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Rare Occurrence (rare + occurrence)
Selected AbstractsRare occurrence of CD30+ circulating cells in patients with cutaneous CD30+ anaplastic large cell lymphoma: a study of nine patientsBRITISH JOURNAL OF DERMATOLOGY, Issue 2 2003O. Dereure SummaryBackground The presence of a significant percentage of circulating atypical lymphocytes in peripheral blood has already been demonstrated in systemic CD30+ anaplastic large cell lymphoma (ALCL), which implies that a leukaemic component may be present in this subset of lymphomas. However, no similar data are available for the cutaneous counterpart of this particular lymphoproliferation. Objectives To assess the presence of atypical cells, CD30+ lymphocytes and of a dominant T-cell clone in peripheral blood in a series of patients with cutaneous CD30+ ALCL. Materials and methods Nine patients with either primary (four) or secondary (five) cutaneous CD4+ CD30+ ALCL were selected. The percentage of CD30+ CD4+ lymphocytes among peripheral blood mononuclear cells (PBMC) was determined by flow cytometry and the presence of a dominant circulating T-cell clone was assessed by polymerase chain reaction targeting the T-cell receptor , chain. A control group composed of apparently healthy individuals was similarly studied at the same time. Results The mean percentage of CD30+ cells in PBMC was slightly higher in patients than in controls (3·9% vs. 2·7%) but the difference was not statistically significant. Only two patients displayed more than 5% CD30+ cells, both of whom had a minor tumour burden. A dominant circulating T-cell clone was detected in only three cases, including these two latter patients. Conclusions The occurrence of a significant percentage of CD30+ CD4+ circulating cells is rare in active cutaneous CD30+ ALCL, either primary or secondary. This percentage is not related to the apparent skin tumour burden but a significant figure appeared to be correlated with the detection of a dominant T-cell clone in peripheral blood. Overall, these data show that, unlike mycosis fungoides, peripheral blood involvement seems infrequent in cutaneous CD30+ ALCL. The hypothesis that a high percentage of CD30+ circulating cells might be related to the presence of a cryptic systemic disease cannot be ruled out. [source] Isograds and P,T evolution in the eastern Lepontine Alps (Graubünden, Switzerland)JOURNAL OF METAMORPHIC GEOLOGY, Issue 3 2002T. Nagel Abstract Reactions producing Al-rich index minerals in the south-eastern part of the Lepontine Dome (Central Alps, Switzerland) are investigated using mineral distribution maps, microstructural observations and equilibrium phase diagrams. The apparent staurolite mineral zone boundary corresponds to the paragonite breakdown reaction Pg + Grt + Qtz = Pl + Al2O3 + W. Equilibrium phase diagrams show that most natural metapelites do not contain staurolite or alumosilicates as long as univalent cations are predominantly accommodated in white mica. For a wide range of metapelitic compositions the paragonite breakdown releases sufficient Al for the formation of these minerals. Rare occurrences of staurolite and kyanite, north of the formerly mapped mineral zone boundaries, coexist with paragonite and are restricted to extremely Al-rich bulk compositions. The stable branch of the kyanite-forming paragonite breakdown reaction above 660 °C yields an additional mapable isograd. The second set of Al-releasing reactions is biotite-producing phengite breakdown. However, these reactions are less suitable to produce well defined reaction isograds in the field as they are more continuous and their progress is strongly dependent on bulk composition. Well developed fibrolite in metapelites does not appear until staurolite starts to breakdown. We conclude that amphibolite facies conditions in the study area were attained by decompression, without substantial heating at low pressures. [source] The prognosis and incidence of social phobia in an elderly population.ACTA PSYCHIATRICA SCANDINAVICA, Issue 1 2010A 5-year follow-up Karlsson B, Sigström R, Waern M, Östling S, Gustafson D, Skoog I. The prognosis and incidence of social phobia in an elderly population. A 5-year follow-up. Objective:, To examine the prognosis and incidence of social fears and phobia in an elderly population sample followed for 5 years. Method:, A general population sample (N = 612) of non-demented men (baseline age 70) and women (baseline age 70 and 78,86) was investigated in 2000,2001 