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)

Between January 1981 and December 1990, 41 patients were operated on for hepatic hydatid disease, representing 18% of the overall surgery for liver disease performed in our institution during the same period of time. The mean age of the patients was 39.5 years (range 17-71 years), 24 were females and 17 males. The most common clinical sign was abdominal pain (59%) with 3 patients jaundiced at the time of admission. 2 patients suffered from acute rightsided pneumonia, one of them with a very important productive cough due to bronchial perforation of the hydatid cyst of the liver. One patient was admitted due to anaphylactic shock with collapse. For 14 patients (34%) the discovery of the disease was casual, either by clinical hepatomegaly or by X-ray investigations performed for other reasons. We performed 18 pericystectomies, 4 subtotal pericystectomies and 16 liver resections. Only 3 patients were treated by partial cystectomy with removal of the prominent part of the cyst. Several other surgical procedures were performed concomitantly, including 2 pulmonary lobectomies, 1 cystojejunostomy, and pericystectomies for other hepatic or extrahepatic localizations of hydatid cysts. Rupture of the cyst into the bile duct was diagnosed in 13 patients. One patient presented with a peritoneal rupture of the cyst and 2 patients had a fistula through the diaphragm into the pleural cavity. Our postoperative morbidity is acceptable (10 patients, 25%). A bilioma and a biliary leak were successfully treated by percutaneous drainage, a postoperative hemorrhage required surgical treatment, four patients experienced pulmonary complications and the three others minor wound abscesses.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Surgical treatment of hepatic hydatidosis]. 192 52

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 patient with gout and schizophrenia is described who during a schizophrenic paroxysm with paranoid-hypochondriac-hallucinatory syndrome attempted to commit suicide and took 200 tablets milurit (20 g). He developed the picture of acute intoxication with nausea, vomiting, profuse diarrhea, abdominal pain, flushing, temperature, collapse manifestations, hepatomegaly, direct hyperbilirubinemia, elevated transaminase, leukopenia, accelerated ESR. After reanimation and infusion therapy, the patient recovered within 4 days and 2 weeks later all blood indices reached the limits of the norm.
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PMID:[Acute allopurinol (milurit) poisoning]. 402 4

Adjuvant chemo- and immuno-therapy with dacarbazine (1st to 5th day 250 mg/m2 daily) and BCG (6th day 0.01 ml intracutaneously)was administered to a 50-year-old male patient in a three-week cycle after surgical removal of a superficially spreading malignant melanoma. Metoclopramide was used as an antiemetic. During the second therapy cycle sudden severe vascular collapse with increasing hepatomegaly and signs of acute hepatic failure occurred leading to death after two days. At necropsy a Budd-Chiari syndrome with thombosed hepatic veins and congestive liver parenchyma necroses was found. The cause was hyperergic endophlebitis combined with severe infiltration of the vascular walls by eosinophilic granulocytes. In association with 5 more similar cases from other clinics (personal communications) this picture must be assumed to be a complication of dacarbazine treatment.
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PMID:[Budd-Chiari syndrome during treatment with dacarbazine (DTIC) (author's transl)]. 698 51

A prospective study of the clinical pattern and course of primary biliary cirrhosis based on the data of 236 patients (211 females, 25 males) in an international randomized trial has been performed mainly using life-table technique. The median follow-up period has been 18 mo. After the entry into the study, at which time the median duration of symptoms was 2.1 yr, the estimated 5-yr increase in the cumulative percentage of patients was for pruritus from 75% to 95%, jaundice 59% to 82%, hepatomegaly 54% to 86%, pigmentation 54% to 85%, xanthomas 27% to 50%, GI bleeding 17% to 46%, ascites 7% to 49%, and vertebral collapse 3% to 20%. The frequency of cirrhosis increased from 30% to 82% in 4 yr. The mitochondrial antibody titer showed a high rate of progression with time. In analysis of subgroups, age, histologic stage, and particularly the serum bilirubin level were important prognostic factors. Sex, duration of symptoms, and character of first symptom or sign had no independent prognostic influence. The most important indication of seriously progressive course was rapid development of severe cholestasis independent of the histologic stage.
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PMID:Clinical pattern and course of disease in primary biliary cirrhosis based on an analysis of 236 patients. 735 46

