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)

Case reports of benign hepatoma in young women taking oral contraceptives (OCs) are known to exist. A benign hepatic adenoma was discovered in a young woman who had been taking an OC (Ortho-Novum 2) for 7 years. Although hepatic adenoma is a rare tumor, the increasing number of reports of its occurrence strongly suggest an association between OCs and benign hepatic adenoma. Diagnosis must be suspected in any young woman taking OCs and developing signs of acute cholecystitis with hepatomegaly or mass, or presenting with signs and symptoms of nontraumatic intraabdominal hemorrage. Rupture of the tumor is life-threatening. Treatment should be removal of the tumor whenever possible.
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PMID:Benign liver cell adenoma associated with use of oral contraceptive agents. 17 59

Focal nodular hyperplasia and adenoma of the liver together represent approximately 2% of all primary hepatic tumors and tumor-like lesions in childhood. This study reports the clinical and pathologic features of focal nodular hyperplasia in three children, all females between 27 months and 15 years of age with asymptomatic hepatomegaly. In contrast, massive hemoperitoneum from a ruptured, hemorrhagic mass was the presentation of the hepatic adenoma in a 14-year-old girl. There was no history of administration of steroids in these four children. Angiography in two cases (one case each of focal nodular hyperplasia and hepatic adenoma) revealed hypervascular lesions with abnormal tortuous vessels suggesting a malignant tumor. Pathologically, the adenoma and focal nodular hyperplasia were readily distinguishable and the necessity for this differentiation was reviewed. All four patients are currently doing well.
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PMID:Focal nodular hyperplasia and adenoma of the liver. A pediatric experience. 23 73

With the advent of whole body computed tomography (CT), an early diagnosis of hepatic tumors may be posible and laparotomy for diagnosis unnecessary. The CT scanner used was the whole body Delta unit. Patients were examined after an iv dose of 100 cc of Renograffin 60. Solid and cystic components of lesions can thus be determined. Case reports are given of 2 patients. The 1st patient had been taking Ovulen-21 for 21 months. Her liver scan revealed a large filling defect in the right lobe of the liver. Abdominal angiography showed an avasuclar mass displacing the right kidney. A CT scan depicted a large mass replacing the right lobe of the liver. The density of the mass indicated a fluid-filled cyst. At laparotomy a cyst involving the entire right lobe of the liver was found. Several nodules were present in the cyst wall. The diagnosis was cyst-adenoma of the liver. The other patient had taken Ovulen-21 for 7 years. An enlarged liver and symptoms of cholecystitis were present. The CT scan revealed a bolbous mass in the right lobe of the liver. The tumor was of the same density as the liver. At laparotomy a large hepatic adenoma was found. A 2nd mass was present in the left lobe. The tumors were not resected; cholecystectomy was done. Because of bleeding, a hepatic artery was ligated. Postoperatively, a repeat CT scan showed a large area of decreased density. At reoperation a large hematoma with necrosis of the hepatic adenoma was found and drained. The CT is an accurate method of performing percutaneous biopsies and aspiration procedures. An exact position of the needle tip can be obtained. CT-guided biopsies have also been done on masses in other areas.
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PMID:Benign hepatic tumors and cysts in women using oral contraceptive agents: computed tomography as a diagnostic aid. 86 89

In this case report, it is emphasized that duration of exposure to an oral contraceptive is not of prime importance. The patient was a 30-year-old woman complaining of sudden onset of severe epigastric pain which radiated to the right upper abdomen and the right shoulder. She had been using anovulatory pills for 18 months. A mass was felt in the right upper abdomen. The white cell count was 21,000/cu mm. Acute cholecystitis was the original diagnosis. In the next 4 days the bilirubin rose to 2.9 mg% and the alkaline phosphatase to 23.1 KA units. At laparotomy the gallbladder was found to be normal. Hepatomegaly was noted. A liver biopsy was taken. Subsequently a liver scan showed a large defect in the right lobe of the liver. Selective angiography of the right hepatic artery showed a large vascular mass. The diagnosis was changed to hepatic adenoma with secondary hemorrhage. Before further surgery the patient suffered a cardiorespiratory arrest and died. At autopsy a hepatic adenoma was found in the right lobe. A large embolus was found blocking the pulmonary arteries. The embolus was considered the cause of the sudden death.
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PMID:Fatal outcome of an hepatic adenoma following short term oral contraceptive use. 92 51

