Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0038002 (splenomegaly)
 9,873 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 51-year-old female with systemic lupus erythematosus (SLE) was admitted in November 1987 because of general fatigue and muscular weakness. She was treated with prednisolone (PSL) 30 mg and azathioprine (AZP) 50 mg after failure in the management of thrombocytopenia by PSL 15 mg. She exhibited no splenomegaly. Muscular atrophy and weakness were seen in the proximal muscles. Her platelet count was 44,000/microliters. A bone marrow aspiration revealed an increase in megakaryocytes. The blood chemistry revealed a normal CPK level and an elevated LDH level, indicating a presence of steroid myopathy. A splenectomy was performed after an increase of platelet count by giving gamma-globulin 400 mg/kg for 5 days. The platelet count rose to 368,000/microliters on the 46th postoperative day. She was treated with PSL 5 mg and AZP 50 mg as postsplenectomy therapy. The splenectomy did not adversely affect other aspects of SLE, in particular, renal function. She had no major complications in the postoperative period. Her platelet count reached a plateau 4 months later and revealed 115,000/microliters 18 months postoperatively.
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PMID:[Effect of splenectomy for management of thrombocytopenia associated with systemic lupus erythematosus: a case report]. 231 6

A 60-year-old man with primary splenic hemangiosarcoma (PSH) presented with weakness, weight loss, abdominal pain, and anemia. Physical examination revealed hepatomegaly, ascites, and firm, huge splenomegaly. Ultrasonography showed many nodular structures characterized by hypoechogenic and hyperechogenic areas. The patient also had portal hypertension, which was confirmed by physical findings and by measurement of portal vein pressure during operation. A liver-spleen scan using Tc-99m sulfur colloid and Tc-99m labeled heat denatured erythrocytes failed to demonstrate any splenic uptake, a reliable feature of functional asplenia. Although on a total body scan with Ga-67 citrate there was no splenic uptake, there was gallium uptake in the liver, where the presence of the metastatic lesion was histopathologically verified and confirmed by operation. There was also uptake in the middle zones of the lungs. Ga-67 citrate imaging appears to be helpful in the diagnosis of metastasis of PSH, and PSH can rarely cause portal hypertension.
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PMID:Functional asplenia and portal hypertension in a patient with primary splenic hemangiosarcoma. 234 Jun 75

A 37-year-old white woman presented with bilateral symmetrical ascending motor weakness. Subsequent evaluation revealed she was suffering from infectious mononucleosis. This case demonstrates that one should consider mononucleosis when presented with an idiopathic disease of the nervous system. The classic features of mononucleosis, such as pharyngitis, lymphadenopathy or splenomegaly, need not be present.
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PMID:The neurological sequelae of mononucleosis. 236 87

The proband, a 17-year-old boy, was admitted to our department because of the difficulty in standing on heel. Physical examination revealed a marked weakness and atrophy of bilateral lower legs, especially anterior tibial muscles. Patellar and Achilles tendon reflexes were abolished. Marked hepatomegaly and moderate splenomegaly were noted on abdominal echogram and CT scanning. Serum creatine kinase, lactate dehydrogenase, GOT and GPT were markedly increased. There were no abnormal findings in thyroid function, serum lipid analysis and serum lactate level after ischemic forearm exercise test. EMG of anterior tibial and calf muscles showed a mixture of myogenic and neurogenic patterns and biopsy specimen of calf muscle was compatible with a dystrophic change. Liver biopsy specimen revealed no noticeable change except a slight ballooning of hepatocytes in light microscopy. However, electron microscopic examination showed a marked increase of intracellular vesicles and enlarged smooth ER in which low-density, cotton-like materials were contained. In family study, both his father and paternal uncle were also affected with advanced scapuloperoneal-type myopathy associated with a marked elevation of serum creatine kinase and hepatomegaly. The disorder differs from Miyoshi's distal muscular dystrophy, which shows an early involvement of flexor muscles in lower extremities and is inherited as an autosomal-recessive trait. Although the etiology of hepatomegaly in this case remains to be elucidated, the special findings on electron microscopic study imply the possibility of some unknown metabolic disorder involving both muscle and liver. This disease seems to be a new type of scapuloperoneal-type myopathy, probably having an autosomal-dominant inheritance.
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PMID:[Familial scapuloperoneal-type myopathy associated with a marked elevation of serum creatine kinase and hepatomegaly]. 275 61

