• The Journal of the Korean dental association
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• v.17 no.4 s.119
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• pp.291-296
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• 1979

This patient, 30 year-old korean male, was admitted with complaints of the gingival enlargement and spontaneous bleeding of the gingival tissue with acute leukemia. In reviewing this case, the following points should have been understood; 1. This is a case of the gingivitis, gingival enlargement, gingival bleeding, so the patient showed the masticatory disturbances. 2. The generalized weakness, petechia in skin, anemia, and hepatomegaly on palpation were seen. 3. With the histopathological examination by means of microscopic views, numerous myelocytic cells, of which their shapes were in irregular, were appeared. 4. The prognosis was poor in spite of any other internal treatments.

• Journal of Chest Surgery
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• v.22 no.3
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• pp.456-462
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• 1989

We experienced one case of the calcified giant thrombosis in the enlarged right atrium and coronary sinus with markedly dilated persistent left superior vena cava and absent right superior vena cava in 29year old female patient. We supposed that the dilatation of persistent left superior vena cava was due to poststenotic dilatation secondary to obstruction on orifice of coronary sinus by thrombosis. The giant thrombosis in the right atrium and coronary sinus was successfully resected. She had improvement on preoperative chest discomfort but, the moderate hepatomegaly was developed and then she was discharged with incompletely recovered state due to economical poverty on postoperative 6th weeks. The continuous follow-up and study are indeed necessary for further evaluation of pathology and etiology.

• Journal of Veterinary Clinics
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• v.19 no.3
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• pp.337-341
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• 2002

In 2001, eight small-sized dogs and three medium-sized dogs with or without clinical signs such as vomiting, dyspnea and enlargement of superficial lymph nodes were diagnosed as lymphoma by radiography, ultrasonography and cytology. Among them, six were female mainly over 7 years old and others were male around 4 years old. There were six cases of multicentric form lymphoma, three cases of mediastinal form, an alimentary and an extranodal form, respectively. Radiographic findings were enlarged cranial mediastinal lymph node, sternal lymph node, and tracheobronchial lymph nodes on thorax and hepatomegaly, splenomegaly, and enlarged sublumbar lymph nodes on abdomen. Ultrasonographic findings revealed enlarged abdominal lymph nodes and multifocal hypoechoic lesions on the liver. Especially, severe wall thickness of small bowel was observed in a dog with alimentary lymphoma. Three dogs were dead during chemotherapy, and three dogs were dead without any treatment. Three dogs did not return and two treated dog showed normal status. Radiography and ultrasonography, although not giving an final diagnosis for lymphoma, are useful for assessment and diagnosis of lymphoma.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.5 no.1
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• pp.88-90
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• 2002

Chilaiditi's sign is a radiographic term used when the hepatic flexure of colon is seen interposed between the liver and right hemidiaphragm. Mostly asymptomatic, Chilaiditi's syndrome can present with abdominal pain, nausea, vomiting, anorexia, abdominal distension, tender hepatomegaly and change in bowel habits. Uncommon in childhood, the incidence seems to increase with age. We have experienced a case of Chilaiditi's syndrome presenting with vomiting in a 15-month-old boy. The patient recovered uneventfully.

• Clinical and Experimental Pediatrics
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• v.52 no.11
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• pp.1279-1282
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• 2009

Hepatomegaly and liver dysfunction might develop in patients with diabetes mellitus due to glycogen deposition or nonalcoholic steatohepatitis. We experienced a case of hepatic glycogenosis in a patient with type 1 diabetes mellitus who presented with recurrent hypoglycemia, suggesting impairment of glycogenolysis and gluconeogenesis. A 10-year-old girl with a 4-year history of type 1 diabetes mellitus was admitted because of recurrent hypoglycemia and abdominal pain in the right upper quadrant. She had Cushingoid features and hepatomegaly that extended 6 cm below the right costal margin. Laboratory data and radiologic examination revealed elevated liver enzyme levels due to fatty liver. Periodic acid-Schiff (PAS) staining revealed intense glycogen deposition in the cytoplasm of the hepatocytes and PAS reactivity was lost with diastase treatment. At 2 months after administration of glucagon injection and uncooked cornstarch between meals and at bedtime, the hypoglycemic episodes and liver dysfunction improved. It is important to distinguish hepatic glycogenosis from steatohepatitis, because it is possible to prevent excessive hepatic glycogen storage in hepatic glycogenosis cases by strictly controlling blood glucose level and by glucagon administration. To prevent severe hypoglycemic symptoms accompanied by hepatic glycogenosis, we suggest that uncooked cornstarch, which is effective in maintaining blood glucose level, can also be administered.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.20 no.1
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• pp.24-28
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• 2020

