We have experienced a case of hepatoblastoma in 22 months old male infant who presented with abdominal distention and hepatomegaly. Diagnosis was confirmed by X-ray of abdomen, liver scan and histological findings. A brief review of literature was also presented.
Abdomen ; Diagnosis ; Hepatoblastoma* ; Hepatomegaly ; Humans ; Infant ; Liver ; Male

It is essential to estimate the liver size in the diagnosis of liver disease. Many approaches have beenattempted in the evaluation of liver size such as measurement of length, area and volume. Among these, area andvolume measurements are accurate but complicated. So various linear measurements including Pfahler's method havebeen commonly used. But to our knowledge, there was no report about linear diameter of liver in Korean adualts. Asingeneral, larger patients are likely to have larger liver than smaller ones, it seems to be more ideal toevaluate the liver size using relative ratio rather than absolute linear diameters. The main objectives of ourinvestigation were to determine the various diameters of normal and enlarged liver and the criteria ofhepatomegaly in Korean adults using absolute and relative ments. Our cases consisted of 95 clinically normalsubjects and 51 patients suffering from liver disease and diagnosed to have hepatomegaly on abdominal palpationand simple abdomen in the Dept. of Radiology, St, Mary Hospital during the period of 6 months since Jan. 1981. Wemeasured the liver size using 3 linear diameters. And as the reference measurement, the distance from the rightmargin of the liver to the left margin of spleen was also measured. We called this “abdominal transversediameter”(ATD). The results were as follows; 1. The diameters of liver were 13.4±1.6cm, 18.4±2.4cm, 19.2±2.6cmin normal group and 18.8±3.1cm, 23.5±3.0cm, 24.2±3.2cm in hepatomegaly group using midline verticaldiameter(MIVD), maximum vertical diameter (MAVD) and diagonal diameter(DD), respectively. The difference betweentwo groups were statistically very significant in every method(p<0.01). 2. The 99% tolerance limits of liverdiameters were 13.0-13.8cm, 17.8-19.0cm, 18.5-19.9cm in normal and DD, respectively. The midpoints between theupper limit of normal group and the lower limit spectively. These points are warranted to suggest criterias ofhepatomegaly. 3. There were statistical significant difference in the ratio of each diameter to ATD between normaland hepatomegaly group (p<0.01). We called this“hepato-abdominal ratio”. The “hepato-abdominal in hepatomegalygroup using MIVD, MAVD and DD, respectively. 4. The 99% tolerance limits of “hepato-abdominal ratio” were0.43-0.45, 0.59-0.63, 0.62-0.64 in normal group and 0.60-0.62, 0.75-0.77, 0.77-0.79 in hepatomegaly group usingMAVD, MAVD and DD, respectively. The midpoints between the upper limit of normal group and the lower limit ofhepatomegaly group were 0.52, 0.69, 0.70 using MIVD, MAVD and DD, respectively. These points are alsowarranted tosuggest criterias of hepatomegaly.
Abdomen ; Adult ; Diagnosis ; Hepatomegaly ; Humans ; Liver Diseases ; Liver ; Methods ; Spleen

A boy aged 11 years was admitted due to intermittent weakness and difficulty in walking for 6 years, and hepatomegaly, glycopenia and unconsciousness for 4 years. The laboratory examinations showed severe metabolic acidosis, hypoglycemia, and abnormal liver function. CT scan showed marked liver enlargement with fat density shadow. The boy was given fluid infusion, correction of acidosis, intravenous injection of glucose, L-carnitine, compound vitamin B, and coenzyme Q10, but he was in a persistent coma and it was difficult to correct refractory metabolic acidosis and hypoglycemia. The boy died. Blood and urinary organic acid screening and gene detection confirmed that the boy had late-onset glutaric aciduria type II (GAIIc) caused by electron-transferring-flavoprotein dehydrogenase (ETFDH) gene defect. GAIIc is an inherited metabolic disease with a low incidence, resulting in a high misdiagnosis rate. GAIIc should be considered for children with recurrent weakness or reduced activity endurance, hypoglycemia, and marked liver enlargement with abnormal liver function. Urinary organic acid analysis and blood tandem mass spectrometry can help with the early diagnosis of GAIIc, and ETFDH gene analysis helps to make a confirmed diagnosis.
Child ; Hepatomegaly ; etiology ; Humans ; Hypoglycemia ; etiology ; Male ; Multiple Acyl Coenzyme A Dehydrogenase Deficiency ; diagnosis ; Muscle Weakness ; etiology

The authors describe three cases of hepatocellular carcinoma presenting with the initial manifestation of an intracranial lesion without primary hepatic symptoms and signs. The only clue of hepatic dysfuntion was mild elevation of the SGOT, SGPT, AIK-Pase and hepatomegaly. The diagnosis could not be made until operation. All three cases of hepatoma had metastasis to cranium. The literauture of hepatoma with cranial metastasis is reviewed.
Alanine Transaminase ; Aspartate Aminotransferases ; Carcinoma, Hepatocellular* ; Diagnosis ; Hepatomegaly ; Neoplasm Metastasis* ; Skull

The authors describe three cases of hepatocellular carcinoma presenting with the initial manifestation of an intracranial lesion without primary hepatic symptoms and signs. The only clue of hepatic dysfuntion was mild elevation of the SGOT, SGPT, AIK-Pase and hepatomegaly. The diagnosis could not be made until operation. All three cases of hepatoma had metastasis to cranium. The literauture of hepatoma with cranial metastasis is reviewed.
Alanine Transaminase ; Aspartate Aminotransferases ; Carcinoma, Hepatocellular* ; Diagnosis ; Hepatomegaly ; Neoplasm Metastasis* ; Skull

