1/11. A case report of aspergillus hypersensitivity syndrome with obstructing bronchial aspergillosis.INTRODUCTION: A 62-year-old Indian male with diabetes mellitus presented with atypical, overlap features of aspergillus hypersensitivity syndrome and obstructing bronchial aspergillosis. CLINICAL PICTURE: He was febrile and tachypnoeic with diffuse crepitations and wheezing. Chest X-ray was normal but eosinophil count was 2900/mm3 and Ig E > 10,000 IU/ml. TREATMENT AND OUTCOME: He responded initially to high dose steroid therapy but deteriorated subsequently from extensive mucus plugging of the bronchial tree which resulted in respiratory failure and death. He was hiv-negative. CONCLUSION: culture and histologic examination of bronchoscopically identified tracheobronchial mucus plugs should be performed as early treatment may be life-saving.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
2/11. Rapid progress of acute suppurative cholangitis to secondary sclerosing cholangitis sequentially followed-up by endoscopic retrograde cholangiography.A 66-year-old man was admitted to our hospital because of right hypochondralgia and fever after colonic polypectomy. Endoscopic examination revealed purulent bile excretion from the duodenal papilla orifice; based on this finding, acute suppurative cholangitis was diagnosed. An endoscopic retrograde cholangiography revealed no abnormality in the biliary tree. However, chronic cholestasis persisted, and endoscopic cholangiography performed 4 months later disclosed a beaded appearance of the intrahepatic bile ducts; this sign is a characteristic finding of sclerosing cholangitis. This is the first report of rapid progression of acute suppurative cholangitis to secondary sclerosing cholangitis sequentially followed-up by endoscopic retrograde cholangiography.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
3/11. Molecular changes track recurrence and progression of bronchial precancerous lesions.diagnosis of lung cancer is reached when the disease had grown to advanced stages in its natural history. Thus, novel molecular markers for early detection and risk assessment are needed. Here we performed a longitudinal study of 20 morphological lesions occurred in the bronchial tree of high-risk patients. The lesions were subjected to life (laser-induced fluorescence endoscopy) bronchoscopy and molecular follow-up over a 4 year period. The presence and the persistence of specific genetic alterations were correlated with recurrence or progression of the disease on follow-up. Molecular lesions considered as highly predictive of invasion could be prospectively used as diagnostic markers of high risk for lung cancer in exposed patients.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
4/11. Familial idiopathic intracranial hypertension.AIM: To analyze the development and occurrence of the idiopathic intracranial hypertension and consequent visual loss in a family affected with idiopathic intracranial hypertension. methods: We studied 15 members of the same family and found six of them affected with idiopathic intracranial hypertension, which was accompanied with visual loss as a repercussion of the disease. Idiopathic intracranial hypertension was diagnosed on neurological and radiological examination. Visual examination to establish visual loss included fundoscopy, visual acuity, visual field testing, and ultrasonography of the optic nerve. RESULTS: The construction of a family tree and detailed examination of 15 family members revealed idiopathic intracranial hypertension with visual disturbances, even amaurosis, and different stages of visual field constriction in three members of the family: the mother and her two daughters. Due to the symptoms of idiopathic intracranial hypertension, such as headaches, nausea, vertigo, and the presence of transient visual obscuration and papilledema, in three other members of this family (aged 16, 17, and 25 years), we considered a presumptive diagnosis of idiopathic intracranial hypertension, and the need for thorough follow-up. Medical data on the family grandmother, who died 34 years ago, suggested that she also had symptoms of idiopathic intracranial hypertension. One of the patients underwent surgical treatment by a lumbo-peritoneal shunt operation worsening of the symptoms. CONCLUSION: It is very important to include idiopathic intracranial hypertension in differential diagnosis of papilledema and recognize it in early stages to prevent vision loss. Current successful therapeutic approaches and close follow-up of such patients require teamwork of neurologists, ophthalmologists, and neurosurgeons.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
5/11. Posterior stabilisation of a malignant cervico-thoracic vertebral bone defect.Oesophageal cancer is frequently complicated by malignant fistulae. necrosis of the tumour following radiotherapy or chemotherapy may lead to the development of fistulae between the oesophagus and adjacent tissues and organs. We report the expansion of an extra-luminal oesophageal cancer after resection, invading the cervico-thoracic spine, fortunately without neurological deficit, and leading to instability and formation of a malignant fistula linking the tracheo-bronchial tree to the subarachnoidal space. To prevent imminent paraplegia and to alleviate severe pain, we rigidly stabilised the spine at the cervico-thoracic junction using an angle-stable system through a single posterior approach. Further postoperative follow-up revealed no signs of neurological deterioration. Cervico-thoracic stability was preserved until the patient died nearly five months postoperatively. This case shows that posterior stabilisation and decompression may be a palliative option for patients with imminent paraplegia and severe pain due to advanced tumour infiltration of the cervico-thoracic spine.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
