Cystic fibrosis is a genetic disorder that affects the lungs and digestive system. It arises from mutations in the CFTR gene that result in defective chloride channels. This causes thick, sticky mucus to build up in the lungs, liver, and pancreas. Common symptoms include salty-tasting skin, persistent lung infections, and gastrointestinal issues. Diagnosis involves newborn screening, sweat testing, pulmonary function tests, and genetic testing. Treatment focuses on airway clearance, antibiotics, nutrition, and potentially lung transplantation. While there is no cure, advances in care have improved life expectancy in recent decades.
Cystic Fibrosis Care as a model for chronic disease care in the clinic. A presentation made on April 29, 2017 to the Wisconsin Chapter of the American Association of Medical Assistants at Lacrosse, WI.
* what to ask in chronic cough
* what investigations you would send
* characters of cough
* specific cough pointers
* undergraduate seminar in pediatrics
Goodpasture syndrome (GPS) also known as Goodpasture’s disease is a rare autoimmune disease in which antibodies attack the basement membrane in lungs and kidneys, leading to bleeding from the lungs and to kidney failure
Some forms of the disease involve just the lung or the kidney, most times, both.
Men are eight times more likely to be affected than women. The disease most commonly occurs in early adulthood.
Cystic Fibrosis Care as a model for chronic disease care in the clinic. A presentation made on April 29, 2017 to the Wisconsin Chapter of the American Association of Medical Assistants at Lacrosse, WI.
* what to ask in chronic cough
* what investigations you would send
* characters of cough
* specific cough pointers
* undergraduate seminar in pediatrics
Goodpasture syndrome (GPS) also known as Goodpasture’s disease is a rare autoimmune disease in which antibodies attack the basement membrane in lungs and kidneys, leading to bleeding from the lungs and to kidney failure
Some forms of the disease involve just the lung or the kidney, most times, both.
Men are eight times more likely to be affected than women. The disease most commonly occurs in early adulthood.
Dans le cadre du séminaire de la Société Démographique Francophone de Belgique (SDFB), l’Institut Bruxellois de statistique et d’Analyse (IBSA) a présenté l’exposé suivant : « Projections démographiques communales bruxelloise 2015 – 2025 ».
Intervenants : Benoît Laine (Bureau Fédéral du Plan) et Jean-Pierre Hermia (IBSA).
In het kader van het seminarie van de Société Démographique Francophone de Belgique (SDFB) heeft het Brussels Instituut voor Statistiek en Analyse (BISA) de volgende presentatie gegeven : « Bevolkingsprojecties voor de Brusselse gemeenten 2015-2025 ».
Sprekers : Benoît Laine (Federaal Planbureau) en Jean-Pierre Hermia (BISA)
causes and pathophysiology of obstetric sepsis simplified
Sepsis = ancient greek word ‘sepein’ = ‘to rot’
2016 SCCM definition –
life threatening organ dysfunction caused by a regulated host response to infection.
Sepsis exists on a continuum of severity ranging from infection and bacteremia to sepsis and septic shock, which can lead to MODS and death
Genital tract causes: chorioamnionitis, endometritis, septic abortion, wound infection after vaginal tear, episiotomy, or Caesarean section
Renal causes: lower urinary tract infection, pyelonephritis
Respiratory causes: pneumonia—bacterial, viral; tuberculosis
Intraperitoneal causes: ruptured appendix, acute appendicitis, acute cholecystitis, bowel infarction
Other causes: breast infection, septic pelvic thrombophlebitis, necrotizing fasciitis, malaria, miliary tuberculosis.
Most of what is known concerning sepsis comes from study of endotoxin -lipopolysaccharide-LPS
The lipid A moiety is bound by mononuclear blood cells,becomes internalized and stimulates release of mediators and a series of complex downstream events. Clinical effects manifested by cytokine effects.
Most of the pathogens produce endotoxins e.g klebsiella , some produce exotoxin eg. Clostridium,Staph
Schistosomiasis (Bilharziasis)Several species of parasitic blood flukes (trematodes), of which the most important are Schistosoma mansoni, S. japonicum, S. mekongi and S. haematobium.
Nutraceutical market, scope and growth: Herbal drug technologyLokesh Patil
As consumer awareness of health and wellness rises, the nutraceutical market—which includes goods like functional meals, drinks, and dietary supplements that provide health advantages beyond basic nutrition—is growing significantly. As healthcare expenses rise, the population ages, and people want natural and preventative health solutions more and more, this industry is increasing quickly. Further driving market expansion are product formulation innovations and the use of cutting-edge technology for customized nutrition. With its worldwide reach, the nutraceutical industry is expected to keep growing and provide significant chances for research and investment in a number of categories, including vitamins, minerals, probiotics, and herbal supplements.
Observation of Io’s Resurfacing via Plume Deposition Using Ground-based Adapt...Sérgio Sacani
Since volcanic activity was first discovered on Io from Voyager images in 1979, changes
on Io’s surface have been monitored from both spacecraft and ground-based telescopes.
