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)

We analyzed the efficacy of splenic irradiation in a population of patients with hematologic diseases. The records of the Radiation Oncology Division, Naval Medical Center San Diego were retrospectively reviewed for all patients treated with splenic irradiation (SI) between January 1, 1990 and March 1, 2001. The charts of 17 patients were identified: 5 patients had chronic myelogenous leukemia, 4 had chronic lymphocytic leukemia, 4 had idiopathic myelofibrosis, 2 had polycythemia vera, and 1 patient each had idiopathic thrombocytopenic purpura and acute myelogenous leukemia. Patient ages ranged from 37 to 88 years. Sixteen of 17 suffered from symptomatic splenomegaly. Twenty-six courses of splenic irradiation were delivered to these 17 patients. Treatment courses generally consisted of two fractions of 50 cGy in the first week, two fractions of 75 cGy the second week, and two fractions of 100 cGy the third week. Blood counts were checked prior to each treatment. Seven of the 17 patients died 1 month or less after SI due to the terminal nature of their disease. Twenty-two of 25 treatment courses for splenomegaly resulted in decreased pain and symptoms. Five patients required two treatment courses for splenomegaly, and one patient required five treatment courses. Three of four patients treated for thrombocytopenia demonstrated improvement, but only one was evaluable for more than 2 weeks due to disease-related mortality. Three of five patients treated for leukocytosis had significant improvement. In general, patients suffered few significant complications from this palliative intervention. Splenic irradiation can effectively palliate symptomatic splenomegaly in patients for whom splenectomy is not an option. Retreatment is possible. Splenic irradiation is less effective in the treatment of thrombocytopenia or leukocytosis.
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PMID:Palliative irradiation of the spleen. 1271 92

Two cases of Polycystic hydatid disease (PH) are reported from the same municipal district of the Brazilian Amazon region (Sena Madureira, Acre). Both had a similar clinical presentation and course over two years of follow-up. Initially the patients complained of pain in the right hypochondrium or upper abdomen and presented obstructive jaundice, fever, increased abdominal volume and weight loss. By image analysis, in addition to splenomegaly, multiple and coalescent cysts were detected in the liver. Serum samples were reactive by counterimmunoelectrophoresis. Treatment with albendazole resulted in partial improvement, with symptomatic relief and reduction in size of the lesions. This report stresses the importance of performing clinical-epidemiological studies of polycystic hydatid disease in the Brazilian Amazon and especially in the municipality of Sena Madureira where many other cases of PH may remain undiagnosed.
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PMID:[Polycystic hydatid disease: report of two cases from the city of Sena Madureira, Acre, in Brazilian Amazon]. 1271 68

A total of eighty-one consecutive cases of Kala-azar admitted in all four medicine units of Mymensingh Medical College Hospital during the period from January 2002 to mid August 2002 were included in this study. The number of the patients clearly indicates that the burden of Kala-azar in this region is significant and expanding, which constituted 1.90% of total admission in all 4 medicine units during this period. Majority of the patients were of 20-29 years of age. Male to female ratio was 1.38:1. Maximum number of the patients were of poor socio-economic group with history of housing made up of mud and having close proximity with cattle house. Fever and splenomegaly (100%) were the predominant features. Hepatomegaly was found in 91.36% of the cases. Other clinical manifestations were weight loss (79.01%), normal or increased appetite (65.43%), generalized weakness (72.84%), pallor (69.13%), cough (25.92%), jaundice (17.28%), abdominal Pain (12.34%), hyperpigmentation (9.88%), ascites (4.94%) and bleeding manifestations (4.94%). Notable concomitant illnesses were urinary tract infection (7.40%), pulmonary tuberculosis (3.70%), malaria (1.23%), scabies (4.94%), heart failure (3.70%) and chronic liver disease (2.47%). Due to wide diversity of clinical presentations, clinical features of kala-azar should be evaluated in details which will pave the hidden cases into light.
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PMID:Clinical profile of Kala-azar in adults: as seen in Mymensingh Medical College Hospital, Mymensingh, Bangladesh. 1271 42

We report the case of a young patient native of the Ivory Coast who suffered from homozygous hemoglobin C disease. He presented with the usual findings of this hemoglobinopathy: a moderate hemolytic anemia and a massive, painful and even disabling splenomegaly. Pain completely disappeared following splenectomy. However, postoperative course was complicated by portal venous thrombosis, which was medically treated. No deficiency of natural coagulation inhibitors could be demonstrated, so splenectomy was the only factor predisposing to thrombosis. We consider that in only very few cases of hemoglobin C disease, splenectomy (preceded by prophylactic antipneumococcic vaccine) may be indicated from pain and risk of spontaneous splenic rupture.
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PMID:[Hemoglobinopathy C and splenomegaly in an Ivory Coast patient. Value of splenectomy]. 1280 78

A 32-year-old man who had previously undergone chemotherapy for testicular seminoma 11 years ago was admitted to our hospital with a pain in the right leg. Computed tomography (CT) and bone scintigraphy revealed splenomegaly and multiple bone disease. Laboratory examination showed thrombocytopenia. The pathologic diagnosis of the resected spleen and the biopsied rib was angiosarcoma. We found no reports of angiosarcoma following previous chemotherapy for testicular cancer. It is unclear whether the angiosarcoma is a secondary neoplasm induced by the chemotherapy or not. However, the patient had a chromosomal aberration of the peripheral lymphocytes. The chemotherapy might also have affected the chromosomal aberration presumably in endothelial progenitor cells causing the development of a secondary neoplasm. To our knowledge, this is the first case of angiosarcoma after chemotherapy for testicular seminoma.
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PMID:[Angiosarcoma of the spleen 11 years after chemotherapy for testicular seminoma: a case report]. 1451 82