Type III Glycogen storage disease (Type III GSD, OMIM#232400) is a genetic metabolic disorder in which undigested glycogen accumulates in the organs due to lack of glycogen debranching enzyme caused by AGL mutation. The clinical symptoms of type III GSD include hepatomegaly, delayed growth, hypoglycemia and muscle weakness. These clinical symptoms are similar to those of other types of GSD, making it difficult to distinguish clinically. The authors report a case of type III GSD diagnosed by gene panel sequencing. A 11-month old male patient was presented with hepatomegaly. In liver biopsy, glycogen was accumulated in hepatocytes, suggesting GSDs. For differential diagnosis of types of GSD, gene panel sequencing for GSDs was performed. As a result, two novel pathogenic compound heterozygous variants: c.311_312del (p.His104Argfs*15) and c.3314+1G>A in AGL were detected and the patient was diagnosed as type III GSD. After diagnosis, he started dietary treatment with cornstarch, and has been free from complications. After two years, two same variants were also identified in the chorionic villous sampling of the pregnant mother, and the fetus was diagnosed as type III GSD. Gene panel sequencing is useful for diagnosis of disease which is indistinguishable by clinically and has high genetic heterogeneity, such as GSD. After diagnosis, familial genetic analysis can provide adequate genetic counseling and rapid diagnosis.

• Asian Pacific Journal of Cancer Prevention
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• v.17 no.1
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• pp.215-217
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• 2016

Background: Chronic lymphocytic leukemia (CLL) is the most frequent form of leukemia in Europe and North America, and it mainly affects older individuals. Many approaches are implemented in the management of CLL from a watch and wait (W&W) strategy to chemotherapeutic regimens. We here reviewed our clinical practice for the relevance of the W&W strategy in Lebanese CLL patients. Materials and Methods: A total of 95 patients with CLL diagnosed in four institutions in Lebanon, between 1992 and 2013, were selected and their files were reviewed. Characteristics of these patients were noted including age, sex, RAI and Binet scores, CBC values, presence of hepatomegaly or splenomegaly, performance of bone marrow biopsy or peripheral blood flux cytometry for diagnosis, adoption of W&W strategy, different chemotherapeutic regimens and the indications for treatment. Results: Some 38 patients (40%) diagnosed with CLL were women and 57 (60%) were men with a mean age of 65.1 years [36-89]. Of the total, 50.5%, 17.2%, 14%, 7.5% and 10.8% had an RAI score at diagnosis of 0, 1, 2, 3 and 4, respectively, while 65.6%, 17.2% and 17.2% had Binet scores of I, II and III. The mean lymphocyte count at diagnosis was $39885/mm^3$ [1596-290000], the mean hemoglobin level was 12.7 g/dl [6.2-17] and the mean platelet count was $191255/mm^3$ [14000-458000]. While 26.3% of patients with CLL had splenomegaly, only 7.4% had hepatomegaly. Some 33.7% had undergone a bone marrow biopsy, 66.3% flow cytometry of circulating blood and 5.3% a lymph node biopsy. Overall, the W&W was adopted in 62.4% (58) of patients with a mean duration of 37.7 months [3-216]. The W&W was used in 82.6%, 73.3%, 46.2%, 14.3% and 0% of patients having RAI scores of 0, 1, 2, 3 and 4, respectively, and, it was used in 80%, 46.7% and 6.25% with Binet scores of I, II and III. The most frequent indication for treatment was anemia and thrombocytopenia, accounting for 32.7% of cases. The most frequently used chemotherapeutical regimens were chlorambucil until the end of the last century and flufarabine-cyclophosphamide-rituximab during the last decade. Conclusions: This retrospective review of CLL clinical practice showed an important implementation of the W&W strategy with a long duration, especially in early stage cases with low RAI or Binet scores.