Systemic amyloidosis results from the deposition of insoluble, fibrous amyloid proteins. It occurs mainly in the extracellular spaces of multiple organs and tissues including the kidney, heart, and liver. Although amyloid deposition in the liver is common in patients with systemic amyloidosis, clinically apparent liver disease is relatively rare. Indeed, most patients with systemic amyloidosis manifest only minimal to moderate hepatomegaly and trivial abnormalities in liver function tests. Recently, we experienced two cases of patients who presented with abnormalities in liver function tests and hepatomegaly as manifestations of systemic amyloidosis. We report these cases with a review of the relevant literatures.
Adult ; Amyloidosis/complications/*pathology ; Female ; Hepatomegaly/complications/*diagnosis/pathology ; Humans ; *Liver Function Tests ; Male ; Middle Aged

In 1953, Lichtenstein suggested to use “Histiocytosis X” as an unifying diagnostic term to represent Eosinophilic granuloma, Hand-Schiiller-Christian disease, and Letterer-Siwe disease. The author experienced a case of Hand-Schuller-Christian disease in six year old girl. She has been treated under the diagnosis of Calves disease. But later, it was confirmed as Hand-Schiiller-Christian disease by clinically and pathologically, involving multiple region of entire skeletal system, but sparing the musculocutaneous and nervous system. Clinically, anemia, malaise, high fever and hepatomegaly were present. Co60 isotope irradiation combined with antibiotics was done for 87 days twice weekly in a dose of 50 R at a time. At the time of discharge clinical symptoms were subsided. In X-Ray check, one year after treatment, bony defects were consolidated with new bone and the result was successful.
Anemia ; Anti-Bacterial Agents ; Diagnosis ; Eosinophilic Granuloma ; Female ; Fever ; Hepatomegaly ; Histiocytosis, Langerhans-Cell ; Humans ; Nervous System

Langerhans cell sarcoma (LCS) is a neoplastic proliferation of Langerhans cells with malignant cytological features and multi-organ involvement that typically has a poor prognosis. We experienced 2 cases of LCS in children less than 2 years of age and report them based primarily on CT and MR findings. Both children had findings of hepatosplenomegaly with low-attenuation nodular lesions, had multiple lymphadenopathy, and had shown recurrent lesions invading the skull during follow-up after chemotherapy.
Female ; Hepatomegaly/diagnosis ; Humans ; Infant ; Langerhans Cell Sarcoma/*diagnosis ; Magnetic Resonance Imaging ; Mediastinal Neoplasms/*diagnosis ; Neoplasm Recurrence, Local ; Skull Neoplasms/*diagnosis ; Splenomegaly/diagnosis ; Tomography, X-Ray Computed

The clinical syndrome of venoocclusive disease of the liver is one of several manifestations of regimen-related toxicity that can occur after high-dose cytoreductive therapy. Hepatic dysfunction after bone marrow transplantation may result from a number of causes such as pretransplant chemoradiation, graft-versus host disease, drugs for prophylaxis of graft-versus host disease, venoocclusive disease, various infections, and infiltration of recurrent malignancy. The clinical distinction of these causes may be difficult and the treatment of each cause is also quite different. Therefore the diagnosis of veno-occlusive disease is important. Veno-occlusive disease affects zone 3 of the liver acinus and produces a syndrome of jaundice, painful hepatomegaly, and fluid retention. Veno-occlusive disease occurs in up to 50% of the patients who undergo BMT and is usually associated with a high mortality rate. In Korea, there are a few case reports on venoocclusive disease after BMT which were only confirmed by clinical symptoms. This is a first report of two cases of hepatic veno-occlusive disease after allogenic BMT, which were proven by laparoscopic liver biopsy in our country.
Biopsy* ; Bone Marrow Transplantation* ; Bone Marrow* ; Diagnosis ; Hepatic Veno-Occlusive Disease* ; Hepatomegaly ; Humans ; Jaundice ; Korea ; Leukemia ; Liver* ; Mortality

The clinical syndrome of venoocclusive disease of the liver is one of several manifestations of regimen-related toxicity that can occur after high-dose cytoreductive therapy. Hepatic dysfunction after bone marrow transplantation may result from a number of causes such as pretransplant chemoradiation, graft-versus host disease, drugs for prophylaxis of graft-versus host disease, venoocclusive disease, various infections, and infiltration of recurrent malignancy. The clinical distinction of these causes may be difficult and the treatment of each cause is also quite different. Therefore the diagnosis of veno-occlusive disease is important. Veno-occlusive disease affects zone 3 of the liver acinus and produces a syndrome of jaundice, painful hepatomegaly, and fluid retention. Veno-occlusive disease occurs in up to 50% of the patients who undergo BMT and is usually associated with a high mortality rate. In Korea, there are a few case reports on venoocclusive disease after BMT which were only confirmed by clinical symptoms. This is a first report of two cases of hepatic veno-occlusive disease after allogenic BMT, which were proven by laparoscopic liver biopsy in our country.
Biopsy* ; Bone Marrow Transplantation* ; Bone Marrow* ; Diagnosis ; Hepatic Veno-Occlusive Disease* ; Hepatomegaly ; Humans ; Jaundice ; Korea ; Leukemia ; Liver* ; Mortality