Cystic fibrosis is a genetic disorder that affects the lungs and digestive system. It is caused by a defective gene that causes thick, sticky mucus to build up in the lungs, liver, pancreas, and other organs. On radiological evaluation, chest x-rays and CT scans are used to examine lung abnormalities like bronchial wall thickening, bronchiectasis, mucus plugging. Abdominal imaging reveals pancreatic abnormalities such as cysts and atrophy as well as hepatobiliary manifestations like cirrhosis and gallstones. Imaging also detects gastrointestinal complications including meconium ileus in newborns, distal intestinal obstructive syndrome, and intussusception.
THIS PRESENTATION INCLUDES DEFINITION, OVERVIEW, PATHOLOGY, CLINICAL FEATURES, ASSESSMENT AND PT MANAGEMENT OF CYSTIC FIBROSIS. THIS PPT WILL BE VERY USEFUL FOR FINAL YEAR BPT STUDENTS. IT COVERS BASIC KNOWLEDGE REGARDING THE DISEASE AND ALLOWS BETTER UNDERSTANDING. IT IS MADE ONLY FOR LEARNING AND EXAM PURPOSE.
Bronchiectasis
A condition characterized by chronic permanent dilation & destruction of bronchi due to destructive changes in the elastic and muscular layers of bronchial walls.
The common thread in the pathogenesis of bronchiectasis consists of difficulty clearing secretions & recurrent infections with a “vicious circle” of infection and inflammation resulting in airway injury and remodelling.
PLEASE REFER TO REFERENCE TEXTBOOKS FOR CLARITY.
Abdominal TB can involve any part of GIT from mouth to anus, the peritoneum and pancreato-billiary system.
Total EP TB accounts for about 10-12% of total no. of TB cases, out of which 11-16% are abdominal koch.
Sixth most frequent EP TB after lymphatics, genitourinary, bone & joint, milliary & meningeal TB.
Caused by M. tuberculosis, M. bovis & NTM.
Age group 20-40 most commonly affected & slight female preponderance has been described.
Before era of HIV infection > 80% TB was confined to lung
Extrapulmonary TB increases with HIV
40 –60% TB in HIV+ pt are extrapulmonary
Globally, proportion of co-infected pt > 8 %
~ 0.4 million people in India are co-infected.
In one study, 16.6% abdominal TB pt in Bombay was HIV +.
Mechanisms by which M. tuberculosis reach the GIT:
Hematogenous spread from primary lung focus
Ingestion of bacilli in sputum from active pulmonary focus.
Direct spread from adjacent organs.
Via lymph channels from infected LN
Rare Mechanism:
Contiguous spread of infection from a fallopian tube
TB peritonitis as complication of peritoneal dialysis
Ileum > caecum > ascending colon > jejunum
>appendix > sigmoid > rectum > duodenum
> stomach > oesophagus
More than one site may be involved
Ileum > caecum > ascending colon > jejunum
>appendix > sigmoid > rectum > duodenum
> stomach > oesophagus
More than one site may be involved
Ileum > caecum > ascending colon > jejunum
>appendix > sigmoid > rectum > duodenum
> stomach > oesophagus
More than one site may be involved
THIS PRESENTATION INCLUDES DEFINITION, OVERVIEW, PATHOLOGY, CLINICAL FEATURES, ASSESSMENT AND PT MANAGEMENT OF CYSTIC FIBROSIS. THIS PPT WILL BE VERY USEFUL FOR FINAL YEAR BPT STUDENTS. IT COVERS BASIC KNOWLEDGE REGARDING THE DISEASE AND ALLOWS BETTER UNDERSTANDING. IT IS MADE ONLY FOR LEARNING AND EXAM PURPOSE.
Bronchiectasis
A condition characterized by chronic permanent dilation & destruction of bronchi due to destructive changes in the elastic and muscular layers of bronchial walls.
The common thread in the pathogenesis of bronchiectasis consists of difficulty clearing secretions & recurrent infections with a “vicious circle” of infection and inflammation resulting in airway injury and remodelling.
PLEASE REFER TO REFERENCE TEXTBOOKS FOR CLARITY.
