Lymphangioleiomyomatosis (LAM) is a rare lung disease that affects premenopausal women. It involves the proliferation of abnormal smooth muscle-like cells (LAM cells) in the lungs, lymphatic system, and kidneys. This leads to cyst formation in the lungs and lymphatic obstruction, causing symptoms like shortness of breath, pneumothorax, chylous ascites, and lymphangioleiomyomas. The disease has links to tuberous sclerosis complex and is exacerbated by estrogen. Diagnosis involves imaging and biopsy to identify LAM cells. Treatment focuses on managing symptoms, with lung transplantation as a last resort.
New technology called Electromagnetic Navigation Bronchoscopy® (ENB) that uses virtual bronchoscopy and real time 3-dimensional CT images that enable me to localize these peripheral lung nodules for diagnosis and treatment. This outpatient procedure is minimally invasive and therefore has a small risk of pneumothorax (2-3%) and its published diagnostic yield rates range from 67% - 86%
New technology called Electromagnetic Navigation Bronchoscopy® (ENB) that uses virtual bronchoscopy and real time 3-dimensional CT images that enable me to localize these peripheral lung nodules for diagnosis and treatment. This outpatient procedure is minimally invasive and therefore has a small risk of pneumothorax (2-3%) and its published diagnostic yield rates range from 67% - 86%
An approach to Interstitial Lung Disease / Diffuse Parenchymal Lung DiseaseThomas Kurian
YouTube link: https://youtu.be/gPr31qrivUc
An approach to Diffuse Parenchymal Lung disease / Interstitial Lung disease with emphasis on the idiopathic causes.
An approach to Interstitial Lung Disease / Diffuse Parenchymal Lung DiseaseThomas Kurian
YouTube link: https://youtu.be/gPr31qrivUc
An approach to Diffuse Parenchymal Lung disease / Interstitial Lung disease with emphasis on the idiopathic causes.
multiple myloma
By: Nader Amir Al-assadi
Supervised by : Dr/ Ghazi Alariqe
taiz university
Multiple myeloma (MM) is a plasma cell malignancy in which monoclonal plasma cells proliferate in bone marrow, resulting in an over abundance of monoclonal para protein (M protein), destruction of bone, and displacement of other hematopoietic cell lines.
The precise etiology of MM has not yet been established.
Roles have been suggested for a variety of factors, including genetic causes, environmental or occupational causes,radiation, chronic inflammation, and infection .
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.
Anti ulcer drugs and their Advance pharmacology ||
Anti-ulcer drugs are medications used to prevent and treat ulcers in the stomach and upper part of the small intestine (duodenal ulcers). These ulcers are often caused by an imbalance between stomach acid and the mucosal lining, which protects the stomach lining.
||Scope: Overview of various classes of anti-ulcer drugs, their mechanisms of action, indications, side effects, and clinical considerations.
These lecture slides, by Dr Sidra Arshad, offer a quick overview of physiological basis of a normal electrocardiogram.
Learning objectives:
1. Define an electrocardiogram (ECG) and electrocardiography
2. Describe how dipoles generated by the heart produce the waveforms of the ECG
3. Describe the components of a normal electrocardiogram of a typical bipolar leads (limb II)
4. Differentiate between intervals and segments
5. Enlist some common indications for obtaining an ECG
Study Resources:
1. Chapter 11, Guyton and Hall Textbook of Medical Physiology, 14th edition
2. Chapter 9, Human Physiology - From Cells to Systems, Lauralee Sherwood, 9th edition
3. Chapter 29, Ganong’s Review of Medical Physiology, 26th edition
4. Electrocardiogram, StatPearls - https://www.ncbi.nlm.nih.gov/books/NBK549803/
5. ECG in Medical Practice by ABM Abdullah, 4th edition
6. ECG Basics, http://www.nataliescasebook.com/tag/e-c-g-basics
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Ozempic: Preoperative Management of Patients on GLP-1 Receptor Agonists Saeid Safari
Preoperative Management of Patients on GLP-1 Receptor Agonists like Ozempic and Semiglutide
ASA GUIDELINE
NYSORA Guideline
2 Case Reports of Gastric Ultrasound
Title: Sense of Smell
Presenter: Dr. Faiza, Assistant Professor of Physiology
Qualifications:
MBBS (Best Graduate, AIMC Lahore)
FCPS Physiology
ICMT, CHPE, DHPE (STMU)
MPH (GC University, Faisalabad)
MBA (Virtual University of Pakistan)
Learning Objectives:
Describe the primary categories of smells and the concept of odor blindness.
