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)

A 40-year-old woman was admitted because of increasing exertional dyspnea. Right heart failure was suggested by the presence of hepatomegaly, pretibial edema and also echocardiographic findings. Physical examination and echocardiography showed no evidence of valvular disease or congenital heart disease except for right ventricular dilatation and tricuspid regurgitation. The ventricular septum deviated toward the left ventricle throughout the cardiac cycle, but left ventricular function was preserved. Severe pulmonary hypertension averaging 44 mmHg was revealed by cardiac catheterization. Digital subtraction angiography and pulmonary blood flow scintigraphy showed no evidence of pulmonary artery embolism, and no interstitial pulmonary lesions that might have caused pulmonary hypertension were recognized. Hypergammaglobulinemia suggested an autoimmune disorder, and signs of systemic lupus erythematosus (SLE), such as pleural effusion, proteinuria, lymphocytopenia, LE cell phenomenon and antinuclear antibodies were present. Several autoimmune diseases are known to be causative factors of pulmonary hypertension. However, only ten cases of SLE complicated by pulmonary hypertension have been reported the present one. These cases were characterized by a high incidence of Raynaud's phenomenon and positivity for anti-RNP antibody. In our present case, SLE activity was suppressed using prednisolone, but pulmonary hypertension persisted and the patient eventually died due to right cardiac failure. Judging from the clinical course of the ten reported cases of SLE-pulmonary hypertension, there seems to be no hope of improving the pulmonary hypertension once it has become established. Therefore it is important to detect and cure pulmonary hypertension as early as possible.
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PMID:[A case of lupus erythematosus preceded by right heart failure due to pulmonary hypertension]. 174 69

Heart-lung transplantation for treatment of end-stage cardiopulmonary disease continues to be plagued by many problems. Three primary ones are the technical difficulties that can be encountered, particularly in those patients who have undergone previous cardiac operations, the additional restriction on donor availability imposed by the lack of satisfactory preservation techniques, and the need for lung size compatibility. Two of these difficulties and others surfaced postoperatively in a heart-lung transplant recipient who presented a series of unique operative and therapeutic challenges. A 42-year-old woman with chronic pulmonary hypertension and previous atrial septal defect repair underwent a heart-lung transplantation in August 1985. The operative procedure was expectedly complicated by bleeding from extensive mediastinal adhesions from the previous sternotomy and bronchial collateralization. Excessive chest tube drainage postoperatively necessitated reoperation to control bleeding from a right bronchial artery tributary. Phrenic nerve paresis, hepatomegaly, and marked abdominal distention caused persistent atelectasis and eventual right lower lobe collapse. Arteriovenous shunting and low oxygen saturation necessitated right lower lobectomy 15 days after transplantation, believed to be the first use of this procedure in a heart-lung graft recipient. Although oxygenation improved dramatically, continued ventilatory support led to tracheostomy. An intensive, psychologically oriented physical therapy program was initiated to access and retrain intercostal and accessory muscles. The tracheostomy cannula was removed after 43 days and gradual weaning from supplemental oxygen was accomplished. During this protracted recovery period, an episode of rejection was also encountered and successfully managed with steroid therapy. The patient continued to progress satisfactorily and was discharged 83 days after transplantation. She is well and active 20 months after discharge.
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PMID:Postoperative complications necessitating right lower lobectomy in a heart-lung transplant recipient with previous sternotomy. 311 65

Although myasthenia gravis (MG) is frequently mentioned in standard textbooks and journal articles as a rare cause for pulmonary hypertension and right heart failure, no case can actually be found in the literature. The case described in this report is the first documented case of chronically decompensated MG manifesting itself as pulmonary hypertension, severe right heart failure, and functional prolapse of both the mitral and tricuspid valves. Interestingly, no hepatic biochemical abnormalities were present in spite of significant congestive hepatomegaly.
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PMID:Rare cardiopulmonary complications of chronically decompensated myasthenia gravis. 742 Apr 43

We report the case of a young man first seen by is in 1989 at the age of 20 years. The diagnoses of hypertrophic cardiomyopathy, Wolf-parkinson-White syndrome, congestive heart failure and pulmonary hypertension were made. One month later the patient had jaundice and hepatomegaly and a diagnosis of acute viral hepatitis A was established by laboratory findings. The ALT and AST levels were persistently elevated, seven times the normal mean, during six years. Two liver biopsies in 1991 and 1993 showed liver injury secondary to congestive heart failure (CHF) as the only abnormality. This case illustrates the importance of liver injury secondary to CHF as a cause of a marked and persistent increase of ALT and AST that resembles that of other liver diseases.
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PMID:[A case of elevated transaminases caused by congestive heart failure simulating chronic hepatitis]. 938 Sep 78

