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

We report a case of scrub typhus pneumonitis in a laboratory worker who apparently acquired it through the respiratory tract. The patient was suffering from fever, cough and dyspnea. He had both cervical and axillary lymphadenopathy, and hepatomegaly. A chest X-ray showed interstitial infiltrates. A diagnosis of scrub typhus was established upon isolation of Orientia tsutsugamushi. 12 days before the patient showed symptoms, he had purified O. tsutsugamushi proteins from infected cells using an ultrasonication method which could generate aerosols containing O. tsutsugamushi.
 ...
PMID:Scrub typhus pneumonitis acquired through the respiratory tract in a laboratory worker. 1126 62

The clinical profile of one hundred and six biopsy proved patients with sarcoidosis is reported from New Delhi. The disease occurred more frequently in males (68 of the 106; 64%) in their fifth decade. Dry cough (83%), exertional dyspnoea (61%), fever (56%) and joint pains (44%) were frequent symptoms. Hepatomegaly (45%), peripheral lymphadenopathy (45%), cutaneous involvement (42%), presence of crepitations (36%) were frequent signs. In addition, ocular symptoms (18%), parotid gland enlargement (12%), facial nerve palsy (8%) were observed. Audible wheeze was present in nine patients. Radiologically, 25 (24%), 67 (63%) and 14 (13%) of patients belonged to stage I, II and III respectively at presentation. More than one sibling was involved in six families. Pulmonary functions revealed mixed obstructive and restrictive ventilatory defect. However, obstructive ventilatory defect was predominant. Systemic steroids were used in 81 patients who regularly followed up. Frequent relapses occurred on tapering off or stopping the corticosteroids in 21 patients. History of malaise (p < 0.05), presence of crepitations (p < 0.05), wheezing (p < 0.05), peripheral blood eosinophilia (p < 0.05) and FEV1/FVC (%) < 65% of the predicted value (p < 0.05), were independent predictors of relapse. Hyperuricaemia, hitherto unreported in patients with sarcoidosis was observed in 41% of patients for whom values were available. Two patients died; one from cardiomyopathy and another from stroke. In summary, constitutional symptoms such as fever, weight loss and pulmonary infiltrates, were more frequently encountered in Indian patients with sarcoidosis as compared to western studies. A high index of clinical suspicion and histopathological confirmation early in the illness are required to ascertain the diagnosis lest these patients will get treated as tuberculosis with potentially hepatotoxic drugs.
 ...
PMID:Clinical characteristics, pulmonary function abnormalities and outcome of prednisolone treatment in 106 patients with sarcoidosis. 1157 54

A 56-year-old woman presented with general fatigue, dyspnea on exercise, and hepatomegaly subsequent to secondary implantation of a pacemaker. On admission, cardiac echo examination showed tricuspid valve regurgitation due to a migrated looped lead. At surgery, there was evidence of severe stenosis at both orifices of the superior and inferior vena cavae due to fibrous tissue around the leads. We removed the fibrous tissue, pacing leads, and generator. New leads were anchored onto the epicardium and a generator was inserted under the rectus muscle. The postoperative course was satisfactory and the symptoms disappeared.
 ...
PMID:An operative case of inferior vena cava stenosis due to fibrosis around permanent pacemaker leads. 1191 93

A 52-year-old man was admitted with diarrhea, near dehydration and dyspnoea. After many years of alcohol consumption, he had stopped drinking alcohol over a very short space of time and had eaten very little or nothing. He had tachypnoea, hypotension and an enlarged liver. Laboratory analysis revealed metabolic acidosis with an elevated anion gap, ketone bodies in the urine, increased free fatty acid levels and beta-hydroxybutyrate in the serum, fitting the picture of alcoholic ketoacidosis. The syndrome is explained by metabolic changes induced by chronic alcohol consumption and ketogenesis as the result of fasting and dehydration. Treatment consisted of correction of the fluid deficit and administration of glucose, after which the patient made a rapid recovery.
 ...
PMID:[Ketoacidosis after cessation of chronic alcohol consumption]. 1205 Oct 65

