Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019214 (hepatosplenomegaly)
 4,408 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Eight bilharzial patients with hepatosplenomegaly who underwent splenectomy under ether anaesthesia were studied. The levels of iron, zinc, titanium, aluminium, calcium, magnesium and phosphorus were determined in the plasmas and RBCs both preoperatively and up to four days postoperatively. Preoperatively the bilharzial patients were found to have subnormal levels of the studied elements except aluminium in plasma. The effect of ether anaesthesia and surgery (splenectomy) was marked on the second postoperative day. All the elements displayed a tendency towards reaching preoperative levels by the fourth day except titanium which was still elevated. Metal ions particularly zinc and magnesium should be monitored in bilharzial patients in general and in the advanced cases in particular, who are undergoing major surgery. This should be done as a precautionary measure especially since these patients have subnormal metal ion levels.
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PMID:Some metal ion changes in plasma and erythrocytes following ether anaesthesia and surgery in bilharzial patients. 60 43

Although anemia has not been widely appreciated as a complication of primary hyperparathyroidism, 5.1% of the individuals with this disorder seen at the Massachusetts General Hospital since 1962 had a normochromic, normocytic anemia that could not be related to blood loss,a deficiency state, or uremia. The anemic group had more advanced bone disease and higher levels of serum calcium, alkaline phosphatase, and parathyroid hormone than the nonanemic group. Results of bone marrow biopsies performed in five patients showed variable degrees of myelofibrosis. However, none of the patients had hepatosplenomegaly, a myelophthisic peripheral blood smear, leukopenia, or thrombocytopenia. Removal of the abnormal parathyroid glands led to improvement or correction of the anemia.
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PMID:Anemia in primary hyperparathyroidism. 85 57

We have characterized CD4-CD8- double-negative (DN) alpha beta TCR+ T cells from a patient with immunodeficiency, lymphocytosis, lymphadenopathy, and hepatosplenomegaly. The majority of peripheral blood lymphocytes were DN alpha beta TCR+ T cells as evaluated by FACS and biochemical analysis. The DN T cells showed the following phenotype: alpha beta TCR+, gamma delta TCR-, CD2+, CD3+, CD4-, CD5+, CD7-, CD8-, CD16-, CD25-, CD26-, CD28+, CD45RO-, CD45RA+, CD57+, and HLA-DR+. Both southern blot analysis of TCR genes and FACS analysis applying a panel of V beta and V alpha monoclonal antibodies (MoAbs) indicated a polyclonal T-cell expansion. Thymic biopsy showed normal histology, whereas lymph node biopsy samples showed altered histological and immunohistological patterns with markedly expanded paracortical areas containing the DN T cells of the same phenotype as found in peripheral blood T cells. In functional studies, the DN T cells showed a profoundly reduced proliferative response upon stimulation with mitogens as well as MoAbs against the TCR/CD3 complex, CD2, and CD28, respectively. Addition of exogenous interleukin-2 (IL-2) only minimally augmented the proliferative response. In contrast, the addition of a combination of Ca2+ ionophore and phorbol 12-myristate 13-acetate (PMA) restored the proliferative response of the DN T cells to almost normal levels. This observation strongly suggests that the protein kinase C activity of the DN T cells was intact, but that the normal mechanism for transmembrane signal transduction was impaired in these unusual DN T cells.
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PMID:Phenotypical and functional characterization of double-negative (CD4-CD8-) alpha beta T-cell receptor positive cells from an immunodeficient patient. 183 26

46-year-old male patient was born in Niigata Prefecture and thereafter lived in Tokyo. In late January 1985, he noticed swelling of the bilateral inguinal lymph-nodes followed by fever and lumbago. In February, he consulted a local doctor and hepatosplenomegaly, marked leukocytosis and renal dysfunction were pointed out and he was referred to our hospital on February 22nd. The clinical laboratory data on admission were as follows; WBC 23,200/microliter, serum-Ca 18.4 mg/dl, BUN 85.3 mg/dl, creatinine 5.4 mg/dl, antibody to ATLV x160. ATL was diagnosed by biopsy of lymph nodes and examinations of peripheral blood and bone marrow hemogram. Remission was achieved in March by the treatment with adriacin. Renal failure and hypercalcemia also improved. However his respiratory dysfunction gradually worsened. The chest radiographies++ showed pulmonary edema, although there was no clinical evidence of heart failure. When his condition became stable, TBLB was performed and revealed extensive deposition of calcium along alveolar septae, suggesting that pulmonary edema was induced by the metastatic calcification of the lung. After the second treatment for ATL, he died of pneumonia. The autopsy showed calcium deposition not only in the lung but in pyramids of the kidney and in sub-serous layer of the small intestine. There was no tumor cell invasion into the bone or parathyroid gland. High urinary c-AMP together with normal levels of PTH suggested that the hypercalcemia in this case was induced by PTH-related protein. It was concluded that careful treatment for hypercalcemia is important as regards the occurrence of pulmonary edema.
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PMID:[An autopsy case of adult T-cell leukemia complicated with metastatic calcification of the lung]. 204 Dec 50