• Journal of The Korean Society of Inherited Metabolic disease
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• v.15 no.1
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• pp.18-24
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• 2015

Glycogen storage disease type Ib (GSD Ib) is one of the rare inherited metabolic disease caused by mutation of SLC37A4 gene. Clinical characteristics include hepatomegaly, hypoglycemia, lactic acidosis, hyperlipidemia and high serum uric acid concentration. The authors analyzed clinical and molecular characteristics of three Korean patients (one male and two females) with GSD Ib by retrospective review of medical records. Two patients were diagnosed in toddler period by hypoglycemia and hepatomegaly. One patient was diagnosed by growth retardation and short stature in puberty. c.412T>C (p.Trp138Arg) (3/6 alleles, 50.0%) was most frequently observed, following by p.Leu348Valfs*53 (1 allele), p.Pro191Leu (1 allele), p.Ala148Val (1 allele) in molecular analysis. Uncooked corn starch and allopurinol was administered. Because all three patients had neutropenia and recurrent infections, G-CSF was administered. Two patients had severe osteoporosis needing calcium supplement. The patient who diagnosed at puberty had relatively poor prognosis demonstrated by having severe infection and complications in liver and kidney.

• Journal of Chest Surgery
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• v.14 no.1
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• pp.26-32
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• 1981

Three cases of tricuspid atresia were treated by Fontan varieties of operation in this department in 1980. The first case was 19 year old girl who underwent Glenn operation at the age of 6 years. Her second operation was done with ASD closure and 16mm Ionescu-Shiley valved conduit insertion between right atrium and main pulmonary artery. The second case was a 5 year old boy who underwent Kreutzer operation successfully utilizing 14mm Ionescu-Shiley va]ved conduit. The above mentioned 2 cases were Type Ib after Keith`s classification, whose immediate postoperative courses were complicated by pleura] effusion [in 2nd case chylothorax] hepatomegaly, and ascites. Those complications were relieved completely by medical treatment and closed thoractomy; Postoperative follow-up up to 11 months and 1 year periods were satisfactory with disappearance of cyanosis and dyspnea. The third case was a 8 year old boy who had complete TGA with TA [Keith`s Type IIb] who underwent Kreutze`s operation utilizing 14mm Ionescu-Shiley valved conduit, he died of low cardiac output Immediately after open heart surgery.

• Environmental Mutagens and Carcinogens
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• v.17 no.2
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• pp.78-91
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• 1997

Peroxisome is a single membrane-bounded organelle found in hepatic parenchymal cells and kidney tubular epithelial cells. A number of enzymes exist in peroxisome contributing to anabolic and catabolic peroxisomal functions. Extramitochontriai $\beta$-oxidation of fatty acid is a major function of peroxisome. Peroxisomes can be proliferated by many structually unrelated compounds such as hypolipidemic drugs, plasticizers, pesticides, some pharmaceutical agents and high fat diet. These chemicals, called peroxisome proliferators, act via the peroxisome proliferator activated receptor, to induce peroxisome proliferation, hepatomegaly and hepatocellular carcinoma in rodent. The clear mechanisms of peroxisome proliferator-induced hepatocarcinogenesis have been not demonstrated. Since they are not genotoxic, biochemical changes or changes in gene expressions may be involved. A free radical theory has been suggested based on the finding of oxidative damages of macromolecules by hydrogen peroxide released in the peroxisomal $\beta$-oxidation of fatty acid. Increased cell proliferation by a peroxisome proliferator has been also thought to be an important factor in the hepatocarcinogenesis as suggested in other cases of nongenotoxic carcinogenesis. The alternation of eicosanoid concentrations by peroxisome proliferators may be important in the peroxisome proliferator-induced hepatocarcinogenesis since peroxisome proliferators decrease the concentration of eicosanoids, and the peroxisome proliferator ciprofibrate-eicosanoid combination is comitogenic and costimulates some mitogenic signals in hepatocytes. All of proposed mechanisms should be considered in the peroxisome prolifrator-induced hepatocarcinogenesis.