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)

For over 15 years, upper respiratory tract obstruction due to adenotonsillar hypertrophy has been known to cause hypoxia, hypercapnia, increased pulmonary vascular resistance and thereby cor pulmonale and congestive heart failure. This is now an uncommon but not rare entity and three recent cases prompted this report. The typical patient is dyspneic with retractions, cyanosis, occasional periods of apnea and somnolence. Edema and hepatomegaly and at times splenomegaly are common. X-rays show cardiomegaly, which on electrocardiogram is found to involved mainly the right ventricle. The strict definition of cor pulmonale is right ventricular hypertrophy secondary to lung disease or abnormal pulmonary function, a definition that may logically be stretched to include abnormal respiratory function secondary to upper airway pathology. The mechanisms by which this occurs are generally agreed upon. Hypoxia has been demonstrated to cause pulmonary vasoconstriction. Acidosis and hypercapnia are thought by some to have the same effect. Pressure across the pulmonary vascular bed is also increased, as predicted by Poiseuille's law, by the high rate of blood flow required to maintain tissue oxygenation with poorly oxygenated blood. Conditions producing hypoxia of hypercapnia or both lead to hypertrophy and eventually to dilatation of the right ventricle. Three cases of children who underwent cardiac catheterization while suffering from cor pulmonale due to adenotonsillar hypertrophy are reported. Right ventricular pressure averaged 44/5, PAO2 72, pH 7.32, and PACO2 52. All were clinically improved following adenotonsillectomy. Cardiac catheterization was repeated in one case, with right ventricular pressure dropping from 44/5 to 21/2, pulmonary vascular resistance from eight units to three, and PACO2 from 62 to 44.
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PMID:Cardiac and pulmonary failure secondary to adenotonsillar hypertrophy. 95 48

By the end of 1990, 51 symptomatic children with perinatally acquired HIV infection had been admitted to Baragwanath Hospital. Of 42 children who were followed up for at least 3 months, 15 (35.7%) died and 16 (38.1%) were lost to follow-up. The case fatality rate for these children lies between 35.7% and 73.8%. Most children became symptomatic before the age of 6 months and presenting features seen in over 70% of cases included lymphadenopathy, failure to thrive and hepatomegaly. Surviving children had recurrent admissions to hospital, predominantly for respiratory distress. Many had bacterial pneumonias. Cardiac involvement (cardiomyopathy and cor pulmonale), recurrent serious bacterial infections and neurodevelopmental abnormalities were common. Our experience confirms that vertically acquired HIV infection has a relatively short incubation period and progresses rapidly with cardiorespiratory symptoms predominating. Five HIV seroprevalence studies have been conducted in pregnant women attending Baragwanath Hospital and the Soweto clinics since 1988. The calculated doubling time is between 7 and 21 months. At the end of 1990 the HIV seroprevalence rate in pregnant women was 0.82% (95% confidence limits 0.44-1.19%).
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PMID:AIDS--the Baragwanath experience. Part II. HIV infection in pregnancy and childhood. 150 37

A 10-year-old boy with idiopathic scoliosis presented with exertional dyspnea, lower leg edema since two weeks prior to admission. Physical examination revealed neck venous engorgement, moist rales, mild hepatomegaly, ascites and lower leg edema in addition to the scoliosis. Chest x-ray films showed cardiomegaly, pulmonary congestion and pneumonic infiltration of both lower lobes with bilateral pleural effusion. Electrocardiograms showed right axis deviation and right atrial hypertrophy. Two-dimensional echocardiography revealed dilatation of the right heart chambers and pulmonary artery. He was treated initially with mechanical ventilation, antibiotics, bronchodilators, diuretics, vasodilators, and digoxin with improvement. Unfortunately the patient became comatose after an episode of cardiopulmonary arrest. He did not recover from this tragedy and was discharged against medical advice in comatose state. Scoliosis may be complicated with pneumonia and cor pulmonale in children. Early surgical correction of the scoliosis may prevent the development of cor pulmonale. Combined medical and surgical managements should be carried out aiming at scoliotic heart disease.
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PMID:[Idiopathic scoliosis and pneumonia complicated with cor pulmonale. Report of one case]. 227 31

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

A computerised, retrospective study of 818 patients with sarcoidosis was analysed to study the prognosis of each individual manifestation of the disease. The good prognosis of erythema nodosum, acute arthritis and bilateral hilar lymphadenopathy was confirmed, though 16 per cent of patients presenting with erythema nodosum pursued a chronic course. Cor pulmonale and nephrocalcinosis reflected the poorest prognosis while lupus pernio and sarcoidosis of the mucosa of the upper respiratory tract rarely resolved. Bone sarcoidosis also implied chronicity but in four of 31 patients there was no clinical evidence of disease activity two years after the initial diagnosis, although naturally the bone radiograph was still abnormal. Hepatomegaly carried a worse prognosis than splenomegaly, or indeed, than the finding of pulmonary mottling without hilar glands--a stage three chest radiograph.
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PMID:Prognostic factors predicting the outcome of sarcoidosis: an analysis of 818 patients. 665 15

