Glycogen storage disease type Ia (GSD-Ia; also called von Gierke disease) is an autosomal recessive disorder of carbohydrate metabolism caused by glucose-6-phosphatase deficiency. There have been many reports describi...Glycogen storage disease type Ia (GSD-Ia; also called von Gierke disease) is an autosomal recessive disorder of carbohydrate metabolism caused by glucose-6-phosphatase deficiency. There have been many reports describing hepatic tumors in GSD patients; however, most of these reports were of hepatocellular adenomas, whereas there are only few reports describing focal nodular hyperplasia (FNH) or hepatocellular carcinoma (HCC). We report a case with GSD-Ia who had undergone a partial resection of the liver for FNH at 18 years of age and in whom moderately differentiated HCC had developed. Preoperative imaging studies, including ultrasonography, dynamic computer tomography (CT) and magnetic resonance imaging, revealed benign and malignant features. In particular, fluorodeoxyglucose-positron emission tomography (FDG-PET)/CT revealed the atypical findings that FDG accumulated at high levels in the non-tumorous hepatic parenchyma and low levels in the tumor. Right hemihepatectomy was performed. During the perioperative period, high-dose glucose and sodium bicarbonate were administered to control metabolic acidosis. He had multiple recurrences of HCC at 10 mo after surgery and was followed-up with transcatheter arterial chemoembolization. The tumor was already highly advanced when it was found by chance; therefore, a careful follow-up should be mandatory for GSD-I patients as they are at a high risk for HCC, similar to hepatitis patients.展开更多
Background of the Study, Aims and Objectives: There are very few studies on histological patterns of diabetic nephropathy in our part of country. The aim of this study was to evaluate the renal involvement in patients...Background of the Study, Aims and Objectives: There are very few studies on histological patterns of diabetic nephropathy in our part of country. The aim of this study was to evaluate the renal involvement in patients with Type-2 diabetes mellitus (T2DM), assess the histopathological changes and establish a clinico-pathological correlation. Subjects, Method and Materials: Thirty two Type 2 DM patients with nephropathy, after screening consecutive hundred(100) Type 2 Diabetics admitted to the Medicine Department were evaluated for renal involvement by kidney biopsy and histopathological study. Statistical analysis was done by student’s t-test, chi-square and linear regression analysis. Results: Thirty two patients (32) with diabetic nephropathy (20 males and 12 females) formed the study group out of hundred (100) consecutive Type-2 diabetes mellitus patients (58 males and 42 females) admitted to Medicine Department of SCB Medical College Hospital, Cuttack. The frequency of occurrence of clinical diabetic nephropathy was 32%. Most of the patients were having duration of DM of 6-10 years (87.5%). Pedal edema was found in 96.87%, hypertension in 87.5% patients respectively. Regression analysis showed that durations of DM and HbA1c were the two significant risk factors (P Histopathologically, diffuse glomerulosclerosis was the most common form of renal abnormality found in 93.75% followed by nodular glomerulosclerosis in 62.50% with overlap in many patients, membranous nephropathy in 12.5% and focal necrotising glomerulonephritis in 6.25% respectively. There was no statistically significant clinicopathological correlation observed between clinical and biochemical parameters in patients harbouring the two predominant histological types of nephropathy i.e. diffuse and nodular glomerulosclerosis with respect to age, sex, duration of diabetes, body mass index, systolic blood pressure, HbA1c, 24 hour urinary protein, creatinine clearance, serum urea, serum creatinine or lipid profile. Conclusion: Durations of diabetes and HbA1c were found to be strongly associated with development of diabetic nephropathy. Diffuse glomerulosclerosis was the most common form of renal abnormality found in 93.75% followed by nodular glomerulosclerosis in 62.50% of patients.展开更多
文摘Glycogen storage disease type Ia (GSD-Ia; also called von Gierke disease) is an autosomal recessive disorder of carbohydrate metabolism caused by glucose-6-phosphatase deficiency. There have been many reports describing hepatic tumors in GSD patients; however, most of these reports were of hepatocellular adenomas, whereas there are only few reports describing focal nodular hyperplasia (FNH) or hepatocellular carcinoma (HCC). We report a case with GSD-Ia who had undergone a partial resection of the liver for FNH at 18 years of age and in whom moderately differentiated HCC had developed. Preoperative imaging studies, including ultrasonography, dynamic computer tomography (CT) and magnetic resonance imaging, revealed benign and malignant features. In particular, fluorodeoxyglucose-positron emission tomography (FDG-PET)/CT revealed the atypical findings that FDG accumulated at high levels in the non-tumorous hepatic parenchyma and low levels in the tumor. Right hemihepatectomy was performed. During the perioperative period, high-dose glucose and sodium bicarbonate were administered to control metabolic acidosis. He had multiple recurrences of HCC at 10 mo after surgery and was followed-up with transcatheter arterial chemoembolization. The tumor was already highly advanced when it was found by chance; therefore, a careful follow-up should be mandatory for GSD-I patients as they are at a high risk for HCC, similar to hepatitis patients.
文摘Background of the Study, Aims and Objectives: There are very few studies on histological patterns of diabetic nephropathy in our part of country. The aim of this study was to evaluate the renal involvement in patients with Type-2 diabetes mellitus (T2DM), assess the histopathological changes and establish a clinico-pathological correlation. Subjects, Method and Materials: Thirty two Type 2 DM patients with nephropathy, after screening consecutive hundred(100) Type 2 Diabetics admitted to the Medicine Department were evaluated for renal involvement by kidney biopsy and histopathological study. Statistical analysis was done by student’s t-test, chi-square and linear regression analysis. Results: Thirty two patients (32) with diabetic nephropathy (20 males and 12 females) formed the study group out of hundred (100) consecutive Type-2 diabetes mellitus patients (58 males and 42 females) admitted to Medicine Department of SCB Medical College Hospital, Cuttack. The frequency of occurrence of clinical diabetic nephropathy was 32%. Most of the patients were having duration of DM of 6-10 years (87.5%). Pedal edema was found in 96.87%, hypertension in 87.5% patients respectively. Regression analysis showed that durations of DM and HbA1c were the two significant risk factors (P Histopathologically, diffuse glomerulosclerosis was the most common form of renal abnormality found in 93.75% followed by nodular glomerulosclerosis in 62.50% with overlap in many patients, membranous nephropathy in 12.5% and focal necrotising glomerulonephritis in 6.25% respectively. There was no statistically significant clinicopathological correlation observed between clinical and biochemical parameters in patients harbouring the two predominant histological types of nephropathy i.e. diffuse and nodular glomerulosclerosis with respect to age, sex, duration of diabetes, body mass index, systolic blood pressure, HbA1c, 24 hour urinary protein, creatinine clearance, serum urea, serum creatinine or lipid profile. Conclusion: Durations of diabetes and HbA1c were found to be strongly associated with development of diabetic nephropathy. Diffuse glomerulosclerosis was the most common form of renal abnormality found in 93.75% followed by nodular glomerulosclerosis in 62.50% of patients.
