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Query: UMLS:C0000737 (abdominal pain)
31,184 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

An unusual case of tuberous sclerosis is presented in which splenomegaly and abdominal pain predominated. The clinical manifestations of the disease are discussed, and the generalized hamartonmatous nature of the diseases is emphasized.
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PMID:Hamartoma of the spleen: a manfestation of tuberous sclerosis. 116 74

The renal angiomyolipomas are unusual benign tumors which more than 50 per cent, are associated with tuberous sclerosis or intestinal linphangiomatosis. They can be discovered in a radiologic study by chance or to be associated with flank and abdominal pain and hematuria. The ultrasonographic imagen is characteristic and its management is conservative.
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PMID:[Renal angiomyolipoma in childhood: a clinical case]. 128 30

We describe the case of a previously healthy young woman admitted to hospital for abdominal pain and symptoms of shock. The preoperative diagnosis arrived at was polycystic kidney disease associated with ruptured angiomyolipoma. This combined pathology is pathognomonic of tuberous sclerosis; skin signs of the disease were also present in the patient, although undetected till then. The lack of neurological symptoms was an extraordinary finding. Cure is achieved at the cost of nephrectomy.
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PMID:[Angiomyolipoma and polycystic kidney secondary to Bourneville's tuberous sclerosis]. 219 91

Renal angiomyolipoma (AML) is an uncommon, benign neoplasm of a hamartomatous origin that presents as multiple bilateral lesions occasionally associated with tuberous sclerosis, or as a single unilateral lesion. They are generally asymptomatic, and can manifest the following clinical triad: abdominal pain, palpable mass and hematuria. Retroperitoneal hemorrhage is a rare complication (9%). We report 3 cases of spontaneous rupture of renal AML with massive hemorrhage; two of these cases were associated with tuberous sclerosis. This complication was the presenting feature of renal AML in two cases. Radiologic diagnosis and management of this tumor type are highlighted.
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PMID:[Retroperitoneal hemorrhage as a complication of renal angiomyolipoma]. 233 Nov 62

We report on a 25-year-old patient diagnosed as having Bourneville tuberous sclerosis with a giant angiomyolipoma 16 X 12 cm. in diameter, and two small angiomyolipomas in the left kidney, multiple asymptomatic angiomas in the right kidney and two 1 cm. diameter angiomas in the liver. The presenting features were intense left-sided abdominal pain of sudden onset, frank hematuria, acute anemic syndrome, nausea and vomiting. Patient work up included x-ray of chest, abdomen, and skull, bilateral renal arteriography and ultrasound were diagnostic of Bourneville tuberous sclerosis with multiple bilateral renal angiomyolipomas. The hematologic and hemodynamic compromise and the almost completely absent intact parenchyma in her left kidney did not permit a conservative surgical procedure and the patient was submitted to a left nephrectomy. The patient had the following characteristic features of Bourneville's disease: epilepsy, intracranial calcifications on CT, sebaceous adenomas on face, fibromas under nails of left hand and foot, bony lesions in the form osteosclerosis and chylous pleural effusion. Her intelligence level was normal and no optic nerve phakomas were observed. The literature on angiomyolipoma and Bourneville's tuberous sclerosis is reviewed. The importance of using ultrasound and CT in combination to diagnose and follow up renal angiomyolipomas is highlighted. Treatment of angiomyolipomas must be based on two parameters: symptoms and size. Symptomatic angiomyolipomas warrant angiographic work up and selective arterial embolization or the most conservative surgical procedure possible (enucleation, partial nephrectomy). Asymptomatic angiomyolipomas warrant CT and/or ultrasound examination every 6 or 12 months depending on size.
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PMID:[Bilateral angiomyolipomas of the kidney in Bourneville's tuberous sclerosis]. 268 46

A 40-year-old housewife was found to have a renal mass on her left side through an ultrasonogram at the hospital during a medical check up. The patient had a dull pain in her left flank intermittently for three years. She had no personal or family history of stigmas of tuberous sclerosis. The mass showed a high echogenicity on the ultrasonogram and a low density (-84HU) on the CT scan. Preoperative diagnosis was reported as renal angiomyolipoma and an enucleation of the tumor was performed with CUSA. A statistical study was done on data taken from 429 cases of renal angiomyolipoma in the Japanese literature, including our case. The male to female ratio was 1 to 2.9. Thirty three percent of the cases were associated with tuberous sclerosis. The ratio of bilateral cases to unilateral ones was 1:4. The main clinical signs were abdominal pain, abdominal mass and gross hematuria. Nephrectomy was done in 71.0% of the cases, partial nephrectomy and enucleation in 13.7% and embolization in 2.2%. Through the composition of the CT scan and the ultrasonogram, preoperative diagnosis has become possible in many cases, so recently the ratio of nephrectomy has been decreasing. We think partial nephrectomy and enucleation compose the most effective therapy for renal angiomyolipoma.
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PMID:[Angiomyolipoma of the kidney: a case report and a statistical study of 429 cases in Japan]. 269 39

