein purpura (HSP). Henoch-Schönlein purpura is the most common systemic vasculitis in childhood. Presenting symptoms include a rash, arthralgia, abdominal pain, and, frequently, ...
Herman C - - 2005
We present a case of a 53-year-old black male who developed a factor VIII inhibitor during his treatment for hepatitis C virus infection with peginterferon. The development of inhibitors against factor VIII during peginterferon therapy has been rarely reported. We have reviewed the current literature and the following case presentation ...
Cogar Bryan D - - 2005
Henoch-Schonlein purpura (HSP) is the most common acute vasculitis in the pediatric population, with an incidence of 10-14 per 100,000. The classic presentation of this disorder includes erythematous papules followed by palpable purpura in the lower extremities, trunk, and face, arthralgia or arthritis, abdominal pain, gastrointestinal bleeding, and nephritis. While ...
Puapong Devin - - 2005
Although relatively rare, intracranial hemorrhage remains the most common cause of immune thrombocytopenic purpura-related mortality [Medeiros D. Current controversies in the management of idiopathic thrombocytopenic purpura during childhood. Pediatr Clin North Am . 1996;43:757-72]. The required decompressive treatment has the potential for substantial blood loss and must often be delayed ...
Mayer Sebastian A - - 2005
Several disease states manifest as thrombotic microangiopathies (TMA), most prominently thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome (HUS). The recent discovery of the von Willebrand factor cleaving protease ADAMTS-13 (a disintegrin and metalloprotease with thrombospondin type 1 motif), found to be deficient in TTP, has helped separate these entities. ...
Ploy Marie-Cécile - - 2005
We reported the case of a child who died of purpura fulminans. The diagnosis of Neisseria meningitidis serogroup C could be assessed using postmortem specimens collected up to 10 h after death. We were able to identify the bacteria by culture and/or PCR on samples without having autopsy performed. Soluble ...
Al Aly Ziyad - - 2005
Thrombotic thrombocytopenic purpura (TTP), characterized by thrombocytopenia and microangiopathic hemolytic anemia, is a relatively rare disorder. The majority of cases have no defined causes. TTP has been reported in association with many drugs, but not with imatinib mesylate. We report a 22-year-old African-American woman who developed idiopathic hypereosinophilic syndrome. She ...
Barnes C - - 2005
Intracranial haemorrhage (ICH) is a dramatic and potentially life-threatening presentation of children with thrombocytopenia. Management is limited to supportive care. Recent evidence suggests that ongoing bleeding following the initial ICH may result in greater neurological morbidity and mortality. Haemostatic agents, including recombinant factor VIIa (rFVIIa), a product licensed for use ...
Daram Sumanth R - - 2005
A hallmark of the clinical syndrome of thrombotic thrombocytopenic purpura (TTP) is evidence of microangiopathic hemolytic anemia. The presence of schistocytes on the peripheral blood smear, elevated plasma lactic dehydrogenase, and decreased haptoglobin concentration are used as evidence of microangiopathic hemolytic anemia to make a diagnosis of TTP. This report ...
Erbagci Zulal - - 2005
Progressive pigmentary purpura is a rare condition characterized by lymphocytic capillaritis histologically causing various clinical entities which are also named as persistent pigmented purpuric dermatoses. It is generally idiopathic; however, rare cases secondary to drugs and various diseases have been reported. In this report we describe a case of progressive ...
Binstadt Bryce A - - 2005
Henoch-Schönlein purpura (HSP) is a common childhood vasculitis. Abdominal pain is a common feature of HSP, often leading to surgical consultation for evaluation of possible intussusception. Appendicitis is a rare complication of HSP, and in each of the 3 reported cases, appendectomy preceded the appearance of the purpuric rash. More ...
Manadan Augustine M - - 2005
OBJECTIVES: To describe the association between thrombotic thrombocytopenic purpura (TTP) and systemic sclerosis (SSc) and the methods to distinguish TTP from scleroderma renal crisis (SRC). METHODS: A case of TTP that developed in a patient with preexisting SSc is described. Medline/PubMed was searched for literature pertaining to an association between ...