Abdominal TB can involve any part of GIT from mouth to anus, the peritoneum and pancreato-billiary system.
Total EP TB accounts for about 10-12% of total no. of TB cases, out of which 11-16% are abdominal koch.
Sixth most frequent EP TB after lymphatics, genitourinary, bone & joint, milliary & meningeal TB.
Caused by M. tuberculosis, M. bovis & NTM.
Age group 20-40 most commonly affected & slight female preponderance has been described.
Before era of HIV infection > 80% TB was confined to lung
Extrapulmonary TB increases with HIV
40 –60% TB in HIV+ pt are extrapulmonary
Globally, proportion of co-infected pt > 8 %
~ 0.4 million people in India are co-infected.
In one study, 16.6% abdominal TB pt in Bombay was HIV +.
Mechanisms by which M. tuberculosis reach the GIT:
Hematogenous spread from primary lung focus
Ingestion of bacilli in sputum from active pulmonary focus.
Direct spread from adjacent organs.
Via lymph channels from infected LN
Rare Mechanism:
Contiguous spread of infection from a fallopian tube
TB peritonitis as complication of peritoneal dialysis
Ileum > caecum > ascending colon > jejunum
>appendix > sigmoid > rectum > duodenum
> stomach > oesophagus
More than one site may be involved
Ileum > caecum > ascending colon > jejunum
>appendix > sigmoid > rectum > duodenum
> stomach > oesophagus
More than one site may be involved
Ileum > caecum > ascending colon > jejunum
>appendix > sigmoid > rectum > duodenum
> stomach > oesophagus
More than one site may be involved
Empyema is a collection of pus in the cavity between the lung and the membrane that surrounds it (pleural space). Caused by an infection that spreads from the lung and leads to an accumulation of pus in the pleural space, the infected fluid can build up to a quantity of a pint or more, which puts pressure on the lungs, causing shortness of breath and pain. Risk factors include recent lung conditions like bacterial pneumonia, lung abscess, thoracic surgery, trauma or injury to the chest.
An undergraduate lecture on Congenital Anomalies, Inflammatory & Neoplastic Disorders of Esophagus for medical students delivered by Dr Muhammad Omair Riaz
ARTIFICIAL INTELLIGENCE IN HEALTHCARE.pdfAnujkumaranit
Artificial intelligence (AI) refers to the simulation of human intelligence processes by machines, especially computer systems. It encompasses tasks such as learning, reasoning, problem-solving, perception, and language understanding. AI technologies are revolutionizing various fields, from healthcare to finance, by enabling machines to perform tasks that typically require human intelligence.
MANAGEMENT OF ATRIOVENTRICULAR CONDUCTION BLOCK.pdfJim Jacob Roy
Cardiac conduction defects can occur due to various causes.
Atrioventricular conduction blocks ( AV blocks ) are classified into 3 types.
This document describes the acute management of AV block.
Lung Cancer: Artificial Intelligence, Synergetics, Complex System Analysis, S...Oleg Kshivets
RESULTS: Overall life span (LS) was 2252.1±1742.5 days and cumulative 5-year survival (5YS) reached 73.2%, 10 years – 64.8%, 20 years – 42.5%. 513 LCP lived more than 5 years (LS=3124.6±1525.6 days), 148 LCP – more than 10 years (LS=5054.4±1504.1 days).199 LCP died because of LC (LS=562.7±374.5 days). 5YS of LCP after bi/lobectomies was significantly superior in comparison with LCP after pneumonectomies (78.1% vs.63.7%, P=0.00001 by log-rank test). AT significantly improved 5YS (66.3% vs. 34.8%) (P=0.00000 by log-rank test) only for LCP with N1-2. Cox modeling displayed that 5YS of LCP significantly depended on: phase transition (PT) early-invasive LC in terms of synergetics, PT N0—N12, cell ratio factors (ratio between cancer cells- CC and blood cells subpopulations), G1-3, histology, glucose, AT, blood cell circuit, prothrombin index, heparin tolerance, recalcification time (P=0.000-0.038). Neural networks, genetic algorithm selection and bootstrap simulation revealed relationships between 5YS and PT early-invasive LC (rank=1), PT N0—N12 (rank=2), thrombocytes/CC (3), erythrocytes/CC (4), eosinophils/CC (5), healthy cells/CC (6), lymphocytes/CC (7), segmented neutrophils/CC (8), stick neutrophils/CC (9), monocytes/CC (10); leucocytes/CC (11). Correct prediction of 5YS was 100% by neural networks computing (area under ROC curve=1.0; error=0.0).
