Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0036690 (sepsis)
 59,461 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Primary isolated IgM deficiency accounted for 0.1% of hospital admissions; secondary IgM deficiency for 2.0%. Although 19% were asymptomatic the rest of 89 subjects (4M:1F) suffered infection (60%), septicemia (36%), atopy (22%), splenomegaly (11%), neoplasia(7%) and autoimmune disorders (3%) with a mortality of 10%. Serious early treatment is needed to avert death from unopposed spread of organisms throughout the blood. Qualitative IgM deficiency (absence of isohemagglutinins) and delayed maturation of IgM can result in similar symptomatology.
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PMID:IgM deficiency. 5 91

A retrospective review of 19 patients with documented myeloid metaplasia undergoing, elective splenectomy during the past ten years at the Peter Bent Brigham Hospital is presented. The primary indications for splenectomy in 17 of these 19 were either hypersplenism or symptomatic splenomegaly. Eighteen of the 19 underwent both 59Fe-ferrokinetic studies and 51Cr-sequestration studies or, alternatively, 111In-marrow scintigraphy as a part of their routine preoperative evaluation. The death from sepsis of one patient six weeks post-operatively, whose marrow function was poor and whose level of splenic sequestration was minimal, confirms the efficacy of these studies in the preoperative prediction of hematologic response to splenectomy. Eighteen of the 19 patients benefited from the operation in terms of symptomatic relief and/or hematologic improvement, although surgery presumably did nothing to prolong survival in these patients. We conclude that splenectomy is indicated as a palliative maneuver for carefully selected patients with myeloid metaplasia without prohibitive operative risk, provided the criteria for selection of patients are adhered to and the surgeon and hematologist work together as a team.
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PMID:Splenectomy in myeloid metaplasia. 7 43

Three cases of malignant histiocytosis occurring in children aged 2 months, 10 months and 14 years, are described. In all children the diagnosis was based on anaemia, granulocytopenia or thrombocytopenia, splenomegaly and marked erythrophagocytosis by bone marrow and lymph node atypical histiocytes. Two children aged 10 months and 14 years, underwent splenectomy after which combined chemotherapy with cyclophosphamide, vincristine and prednisone (COP) was started. In the older child a complete remission was achieved. The younger child died soon after the onset of the treatment. The youngest child was treated with bleomycin, adriamycin, cyclophosphamide, vincristine and prednisone (BACOP). He died of pneumonia and sepsis two months after the start of the treatment.
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PMID:Malignant histiocytosis. Histiocytic medullary reticulosis. 7 57

This review is concerned with normal splenic function, mechanisms and consequences of splenomegaly, hypersplenism, the medical indications for splenectomy and the various aspects of hyposplenism. The potential probelm of lethal septicemia in hyposplenic or asplenic patients is also considered.
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PMID:Splenic function: normal, too much and too little. 37 97

Splenomegaly is usually the result of systemic disease. The differential diagnosis can logically be subdivided into infectious, hematologic, metabolic, vascular, and neoplastic diseases which result in abnormalities of the lymphoid, reticuloendothelial, or vascular components of the spleen. Splenic enlargement increases the risk of traumatic rupture of the spleen. Splenectomy, although indicated in some conditions, does not always relieve the hypersplenic state, and its benefit must be weighed against the hazard of life-threatening episodes of sepsis.
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PMID:Splenomegaly in children. Identifying the cause. 42 50

Twenty infants and young children with hereditary fructose intolerance (HFI) were admitted to hospital. None was diagnosed at admission. Referals were for vomiting of unknown aetiology (16X), pyloric stenosis or hiatus hernia (5X), toxic condition (3X), and hepatomegaly of unknown origin (5X). Feeding difficulties (20X), vomiting (18X), and failure to thrive (16X) were leading symptoms. The most frequent clinical findings were hepatomegaly (18X), pallor (14X), haemorrhages (13X). Ascites, oliguria, tachypnoea, fever, splenomegaly and rickets were less frequent. Laboratory findings were indicative of disturbed hepatic and renal tubular function and also of disturbed intermediary metabolism (hypokaliaemia, hypophosphataemia). However, hypoglycaemia was found in only 4 out of 15 patients tested. Differential diagnosis after hospital admission centered on metabolic disorders such as glycogenoses, galactosaemia, tyrosinosis, or Wilson's disease. Hepatitis, toxic hepatosis, liver tumour, intrauterine infection and sepsis were also considered. Eleven children had first ingested fructose within the first 6 weeks of life. The diagnosis was usually established only many weeks or months after first fructose intake and appearance of symptoms. This documents how difficult the diagnosis of this disease can be both in practice and in hospital. The course was severe in 11 children and lethal in 4. In only 5 patients was the course mild. The 16 survivors are doing well under fructose-exclusion diet. Irreversible visual impairment after intraocular haemorrhage occurred once. In each case HFI could have been suspected immediately, had a detailed nutritional history been taken. Practising paediatricians should know the composition of commonly used infant formulae. They should never prescribe sugared condensed milk for intractable vomiting prior to excluding HFI. Solution for intravenous infusion containing fructose and sorbitol are life-threatening for undiagnosed HFI patients.
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PMID:Hereditary fructose intolerance in early childhood: a major diagnostic challenge. Survey of 20 symptomatic cases. 73

