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Selected AbstractsAcute pseudo-obstruction of the colon (Ogilvie's syndrome) following instrumental vaginal deliveryINTERNATIONAL JOURNAL OF CLINICAL PRACTICE, Issue 10 2006A. KAKARLA Summary Acute pseudo-obstruction of the colon (Ogilvie's syndrome) is an adynamic ileus without mechanical obstruction of the bowel. Predisposing factors include: abdominal and pelvic surgery, or trauma, or severe pre-existing systemic illness. In obstetrics, many cases have been reported after caesarean delivery, but none following a vaginal delivery. Conservative and pharmacological therapies are effective in many patients, but surgical intervention may be required. Early diagnosis and appropriate treatment is imperative to avoid caecal rupture, faecal peritonitis and the associated high maternal mortality. High index of clinical suspicion and proper assessment of the gastrointestinal system in the post-surgical patient are vital to the management of this uncommon but potentially serious condition met with in obstetrics practice. [source] Primary Malignant Melanoma of the Maxillary GingivaDERMATOLOGIC SURGERY, Issue 3 2003Betül Gözel Ulusal MD BACKGROUND Mucosal malignant melanoma arising from the mucosa of the head and neck region is a rare entity, accounting for approximately 0.2% of all melanomas. Most of these lesions (80%) have occurred on the maxillary anterior gingival area, especially on the palatal and alveolar mucosa. OBJECTIVE Mucosal malignant melanomas are more aggressive than cutaneous melanomas. On the other hand, complex anatomy of this area makes complete surgical excision difficult. Thus, early diagnosis and treatment are important. METHODS We presented primary malignant melanoma of the maxillary gingiva in two cases. CONCLUSION In mucosal malignant melanoma, survival rates may be increased by early diagnosis and treatment. The clinician must carefully examine oral cavity, and pigmented lesions should be biopsied. Because some melanomas may be amelanotic, a high index of suspicion is necessary. [source] An international perspective on Tourette syndrome: selected findings from 3500 individuals in 22 countriesDEVELOPMENTAL MEDICINE & CHILD NEUROLOGY, Issue 7 2000Roger D Freeman MD We have established a multisite, international database of 3500 individuals diagnosed with Tourette syndrome (TS). The male:female ratio is 4.3:1 for the total sample, with wide variation among sites; the male excess occurs at every site. Anger control problems, sleep difficulties, coprolalia, and self-injurious behavior only reach impressive levels in individuals with comorbidity. Anger control problems are strongly correlated with comorbidity, regardless of site, region, or whether assessed by neurologists or psychiatrists. The mean age at onset of tics is 6.4 years. At all ages, about 12% of individuals with TS have no reported comorbidity. The most common reported comorbidity is attention-deficit-hyperactivity disorder. Males are more likely to have comorbid disorders than females. The earlier the age at onset, the greater the likelihood of a positive family history of tics. An understanding of the factors producing these and other variations might assist in better subtyping of TS. Because behavioral problems are associated with comorbidity, their presence should dictate a high index of suspicion of the latter, whose treatment may be at least as important as tic reduction. The established database can be used as the entry point for further research when large samples are studied and generalizability of results is important. [source] Role of Contrast Echocardiography in the Assessment of Myocardial RuptureECHOCARDIOGRAPHY, Issue 1 2003Sumit Mittle M.D. Left ventricular free wall rupture is known to complicate acute myocardial infarction and is the second most common cause of inhospital mortality in this patient population. Contrary to widely held medical belief, this does not always result in immediate fatal pericardial tamponade with hemodynamic collapse. Up to 40% of such occurrences are subacute and may evolve over hours or even days. A high index of suspicion and accurate diagnostic tests are required to identify and treat these patients with emergent surgery. Echocardiography has emerged as an important diagnostic modality to identify this catastrophic condition. Although the literature has scattered reports on the role of transesophageal and transthoracic echocardiography in diagnosing free wall rupture, to date, only one report in the literature used ultrasound contrast agents to better delineate echocardiographic findings. We will present two cases in which echocardiography with use of intravenous ultrasound contrast agents was instrumental in helping to exclude rupture in one case and help identify rupture in another. (ECHOCARDIOGRAPHY, Volume 20, January 2003) [source] Anabolic steroid users' attitudes towards physiciansADDICTION, Issue 