and in 2005,2006 with semi-structured psychiatric examinations including the Comprehensive Psychopathological Rating Scale, and the Mini International Neuropsychiatric Interview. Social phobia was diagnosed according to the DSM-IV criteria. Results:, Among nine individuals with DSM-IV social phobia in 2000, 5 (55.6%) had no social fears in 2005, and 1 (11.1%) still met the criteria for DSM-IV social phobia. Among individuals without DSM-IV social phobia in 2000 (N = 603), 12 (2.0%) had DSM-IV social phobia in 2005. Conclusion:, These findings challenge the notion that social phobia is a chronic disorder with rare occurrence in old age. [source] Heart rate and QT variability in children with anxiety disorders: A preliminary reportDEPRESSION AND ANXIETY, Issue 2 2001Vikram K. Yeragani M.B.B.S. Abstract This study compared beat-to-beat heart rate and QT variability in children with anxiety disorders (n=7) and normal controls (n=15) by using an automated algorithm to compute QT intervals. An increase in QT variability appears to be associated with a higher risk for sudden cardiac death. A decrease in heart rate variability is also linked to significant cardiovascular events. Supine detrended QT variability, QT variability corrected for mean QT interval, and QTvi (a log ratio of QT variance normalized for mean QT over heart rate variability normalized for mean heart rate) were significantly higher in children with anxiety compared to controls (P<0.05). The largest Lyapunov Exponent (LLE) of heart rate time series was significantly lower (P<0.05) in children with anxiety compared to controls. These findings suggest a relative increase in sympathetic activity and a relative decrease in cardiac vagal activity in children with anxiety disorders, and are discussed in the context of the effects of tricyclics on cardiac autonomic function in children, and the rare occurrence of sudden death during tricyclic antidepressant treatment. Depression and Anxiety 13:72,77, 2001. © 2001 Wiley-Liss, Inc. [source] Sebaceous Carcinoma, Basal Cell Carcinoma, Trichoadenoma, Trichoblastoma, and Syringocystadenoma Papilliferum Arising within a Nevus SebaceusDERMATOLOGIC SURGERY, Issue 12p2 2004Christopher J. Miller MD Background. Nevus sebaceus has a well-documented potential to develop a wide variety of neoplasms of both epidermal and adnexal origins. It is highly unusual for more than three tumors to arise simultaneously within a single nevus sebaceus. Sebaceous carcinoma arising within a nevus sebaceus is a rare occurrence. Objective. The objective was to report the case of a patient with a nevus sebaceus that simultaneously developed five distinct neoplasms of epidermal and various adnexal origins and to report the fourth case of sebaceous carcinoma arising within a nevus sebaceus. Methods. A 45-year-old woman presented with a nevus sebaceusthat contained five separate neoplasms, including sebaceous carcinoma, basal cell carcinoma, trichoadenoma, trichoblastoma, and syringocystadenoma papilliferum. Results. Complete excision of the nevus sebaceus and the five tumors was performed. Systemic work-up showed no evidence of metastatic disease or association with Muir-Torre syndrome. Conclusion. This case report highlights the diverse neoplastic potential of nevus sebaceus and demonstrates the capacity of this hamartoma to develop aggressive tumors, such as sebaceous carcinoma. Prophylactic excision or at least close clinical surveillance for sudden development of new growths is warranted in all cases of nevus sebaceus. [source] Neurotoxicity upon infusion of dimethylsulfoxide-cryopreserved peripheral blood stem cells in patients with and without pre-existing cerebral diseaseEUROPEAN JOURNAL OF HAEMATOLOGY, Issue 6 2007Lutz P. Mueller Abstract Objective:, Toxicity related to the infusion of dimethylsulfoxide (DMSO)-cryopreserved peripheral blood stem cells (DMSO-PBSC) mainly comprises cardiovascular events. Fatal neurotoxicity has been reported in a few cases. DMSO represents the putative causative agent of such rare toxicities and elaborate strategies to replace DMSO would benefit from the identification of predisposing factors for DMSO-related toxicities. Methods:, Here, we report on DMSO-related neurotoxicity in a series of patients (n = 51) receiving DMSO-PBSC. The analyzed patient-series included eight patients with pre-existing cerebral disease, partially with a history of epileptic seizures. Results:, Neurotoxicity was observed in only one patient who suffered from a generalized