We examined the clinical characteristics of six patients with myelofibrosis secondary to myeloproliferative diseases whose clinical courses were complicated by pulmonary hypertension to determine possible causal links between the two disorders. Six patients (four male, two female), with diagnoses of myeloproliferative disease, myelofibrosis (one with polycythemia vera, three with agnogenic myeloid metaplasia, one with unclassified myeloproliferative syndrome, one with essential thrombocytosis), and pulmonary hypertension are presented. Measurement of the pulmonary artery pressure was performed by Doppler echocardiography in all patients and by right sided heart catheterization in four patients. The range of resting pulmonary artery systolic pressure was 35 to 47 mmHg above the mean right atrium by echocardiography. One patient had autopsy evidence of pulmonary myeloid metaplasia and interstitial fibrosis; another had acute leukemic infiltration of the lung parenchyma. All patients had thrombocytosis; symptomatology in one patient with marked thrombocytosis improved with plateletpheresis. Two patients suffered systemic thrombosis. All patients had severe hepatomegaly. Two patients had evidence of left ventricular dysfunction. The interval between the development of dyspnea and death was less than seven months in five of the patients. A causal link between pulmonary hypertension and myelofibrosis secondary to myeloproliferative diseases is suggested for each patient. Hematopoietic infiltration of the pulmonary parenchyma, portal hypertension, thrombocytosis, hypercoagulability, and left ventricular failure may account in part for the development of pulmonary hypertension in these patients. Patients with myelofibrosis and dyspnea should have Doppler echocardiography to evaluate pulmonary artery pressures.
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PMID:Pulmonary hypertension in patients with myelofibrosis secondary to myeloproliferative diseases. 992 5

The purpose of the present study was to evaluate the effectiveness of fish oil in preventing tissue pathologies associated with monocrotaline (MCT) toxicity. Twenty-four weanling rats were randomly assigned to one of two groups: (1) 12 to a group fed a diet containing 15% (w/w) corn oil (control) and (2) 12 to a group fed a diet containing fish oil (13%) and corn oil (2%) as the source of fat. Rats were fed for 4 weeks prior to MCT treatment. Six rats in each group were subcutaneously injected with MCT and six injected with its vehicle (water) and all were continued on their respective diets. All rats were sacrificed 3 weeks after injection. In rats receiving MCT, we observed severe interstitial pneumonia, septal fibrosis, vasculitis with virtual obliteration of the lumen of the small arteries and arterioles, right ventricular hypertrophy (RVH), and hepatomegaly and hepatocyte vacuole formation. Dietary fish oil significantly reduced septal fibrosis and development of pneumonia. There was a slight, but statistically insignificant decrease in vasculitis and fish oil did not prevent RVH (pulmonary hypertension). In addition, fish oil effectively protected the MCT-treated rats from development of hepatocyte vacuoles (steatosis), hepatic inflammation and vasculitis, increased presence of fibroblasts and collagen deposition in the centrilobular and, to a lesser extent, in the periportal spaces. These results suggest that lung parenchymal inflammation can be attenuated without altering the course of development of pulmonary hypertension in the MCT model. These results also indicate that fish oil protects against inflammation and fibrosis in the lung and liver, and against hepatocyte vacuole formation in MCT-treated rats.
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PMID:Dietary fish oil protects against lung and liver inflammation and fibrosis in monocrotaline treated rats. 1204 31

Management of severe tricuspid regurgitation late after left heart valve operation is controversial. There has been reluctance to the operation due to the high risk of repeat operation, coexistent right ventricular dysfunction and pulmonary hypertension. We investigated 6 cases of isolated tricuspid valve surgery (tricuspid valve replacement: 2, tricuspid valve plasty: 4) late after left heart valve operation. Before operation, these patients showed poor general condition (4 cases were NYHA III or IV) but good left ventricular function [mean ejection fraction (EF) 66 +/- 9.6%]. Hospital mortality was 0% and the mean mid-term actual event-free survival over 2 +/- 1.8 years was 80%. Most of patients demonstrated NYHA I and improvement of hepatomegaly after operation. There were many complications in 2 cases that had been performed previous operation more than 20 years before. In summary, the patient who complained symptoms due to right heart ventricular failure and showed good left ventricular function after left heart valve surgery should be considered to undergo tricuspid valve operation before the occurrence of other complications.
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PMID:[A study of isolated tricuspid valve surgery late after left heart valve operation]. 1205 60