9 2004Harrison G. Pope ABSTRACT Aims To assess anabolic-androgenic steroid (AAS) users' trust in the knowledge and advice of physicians. Design Interviews of AAS users and non-users. Setting Research offices. Participants Eighty weight-lifters (43 AAS users, 37 non-users) recruited by advertisement in Massachusetts and Florida, USA. Measurements Personal interviews and questionnaire responses, including subjects' ratings of physicians' knowledge regarding various health- and drug-related topics. AAS users also rated their level of trust in various sources of information about AAS. Findings Both groups of subjects gave physicians high ratings on knowledge about general health, cigarette smoking, alcohol, and conventional illicit drugs, but gave physicians markedly and significantly lower ratings on knowledge about AAS. When rating sources of information on AAS, users scored physicians as no more reliable than their friends, Internet sites, or the person(s) who sold them the steroids. Forty percent of users trusted information on AAS from their drug dealers at least as much as information from any physician that they had seen, and 56% had never revealed their AAS use to any physician. Conclusion AAS users show little trust in physicians' knowledge about AAS, and often do not disclose their AAS use to physicians. These attitudes compromise physicians' ability to educate or treat AAS users. Physicians can respond to these problems by learning more about AAS and by maintaining a high index of suspicion when evaluating athletic male patients. [source] Necrotizing fasciitis of the head and neck: A report of two patients and reviewHEAD & NECK: JOURNAL FOR THE SCIENCES & SPECIALTIES OF THE HEAD AND NECK, Issue 5 2002Deowall Chattar-Cora MD Abstract Background Necrotizing fasciitis is a disfiguring condition that can be fatal. The head and neck region is rarely affected. However, when involved, the functional and cosmetic sequelae can be considerable. Materials and Methods We present two case histories, discuss salient diagnostic points, treatment, and review published data on this topic. Results With a timely diagnosis we were able to diagnose and appropriately treat these patients. Conclusions Necrotizing fasciitis is a disfiguring condition that can be fatal if not diagnosed in a timely fashion. Diagnosis and treatment require a high index of suspicion, immediate operative intervention, broad-spectrum antibiotics, and appropriate supportive care. © 2002 Wiley Periodicals, Inc. [source] Hemicrania Continua-Like Headache Associated With Internal Carotid Artery Dissection May Respond to IndomethacinHEADACHE, Issue 1 2007Avi Ashkenazi MD Hemicrania continua (HC) is an idiopathic, chronic disorder characterized by a continuous, strictly unilateral headache associated with ipsilateral cranial autonomic symptoms. The symptoms of HC typically respond dramatically to indomethacin therapy. We describe a patient with traumatic internal carotid artery dissection, who presented with a clinical picture mimicking HC that initially responded to indomethacin. Patients with a clinical picture similar to HC should be managed with a high index of suspicion for a possible cervical arterial dissection. [source] Infective endocarditis in a hemodialysis patient: A dreaded complicationHEMODIALYSIS INTERNATIONAL, Issue 4 2007Claudia SCHUBERT Abstract Infection is the most common cause of death in hemodialysis patients, after cardiovascular disease. Dialysis access infections, with secondary septicemia, contribute significantly to patient mortality. The most common source is temporary catheterization. Bacteremia occurs commonly in patients receiving hemodialysis, with infective endocarditis being a relatively uncommon, but potentially lethal complication. Valvular calcification is the most significant risk factor. The diagnosis of infective endocarditis is made clinically and confirmed with the echocardiographic modified Duke's criteria. The most common pathogen is Staphylococcus aureus and the mitral valve is the most common site. Staphylococcus aureus infective endocarditis is commonly associated with embolic phenomenon. A high index of suspicion is critical in the early recognition and management of infective endocarditis. However, prevention of bacteremia is undoubtedly the best strategy with the early placement of arteriovenous fistulae. In the case of temporary catheterization, the use of topical mupirocin or polysporin and gentamicin and/or citrate locking is beneficial. Although catheter salvage has not been studied in randomized trials, catheter removal remains standard therapy during bacteremia. [source] Angioleiomyoma: a clinical, pathological and radiological reviewINTERNATIONAL JOURNAL OF CLINICAL PRACTICE, Issue 6 2004P. Ramesh Summary Angioleiomyoma is a benign tumour arising from the vascular smooth muscle (tunica media) and presents commonly between third and fifth