Sixty cats with hematologic abnormalities indicative of non-lymphoid hematopoietic neoplasia were classified into two groups, myelodysplastic syndromes (MDS) and acute myelogenous leukemias (AML), using criteria developed for human patients with similar diseases. Cats with myeloblast counts in bone marrow of less than 30% were classed as MDS and cats with myeloblast counts of 30% or greater were classed as AML. The clinical, laboratory, and postmortem findings in each group were described and compared. Clinical signs of disease were similar in both groups, the most common being inappetance, lethargy, and weakness. Non-regenerative anemia, macrocytosis, neutropenia, and thrombocytopenia were frequent hemogram abnormalities in both groups. Diagnostically useful differences in physical and peripheral blood findings were a higher prevalence of splenomegaly and/or hepatomegaly, thrombocytopenia, and severe anemia in the AML group. Circulating myeloblasts were found only in cats in the AML group. Outcome of disease was similar in both groups; 85% of the cats in each group died or were euthanatized within one week of diagnosis. In cats that were necropsied, extramedullary leukemic infiltrates were found in all cats in the AML group and in none of the cats in the MDS group.
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PMID:Non-lymphoid hematopoietic neoplasia in cats: a retrospective study of 60 cases. 282 80

This investigation was undertaken to study the clinical manifestations, development and course of the Norrbottnian type of Gaucher disease--a type III variant--with emphasis on central nervous system symptomatology and function, to correlate clinical signs with laboratory, neuropathological and biochemical findings, to evaluate effects of splenectomy on the course and severity of the disease and to investigate the effect of bone marrow transplantation. Clinical methods applied were neuropaediatric follow-up examinations, psychometric tests and motor age tests. Conventional neurophysiological, haematological and clinico-chemical methods were used. The investigation comprised 22 patients, 10 girls and 12 boys, in all of whom the clinical diagnosis was confirmed by enzymatic tests. The median age at diagnosis was 1.9 years. The clinical pattern at diagnosis was usually that of an alert child with normal intelligence, short stature, splenomegaly, a tendency to bleeding and ocular manifestations. The course was slowly progressive but varied considerably between patients. The median age at death in a representative group of patients was 11.8 years. Early motor development was delayed in the lower limbs but normal in the upper. Eight patients later developed ataxia and six patients signs of mild spastic paraparesis which usually appeared many years after splenectomy. IQ tended to decrease with age. Early splenectomy resulted in lower IQ scores than late splenectomy. With progression of the disease, EEG abnormalities became increasingly frequent, more markedly among splenectomized patients. Thirteen patients had abducens nerve weakness and ten had age dependent abnormalities of horizontal gaze. Retinal infiltrates were characteristic, mainly among splenectomized patients. At, autopsy, Gaucher cell accumulations were found in the adventitia of brain venules, most frequently in cerebral and cerebellar subcortical white matter. Intraneuronal storage of glucosylceramide was observed. The highest concentrations of glucosylceramide were in the cerebellum and cerebral subcortical white matter of splenectomized patients. The fatty acid composition of glucosylceramide from these regions indicated an extracerebral origin in splenectomized patients, but mainly cerebral in nonsplenectomized. Psychosine was found, the highest concentrations in cerebral and cerebellar cortex. Bone marrow transplantation was performed in a nine-year old girl. A three-year follow-up showed very encouraging results both biochemically and clinically. The Norrbottnian type of Gaucher disease is a well defined nosological entity with a characteristic course and clinical manifestations.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Gaucher disease--Norrbottnian type (III). Neuropaediatric and neurobiological aspects of clinical patterns and treatment. 329 44

Neurologic complications can occur during the course of brucellosis. In a 25-year-old patient admitted July 3, 1984 because of loss of weight (10 kg) brucellosis (Brucella melitensis) was diagnosed on the basis of splenomegaly, positive agglutination and positive blood and bone marrow cultures. Therapy with cotrimazol was performed for eight weeks. On July 31, 1984 the patient was readmitted because of weakness in both legs. Neurologic examination revealed slight weakness of the hip extensors and a marked weakness of the knee flectors and foot extensors without loss of sensation. The cerebrospinal fluid had a protein concentration of 3.6 g/l and a white blood cell content of 410/3 (39% granulocytes and 57% lymphocytes). Radiologic examinations ruled out spondylodiscitis. Meningoradiculitis was diagnosed in view of the inflammatory changes in the cerebrospinal fluid. After therapy with prednisone, rifampicin and doxicycline the neurologic alterations improved markedly. The clinical features and prevalence of this neurologic complication as reported in the literature are discussed.
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PMID:[Lumbosacral meningoradiculitis as a late manifestation of brucellosis (Brucella melitensis)]. 395 82

