Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Calcified constructive pericarditis was discoverd in a 14 year old boy who presented with isolated hepatomegaly. No cause could be found. He was cured by surgery.
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PMID:[Calcified constrictive pericarditis manifested by isolated hepatomegaly]. 53 49

Two siblings with marked dwarfism, now 11 and 19 years of age, have been followed from infancy. The girl had frequent episodes of pneumonitis and presented at age 4 years with hepatic enlargement and ascites which proved to be due to constrictive pericarditis. The boy presented with growth failure and pseudohydrocephalus. He had fibrous dysplasia of the tibia and a pathologic fracture; acute hepatic congestion followed physical activity at age 13 years and led to the diagnosis of constrictive pericarditis. Muscle function was normal, there was no evidence for a primary liver disorder, and mental development was normal so that the coined word "mulibray" seemed inappropriate. Pericardiectomy produced only partial improvement; both patients have hepatic enlargement and continue to need diuretics. A third patient with dwarfism, frequent respiratory infections, and pericardial calcification has certain features of the syndrome.
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PMID:Constrictive pericarditis with dwarfism in two siblings (mulibrey nanism). 125 13

Symptomatic occlusion of hepatic veins, the Budd-Chiari syndrome (BCS), may be on the increase in women taking oral contraceptives. 17 liver scans in 7 patients with confirmed BCS over a 7-year period were studied. 6 of the 7 patients were women. When more than 1 vein is occluded BCS results, with hepatomegaly, abdominal pain, ascites, and hepatic histology showing centrizonal sinusoidal distention, hemorrhage, and necrosis. Mortality has been high. BCS is associated with polycythemia, oral contraceptive use, malignancy, trauma, and congenital abnormalities. The scintigram appearance with radiocolloid is usually characteristic but a similar appearance has rarely been reported in cirrhosis of the liver and in 1 case of contrictive pericarditis. Excessive uptake in the midline with markedly diminished activity at the periphery may be the 1st clue that BCS is present. Confusing conditions with incomplete BCS include partial hepatectomy, radiation injury, fortuitous segmental involvement by diffuse or focal liver disease, and rarely hepatic artery occlusion. Treatment is by the porto-caval shunting operation. Venous obstruction as shown venographically has had good correlation with liver scans. After the shunt procedure, hepatic artery flow to the affected lobes has increased as the pressure falls and underperfusion of the hypertorphied midline section. After 14 months, the midline area has shown no uptake, possibly because of atrophy. Radiocolloid uptake also appears in the ribs, spine, and lung. This uptake recedes when the liver function improves.
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PMID:Liver scan in Budd-Chiari syndrome. 126 39

A clinical case of idiopathic hypereosinophilic syndrome mimicking seronegative rheumatoid arthritis with a twenty year follow-up is reported. The patient showed other sign of the disease, such as pericarditis, gastroenteritis and hepatomegaly. Among the laboratory findings the elevated levels of aldolase and LDH 2, never reported previously, are stressed.
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PMID:An idiopathic hypereosinophilic syndrome mimicking seronegative rheumatoid arthritis: 20-year follow-up with clinical and laboratory findings. 155 Dec 84

Between 1980 and 1990 12 patients (5 male, 7 female) were operated on for acute infectious pericarditis at a mean age of 42 years. The infections were 6 bacterial (purulent 4, abscess 2), 4 tuberculous, 1 viral and 1 Candida. Pericarditis resulted from contiguous spread of infection from bilateral pneumonia in 3 patients, from subphrenic abscess in 2 and followed bacteremia in 1. Clinical signs were: tamponade/shock in 9, elevated jugular venous pressure in 11, edema in 6, hepatomegaly in 6, ascites in 1, and pericardial friction rub in 3. A preoperative pericardiocentesis in 9 patients allowed only 4 positive microbiological diagnoses and was an insufficient drainage in all cases. The preoperative mean NYHA class was 3.3. The pericardectomy was total in 9 patients and partial in 3. Total mortality was 1/12 patients (8%) with one late death due to recurrent tuberculous pericarditis. No patient with purulent pericarditis died. Another recurrence occurred 6 months after acute viral pericarditis. Atrial fibrillation in one patient was the only postoperative complication. After a mean follow-up period of 48.5 months no cardiac constriction had occurred in 11 surviving patients Actuarial survival after pericardectomy is 100% after 1 month and remains 91% after 5 years. The mean NYHA class has significantly improved to 1.2 (p less than 0.05) at the end of the follow-up. We conclude that pericardectomy combined with a specific antimicrobial therapy is a safe treatment for acute infectious and especially purulent pericarditis with low mortality and excellent longterm results. Early pericardectomy allows rapid decompression of the heart, removal of intrapericardial adhesions and infected tissue and prevents late constriction.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Pericardectomy and acute infectious pericarditis]. 173 22

