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)

We report a case of non-Hodgkin's lymphoma (NHL) presenting with acute renal failure. A-56-year-old male was admitted to our hospital on October, 1997 with fever and renal dysfunction. Physical examination showed no abnormality except for hepatomegaly. Body surface lymphadenopathy was not observed. Computed tomography (CT) of the abdomen showed markedly enlarged kidneys bilaterally and a mass of soft tissue density, which was considered as a swelling lymph node, around the aortic artery. The renal biopsy revealed parenchymal involvement of the NHL cells without normal tubulo-interstitial structure, but the glomeruli were almost intact. Our case rapidly fell into oliguria and acute renal failure, hence needed hemodialysis. After chemotherapy was performed, his renal function gradually improved and the kidney became smaller on subsequent CT. Unfortunately, the patient happened to suffer from methicillin-resistant staphylococcus aureus (MRSA) infection in a neutropenic state and died. Necropsy revealed recovery of the renal interstitium without residual NHL cells. Renal lymphoma without any other organ or nodal involvement is a rare type of NHL, which considered primary renal lymphoma (PRL). However, we believe this case to have been a result of lymphomatous infiltration of the kidneys in disseminated lymphoma.
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PMID:[A case of non-Hodgkin's lymphoma presenting with acute renal failure diagnosed by renal biopsy]. 1050 45

The Human T-Cell lymphotropic virus type I (HTLV-I) is endemic in the Caribbean basin, Japan, Central Africa and South Pacific. It as been associated to Lymphoma-Leukemia of Adult T-Cells (ATLL) and a progressive spastic paraparesis (TSP/HAM). The nationwide seroprevalence of Panama is of 1-2%. We report a case of a 73 year old male, albino, single, patient, Panamanian descendent from Jamaican immigrants who presented a skin disorder which started 1 year ago, characterized by the appearance of infiltrative, intensely pruritic papules, nodules and a non exfoliative erythroderna involving face, neck, trunk and extremities. He also had painless enlarged cervical lymph nodes, non tender hepatomegaly. Laboratory studies revealed a keukocyte count of 128,000/ml wit 67% atypical lymphocytes, serum calcium was 12.5 mg/dl, DL in 583 UNI, "flower cells" and atypical lymphocytes with hyperlobulated nuclear contour was observed in the peripheral blood smear, seropositivity to TLV-I detected by enzyme-linked immunosorbent assay (Elisa) and confirmed by Western blot assay. The skin biopsy shows a bandlike dermal infiltrates of atypical lymphoid cells with epidermotropism and Pautrier's microabscesses. Once the treatment was initiated with prednisone, cyclophosphamide, and systemic antibiotics for a bronchopneumonic process most of the cutaneous lesions cleared up although the clinical condition of our patient became progressively worse and died after a acute renal failure and a lower gastrointestinal bleeding. In 1986, the tree first cases of ATLL were identified in Panama, there has not been apparently new cases reported until now.
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PMID:[T-cell lymphoma/leukemia secondary to HTLV-1 in adults. Report of a case]. 1099 95

Severe adverse effects due to 3,4-methylenedioxymethamphetamine (MDMA, ecstasy) are reported with increasing frequency in the medical literature. The signs of acute toxicity most often seen are fulminant hyperthermia, hyperexcitatory states, acute renal failure and hyponatraemia. In 1992, hepatotoxicity was also described with unexplained jaundice and hepatomegaly after ingestion of MDMA. We report a case of severe toxic hepatitis following ingestion of MDMA with fulminant hepatic failure which required auxiliary liver transplantation. The diagnosis was necrotic toxic hepatitis following ecstasy ingestion. The outcome was successful, and the patient was discharged from ICU 20 d after surgery. Hepatotoxic effects of MDMA seem infrequent, but may be lethal; liver transplantation is the ultimate therapeutic option in some cases.
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PMID:Ecstasy ingestion and fulminant hepatic failure: liver transplantation to be considered as a last therapeutic option. 1130 31