A 6-year-old girl with cerebral palsy developed conscious disturbance and generalized convulsion after one-hour hot herb drug bath. Physical examination on admission revealed rectal temperature 41 degrees C, hot skin, respiration 46/min, regular heart beat 98/min, BP 130/60 mmHg, Glascow coma scale 4 (E2M1V1), soft and flat abdomen, no hepatosplenomegaly, no skin rash, no focal neurological sign, increased generalized muscle ton. Laboratory data showed CBC: WBC 20400 cumm (Neutrophils 31%, Lymphocytes 69%), Hb 11.6gm%, ESR 11 mm/hr, arterial blood gas: PH 7.077, PO2 43mmHg, PCO2 57.1mmHg, HCO3- 16 mEq/L, BE-11.5mEq/L, serum sodium 143 mEq./L, potassium 5.2 mEq/L, chloride 101 mEq/L, free calcium ion 3.8mg%, GOT 63IU/L, GPT 263 IU/L, amylase 193 IU/L, alkaline phosphatase 388 IU/L, LDH 1245 IU/L, CPK 677 IU/L, total bilirubin 0.8 mg/dl, direct type 0.1 mg/dl, BUN 18 mg/dl, Glucose 35 mg/dl. Urinalysis revealed proteinuria( ) trace hematuria and pyuria, but no cast. Lumbar puncture is within normal limits. Bacteriology including blood and CSF are normal. Multiple organ failure was noted at that time. Intensive cooling methods were performed including central and peripheral cooling. We used luminal and valium to control the seizure. Condition didn't improve. Afterwards cardiopulmonary arrest developed. Patient expired 8 hours after admission despite of resuscitation. Heat stroke in infancy and childhood is different from that in adulthood. The predisposing factors are high ambient temperature, dehydration, very young baby, sweat gland dysfunction, or ectodermal dysplasia. Definition of heat stroke includes 1) rectal temperature above 41 degrees C, 2) behavioral change, 3) warm skin, wet or dry.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Status epilepticus induced by prolonged immersion in hot herb bath: report of one case]. 263 19

Plasma cell leukemia (PCL) can be considered the leukemic variant of multiple myeloma. The diagnosis is based on hematological features, including a plasmacytosis exceeding 2 x 10(9)/l and any evidence of a clonal plasma cell proliferation. There are two forms of PCL: the primary form occurring in individuals without preceding multiple myeloma, and the secondary form arising as a late manifestation in patients with multiple myeloma. From 1974 to 1988 we diagnosed 8 primary PCL cases out of a total 301 multiple myeloma cases (incidence, 2.6%) and a total of 847 acute leukemia cases (incidence, 0.9%). During the same period we observed in 7 multiple myeloma patients a terminal PCL, for an incidence of PCL in myeloma of 2.3%. Most clinical characteristics were similar in both types of plasma cell leukemia. In particular we found no difference in the average age and in the incidence of bone pain, hepatosplenomegaly, lytic bone lesions. None of our cases showed a clinically relevant lymphadenopathy either as presenting symptom or during the course of the disease. The values for hemoglobin, leukocytes, plasma cells, serum creatinine and calcium did not differ significantly between the two groups of patients. The median survival was 7 months for patients with primary PCL and 1 month for patients with secondary PCL. 5 of the 8 patients with primary PCL obtained a response to conventional myeloma therapy including single alkylating agents, with a duration ranging from 7 to 44 months. Only 1 of the patients with secondary PCL had a partial response after combination chemotherapy.
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PMID:Plasma cell leukemia: a report on 15 patients. 269 96

