1/7. adult Wilms' tumor associated with polycythemia--a case report.Both Wilms' tumor (nephroblastoma) in adults and polycythemia in Wilms' tumor are rare. Herein we report an extremely rare case of adult Wilms' tumor associated with polycythemia. A 41-year-old female was incidentally found to have right renal mass by abdominal sonography in a routine health examination. Laboratory examination revealed polycythemia (hemoglobin 20.2 g/dL). Although physical examination was unremarkable, CT scan revealed an homogeneous mass at the middle pole of right kidney, and chest x-ray revealed no metastatic lesions. Right radical nephrectomy was performed smoothly. Grossly, the tumor of 5 x 4.5 x 4.5 cm in size was well circumscribed, and had no vascular structure or collecting system involvement. Microscopic features were consistent with adult nephroblastoma. The post-operative course was uneventful. At follow-up, the patient was well, showed no evidence of recurrence and her hemoglobin level had returned to normal (hemoglobin 14.5 g/dl). We suggest that the relationship between polycythemia and Wilms' tumor should be carefully evaluated before surgical treatment.- - - - - - - - - - ranking = 1keywords = physical examination, physical (Clic here for more details about this article) |
2/7. Secondary polycythemia as a paraneoplastic syndrome of testicular seminoma.A 45-year-old man was referred to our hospital because of polycythemia. A physical examination revealed a large tumor in his scrotum enlarged to the size of 13 x 10 cm. A laboratory examination revealed severe erythrocytosis with a red blood cell count of 6,820 x 10(9)/L, a hemoglobin concentration of 21.2 g/dL, and a hematocrit of 59.8%. The total red cell volume was increased. A right radical orchidectomy was done with minimum bleeding, and he was diagnosed as having pure seminoma. After the operation, polycythemia improved spontaneously. polycythemia is a rare complication of seminoma and only two cases have been reported previously. The precise mechanism of polycythemia in our patient could not be clearly evaluated, but clinical course did indicate a close relationship between two distinct disorders.- - - - - - - - - - ranking = 1keywords = physical examination, physical (Clic here for more details about this article) |
3/7. Erythrocytosis due to bisphosphoglycerate mutase deficiency with concurrent glucose-6-phosphate dehydrogenase (G-6-PD) deficiency.A 28-year-old asymptomatic male of Iranian Jewish (Meshadi) heritage was found on routine exam to have an erythrocytosis (RBC = 6.22 x 10(12)/l, Hgb = 19.2 g/dl, Hct = 58.9%). splenomegaly was absent on physical exam. There was no family history of erythrocytosis. His oxygen dissociation curve was left-shifted with a p50 of 19 mmHg (normal = 25-32 mmHg). Hemoglobin electrophoresis showed no abnormalities. dna sequencing of the hemoglobin beta globin gene and both alpha globin genes did not reveal a mutation. A 2,3-bisphosphoglycerate (BPG) level was markedly decreased at 0.3 micromol/g Hb (normal = 11.4-19.4 micromol/g Hb). The patient's bisphosphoglycerate mutase (BPGM) enzyme activity was also markedly decreased at 0.16 IU/g Hb (normal = 4.13-5.43 IU/g Hb). A red cell enzyme panel revealed a markedly decreased G-6-PD level (0.3 U/g Hb, normal = 8.6-18.6 U/g Hb). His parents and a brother were also available for evaluation. Both parents showed normal 2,3-BPG levels but BPGM activity approximately 50% of normal. Paradoxically, the brother showed normal BPGM activity but a slightly decreased 2,3-BPG level. All family members had markedly decreased G-6-PD activity. dna sequencing of the BPGM gene showed the propositus to be homozygous for 185 G-->A, Arg 62 Gln in exon 2. Thus, the erythrocytosis in this patient is secondary to low 2,3-BPG levels, due to a deficiency in BPG mutase. This appears due to consanguinity within this family.- - - - - - - - - - ranking = 0.06900953420142keywords = physical (Clic here for more details about this article) |