tonic seizure upon infusion of DMSO-PBSC and for which the clinical course is reported herein. No neurotoxicity was observed in the group of patients with pre-existing neurological disease. Furthermore, no neurotoxicity was observed in patients who received particularly large volumes of DMSO. In all patients, mild non-neurological side effects occurred but besides the reported seizure no other severe adverse events were observed upon PBSC-infusion. Conclusions:, To our knowledge, this is the first report addressing the identification of predisposing factors for DMSO-related neurotoxicty. We conclude that infusion of DMSO-PBSC can be performed safely in patients with pre-existing cerebral disease despite the rare occurrence of severe neurotoxicity. Retrospective multicenter studies are warranted to identify patients who would benefit from elaborate methods of DMSO-replacement. [source] Acute isolated cerebral mucormycosis in a patient with high grade non-Hodgkins lymphomaEUROPEAN JOURNAL OF NEUROLOGY, Issue 4 2000M. Zarei A 57-year-old female in complete remission of grade IV non-Hodgkin lymphoma whilst on intensive chemotherapy, suddenly developed unilateral hemispheric stroke with a fatal outcome in 3 days. She was apyrexial and had received antifungal prophylaxis during her treatment. Post-mortem examination showed complete thrombosis of the internal carotid artery leading to infarction in the territory of the middle and anterior cerebral arteries. Microscopic examination of the brain showed involvement of intra-cranial vessel walls and brain parenchyma by mucormyces, with no evidence of systemic mucormycosis. Isolated cerebral mucormycosis is a rare occurrence, more commonly found in intravenous drug abusers, but can occur in patients with haematological malignancy. [source] A Question of Rites?HISTORY COMPASS (ELECTRONIC), Issue 5 2006Perspectives on the Colonial Encounter with Sati Although a rare occurrence, sati has become a highly controversial issue in modern India. In the wake of the now notorious burning of Roop Kanwar in 1987, sati and its glorification became a terrain on which wider issues about religion, identity, modernity and tradition were contested. In this debate both supporters and opponents of sati invoked the rhetoric of ,rights'. It is generally agreed that such terms in the contemporary debate have their roots in the colonial period; some supporters of sati go as far as to argue that those who condemn sati as a violation of women's rights are adopting a ,Western' perspective without appreciating sati's ,true' social, religious and cultural significance. In doing so, however, they assume a homogenous and consistent colonial condemnation of sati. New perspectives suggest, however, that the British response to sati was more multifaceted than this allows and the link between colonial discourses and modern protagonists more complex. [source] Langerhans cell histiocytosis: fascinating dynamics of the dendritic cell,macrophage lineageIMMUNOLOGICAL REVIEWS, Issue 1 2010R. Maarten Egeler Summary:, In its rare occurrence, Langerhans cell histiocytosis (LCH) is a dangerous but intriguing deviation of mononuclear phagocytes, especially dendritic cells (DCs). Clinically, the disease ranges from self-resolving or well manageable to severe and even fatal. LCH lesions in skin, bone, and other sites contain high numbers of cells with phenotypic features resembling LCs admixed with macrophages, T cells, eosinophils, and multinucleated giant cells. Here we review current progress in the LCH field based on two central questions: (i) are LCH cells intrinsically aberrant, and (ii) how does the lesion drive pathogenesis? We argue that LCH cells may originate from different sources, including epidermal LCs, tissue Langerin+ DCs, or mononuclear phagocyte precursors. Current and prospective in vitro and in vivo models are discussed. Finally, we discuss recent insights into plasticity of T-helper cell subsets in light of the lesion microenvironment. LCH continues to provide urgent clinical questions thereby inspiring innovative DC lineage research. [source] Subcutaneous emphysema during restorative dentistryINTERNATIONAL JOURNAL OF PAEDIATRIC DENTISTRY, Issue 3 2007ROBERT J. STEELMAN Background., Subcutaneous emphysema is a rare occurrence in the dental setting. When it does occur, the entity may be mistaken for an anaphylactic reaction to a local anaesthetic agent or other medications used in dental surgery. Case Report., During restorative treatment of a left second