Cellular and Molecular Mechanism of Liver Fibrosis: A Critical Insight

Research Journal of Pharmacy and Technology ◽

10.52711/0974-360x.2021.01066 ◽

2021 ◽

pp. 6147-6154

Author(s):

Gnyana Ranjan Parida ◽

Gurudutta Pattnaik ◽

Amulyaratna Behera

Keyword(s):

Liver Fibrosis ◽

Cellular Level ◽

Advanced Cirrhosis ◽

Life Threatening ◽

Critical Insight ◽

Asymptomatic Individuals ◽

Future Direction ◽

Collagen Type 1 ◽

Limited Scope

Diseases are there, some are curable but certain diseases are life threatening. The dreadfulness of liver fibrosis created much attention in 21st century among pharmaceutical researchers. Usually liver fibrosis is asymptomatic and patients with advanced cirrhosis symptoms will have limited scope for reversibility. Recognition of asymptomatic individuals by considering them as critical is the fundamental step to control the liver-related morbidity and mortality. By reviewing the previous mortality analysis around the globe it is reported that liver fibrosis is the second leading cause of mortality in US, fifth most common cause of death in Germany, UK as well as tenth most frequent cause of fatality in India and around 10 lakh patients are diagnosed globally in every year. Liver fibrosis often leads to cirrhosis, hepatocellular carcinoma and progress towards liver failure which results due to excessive deposition of extracellular matrix (ECM) proteins, especially collagen type-1 contributed by hepatic stellate cells. The current study is based on for analyzing the molecular basis of liver fibrosis so that, it will be helpful for finding its route cause in cellular level which will guide as a future direction for developing new theranostic approaches for the early diagnosis of liver fibrosis.

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Congenital Craniofacial Plexiform Neurofibroma in Neurofibromatosis Type 1

Diagnostics ◽

10.3390/diagnostics11020218 ◽

2021 ◽

Vol 11 (2) ◽

pp. 218

Author(s):

Antonella Cacchione ◽

Alessia Carboni ◽

Mariachiara Lodi ◽

Rita De Vito ◽

Andrea Carai ◽

...

Keyword(s):

Neurofibromatosis Type 1 ◽

Correct Diagnosis ◽

Plexiform Neurofibroma ◽

Neurofibromatosis Type ◽

Detection Techniques ◽

Life Threatening ◽

Magnetic Resonance Imaging Mri ◽

Diagnostic Work ◽

Radiological Methods

We present a case demonstrating the performance of different radiographical imaging modalities in the diagnostic work-up of a patient with neurofibromatosis type 1 (NF1) and plexiform neurofibroma (PN). The newborn boy showed an expansive-infiltrative cervical and facial mass presented with macrocrania, craniofacial disfigurement, exophthalmos and glaucoma. A computer tomography (CT) and a magnetic resonance imaging (MRI) were performed. The CT was fundamental to evaluate the bone dysmorphisms and the MRI was crucial to estimate the mass extension. The biopsy of the lesion confirmed the suspicion of PN, thus allowing the diagnosis of NF1. PN is a variant of neurofibromas, a peripheral nerves sheath tumor typically associated with NF1. Even through currently available improved detection techniques, NF1 diagnosis at birth remains a challenge due to a lack of pathognomonic signs; therefore congenital PN are recognized in 20% of cases. This case highlights the importance of using different radiological methods both for the correct diagnosis and the follow-up of the patient with PN. Thanks to MRI evaluation, it was possible to identify earlier the progressive increasing size of the PN and the possible life threatening evolution in order to perform a tracheostomy to avoid airways compression.

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Risk stratification in cardiogenic shock: a focus on the available evidence

Heart Failure Reviews ◽

10.1007/s10741-021-10140-7 ◽

2021 ◽

Author(s):

C. Sciaccaluga ◽

G. E. Mandoli ◽

N. Ghionzoli ◽

F. Anselmi ◽

C. Sorini Dini ◽

...