decades of life. Although there are sporadic reports about this tumour in the literature, none describes all the information in detail. This review is an attempt to collate all the facts in one concise article. Angioleiomyoma presents as a painful mass in approximately 60% of the cases. One of the distinct clinical feature noted is the increase in size of the swelling with physical activity of the involved part, especially in the hand. It should be considered in the differential diagnosis of painful nodular lesions of the extremity. Pre-operative diagnosis is difficult, but with a high index of suspicion and awareness, it is possible. The use of ultrasound and magnetic resonance imaging should be considered. It causes minimal morbidity and excision is usually curative. Histological examination using smooth muscle Actin stain portraits the smooth muscle bundles clearly. [source] Primary vesical actinomycosis: A case report and literature reviewINTERNATIONAL JOURNAL OF UROLOGY, Issue 10 2007Sabah Al-Kadhi Abstract: Pelvic actinomycosis is a chronic granulomatous infection caused by the gram-positive anaerobic bacteria, Actinomyces Israelli. Vesical lesions, particularly without any obvious source of infection, are rare. We report a case of primary vesical actinomycosis which presented as a bladder mass, giving suspicion of malignancy. Repeated trans-urethral deep resections and histo-pathological examinations revealed the true nature of the mass, which was treated by medical means. A high index of suspicion of potentially benign bladder lesions, particularly in a younger age group of patients, is advocated, to prevent unnecessary major surgical procedures. [source] Necrotising fasciitis: a new management algorithm based on clinical classificationINTERNATIONAL WOUND JOURNAL, Issue 3 2004Paul S Carter Abstract Necrotising fasciitis is a rare infection of the subcutaneous tissues. If untreated, it is invariably fatal, and thus a high index of suspicion for the diagnosis is required. The disease's manifestation can range from a fulminant presentation to a subtle and insidious development. The priority in every case is to proceed to radical surgical debridement. On review of the literature and based on our clinical experience, we propose a new classification based on clinical presentation and suggest an algorithm to facilitate the management of this devastating condition. Increasing awareness should be given to the management of the large wounds resulting from the surgical debridement of necrotising fasciitis. [source] Thrombotic thrombocytopenic purpura: 24 years of experience at the American University of Beirut Medical CenterJOURNAL OF CLINICAL APHERESIS, Issue 3 2004Ali Shamseddine Abstract Thrombotic thrombocytopenic purpura (TTP) is a hematological syndrome defined by the presence of thrombocytopenia and microangiopathic hemolytic anemia without a clinically apparent etiology. Patients may also suffer from fever in addition to neurological and renal impairment. Treatment should be initiated as soon as possible, otherwise this rare disease can be fatal. The main treatment options include therapeutic plasma exchange, fresh frozen plasma infusion, and adjuvant agents such as steroids and antiplatelet drugs. A search of patient records was carried out at the American University of Beirut Medical Center looking for patients who developed TTP over a 24-year period extending from 1980 to 2003. Relevant information was collected and analyzed. A total of 47 records were found. All presented with anemia and thrombocytopenia, 83% had neurological symptoms, 61.7% had fever and 34% had renal impairment. All patients were treated with a multimodality regimen including therapeutic plasma exchange, FFP infusion, steroids, antiplatelet agents, vincristine and others. 38 (81%) cases achieved complete remission. Out of these, 12 (31.6%) relapsed and responded to treatment. Patients who did not receive plasma exchange were more likely to relapse (P = 0.032). A second relapse was observed in 6 cases. The overall mortality rate from TTP over 24 years was 21.3%. TTP remains a fatal disease. A high index of suspicion should, therefore, always be present. Treatment options should be further developed and patients should directly be referred to tertiary care centers. J. Clin. Apheresis 19:119,124, 2004. © 2004 Wiley-Liss, Inc. [source] Cutaneous Rosai-Dorfman DiseaseJOURNAL OF CUTANEOUS PATHOLOGY, Issue 1 2005L. Lin Rosai-Dorfman disease, or sinus histiocytosis with massive lymphadenopathy (SHML), is a rare benign disease of unknown etiology that involves the cervical lymph nodes. Histologic findings include emperiopolesis of lymphocytes and S-100 protein positivity. Extranodal involvement occurs in 30,40% of cases, often involving skin. However, cutaneous Rosai-Dorfman disease without lymph node involvement is uncommon. We describe a case of cutaneous Rosai-Dorfman disease and discuss its