primary molar air was introduced under an operculum. Communication with submandibular and sublingual spaces to the mediastinum was established. Conclusion., Therapy for subcutaneous emphysema involves, correct diagnosis and antibiotic coverage. Subcutaneous air will resolve over time so observation is indicated. [source] Myiasis after study trip to French GuianaJOURNAL DER DEUTSCHEN DERMATOLOGISCHEN GESELLSCHAFT, Issue 5 2010Heike A. Schreiber Summary Cutaneous myiasis is usually a harmless tropical disease caused by infestation with larvae from a variety of flies. Because of its rare occurrence in Europe, it is often misdiagnosed. Increased travel to tropical regions has correspondingly increased the number of cases observed in Europe. The furuncular type of cutaneous myiasis in a 31-year-old biology student was diagnosed upon his return from French Guiana. The student cultured one of the larvae to obtain a botfly. This case is discussed in the light of the current literature on pathogenesis, incidence and therapy of cutaneous myiasis. [source] Non-hematopoietic cutaneous metastases in children and adolescents: thirty years experience at St. Jude Children's Research HospitalJOURNAL OF CUTANEOUS PATHOLOGY, Issue 10 2000W. A. Wesche Background: The spectrum of cutaneous metastasis of non-hematopoietic neoplasms in the pediatric population is not well documented. We report the histologic diversity of this unusual process over a 30-year period at a tertiary care center for pediatric malignancy (St. Jude Children's Research Hospital, Memphis, TN, USA). Methods: Of 1,971 pathology accessions which included histologic material on skin (1,604 surgical cases and 367 autopsy cases) we found 40 cases (2% of total skin accessions) coded for metastatic non-hematopoietic malignancy. Results: The patients (n=34) ranged in age from 1 month to 20 years (mean=9.8 years) and had a male:female ratio of 1:1. The histologic diagnoses were as follows: rhabdomyosarcoma NOS (6 cases), embryonal rhabdomyosarcoma (4 cases), alveolar rhabdomyosarcoma (4 cases), neuroblastoma (8 cases), osteosarcoma (2 cases), choriocarcinoma (2 cases), peripheral neuroepithelioma or Ewing's sarcoma (2 cases), malignant rhabdoid tumor (1 case), paraganglioma (1 case), nasopharyngeal carcinoma (1 case), sarcoma NOS (1 case), colon adenocarcinoma (1 case), and malignant melanoma (1 case). Conclusions: Cutaneous or subcutaneous metastasis of non-hematopoietic malignancies in children and adolescents is a rare occurrence but in a high percentage of cases may be the first manifestation of disease. The tumors most likely to metastasize to the skin in children are rhabdomyosarcoma and neuroblastoma and they are more likely than adult malignancies to disseminate to multiple distant sites. [source] Peritoneal mesothelioma presenting as an acute surgical abdomen due to jejunal perforationJOURNAL OF DIGESTIVE DISEASES, Issue 4 2007Nikolaos S SALEMIS BACKGROUND: Peritoneal mesothelioma is a rare disease associated with poor prognosis. Acute abdomen as the first presentation is an extremely rare occurrence. We report an exceptional case of a patient who was found to have a jejunal perforation due to infiltration of peritoneal mesothelioma. METHODS: A 62-year-old man was admitted with clinical signs of peritonitis. Computerized tomographic scans showed a mass distal to the ligament of Treitz, thickening of the mesentery and a small amount of ascites. RESULTS: Emergency laparotomy revealed a perforated tumor 15 cm distal to the ligament of Treitz and diffuse peritoneal disease. Segmental small bowel resection and suboptimal cytoreduction were performed. Histopathology and immunohistochemistry showed infiltration of malignant mesothelioma. During the postoperative period pleural mesothelioma was also diagnosed. Despite adjuvant chemotherapy, the patient died of disseminated progressive disease 7 months after surgery. CONCLUSIONS: Peritoneal mesothelioma is a rare malignancy with grim prognosis. Small bowel involvement is a poor prognostic indicator. Our case of a small bowel perforation due to direct infiltration by peritoneal mesothelioma appears to be the first reported in the English literature. [source] Sinusoidal heart rate pattern: Reappraisal of its definition and clinical significanceJOURNAL OF OBSTETRICS AND GYNAECOLOGY RESEARCH (ELECTRONIC), Issue 3 2004Houchang D. Modanlou Abstract Objectives: To address the clinical significance of sinusoidal heart rate (SHR) pattern and review its occurrence, define its