We conducted a case record study comparing liver tests abnormalities in 20 malaria-related acute renal failure cases without cerebral malaria, 52 cerebral malaria cases without other organ impairment, 189 cases of nonsevere malaria associated with a high parasite burden, and 131 cases of mild Plasmodiumfalciparum malaria. Jaundice and hepatomegaly were significantly associated with renal failure (adjusted odds ratio [AOR], 3.3, 95% confidence interval [CI], 1.3-8.6, P = 0.01; and AOR, 1.7 95% CI, 1.13-2.4, P = 0.01) but not with cerebral malaria (AOR, 1, 95% CI, 0.5-2, P = 0.8; and AOR, 1.08, 95% CI, 0.8-1.8, P = 0.5). Patients with acute renal failure were significantly older and had increased liver abnormalities compared with other groups. Although an increase in the proportion of mature schizonts over ring forms was significantly associated with cerebral malaria, it did not seem to have affected acute renal failure. These results suggested that cytoadherence was not the main determinant for renal failure and that jaundice itself may have potentiated the effects of hypovolemia.
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PMID:Association of hepatomegaly and jaundice with acute renal failure but not with cerebral malaria in severe falciparum malaria in Thailand. 1179 81

The hepatorenal syndrome (HRS) is a unique form of acute renal failure with entirely normal renal histology in advanced liver disease. Its diagnosis is made by exclusion of all causes of renal failure and by all the five major criteria as set by the International Ascites Club. The presence of hepatomegaly, poor nutritional status, and oesophageal varices at endoscopy are associated with a high risk of HRS. The liver tests, the Child-Pugh score, are of no value in prediction of its occurrence. Contraction of the effective blood volume, which may lead to renal hypoperfusion with preferential renal cortical ischaemia, is proposed pathogenesis of the condition. Because understanding of the pathogenesis of HRS is incomplete, therapy is supportive only. Optimal fluid management is vital as there is almost invariably a reduction in effective arterial blood volume. Dopamine, frusemide and haemofiltration may be helpful in management of fluid overload but do not affect renal function. TIPS has been used successfully in small series of patients. The vasopressin analog also has been used with early excellent response. The treatment of HRS has been discouraging and the only proven cure for HRS is liver transplantation at this point of time.
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PMID:Hepatorenal syndrome: pathophysiology and treatment. 1224 Aug 52

Glycogen storage diseases (GSDs) or glycogenoses comprise several rare inherited diseases caused by abnormalities of the enzymes that regulate the synthesis or degradation of glycogen. We report on a male patient with type Ia GSD (GSD Ia) who was followed-up for more than 20 years. He had been diagnosed with GSD Ia based on biochemical tests and the glucose-6-phosphatase (G6Pase) enzyme assay from a liver biopsy at 6 years old, due to problems of hepatomegaly, growth retardation, and recurrent hypoglycemic episodes. The introduction of uncooked cornstarch improved his quality of life only in the first 8-year follow-up period. At 17 years old, gouty arthritis with multiple tophi and generalized xanthomatosis developed. Later, hepatocellular adenoma, nephrolithiasis, and gastrointestinal bleeding occurred at the age of 20, 23, and 24 years, respectively. At 26 years old, he suffered from acute renal failure and polyradiculoplexopathy. The problem of delayed puberty persisted. The story of this patient illustrates the multisystemic nature of GSD Ia and highlights the need for careful dietary therapy and long-term follow-up.
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PMID:A 20-year follow-up of a male patient with type Ia glycogen storage disease. 1284 28

