A detailed description of sarcoidosis, pulmonary in specific but also covering the other systems. a rare entity in india or a better way to say, often an overlooked disease.
Prof. Md. Khairul Hassan Jessy
Professor of Respiratory Medicine
National Institute of Diseases of the Chest and Hospital (NIDCH)
Mohakhali, Dhaka, Bangladesh
A detailed description of sarcoidosis, pulmonary in specific but also covering the other systems. a rare entity in india or a better way to say, often an overlooked disease.
Prof. Md. Khairul Hassan Jessy
Professor of Respiratory Medicine
National Institute of Diseases of the Chest and Hospital (NIDCH)
Mohakhali, Dhaka, Bangladesh
Interstitial lung disease is a general category that includes many different lung conditions. All interstitial lung diseases affect the interstitium, a part of the lungs' anatomic structure.
Some of the types of interstitial lung disease include:
Interstitial pneumonia: Bacteria, viruses, or fungi may infect the interstitium of the lung. A bacterium called Mycoplasma pneumonia is the most common cause.
Idiopathic pulmonary fibrosis : A chronic, progressive form of fibrosis (scarring) of the interstitium. Its cause is unknown.
Nonspecific interstitial pneumonitis: Interstitial lung disease that's often present with autoimmune conditions (such as rheumatoid arthritis or scleroderma).
Interstitial lung disease is a general category that includes many different lung conditions. All interstitial lung diseases affect the interstitium, a part of the lungs' anatomic structure.
Some of the types of interstitial lung disease include:
Interstitial pneumonia: Bacteria, viruses, or fungi may infect the interstitium of the lung. A bacterium called Mycoplasma pneumonia is the most common cause.
Idiopathic pulmonary fibrosis : A chronic, progressive form of fibrosis (scarring) of the interstitium. Its cause is unknown.
Nonspecific interstitial pneumonitis: Interstitial lung disease that's often present with autoimmune conditions (such as rheumatoid arthritis or scleroderma).
References
Fisherman's Pulmonary Diseases & Disorders 5th ed
Murray & Nadel's Textbook of Respiratory Medicine 6th ed
Croatian & Douglas Respiratory Medicine 5th ed
Harrison's Principle of Internal Medicine 19th edition
NEJM Article
Management of acute lymphoblatic leukemia with light on etiology, clinical features, diagnosis and different aspects of management including chemotherapy and radiation therapy
- Video recording of this lecture in English language: https://youtu.be/lK81BzxMqdo
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CDSCO and Phamacovigilance {Regulatory body in India}NEHA GUPTA
The Central Drugs Standard Control Organization (CDSCO) is India's national regulatory body for pharmaceuticals and medical devices. Operating under the Directorate General of Health Services, Ministry of Health & Family Welfare, Government of India, the CDSCO is responsible for approving new drugs, conducting clinical trials, setting standards for drugs, controlling the quality of imported drugs, and coordinating the activities of State Drug Control Organizations by providing expert advice.
Pharmacovigilance, on the other hand, is the science and activities related to the detection, assessment, understanding, and prevention of adverse effects or any other drug-related problems. The primary aim of pharmacovigilance is to ensure the safety and efficacy of medicines, thereby protecting public health.
In India, pharmacovigilance activities are monitored by the Pharmacovigilance Programme of India (PvPI), which works closely with CDSCO to collect, analyze, and act upon data regarding adverse drug reactions (ADRs). Together, they play a critical role in ensuring that the benefits of drugs outweigh their risks, maintaining high standards of patient safety, and promoting the rational use of medicines.
New Drug Discovery and Development .....NEHA GUPTA
The "New Drug Discovery and Development" process involves the identification, design, testing, and manufacturing of novel pharmaceutical compounds with the aim of introducing new and improved treatments for various medical conditions. This comprehensive endeavor encompasses various stages, including target identification, preclinical studies, clinical trials, regulatory approval, and post-market surveillance. It involves multidisciplinary collaboration among scientists, researchers, clinicians, regulatory experts, and pharmaceutical companies to bring innovative therapies to market and address unmet medical needs.
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NVBDCP.pptx Nation vector borne disease control programSapna Thakur
NVBDCP was launched in 2003-2004 . Vector-Borne Disease: Disease that results from an infection transmitted to humans and other animals by blood-feeding arthropods, such as mosquitoes, ticks, and fleas. Examples of vector-borne diseases include Dengue fever, West Nile Virus, Lyme disease, and malaria.
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
Title: Sense of Taste
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 structure and function of taste buds.
Describe the relationship between the taste threshold and taste index of common substances.
Explain the chemical basis and signal transduction of taste perception for each type of primary taste sensation.
