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Query: UMLS:C0002871 (anemia)
 52,094 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

The authors report about one case of bilateral renal angiomyolipoma, which is interesting because it is associated with tuberous sclerosis and was revealed by chronic anemia, and because of the treatment chosen, which consisted in the selective embolization of the neovessels. Follow-up at one year showed the reduction of the tumoral mass and the proper function of the remaining renal parenchyma.
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PMID:[Therapeutic embolization of a renal angiomyolipoma associated with tuberous sclerosis]. 143 Nov 85

Transfusion of homologous blood components is associated with immunological (incompatibility, alloimmunization, immunosuppression) and infectious risks (hepatitis, cytomegalovirus, HIV and other agents). Endoprosthetic surgery of the hip and knee frequently requires transfusion. Preoperative deposit of autologous blood can reduce homologous transfusion requirements. The simplest method is liquid storage of whole blood. In order to re-examine the efficiency of our present scheme of preoperative deposit, we studied patients scheduled for endoprosthetic surgery with respect to the amount of blood deposited, stimulation of erythropoiesis, and homologous blood requirements at the time of operation. PATIENTS AND METHODS. Sixty-seven consecutive patients (33 men, 34 women) scheduled for endoprosthetic replacement of hip or knee or for revision arthroplasty of the hip were studied. Patients with anemia, coagulopathies, coronary heart disease, severe obstructive or restrictive pulmonary disease, cerebral sclerosis, syncopes and seizures were excluded from preoperative deposit. Patients deposited 450 ml at weekly intervals, with occasionally slightly higher or lower volumes. A patient was temporarily deferred when the hemoglobin concentration prior to donation fell below 11 g/dl. Blood was collected in CPDA-1 buffer. The aim was a deposit of three units. In patients undergoing exchange reoperation of a total hip arthroplasty, intra- and postoperative autotransfusion with a cell separator was employed in addition to preoperative donation. RESULTS. The age of the patients ranged from 43 to 83 years (mean +/- SD: 61.2 +/- 9.1). The differences between men and women with respect to height (172.9 +/- 6.8 vs 160.6 +/- 7.4 cm; p less than or equal to 0.001), weight (75.7 +/- 11.2 vs 69.1 +/- 11.0 kg; p less than or equal to 0.05), calculated blood volume (p less than or equal to 0.001), and erythrocyte volume prior to donation (p less than or equal to 0.001) were significant. A total of 185 units was deposited. Men donated 1350 (450-1970) ml blood (median, range) and women 1260 (340-1450) ml (p less than or equal to 0.01). Hemoglobin concentrations decreased significantly from an average of 14.7 g/dl in men and 13.8 g/dl in women prior to donation to 13.4 g/dl and 12.3 g/dl preoperatively (p less than or equal to 0.0001 for both groups). The donation was not associated with serious complications. For 4 patients the scheduled operation was deferred for a longer term. Forty-six patients (23 men, 23 women) underwent total hip arthroplasty, 12 (4 men, 8 women) exchange of total hip arthroplasty, and 5 (3 men, 2 women) endoprosthetic knee surgery. In total hip arthroplasty men required 0 to 500 ml homologous packed red cells (median=0), women 0 to 1250 ml (median=0;p less than or equal to 0.05). Thirty-nine (69.6%) of the patients, 19 (82.6%) men and 13 (56.5%) women, did not require homologous transfusion.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:[Preoperative autologous blood deposit and liquid storage for replacement arthroplasty]. 258 31

An adolescent with anemia and weight loss was found to have bilateral renal cell carcinoma (hypernephroma). Further investigation revealed an underlying tuberous sclerosis that had escaped previous clinical detection. Several reports of this association were subsequently found when the world's literature was reviewed. Physicians treating patients with tuberous sclerosis should be aware of the possible development of these renal malignancies in their patients.
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PMID:Renal cell carcinoma in patients with tuberous sclerosis. 331 62

Small polydisperse circular DNA (spcDNA) in Fanconi anemia (FA) was analyzed from cultured fibroblast-like cells by electron microscopy. Application of the mica-press adsorption technique for the semi-quantitative determination of spcDNA amounts to three FA and three normal control skin-derived fibroblast strains revealed 85-fold increased levels of spcDNA in the FA cells. An even higher excess over controls was suggested when the FA fibroblasts were propagated for up to 11 serial in vitro passages, consistent with the short replicative life-span of primary FA cells and their rapid transition into a poorly dividing state, in which spcDNA reportedly further increases. In addition, contour length distributions of gradient-purified spcDNA preparations from five FA fibroblast strains were compared with those from five normal control strains. Mean spcDNA contour lengths were significantly greater in the FA than in the control cells. The reported findings of increased spcDNA amounts and sizes in FA coincide with a similar association of chromosome instability and abnormal spcDNA formation previously observed in cultured cells derived from angiofibromas in tuberous sclerosis. Circumstantial evidence from the present study in the paradigmatic chromosome breakage syndrome FA further supports the suggestion that a common mechanism underlies chromosome instability and the surplus generation of spcDNA. Notably, this apparent mechanism is functional in homonuclear primary cell strains with a distinct inherited basis of their chromosome instability, and is not restricted to heteroploid and neoplastoid cell lines.
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PMID:Increased amount and contour length distribution of small polydisperse circular DNA (spcDNA) in Fanconi anemia. 767 70