clinical and histopathologic findings. A 69-year-old Caucasian male presented with a two-year history of a slowly enlarging violaceous mass on the mid-upper abdomen. He had a history of renal cell carcinoma two-year ago and was asymptomatic without any systemic symptoms. Physical examination revealed a large, violaceous mass of mid-upper abdomen with several violaceous nodules surrounding the periphery. There was no palpable lymphadenopathy. Punch biopsy showed diffuse dermal infiltrates composed of central xanthomatous cells surrounded by lymphoplasmacytic cells. Focal germinal center was noted. Immunostaining revealed strong S-100 and CD68 reactivity of central xanthomatous cells and kappa and lambda polyclonal lymphoplasmacytic cells. Special stains for infectious disorder were negative. Cutaneous Rosai-Dorfman disease is a benign reactive process, often under-recognized due to lack of systemic symptoms and lymphadenopathy. A high index of diagnostic awareness is required for its recognition. [source] Esophageal perforation as a complication of esophagogastroduodenoscopyJOURNAL OF HOSPITAL MEDICINE, Issue 3 2008Nisha L. Bhatia MD Abstract Fifty years ago, esophageal perforation was common after rigid upper endoscopy. The arrival of flexible endoscopic instruments and refinement in technique have decreased its incidence; however, esophageal perforation remains an important cause of morbidity and mortality. This complication merits a high index of clinical suspicion to prevent sequelae of mediastinitis and fulminant sepsis. Although the risk of perforation with esophagogastroduodenoscopy alone is only 0.03%, this risk can increase to 17% with therapeutic interventions in the setting of underlying esophageal and systemic diseases. A wide spectrum of management options exist, ranging from conservative treatment to surgical intervention. Prompt recognition and management, within 24 hours of perforation, is critical for favorable outcomes. Journal of Hospital Medicine 2008;3:256,262. © 2008 Society of Hospital Medicine. [source] Bladder pheochromocytoma encountered on sonographyJOURNAL OF MEDICAL IMAGING AND RADIATION ONCOLOGY, Issue 3 2004M Çelikta Summary Pheochromocytomas of the bladder are rare neoplasms, constituting <0.06% of all vesical tumours. Common presenting features of this tumour include episodes of sweating, hypertension, haematuria and postmicturition syncope. We describe a case of bladder pheochromocytoma in a 66-year-old man whose only symptom of macroscopic haematuria was initially assessed with ultrasonography. Clinical presentation highlights the need for a high index of suspicion during sonographic evaluation of bladder neoplasms because such tumours might present without symptoms of adrenergic excess. [source] Acute mesenteric venous thrombosis due to protein S deficiency in a pregnant womanJOURNAL OF OBSTETRICS AND GYNAECOLOGY RESEARCH (ELECTRONIC), Issue 4 2009p Atakan Al Abstract Acute mesenteric venous thrombosis is a rare and potentially fatal disease, which often occurs in medically compromised elderly patients. Isolated mesenteric venous thrombosis may be encountered in young women who have underlying hypercoagulable disease. We report a case of mesenteric venous thrombosis in a young pregnant woman in whom protein S deficiency was diagnosed at a later stage. The patient underwent extensive bowel resection. On follow-up she had developed an obstruction on the intestinal anastomosis. The anastomosis was revised, but the patient died of intervening complications 3 months after the operation. Early management of acute mesenteric venous thrombosis relies on early diagnosis, which requires a high index of suspicion. The condition must be considered during evaluation of persistent abdominal pain in pregnant women with hypercoagulable disorder. [source] Primary adrenal insufficiency in childhood and adolescence: Advances in diagnosis and managementJOURNAL OF PAEDIATRICS AND CHILD HEALTH, Issue 11 2004PJ Simm Objectives: Primary adrenal insufficiency occurring in childhood and adolescence is due to abnormalities of gland development, gland responsiveness, and steroid biosynthesis or target organ response. Causes include autoimmune Addison's disease, tuberculosis, HIV, adrenoleukodystrophy, adrenal hypoplasia congenita and syndromes including triple A and IMAGe. We aimed to define the causes of adrenal insufficiency for a cohort of children in Melbourne. Methods: We reviewed the frequency and variety of presentation of primary adrenal insufficiency to the Royal Children's Hospital over the past 10 years through an audit of patient records, collating demographic information, presentation and investigations. Results: Sixteen cases (13 male, 3 female) of primary adrenal insufficiency were diagnosed at this hospital between January 1993 and July 2003. Median age at presentation was 7.7 years (range: birth to 14.8 years). Symptoms at presentation included