Cases reported "von Hippel-Lindau Disease"

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1/13. Peripheral primitive neuroectodermal tumor of the small bowel mesentery: a case showing perforation at onset.

    A case of peripheral primitive neuroectodermal tumor of the small bowel mesentery with an uncommon clinical onset is reported. A 40-year-old man was admitted to hospital because of acute severe abdominal pain. Chest X-ray revealed a free air sign beneath the diaphragm. At emergency surgery a mass measuring 11.0 x 8.0 cm with perforation was located in the jejunal mesenteric region. Histologically the resected lesion consisted of sheets of undifferentiated small round cells forming abortive Homer Wright rosettes. Some spindle-shaped cells showed perivascular pseudorosettes. Immunohistochemical study revealed that the tumor cells expressed positivity against CD99 (MIC2), neuron-specific enolase, synaptophysin and vimentin. To the authors' knowledge this is the first documentation of peripheral primitive neuroectodermal tumor of the small bowel mesentery with perforation at onset.
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2/13. Small intestinal infarction: a fatal complication of systemic oxalosis.

    Primary hyperoxaluria is a rare genetic disorder characterised by calcium oxalate nephrolithiasis and nephrocalcinosis leading to renal failure, often with extra-renal oxalate deposition (systemic oxalosis). Although ischaemic complications of crystal deposition in vessel walls are well recognised clinically, these usually take the form of peripheral limb or cutaneous ischaemia. This paper documents the first reported case of fatal intestinal infarction in a 49 year old woman with systemic oxalosis and advocates its consideration in the differential diagnosis of an acute abdomen in such patients.
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3/13. Ruptured true aneurysm of the splenic artery: an unusual cause of haemoperitoneum.

    True aneurysm of the splenic artery is rare. Two cases of ruptured true splenic artery aneurysms are presented. The first patient was a 62-year-old female who presented within 6 hours of the onset of symptoms. The other was a 27-year-old non-alcoholic male patient who was admitted in a state of shock after 2 days of observation in a peripheral hospital. Both patients had haemoperitoneum and were subjected to exploratory laparotomy. Aneurysmectomy was performed in both the patients in addition to left splenopancreatectomy in the first case and splenectomy in the second. However, due to the prolonged preoperative shock, the second patient succumbed on the third postoperative day.
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4/13. Recurrent haemoperitoneum in a mild von Willebrand's disease combined with a storage pool deficit.

    Haemoperitoneum secondary to haemorrhagic corpus luteum has been described in severe bleeding disorders such as afibrinogenaemia, type 3 von Willebrand's disease and patients under oral anticoagulation. We have studied one patient who presented three episodes of severe bleeding at ovulation, requiring surgery twice, with the diagnosis of mild von Willebrand's disease and mild storage pool deficiency. Mild von Willebrand's disease (associated with other thrombopathies or coagulopathies) should be considered in this pathology, although physicians would prefer to find a severe haemorrhagic disorder as the underlying condition in these cases.
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5/13. Spontaneous rupture of adrenal pheochromocytoma with capsular invasion.

    A 67-year-old Japanese man developed a sudden onset of severe right-side upper abdominal pain, nausea and vomiting. On hospitalization, physical examination revealed sweating, tachycardia, hypertension and the appearance of peripheral vasoconstriction. An urgent computed tomography scan with contrast demonstrated a large hematoma in the right retroperitoneal space. A phentolamine test and an 131iodine metaiodobenzylguanidine scan suggested pheochromocytoma. An elective right adrenalectomy was successfully performed after pretreatment for sufficient volume replacement with continuous administration of alpha- and beta-adrenergic blocking agents. Pathological diagnosis was an adrenal pheochromocytoma 9.0 x 6.5 cm in diameter with evidence of capsular invasion, which could be associated with a tear in the capsule.
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6/13. Systemic multiple aneurysms of the extracranial internal carotid artery, intracranial vertebral artery, and visceral arteries: case report.