Recognize different abnormalities of taste perception and their causes.
Key Topics:
Significance of Taste Sensation:
Differentiation between pleasant and harmful food
Influence on behavior
Selection of food based on metabolic needs
Receptors of Taste:
Taste buds on the tongue
Influence of sense of smell, texture of food, and pain stimulation (e.g., by pepper)
Primary and Secondary Taste Sensations:
Primary taste sensations: Sweet, Sour, Salty, Bitter, Umami
Chemical basis and signal transduction mechanisms for each taste
Taste Threshold and Index:
Taste threshold values for Sweet (sucrose), Salty (NaCl), Sour (HCl), and Bitter (Quinine)
Taste index relationship: Inversely proportional to taste threshold
Taste Blindness:
Inability to taste certain substances, particularly thiourea compounds
Example: Phenylthiocarbamide
Structure and Function of Taste Buds:
Composition: Epithelial cells, Sustentacular/Supporting cells, Taste cells, Basal cells
Features: Taste pores, Taste hairs/microvilli, and Taste nerve fibers
Location of Taste Buds:
Found in papillae of the tongue (Fungiform, Circumvallate, Foliate)
Also present on the palate, tonsillar pillars, epiglottis, and proximal esophagus
Mechanism of Taste Stimulation:
Interaction of taste substances with receptors on microvilli
Signal transduction pathways for Umami, Sweet, Bitter, Sour, and Salty tastes
Taste Sensitivity and Adaptation:
Decrease in sensitivity with age
Rapid adaptation of taste sensation
Role of Saliva in Taste:
Dissolution of tastants to reach receptors
Washing away the stimulus
Taste Preferences and Aversions:
Mechanisms behind taste preference and aversion
Influence of receptors and neural pathways
Impact of Sensory Nerve Damage:
Degeneration of taste buds if the sensory nerve fiber is cut
Abnormalities of Taste Detection:
Conditions: Ageusia, Hypogeusia, Dysgeusia (parageusia)
Causes: Nerve damage, neurological disorders, infections, poor oral hygiene, adverse drug effects, deficiencies, aging, tobacco use, altered neurotransmitter levels
Neurotransmitters and Taste Threshold:
Effects of serotonin (5-HT) and norepinephrine (NE) on taste sensitivity
Supertasters:
25% of the population with heightened sensitivity to taste, especially bitterness
Increased number of fungiform papillae
2. Sarcoidosis
• Multisystem disorder of unknown etiology
that most commonly affects the lungs, but
can also affect other organs.
• Beethoven is thought to have been the first
person described with this condition.
4. History of sarcoidosis
• In 1899, the pioneering Norwegian
dermatologist Caesar Boeck describe skin
nodules characterized by compact,
sharply defined foci of "epithelioid cells
with large pale nuclei and also a few giant
cells .
• Thinking this resembled sarcoma, he
called the condition "multiple benign
sarcoid of the skin.
5. Epidemiology
• All racial .
• All ethnic groups.
– More prevalent in Swedes, Danes, and US
blacks
• All ages (with the incidence peaking at 20 to 39 years).
• M-F ratio 2:1.
• Affects siblings of first- or second- degree
relatives in 15% of patients with
sarcoidosis.
6. Etiology and Pathogenesis
• Cause is unknown, although both genetic and
environmental factors suspected.
• Theory that disease develops in genetically
predetermined hosts who are exposed to certain
environmental agents that trigger an
exaggerated inflammatory immune response
leading to granuloma formation.
7. • Hallmark is noncaseating granulomas,
composed of a central core of epithelioid
histocytes and multinucleated giant cells.
• Activated T cells and macrophages
accumulate at site of inflammation.
• Release chemo attractants and GF’s lead
to cellular proliferation and granuloma
formation.
• Progressive granulomatous inflammation
leads to injury, dysfunction, and
destruction of the affected organs.
10. The following have been suggested as
possible candidates that might play a
role in causing sarcoidosis:
• Mycobacteria, such as Mycobacterium tuberculosis, and
atypical pathogens have been suggested.
• Fungi and viruses, particularly Mycoplasma, Chlamydia,
and Epstein-Barr virus, have been unconvincingly
implicated.
11. Environmental Causes
• Some of the earliest studies of sarcoidosis reported
associations with exposures to irritants found in rural
settings, such as emissions from wood-burning stoves
and tree pollen.
• More recently, associations with sarcoidosis and
exposure to inorganic particles, insecticides, and
moldy environments have been reported.
• Occupational studies have shown positive associations
with service in the U.S. Navy, metalworking,
firefighting, and the handling of building supplies.