Pleuropulmonary amebiasis is the common and pericardial amebiasis the rare form of thoracic amebiasis. Low socioeconomic conditions, malnutrition, chronic alcoholism, and ASD with left to right shunt are contributing factors to the development of pulmonary amebiasis. Although no age is exempt, it commonly occurs in patients aged 20 to 40 years, with an adult male to female ratio of 10:1. Children rarely develop thoracic amebiasis: when it does occur there is an equal sex distribution. The infection usually spreads to the lungs by extension of an amebic liver abscess. Infection may pass to the thorax directly from the primary intestinal lesion through hematogenous spread, however. Lymphatic spread is one possible route. Inhalation of dust containing cysts and aspiration of cysts or trophozoites of E histolytica in the lungs are some other hypothetical routes. The lung is the second most common extraintestinal site of amebic involvement after the liver. Usually the lower lobe, and sometimes the middle lobe of the right lung, are affected, but it may affect any lobe of the lungs. The patient develops fever and right upper quadrant pain that is referred to the tip of the right shoulder or in between the scapula. Hemophtysis is common. The diagnosis of thoracic amebiasis is suggested by the combination of an elevated hemidiaphragm (usually right), hepatomegaly, pleural effusion, and involvement of the right lung base in the form of haziness and obliteration of costophrenic and costodiaphragmatic angles. Infection is usually extended to the thorax by perforation of a hepatic abscess through the diaphragm and across an obliterated pleural space, producing pulmonary consolidation, abscesses, or broncho-hepatic fistula. Empyema develops when a liver abscess ruptures into the pleural space. Rarely, a posterior amebic liver abscess can burst into the inferior vena cava and develop an embolism of the inferior vena cava and thromboembolic disease of the lungs with congestive cardiac failure or corpulmonale. Diagnosis by finding E histolytica in stool specimens is of limited value. In a limited number of cases amebae might be found in aspirated pus or expectorated sputum. "Anchovy sauce-like" pus or sputum may be found. Presence of bile in sputum indicates that the pus is of liver origin. Serological tests are of immense value in diagnosis. Liver enzymes are usually normal and neutrophilic leucocytosis may or may not be found. ESR is invariably elevated. Anti-amebic antibodies can be detected by ELISA, IFAT, and IHA. Amebic antigen can be detected from serum and pus by ELISA. Detection of Entamoeba DNA in pus or sputum may be a sensitive and specific method. Pleuropulmonary amebiasis is easily confused with other illnesses and is treated as pulmonary TB, bacterial lung abscesses, and carcinoma of the lung. A single drug regimen with metronidazole with supportive therapy usually cures patients without residual anomalies. Aspiration of pus from empyema thoracis may be needed for confirmation and therapeutic purposes. The pericardium is usually involved by direct extension from the amebic abscess of the left lobe of the liver, sometimes from the right lobe of the liver, and rarely from the lungs or pleura. An initial accumulation of serous fluid due to reactive pericarditis followed by intrapericardial rupture may develop either (1) acute onset of severe symptoms with chest pain, dyspnea, and cardiac tamponade, shock, and death, or (2) progressive effusion with thoracic cage pain, progressive dyspnea, and fever. Chest radiograph, ultrasound examination, and CT scan usually confirm the presence of a liver abscess in continuity with the pericardium and fluid within the pericardial sac with or without the fistulous tract. Echocardiography may demonstrate fluid in the pericardial cavity. Patients should be cared for in the ICU and ambecides should be started without delay. Pericardiocentesis usually confirms the diagnosis and improves the general condition of the patient. Aspiration of the accumulated fluid should be performed urgently in cardiac tamponade; repeated aspiration may be needed. Surgical drainage should be done if needed. Acanthamoeba, a free-living ameba, may also infect the lungs in the form of pulmonary nodular infiltration and pulmonary edema in association with amebic meningoencephalitis in immunocompromised patients. It usually spreads to the meninges of the brain by way of the blood from its primary lesion in the lung or skin. Early diagnosis and institution of treatment may be life saving for these patients. A literature review shows that HIV/AIDS patients are not prone to infection with E histolytica. It is now clear that there are an increasing number of HIV-seropositive patients among amebic liver abscess patients, however, which suggests that although the incidence of intestinal infection is not high among HIV-seropositive or AIDS patients they are more susceptible to an invasive form of the disease.
 ...
PMID:Thoracic amebiasis. 1209 41

Three infants with documented mitochondrial fatty acid oxidation disorders are described in this report. Case 1. Carnitine/acylcarnitine translocase deficiency. (CACT) (OMIM 212138) A two-day-old male developed sudden cardiac arrest 48 hours postpartum, with a previous history of early death (day 2) in siblings with a history of parental consanguinity; somnolence, inactivity, refusal to suck within 24 h, hepatomegaly, persistent hypoglycemia, hypocalcemia, hyperkalemia and severe metabolic acidosis prior to cardiac arrest. Dried blood spots by tandem mass spectrometry demonstrated 10 x elevation of palmitoylcarnitine, moderate elevation of oleylcarnitine, steroylcarnitine and myristoylcarnitine. Case 2. Medium chain acyl CoA dehydrogenase (MCAD) deficiency. (OMIM 212139) A six-week-old male infant, developed sudden cardiac arrest after contacting a viral illness, resuscitated successfully in the first episode, only to succumb during the second episode, 2 weeks apart. Plasma acylcarnitine via tandem mass spectrometry was reported normal; however, urine organic acids via gas liquid chromatography and mass spectrometry demonstrated characteristic metabolites consistent with MCADD. Case 3. Carnitine deficiency, systemic primary. (CDSP) (OMIM 212140) A one-year-old girl with progressive dyspnea since birth and a history of parental consanguinity. Severe dilated cardiomyopathy with episodes of cardiac decompensations, hepatomegaly, anemia, generalized hypotonia, but no hypoglycemia were demonstrated prior to cardiac arrest. Extremely low carnitine level noted in dried blood spots via tandem mass spectrometry.
 ...
PMID:Mitochondrial fatty acid oxidation disorders in Thai infants: a report of 3 cases. 1240 51

