scholarly journals Fibrolamellar liver cancer: the modern concept

2020 ◽  
pp. 134-142
Author(s):  
E. Yu. Antonova ◽  
V. V. Breder ◽  
E. A. Moroz ◽  
K. K. Laktionov ◽  
I. A. Dzhanyan ◽  
...  

Fibrolamellar hepatocellular carcinoma (FLC) is a relatively rare primary liver tumor of unknown etiology (chronic infections with hepatitis B or C viruses, chronic alcohol poisoning, cirrhosis of the liver), which occurs mainly in the young population. FLK is more often diagnosed already at common stages due to the absence of pronounced clinical manifestations in patients for a long time. In pathomorphological diagnostics, FLC is a cluster of large polygonal eosinophilic cells with a clearly defined vesicular nucleus, surrounded by abundant growths of lamellar fibrosis. In most cases, collagen fibers are arranged in parallel strands; in metastatic tumors, collagen fibers can be arranged haphazardly (the so-called “tangled fibers”).For many years, attempts have been made to identify reliable markers for the detection and differential diagnosis of FLC and to determine the cause of tumors in young patients, and the molecular mechanisms of FLC carcinogenesis have been studied. In 2014 during the full transcriptome analysis of FLC samples, the chimeric transcript DNAJB1PRKACA was discovered, which is formed as a result of deletion of a section of chromosome 19 with a size of 400 thousand nucleotide pairs and leads to the fusion of two genes, which Is found in most FLC samples. Surgery is the only curative treatment of this tumor type and radical method of treating the disease. Given that lymph node involvement is an important prognostic factor, complete periportal lymphadenectomy should be performed as part of radical surgery for patients with FLK. The role and possibilities of locoregional and drug-based treatment methods are not fully clear, and the search for effective treatment regimens and potential targets specific to this form of HCR is urgent. Studies show conflicting results for different chemotherapy regimens and the use of targeted therapy. The literature describes isolated clinical cases of successful use of immunotherapy in patients with PD-L1-expressing tumors. The most important condition for successful treatment is an in-depth study of the molecular mechanisms of FLC carcinogenesis. This review presents current data on epidemiology, classification, clinico-morphological, molecular and genetic aspects, as well as some diagnostic features and FLC treatment modalities.

2017 ◽  
Vol 25 (2) ◽  
pp. 142-147
Author(s):  
Rabeya Akther ◽  
Humayra Jesmin

Adult onset Still’s disease (AOSD) is a rare systemic inflammatory disease of unknown etiology and pathogenesis that presents in 5 to 10% of patients as fever of unknown origin (FUO) accompanied by systemic manifestations. We report an interesting case of a 63-year-old Bangladeshi male who presented with one-month duration of FUO along with, severe weakness, oral thrush, high WBC count, low RBC count and low hemoglobin concentration. After extensive workup, potential differential diagnoses were ruled out and the patient was diagnosed with AOSD based on the Yamaguchi criteria. The case history, incidence, pathogenesis, clinical manifestations, differential diagnoses, diagnostic workup, treatment modalities, and prognosis of AOSD are discussed in this case report.J Dhaka Medical College, Vol. 25, No.2, October, 2016, Page 142-147


2017 ◽  
Vol 16 (1) ◽  
pp. 57-59
Author(s):  
Shahed Ahmad Chowdhury ◽  
Samira Taufiq Reshma ◽  
Shanjana Islam

Castleman’s disease, also known as angiofollicular lymph node hyperplasia, is a rare disease with two known expansion types, unicentric and multicentric, which plays a major role in determining therapy. The rare multicentric type is a lymphoproliferative disorder of unknown etiology and is characterized by various clinical manifestations and multiple organ involvement. This disease runs a more aggressive course and a poor prognosis. Optimal therapies have not been well established till now. We here report a case of rare Multicentric Castleman's Disease (MCD) in a 21yrs old female. She presented with slowly enlarging lymph nodes in cervical and inguinal regions which lead to a histological diagnosis of this rare condition. Its clinical features, types, relevant investigations and current treatment modalities are discussed. Though rare, early suspicion of this condition may relieve the suffering, avoid unnecessary investigations, give opportunity to choose treatment options and can save lives.Chatt Maa Shi Hosp Med Coll J; Vol.16 (1); Jan 2017; Page 57-59


2012 ◽  
Vol 2012 ◽  
pp. 1-5
Author(s):  
Alejandro Rodríguez Morata ◽  
Ana Hidalgo Conde ◽  
Carlos de la Cruz Cosme ◽  
Susana Gómez Ramírez ◽  
Rafael Gómez Medialdea

Introduction. Behçet's disease (BD) is a form of vasculitis of unknown etiology which is rare in our environment. It is characterized by a variety of clinical manifestations and usually affects young adults. Recurrent oral and genital ulcers are a characteristic and extremely frequent symptom, but mortality is linked with more significant symptoms such as aortic pseudoaneurysm, pulmonary pseudoaneurysm, and cerebral venous thrombosis.Patient and Method. We present a case of a young male with atypical BD and severe polyvascular involvement (previous cerebral venous thrombosis and current peripheral venous thrombosis, acute ischemia, and peripheral arterial pseudoaneurysm) who required urgent surgical intervention due to a symptomatic external iliac pseudoaneurysm.Result. The pseudoaneurysm was successfully treated, we performed an iliofemoral bypass, and we treated it with steroids and immunosuppressive therapy.Conclusions. These rare clinical manifestations highlight the importance of considering BD in young patients, even in usual cases of vascular intervention, whether arterial or venous in nature.