Franchini Massimo - - 2005
Thrombotic events in patients with inherited bleeding disorders occur only rarely. However, in some cases, the co-existence of acquired or inherited prothrombotic risk factors may overcome the hypocoagulative state, modulating the clinical phenotype to a decrease in bleeding symptoms or even to an increase in the likelihood of developing thrombotic ...
Durso Lisa M - - 2005
We report an agricultural fair-associated shiga-toxigenic Escherichia coli O157:H7 (STEC O157) outbreak that was unusual in that it affected both livestock exhibitors and visitors. Twenty-five human cases of STEC O157 infection were detected after the Fort Bend County Fair in Rosenberg, Texas, which ran from 9/26/03 to 10/04/03. Seven cases ...
Guvenc Birol - - 2004
BACKGROUND: Thrombotic thrombocytopenic purpura (TTP) is a multisystem disorder characterised by platelet aggregation causing microvascular occlusion. Early diagnosis and utilization of plasmapheresis can provide an improvement in prognosis. CASE REPORT: A 17 year old male with classical findings of TTP was later diagnosed as systemic lupus erythematosus (SLE). Plasmapheresis resulted ...
Gulati Taruna - - 2004
Henoch Schonlein Purpura with acute rheumatic carditis is a rare entity and only few cases have been reported. An 8 year-old-girl presented with abdominal pain, arthralgia and rashes and was diagnosed as a case of Henoch Schonlein Purpura. She was managed conservatively and discharged. She was readmitted after 1 week ...
Cakir N - - 2004
We present two brothers who came to us with similar complaints within a two-month interval and who were diagnosed as having Henoch-Schönlein purpura. Interestingly, the two brothers were prisoners in the same jail. In addition, we shall review the small number of familial Henoch-Schönlein purpura cases that have been reported ...
Almehmi Ammar - - 2004
Thrombotic thrombocytopenic purpura, a rare clinical syndrome characterized by thrombocytopenia and microangiopathic hemolytic anemia, was almost uniformly fatal until the introduction of plasma exchange in 1970. The thrombotic thrombocytopenic purpura following cardiovascular surgeries is relatively a new entity with high mortality if untreated. Plasma exchange is the most important therapy ...
Yomtovian Roslyn R Department of Pathology, The University Hospitals of Cleveland, Case Western Reserve University School of Medicine, Cleveland, OH, - - 2004
Deficiency of von Willebrand factor (VWF) cleaving protease ADAMTS13 has been demonstrated to be the proximate cause of a subset of thrombotic microangiopathic haemolytic anaemias (MAHA) typical for thrombotic thrombocytopenic purpura (TTP). ADAMTS13 gene mutations cause the hereditary form; acquired deficiency has been attributed to presence of an autoantibody, which ...
Inagaki Takuji - - 2004
Breath-holding spells (BHS) are commonly seen in childhood. However, there are no case reports of BHS occurring in adolescents or young adults. We report two young adult cases and discuss the pathogensis, both physically and psychologically. BHS occurred for 1-2 minutes after hyperventilation accompanied by cyanosis in both cases. Oxygen ...
André-Kerneïs Elisabeth - - 2003
We report the case of a young healthy woman who presented an early overanticoagulation when receiving acenocoumarol for a first thromboembolic episode. The patient had none of the risk factors known to influence the response to the coumarinic derivative except that she carried the rare *3 allelic variant of the ...
Radimer Matthew C - - 2003
A case report is presented of a naval aviator who suffered a transient ischemic attack (TIA) 1 mo after experiencing right leg pain and swelling following a 4-d drive. Although a venous thrombotic source was never discovered, the presence of both a patent foramen ovale and a thrombophilic condition supports ...
Chak W K - - 2003
Thrombotic thrombocytopenic purpura is a rare but serious condition in childhood. It can be idiopathic or a complication of other diseases or drug therapy. We report on a 12-year-old Chinese girl who presented with fulminant systemic lupus erythematosus with progressive renal failure, pancytopenia, and cerebral dysfunction due to thrombotic thrombocytopenic ...