CONCLUSIONS: 5YS of LCP after radical procedures significantly depended on: 1) PT early-invasive cancer; 2) PT N0--N12; 3) cell ratio factors; 4) blood cell circuit; 5) biochemical factors; 6) hemostasis system; 7) AT; 8) LC characteristics; 9) LC cell dynamics; 10) surgery type: lobectomy/pneumonectomy; 11) anthropometric data. Optimal diagnosis and treatment strategies for LC are: 1) screening and early detection of LC; 2) availability of experienced thoracic surgeons because of complexity of radical procedures; 3) aggressive en block surgery and adequate lymph node dissection for completeness; 4) precise prediction; 5) adjuvant chemoimmunoradiotherapy for LCP with unfavorable prognosis.
New Directions in Targeted Therapeutic Approaches for Older Adults With Mantl...i3 Health
i3 Health is pleased to make the speaker slides from this activity available for use as a non-accredited self-study or teaching resource.
This slide deck presented by Dr. Kami Maddocks, Professor-Clinical in the Division of Hematology and
Associate Division Director for Ambulatory Operations
The Ohio State University Comprehensive Cancer Center, will provide insight into new directions in targeted therapeutic approaches for older adults with mantle cell lymphoma.
STATEMENT OF NEED
Mantle cell lymphoma (MCL) is a rare, aggressive B-cell non-Hodgkin lymphoma (NHL) accounting for 5% to 7% of all lymphomas. Its prognosis ranges from indolent disease that does not require treatment for years to very aggressive disease, which is associated with poor survival (Silkenstedt et al, 2021). Typically, MCL is diagnosed at advanced stage and in older patients who cannot tolerate intensive therapy (NCCN, 2022). Although recent advances have slightly increased remission rates, recurrence and relapse remain very common, leading to a median overall survival between 3 and 6 years (LLS, 2021). Though there are several effective options, progress is still needed towards establishing an accepted frontline approach for MCL (Castellino et al, 2022). Treatment selection and management of MCL are complicated by the heterogeneity of prognosis, advanced age and comorbidities of patients, and lack of an established standard approach for treatment, making it vital that clinicians be familiar with the latest research and advances in this area. In this activity chaired by Michael Wang, MD, Professor in the Department of Lymphoma & Myeloma at MD Anderson Cancer Center, expert faculty will discuss prognostic factors informing treatment, the promising results of recent trials in new therapeutic approaches, and the implications of treatment resistance in therapeutic selection for MCL.
Target Audience
Hematology/oncology fellows, attending faculty, and other health care professionals involved in the treatment of patients with mantle cell lymphoma (MCL).
Learning Objectives
1.) Identify clinical and biological prognostic factors that can guide treatment decision making for older adults with MCL
2.) Evaluate emerging data on targeted therapeutic approaches for treatment-naive and relapsed/refractory MCL and their applicability to older adults
3.) Assess mechanisms of resistance to targeted therapies for MCL and their implications for treatment selection
Couples presenting to the infertility clinic- Do they really have infertility...Sujoy Dasgupta
Dr Sujoy Dasgupta presented the study on "Couples presenting to the infertility clinic- Do they really have infertility? – The unexplored stories of non-consummation" in the 13th Congress of the Asia Pacific Initiative on Reproduction (ASPIRE 2024) at Manila on 24 May, 2024.
Prix Galien International 2024 Forum ProgramLevi Shapiro
June 20, 2024, Prix Galien International and Jerusalem Ethics Forum in ROME. Detailed agenda including panels:
- ADVANCES IN CARDIOLOGY: A NEW PARADIGM IS COMING
- WOMEN’S HEALTH: FERTILITY PRESERVATION
- WHAT’S NEW IN THE TREATMENT OF INFECTIOUS,
ONCOLOGICAL AND INFLAMMATORY SKIN DISEASES?