Explain the structure and location of the olfactory membrane and mucosa, including the types and roles of cells involved in olfaction.
Describe the pathway and mechanisms of olfactory signal transmission from the olfactory receptors to the brain.
Illustrate the biochemical cascade triggered by odorant binding to olfactory receptors, including the role of G-proteins and second messengers in generating an action potential.
Identify different types of olfactory disorders such as anosmia, hyposmia, hyperosmia, and dysosmia, including their potential causes.
Key Topics:
Olfactory Genes:
3% of the human genome accounts for olfactory genes.
400 genes for odorant receptors.
Olfactory Membrane:
Located in the superior part of the nasal cavity.
Medially: Folds downward along the superior septum.
Laterally: Folds over the superior turbinate and upper surface of the middle turbinate.
Total surface area: 5-10 square centimeters.
Olfactory Mucosa:
Olfactory Cells: Bipolar nerve cells derived from the CNS (100 million), with 4-25 olfactory cilia per cell.
Sustentacular Cells: Produce mucus and maintain ionic and molecular environment.
Basal Cells: Replace worn-out olfactory cells with an average lifespan of 1-2 months.
Bowman’s Gland: Secretes mucus.
Stimulation of Olfactory Cells:
Odorant dissolves in mucus and attaches to receptors on olfactory cilia.
Involves a cascade effect through G-proteins and second messengers, leading to depolarization and action potential generation in the olfactory nerve.
Quality of a Good Odorant:
Small (3-20 Carbon atoms), volatile, water-soluble, and lipid-soluble.
Facilitated by odorant-binding proteins in mucus.
Membrane Potential and Action Potential:
Resting membrane potential: -55mV.
Action potential frequency in the olfactory nerve increases with odorant strength.
Adaptation Towards the Sense of Smell:
Rapid adaptation within the first second, with further slow adaptation.
Psychological adaptation greater than receptor adaptation, involving feedback inhibition from the central nervous system.
Primary Sensations of Smell:
Camphoraceous, Musky, Floral, Pepperminty, Ethereal, Pungent, Putrid.
Odor Detection Threshold:
Examples: Hydrogen sulfide (0.0005 ppm), Methyl-mercaptan (0.002 ppm).
Some toxic substances are odorless at lethal concentrations.
Characteristics of Smell:
Odor blindness for single substances due to lack of appropriate receptor protein.
Behavioral and emotional influences of smell.
Transmission of Olfactory Signals:
From olfactory cells to glomeruli in the olfactory bulb, involving lateral inhibition.
Primitive, less old, and new olfactory systems with different path
The prostate is an exocrine gland of the male mammalian reproductive system
It is a walnut-sized gland that forms part of the male reproductive system and is located in front of the rectum and just below the urinary bladder
Function is to store and secrete a clear, slightly alkaline fluid that constitutes 10-30% of the volume of the seminal fluid that along with the spermatozoa, constitutes semen
A healthy human prostate measures (4cm-vertical, by 3cm-horizontal, 2cm ant-post ).
It surrounds the urethra just below the urinary bladder. It has anterior, median, posterior and two lateral lobes
It’s work is regulated by androgens which are responsible for male sex characteristics
Generalised disease of the prostate due to hormonal derangement which leads to non malignant enlargement of the gland (increase in the number of epithelial cells and stromal tissue)to cause compression of the urethra leading to symptoms (LUTS
Flu Vaccine Alert in Bangalore Karnatakaaddon Scans
As flu season approaches, health officials in Bangalore, Karnataka, are urging residents to get their flu vaccinations. The seasonal flu, while common, can lead to severe health complications, particularly for vulnerable populations such as young children, the elderly, and those with underlying health conditions.
Dr. Vidisha Kumari, a leading epidemiologist in Bangalore, emphasizes the importance of getting vaccinated. "The flu vaccine is our best defense against the influenza virus. It not only protects individuals but also helps prevent the spread of the virus in our communities," he says.