For daily clinical practice an isovolemic hemodilution down to an arterial O2 content of 10 ml/dl, corresponding to a hemoglobin content of 7.5 g/dl or a hematocrit value of 22.5%, is described as a tolerable value, as long as normovolemia and normoxia (no disturbances of lung function) are guaranteed and local restrictions in perfusion (coronary or cerebral sclerosis) are excluded. This value is not derived from the mixed venous O2 status but from the situation of the myocardium as the main limiting organ for anemic hypoxemia. Compensation of anemia is regulated hemodynamically: First, by an increase in stroke volume; secondary, by an increase in heart frequency and, tertiary, by an increase in venous utilization. The last may reach 100% without any restrictions from the so-called critical mixed venous pO2 as a possible limiting factor for hemodilution.
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PMID:[Critical limits of hemodilution: theoretical principles]. 769 Jun 62

Symptomatic uterine lymphangioleiomyomatosis (LAM) simulating high-stage uterine sarcoma in a patient with tuberous sclerosis complex is reported. A 49-year-old female presented with abdominal pain and anemia. Preoperative workup revealed a uterine mass and a large amount of peritoneal free fluid and possible metastatic implant along the lateral edge of the liver. The patient also had a large right pleural effusion. A fungating anterior uterine fundal mass with apparent perforation and intraabdominal hemorrhage was found on laparotomy. A portion of the mass was excised and initially interpreted as an endometrial stromal sarcoma. Microscopic examination revealed multiple vascular epithelioid smooth muscle proliferations in the uterus and serosal surface of the fallopian tube and periaortic lymph node lymphangioleiomyomas. The uterine, fallopian tube, and nodal lesions were positive for smooth muscle actin, desmin, and HMB-45, findings characteristic of LAM. Additional examination of the patient revealed stigmata of tuberous sclerosis complex. Although uterine LAM is uncommon, it may be associated with pelvic and/or abdominal symptoms and may simulate a primary uterine mesenchymal neoplasm.
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PMID:Lymphangioleiomyomatosis of the uterus simulating high-stage endometrial stromal sarcoma. 894 80

Tuberous sclerosis is a hereditary autosomal-dominant disease characterized by hamartomas that can develop in any organ. We report herein the case of a 34-year-old female with tuberous sclerosis and a huge abdominopelvic mass that started growing quickly 2 years after its diagnosis. The patient had undergone several previous operations for hydrocephalus and cerebral tubers, and a nephrectomy for right renal angiomyolipoma. On admission, she was in poor general health with renal failure, severe anemia, and weight loss. A laparotomy revealed that the tumor occupied the pelvis, the lower and part of the upper abdomen, and was hypervascularized, with an extremely irregular surface covered in nodules, vegetations, and areas of hemorrhagic necrosis. The development of the mass and the impossibility of recognizing the internal genital organs led us to assume that the formation had originated from these. Frozen-section examination indicated an undifferentiated tumor that had not been completely resected. Her postoperative course was complicated by bronchopneumonia and progressive renal failure. The patient died 10 days after surgery due to cardiorespiratory failure. A histological diagnosis of epithelioid angiomyolipoma was confirmed. Although it is presently impossible to determine whether angiomyolipoma with predominant epithelioid cells is more aggressive than typical angiomyolipoma, it definitively demonstrated local aggressive behavior in this patient.
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PMID:Giant abdominopelvic epithelioid angiomyolipoma associated with tuberous sclerosis: report of a case. 1055 39

We report a 26 years old male with a tuberous sclerosis with multiple and bilateral kidney cysts and angiomyolipomas. The patient presented to the emergency room with a severe abdominal pain and anemia, secondary to a bleeding angiomyolipoma. The patient rejected blood transfusions due to his religious beliefs. A selective angiography was performed confirming diagnosis and the lesion artery was selectively embolized, stopping the bleeding immediately. The patient had a satisfactory evolution thereafter. This is a rare lesion and the fact that the patient was a Jehovah witness that rejected blood transfusions, required an innovative medical approach.
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PMID:[Hemorrhagic tuberous sclerosis. Report of a Jehovah Witness patient]. 1083 24

A 78 year-old woman, suffering from a von Recklinghausen's disease sought medical assistance for hematemesis with anemia. This patient had previously experienced an amputation of the right arm for gangrene. Gastric fibroscopy unveiled a deep chronic ulcus developed in the antrum, highly suspect of malignancy. Multiple biopsies of the ulcer showed mainly interstitial gastritis. The persistence of the hematemesis imposed a subtotal gastrectomy. Pathological examination of the operative specimen evidenced an ischemic ulcer caused by arterial intimal muscular fibrodysplasia with associated neurofibromatosis in the neighboring sub-mucosal layer. This case report highlights the frequent association of phacomatosis especially von Recklinghausen's disease, with vascular lesions whose clinical expression mainly depends on the involved vascular area.
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PMID:[Intimal muscular fibrodysplasia responsible for an ischemic gastric ulcer in a patient with a von Recklinghausen's disease: a case report]. 1124 May 32

A 46-year-old woman with edema and pancytopenia was referred for further evaluation. She was diagnosed as tuberous sclerosis with clinical manifestations such as facial adenoma sebaceous, ungual and periungual fibroma, subependymal nodules and renal angiomyolipoma. Her edema seemed due to hypercardiac function induced by massive anemia. X-ray revealed extraordinary thickening of the cortex of long bones of the extremities as well as patchy osteosclerotic findings in vertebra, suggesting that hematopoietic space was significantly reduced. Pancytopenia improved after splenectomy. Histological examination revealed several intrasplenic hemangiomas but its relationship to hypersplenism was not clear. It seemed that her massive pancytopenia was induced by a combination of hypersplenism and significant reduction in hematopoetic space. In tuberous sclerosis, various systemic complications sometimes induce severe hematological abnormalities. According to previous literatures, the present case of tuberous sclerosis manifested the most outstanding hematological complications.
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PMID:Pancytopenia in tuberous sclerosis. 1138 23


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