characteristics, and explain its physiopathology. Background: In 1972, Manseau et al. and Kubli et al. described an undulating wave form alternating with a flat or smooth baseline fetal heart rate (FHR) in severely affected, Rh-sensitized and dying fetuses. This FHR pattern was called ,sinusoidal' because of its sine waveform. Subsequently, Modanlou et al. described SHR pattern associated with fetal to maternal hemorrhage causing severe fetal anemia and hydrops fetalis. Both Manseau et al. and Kubli et al. stated that this particular FHR pattern, whatever its pathogenesis, was an extremely significant finding that implied severe fetal jeopardy and impending fetal death. Undulating FHR pattern: Undulating FHR pattern may be due to the following: (1) true SHR pattern; (2) drugs; (3) pre-mortem FHR pattern; (4) pseudo-SHR pattern; and (5) equivocal FHR patterns. Fetal conditions associated with SHR pattern: SHR pattern has been reported with the following fetal conditions: (1) severe fetal anemia of several etiologies; (2) effects of drugs, particularly narcotics; (3) fetal asphyxia/hypoxia; (4) fetal infection; (5) fetal cardiac anomalies; (6) fetal sleep cycles; and (7) sucking and rhythmic movements of fetal mouth. Definition of true SHR pattern: Modanlou and Freeman proposed the following definition for the interpretation of true SHR pattern: (a) stable baseline FHR of 120,160 bpm; (b) amplitude of 5,15 bpm, rarely greater; (c) frequency of 2,5 cycles per minute; (d) fixed or flat short-term variability; (e) oscillation of the sinusoidal wave from above and below a baseline; and (f) no areas of normal FHR variability or reactivity. Physiopathology: Since its early recognition, the physiopathology of SHR became a matter of debate. Murata et al. noted a rise of arginine vasopressin levels in the blood of posthemorrhagic/anemic fetal lamb. Further works by the same authors revealed that with chemical or surgical vagotomy, arginine vasopressin infusion produced SHR pattern, thus providing the role of autonomic nervous system dysfunction combined with the increase in arginine vasopressin as the etiology. Conclusion: SHR is a rare occurrence. A true SHR is an ominous sign of fetal jeopardy needing immediate intervention. The correct diagnosis of true SHR pattern should also include fetal biophysical profile and the absence of drugs such as narcotics. [source] Pulmonary aspergilloma with renal oxalosis: fatal effect at a distanceMYCOSES, Issue 3 2009Pradeep Vaideeswar Summary Some species of the fungus Aspergillus, especially Aspergillus niger, produce oxalic acid as a fermentation byproduct. The acid combines with calcium ions at physiological pH to form insoluble calcium oxalate crystals that are mainly deposited at local sites. This is often seen in the lungs, where the crystals tend to potentiate the destructive capacity of the fungus. In rare instances, there is hyperoxaluria and deposition of the crystals in the renal tubules. We report this rare occurrence in a 59-year-old man with pulmonary aspergilloma and acute renal failure. To the best of our knowledge, this is the fifth case to be reported. [source] Chronic demyelinating polyneuropathy in graft-versus-host disease following allogeneic bone marrow transplantationNEUROPATHOLOGY, Issue 1 2002Toshiko Nagashima In recent years a novel problem has arisen in organ transplantation medicine, namely GVHD. The nervous system has been involved mainly at the level of the CNS and this can lead to a serious outcome for the patient. In rare cases, peripheral nerves may be affected and show acute or chronic polyneuropathy. Here a case is reported of polyneuropathy associated with chronic GVHD. A 32-year-old man, suffering from chronic GVHD following an allogeneic bone marrow transplantation (BMT) for malignant lymphoma at the age of 25, developed a motor dominant polyneuropathy 5 years later. Electrophysiologic studies demonstrated the demyelinating type of polyneuropathy. Biopsy specimens from skin and skeletal muscle disclosed perivascular lymphocytic infiltrates expressing T-cell markers. The sural nerve showed a loss of myelinated nerve fibers with epineurial fibrosis and rare occurrence of T cells, but without obvious vasculitic changes. The present case suggested that polyneuropathy could develop in association with chronic GVHD in some patients with a longstanding disease course. [source] Unusual presentation of a hypoglossal nerve neurofibromaORAL SURGERY, Issue 4 2009V. Patel Abstract Neurofibromatosis type 1 genetic