This year, the flu season is expected to coincide with a potential increase in other respiratory illnesses. The Karnataka Health Department has launched an awareness campaign highlighting the significance of flu vaccinations. They have set up multiple vaccination centers across Bangalore, making it convenient for residents to receive their shots.
To encourage widespread vaccination, the government is also collaborating with local schools, workplaces, and community centers to facilitate vaccination drives. Special attention is being given to ensuring that the vaccine is accessible to all, including marginalized communities who may have limited access to healthcare.
Residents are reminded that the flu vaccine is safe and effective. Common side effects are mild and may include soreness at the injection site, mild fever, or muscle aches. These side effects are generally short-lived and far less severe than the flu itself.
Healthcare providers are also stressing the importance of continuing COVID-19 precautions. Wearing masks, practicing good hand hygiene, and maintaining social distancing are still crucial, especially in crowded places.
Protect yourself and your loved ones by getting vaccinated. Together, we can help keep Bangalore healthy and safe this flu season. For more information on vaccination centers and schedules, residents can visit the Karnataka Health Department’s official website or follow their social media pages.
Stay informed, stay safe, and get your flu shot today!
These simplified slides by Dr. Sidra Arshad present an overview of the non-respiratory functions of the respiratory tract.
Learning objectives:
1. Enlist the non-respiratory functions of the respiratory tract
2. Briefly explain how these functions are carried out
3. Discuss the significance of dead space
4. Differentiate between minute ventilation and alveolar ventilation
5. Describe the cough and sneeze reflexes
Study Resources:
1. Chapter 39, Guyton and Hall Textbook of Medical Physiology, 14th edition
2. Chapter 34, Ganong’s Review of Medical Physiology, 26th edition
3. Chapter 17, Human Physiology by Lauralee Sherwood, 9th edition
4. Non-respiratory functions of the lungs https://academic.oup.com/bjaed/article/13/3/98/278874
2. Lymphangioleiomyomatosis (LAM) is a rare disorder resulting
from proliferation in the lung, kidney, and axial lymphatics of
leiomyoma (LAM cells) that exhibit features of
neoplasia. Cystic destruction of the lung with progressive
pulmonary dysfunction(pneumothorax , dyspnea), and the
presence of abdominal tumors (eg, angiomyolipomas
[AML], lymphangioleiomyomas) characterize the disease.
4. LAM typically occurs in premenopausal
women, suggesting the involvement of
female hormones in disease pathogenesis.
LAM can occur with increased frequency in
patients with tuberous sclerosis complex
(TSC), an autosomal dominant disorder due
to mutations in the TSC1 or TSC2 gene.
5. Proliferation of lymphangioleiomyomatosis (LAM) cells
may obstruct bronchioles,possibly leading to airflow
obstruction, air trapping, formation of bullae, and
pneumothoraces. Obstruction of lymphatics may result in
lymphangioleiomyomas, chylothorax, and chylous ascites.
Obstruction of venules may result in hemosiderosis and
hemoptysis. Excessive proteolytic activity, which relates
to an imbalance of the elastase/alpha1-antitrypsin system
or metalloprotease (MMPs) and their inhibitors (tissue
inhibitors of metalloproteases [TIMPs]), may be
important in lung destruction and formation of cysts.
PATHOLOGY
8. Race
No racial predilection has been reported for
lymphangioleiomyomatosis (LAM).
Sex
Lymphangioleiomyomatosis (LAM) primarily is a disease of
women (esp in premenopausal time /pregnancy) ;
however, rare case reports describe LAM in men with TSC.
Age
Although primarily a disease of women of childbearing
age, lymphangioleiomyomatosis (LAM) has also been reported
in postmenopausal patients.
EPIDEMIOLOGY:
9. CLINICAL MANIFESTATIONS
Common lymphangioleiomyomatosis (LAM) symptoms include the
following:
Dyspnea
Cough
Less common symptoms (findings) are associated with the
following:
Pneumothorax, pneumoperitoneum
Chylothorax, chylous ascites
Lymphedema
Exacerbations of LAM have been reported to occur during
pregnancy and menstruation, as well as with exogenous estrogen
use.