Budd-Chiari syndrome is caused by the obstruction of the hepatic veins or of the inferior vena cava. It is characterized by the classic symptomatological triad: ascites, hepatomegaly, and abdominal pain. In 2/3 cases its etiology remains unknown. Budd-Chiari syndrome may be associated with polycythemia vera, neoplasms, chronic leukemia, congenital abnormalities, hypercoagulation conditions, pregnancy, oral contraceptives, and constrictive pericarditis. Even though its clinical diagnosis is difficult, radiology plays a decisive role with US, CT, MR imaging and, above all, angiography; the latter, together with liver biopsy, generally provides with an unquestionable diagnosis. Through the definition of stage of the disease, of level (intrahepatic, venous, caval, cardiac), of type (intrinsic or extrinsic), and degree of both obstruction and consequent development of collateral channels, radiology determines which patients should undergo a medical or a surgical treatment. In some case, percutaneous angioplasty can be performed. Four cases of Budd-Chiari syndrome, including two children, were investigated with US, CT, angiography, and liver biopsy; MR imaging was also employed in one case. The underlying cause was identified in 3 patients: constrictive pericarditis of probable congenital origin and web occlusion of the inferior vena cava near the right atrium in the 2 children; hepatic vein thrombosis due to essential thrombocythemia in the third case. In the fourth patient, thrombosis of the inferior vena cava and hepatic veins was unexplained. The diagnosis was established by means of liver biopsy and phlebography of the hepatic veins. Good diagnostic information was also supplied by non-invasive techniques, such as US, CT, and MR imaging.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Diagnostic imaging of Budd-Chiari syndrome in adults and children]. 218 5

A Salmonella typhimurium outbreak in 1-to-2-week-old broiler flocks in Mexico is reported. Clinical signs were growth retardation, blindness, twisted necks, and lameness. Gross lesions consisted of hypopyon, panophthalmitis, hepatomegaly with necrotic foci, enlarged spleen, pericarditis, coagulated and unabsorbed yolks, and purulent arthritis. Mortality and cull rates in different flocks ranged from 1.7% to 10.6% during the first two weeks of age. All internal organs, eyes, and hock joints of diseased chickens that were cultured were Salmonella-positive. The bacteria were also isolated from the breeder source flock. Disease was thought to be transmitted through eggs at hatch.
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PMID:Salmonella typhimurium outbreak in broiler chicken flocks in Mexico. 218 96