In Bucaramanga, Colombia, dengue haemorrhagic fever (DHF) has become endemo-epidemic since 1992. A cross-sectional study covering a period of 10 years (February, 1992 to February, 2002) was undertaken in children under 13 years of age hospitalized at the University Hospital. Observations were recorded on the clinical features, laboratory tests and the natural development of the disease. A total of 763 patients were examined, of whom 617 were classified as having DHF according to the WHO criteria (9.1% Grade I, 61.5% Grade II, 21.7% Grade III and 7.5% Grade IV). One hundred forty six patients could not be classified. The highest incidence took place in 1997, 1998 and 2001. Seventy four per cent of patients came from the metropolitan area of Bucaramanga; 48% were males; 0.3%, newborns; 11.8%, infants; 23%, pre-school children, and 64.9%, school children. The most important clinical features were fever and haemorrhagic manifestations (100%); vomiting (60%); abdominal pain (57%); headache (50%); osteomyalgia (40.8%); hepatomegaly (33%), and macular rash (29%). Among the haemorrhagic manifestations we found petechiae (56%); positive tourniquet test (35%); gastrointestinal bleeding (34%), and epistaxis (32%). Serous effusion was found in 17.7% of cases. Alarm signs of shock were found in 29%. Fifty two per cent had leucopenia and 37.3% atypic lymphocytes. Among other unusual manifestations were hepatitis, encephalopathy, alithiasic cholecystitis, acute renal failure, haemophagocytic syndrome and coinfections. Of the 617 cases, 12 died (1.5%).
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PMID:[Dengue haemorrhagic fever in children: ten years of clinical experience]. 1287 57

A total of 455 confirmed and reported to the Bulgarian Ministry of Health leptospirosis cases between 1989 and 2001 were analysed. Overall incidence of the disease was 0.42/100.000 population (range 0.20-0.64/100.000). The overall fatality rate was 6.6%. Infection was acquired through occupational (30.3%), recreational (45.1%), and accidental (4.6%) exposure or was home contracted (5.1%). A tendency towards the main mode of transmission moving from occupational to recreational exposure was noted. Fishing and livestock farming together accounted for more than half of all leptospirosis cases. Leptospirosis was contracted more frequently through indirect contact with animal excreta and less commonly through direct contact with animal urine or tissue. Serovars belonging to 10 different serogroups caused infection during the 13-year study period. Two serogroups, Icterohaemorrhagiae and Pomona, accounted for more than 87% of all leptospirosis cases. The third main cause was serogroup Australis. The significant change in the infecting serovars has to be related to widely performed vaccinations of cattle and pigs but still inefficient measures for rodent control. The most frequently reported symptoms were fever, myalgia, jaundice, and hepatomegaly. Anicteric cases (40.3%) had milder flu-like course. Acute renal failure was reported in 52 (33.8%) of the patients, all of them with icteric leptospirosis. Knowledge of epidemiological, clinical, and serological features of leptospirosis in Bulgaria during the 13-year study period is an appropriate base to outline measures for successful prevention and early diagnosis of the disease.
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PMID:Human leptospirosis in Bulgaria, 1989-2001: epidemiological, clinical, and serological features. 1472 64

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

Idiopathic myelofibrosis is characterized by bone marrow fibrosis, anemia, leukoerythroblastosis, and extramedullary hematopoiesis in many organs. Renal abnormalities in idiopathic myelofibrosis have been rarely described in the literature and include extramedullary hematopoiesis in the pararenal or retroperitoneal areas resulting in obstructive uropathy and hemtopoietic cell infiltration in tubulointerstitial area and urolithiasis. These lead to azotemia or acute renal failure, which may respond well to radiotherapy and adjuvant chemotherapy. To our knowledge, there has been only one case report of nephrotic syndrome associated with glomerulonephritis in a myelofibrosis patient; however, no effective treatment was described. Herein, we report the case of a patient with idiopathic myelofibrosis who initially presented with hepatomegaly, anemia, and leukoerythroblastosis. A nephrotic syndrome developed 7 years after initial diagnosis. Renal biopsy disclosed the unique pathological finding of simultaneous mesangial proliferative glomerulonephritis, renal extramedullary hematopoiesis, and gouty nephropathy. Despite treatment with busulfan, proteinuria persisted that implied irreversible glomerular injury and a terminal prognosis. We focus on the unusual pathological finding and the association between nephrotic syndrome and idiopathic myelofibrosis.
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PMID:Idiopathic myelofibrosis associated with renal extramedullary hematopoiesis and nephrotic syndrome: case report. 1564 Dec 21


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