During a 16-month period children presenting to a pediatric outpatient facility from an area with a high tuberculosis incidence (> 400/100,000) and suspected of having respiratory tuberculosis (TB) were evaluated for close contact with adult pulmonary tuberculosis, weight loss, symptom duration, respiratory signs, lymphadenopathy and hepatosplenomegaly and by chest radiography and tuberculin testing (Mantoux or tine). Probable tuberculosis was diagnosed in 258 children and was confirmed in 109 (42%) patients with a mean age of 31 months by culture of Mycobacterium tuberculosis from gastric aspirate or another source. Eleven children with confirmed TB had a normal chest radiograph. After review of special investigations, clinical course and follow-up of the remaining 149 children, 86 children (58%) with a mean age of 32.4 months were considered to have probable TB and 63 (42%) with a mean age of 27 months not to have TB. Significantly fewer children in the "not TB" group than in the confirmed and probable TB groups had a close adult pulmonary tuberculosis contact (13 (21%) and 95 (49%), respectively; P < 0.01). There was no difference between the "not TB" group and the confirmed and probable TB groups in the proportion presenting with weight loss, cough or other respiratory symptoms, a symptom duration > 2 weeks, the presence of bronchial breathing, wheeze, hepatomegaly or splenomegaly or peripheral lymphadenopathy. Final diagnoses in the "not TB" group included bacterial or viral pneumonia or bronchopneumonia in 37, asthma often accompanied by segmental collapse in 9 and cavitating pneumonia in 3 children.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Respiratory tuberculosis in childhood: the diagnostic value of clinical features and special investigations. 776 Nov 83

In a personal series of 107 patients, we describe clinical presentations, methods of recognition and therapeutic management of inherited fatty acid oxidation (FAO) defects. As a whole, FAO disorders appear very severe: among the 107 patients, only 57 are still living. Including 47 siblings who died early in infancy, in total 97 patients died, of whom 30% died within the first week of life and 69% before 1 year. Twenty-eight patients presented in the neonatal period with sudden death, heart beat disorders, or neurological distress with various metabolic disturbances. Hepatic presentations were observed in 73% of patients (steatosis, hypoketotic hypoglycaemia, hepatomegaly, Reye syndrome). True hepatic failure was rare (10%); cholestasis was observed in one patient with LCHAD deficiency. Cardiac presentations were observed in 51% of patients: 67% patients presented with cardiomyopathy, mostly hypertrophic, and 47% of patients had heart beat disorders with various conduction abnormalities and arrhythmias responsible for collapse, near-miss and sudden unexpected death. All enzymatic blocks affecting FAO except CPT I and MCAD were found associated with cardiac signs. Muscular signs were observed in 51% of patients (of whom 64% had myalgias or paroxysmal myoglobinuria, and 29% had progressive proximal myopathy). Chronic neurologic presentation was rare, except in LCHAD deficiency (retinitis pigmentosa and peripheral neuropathy). Renal presentation (tubulopathy) and transient renal failure were observed in 27% of patients. The diagnosis of FAO disorders is generally based on the plasma acylcarnitine profile determined by FAB-MS/MS from simple blood spots collected on a Guthrie card. Urinary organic acid profile and total and free plasma carnitine can also be very helpful, mostly in acute attacks. If there is no significant disturbance between attacks, the diagnosis is based upon a long-chain fatty acid loading test, fasting test, and in vitro studies of fatty acid oxidation on fresh lymphocytes or cultured fibroblasts. Treatment includes avoiding fasting or catabolism, suppressing lipolysis, and carnitine supplementation. The long-term dietary therapy aims to prevent periods of fasting and restrict long-chain fatty acid intake with supplementation of medium-chain triglycerides. Despite these therapeutic measures, the long-term prognosis remains uncertain.
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PMID:Recognition and management of fatty acid oxidation defects: a series of 107 patients. 1040 81