weakness, increased pigmentation, abdominal pain, nausea, developmental delay or a reduction in school performance. Four patients presented with adrenal crisis. Median adrenocorticotrophic hormone (ACTH) at diagnosis was 246 pmol/L (range 30,969 pmol/L). Autoantibodies were positive in five patients. Five patients had elevation of very long chain fatty acids. Five patients were diagnosed with autoimmune adrenal insufficiency, five with adrenal hypoplasia congenita, five with adrenoleukodystrophy and one with IMAGe syndrome. Conclusions: A high index of suspicion results in earlier detection and possible prevention of adrenal crisis with a reduction in associated morbidities. Definitive diagnosis is now possible for almost all cases of primary adrenal insufficiency using technologies for screening autoimmunity, adrenoleukodystrophy (ALD) and genetic screening. [source] Calciphylaxis , a topical overviewJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 5 2006G Arseculeratne Abstract ,Calciphylaxis', a calcification syndrome associated with ischaemic cutaneous necrosis, is acquired naturally in humans in disease states. It is a life and limb-threatening complication, usually observed in patients with renal disease and secondary hyperparathyroidism, but known to occur in the absence of renal or parathyroid disease. The reported mortality rate, which ranges from 60,80%, relates to wound infection, sepsis and organ failure. It is a small-vessel vasculopathy, which is estimated to occur in about 4% of haemodialysis patients. Clinically, violaceous, reticulate areas of cutaneous necrosis and eschar may be evident, particularly in the extremities. In addition to the clinical picture, a raised calcium phosphorous product, an elevated parathyroid hormone level, radiographic evidence of vessel and soft-tissue calcification and the finding of mural calcification affecting small arteries and arterioles on histopathology help to confirm the diagnosis of this entity which generally has a poor prognosis. A high index of suspicion and an active multidisciplinary management approach, with rigorous attention to wound care and prevention of sepsis, are vital in the management of these patients. In this overview, we discuss the pathophysiology, clinical features and associations, risk factors, diagnosis and management issues relating to calciphylaxis. [source] Cutaneous T-cell lymphomaJOURNAL OF THE EUROPEAN ACADEMY OF DERMATOLOGY & VENEREOLOGY, Issue 2 2003EA Kotz ABSTRACT Cutaneous T-cell lymphoma (CTCL) is a neoplasm of helper T cells whose first manifestations usually appear in the skin. The various forms of CTCL are distinguished by both clinical features and histopathology. Early on, the diagnosis may be difficult to establish because of its numerous, and often non-specific, clinical presentations. Further, the pathological findings of early lesions may lack the diagnostic features observed in well-developed or advanced disease. The diagnosis of CTCL must be considered in any patient with a chronic, therapy-resistant condition of the skin. In patients with non-specific histological findings, a high index of suspicion and multiple biopsies may eventually lead to a diagnosis of CTCL. Once the diagnosis of CTCL is established, accurate staging is essential both for its effect on treatment decisions and for its prognostic value. In general, CTCL is a chronic, slowly progressive disease with a long evolution. The development of tumours is a poor prognostic sign, as is erythroderma. The Sezary syndrome is a distinct form of erythrodermic CTCL that is characterized by exfoliative erythroderma, lymphadenopathy, lymphocytosis, intense pruritus, and circulating large, abnormal lymphocytes (Sezary cells). When death does occur, it is most often due to septicemia. Treatment of CTCL must be tailored to the individual patient. The most commonly employed treatment options are photochemotherapy and topical chemotherapy. [source] Imported Malaria in Children: A Comparative Study Between Recent Immigrants and Immigrant Travelers (VFRs)JOURNAL OF TRAVEL MEDICINE, Issue 4 2010Juan Arnáez MD Background. In Europe, imported malarial cases occur in returning travelers and immigrants mostly from African countries. There have been an increasing number of cases in the past years in Spain. Methods. An analysis of all cases of malaria who attended at the Hospital of Mostoles in the Southwest of Madrid from 1995 to 2007 was performed. Clinical, epidemiological, laboratory, and parasitological findings were analyzed and compared between immigrants coming from endemic countries (recent immigrants) and children who traveled to endemic areas to visit friends and relatives (VFRs). Results. Sixty cases of imported malaria were detected. Most of the cases (59 of 60) were acquired in sub-Saharan Africa. The most common species was Plasmodium falciparum (43 of 60). Microscopic examination was positive in 95%, and