Vasculitis may accompany rheumatoid arthritis. One must distinguish between vascular involvement associated with the pathogenesis of rheumatoid arthritis, isolated digital vasculitis, and the syndrome of clinical rheumatoid vasculitis. The cause of clinical rheumatoid vasculitis is unknown. High titers of rheumatoid factor, cryoglobulins, diminished circulating complement, an increased prevalence of HLA-DR4, and the pathologic findings suggest an immune etiology. However, similar, but perhaps less pronounced, abnormalities occur in uncomplicated rheumatoid arthritis, and these findings are not universal in complicating vasculitis. Classic cutaneous clinical manifestations include ischemic ulcers, digital gangrene, and palpable purpura. Mononeuritis multiplex is another classic presentation of rheumatoid vasculitis. Small digital infarctions may accompany other manifestations in clinical vasculitis or may occur alone as isolated digital arteritis, in which case the prognosis is relatively favorable. Weight loss, pleuritis, pericarditis, ocular inflammation, splenomegaly, hepatomegaly, and Felty's syndrome have also been reported in association with rheumatoid vasculitis. Although renal involvement has been considered unusual in rheumatoid vasculitis, several studies suggest that this may be more common than previously recognized. Ideally, a biopsy or an angiogram confirms the diagnosis of rheumatoid vasculitis, but often the diagnosis rests upon the clinical picture. In general, blind biopsies are not helpful, although one series indicated that a blind rectal biopsy may be an exception to this rule. An elevated erythrocyte sedimentation rate, increased C-reactive protein level, anemia, thrombocytosis, hypoalbuminemia, and a positive rheumatoid factor are common laboratory findings. Leukocytosis, hypergammaglobinemia, leukocytopenia, an elevated creatinine level, and minimal abnormalities of the urinary sediment also occur in patients with rheumatoid vasculitis. However, these abnormalities overlap in patients with uncomplicated rheumatoid arthritis, and their role in distinguishing rheumatoid vasculitis from uncomplicated rheumatoid arthritis is limited. Other immunologic tests have no established clinical role in diagnosing rheumatoid vasculitis. Therapy depends upon the clinical manifestation of rheumatoid vasculitis. Uncomplicated rheumatoid arthritis deserves appropriate therapy, and general attention to nutrition, cessation of tobacco, and control of blood pressure are indicated for all patients. Isolated digital vasculitis generally requires no more than the usual treatment for uncomplicated rheumatoid arthritis. Appropriate dermatologic management is indicated for ischemic ulcers. Most clinical experience in managing more symptomatic rheumatoid vasculitis has focused on glucocorticosteroids, D-penicillamine, and cytotoxic immunosuppressive drugs.(ABSTRACT TRUNCATED AT 400 WORDS)
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PMID:Vasculitis associated with rheumatoid arthritis. 218 61

Since 1957, 30 patients with constrictive pericarditis have been treated surgically in Kyushu University Hospital. The surgical approaches were the left anterolateral thoracotomy in 17 patients (group I); the median sternotomy without cardiopulmonary bypass (CPB) in 6 patients (group II); and the median sternotomy with CPB in 7 patients (group III). The hospital mortality was 3.3 percent. The mean postoperative follow-up period was 11.7 years (longest 30 years). The actuarial survival rate at 5 years postoperatively was 88% in total cases (100% in group I, 82% in group II and 52% in group III), 88% at 10 years, 75% at 15 years and 67% at 20 years. Several patients in group III, who underwent complete pericardiectomy using CPB showed severe congestive heart failure and arrhythmia postoperatively. The comparative study between an poor result group (patients who died within 10 years post-operatively) and a good result group (patients who survived more than 10 years postoperatively revealed that preoperative hepatomegaly, atrial fibrillation and the interval between the onset of symptoms and the pericardiectomy influenced the survival rate significantly. These results suggested that pericardiectomy using CPB was a safe method for removing the calcified pericardium in the patient with severe constrictive pericarditis. However, a careful long term follow-up was necessary for the patient with severe myocardial damage even though the complete pericardiectomy was performed.
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PMID:[Surgical treatment of constrictive pericarditis]. 221 78

One year after the diagnosis of HIV infection, a 34-year-old man developed marked but painless swelling of the left parotid of uncertain cause. The swelling completely regressed under 60Co gamma radiation. Subsequently subfebrile temperatures were noted, together with nausea and back pain. Hepatomegaly with signs of biliary stasis occurred, LDH levels rose to 808 U/l, and pleural effusion and pericarditis with pericardial effusion occurred. Histological examination of inguinal lymph nodes revealed HIV-associated Burkitt's lymphoma in stage IVb. A full but short remission set in during a six-drug COP-BLAM treatment regimen. The patient died six months after the diagnosis had been made of rapidly spreading recurrence.
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PMID:[Burkitt's lymphoma in HIV infection]. 240 85


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