"Computed tomography can show normal hepatic parenchyma, numerous small nodules, or larger coalesced nodules spanning several centimeters. On nuclear medicine imaging, these lesions may take up sulfur colloid but will remain iso-or hypodense in both arterial and portal venous phases, which can distinguish NRH from FNH . The use of MRI to enhance diagnostic accuracy is still controversial. "
[Show abstract][Hide abstract] ABSTRACT: Nodular regenerative hyperplasia (NRH) is an uncommon condition, but an important cause of noncirrhotic intrahepatic portal hypertension (NCIPH), characterized by micronodules of regenerative hepatocytes throughout the liver without intervening fibrous septae. Herein, we present a case of a thirty-seven-year-old female with systemic lupus erythematosus (SLE) who was discovered to have significant esophageal varices on endoscopy for dyspepsia. Her labs revealed a slight elevation in the alkaline phosphatase and mild thrombocytopenia. Abdominal MRI revealed seven focal hepatic masses, splenomegaly, no ascites, and a patent portal vein. Ultrasound-guided core biopsy was reported as focal nodular hyperplasia. However, her varices persisted despite treatment with beta-blockers and four additional upper endoscopies with banding. She was subsequently referred for a surgical opinion. At that time, given her history of SLE, azathioprine use, and portal hypertension, suspicion for NRH was raised. Given her normal synthetic function and lack of parenchymal liver disease, the patient was offered surgical shunting. During shunt surgery, a liver wedge biopsy was also performed and this confirmed NRH. An upper endoscopy six weeks after shunting verified complete resolution of varices. Currently, fifteen months after surgery duplex ultrasonography demonstrates shunt patency and the patient is without recurrence of her portal hypertension.
Case Reports in Medicine 08/2012; 2012:965304. DOI:10.1155/2012/965304
[Show abstract][Hide abstract] ABSTRACT: Cystinosis is a rare autosomal-recessive disorder characterized by the intralysosomal accumulation of cystine, which is responsible for widespread tissue destruction. Liver biopsy specimens of patients with cystinosis show cystine crystal formation in Kupffer cells. However, significant liver disease and portal hypertension is not a common complication of cystinosis. We report the case histories of 2 young men with poorly treated nephropathic cystinosis who developed noncirrhotic portal hypertension with evidence of nodular regenerative hyperplasia (NRH).
Liver biopsy examinations, upper and lower endoscopy with biopsy examination, imaging studies, venous pressure measurements, and laboratory investigations were used to evaluate the causes of the liver disease and portal hypertension.
Histologic examination of liver biopsy specimens from both patients showed changes characteristic of NRH with portal hypertension documented by measurement of pressure gradients. In addition, endoscopy in the first patient showed varices and portal hypertensive gastropathy.
NRH was confirmed by histologic examination of the liver in both patients and is the likely cause of their portal hypertension. NRH may represent a rare, late complication of cystinosis, although the mechanism remains undefined.
[Show abstract][Hide abstract] ABSTRACT: The association of biliary atresia (BA) and idiopathic thrombocytopenic purpura (ITP) is extremely rare, with only 2 cases being reported in the literature. This report describes the very rare case of a 1-year-old boy with BA complicated with ITP after cholangitis and the successful steroid treatment of ITP. We review the literature on this type of relationship between BA and ITP and discuss the clinical features of this complication. Furthermore, the possible cause of the onset of ITP complicated with BA was explored in this report.
Journal of Pediatric Surgery 02/2009; 44(1):e21-3. DOI:10.1016/j.jpedsurg.2008.09.034 · 1.31 Impact Factor
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