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Fetal haemoglobin in homozygous sickle cell disease.

Synthesis of fetal haemoglobin is prolonged in homozygous sickle cell disease. Its intracellular distribution is irregular and red cells containing high levels of Hb F enjoy greater protection from sickling and subsequent destruction. This relationship is reflected in a number of associations between Hb F level and the haematological and clinical parameters of the disease. High fetal haemoglobin levels are associated with more normal red cell survival, more normal oxygen affinity, and more normal red cell characteristics whereas clinically they are associated with less evidence of vessel obstruction, persistence of splenomegaly, more normal skeletal development and body habitus, and a generally more benign clinical course. Attempts to prolong Hb F synthesis might therefore be expected to lead to amelioration of the clinical features of the disease. The factors leading to persistence of Hb F synthesis is SS disease are largely unknown although they appear to be operative as early as the first three months of life.

Adolescent↗

[Torsion of the spleen--an unusual cause of sudden acute abdomen].

Torsion of a wandering spleen is rare and has been diagnosed in about 0.2%-0.3% of a large group of patients who required splenectomy. Abnormalities in the ligamentous structures that fix the spleen are thought to be responsible for its abnormal position and for its torsion. Patients may present with various symptoms ranging from mild intermittent abdominal pain to an acute abdomen. Computed tomography leads to correct diagnosis.

Abdomen, Acute↗

Spontaneous rupture of the spleen.

A 40-year-old woman with bronchial asthma was admitted as a medical emergency with suspected myocardial infarction. Spontaneous splenic rupture was diagnosed during hospitalization and splenectomy successfully performed. No histopathologic changes or signs of previous injury or adhesions were found.

Adult↗

Wandering spleen.

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Abdominal Pain↗

[Widespread embolism in tricuspid and mitral endocarditis caused by chronic lymphangitis. Case report].

Infective endocarditis, a serious microbial infection of the cardiac endothelial surface, may involve any heart valve. However, right-sided endocarditis is uncommon in non-intravenous drug abusers without underlying heart disease, and the contextual involvement of the left-sided valve is exceptional. A 63-year-old man with no evidence of intravenous drug abuse or heart disease, presented with persistent fever, worsening of breath, and aphasia. His medical history was notable for mild arterial hypertension and serious lymphangitis with cutaneous erosions on the legs. Transesophageal echocardiography was performed for suspicious endocarditis and showed a pedunculated and highly mobile vegetation adhered to the atrial portion of the posterior leaflet of the mitral valve, protruding into the left ventricle through the valvar orifice. Another large vegetation was seen at the tricuspid valve surface and protruded into the right ventricle during diastole. Cerebral and thoraco-abdominal computed tomography scan revealed multiple embolism to the left kidney, spleen, lungs and central nervous system. Blood cultures identified Staphylococcus aureus. The only risk factor was large skin sepsis. Despite successful antibiotic therapy, the patient died for development of renal and respiratory failure.

Chronic Disease↗

Splenic syndrome at mountain altitudes in sickle cell trait. Its occurrence in nonblack persons.

Six consecutive cases of splenic syndrome at mountain altitudes in persons with sickle cell trait are reported and the literature is reviewed. All six cases occurred in men who experienced the acute onset of severe left-upper-quadrant abdominal pain within 48 hours of arrival in Colorado from lower altitudes. All six patients were phenotypically nonblack. Three patients experienced their symptoms at moderate altitudes of 1,609 to 2,134 m (5,280 to 7,000 ft) above sea level. All recovered with medical management and none required splenectomy, although functional hyposplenia was a sequela in at least one patient. The possibility that nonblack persons with sickle cell trait may be at greater risk than black persons with sickle cell trait for the development of splenic syndrome at moderate altitude is discussed.

Adolescent↗