One splenectomized and 6 intact coyotes (Canis latrans), and 2 coydogs were experimentally inoculated with a recent isolate of Babesia gibsoni. The disease was mild in intact animals, was fatal in the splenectomized coyote, and was characterized by a regenerative hemolytic anemia with the PCV decreasing to 16% in intact animals and to 6% in the splenectomized coyote. Peak parasitemia ranged from 3% to 21% of erythrocytes infected and was inversely correlated to PCV. Serum lactate dehydrogenase, bilirubin, and globulin concentrations were increased in all infected animals. Three weeks after inoculation, specific antibody titers increased to 1:65,536 and remained elevated in the chronically infected animals. The splenectomized coyote had progressive weakness until death, 24 days after inoculation. Intact animals had splenomegaly and anorexia at the height of infection. The splenectomized coyote had generalized edema, omental petechiae, renal and hepatic degeneration, membrano-proliferative glomerulonephritis and congestion, extramedullary hematopoiesis, lymphoid hyperplasia, and severe hemosiderosis in an accessory spleen. The only consistent change in the intact animals was splenomegaly.
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PMID:Experimental babesiosis in coyotes and coydogs. 397 Apr 35

Two cases of Yersinia enterocolitica septicemia occurred in a breeding group of 22 adult patas monkeys (Erythrocebus patas). Affected animals had acute clinical signs of depression, weakness, dehydration, hypothermia, hepatomegaly and pronounced leukopenia. Both animals died a few hours after treatment was initiated. Gross necropsy findings included jaundice, fluid in body cavities, hepatomegaly, splenomegaly, multiple white foci within the liver and spleen, generalized lymph node enlargement and numerous mucosal ulcerations in the colon. Primary histopathological lesions were multifocal hepatic necrosis, splenic necrosis, chronic ulcerative enteritis and diaphragmatic myositis with necrosis and edema. Yersinia enterocolitica was cultured from the liver, spleen, lung, jejunum and rectum. Wild rodents, particularly mice, may have been a source of infection for these animals, as the monkeys were housed in a rural, indoor-outdoor facility. A preliminary culture survey showed that some clinically normal patas monkeys harbored the organism in their intestinal tracts.
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PMID:Naturally occurring Yersinia enterocolitica septicemia in patas monkeys (Erythrocebus patas). 405 42

Investigations of schistosomiasis mansoni in 218 Yemeni agricultural workers in the San Joaquin Valley of California revealed a prevalence of 56%. In those infected, quantitative egg counts performed by the Kato thick smear method revealed that 57% had light infections (1-100 eggs/g), 27% moderate infections (101-400 eggs/g), and 16% heavy infections (greater than 400 eggs/g; mean--918 eggs/g). The Yemeni had been migrating to the USA for the past 20 years, a period in which the prevalence of schistosomiasis had remained constant in the Yemen. The prevalence of schistosomiasis in those who had been away from Yemen for less than 5 years was 59% with a mean egg output of 236 eggs/g, but in those away for more than 5 years (up to 20 years) it was 32% with a mean egg output of 75% eggs/g. This is in spite of the fact that 75% of the latter had returned to Yemen for short visits. Statistical analysis by the Fisher's exact probability test revealed a significantly lower egg output in those away from Yemen more than 5 years. On the basis of these findings the mean life span of the Yemen strain of Schistosoma mansoni in man was estimated to be between 5 and 10 years. The presence of disease was assessed in this population by traditional medical means without prior knowledge of the status of the infection in the individuals examined. Under these circumstances, there were no differences in symptoms such as weakness, diarrhea and abdominal pain among the uninfected proportion of the population, total infected group and a small subgroup of those most heavily infected. None of the patients had hepatomegaly and only two had splenomegaly--one lightly and the other heavily infected. Because of the toxicity of antischistosomal drugs and the lack of treatment facilities only the 22 most heavily infected (greater than 200 eggs/g) of the 122 individuals with schistosomiasis mansoni were treated with niridazole. In addition the two individuals with splenomegaly were treated with antimony dimercaptosuccinate. Side effects, though common, were not severe. Although follow-up in this migrant population was poor the nine patients examined 3 to 7 months after treatment showed a mean decrease in egg output of 97%.
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PMID:Schistosomiasis mansoni in Yemeni in California: duration of infection, presence of disease, therapeutic management. 445 Dec 30


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