Keyword(s):

Risk Stratification ◽

Cardiogenic Shock ◽

Clinical Syndrome ◽

Diagnostic Assessment ◽

Prognostic Scores ◽

Current Evidence ◽

Efficient Resource ◽

Threatening Condition ◽

Life Threatening ◽

Future Direction

AbstractCardiogenic shock is a clinical syndrome which is defined as the presence of primary cardiac disorder that results in hypotension together with signs of organ hypoperfusion in the state of normovolaemia or hypervolaemia. It represents a complex life-threatening condition, characterized by a high mortality rate, that requires urgent diagnostic assessment as well as treatment; therefore, it is of paramount important to advocate for a thorough risk stratification. In fact, the early identification of patients that could benefit the most from more aggressive and invasive approaches could facilitate a more efficient resource allocation. This review attempts to critically analyse the current evidence on prognosis in cardiogenic shock, focusing in particular on clinical, laboratoristic and echocardiographic prognostic parameters. Furthermore, it focuses also on the available prognostic scores, highlighting the strengths and the possible pitfalls. Finally, it provides insights into future direction that could be followed in order to ameliorate risk stratification in this delicate subset of patients.

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Direct comparison of 3D and 2D cultivation reveals higher osteogenic capacity of elderly osteoblasts in 3D

Journal of Orthopaedic Surgery and Research ◽

10.1186/s13018-020-02153-z ◽

2021 ◽

Vol 16 (1) ◽

Author(s):

Stephan Payr ◽

Elizabeth Rosado-Balmayor ◽

Thomas Tiefenboeck ◽

Tim Schuseil ◽

Marina Unger ◽

...

Keyword(s):

Gene Expression ◽

Collagen Type ◽

3D Culture ◽

Osteogenic Potential ◽

Donor Age ◽

Collagen Type 1 ◽

2D And 3D ◽

Human Primary Osteoblasts ◽

Gene Expression Levels

Abstract Background The aim of this study was the investigation of the osteogenic potential of human osteoblasts of advanced donor age in 2D and 3D culture. Methods Osteoblasts were induced to osteogenic differentiation and cultivated, using the same polystyrene material in 2D and 3D culture for 2 weeks. Samples were taken to evaluate alkaline phosphatase (ALP) activity, mineralization and gene expression. Results Osteoprotegerin (OPG) levels were significantly increased (8.2-fold) on day 7 in 3D compared to day 0 (p < 0.0001) and 11.6-fold higher in 3D than in 2D (p < 0.0001). Both culture systems showed reduced osteocalcin (OC) levels (2D 85% and 3D 50% of basic value). Collagen type 1 (Col1) expression was elevated in 3D on day 7 (1.4-fold; p = 0.009). Osteopontin (OP) expression showed 6.5-fold higher levels on day 7 (p = 0.002) in 3D than in 2D. Mineralization was significantly higher in 3D on day 14 (p = 0.0002). Conclusion Advanced donor age human primary osteoblasts reveal significantly higher gene expression levels of OPG, Col1 and OP in 3D than in monolayer. Therefore, it seems that a relatively high potential of bone formation in a natural 3D arrangement is presumably still present in osteoblasts of elderly people. Trial registration 5217/11 on the 22nd of Dec. 2011.

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EXPRESS: Pulmonary Hypertension Associated With Neurofibromatosis Type 2

Pulmonary Circulation ◽

10.1177/20458940211029550 ◽

2021 ◽

pp. 204589402110295

Author(s):

Hirohisa Taniguchi ◽

Tomoya Takashima ◽

Ly Tu ◽

RaphaÃ«l Thuillet ◽

Asuka Furukawa ◽

...

Keyword(s):

Pulmonary Hypertension ◽

Neurofibromatosis Type ◽

Pulmonary Vascular Remodeling ◽

Life Threatening ◽

History Of ◽

Pulmonary Arterial ◽

First Time

Although precapillary pulmonary hypertension (PH) is a rare but severe complication of patients with neurofibromatosis type 1 (NF1), its association with NF2 remains unknown. Herein, we report a case of a 44-year-old woman who was initially diagnosed with idiopathic pulmonary arterial hypertension (IPAH) and treated with PAH-specific combination therapy. However, a careful assessment for a relevant family history of the disease and genetic testing reveal that this patient had a mutation in the NF2 gene. Using immunofluorescence and Western blotting, we demonstrated a decrease in endothelial NF2 protein in lungs from IPAH patients compared to control lungs, suggesting a potential role of NF2 in PAH development. To our knowledge, this is the first time that precapillary PH has been described in a patient with NF2. The altered endothelial NF2 expression pattern in PAH lungs should stimulate work to better understand how NF2 is contributing to the pulmonary vascular remodeling associated to these severe life-threatening conditions.