Here, we present the highest spatial resolution images of Io ever obtained from a groundbased telescope. These images, acquired by the SHARK-VIS instrument on the Large
Binocular Telescope, show evidence of a major resurfacing event on Io’s trailing hemisphere. When compared to the most recent spacecraft images, the SHARK-VIS images
show that a plume deposit from a powerful eruption at Pillan Patera has covered part
of the long-lived Pele plume deposit. Although this type of resurfacing event may be common on Io, few have been detected due to the rarity of spacecraft visits and the previously low spatial resolution available from Earth-based telescopes. The SHARK-VIS instrument ushers in a new era of high resolution imaging of Io’s surface using adaptive
optics at visible wavelengths.
Introduction:
RNA interference (RNAi) or Post-Transcriptional Gene Silencing (PTGS) is an important biological process for modulating eukaryotic gene expression.
It is highly conserved process of posttranscriptional gene silencing by which double stranded RNA (dsRNA) causes sequence-specific degradation of mRNA sequences.
dsRNA-induced gene silencing (RNAi) is reported in a wide range of eukaryotes ranging from worms, insects, mammals and plants.
This process mediates resistance to both endogenous parasitic and exogenous pathogenic nucleic acids, and regulates the expression of protein-coding genes.
What are small ncRNAs?
micro RNA (miRNA)
short interfering RNA (siRNA)
Properties of small non-coding RNA:
Involved in silencing mRNA transcripts.
Called “small” because they are usually only about 21-24 nucleotides long.
Synthesized by first cutting up longer precursor sequences (like the 61nt one that Lee discovered).
Silence an mRNA by base pairing with some sequence on the mRNA.
Discovery of siRNA?
The first small RNA:
In 1993 Rosalind Lee (Victor Ambros lab) was studying a non- coding gene in C. elegans, lin-4, that was involved in silencing of another gene, lin-14, at the appropriate time in the
development of the worm C. elegans.
Two small transcripts of lin-4 (22nt and 61nt) were found to be complementary to a sequence in the 3' UTR of lin-14.
Because lin-4 encoded no protein, she deduced that it must be these transcripts that are causing the silencing by RNA-RNA interactions.
Types of RNAi ( non coding RNA)
MiRNA
Length (23-25 nt)
Trans acting
Binds with target MRNA in mismatch
Translation inhibition
Si RNA
Length 21 nt.
Cis acting
Bind with target Mrna in perfect complementary sequence
Piwi-RNA
Length ; 25 to 36 nt.
Expressed in Germ Cells
Regulates trnasposomes activity
MECHANISM OF RNAI:
First the double-stranded RNA teams up with a protein complex named Dicer, which cuts the long RNA into short pieces.
Then another protein complex called RISC (RNA-induced silencing complex) discards one of the two RNA strands.
The RISC-docked, single-stranded RNA then pairs with the homologous mRNA and destroys it.
THE RISC COMPLEX:
RISC is large(>500kD) RNA multi- protein Binding complex which triggers MRNA degradation in response to MRNA
Unwinding of double stranded Si RNA by ATP independent Helicase
Active component of RISC is Ago proteins( ENDONUCLEASE) which cleave target MRNA.
DICER: endonuclease (RNase Family III)
Argonaute: Central Component of the RNA-Induced Silencing Complex (RISC)
One strand of the dsRNA produced by Dicer is retained in the RISC complex in association with Argonaute
ARGONAUTE PROTEIN :
1.PAZ(PIWI/Argonaute/ Zwille)- Recognition of target MRNA
2.PIWI (p-element induced wimpy Testis)- breaks Phosphodiester bond of mRNA.)RNAse H activity.
MiRNA:
The Double-stranded RNAs are naturally produced in eukaryotic cells during development, and they have a key role in regulating gene expression .
Richard's aventures in two entangled wonderlandsRichard Gill
Since the loophole-free Bell experiments of 2020 and the Nobel prizes in physics of 2022, critics of Bell's work have retreated to the fortress of super-determinism. Now, super-determinism is a derogatory word - it just means "determinism". Palmer, Hance and Hossenfelder argue that quantum mechanics and determinism are not incompatible, using a sophisticated mathematical construction based on a subtle thinning of allowed states and measurements in quantum mechanics, such that what is left appears to make Bell's argument fail, without altering the empirical predictions of quantum mechanics. I think however that it is a smoke screen, and the slogan "lost in math" comes to my mind. I will discuss some other recent disproofs of Bell's theorem using the language of causality based on causal graphs. Causal thinking is also central to law and justice. I will mention surprising connections to my work on serial killer nurse cases, in particular the Dutch case of Lucia de Berk and the current UK case of Lucy Letby.
Seminar of U.V. Spectroscopy by SAMIR PANDASAMIR PANDA
Spectroscopy is a branch of science dealing the study of interaction of electromagnetic radiation with matter.
Ultraviolet-visible spectroscopy refers to absorption spectroscopy or reflect spectroscopy in the UV-VIS spectral region.