From an association of nearly 50 years, the author had diagnosed biopsy proven 200 cases of sarcoidosis in Eastern India during the past three decades. It appears that most of these cases follow a distinct clinical pattern and presentation. The clinical course and prognosis differ considerably from that seen in Caucasians, Afro-Americans, South-African Bantus and Japanese. Males, above 40 years, coming largely from atopic and wealthier section of society (a particular business community with physicians, nurses with their families and other professionals). Patients present with constitutional symptoms (97%) like slow unrecognized fever with little malaise (fever-malaise dissociation in 70%), arthralgia (61%) or lone-myalgia (13%), appreciable loss of weight (33%), irritability, anorexia, respiratory symptoms (93%) like cough, dyspnea, etc., hepatomegaly (43.5%), splenomegaly (32.5%), lymphadenopathy (22%) with raised ESR (91%), hypergammaglobulinaemia (41.5%), hypercalciuria (40.5%), raised serum angiotensin converting enzyme (SACE) in 70.5% advance disease in chest radiograph (68%), positive 67-gallium scan and clinico-radiological dissociation in 81% (alarming looking chest radiograph with few physical signs). Course and prognosis also differ from the West. A different treatment schedule, avoiding oral prednisolone, has been found quite effective.
 ...
PMID:Sarcoidosis: a journey through 50 years. 1243 38

Polycystic liver disease (PCLD) is a rare inherited disorder, often associated with polycystic disease of the kidney. Although liver failure is unusual, some patients suffer from hepatic enlargement associated with severe complications such as abdominal distention, cachexia and dyspnea. Until recently, many surgical attempts had been made to reduce hepatic size, however, results have been unsatisfactory [3, 9, 10]. Today, liver transplantation is recommended as a therapeutic option, and excellent outcome has been demonstrated [1, 2, 4, 5, 6, 8, 11]. In this paper, we present the first case study of total hepatectomy and partial liver transplantation for PCLD, from a living, related donor. The patient is a 38-year-old man with PCLD who underwent living related liver transplantation (LRLT). He is alive and well 21 months after the operation, with complete resolution of the symptoms. He has returned to his previous job, with a marked improvement in his quality of life. Our experience demonstrates that LRLT can be an option for treatment of PCLD.
 ...
PMID:Living related liver transplantation for polycystic liver disease. 1246 64

A 45-day-old patient was admitted with dyspnea, hepatomegaly, tachycardia, holosystolic murmur in the precordial region, and continuous murmur at the right hypochondrium. Four cutaneous angiomas were noted. Instrumental examinations revealed congestive heart failure and multiple focal lesions in the liver with typical features of hemangiomas. The therapy with subcutaneous interferon-alfa-2a (IFN-alpha) was administered for 12 months with progressive regression of cutaneous hemangiomas, liver lesions, and cardiological alterations. IFN-alpha therapy was effective without any significant adverse effects.
 ...
PMID:Recombinant interferon alfa 2a in hepatic hemangiomatosis with congestive heart failure: a case report. 1255 28

Sinus of Valsalva aneurysms are uncommon. Aortic sinus aneurysm may be complicated by endocarditis or rupture. A 26 year old native Ghanaian presented with dyspnoea, raised jugular venous pressure (JVP), tender hepatomegaly, peripheral oedema, a thrill and a continuous murmur at the upper left sternal edge. Two-dimensional doppler echocardiography with colour flow mapping revealed a large aneurysm of the right sinus of Valsalva (4 cm diameter) that abutted the right ventricular out-flow tract with distortion of the pulmonary valve. Colour flow revealed left to right shunting of blood from the aortic root into the right atrium. A year later he presented with a febrile illness, weight loss, night sweats and was diagnosed as having culture negative infective endocarditis. Following a course of antibiotics, he underwent successful cardiopulmonary bypass surgery with repair of the ruptured aneurysm of the right sinus of Valsalva.
 ...
PMID:Rupture of sinus of Valsalva aneurysm: case report. 1286 11


<< Previous 1 2 3 4 5 6 7 8 9 10 Next >>