- ARTIFICIAL INTELLIGENCE AND ETHICS
- GENE THERAPY
- BEYOND BORDERS: GLOBAL INITIATIVES FOR DEMOCRATIZING LIFE SCIENCE TECHNOLOGIES AND PROMOTING ACCESS TO HEALTHCARE
- ETHICAL CHALLENGES IN LIFE SCIENCES
- Prix Galien International Awards Ceremony
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2. INTRODUCTION
Is an inherited multisystem disorder of children and
adults
It is the most common life-limiting recessive genetic
trait among white persons.
Is responsible for most cases of exocrine pancreatic
insufficiency in early life and is the major cause of
severe chronic lung disease in children.
Responsible for many cases of salt depletion, nasal
polyposis, pansinusitis, rectal prolapse, pancreatitis,
cholelithiasis, and insulin-dependent
hyperglycemia.
3. GENETIC
The cystic Fibrosis gene was isolated in
1989 and mapped to chromosome 7.
More than 1300 different mutations of this
gene have been identified, all of which
cause a defect in chloride transport
across the cell membrane;
The severity of chloride channel
dysfunction varies widely with different
mutations.
4. PATHOPHYSIOLOGY
Dysfunction of the cystic fibrosis transmembrane
conductance regulator protein (CFTR).
CFTR is expressed largely in epithelial cells of airways, the
gastrointestinal tract (including the pancreas and biliary
system), the sweat glands, and the genitourinary system.
It functions as a chloride channel and has other regulatory
functions
The alteration in chloride transport is associated with
production of abnormally thick secretions in glandular
tissues. The lung bronchioles and pancreatic ducts are
primarily affected, often resulting in progressive destruction
of these organs
5.
6. PATHOPHYSIOLOGY
The result is that these secretions are retained and
obstruct airways, starting with those of the smallest
caliber, the bronchioles.
In the pancreatic and biliary ducts (and in the vas
deferens), leading to desiccation of proteinaceous
secretions and obstruction.
The function of sweat gland duct cells is to absorb
rather than secrete chloride, salt is not retrieved
from the isotonic primary sweat as it is transported
to the skin surface; chloride and sodium levels are
consequently elevated
7. Sequelae
Chronic infection in CF is limited to the airways. A
likely explanation for infection is a sequence of
events starting with failure to clear inhaled bacteria
promptly and then proceeding to persistent
colonization and an inflammatory response in
airway walls.
High prevalence in patients with CF of airway
colonization with Staphylococcus aureus,
Pseudomonas aeruginosa, and Burkholderia
cepacia, organisms that rarely infect the lungs of
other individuals
8.
9. Clinical presentation
Most common signs/symptoms: Recurrent respiratory tract
infections
• Other signs/symptoms
o With mild disease, may be asymptomatic
o Chest
• Wheezing, chronic cough, hemoptysis, progressive respiratory
failure, cor pulmonale
• Digital clubbing
• Pansinusitis and nasal polyps
o Intestinal tract
• Meconium peritonitis, meconium ileus (10-15% of newborns
with CF), distal intestinal obstruction syndrome = meconium ileus
equivalent, intussusception, rectal prolapse.
10. Clinical presentation
o Hepatobiliary
• Cirrhosis, portal hypertension, hypersplenism, esophageal
varices that may bleed, cholelithiasis
o Pancreas
• Steatorrhea: Frequent, bulky, greasy, large, foul-smelling stools
that float in water
• 8% develop diabetes mellitus
• Occasional deficiency of vitamins A, D, E, and K
o Urogenital
• Delayed sexual development, azoospermia due to failure of
vas deferens to develop, undescended testes, hydrocele,
secondary amenorrhea
o Dry skin, salty taste to skin
o Failure to thrive
11.
12. RADIOLOGICAL EVALUATION
Medical imaging provides an invaluable service for the
diagnosis and treatment of cystic fibrosis.
Each modality is valuable in detecting different symptoms
that can arise due to cystic fibrosis.
Possible connections between abdominal and pulmonary
pathologies have increased the likelihood of catching cystic
fibrosis using multiple imaging modalities together.
