International Biological and Biomedical Journal
Volume:6 Issue: 3, Summer 2020

In Budd-chiari syndrome (BCS), obstruction of the hepatic venous outflow tract occurs at a site from the small hepatic veins to the junction of the inferior vena cava with the right atrium, and this syndrome can have various causes. Remarkably, BCS can be classified as primary or secondary; primary BCS is caused by venous thrombus or phlebitis, while secondary BCS is due to venous compression or invasion of an extrinsic lesion, such as a tumor, abscess, or cyst. Antiphospholipid syndrome (APS) is an acquired cause of primary BCS.

Case Report:

A 24-year-old man, with complaints of abdominal pain, abdominand anorexia starting three months earlier, was admitted to Rasht Razi Hospital. The patient's pain did not relate to feeding, defecation and positioning. The patient had nausea and vomiting half an hour after feeding. He also mentioned a weight loss of about 5 kg and also,had blood pressure. He had no history of smoking, alcohol and opiums and had not any specific illness in his family. Due to abdominal distension and hepatosplenomegaly, an ultrasound examination was performed for the patient, ascites were diagnosed and the patient was subjected to ascites fluid paracentesis. High gradient of albumin and low protein were reported. The patient was first examined for cardiac examination and heart echocardiography and other examinations were reported as normal. A CT scan of the abdomen, pelvic and doppler ultrasonography was performed that showed ascites and enlargement of the caudate lobe. The findings were compatible with BCS. The diagnosis of BCS was confirmed by a hemodynamic study, and further investigation for the cause of BCS was positive. Antinuclear antibodies, deficiency of protein C and S were detected. LA was not present and aCL (IgM, IgG) were positive. Moreover, serology was negative for hepatitis B or C and serum protein electrophoresis was demonstrated high levels of gamma globulin. We concluded that the patient had chronic BCS secondary to APS in association with autoimmune hepatitis. Ultimately, the patient was treated with prednisolone 60 mg/daily and warfarin, and was referred to a transplantation-equipped center for liver transplantation.

In conclusion, BCS, although infrequent, is not a rare complication in patients with APS and may be the first clinical manifestation of this syndrome. In this patient, BCS caused by the initiation of AIH and secondary APS.

Protein aggregation is phenomenon wherein protein loses its native structure and aggregates due to the adaption of non-native conformation. Amyloid aggregation formed by the accumulation of various proteins causes many diseases in humans and other organisms. Antioxidants can prevent proteins aggregation. Pulicaria undulata  extract along with phenolic compounds can increase protein stability and prevent the aggregation of proteins. The aim of this study is to investigate the aggregation of reduced κ-casein in the presence and absence of P.undulata extract. Interaction between the extract and the κ-casein was investigated by Thioflavin T fluorescence, intrinsic fluorescence intensity, 1-Anilino-8-naphthalene sulfonic acid( ANS) binding assay and circular dichroism (CD) spectroscopy. The result of ThT binding assay indicated that the increase fluorescence intensity of 1,4- dithiothreithol(DTT)-reduced κ-casein is a result of amyloid fibril formation increased in the presence of different concentrations of P.undulata extract in the lag phase.  This indicates that P.undulata extract  reduces amyloid formation. And fluorescence test of reduced κ-casein indicates a high fluorescence intensity in the absence of P.undulata extract , and the fluorescence decreases when smallest amount of P.undulata extract was used. Using a fluorescence study of far-CD spectrum of κ-casein in the presence and absence of P.undulata extract revealed that the extract prevents changes in secondary structure in the protein. P.undulata extract interacts with the hydrophobic region of κ-casein and prevents hydrophobic-hydrophobic protein interaction and therefore protein aggregation.

Acute Lymphoblastic Leukaemia (ALL) a malignant proliferation of immature lymphoid cells is a biologically heterogeneous disorder with variable outcomes in adults. We presents a case of a 52 year old woman who presented with fever, anaemia and peripheral lymphadenopathy of four weeks duration, for which she was managed for disseminated tuberculosis. Complete blood count and a bone marrow aspiration constituted part of her initial investigations. Complete blood count and examination of bone marrow aspiration cytology films were in keeping with acute lymphoblastic leukaemia (ALL-L3). Patient had supportive treatment and managed with Cyclophosphamide, Vincristine (Oncovin), Cytosine Arabinoside and Prednisolone (COAP Regimen), achieved complete clinical and haematologic remission, and has remained disease free after consolidation and maintenance therapy. No evidence of haematologic or clinical relapse at five years and currently in her ninth year post diagnosis and treatment. With high index of suspicion, timely investigation and referral, satisfactory outcomes are achievable in managing some patients with acute lymphoblastic leukaemia.

The present paper refers to some of the most critical aspects of the management of adult-T cell leukaemia/lymphoma (ATL), which is a rare malignancy that affect the 5-7% of infected with human T cell-lymphotropic virus-1 (HTLV-1). ATL has a geographical distribution that includes countries such as Japan, the Caribbean, Central and South America and sub-Saharan Africa. The ATL management includes the use of antivirals, monoclonal antibodies, chemotherapy, radiotherapy, hematopoietic stem cell transplantation (HSCT) and more modern experimental approach, which are promising as immunotherapy. Despite available therapy the prognosis  of ATL remains poor, but it may improve in the upcoming years with the parallel advancement in the basic medical sciences.