LAM may be present in approximately 30-40% of patients with
tuberous sclerosis complex (TSC).
10. Causes
The etiology of lymphangioleiomyomatosis
(LAM) is unknown; however, the fact that the
condition occurs primarily in premenopausal
women and that it is exacerbated by high
estrogen states suggests a role for hormones.
The link with TSC suggests a genetic
component.
11. Laboratory Studies
Vascular endothelial growth factor-D (VEGF-D)
levels, above a certain threshold, are found in
lymphangioleiomyomatosis (LAM) but not other
cystic lung diseases. Hence, a serologic test for
VEGF-D may be useful for diagnosis. A prior study
has shown that high levels of VEGF-D are
associated with lymphatic involvement
(eg, lymphangioleiomyomas, adenopathy) in LAM.
12. Imaging Studies
*Chest radiographs in lymphangioleiomyomatosis (LAM)
may be normal. Fine reticular or reticulo-nodular interstitial
infiltrate with preserved lung volumes is the most commonly
observed abnormality. Pleural effusions may be present.
Patients may present with pneumothorax.
*CT or MRI scanning of the head may reveal an incidental or
symptomatic meningioma, which occurs with increased
frequency in patients with LAM .
*On bone densitometry, patients with LAM exhibit
accelerated osteoporosis
13.
14. *CT scan and high-resolution CT scan findings include
the following:
Diffuse thin-walled cysts - The defining appearance in LAM
Adenopathy and thoracic duct dilatation
Pleural effusion
Pneumothorax
Ground-glass opacities - May be present, perhaps representing alveolar
hemorrhage or interstitial disease
Pericardial effusion
Multifocal multinodular pneumocyte hyperplasia (MMPH) - Can be seen in
patients with tuberous sclerosus complex (TSC), but the pathology is distinct
from LAM
*Abdominal imaging by either ultrasound or CT scan may
demonstrate the following:
Angiomyolipoma (AML) - Benign tumors (kidney, liver, spleen) containing
smooth muscle, thick-walled blood vessels, and mature adipose tissue
Retroperitoneal or mediastinal lymphangioleiomyomas
15.
16. Other Tests
Pulmonary function testing, decreased diffusing capacity for
carbon monoxide is the most common abnormality seen, and it
is often markedly reduced.Hypoxemia at rest, worsening with
exercise, is a common finding.
On spirometry, airflow obstruction is the most frequent
abnormality; restriction or mixed obstruction and restriction can
also be seen.
Lung volumes may show an increased ratio of residual volume
to total lung capacity
17. Procedures
Histologic diagnosis can be made by performing an open
lung, video-assisted thoracoscopic, or transbronchial biopsy
(TBB); the amount of tissue obtained from TBB may be
insufficient to confirm a diagnosis. Lymphangioleiomyomatosis
(LAM) cells react with human melanoma black (HMB)–45, an
antibody generated against an extract of melanoma.HMB-45
staining is used for the identification of LAM cells and may help
in confirming LAM on TBB.
With classic high-resolution CT scans of the lung and associated
findings of LAM (eg, tuberous sclerosis
complex, angiomyolipoma, lymphangioleiomyomas), histologic
confirmation may be unnecessary.
19. Microscopic pathology
In histological sections of the lung, the following are observed:
Proliferation of lymphangioleiomyomatosis (LAM) cells (spindle-
shaped cells with small nuclei, larger epithelioid cells with clear
cytoplasm and round nuclei) having a smooth muscle cell
phenotype.
Loss of alveoli with cyst formation
Aggregates of LAM cells abutting cystic spaces
Distal airway narrowing, thickened arterial walls with venous
occlusion, and hemosiderosis
20.
21. Macroscopic pathology
Cysts are evenly distributed in all lung fields. Lymph nodes
(retroperitoneal and pelvic) may appear pale and spongy; large
chyle-filled cysts can be found within the axial lymphatic system.
The thoracic duct may be large, spongy, and sausage-like.