the polymerase chain reaction (PCR) for Plasmodium achieved additional diagnosis in seven cases. Fourteen cases were VFRs; none of them used appropriate malaria chemoprophylaxis. Fever and thrombocytopenia were significantly more common among VFRs. They also had significantly higher parasite density. Twelve cases were asymptomatic at the time of diagnosis; all of them were recent immigrants. Conclusions. VFRs account for a significant number of childhood malarial cases. These patients had not taken malaria chemoprophylaxis and malarial cases were more severe. VFR children are a high-risk group, and pretravel advice should underline the risk for malaria. Recent immigrants can be asymptomatic and parasitemias are lower. Therefore, a high index of suspicion is necessary, and PCR for Plasmodium should be performed in case of negative thick smears. [source] Successful use of short-term mechanical ventilation to manage respiratory failure secondary to profound hypokalemia in a cat with hyperaldosteronismJOURNAL OF VETERINARY EMERGENCY AND CRITICAL CARE, Issue 5 2008Tara N. Hammond DVM Abstract Objective , To report successful management of respiratory failure due to severe hypokalemia in a cat with hyperaldosteronism, including short-term mechanical ventilation strategies and aspects of medical and surgical treatment. Case Summary , A cat presented with bilateral pelvic limb weakness that rapidly progressed to tetraparesis and respiratory muscle failure. Point-of-care testing revealed severe hypokalemia (1.9 mmol/L) and mild azotemia. Initial management included endotracheal intubation, mechanical ventilation, and aggressive potassium supplementation. Spironolactone was started due to a high index of suspicion for hyperaldosteronism. A right adrenal mass visualized during abdominal ultrasonographic examination and a serum aldosterone level greater than 3329 pmol/L confirmed the diagnosis. The cat made a full recovery following surgical removal of a right adrenal adenoma. New or Unique Information Provided , We report successful management of respiratory failure in a cat with hyperaldosteronism using short-term mechanical ventilation. Respiratory failure due to severe hypokalemia should be considered a complication of hyperaldosteronism in cats and may require mechanical ventilation. However, full recovery is possible. [source] Valproate-induced Parkinsonism in epilepsy patientsMOVEMENT DISORDERS, Issue 1 2007Dominic Jamora MD Abstract We systematically examined 226 epilepsy patients in a tertiary-referral center and found 6 (5.04%) to have valproate-induced Parkinsonism. There was a significantly higher prevalence of patients with Parkinsonism in the group of patients treated with valproate compared to those who were on other antiepileptic drugs (6 [5.04%] of 119 vs. 0 [0%] of 107; ,2 = 5.54; P = 0.025). These six patients had been on valproate for more than 3 years (mean, 75.67 ± 25.32 months) at an average dose of 750 ± 273.86 mg/day. The valproate doses were decreased or discontinued with supplementation from another antiepileptic medication. The mean UPDRS motor score significantly improved from 10.67 ± 5.1 to 4.75 ± 2.75 (P < 0.05). There was no relapse of seizures. Clinicians working in tertiary-referral centers should have a high index of suspicion for valproate-induced Parkinsonism. Early recognition and switching into another antiepileptic medication may help reduce unnecessary suffering in these patients. © 2006 Movement Disorder Society [source] Laryngeal histoplasmosis in an immunocompetent patient from a non-endemic region: case reportMYCOSES, Issue 6 2009Teresa C. A. Ferrari Histoplasma capsulatum infection involving the larynx is a rare manifestation, especially in immunocompetent individuals and a high index of suspicion is needed to establish the diagnosis correctly. We report a case of a 50-year-old Brazilian man who presented with progressive hoarseness and throat pain for 4 months. Laryngoscopy showed a supraglottic vegetant lesion, and the biopsies chronic granulomatous inflammation without any specific agent. A second laryngoscopy with biopsies was performed and after 17 days of incubation in specific medium, H. capsulatum was isolated. The patient was successfully treated with amphotericin B. [source] Mucormycotic pseudoaneurysm of the common carotid artery with tracheal involvementMYCOSES, Issue 4 2008S. Hashemzadeh Summary Mucormycosis is an emerging and fatal fungal infection. A high index of suspicion and the knowledge of its potential manifestations are essential for early diagnosis. We describe a patient with acute lymphoblastic leukaemia (L2 subtype) who developed a neck mass following a course of induction chemotherapy. Doppler ultrasonography and angiography of the neck revealed a pseudoaneurysm of the right common carotid artery. The patient then developed haemoptysis. Surgical exploration revealed a necrotic right common carotid artery