14. PRE-NATAL DIAGNOSIS
pre-natal diagnosis of CF and post-natal complications is
well documented
Fetuses with CF have been associated with hyperechogenic
fetal bowel detected by ultrasound during the second and
third trimesters of pregnancy.
Bowel is considered hyperechogenic if its echogenicity
(brightness) is equal to or greater than that of the adjacent
iliac bone
17. Radiography
Chest x-rays are insensitive to the early changes of cystic
fibrosis, with changes seen on HRCT in 65% of patients with
CF and normal CXR. Later changes include:
Hyperinflation
bronchiectasis
lobar collapse
pulmonary arterial enlargement due to pulmonary
arterial hypertension is seen in patients with long
standing disease
18.
19.
20. Brasfield scoring system
air trapping linear
markings
nodular cystic
lesions
large lesions general severity
0: absent
4: most severe
0: absent
4: most severe
0: absent
4: most severe
0: absent
3: segmental/lobar
atelectasis and
pneumonia
5: multiple
atelectasis and
pneumonia
0: absent
1-3: increasing severity
4: complications
(cardiac enlargement,
pneumothorax)
5: most severe
Is a scoring system for cystic fibrosis. It is based on plain film radiographic findings
and has been reported to have a good correlation with pulmonary function
Overall score: 25-total de merit points (the lower the overall score, the worse the
disease severity)
Minimum possible score: 3
21. HRCT chest
HRCT has become indispensable in the monitoring of CF
patients, and is used to guide therapy and assess
response to treatment, as it not only correlates with lung
function.
Typically scans are repeated every 6 to 18 months
depending on institution and clinical course.
Mucous plugging is of particular importance as it is
thought to precede infective exacerbations and thus
identification of such plugging may be used to trigger
changes in therapy.
The use of low dose protocols is able to significantly reduce cumulative radiation dose
22. HRCT chest findings
bronchial wall
and / or
Peribronchial
interstitial
thickening
acute infectious
bronchiolitis
bronchiectasis mosaic
attenuation
pattern
mucus plugging
within bronchi
especially early in
the disease
• especially early
in the disease
• tree-in-bud
appearance
• centrilobular
nodular
opacities
• branching
opacities
• progresses with
time from
cylindrical to
varicose to
cystic
• signet ring sign
due to air
trapping
best seen on
expiratory scans
finger in glove
appearance
23.
24.
25.
26. The nose and sinuses
The upper airways are very frequently affected in CF, with
over 75% of patients reporting some kind of sinus or nasal
symptom such as nasal or sinus obstruction, nasal
discharge, post-nasal drip and facial pain
Imaging findings includes small hypoplasic sinuses,
thickened nasal turbinates and thickened mucosa of the
sinuses.
Chronic inflammation and thickening of the mucosa of
the nasal cavities and sinuses result in formation of
inflammatory polyps and the accumulation of mucus in
obstructed sinuses
27.
28. Extra pulmonary manifestation
Abdominal manifestations in cystic fibrosis are common,
varied and nearly all organ systems can be
affected. Only 39% of patients with cystic fibrosis (CF)
have pulmonary symptoms as their sole complaint.
This include:
Pancreatic manifestations
Hepatobiliary manifestations
Gastrointestinal manifestations
Renal manifestations
Musculoskeletal manifestations
29. Pancreatic manifestations
Autolysis of the pancreas due to viscous pancreatic
enzymes and obstructed pancreatic ducts is known to
start during intra-uterine life
This leads to fibrosis, atrophy and replacement of the
pancreas by fat which clinically results in exocrine
pancreatic insufficiency and malabsorption in up to 90%
of patients.
Pancreatic cysts are a relatively common finding in CF
and can vary in size and number. Rarely, cysts can
replace the entire pancreas in a condition called
pancreatic cystosis, which can cause pain through mass
effect
30.
31.
32.
33. Hepatobiliary manifestations
Hepatobiliary manifestations are common in CF and
include fatty infiltration of the liver (steatosis), focal
biliary cirrhosis with portal hypertension,
microgallbladder and gallstones.