22. Figure 1. Chest CT scan of a patient with LAM (A)
showing numerous thin-walled cysts distributed
throughout the lungs. (B) The lung parenchyma is
almost completely replaced by very small
cysts. Figure 2. Abdominal CT scan of a patient
with LAM showing angiomyolipomas involving both
kidneys. Figure 3. Abdominal CT scan of a patient
with LAM showing a large lymphangioleiomyoma
located in the retroperitoneal area and
surrounding the aorta and inferior vena cava.
Figure 4 A and B. LAM nodule comprising spindle-
shaped cells and larger epithelioid cells (A).
Nodules of various sizes (B) are seen in involved
lung (hematoxylin-eosin; original magnification
x50). Figure 5. Immunohistochemistry of LAM
cells. Immunoperoxidase method and
counterstaining with hematoxylin. A and B:
Immunoreactivity with a-smooth muscle actin
antibodies. LAM cells show strong reactivity (A).
Pulmonary vascular smooth muscle cells are also
strongly positive (arrow). LAM cells in the walls of
the lung cysts are also strongly reactive (arrow)
(B) (original magnification x250 for each). C:
Immunoreactivity with monoclonal antibody HMB-
45. Immunoreactive cells are distributed in the
periphery of LAM lung nodules (arrow) (original
magnification x250). D: Immunoreactivity with
monoclonal antibody HMB-45. Higher-
magnification view of tissue shown in C. A strong
granular reaction is present in large epithelioid
LAM cells adjacent to epithelial cells covering LAM
lung nodules (arrow) (D)
23. In involved lymph nodes and the thoracic duct, there are
interlacing bundles of LAM cells, which may invade the walls of
the lymphatics.
Immunohistochemical staining of LAM lesions demonstrates the
following:
Reactivity with anti–alpha-smooth actin antibodies, which is
consistent with smooth-muscle differentiation
Estrogen and progesterone receptors
Immunoreactivity with the monoclonal antibody HMB-45, which
recognizes LAM cells with epithelioid features; rarely, spindle
cells are also HMB-45 positive
Renal and hepatic AMLs, as well as lymphangioleiomyomas, can
also be detected with HMB-45 antibody
24. Figure 6. Left panel: close-up of LAM
nodule (hematoxylin-eosin). Right
panel: same nodule showing positive
immunocytochemistry stain for HMB
45 (original magnification x200).
Figure 7. Characteristics of LAM cells
(A-C). Reaction of LAM cells cultured
from lung and pulmonary artery
smooth muscle cells (PASM) with
monoclonal antibody against SMA (A).
Reaction of cultured LAM cells and
melanoma cells (MALME-3M) with
monoclonal antibody HMB-45 (B).
Fluorescence in situ hybridization
(FISH) for TSC1 (green) and TSC2 (red)
in LAM cells showing normal presence
of two of each alleles as well as
abnormal presence of TSC2 alleles
(left) (C). FISH for TSC1 (green, arrow)
and TSC2 (red, arrowhead) in LAM cell
with one (right) or two TSC2 (left)
alleles (C). Bar, 20 µm.
25. TREATMENT
General care for patients with lymphangioleiomyomatosis (LAM) addresses
the following findings:
Pleural effusions - Consider chemical pleurodesis; surgical obliteration of
the pleural space; medium-chain triglyceride (MCT [not a component of
chyle]), lipid-free diet to reduce chyle flow (utility unknown)
Ascites - Paracentesis, MCT diet (utility unknown)
Airways disease and hypoxemia - Bronchodilators may be of benefit[25] ;
supplemental oxygen, pulmonary rehabilitation, smoking cessation
Standard vaccination for respiratory infections
Osteoporosis - Standard surveillance and treatment; avoid exogenous
estrogens
Lung transplantation
26. Possible options for hormonal manipulation include the
following:
Medroxyprogesterone - Utility not known; recent case series does
not support its use
Gonadotropin-releasing hormone agonists - Utility not known; few
case reports support their use
Tamoxifen does not appear to be effective and is not recommended
due to estrogen receptor agonist activity
Rate of decline in lung function trends to be less in postmenopausal
women (eg, surgical oophorectomy, age)
27. New experimental therapies include the following:
Rapamycin - Shrinks angiomyolipomas and improves lung
function, but response is not sustained
Doxycycline - Antiangiogenic, antibiotic, and matrix
effects
Aromatase inhibitors - Antiestrogenic effect