Ultraviolet-visible spectroscopy is an analytical method that can measure the amount of light received by the analyte.
Multi-source connectivity as the driver of solar wind variability in the heli...Sérgio Sacani
The ambient solar wind that flls the heliosphere originates from multiple
sources in the solar corona and is highly structured. It is often described
as high-speed, relatively homogeneous, plasma streams from coronal
holes and slow-speed, highly variable, streams whose source regions are
under debate. A key goal of ESA/NASA’s Solar Orbiter mission is to identify
solar wind sources and understand what drives the complexity seen in the
heliosphere. By combining magnetic feld modelling and spectroscopic
techniques with high-resolution observations and measurements, we show
that the solar wind variability detected in situ by Solar Orbiter in March
2022 is driven by spatio-temporal changes in the magnetic connectivity to
multiple sources in the solar atmosphere. The magnetic feld footpoints
connected to the spacecraft moved from the boundaries of a coronal hole
to one active region (12961) and then across to another region (12957). This
is refected in the in situ measurements, which show the transition from fast
to highly Alfvénic then to slow solar wind that is disrupted by the arrival of
a coronal mass ejection. Our results describe solar wind variability at 0.5 au
but are applicable to near-Earth observatories.
Professional air quality monitoring systems provide immediate, on-site data for analysis, compliance, and decision-making.
Monitor common gases, weather parameters, particulates.
(May 29th, 2024) Advancements in Intravital Microscopy- Insights for Preclini...Scintica Instrumentation
Intravital microscopy (IVM) is a powerful tool utilized to study cellular behavior over time and space in vivo. Much of our understanding of cell biology has been accomplished using various in vitro and ex vivo methods; however, these studies do not necessarily reflect the natural dynamics of biological processes. Unlike traditional cell culture or fixed tissue imaging, IVM allows for the ultra-fast high-resolution imaging of cellular processes over time and space and were studied in its natural environment. Real-time visualization of biological processes in the context of an intact organism helps maintain physiological relevance and provide insights into the progression of disease, response to treatments or developmental processes.
In this webinar we give an overview of advanced applications of the IVM system in preclinical research. IVIM technology is a provider of all-in-one intravital microscopy systems and solutions optimized for in vivo imaging of live animal models at sub-micron resolution. The system’s unique features and user-friendly software enables researchers to probe fast dynamic biological processes such as immune cell tracking, cell-cell interaction as well as vascularization and tumor metastasis with exceptional detail. This webinar will also give an overview of IVM being utilized in drug development, offering a view into the intricate interaction between drugs/nanoparticles and tissues in vivo and allows for the evaluation of therapeutic intervention in a variety of tissues and organs. This interdisciplinary collaboration continues to drive the advancements of novel therapeutic strategies.
4. Introduction
• Cystic fibrosis (mucoviscidosis) is a genetic disorder.
• Different people may have different degrees of symptoms.
• CF is inherited in an autosomal recessive manner.
• CF is most common among people of Northern European ancestry.
• There is no cure for cystic fibrosis.
5. Historical Timeline
1938
1965
1965
1985
1989
Carl von Rokitansky described a case of fetal death with meconium peritonitis.
Dorothy Hansine Andersen published an article, “Cystic Fibrosis of the Pancreas and Its Relation to Celiac Disease”.
Paul di Sant’Agnese discovered abnormalities in sweat electrolytes.
Cystic fibrosis was identified as an autosomal recessive disease.
The genetic defect for CF was located on chromosome 7.
The gene encoding the cystic fibrosis transmembrane conductance regulator (CFTR) was identified.
19th
6. Epidemiology
• Prevalence
• Life expectancy
Population
Caucasian (US)
Hispanic
African American
Native American
Asian (US)
Epidemiologic
1 in 1,900-3,700
1 in 8,000-9,000
1 in 15,300
1 in 40,000
1 in 10,000
8. Cause
The CFTR chloride channel
• chloride channel
• Member of ABC family of transporters
• CFTR also regulates epithelial sodium channel
9. Classes of cystic fibrosis-causing mutations
• Class I:- No synthesis of CFTR protein
• Class II:- Defective processing of CFTR protein
∆F508 (accounts for 66-70% CF cases)
• Class III:- Defective Regulation
• Class IV:- Defective Conductance
• Class V:- Reduced production of CFTR
Cause
10. Pathophysiology
• Most of the damage in CF is due to blockage of the narrow passages of affected
organs with thickened secretions.
15. Inflammation and infection cause injury and structural changes to the lungs
• In the early stages:coughing, copious sputum production, and decreased ability to
exercise are common.
• later stages:Bronchiectasis,hemoptysis, pulmonary hypertension, heart failure
,hypoxia, and respiratory failure
Symptoms
20. Cystic fibrosis may be diagnosed by many different methods including
• newborn screening
• sweat testing
• Pulmonary function testing (PFT)
• CT CHEST
• genetic testing
Diagnosis