2020 ◽  
Vol 9 (1) ◽  
pp. 56-59
Author(s):  
Prakash Banjade ◽  
Prakash Poudel Jaishi ◽  
Jonas Malla

Adult -onset Still’s disease (AOSD) is a rare, idiopathic, inflammatory disorder of unknown etiology and pathogenesis that presents in 5 to 10% of patients as fever of unknown origin (FUO )exclusion characterized by generalized migratory joint ache, blanchable rash, fever and other systemic manifestations. We report an interesting case of a 23 year old Nepalese lady from Okhaldunga who presented with one-month duration of FUO along with sore throat, fever, vomiting, generalized joint ache, erythematous blanchable rash, headache and visual impairment. On examination there was hepatomegaly and investigations showed raised liver enzymes, serum ferritin and fibrinogen.After extensive workup, potential differential diagnoses were ruled out She was diagnosed to have Adult -onset Still’s disease based on Yamuguchi criteria after exclusion of other potential differentials.The patient partially responded to prednisolone and later methotrexate was prescribed which improved her symptoms. The case history, incidence, pathogenesis, clinical manifestations, differential diagnoses, diagnostic workup, treatment modalities, and prognosis of AOSD are discussed in this case report.


Author(s):  
Zen Ahmad

Corona Virus Disease (Covid-19) is a contagious disease caused by Severe Acute Respiratory Syndrome Coronavirus 2 (SARS-CoV-2) which was discovered in December 2019 in China. This disease can cause clinical manifestations in the airway, lung and systemic. The World Health Organization (WHO) representative of China reported a pneumonia case with unknown etiology in Wuhan City, Hubei Province, China on December 31, 2019. The cause was identified as a new type of coronavirus on January 7, 2020 with an estimated source of the virus from traditional markets (seafood market). ) Wuhan city


GYNECOLOGY ◽  
2020 ◽  
Vol 22 (3) ◽  
pp. 6-10
Author(s):  
Sergey A. Martynov

The aim of the review was to summarize information on clinical manifestations, diagnostic features, as well as methods for correction of scar defects after cesarean section (CS) outside pregnancy. Lack of the scar after CS, niche, isthmocele are a myometrium defect in the area of scar after CS, which is most often detected by ultrasound, sonohysterography or magnetic resonance imaging and is manifested by postmenstrual bleeding from the genital tract. In some cases, it can cause menorrhagia, dyspareunia, pelvic pain, infertility, uterine rupture during subsequent pregnancy and childbirth. Conservative therapy or surgical treatment with laparoscopy, laparotomy or vaginal approach is carried out depending on the symptoms, size of the defect, the thickness of the residual myometrium, as well as the womans reproductive plans.


2020 ◽  
Vol 16 ◽  
Author(s):  
Alexander K. C. Leung ◽  
Joseph M. Lam ◽  
Kin Fon Leong ◽  
Kam Lun Hon

Background: Vitiligo is a relatively common acquired pigmentation disorder that can cause significant psychological stress and stigmatism. Objective: This article aims to familiarize physicians with the clinical manifestations, evaluation, diagnosis, and management of vitiligo. Methods: A Pubmed search was conducted in Clinical Queries using the key term "vitiligo". The search included metaanalyses, randomized controlled trials, clinical trials, observational studies, and reviews. The search was restricted to the English literature. The information retrieved from the above search was used in the compilation of the present article. The information retrieved from the above search was used in the compilation of the present article. Results: Approximately one quarter of patients with vitiligo have the onset before 10 years of age. Genetic, immunological, neurogenic and environmental factors may have a role to play in the pathogenesis. Vitiligo typically presents as acquired depigmented, well-demarcated macules/patches that appear milk- or chalk-white in color. Lesions tend to increase in number and enlarge centrifugally in size with time. Sites of predilection include the face, followed by the neck, lower limbs, trunk, and upper limbs. The clinical course is generally unpredictable. In children with fair skin, no active treatment is usually necessary other than the use of sunscreens and camouflage cosmetics. If treatment is preferred for cosmesis, topical corticosteroids, topical calcineurin inhibitors, and narrowband ultraviolet B phototherapy are the mainstays of treatment. Conclusion: The therapeutic effect of all the treatment modalities varies considerably from individual to individual. As such, treatment must be individualized. In general, the best treatment response is seen in younger patients, recent disease onset, darker skin types, and head and neck lesions. Topical corticosteroids and calcineurin inhibitors are the treatment of choice for those with localized disease. Topical calcineurin inhibitors are generally preferred for lesions on genitalia, intertriginous areas, face, and neck. Narrowband ultraviolet B phototherapy should be considered in patients who have widespread vitiligo or those with localized vitiligo associated with a significant impact on the quality of life who do not respond to treatment with topical corticosteroids and calcineurin inhibitors.