A 31-year-old male patient with type Ia glycogen storage disease was admitted to our department complaining of general fatigue and right hypochondriac pain. He exhibited massive hepatomegaly with systemic hypoglycemia, lactic acidosis, hyperuricemia, hyperpyruvatemia and hyperlipemia. The failure of blood glucose levels to increase after a glucagon loading test, and a reduced lactate level on glucose tolerance test were also observed. Various imaging techniques suggested hepatic adenoma with hemorrhage in the tumor, which was confirmed histologically. There was a complete absence of glucose 6-phosphatase activity, as determined by an enzyme assay on resected liver specimens, which proved the case to be type Ia glycogen storage disease. We also reviewed all previously reported cases of hepatic tumor and glycogen storage diseases. We conclude that, since hepatic adenoma is not rare in this disease, and is complicated by hemorrhage, rupture and malignancy, careful follow-ups are necessary.
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PMID:A case of type Ia glycogen storage disease complicated by hepatic adenoma. 217 Feb 59

A 21-year-old woman presented with a 12-month history of epigastric pain, and for 3 months she had noticed a mass in the right hypochondrium. She had taken 'Norinyl-1' (norethisterone 1 mg and mestranol 50 mcg) for 5 years. She smoked 20 cigarettes a day but drank little alcohol. Physical examination revealed irregular hard hepatomegaly 10 cm below the right costal margin. Hepatitis B surface antigen was not detected in the serum and alpha fetoprotein levels were normal ( 10 M.R.C. units). A liver scan showed a large space-occupying lesion in the right lobe of the liver, and liver biopsy revealed a cholangicarcinoma with striking fibrous reaction. Multiple shadows consistent with metastases were present on chest X-ray, but no bony deposits were found on radiological skeletal survey or bone scan. The serum calcium was persistently high (2.74-2.92 mmol/l) but fell on prednisolone therapy. Serum parathyroid hormone levels were normal. A causal relation between oral contraceptives and hepatic adenoma is now generally accepted, and several patients with hepatocellular carcinoma have also been reported. We have been able to find only 1 previous report of cholangiocarcinoma in a young female taking oral contraceptives, and there is 1 report of this tumor in a man taking high doses of anabolic steroids for refractory anemia. This tumor has its peak incidence in the 6th decade and is very rare in the 3rd decade. The association with hypercalcemia due to pseudohyperparathyroidism is well recognized. In only some cases are parathyroid hormone levels raised, and the cause of the pseudohypercalcemia in our patient is unknown.
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PMID:Cholangiocarcinoma and oral contraceptives. 610 61

Forty-one patients (16 females and 25 males) over 10 years of age from five different European centres were studied retrospectively. Of those patients 19 were below the 3rd percentile for height. Hypoglycaemia was still reported in 6 patients. Hepatomegaly was present in 39 out of 40, while 11 out of 27 reported patients had marked hepatomegaly (> 10 cm below the costal margin in the midclavicular line). Adenomas were detected in 11 out of 39 patients, alpha-1-fetoprotein was reported to be within normal limits in a total of 22 patients of whom 6 had adenomas. Blood cholesterol concentration was elevated in 31 out of 38 patients, in 7 greater than 10.0 mmol/l. Blood triglycerides were elevated in 29 out of 34 patients, in 8 patients greater than 4.0 mmol/l. Blood uric acid concentration was elevated in 19 out of 35 patients, 12 of them being treated with allopurinol. Mental development was reported to be normal in 32 out of 37 patients. Since limited information on treatment was available no significant differences between treatment groups could be detected. In order to evaluate the effect of treatment, 20 patients (10 females and 10 males) of one centre were studied before and after at least 5 years of treatment. This treatment consisted of frequent feedings during the day together with nocturnal gastric drip feeding. Patients were divided into responders (n = 16) and non-responders (n = 4) depending on their (change in) SDS (standard deviation score) for height. Liver adenomas were detected in 3 patients, of which one was a non-responder. Alpha-fetoprotein was normal in all patients.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:The long-term outcome of patients with glycogen storage disease type Ia. 831 26