A 59-year-old man had undergone aortic and mitral valve replacement (DVR) for rheumatic aortic and mitral valve stenosis 15 years ago. At that time, echocardiography did not detect tricuspid regurgitation (TR), and catheterization data showed right atrial pressure v wave of 8 mmHg and pulmonary artery pressure of 27/12 (17) mmHg. One year after DVR, hepatomegaly and jugular venous dilatation appeared, and after 5 years edema of both legs became apparent. After 7 years, chest X-ray showed an increase of cardio-thoracic ratio, and for the first time, echocardiography detected mild TR. Fifteen years after DVR, severe general fatigue, shortness of breath and hepatomegaly could not be controlled with medication. Catheterization data showed right atrial pressure v wave of 23 mmHg and pulmonary artery pressure of 28/13 (17) mmHg. Right ventriculography showed progression of severe TR. Tricuspid valve replacement (TVR) was performed using a St. Jude Medical 31 M mechanical valve under natural cooling and heart beating. The tricuspid valve was only slightly thickened and no subvalvular abnormalities were seen other than a severely dilated tricuspid annulus. Postoperative course was uneventful and he was discharged 44 days after the TVR. He is currently doing well 6 years after the TVR. All terms, he did not have pulmonary hypertension or left-side heart problems. We suspect that the cause of TR was not secondary, and was included in the category of isolated TR. If the left heart is completely treated, as in this case, it is important to follow-up for signs of right heart failure, before TR is detected.
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PMID:[Severe tricuspid regurgitation late after aortic and mitral double valve replacement; report of a case]. 1247 68

Hyperinsulism is a rare cause of persistent hypoglycemia in the neonatal period. Therapy can be accomplished either surgically or pharmacologically. Diazoxide treatment remains the mainstay of medical therapy. Tolerance of diazoxide is usually excellent, but several adverse effects of this drug have been described. A case of severe diazoxide intoxication with fluid retention, congestive heart failure, and respiratory failure is reported. The patient was a 43-day-old infant, affected by persistent and severe hypoglycemia. After the diagnosis, hyperinsulinism was established he was treated with diazoxide (17 mg x kg(-1) daily) and octreotide (12 microg x kg(-1) daily). A few days later he presented with hepatomegaly, severe fluid retention, diffuse edema, congestive heart failure, and respiratory failure requiring mechanical ventilation. After introduction of ACE inhibitors he developed acute renal failure. The clinical condition worsened and he developed pulmonary hypertension requiring high-frequency oscillatory ventilation. Diazoxide was stopped on the 12th day in spite of poor control of blood sugar. During the next 5 days his hemodynamic status dramatically improved and he was weaned from catecholamines: he lost weight, had a negative fluid balance, and the edema disappeared, a normal diuresis resumed and renal function improved. Improvement of respiratory patterns and gas exchange made it possible to switch back to conventional ventilation and then to extubate the patient. Echocardiography demonstrated reduction of the PA pressure to normal and resolution of atrial enlargement. The patient was scheduled for elective subtotal pancreatectomy. Diagnosis and management of diazoxide intoxication are discussed.
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PMID:A case of severe diazoxide toxicity. 1520 Jun 61

Congenital dyserythropoietic anemia (CDA) type I is an inherited disorder characterized by macrocytic anemia with pathognomonic morphologic ultrastructural features of the erythroid precursors. The authors recently cloned the CDAN1 gene and identified one founder missense mutation in all of their Bedouin patients. In a previous study, the authors found that the majority of their 31 Bedouin patients had anemia and jaundice during the first month of life and required blood transfusions; some had persistent pulmonary hypertension. In the present retrospective evaluation of 70 Bedouin patients with CDA type I, the authors more than doubled the number. Forty-five (64%) patients were symptomatic in the neonatal period, 29 (65%) had hepatomegaly, 24 (53%) had early jaundice, 11 (27%) were born small for gestational age, 7 (15%) had persistent pulmonary hypertension, and 6 (13%) had direct hyperbilirubinemia and another 6 (13%) had transient thrombocytopenia. Thirty-six of the symptomatic neonates (80%) required at least one blood transfusion. These results confirm the authors' previous findings and add neonatal manifestations not previously described, particularly hyperbilirubinemia and thrombocytopenia. Early diagnosis of CDA type I may be beneficial in light of the potential efficacy of alpha-interferon in avoiding transfusions in some patients.
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PMID:A comprehensive study of the neonatal manifestations of congenital dyserythropoietic anemia type I. 1554 10


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