Baron Beverly W - - 2003
BACKGROUND: Acquired thrombotic thrombocytopenic purpura (TTP) is an autoimmune disorder. The pathogenesis is believed to be mediated by an autoantibody directed against the metalloproteinase responsible for the degradation of the very-high-molecular-weight multimers of the vWF. The syndrome can be precipitated by a variety of conditions, and certain medications also have ...
Winkler A - - 2003
We present the case of a woman (age: 64 years) with acute thrombotic microangiopathy due to severe acquired ADAMTS-13 (von Willebrand factor-cleaving protease) deficiency. She was successfully treated with plasma exchange therapy and glucocorticosteroids. She relapsed seven months later, and splenectomy led to lasting remission. Pathomechanisms of thrombotic thrombocytopenic purpura, ...
D'Addario Stephen F - - 2003
Minocycline hydrochloride, a synthetic tetracycline, is a systemic antibiotic that has received much attention over the past several years. Currently, minocycline is considered the most widely prescribed oral antibiotic in the management of acne. Minocycline has been associated with autoimmune events, hepatitis, lupus-like syndromes, serum sickness, vasculitis, Sweet's syndrome, and ...
Fisler Rita E - - 2003
Bullous pemphigoid (BP) is an acquired bullous disorder that predominantly affects the elderly. It is rare in children but when it occurs, there is considerable clinical and histologic overlap with other acquired or congenital blistering disorders. A definitive diagnosis of childhood BP requires direct immunofluorescence and, in some cases, characterization ...
Majhail Navneet S - - 2003
Although the antiplatelet agent clopidogrel is associated with an increased incidence of thrombotic thrombocytopenic purpura (TTP), available evidence is not sufficient to establish or disprove a causal relationship. We review and assess the evidence and case reports linking clopidogrel use with TTP and discuss how to approach the risk of ...
Bais A S - - 2003
Osteopetrosis is a rare hereditary metabolic disease associated with a myriad of otolaryngological presentations. Three cases with varying otolaryngological presentations are described here. A brief review of the radiological changes of interest to the otorhinolaryngologist is being made.
Huang Dan-Ching - - 2003
Henoch-Schönlein purpura is one of the most common types of systemic vasculitis in children. Although recurrence is frequent, most cases are benign and self-limited. Standard treatment consists of supportive care and nonsteroid antiinflammatory drugs and/or steroid. Here we report 2 cases of Henoch-Schönlein purpura, both of which had a prolonged ...
Nguyen Roland T D - - 2003
Two cases of scurvy diagnosed following presentation with a purpuric rash are presented. A 44-year-old man developed scurvy as a result of poor dietary intake of vitamin C. This occurred because of a number of factors. including poor dentition, diarrhoea, depression and benzodiazepine/narcotic dependence. A 69-year-old man with acute myeloid ...
Mele Luca - - 2003
Bupropion hydrochloride is an effective drug for people who want stop smoking, and its use has recently increased in many countries. The main side effects of this drug are related to its dopaminergic activity and are dose dependent. To date, no cases of thrombotic thrombocytopenic purpura-hemolytic uremic syndrome (TTP-HUS) have ...
Yen Tzung-Hai - - 2003
This article reports the unexpected discovery of IgA nephropathy in a 26-year-old Chinese woman 1.5 years after the onset of idiopathic dermatomyositis. The patient was taking immunosuppressive agents, prednisolone 25 mg and azathioprine 75 mg daily. Glomerulonephritis associated with idiopathic polymyositis/dermatomyositis is rare. A review of the medical literature indicates ...
Choi Soo Jeong - - 2002
Henoch-Schönlein purpura is an acute, self-limited vasculitis syndrome which shows characteristic skin, joint, renal and gastrointestinal manifestations. It is common in childhood and may also occur in adults with fatal complications such as nephritis and gastrointestinal bleeding. We experienced a case of a 20-year-old woman who presented with palpable purpura ...
Agarwal V - - 2002
Thrombotic thrombocytopenic purpura (TTP) is a rare disease. A high index of suspicion is required for early diagnosis and treatment. It can present in acute as well as chronic forms. Cyclophosphamide has been reported to be beneficial in relapsing TTP. Herein a case of acute TTP evolving into relapsing phase ...
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