disorder is an inherited autosomal dominant trait with variable penetrance and expressivity and occurs in one of every 2000,3300 live births. Neurofibromatosis type 2, on the other hand, afflicts only one in approximately 50 000 people. Within these patients, the frequency of intra-oral involvement of neurofibromas has been reported in a range of 4,7%. Alternatively, neurofibromas in the oral cavity minus the presence of a neurofibromatosis disease has been documented but the prevalence is even less than stated earlier making a solitary neurofibroma of the tongue a rare occurrence. This article reports an unusual presentation of a neurofibroma masquerading as a lipoma with the tumour occurring at the base of the tongue. [source] Effects of Scatter Radiation on ICD and CRT FunctionPACING AND CLINICAL ELECTROPHYSIOLOGY, Issue 6 2008SURAJ KAPA M.D. Background: Effects of direct radiation on implantable cardiac devices have been well studied. However, the effects of scatter radiation are not as clear. Recommendations on management of patients with implantable cardiac devices undergoing radiotherapy are based on limited studies mostly involving pacemakers. We sought to elucidate the effects of scatter radiation on implantable cardioverter-defibrillators (ICDs) and cardiac resynchronization therapy (CRT)-ICDs. Methods: We exposed 12 ICDs and eight CRT-ICDs to 400 cGy of scatter radiation from a 6-MV photon beam. Devices were programmed with nominal parameters and interrogated prior to radiation, after each fraction, upon completion of the radiation course and again 1 week later. A retrospective review of patients undergoing radiotherapy at the Mayo Clinic,Rochester between 2002 and 2007 in whom the device was outside the radiation field was also performed. There were 13 patients with devices undergoing radiotherapy during this time period, 12 of whom were interrogated prior to and after radiation. Results: Interrogation reports were reviewed for device reset or parameter changes. There was no evidence of reset or malfunction during or after radiation. Also, no episodes of device reset, inappropriate sensing or therapy, or changes in programmed parameters were found in our review of patients undergoing radiotherapy. Conclusions: Device reset or malfunction associated with scatter radiation likely represents an unpredictable, rare occurrence. While we see no clear contraindication to radiotherapy in patients with ICDs or CRT-ICDs, precautions should be taken to avoid direct radiation exposure and to closely evaluate patient outcomes before and after the radiation course. [source] Spontaneous regression of malignant lymphoma of the breastPATHOLOGY INTERNATIONAL, Issue 7 2004Kuniko Iihara A complete spontaneous regression of diffuse large B cell lymphoma involving the right breast, confirmed by aspiration cytology, is reported. The patient visited a hospital due to the rapid growth of a tumor in the right breast. Five years previously she underwent a craniotomy for a brain tumor, diagnosed as B-cell malignant lymphoma, and received several courses of irradiation to the brain. Analysis of the breast tumor cells obtained by aspiration revealed lymphoma cells morphologically, which were similar to the tumor cells in the brain expressing CD20. While waiting for further examination, the tumor regressed rapidly and was not palpable after 20 days. An excisional biopsy of the breast exhibited no definite malignant lymphoma cells among a diffuse population of CD45RO and CD8-positive small lymphocytes. Nucleotide sequencing of HCDR3s of the brain tumor and breast tumor cells showed a completely matched sequence, revealing the breast mass to be a metastatic lesion from the tumor of the brain. Although there was no tumorous lesion, the patient received additional chemotherapy and has shown no sign of recurrence in the breast for 7 years. We were able to confirm that the breast lymphoma shown in the aspiration cytology was a metastatic one, which was not proven histologically prior to chemotherapy, and regard the present case as a malignant lymphoma of the breast showing spontaneous regression. The present case shows a rare occurrence of spontaneous regression of diffuse large B cell malignant lymphoma after aspiration and suggests that CD8-positive T cells might be related to the regression. [source] Post dural puncture headache in a pediatric patient with idiopathic intracranial hypertensionPEDIATRIC ANESTHESIA, Issue 9 2005OLUBUKOLA O. NAFIU MD FRCA Summary We describe the