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Low rate of life‐threatening events and limitations in predicting invasive and noninvasive markers of symptoms in a cohort of type 1 Brugada syndrome patients: Data and insights from the GenBra registry

Journal of Cardiovascular Electrophysiology ◽

10.1111/jce.14732 ◽

2020 ◽

Vol 31 (11) ◽

pp. 2920-2928 ◽

Cited By ~ 1

Author(s):

Luciana Sacilotto ◽

Mauricio I. Scanavacca ◽

Natália Olivetti ◽

Carolina Lemes ◽

Gabrielle D. Pessente ◽

...

Keyword(s):

Brugada Syndrome ◽

Life Threatening ◽

Noninvasive Markers ◽

Low Rate

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F.N.10 PHARMACOLOGICAL ANTAGONISM OF ENDOCANNABINOID CB1 RECEPTOR AMELIORATES LIVER FIBROSIS IN A RAT MODEL OF ADVANCED CIRRHOSIS

Digestive and Liver Disease ◽

10.1016/s1590-8658(10)60464-1 ◽

2010 ◽

Vol 42 ◽

pp. S36-S37

Author(s):

F.A. Giannone ◽

M. Domenicali ◽

M. Baldassarre ◽

G. Di Pompo ◽

M. Bernardi ◽

...

Keyword(s):

Liver Fibrosis ◽

Rat Model ◽

Cb1 Receptor ◽

Advanced Cirrhosis

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MULTISYSTEM INFLAMMATORY SYNDROME IN CHILDREN ASSOCIATED WITH COVID-19 IN THE 6-YEAR-OLD PATIENT WITH MANIFESTATION OF TYPE 1 DIABETES MELLITUS WITH DIABETIC KETOACIDOSIS

PEDIATRIA Journal named after G N SPERANSKY ◽

10.24110/0031-403x-2021-100-2-295-300 ◽

2021 ◽

Vol 100 (2) ◽

pp. 295-300

Author(s):

Yu.V. Tikhonovich ◽

◽

A.Yu. Rtishchev ◽

A.A. Glazyrina ◽

D.Yu. Ovsyannikov ◽

...

Keyword(s):

Diabetes Mellitus ◽

Type 1 Diabetes ◽

Type 1 Diabetes Mellitus ◽

Diabetic Ketoacidosis ◽

Clinical Observation ◽

Laboratory Data ◽

Domestic Literature ◽

Life Threatening ◽

Inflammatory Syndrome

For the first time in the domestic literature, the article presents a clinical observation of multisystem inflammatory syndrome in children (MIS-C) associated with COVID-19 in the 6-year-old patient with manifestation of type 1 diabetes mellitus (T1DM) in the form of diabetic ketoacidosis. Anamnestic, clinical and laboratory data are presented on the basis of which two life-threatening diseases was diagnosed, as well as tactics of therapy, which made it possible to achieve a positive result. This clinical observation is compared with observations of foreign colleagues. Possible pathogenetic mechanisms of MIS-C and T1DM comorbidity are discussed.

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Congenital ADAMTS-13 deficiency presenting as life-threatening thrombosis during pregnancy

BMJ Case Reports ◽

10.1136/bcr-2020-239901 ◽

2021 ◽

Vol 14 (8) ◽

pp. e239901

Author(s):

Faheema Hasan ◽

Anshul Gupta ◽

Dinesh Chandra ◽

Soniya Nityanand

Keyword(s):

Late Presentation ◽

Fresh Frozen Plasma ◽

Artery Thrombosis ◽

Fresh Frozen ◽

Life Threatening ◽

A Disintegrin And Metalloproteinase ◽

Thrombospondin Type 1 Repeats ◽

Frozen Plasma ◽

Plasma Infusions

Thrombotic thrombocytopenic purpura (TTP) is a life-threatening disease characterised by thrombocytopenia, microangiopathic haemolytic anaemia and microvascular thrombosis. Congenital TTP accounting for less than 5% of all TTP cases can have a late presentation in adulthood mostly triggered by predisposing factors such as infection, pregnancy and inflammation. We present a case of a 23-year-old woman who presented to us in the postpartum period with mesenteric artery thrombosis with infarcts and later was diagnosed as a case of TTP based on congenital a disintegrin and metalloproteinase with thrombospondin type 1 repeats 13 (ADAMTS-13) deficiency detected on ADAMTS-13 levels and gene sequencing. She was successfully managed initially with therapeutic plasma exchanges and is now on prophylactic fortnightly fresh frozen plasma infusions at 15 mL/kg body weight and continues to be in remission.

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