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Hepatectomy for hepatocellular carcinoma complicated by vasculitis flare
Abdi, Zeinab; Krasnokutsky, Svetlana; Rapkiewicz, Amy; Saxena, Amit; Villanueva, Gerald; Sarpel, Umut
Background. The hepatitis C virus is a major cause of hepatocellular carcinoma. Extrahepatic manifestations of hepatitis C include mixed cryoglobulinemia which can result in ischemic damage to multiple organs. The management of these sequelae in posthepatectomy patients is unclear. Case Report. A 49-year-old male with hepatitis C was found to have a 4 cm hepatocellular carcinoma on surveillance imaging. He underwent portal vein embolization followed by hepatectomy. His postoperative course was complicated by the development of splenic infarcts, small bowel ischemia, skin lesions, and liver damage. Findings of elevated cryocrit and elevated rheumatoid factor suggested the diagnosis of cryoglobulin-related vasculitis. The patient improved on supportive care. Conclusion. Cryoglobulinemia is associated with hepatitis C and may complicate the care of this patient population. The treatment of cryoglobulinemia posthepatectomy patients is complicated by concerns over how medications may affect the regenerating liver. Steroids should be used with caution in this setting. Summary. Brief report of hepatectomy complicated by vasculitis in the context of hepatocellular carcinoma secondary to hepatitis C addresses the management of mixed cryoglobulinemia in post-hepatectomy patients
PMCID:2939435
PMID: 20862343
ISSN: 1607-8462
CID: 112568
Mortality/morbidity in cardiac neonatal lupus and associated maternal/fetal risk factors [Meeting Abstract]
Izmirly P.M.; Saxena A.; Smith Z.; Buyon J.P.
Purpose: The classic cardiac manifestations of neonatal lupus (cardiac-NL) include a spectrum of conduction dysfunction (1<sup>st</sup>, 2<sup>nd</sup>, or 3<sup>rd</sup> degree heart block (CHB)) and more rarely cardiomyopathy which can be absent any conduction disease. This study was undertaken to update the mortality data of cardiac-NL in a large US based cohort and identify associated risk factors to further understand the pathogenesis of anti-SSA/Ro mediated injury and provide evidence based data for counseling women with these antibodies. Methods: Three hundred and one children with cardiac-NL (295 with CHB and 6 with isolated cardiomyopathy) enrolled in the Research Registry for Neonatal Lupus (RRNL) had sufficient medical records for review. The RRNL database was analyzed for the following potential mortality maternal risk factors: age at pregnancy, race/ethnicity, anti-SSB/La antibody status, health status and fetal risk factors: time of diagnosis, exposure to maternal non-fluorinated and fluorinated steroids during pregnancy, method of delivery, and gender. In addition, morbidity was assessed by the frequency of pacemaker placement and cardiac transplant. Results: Follow up of the children ranged from in utero death to adulthood. Of the 301 children with cardiac-NL, 53 (17.6%) died. Thirty percent died in utero or at birth, 41% died prior to six months of postnatal life and the remaining 29% died after 6 months. Mortality was higher among children born to non-Caucasian mothers compared to Caucasian mothers (33% vs 15% p=.0003). Maternal age was equivalent between the groups (29.1 dead vs 29.7 live). The maternal presence of anti-SSB/La antibodies was 74% for those whose children died and 63% for those whose children survived, which was not significant. A maternal diagnosis of Sjorgen's Syndrome and/or Systemic Lupus Erythematosus was not significantly associated with cardiac-NL death (53% in death vs 44%) suggesting that prior knowledge of maternal antibody status did not influence mortality. With regard to fetal factors, 86% of those who died were diagnosed with cardiac-NL during pregnancy compared to 85% who survived. For those diagnosed during pregnancy there was a trend towards early gestational diagnosis for those children that died compared to those that survived (21 vs 23.5 weeks p=.09). There was also a trend toward higher exposure to maternal fluorinated steroids after the diagnosis in children that died (53% vs 40% p=.09), however there was no difference in maternal use of non-fluorinated steroids in those that died vs those that survived (19% vs 16%). Most fetuses were delivered by C-section and this was not significantly associated with death (70% dead vs 75% live). Female gender was also not associated with outcome (49% who died were female vs 52% live). Eighty-five percent of children received a pacemaker, 43% within 9 days of birth, 20% between 9 days and one year. Five children (2%) received a heart transplant. Conclusion: Based on data from this large cohort, 17.6% of children born with cardiac-NL die from complications of the disease. Eighty-five percent required pacing and two percent required cardiac transplantation. Mortality was more prevalent in children born to non Caucasian mothers
EMBASE:70381466
ISSN: 0004-3591
CID: 130944
Long-term impact of radiation on the stem cell and oligodendrocyte precursors in the brain
Panagiotakos, Georgia; Alshamy, George; Chan, Bill; Abrams, Rory; Greenberg, Edward; Saxena, Amit; Bradbury, Michelle; Edgar, Mark; Gutin, Philip; Tabar, Viviane
BACKGROUND: The cellular basis of long term radiation damage in the brain is not fully understood. METHODS AND FINDINGS: We administered a dose of 25Gy to adult rat brains while shielding the olfactory bulbs. Quantitative analyses were serially performed on different brain regions over 15 months. Our data reveal an immediate and permanent suppression of SVZ proliferation and neurogenesis. The olfactory bulb demonstrates a transient but remarkable SVZ-independent ability for compensation and maintenance of the calretinin interneuron population. The oligodendrocyte compartment exhibits a complex pattern of limited proliferation of NG2 progenitors but steady loss of the oligodendroglial antigen O4. As of nine months post radiation, diffuse demyelination starts in all irradiated brains. Counts of capillary segments and length demonstrate significant loss one day post radiation but swift and persistent recovery of the vasculature up to 15 months post XRT. MRI imaging confirms loss of volume of the corpus callosum and early signs of demyelination at 12 months. Ultrastructural analysis demonstrates progressive degradation of myelin sheaths with axonal preservation. Areas of focal necrosis appear beyond 15 months and are preceded by widespread demyelination. Human white matter specimens obtained post-radiation confirm early loss of oligodendrocyte progenitors and delayed onset of myelin sheath fragmentation with preserved capillaries. CONCLUSIONS: This study demonstrates that long term radiation injury is associated with irreversible damage to the neural stem cell compartment in the rodent SVZ and loss of oligodendrocyte precursor cells in both rodent and human brain. Delayed onset demyelination precedes focal necrosis and is likely due to the loss of oligodendrocyte precursors and the inability of the stem cell compartment to compensate for this loss.
PMCID:1913551
PMID: 17622341
ISSN: 1932-6203
CID: 161084