    A rare case of systemic multiple aneurysms located in the extracranial internal carotid artery, intracranial vertebral artery, and intraperitonial arteries is described. A 56-year-old woman was referred to our hospital with suspected rupture of an aneurysm of the right extracranial internal carotid artery. Digital subtraction angiography demonstrated a giant aneurysm in the right extracranial internal carotid artery and an aneurysm of fusiform type of the left intracranial vertebral artery. The extracranial carotid artery aneurysm was successfully resected, with end-to-end anastomosis of the internal carotid artery, preserving the cranial nerves. Five days later, an aneurysm of the left hepatic artery ruptured unexpectedly and was treated with emergency surgery. Other aneurysms in the liver and spleen were identified on postoperative celiac angiography. The patient subsequently underwent an operation for a left intracranial vertebral artery aneurysm by proximal clipping.
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ranking = 1.1937127094679
keywords = nerve
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7/13. Is neuroborreliosis a medical emergency?

    Although lyme disease affects the nervous system in many ways (collectively known as neuroborreliosis), only rarely does it present as a medical emergency. In extreme cases, it may cause (1) encephalitis, (2) a rapidly progressive peripheral neuropathy, or (3) a painful truncal radiculopathy that may be confused with a severe visceral process. Knowing when to consider this spirochetosis in the differential diagnosis requires an understanding of its true clinical spectrum, and of an appropriate diagnostic and therapeutic approach.
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8/13. Acute abdomen by varicella zoster virus induced gastritis after autologous peripheral blood stem cell transplantation in a patient with non-Hodgkin's lymphoma.

    We report on a 54-year-old male patient with an aggressive T cell non-Hodgkin's lymphoma with abdominal manifestation undergoing autologous peripheral blood stem cell transplantation after high-dose chemotherapy in April 2003. About 4 months after transplantation, he developed severe upper abdominal pain. Ultrasound examination, X-ray, computed tomography of the abdomen and cardiac diagnostics could not explain the symptoms. While empiric therapy with high-dose acyclovir was started, we could document herpetic lesions in the gastric antrum by endoscopy. The epigastric pain rapidly decreased within several days after the start of acyclovir therapy. No herpetic skin lesions were observed at any time during the disease. This report demonstrates the importance of viral-induced gastritis in the differential diagnosis of severe abdominal pain in patients receiving autologous peripheral blood stem cell transplantation.
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9/13. intestinal obstruction at the onset of acute lymphoblastic leukemia in a child.

    Surgical complications need not be fatal in acute leukemia. If these are promptly diagnosed and properly treated, the prognosis will improve. This report deals with a case of acute lymphoblastic leukemia presenting with an acute abdomen following surgery for choledochal cyst. A peripheral blood smear and examination of the bone marrow revealed acute lymphoblastic leukemia. The child received transfusions of blood and platelets. Pretreatment with prednisolone was started as therapy for leukemia, and 2 days later, the patient underwent surgery. Therapy was continued until the general condition allowed a more aggressive form of treatment. Complete remission was achieved, and the patient is still in good health 48 months after diagnosis and 15 months after discontinuation of treatment. The favorable outcome in this child shows that prompt surgery is sometimes an essential step in the treatment of childhood leukemia.
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10/13. Thoracoabdominal radiculopathy.

    Thoracic nerve root dysfunction (TNRD) manifested as abdominal pain is an infrequently reported condition. We present data on six patients who had chronic intermittent thoracoabdominal pain originating in the back. Diabetes and osteoarthritis of the spine were the chief causes of these symptoms. The electromyogram in all patients showed changes consistent with an acute radiculopathy. All patients responded to anti-inflammatory therapy in combination with phenytoin, carbamazepine, amitriptyline, or local nerve block. TNRD is a condition that may be diagnosed earlier if clinical suspicion is increased, thus sparing patients excessive testing and surgery, and affording quicker relief.
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