A case is reported of spontaneous intraperitoneal rupture of a kidney in a patient with tuberous sclerosis who presented with left renal colic and left lower quadrant abdominal pain.
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PMID:Spontaneous intraperitoneal rupture of a kidney in a patient with tuberous sclerosis. 371 23

The author recently examined four children of clinical macrocephaly. Their occipitofrontal head circumference was exceeded 2 standard deviations above the mean for chronological age. Megalencephaly with normal ventricular system was proved by computerized tomography or pneumoencephalography. The purpose of this paper is to report four cases with various clinical aspects and to discuss the similarity of these patients. Additionally, previously reported syndromes with macrocephaly and multiple hemangiomas were presented in Table 4. Case 1 A 6 year-old was accidentally admitted because of infectious disease. His development was uneventful. Case 2. A 2 year-old boy was admitted because of delayed speech and delayed walking. He had no neonatal complications and no history of convulsions. Case 3. A 4 year-old boy was accidentally admitted because of abdominal pain. His physical examination exhibited diffuse hemangiomatous lesions on the right side of face, neck and chest, and congenital glaucoma. He was diagnosed as Sturge-Weber anomalad at the age of 2 years. Case 4. A 3 year-old girl was admitted because of distended abdomen. She had widely spread strawberry hemangioma on her right abdominal wall and blue and brownish phacomatosis on her back. GI tract examination showed lymphoid hyperplasia of the colon. Clinical profiles are presented in Table 3. These patients were different from Sotos syndrome (cerebral gigantism), but had the following similar findings besides megalencephaly-1) large birth weight (mean; 3961 g), 2) hypotonic and wasting muscles, 3) clumsy in walking and running, 4) no hereditary tendency. The Cases 1, 3 and 4 had normal mental development. The Case 4 was seemed as a previously undescribed clinical syndrome in which the principal features were megalencephaly, distended abdomen, hypotonic and wasting muscles, lymphoid hyperplasia of the colon, retroperitoneal cavernous hemangioma, and cutaneous hemangioma and neuroma. The relationship between cutaneous involvement and megalencephaly was unknown.
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PMID:[Megalencephaly: a report of 4 children including a previously undescribed congenital syndrome and review of the literature (author's transl)]. 616 78

This review summarizes the clinical and pathological findings of 52 cases of hepatic angiomyolipoma to discern and establish the most pertinent clinical and pathologic characteristics of the tumor. The disease was symptomatic in 60% of the patients. Abdominal pain or distress was the most common symptom, appearing in 37% of the patients, followed by malaise and upper abdominal mass or hepatomegaly. Of the 52 patients, only three (5.8%) showed associated tuberous sclerosis. Antemortem diagnosis of the tumor has been made with increasing frequency with the recent advent of computed tomography (CT) and ultrasound (US). The tumor was usually visualized as a hyperechoic mass by US imaging and as a low density mass less than -20 Housefield units by CT, and was hypervascular on angiography. The tumor was usually yellow to light tan, depending on the amount of fat tissue. Histologically, the tumor was characterized by an admixture of mature fat cells, blood vessels, and smooth muscle cells, with occasional foci of extramedullary hematopoiesis. The amount of smooth muscle component varied and often exhibited hypercellularity, pleomorphism with occasional bizarre giant cells, and moderate motitic activity. These features are considered conducive to an erroneous diagnosis of malignant tumor. However, since no malignant counterpart has been reported, it can easily be accurately differentiated histologically, if one is aware of the entity and can identify the three components of the tumor; blood vessels, smooth muscle cells, and fat. With regard to the histogenesis of angiomyolipoma, primitive mesenchymal cells around blood vessels may be the precursor cells.
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PMID:Angiomyolipoma of the liver: a collective review. 819 5

We report a case of a 21-year-old man with a right renal angiomyolipoma who has been observed for 20 months after successful treatment with selective transcatheter arterial embolization (TAE). In May 1993, he was transferred to our hospital for the treatment of an abdominal mass. Examination revealed a solid and fat-containing right renal tumor on computed tomography (CT) and magnetic resonance imaging (MRI), hypervascular staining with multiple peripheral microaneurysms on renal angiogram, indicating an angiomyolipoma (AML). He did not have any signs of tuberous sclerosis. Because he complained of abdominal pain and the tumor was 9 cm in diameter, TAE of the tumor using polyvinyl alcohol foam was performed to prevent life-threatening hemorrhage. Renal angiogram after TAE demonstrated a complete shut-down of blood supply to the tumor and CT scan, 6 and 20 months after TAE, demonstrated a decrease in size to 4.5 cm in diameter, showing a favorable long-term effect of treatment. He has been symptom-free for 20 months. TAE appears to be a safe and effective treatment for AML, preventing tumor growth and thus possibly hemorrhage or rupture of the tumor over the long -term.
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PMID:[Long-term effect of transcatheter arterial embolization therapy for renal angiomyolipoma]. 875 70


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