occurrence of postdural puncture headache (PPDH) in an adolescent with idiopathic intracranial hypertension (IIH) and its successful management with an epidural blood patch. PPDH is a very rare occurrence in patients with intracranial hypertension and is described as a paradoxical situation in the literature. There are only two previous case reports (in adults) of the possible association. A 15-year-old obese patient with a diagnosis of IIH had an uneventful diagnostic spinal tap using a 22G Quincke needle in the pediatric emergency department but returned 24 h later with PPDH. After a failed trial of conservative management, she had an uneventful but curative epidural blood patch with 15 ml of autologous venous blood and was able to return to school the day after the blood patch. Follow-up review by her neuro-ophthalmologist shows resolution of her headaches, considerable improvement in her visual field defect and resolution of papilledema. This is the first report of PPDH and its successful management with an epidural blood patch in a pediatric patient with IIH. [source] Giant Congenital Nevus with Progressive Sclerodermoid Reaction in a NewbornPEDIATRIC DERMATOLOGY, Issue 4 2001Sean F. Pattee B.S. Giant congenital melanocytic nevi are a rare occurrence in the pediatric population. The risk of malignant transformation associated with these lesions has been well established; however, the management strategies for giant congenital nevi remain controversial. We report an unusual sclerodermoid reaction in a giant congenital nevus in a 6-week-old Caucasian girl. Given its abnormal clinical appearance, the entire lesion was excised. The histology was consistent with an atypical compound/sclerosing spindle and epithelioid cell congenital nevus. No evidence of malignant change was seen histologically. The incidence of malignant transformation in giant congenital nevi has been difficult to calculate. Review of the literature yields an incidence of between 4 and 9%, favoring surgical excision of these lesions where possible. Atypical presentations of giant congenital nevi are rare, and we have found no other reported cases with a stromal change similar to that seen in our patient. We hypothesize that this change may represent an atypical host reaction to the nevus cells. [source] Catastrophic multiple organ ischemia due to an anti-Pr cold agglutinin developing in a patient with mixed cryoglobulinemia after treatment with rituximab,AMERICAN JOURNAL OF HEMATOLOGY, Issue 2 2009Joshua Ruch Cold agglutinin disease occurring with cryoglobulinemia is a rare occurrence. Here, we report a patient with mixed cryoglobulinemia that was treated with rituximab and, after response, developed an anti-Pr cold agglutinin that manifested with hemolysis and microvascular occlusion causing mesenteric ischemia and cerebral infarction. Unlike previous reports of patients with cryoglobulinemia and cold agglutinin disease, our patient did not have a detectable cryoprecipitate when his cold agglutinin manifested. Am. J. Hematol, 2009. © 2008 Wiley-Liss, Inc. [source] Hypereosinophilia associated with cardiac rhabdomyosarcomaAMERICAN JOURNAL OF HEMATOLOGY, Issue 1 2003Vincent Lo Re III Abstract Hypereosinophilia, defined as an absolute eosinophil count exceeding 1,500/mm3, is caused by a limited number of disorders. Its association with malignancy is a rare occurrence that typically denotes extensive metastasis and a poor prognosis. Several theories have been proposed to explain the hypereosinophilia associated with malignancy, focusing on the production of eosinophilopoietic cytokines by tumors. We describe a patient with hypereosinophilia associated with a cardiac rhabdomyosarcoma, review the etiologies of hypereosinophilia, and discuss the possible mechanisms. Am. J. Hematol. 74:64,67, 2003. © 2003 Wiley-Liss, Inc. [source] Crystal structure and kinetic mechanism of aminoglycoside phosphotransferase-2,-IVaPROTEIN SCIENCE, Issue 8 2010Marta Toth Abstract Acquired resistance to aminoglycoside antibiotics primarily results from deactivation by three families of aminoglycoside-modifying enzymes. Here, we report the kinetic mechanism and structure of the aminoglycoside phosphotransferase 2,-IVa (APH(2,)-IVa), an enzyme responsible for resistance to aminoglycoside antibiotics in clinical enterococcal and staphylococcal isolates. The enzyme operates via a Bi-Bi sequential mechanism in which the two substrates (ATP or GTP and an aminoglycoside) bind in a random manner. The APH(2,)-IVa enzyme phosphorylates