Patients are more frequently asymptomatic, but liver
disease is the second most common cause of death in
CF
Splenomegaly as a result of portal hypertension can
be seen as massive enlargement of the spleen causing
pain, dyspnoea and signs of hypersplenism and
sometimes complicated with splenic infarcts or
subcapsular haematomas
34.
35. Gastrointestinal manifestations
Meconium ileus: Bowel blockage of the newborn or
meconium ileus is seen in 15-20% of neonates with CF
and 25% of infants with meconium ileus prove to have
CF
Classically, above the obstruction the bowel is greatly
distended with fluid content, while below this level, the
distal ileum and colon are collapsed. Soon after birth,
usually in 24 to 48 hours, the newborn will present with
abdominal distension and vomiting
36.
37. Gastrointestinal manifestations
Distal intestinal obstructive syndrome: DIOS is the equivalent
of meconium ileus in adults.
It affects up to 15% of CF patients as a result of thickened
intraluminal secretions, undigested food secondary to
exocrine pancreatic insufficiency and impaired bowel
motility
Abdominal radiograph and CT show dilated small bowel
loops with fluid levels and faecal material within the small
bowel (“small bowel faeces”).
38.
39. Gastrointestinal manifestations
Intussusception: Intussusception is more common in CF
patients than in the general population and is seen in 1% of
paediatric patients. It is rare in adulthood and comprises 5%
of all intussusceptions and 1% of bowel obstructions
Imaging reveals a bowel-within-bowel configuration which
is pathognomonic of the condition
40.
41. Gastrointestinal manifestations
Fibrosing colonopathy: has been reported in association
with children prescribed high strength pancreatic enzyme
supplements.
Imaging has a limited role demonstrating non-specific large
bowel wall thickening and final diagnosis is usually obtained
with histology, which depicts submucosal fibrosis
42. Renal manifestation
Nephrolithiasis: is another important differential diagnosis
of acute abdominal pain as there is a reported
increased frequency of renal stone disease in CF in
comparison to the general population.
Initial assessment can be made with a CT of the kidneys,
ureters and
bladder (CT KUB) to confirm the diagnosis or with an
ultrasound scan if obstruction and hydronephrosis is
suspected in a patient with know nephrolithiasis
43.
44. Musculoskeletal manifestations
Hypertrophic pulmonary osteoarthropathy
This condition consists of a triad of clubbing,
symmetric arthritis and periosteal new bone
formation
Firstly associated with brochogenic carcinoma,
it is also recognised in
bronchiectasis, chronic lung inflammation and
infection and CF
45.
46. DIFFERENTIAL DIAGNOSIS
Recurrent aspiration
• Often have predisposing neuromuscular abnormalities
• Bronchiectasis in lower lobes and posterior segments
i.e. the dependent portions of lungs
• Pneumonia can sometimes be related to a preceding episode of choking
or gagging with feeds
Asthma
• Especially when complicated by ABPA and bronchiectasis
• May be seasonal
Bronchopulmonary dysplasia
• Improves as the patient ages
• Recurrent aspiration
• History of prematurity
Immotile cilia syndrome
• Dysfunction of cilia in respiratory and auditory epithelium
• Lower lobe predominant
• May have situs inversus
• May have associated cardiac anomalies
48. 24-year-old man with right-sided pleuritic chest pain
presents to the Emergency Department.
49. ▶ Chest radiograph shows increased lung volumes and diffuse interstitial opacities
emanating from the hila; many of them are parallel, indicative of bronchiectasis
(worse in the upper lungs). Branching tubular densities in the right upper lobe
represent mucus impaction in dilated bronchi. A small right pneumothorax is also
present.
▶ CT findings include bronchial wall thickening, tubular and varicoid bronchiectasis
signet-ring, mucoid-impacted bronchioles causing tree-in-bud, and mosaic
50. 26 years old male chronically ill patient
with nausea vomiting and dehydration
Ct Images of the chest
in lung windows
demonstrated profound,
diffuse, cystic dilatation
of the airway. CT images
of upper abdomen in
soft tissue windows
demonstrate a nodular,
cirrhotic appearing liver,
splenomegaly and
complete fatty
replacement of the
pancreases