Three hundred twenty-five previously untreated patients with chronic lymphocytic leukemia were analyzed to identify significant prognostic factors for survival. Univariate analysis identified the following characteristics associated with survival: (1) clinical characteristics: age, race, sex, performance status, lymphadenopathy, and hepatosplenomegaly; (2) hematologic parameters: WBC count, absolute lymphocyte and granulocyte counts, hemoglobin level, and platelet count; and (3) biochemical parameters: serum albumin, calcium, uric acid, lactate dehydrogenase, alkaline phosphatase, BUN, and creatinine. Multivariate regression analysis in a randomly selected training subset of 217 patients demonstrated that the combination of uric acid, alkaline phosphatase, lactate dehydrogenase, external lymphadenopathy, and age had the strongest predictive relation to survival time. The resulting model was validated in the remaining independent subset of 108 patients and led to classification of patients into low, intermediate, and high-risk groups with five-year survival rates of 75%, 59%, and 14%, respectively, and with distinctively different annual mortality rates (P less than .01). Both the regression model and Rai staging were highly effective in identifying risk groups among the entire patient population (P less than 0.001). Overall the regression model was superior to Rai staging in defining prognostic risk groups. In addition, it was able to separate patients into significantly different risk categories within each Rai stage, thus improving on the prognostic prediction of individual patients with chronic lymphocytic leukemia.
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PMID:Prognosis of chronic lymphocytic leukemia: a multivariate regression analysis of 325 untreated patients. 381 21

A 36-year-old woman, presenting with fever, pancytopenia, hepatosplenomegaly, and striking effacement of the bone marrow by true malignant histiocytes, was found to have no benefit from the systemic administration of cyclophosphamide, vincristine sulfate, doxorubicin, prednisone, and high-dose methotrexate with calcium leucovorin rescue. Striking histologic and clinical improvement was noted after the administration of two cycles of etoposide and amsacrine, each cycle consisting of 100 mg/sq m/day of each agent for five days. We believe that this therapy should be considered for future patients demonstrating aggressive presentations of malignant histiocytosis.
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PMID:Malignant histiocytosis resistant to anthracycline. Response to intensive treatment with etoposide and amsacrine. 387 45

A 25-year-old woman with oxalosis and end-stage renal disease had pancytopenia and massive hepatosplenomegaly associated with extensive bone marrow deposition of calcium oxalate. A ferrokinetic study suggested profound reduction in erythrocyte production, and peripheral blood smears were compatible with myelophthisis and extramedullary hematopoiesis. Mechanical obliteration of bone marrow by calcium oxalate crystals may lead to pancytopenia and hepatosplenomegaly as late extrarenal complications of oxalosis.
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PMID:Pancytopenia and hepatosplenomegaly in oxalosis. 669 53

Congenital erythropoietic porphyria (CEP) is a rare disorder of heme biosynthesis. Skeletal abnormalities have been described in patients with this disease. We report a 25-year-old woman with osteodystrophy from CEP. On examination, mild hepatosplenomegaly, multiple hyperpigmented scars, hypertrichosis, erythrodontia and red coloration of urine were found. Biochemical studies showed increased serum levels of alkaline phosphatase, fasting and total 24-h urinary calcium excretion. Serum 250H vitamin-D concentration was low due to avoidance of sun exposure. Skeletal radiographs disclosed marked vertical and horizontal trabecular pattern and biconcavity of most of the dorsal and lumbar vertebral bodies. Several round sclerotic lesions (1-3 cm in diameter) were seen in the skull, pelvis and one lumbar vertebrae. The sclerotic lesions were augmented in size and number compared to X-rays obtained 8 years before. Bone mineral density (evaluated by DEXA) was markedly reduced at the spine and moderately diminished at the proximal femur and total skeleton. Treatment for 11 months with pamidronate (and the addition of hydrochlorotiazide for the last 6 months) reduced to normal values the serum levels of alkaline phosphatase and fasting urinary calcium. The 24-h urinary excretion of calcium and hydroxyproline were also decreased. The BMD increased in all the skeletal areas with presumably hyperactive bone marrow: spine, head, ribs and pelvis (and total skeleton), but did not change at the extremities and diminished at the femoral neck. Patients with CEP may present osteodystrophy characterized by sclerotic lesions and osteopenia, most likely due to accelerated bone turnover in areas of active bone marrow.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Congenital erythropoietic porphyria: skeletal manifestations and effect of pamidronate treatment. 802 43


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