4/7. polycythemia as the first manifestation of Cushing's disease.A 39-yr-old man presented to our hospital with unexplained erythrocytosis and hypertension. His follow-up for erythrocytosis had begun 2 yr earlier in another hospital and he had been diagnosed with polycythemia rubra vera. On admission to our hospital he was hypertensive (165/95 mmHg) and, except for the presence of moon-like face and facial plethora, his physical examination was normal. His hemoglobin concentration was 19.2 g/dl, and hematocrit was 58.9% with an increased red blood cell mass of 58 ml/kg as measured by radioisotope (Cr51). Blood film, other hematological indices except for elevated leukocyte alkaline phosphatase score, arterial gas analysis, and examination of aspirated bone marrow were all normal. An abdominal ultrasonography showed no evidence of splenomegaly. A diagnosis of probable secondary erythrocytosis was made. Early-morning serum cortisol and 24-h urinary free cortisol concentration as well as serum ACTH were high. serum cortisol was not suppressed by low-dose dexamethasone, but suppressed by high-dose dexamethasone. Pituitary magnetic resonance imaging showed no lesion. After inferior petrosal sinus sampling suggesting right-central ACTH secretion, the patient underwent transnasal-transsphenoidal pituitary adenomectomy. Both hypercortisolemia and erythrocytosis regressed completely after the adenomectomy. After the operation, the patient's hemoglobin concentration and hematocrit decreased steadily, and 1 month post-adenomectomy his hemoglobin is 14.9 g/dl and hematocrit 44.8%. Thus, Cushing's syndrome should be a routine part of evaluation of unexplained polycythemia.- - - - - - - - - - ranking = 1keywords = physical examination, physical (Clic here for more details about this article) |
5/7. Development of polycythaemia vera in a patient with myelofibrosis.In March 1981, a 53-year-old man presented with itching and was diagnosed as having myelofibrosis. There was gradual enlargement of the spleen over the following 5 yr. His spleen had to be removed in February 1986 because of physical discomfort. 3 months post-splenectomy he became polycythaemic. bone marrow examination was consistent with severe myelofibrosis. It was possible to demonstrate erythropoietin-independent BFU-E from peripheral blood, and ferrokinetic studies showed that erythropoiesis was localised to the liver with little bone marrow activity. Thus, despite severe marrow fibrosis, liver erythropoiesis was now polycythaemic, suggesting the coexistence of myelofibrosis and polycythaemia vera.- - - - - - - - - - ranking = 0.06900953420142keywords = physical (Clic here for more details about this article) |
6/7. "Stress" polycythaemia and peripheral facial palsy complications of severe hypertension.An 11-month-old boy had an episode of generalized convulsions followed by a right peripheral facial palsy, which resolved gradually within 3 weeks. Three months later he had another similar episode of convulsions followed by a left peripheral facial palsy. On both occasions it was found that he had polycythaemia. A careful physical examination discovered that the child had severe hypertension. Extensive laboratory investigations did not reveal a cause for his hypertension. Haematologic investigations showed that the polycythaemia was due to a contracted plasma volume as a result of the hypertension. The peripheral facial palsy most probably was due to a blood clot in the facial canal, below the origin of the nerve to m. stapedius, as audiograms were normal and lacrimation preserved. Control of the hypertension resulted in resolution of the facial palsy within 4 weeks and normal haematocrit readings within 6 weeks. It should be stressed that every patient with peripheral facial palsy should be examined for hypertension.- - - - - - - - - - ranking = 1keywords = physical examination, physical (Clic here for more details about this article) |
7/7. Polycythaemia and agoraphobia.Using single photon emission computerized tomography (SPECT), we demonstrated that in patient A, a 20-year-old male with polycythaemia, the cerebral blood flow was decreased (presumably as a result of increased viscosity and or microemboli), probably leading to frightening visual distortions (dysmorphopsia) associated with scintillating specks of bright colors (Teichopsia). This had presumably precipitated agoraphobia. After hospitalization, when the patient had not responded to efforts at systematic desensitization, he improved through a combination of multiple venesections and antiplatelet aggregation therapy (aspirin 75 mg o.d.) over 3 months combined with systematic desensitization. A subsequent SPECT demonstrated an increase in cerebral blood flow to normal levels, which coincided with improvement of agoraphobic symptoms and disappearance of visual distortions on further follow-up. This paper depicts another yet undocumented example of an alarming physical symptom probably leading to a cognitively-based panic sufficient to cause agoraphobia by classical conditioning. It also suggests that prior treatment of such physical symptoms is likely to facilitate the process of systematic desensitization.- - - - - - - - - - ranking = 0.13801906840284keywords = physical (Clic here for more details about this article) |