Hereditary spherocytosis is a congenital haemolytic anaemia due to defect in spectrin-a RBC membrane protein and is transmitted as autosomal dominant. Due to this defect there is presence of characteristic spherical cell in peripheral blood smear and osmotic fragility is increased. Haemolytic anaemia, reticulocytosis, jaundice and splenomegaly are present. This article reports a case of a 9 year old boy who presented with a history of prolonged jaundice since the age of 4 years and recurrent pain in the right upper quadrant of abdomen. Clinical examination revealed jaundice, enlarged liver and marked splenomegaly. Investigations confirmed the diagnosis of hereditary spherocytosis by the presence of spherocytes in blood smear, raised reticulocytes and increased osmotic fragility. The patient was subjected to splenectomy after vaccination against coccobacillus and was discharged after proper advice and on post splenectomy antibiotic prophylaxis.
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PMID:Hereditary spherocytosis. 1582 83

The torsion of spleen on its vascular shank represents an uncommon problem, responsible of acute and chronic pain. The mobile spleen is fixed only through hilus vessels the gastrosplenic ligament. The incidence is unknow, greater in the male with an M:F ratio 6.1 in the first ten years of life, even if an episode of intrauterine torsion has been reported. The diagnosis can be performed with ultrasonography, angiography, scintigraphy and CT scan. There are reported two cases: male of 2.5 years female of 14 years who presented with recurrent pain to the left side, vomit diarrhoea and fever. Objectively a palpable mass was present. Ultrasonography and angio-CT scan of abdomen revealed splenomegaly, ptosis of the spleen and malrotation with signs of obstruction of the vessels. The treatment in both cases was splenectomy. The spleen appeared rotated on its shank and increased of volume, deprived of anatomical structures of fixation. The histological report confirmed the haemorrhagic infarction. The excessive mobility of the spleen, from insufficiency or absence of the ligamentous attachments is case of abdominal pain or acute abdomen, that can complicate with the infarction of the spleen. Angio-CT scan, in the cases here reported, has shown to greater sensibility in comparison to the ultrasonography. The Authors believe that the video-laparoscopic splenopexy, when the diagnosis is made of "wandering spleen" with painful repeated episodes, can be finalized, to the preservation of organ.
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PMID:[Uncommon etiology of acute abdomen in pediatric age: the torsion of spleen]. 1584 92

Familial Mediterranean fever is the best known of the recurrent hereditary autoinflammatory diseases. It predominantly affects subjects of Mediterranean origin, Sephardic Jews in particular. Its gene, MEFV, is located on chromosome 16 and has autosomal recessive transmission, with incomplete penetration. It codes for a protein called pyrin or marenostrin, which is probably involved in the inflammatory process. In most cases, the first episodes appear before the age of 20 years and very rarely after the age of 40. Episodes usually last a few days, although they may extent over several weeks when localized in joints. Fever, occasionally pseudo-malaria, may accompany various symptoms, the most frequent of which are abdominal, articular, pleural or cutaneous. The abdomen is the classic site of this disease, and acute abdominal flares masquerade as abdominal emergencies. Musculoskeletal involvement is revealed by episodes of inflammation of the joints (more often mono- than oligoarthritis) and muscle pain. The flares are usually brief and totally reversible. Flares of thoracic pain corresponding to pleural inflammation and erysipelas-like skin eruptions have been observed. Acute symptoms disappear between flares, but hepatic splenomegaly, swollen lymph nodes or abnormal fundus of the eye may persist. Laboratory findings are typical of nonspecific inflammation, accompanied by moderate hyperleukocytosis during the flares.
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PMID:[Familial Mediterranean fever, clinical and laboratory findings]. 1614 53

A 17-year-old female with massive splenomegaly underwent partial splenic embolization (PSE) for hypersplenism. The postoperative course was characterized by recurrent painful splenic infarctions requiring hydromorphone PCA for an extended period resulting in an unanticipated, prolonged hospital stay. Massive splenomegaly treated with PSE may require an extended hospital stay to achieve pain control. Such patients may subsequently require transition to enteral opioids and weaning.
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PMID:Protracted pain following partial splenic embolization in an adolescent female with hypersplenism. 1623 68

Paracoccidioidomycosis (PCM) in children is rare, and its clinical progression is not clearly understood. The admission and post-admission clinical records of 38 children and teenagers aged up to 14 were studied who had been under treatment for 24-30 months. It is a consecutive case series, 17 retrospective and 21 prospective, from 1977 to 2000, admitted before and after 1990, respectively. The main clinical presentations were lymphatic, abdominal and cutaneous involvement, and fever. The alterations which disappeared more slowly were lymphadenomegaly, hepatomegaly, splenomegaly, and osteoarticular pain. Poor intestinal absorption, esophageal varices and splenic calcification were observed before treatment and persisted as sequelae. There was a satisfactory response to initial treatment in 56.7% of cases. Half the patients became asymptomatic in the ninth month of treatment, and 17+/-8% of cases presented with at least one symptom of the disease after 30 months of treatment. There were five deaths, and treatment failure was frequent and associated in part with the irregular use of antifungal. Treatment with ketoconazole was safe and effective. PCM is a serious systemic disease with slow evolution and high lethality, requiring treatment maintenance for a minimum of 24 months with careful and prolonged follow-up. Studies are necessary to evaluate the efficacy of different antifungals and the ideal treatment length for children with PCM.
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PMID:Clinical evolution of paracoccidioidomycosis in 38 children and teenagers. 1646 90


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