A cross-sectional echocardiographic study of 50 black Zimbabwean children with clinical human immunodeficiency virus (HIV) infection was carried out. The median age was 9 months. Seventy per cent had chronic cough, 60% respiratory distress and 40% cyanosis. Sixty per cent had pericardial effusion and 48% right ventricular hypertrophy (RVH) and dilation. However, the clinical diagnosis of heart failure was difficult as most of these children (80%) had hepatomegaly. These findings suggest that respiratory disease plays a role in the causation of RVH in these children. As cardiac causes of RVH were absent, this was presumed to be due to cor pulmonale. HIV-infected children presenting with respiratory distress may have clinically unapparent cor pulmonale. Early and appropriate management may by beneficial.
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PMID:Cor pulmonale in children with human immunodeficiency virus infection. 767 13

The incidence of heart failure in octogenarians is high and its diagnosis not always easy. In many cases it is made by excess or by omission. Obtaining a history is often difficult. Signs may be masked, false or indicative of another disease process. Dyspnea, edema of the lower limbs and crepitations are relatively non-specific. Jugular distension, tender hepatomegaly and a diastolic gallop are much more valuable. Diagnosis of the underlying etiology also raises problems. While hypertension is commonplace and easy to identify, ischemic heart disease is common and often missed. Tight aortic stenosis must be identified since its treatment is surgical. Hypertrophic cardiomyopathy is often an echocardiographic discovery. Post-embolic chronic cor pulmonale, or secondary to chronic obstructive lung disease, must always be considered in the presence of right heart failure without hypertension or chest pain. Appropriate treatment is dependent upon accurate diagnosis.
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PMID:[Difficulties in the diagnosis of cardiac insufficiency in octogenarians]. 782 52

A 73-year-old woman was presented with altered mental status and disorientation. She was diabetic and hypertensive, and she had experienced an ischemic cerebrovascular accident 3 years ago. Physical examination revealed the findings of chronic obstructive pulmonary disease, cor pulmonale and congestive heart failure. Hepatomegaly, splenomegaly and ascites were found and might be associated with postsinusoidal portal hypertension secondary to congestive heart failure. Laboratory tests showed uremia, lymphocytosis and thrombocytopenia. Neurologic findings were related with uremia and hypoxia. Multiple pathologic lymphadenopathies were seen in abdominal ultrasonography and thoracic computed tomography. Bone marrow histology indicated chronic lymphocytic leukaemia (CLL). The reason for acute renal failure was leukaemic infiltration of the kidneys due to CLL that was shown with renal biopsy. Blood urea nitrogen (BUN) and serum creatinine responded well to cyclophosphamide and methyl prednisolone treatment. In CLL, direct renal involvement is frequently seen in autopsy studies especially in advanced disease, however, renal failure due to leukaemic infiltration is extremely rare.
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PMID:Acute renal failure due to leukaemic infiltration in chronic lymphocytic leukaemia: case report. 1587 23

A young dog was presented for cyanosis and right heart failure. Radiographic and CT characteristics included right heart/pulmonary artery enlargement, hepatomegaly, abdominal effusion, and severe, generalized air-space filling. Focal increased opacities were present in the peripheral lung, as were multiple pulmonary blebs and bullae. Echocardiographic findings were consistent with cor pulmonale and pulmonary hypertension. Bronchoscopic findings were consistent with chronic inflammation. Pulmonary alveolar proteinosis (PAP) was confirmed at necropsy. Pulmonary alveolar proteinosis is an interstitial lung disease that results in accumulation of phospholipoproteinaceous material and should be included as a differential diagnosis for dogs with these clinical and imaging characteristics.
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PMID:Imaging diagnosis--pulmonary alveolar proteinosis in a dog. 2360 Apr 48

A 10-year-old, neutered female, Australian Shepherd was referred for acute respiratory distress and a history of chronic exogenous steroid administration. On thoracic radiographs, a severe increase in mineral opacity characterized as a generalized unstructured interstitial pulmonary pattern, diffuse calcinosis cutis, and moderate hepatomegaly were noted. Cor pulmonale was identified on echocardiography. The patient developed a pneumothorax following sampling and had a cardiac arrest. Postmortem histopathology of the lungs revealed pulmonary interstitial mineralization and alveolar microlithiasis. This report supports including generalized pulmonary mineralization due to chronic exogenous steroid administration as a differential diagnosis for dogs with these clinical and imaging findings.
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PMID:Radiographic characteristics of alveolar microlithiasis and pulmonary ossification following chronic corticosteroid therapy in a dog. 3131 91


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