with anteromedial pseudoaneurysm and adjacent tracheal wall perforation. Local debridement and tracheal repair were performed. Nonseptate hypheal invasion (mucormycosis) was found on the microscopic examination of the excised arterial wall. A subsequent recurrence of pseudoaneurysm was treated with local surgical debridement and intravenous amphotericin B (Fungizone) administration. Although rare, clinicians should be aware of these possible presenting features of mucormycosis as early diagnosis and treatment may potentially improve the survival. [source] Role and prognostic significance of the Ki-67 index in non-Hodgkin's lymphoma,AMERICAN JOURNAL OF HEMATOLOGY, Issue 6 2009Adi Broyde Expression of Ki-67, a nuclear antigen protein present in all cycling cells, is used to determine the growth fraction of tumors. The aim of this study was to evaluate the role and prognostic significance of the Ki-67 proliferation index (PI) in non-Hodgkin's lymphoma. Ki-67 was assayed immunohistochemically in tissue samples of 319 patients with newly-diagnosed non-Hodgkin's lymphoma. In 268 patients, the Ki-67 PI was correlated with clinical course and outcome. The mean Ki-67 PI ranged from 26.6% in indolent lymphomas to 97.6% in very aggressive lymphomas (P < 0.001). The index was <45% in 82.8% of indolent lymphomas and >45% in 85% of aggressive lymphomas (AUC = 0.877, P < 0.001). In patients with diffuse large B-cell lymphoma (n = 141), a Ki-67 PI of 70% was found to significantly discriminate patients with good or bad prognosis (AUC = 0.65, P = 0.004). Three-year survival was 75% ± 5.6% in patients with a low Ki-67 index compared with 55.9% ± 6% in patients with a high index (P = 0.015). In patients with a low IPI (,2), 3-year survival was 94% ± 4% in those with a Ki-67 index ,70% and 64% ± 8.1% in those with a higher index (P = 0.002); in patients with bulky disease (>10 cm), the corresponding 3-year survival by Ki-67 index was 100% and 25% ± 12% (P = 0.012). Our results suggest that the mean Ki-67 PI differs by type of lymphoma. A cut-off value of 45% can help differentiate indolent from aggressive disease. In diffuse large B-cell lymphoma, a cut-off value of 70% can distinguish patients with a good and bad prognosis when combined with other prognostic factors of low IPI score and bulky disease. Am. J. Hematol. 2009. © 2009 Wiley-Liss, Inc. [source] Native BK viral nephropathy in a pediatric heart transplant recipientPEDIATRIC TRANSPLANTATION, Issue 4 2010Farah N. Ali Ali FN, Meehan SM, Pahl E, Cohn RA. Native BK viral nephropathy in a pediatric heart transplant recipient. Pediatr Transplantation 2010: 14:E38,E41. © 2009 Wiley Periodicals, Inc. Abstract:, BK viral nephropathy is a well-documented clinical entity in kidney transplant recipients and a significant cause of morbidity and allograft loss in affected patients. BK viral nephropathy in native kidneys of non-kidney transplant recipients is relatively uncommon, but has been reported in adult patients. We report the occurrence of BK viral nephropathy in a pediatric heart transplant recipient. A 10-yr-old boy with past history of Ewing's sarcoma underwent heart transplantation for dilated cardiomyopathy induced by previous chemotherapy with doxorubicin. Post-transplant course was complicated by grade 3A rejection and CMV colitis. He was diagnosed with native BK viral nephropathy approximately 18 months post-transplant due to mild, but persistent, elevation in serum creatinine associated with proteinuria. BK viral nephropathy affects non-kidney transplant recipients, and a high index of suspicion is necessary for early diagnosis and management of this condition. [source] Post-transplant lymphoproliferative disorder after pediatric liver transplantation: Characteristics and outcomePEDIATRIC TRANSPLANTATION, Issue 3 2009María C. Fernández Abstract:,Purpose:, Post-Transplant Lymphoproliferative Disorder (PTLD) is a life threatening complication in organ transplant recipients. Risk factors include primary Epstein-Barr virus infection, intensity of immunosupression and cytomegalovirus infection. Objectives:, To evaluate the incidence, clinical presentation, risk factors, histopathologic appearance and outcome of pediatric liver recipients with PTLD at our institution. Method:, Retrospective, descriptive and observational analysis. Between November 1992 and December 2005, 383 liver transplants were performed. The diagnosis of PTLD was based on clinical history and physical examination and confirmed by histologic appearance and immunohistologic staining. Knowles' classification was used for histopathologic diagnosis. Results:, The incidence of PTLD was 5.7% (n: 22p). The average onset after tansplantation (OLT) was 24.9 months. Clinical manifestations were malaise, anorexia, fever of more than 3 days, peripheral adenopathy, tonsillar hypertrophy, abdominal mass, hepatosplenomegaly, snoring, interstitial pulmonary infiltrate, G.T.