various 4,6-disubstituted aminoglycoside antibiotics with catalytic efficiencies (kcat/Km) of 1.5 × 103 to 1.2 × 106 (M,1 s,1). The enzyme uses both ATP and GTP as the phosphate source, an extremely rare occurrence in the phosphotransferase and protein kinase enzymes. Based on an analysis of the APH(2,)-IVa structure, two overlapping binding templates specifically tuned for hydrogen bonding to either ATP or GTP have been identified and described. A detailed understanding of the structure and mechanism of the GTP-utilizing phosphotransferases is crucial for the development of either novel aminoglycosides or, more importantly, GTP-based enzyme inhibitors which would not be expected to interfere with crucial ATP-dependent enzymes. [source] Thyroid storm prior to induction of anaesthesiaANAESTHESIA, Issue 10 2004E. A. Hirvonen Summary A 53- year-old woman without a previous history of thyroid disease was scheduled for mastectomy. On arrival in the operating theatre unpremedicated she appeared restless and tachycardic. Midazolam and fentanyl was administered intravenously. Concomitantly, sinus tachycardia developed and a flush reaction was observed in the skin of the thoracic region and neck. The blood pressure increased to 265/160 mmHg and the patient lost consciousness and became apnoeic. Unconsciousness and apnoea lasted for approximately 25 min and the operation was postponed. Further investigations revealed an elevated serum free thyroxine level and suppressed serum thyrotropin diagnostic of hyperthyroidism. The serum TSH receptor antibody concentration was elevated, indicating that the patient was suffering from Graves' disease. We present a case of a previously unknown hyperthyroid patient, with breast cancer, presenting as a thyroid crisis on induction of anaesthesia. Although being quite a rare occurrence, unsuspected thyroid disease should be borne in mind when an agitated patient enters the operating theatre. [source] Benign phylloides tumour with leiomyoma breast: a rare occurrenceANZ JOURNAL OF SURGERY, Issue 6 2009Kavita Kohli MD No abstract is available for this article. [source] Metachronous bilateral primary low-grade mucosa-associated lymphoid tissue non-Hodgkins lymphoma of the breastASIA-PACIFIC JOURNAL OF CLINICAL ONCOLOGY, Issue 3 2009M Helene YILMAZ Abstract We report the rare occurrence of bilateral low-grade MALT lymphoma of the breast in a 73 year old female. The clinical, pathological and treatment details are discussed, with a review of the literature. [source] Cerebral venous sinus thrombosis manifesting as bilateral subdural effusionACTA NEUROLOGICA SCANDINAVICA, Issue 6 2004G. Marquardt Three patients with bilateral subdural effusion, an exclusive manifestation of cerebral venous sinus thrombosis (CVST), are presented. A possible explanation of this rare occurrence is provided, and the differential therapeutic strategies are discussed. We propose to consider CVST in cases of subdural effusions of obscure origin. Appropriate imaging studies should not be delayed if there is suspicion of sinus thrombosis to enable adequate therapy to be started as soon as possible. [source] Generalized eruptive lentiginosis induced by chemotherapyCLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 4 2010D. De Summary Lentigines are characterized by brown macules developing due to increased proliferation of melanocytes in the dermoepidermal junction. Many drugs, including immunosuppressants and immunomodulators, have been shown to cause generalized lentiginosis. We describe a case of lentiginosis induced by cancer chemotherapy, an extremely rare occurrence. [source] Cefuroxime-induced pemphigus erythematosus in a young boyCLINICAL & EXPERIMENTAL DERMATOLOGY, Issue 6 2009A. Baroni Summary Pemphigus erythematosus (Senear,Usher syndrome) is a variant of superficial pemphigus with features of both lupus erythematosus and pemphigus. It affects mainly middle-aged adults, and is rarely observed before the age of 20 years. The case of a 14-year-old boy who showed cutaneous lesions suggestive for pemphigus erythematosus is described. Not all laboratory and histopathological investigations confirmed the hypothesis, so a diagnosis of clinical pemphigus erythematosus was made. Systemic steroid therapy was effective in controlling the disease. This case is interesting because of the rare occurrence of pemphigus erythematosus in adolescence and the possibility of another drug being added to the list of pemphigus inducers. [source] | |||||||||||||||||||||||||||||||