Congenital aortic stenosis accounts for about 5% of cardiac malformations recognized in childhood. It belongs to the category of acyanotic congenital heart disease. These lesions produce a load on the heart because of left ventricular outflow tract obstruction. Severe aortic stenosis in the newborn period (critical aortic stenosis) presents with signs of left sided heart failure (pulmonary edema, poor perfusion), right sided heart failure (hepatomegaly, peripheral edema) and may progress rapidly to total circulatory collapse. We present a case of an infant with critical aortic stenosis presenting with cyanosis, who was entirely dependent on ductal patency for systemic output. When oxygen was given, the ductus started to close, with a worsening of the left sided output and subsequent acidosis. With the right to left shunt across the ductus, the baby was cyanotic and dependent on prostaglandin to keep the ductus open. There was minimal flow across the aortic valve because of the stenosis and extremely poor left ventricular function prior to surgery. After relief of the aortic valvular obstruction, there was finally good antegrade flow across the aortic valve, terminating cyanosis.
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PMID:One day old infant with acyanotic congenital heart disease: critical aortic stenosis. 1056 81

A 30-year-old HBsAg-positive woman was admitted to the hospital because of 6 days of progressive shortness of breath. She was in severe respiratory distress with circulatory collapse. She had an enlarged liver but no stigmata of chronic liver disease or signs of cirrhosis. She had rapidly developed respiratory arrest and was transferred to intensive care unit. Heart ultrasonography and Doppler scan showed right heart straining and high pulmonary artery pressure. Despite cardiovascular and respiratory support she died a few hours after admission. Autopsy revealed combined hepatocellular-cholangiocarcinoma infiltrating the entire liver, metastatic invasion of lung blood vessels and absence of right ventricular hypertrophy. The incidence of hepatocellular-cholangiocarcinoma, a variant of hepatocellular carcinoma, is roughly 2-3% and the presenting symptoms are abdominal pain, weight loss, jaundice, fever or decompensation of liver disease. Associated HBsAg positivity and cirrhosis are reported in 20-30% and 60% of patients, respectively. Metastases to lungs are relatively frequent but this is the first report of hepatocellular-cholangiocarcinoma presented with acute respiratory distress due to massive pulmonary embolism.
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PMID:Combined hepatocellular-cholangiocarcinoma presented with massive pulmonary embolism. 1102 Aug 95

The authors have conducted a prospective investigation on 15 patients with hemodynamically unstable pericardial effusion (main criteria: echocardiographic signs of various degrees of right ventricular diastolic collapse and clinical instability) hospitalized in the Intensive Cardiac Unit (1.97% of all patients) for one year and have compared the results with literature data. The causes of pericardial effusion were neoplasms, infections, rupture of heart of aorta and hypothyroidism. Investigation revealed the most frequent findings: symptoms (dyspnea, retrosternal pain, loading intolerance, nonproductive cough), clinical signs (soft heart sounds, changes in pulmonal findings, fever, jugular venous distention, tachycardia, arterial hypotension and hepatomegaly), laboratory changes (elevated erythrocyte sedimentation rate, leukocytosis), ECG changes (ST-T abnormality, microvoltage, tachycardia) and chest X-rays changes (enlarged cardiac silhouette, pleural effusion). Echocardiography found an average width of pericardial effusion of 2.5 cm (+/- 1.2), frequently thickened pericardium and changes in heart motions. The most used drugs in therapy were indomethacin, antibiotics, analgesics and corticosteroids. In three patients pericardiocentesis, and in two pericardiectomy were performed. Two patients died, 13 patients were discharged from the ICU with an improved health condition. Literature data on this condition are either lacking, or differ from the above findings.
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PMID:Hemodynamically unstable pericardial effusion in the Intensive Cardiac Unit: prospective study. 1467 24


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