6/11. Wayfinding in familiar and unfamiliar environments in a case of progressive topographical agnosia.A 71-year-old right-handed man (F.G.) presents with prosopagnosia and with an inability to recognize famous and familiar buildings. Despite his deficit, F.G. obtained normal scores on neuropsychological tests of executive functions, language, praxis and primary visuoperceptual skills. brain MRI showed atrophy predominantly in the right temporal lobe, particularly in the fusiform gyrus and the parahippocampal cortex. The present study investigated F.G.'s ability to orient himself in familiar and new environments. His wayfinding abilities in a familiar environment (i.e., his hometown) were preserved despite an inability to recognize familiar and famous buildings, monuments and landmarks in this environment. Wayfinding was achieved through a heavy reliance on written indications (e.g., names of restaurants and streets), preservation of a pre-existing cognitive map of this familiar environment, and normal executive functions necessary to plan the execution of a given trajectory. In an unfamiliar environment, F.G.'s topographical agnosia resulted in severe wayfinding difficulties and in the inability to build an adequate cognitive spatial representation. F.G.'s topographical agnosia results from a high-level visuoperceptual deficit, characterized by an inability to access a global configuration of complex visual stimuli such as familiar and famous monuments, and an over-reliance on the processing of local features.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
7/11. Intrahepatic cholangiocarcinoma presenting as the budd-chiari syndrome: a case report and literature review.Intrahepatic cholangiocarcinoma, an increasingly recognized primary tumour of the liver, is associated with a very poor prognosis. A patient with this tumour who presented with budd-chiari syndrome (the first to the authors' knowledge in Western literature and only the third patient overall) secondary to extensive thrombosis in his inferior vena cava extending from the right atrium down to his iliac vessels is described. Neither curative nor palliative intervention was deemed to be an option in this patient, who deteriorated rapidly while on anti-coagulants. Postmortem examination confirmed the radiological findings, and histological analysis revealed characteristic appearances of this tumour within the biliary tree and invasion into the inferior vena cava. Furthermore, biliary dysplasia, which can be a precursor to this cancer, was also noted within some of the bile ducts.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
8/11. IgV gene intraclonal diversification and clonal evolution in B-cell chronic lymphocytic leukaemia.Intraclonal diversification of immunoglobulin (Ig) variable (V) genes was evaluated in leukaemic cells from a B-cell chronic lymphocytic leukaemia (B-CLL) case over a 2-year period at four time points. Intraclonal heterogeneity was analysed by sequencing 305 molecular clones derived from polymerase chain reaction amplification of B-CLL cell IgV heavy (H) and light (C) chain gene rearrangements. Sequences were compared with evaluating intraclonal variation and the nature of somatic mutations. Although IgV intraclonal variation was detected at all time points, its level decreased with time and a parallel emergence of two more represented V(H)DJ(H) clones was observed. They differed by nine nucleotide substitutions one of which only caused a conservative replacement aminoacid change. In addition, one V(L)J(L) rearrangement became more represented over time. Analyses of somatic mutations suggest antigen selection and impairment of negative selection of neoplastic cells. In addition, a genealogical tree representing a model of clonal evolution of the neoplastic cells was created. It is of note that, during the period of study, the patient showed clinical progression of disease. We conclude that antigen stimulation and somatic hypermutation may participate in disease progression through the selection and expansion of neoplastic subclone(s).- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
9/11. Rapidly progressive sclerosing cholangitis following surgical treatment of pancreatic pseudotumor.Two patients with sclerosing cholangitis presented with a distal stricture of the common bile duct in association with pancreatic pseudotumors. jaundice resolved following surgery to correct biliary obstruction, but diffuse cholangiographic abnormalities and clinical evidence of sclerosing cholangitis became evident 2 and 4 months later. Rapid progression of symptomatic disease necessitated liver transplantation in one patient, but the other had a complete response to methotrexate therapy. The rapid disease progression in these two patients may have been triggered by surgery that resulted in a generalized fibroproliferative response of the biliary tree, already affected with localized sclerosing cholangitis contiguous to a pancreatic pseudotumor. We suggest that localized sclerosing cholangitis associated with pancreatic pseudotumors may be a unique variant that can progress rapidly but respond dramatically to antiinflammatory therapy.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
10/11. Hepatic heterotopias in the jejunum: a case study over time showing progressive degenerative changes.Multiple foci of heterotopic liver in the jejunum were sequentially discovered in an infant boy at the ages of 1 day, 2 months, and 4 months. This is the second reported case of jejunal heterotopic liver, a rare entity in any site. Progressive histological changes indicative of biliary duct obstruction were observed in the hepatic heterotopias, which demonstrated no connections to the main body of the liver or biliary tree.- - - - - - - - - - ranking = 1keywords = tree (Clic here for more details about this article) |
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