-tract bleeding, rash, submaxilar mass. Histopathologic diagnosis were Plasmocytic Hyperplasia (n: 10), Polymorphic Lymphoproliferative Disorder (n: 8), Non-Hodgkin Lymphoma (n: 4). Mortality was 18%. Conclusion:, The clinical presentations were protean and not specific. A high index of suspicion is important for early diagnosis as it correlates with more benign lesions and more favorable outcume. The lower mortality rate in our series is concordant with that reported in more recent articles. [source] Neurological presentations of conversion disorders in a group of Singapore childrenPEDIATRICS INTERNATIONAL, Issue 4 2008Wan-yee Teo Abstract Background: Neurological presentations of conversion disorders in children are not uncommon. Conversion disorders mimicking neurological conditions constitute a group of underdiagnosed conditions. Methods: This was a retrospective study of 13 children with neurological presentations of conversion disorders who were admitted to hospital. Patients were followed for 1,4 years. Results: Paralysis was the most common neurological symptom, patients presented with multiple, complex conversion symptoms and other neurological symptoms such as seizures and headache. The affected children underwent complete physical, neurological examination and psychological evaluation. Investigations included blood tests, cranial imaging and electroencephalography. Most common external environmental factors detected were school stress and change in family situation. Five of 13 patients had family members who were reported to have medical conditions with presentations similar to patients' neurological and psychological problem. All the patients were admitted, five patients required multiple admissions. Ten patients eventually had good outcome in terms of academic grades and social functioning. Conclusion: Diagnosis of conversion disorders mimicking neurological conditions can be challenging. There is a need to heighten awareness of this entity for early recognition and diagnosis. Awareness of this entity coupled with a high index of suspicion can facilitate accurate and earlier diagnosis. [source] A Randomized, Prospective Trial of Rituximab for Acute Rejection in Pediatric Renal TransplantationAMERICAN JOURNAL OF TRANSPLANTATION, Issue 12 2008V. Zarkhin We report 1-year outcomes of a randomized study of Rituximab versus standard-of-care immunosuppression (Thymoglobulin and/or pulse steroids) for treatment of biopsy confirmed, acute transplant rejection with B-cell infiltrates, in 20 consecutive recipients (2,23 years). Graft biopsies, with Banff and CADI scores, CD20 and C4d stains, were performed at rejection and 1 and 6 months later. Peripheral blood CMV, EBV and BK viral loads, graft function, DSA, immunoglobulins, serum humanized antichimeric antibody (HACA) and Rituximab, and lymphocyte counts were monitored until 1 year posttreatment. Rituximab infusions were given with a high index of safety without HACA development and increased infections complications. Rituximab therapy resulted in complete tissue B-cell depletion and rapid peripheral B-cell depletion. Peripheral CD19 cells recovered at a mean time of ,12 months. There were some benefits for the recovery of graft function (p = 0.026) and improvement of biopsy rejection scores at both the 1- (p = 0.0003) and 6-month (p < 0.0001) follow-up biopsies. Reappearance of C4d deposition was not seen on follow-up biopsies after Rituximab therapy, but was seen in 30% of control patients. There was no change in DSA in either group, independent of rejection resolution. This study reports safety and suggests further investigation of Rituximab as an adjunctive treatment for B-cell-mediated graft rejection. [source] Primary Cutaneous Fungal Infections in Solid Organ Transplantation: A Case SeriesAMERICAN JOURNAL OF TRANSPLANTATION, Issue 7 2002Peter S. Miele Cutaneous fungal infections in solid-organ transplant patients present in a variety of nonspecific ways, requiring a high index of suspicion to diagnose correctly. In the present series of four transplant recipients, subsequent primary cutaneous fungal infections presented as papules, plaques, ulcers and subcutaneous nodules. Transplantations included one cardiac, two renal and one renal,pancreatic transplant. Fungal infections were limited to the skin; there was no evidence of disseminated disease in any case. The pathogens isolated were Scedosporium apiospermum (Pseudallescheria boydii), Alternaria species, Aspergillus fumigatus, and a coelomycete in the Coniothyrium-Microsphaeropsis complex of dark molds. Individuals were successfully treated with surgical debridement, antifungal agents, and reduction of immunosuppressive therapy. All patients and allografts survived. Accurate diagnosis, aggressive surgery and appropriate antifungal therapy, combined with close outpatient follow-up, optimize the likelihood of a cure in a transplant population. [source] |