Severe splenomegaly and anemia developed in a 5-year-old girl with diffuse lymphangiomatosis of the upper part of the body. Radioisotope scanning and celiac angiography demonstrated lymphangiomatosis of the spleen, a rare but diagnosable condition. Intractable infection in areas of ulcerated skin led to her death from overwhelming sepsis.
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PMID:Lymphangiomatosis with splenic involvement. 98 35

Portal vein thrombosis is a rare complication of ulcerative colitis and is invariably fatal. This report describes a patient with severe Crohn's disease who underwent elective surgery complicated by an anastomotic disruption with faecal peritonitis. Following emergency laparotomy he developed left hypochondrial pain which was a manifestation of splenomegaly consequent upon portal vein thrombosis. Anticoagulation was successful in preventing further spread of the thrombosis as monitored by colour Doppler ultrasound. Severe active disease, surgery and sepsis have been recognized as predisposing factors for thromboembolic complications in inflammatory bowel disease and this patient was exposed to all three. It is conceivable that portal vein thromboses occur more commonly than suspected and ultrasound scanning could ascertain the prevalence if performed prospectively.
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PMID:Portal vein thrombosis in a complicated case of Crohn's disease. 140 98

Wandering spleen is an unusual entity, occurring in both sexes and at any age, but is more frequent in women of reproductive age and in children. Wandering spleen is probably most often a result of congenital anomalies of development of the dorsal mesogastrium, but acquired factors may have a role in certain instances. Patients present most commonly with an asymptomatic mass, mass and subacute abdominal or gastrointestinal complaints or with acute abdominal findings. Clinical diagnosis can be difficult, but noninvasive imaging procedures, such as sonography, nuclear scintigraphy, computed tomography and magnetic resonance imaging are usually diagnostic. Laboratory tests are usually nonspecific, but may occasionally reveal evidence of hypersplenism or functional splenia. Symptoms may remain limited or absent for long periods of time, but complications related to torsion or compression of abdominal organs by the spleen or the pedicle are quite common. Splenomegaly is usually a result of torsion of the pedicle and splenic sequestration. Significant morbidity and mortality rates seem to be considerably less than described in 1933 and limited primarily to patients presenting initially with acute abdominal findings. Management recommendations have varied, but recognition of a significant risk of postsplenectomy sepsis supports a conservative approach. Patients with limited symptomatology may be medically managed until they exhibit worsening symptoms indicating progressive splenic torsion or gastrointestinal compression. Detorsion and splenopexy may be considered a reasonable surgical option even in patients presenting with acute abdomen, if there is no evidence of infarction, thrombosis or hypersplenism. Splenic preservation is especially recommended in extremely young patients who are at particular risk for postsplenectomy sepsis. However, it should be noted that follow-up evaluation data on splenopexy patients are notably lacking. Splenectomy is ideally reserved for patients presenting with acute abdomen and splenic infarction or thrombosis or with hypersplenism and patients in whom splenopexy is technically unfeasible. Subtotal splenectomy and splenic autotransplantation may be of limited value. Pneumococcal, Hemophilus and meningococcal vaccines are indicated before elective splenectomy and shortly after nonelective splenectomy. Antibiotic prophylaxis is recommended for those at particular risk. Prospective studies are unlikely, but extended follow-up information on patients already reported, particularly those managed expectantly or with conservative surgical measures, is needed.
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PMID:The wandering spleen. 141 97

A 33-year-old man was hospitalized because of thrombocytopenia and severe splenomegaly. On admission 78% of peripheral lymphoid cells were abnormally large, with pale cytoplasm. Flow cytometry of the abnormal lymphocytes showed that they expressed CD 2, CD 3, CD 11, CD 16, and CD 56, but not CD 4 nor CD 8, so they were T-cell large granular lymphocytes (T-LGL). Abnormal lymphocytes obtained from a lymph node expressed CD 2, CD 16, CD 38, and CD 56, but not CD 3, CD 4, and CD 8, so they were natural killer(NK) cells. Splenectomy was performed and the operative specimen showed diffuse infiltration of pleomorphic lymphocytes, probably chronic lymphocytic leukemia cells. After splenectomy, the platelet count returned to normal but the lymphocytosis continued. Two years after discharge, chemotherapy was done because of thrombocytopenia and hepatomegaly. The patient died of disseminated intravascular coagulation arising from sepsis. The differences and similarities between peripheral and lymph-node lymphocytes suggest that LGL and NK cells may be differentiated from the same kind of cell, somewhat differentiated from stem cells.
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PMID:[Chronic lymphocytic leukemia with peripheral T lymphocytes expressing CD 2+, CD 3+, CD 4-, CD 8-, CD 16+, and CD 56+ and lymph-node lymphocytes expressing CD 2+, CD 3-, CD 4-, CD 8-, CD 16+, CD 38+, and CD 56+]. 171 68


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