Amyloidosis is not a single disease, but a series of diseases in which there is extracellular deposition of a protein in an abnormal fibrillar form. We report a 48-year old woman with subicteric coloration of scleras and hepatomegaly. Functional liver tests evidenced a high level of alkaline phosphatase and serum gamma-glutamyl transpeptidase with mild increase of bilirubin level. Color Doppler ultra-sonography, showed a hyperkinetic portal hypertension. The liver biopsy found amyloid in sinusoids subendothelially. Immunohistochemical staining for anti lambda light chain immunoglobulin was positive. Bone marrow morphology and immunohistochemistry confirmed lymphoplasmocytic lymphoma with amyloidosis.
Download full-text PDF |
Source |
---|
Ann Pediatr Cardiol
March 2022
Department of Imaging Sciences and Intervention Radiology, Sree Chitra Tirunal Insitute for Medical Sciences and Technology, Thiruvananthapuram, Kerala, India.
An 11-month-old girl was detected to have muscular ventricular septal defect with hyperkinetic pulmonary hypertension (PH) was urgently operated upon. On follow-up, her PH worsened, resulting in right ventricular dysfunction and was later detected to have absent portal vein.
View Article and Find Full Text PDFIn chronic liver disease, the incidence of cirrhosis is increasing. About 1 million deaths from cirrhosis are reported annually by WHO, occupying the 11th position in the hierarchy of pathologies that cause death (1). The prevalence of cirrhosis is often underestimated based on the fact that one third of the patients are asymptomatic (2).
View Article and Find Full Text PDFTremor Other Hyperkinet Mov (N Y)
September 2020
INSERM, U 1127, CNRS UMR 7225, Faculté de Médecine de Sorbonne Université, UMR S 1127, Institut du Cerveau et de la Moelle épinière, ICM, Hôpital Pitié-Salpêtrière, 47-83 Boulevard de l'Hôpital, 75013 Paris, FR.
Background: Genes involved in Tourette syndrome (TS) remain largely unknown. We aimed to identify genetic factors contributing to TS in a French cohort of 120 individuals using a combination of hypothesis-driven and exome-sequencing approaches.
Methods: We first sequenced exons of and in the TS cohort and subsequently sequenced the exome of 12 individuals harboring rare variants in these genes to find additional rare variants contributing to the disorder under the hypothesis of oligogenic inheritance.
Cardiovasc Intervent Radiol
December 2015
Department of Radiology, University of Michigan Medical Center, 1500 E. Medical Center Dr., Ann Arbor, MI, 48109, USA.
Refractory ascites (RA) is thought to complicate the postoperative course of 5-7% (Nishida et al. in Am J Transplant. 6: 140-149, 2006; Gotthardt et al.
View Article and Find Full Text PDFHepatol Int
June 2013
Department of Gastroenterology, G. B. Pant Hospital, New Delhi, 110001, India.
Aims: To evaluate the participation of nitric oxide (NO) and prostaglandin (PGI2) on hyperdynamic state in endotoxemia-induced portal hypertension (EIP) induced by chronic endotoxemia.
Methods: The portal pressure (PP) and mean arterial pressure (MAP) were recorded, in vivo before and after administration of L-NAME (NOS inhibitor) and indomethacin (specific blocker of COX). The vasoactive responses to acetylcholine of thoracic rat aortic rings were studied in vitro before and after nitric oxide and cyclooxygenase blockade using multichannel organ bath.
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!