2021 ◽  
Vol 16 ◽  
Author(s):  
Rajni Sawanny ◽  
Sheersha Pramanik ◽  
Unnati Agarwal

: Breast cancer is the most common type of malignancy among ladies (around 30% of newly diagnosed patients every year). To date, various modern treatment modalities for breast cancer, such as radiotherapy, surgical method, hormonal therapy, and chemotherapeutic drug utilisation, are available. However, adverse drug reactions, therapeutic resistance, metastasis, or cancer reoccurrence chances remain the primary causes of mortality for breast cancer patients. To overcome all the potential drawbacks, we need to investigate novel techniques and strategies previously not considered and treat breast cancer effectively with safety and efficacy. For centuries, we utilise phytochemicals to treat various diseases because of their safety, low-cost & least or no side effects. Recently, naturally produced phytochemicals gain immense attention as potential breast cancer therapeutics because of their ideal characteristics; for instance, they operate via modulating molecular pathways associated with cancer growth and progression. The primary mechanism involves inhibition of cell proliferation, angiogenesis, migration, invasion, increasing anti-oxidant status, initiation of the arrest of the cell cycle, and apoptosis. Remedial viability gets effectively enhanced when phytochemicals work as adjuvants with chemotherapeutic drugs. This comprehensive review revolves around the latest chemopreventive, chemotherapeutic, and chemoprotective treatments with their molecular mechanisms to treat breast cancer by utilising phytochemicals such as vinca alkaloids, resveratrol, curcumin, paclitaxel, silibinin, quercetin, genistein and epigallocatechin gallate. The authors wish to extend the field of phytochemical study for its scientific validity and its druggability.


Immuno ◽  
2021 ◽  
Vol 1 (1) ◽  
pp. 30-66
Author(s):  
Niraj Kumar Jha ◽  
Madhan Jeyaraman ◽  
Mahesh Rachamalla ◽  
Shreesh Ojha ◽  
Kamal Dua ◽  
...  

An outbreak of “Pneumonia of Unknown Etiology” occurred in Wuhan, China, in late December 2019. Later, the agent factor was identified and coined as SARS-CoV-2, and the disease was named coronavirus disease 2019 (COVID-19). In a shorter period, this newly emergent infection brought the world to a standstill. On 11 March 2020, the WHO declared COVID-19 as a pandemic. Researchers across the globe have joined their hands to investigate SARS-CoV-2 in terms of pathogenicity, transmissibility, and deduce therapeutics to subjugate this infection. The researchers and scholars practicing different arts of medicine are on an extensive quest to come up with safer ways to curb the pathological implications of this viral infection. A huge number of clinical trials are underway from the branch of allopathy and naturopathy. Besides, a paradigm shift on cellular therapy and nano-medicine protocols has to be optimized for better clinical and functional outcomes of COVID-19-affected individuals. This article unveils a comprehensive review of the pathogenesis mode of spread, and various treatment modalities to combat COVID-19 disease.


Biomedicines ◽  
2021 ◽  
Vol 9 (7) ◽  
pp. 812
Author(s):  
Guendalina Bastioli ◽  
Maria Regoni ◽  
Federico Cazzaniga ◽  
Chiara Maria Giulia De Luca ◽  
Edoardo Bistaffa ◽  
...  

Parkinson’s disease (PD) is the most common neurodegenerative movement disorder. The neuropathological hallmark of the disease is the loss of dopamine neurons of the substantia nigra pars compacta. The clinical manifestations of PD are bradykinesia, rigidity, resting tremors and postural instability. PD patients often display non-motor symptoms such as depression, anxiety, weakness, sleep disturbances and cognitive disorders. Although, in 90% of cases, PD has a sporadic onset of unknown etiology, highly penetrant rare genetic mutations in many genes have been linked with typical familial PD. Understanding the mechanisms behind the DA neuron death in these Mendelian forms may help to illuminate the pathogenesis of DA neuron degeneration in the more common forms of PD. A key step in the identification of the molecular pathways underlying DA neuron death, and in the development of therapeutic strategies, is the creation and characterization of animal models that faithfully recapitulate the human disease. In this review, we outline the current status of PD modeling using mouse, rat and non-mammalian models, focusing on animal models for autosomal recessive PD.


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