Type Ia Glycogen storage disease is an autosomal recessive hepatic metabolic disease due to a lack of glucose-6-phosphatase (G-6-Pase) activity presenting with growth retardation, lactic acidosis, fasting hypoglycemia with hypoinsulinemia, hyperuricemia, hepatomegaly, and hepatic adenoma with a risk of malignancy. The gene that encodes G-6-Pase was mapped to 17q21. There are some genotype-phenotype correlations. We report a case with delF327 mutation which is devoid of G-6-Pase activity; however clinical presentation in this case differs somewhat. Although correction of hypoglycemia and lactic acidosis with nocturnal intragastric feeding and uncooked starch therapy improves growth failure, mean height of the patients is often less than the target. Normal height and obesity in this case with hepatic steatosis and low hepatic glycogen storage requires clinical reevaluation since there are some overlapping phenotypes between type Ia GSD and metabolic syndrome. The phenomenon may be related to insulin resistance as a consequence of early aggressive nutrition therapy with frequent low glycemic index meals.
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PMID:Obesity and reversed growth retardation in a child with type Ia glycogen storage disease. 2066 51

Hepatic adenoma is a well-defined, benign, solitary tumor of the liver. In individuals with glycogen storage disease I, adenoma tends to occur at a relatively younger age and can be multiple (adenomatosis). Imaging plays a pivotal role in diagnosing hepatic adenoma and in differentiating adenoma from other focal hepatic lesions. Especially in patients with von Gierke disease, in addition to the associated hepatomegaly caused by steatohepatitis and the diffusely reduced attenuation of the liver parenchyma seen on CT, there may be more than one hepatic adenoma in up to 40% of patients. Malignant degeneration of hepatic adenoma into hepatocellular carcinoma can occur and hence imaging is important for prompt diagnosis of adenoma and its complications. In this case report, we present a case of liver adenoma diagnosed by CT scan in a patient with von Gierke disease.
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PMID:CT scan diagnosis of hepatic adenoma in a case of von Gierke disease. 2262 17

Glycogen storage disease Ib is a rare, inherited metabolic disorder caused by glucose-6-phosphatase translocase deficiency. Its main symptoms are hypoglycemia, hyperlipidemia, neutropenia, hepatomegaly, liver adenomas and short stature. The exact mechanism of short stature in this disease is unclear, the most feasible possibility is that it is caused by impairment of growth-hormone and insulin-like growth factor I axis. Here we report the case of a patient who showed typical symptoms of glycogen storage disease Ib since his infancy, his height being under 1 percentile since then. Later-developed hypothyroidism and hypogonadism have also contributed to his short stature. Hypothyroidism was treated but sexual steroid substitution was not started because of an increased risk of hepatic adenomas. Because he developed hepatic adenoma at the age of 23, he had to undergo orthotopic liver transplantation. At the time of the transplantation his height was 128cm. The transplantation was followed by rapid height growth; our patient's height reached 160.3cm 62months after transplantation. We observed that while his IGF-I level increased, his GH level remained unchanged. During the post-transplantation period we ensured adequate calcium and vitamin D supplementation, leaving hormonal substitution unchanged. According to our knowledge, this is the first report of a rapid height growth as big as 32cm, of an individual over the age of 20, not related to endocrine treatment but liver transplantation.
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PMID:Rapid height growth after liver transplantation in adulthood. 2704 Oct 87


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