13. Clinical Presentation
• 30-50% of patients are asymptomatic and are
diagnosed on routine CXR.
• One third have non-specific symptoms of fever,
fatigue, weight loss and malaise.
• A clinical variant of sarcoidosis, Lofgren’s
syndrome, includes constellation of erythema
nodosum, polyarthritis, and BHL. Remission
occurs in 80%.
14. Clinical Presentation
• Onset of sarcoidosis in white patients is usually
asymptomatic.
• African Americans tend to present with an earlier
onset and a more aggressive and severe clinical
course.
• Chronic pulmonary sarcoidosis and the
disfiguring cutaneous lesions of lupus pernio are
also more common in African Americans.
15. Clinical Presentation
• A progressive course is more likely in:
– Age of onset > 40 yrs
– Black race
– Cardiac or renal involvement
– Lupus pernio
– Chronic uveitis
– Hypercalcemia
– Nasal mucosal involvement
– Cystic bone lesions
– Neurosarcoidosis
– Pulmonary fibrosis
16.
17. Systems affected by Sarcoidosis
Cardiac
30%
Palpitations, syncope, dizziness, chest pain,
arrhythmia, sudden death
Cutaneous
25%
Erythema nodosum, lupus pernio, plaques,
subcutaneous nodules, maculopapular eruption,
alopecia, hyper/hypopigmentation
Endocrin
e
Hypo/hyperthyroidism, adrenal insufficiency
Exocrine Painless swelling of parotid gland, keratocon-junctivis
sicca
Hepatic
50-80%
Asymptomatic or abdominal pain, abnormal LFT’s,
hepatomegaly
Lymphatic Extrapulmonary lymphadenopathy, splenomegaly
Signs and symptoms
21. Systems affected by Sarcoidosis
Neurologic
5%
Cranial nerve palsy, seizures, basal
granulomatous meningitis, hypothalamic or
pituitary lesions, hydrocephalus, peripheral
neuropathy, psychiatric
Ocular
20%
Uveitis, chorioretinitis, keratoconjunctivitis,
glaucoma, cataracts, blindness, Heerfordt
syndrome
Pulmonary
90%
Asymptomatic or dyspnea, nonproductive cough,
wheezing, radiographic findings from hilar
adenopathy to fibrosis
Renal Hypercalcemia, hypercalciuria, renal insufficiency
Signs and symptoms
22.
23. 4 Stages of Pulmonary Sarcoidosis
I Bilateral hilar lymphadenopathy
and paratracheal adenopathy
55-90%
remission
II Mediastinal adenopathy with
pulmonary parenchymal
involvements
40-70%
III Pulmonary parenchymal without
adenopathy
10-20%
IV Pulmonary fibrosis with
honeycombing
0-5%
26. Laboratory Studies
• Routine lab evaluation often is
unrevealing.
• Hypercalcemia or hypercalciuria may
occur (NCGs secrete 1,25 vitamin D).
• Hypercalcemia is seen in about 10-13%
of patients, whereas hypercalciuria is 3
times more common.
• An elevated alkaline phosphatase level
suggests hepatic involvement.
• Angiotensin converting enzyme (ACE)
levels may be elevated.
27. • NCGs secrete ACE, which may function
as a cytokine.
• Serum ACE levels are elevated in 60%
of patients at the time of diagnosis.
• Levels may be increased in fluid from
bronchoalveolar lavage or in CSF.
• Sensitivity and specificity as a
diagnostic test is limited (60 and 70%,
respectively).
• There is no clear prognostic value.
• Serum ACE levels may decline in
response to therapy.
• Decisions on treatment should not be
based on the ACE level alone.
28. Imaging Studies
• A chest radiograph is central to
evaluation.
• Routine chest CT scan adds little.
• HRCT of the chest may be helpful.
29. Biopsy specimen
• A biopsy specimen should be obtained
from the involved organ that is most
easily accessed, such as the skin,
peripheral LN, lacrimal glands, or
conjunctiva.
• If diagnosis requires pulmonary tissue,
transbronchial biopsy by means of
bronchoscopy has a diagnostic yield of at
least 85% when multiple lung segments
are sampled.
30. The central histologic finding is the presence of
NCGs with special stains negative for fungus
and mycobacteria.
31. • Sarcoidal granulomas have no unique histologic
features to differentiate them from other granulomas.
• Special stains for acid-fast bacilli and fungi, as well
as cultures of such organisms, are essential.
• If the results of lung biopsy with bronchoscopy are
negative and other organs are not obviously involved,
biopsy of intrathoracic lymph nodes, which are often
enlarged in patients with sarcoidosis, may be
necessary to confirm the diagnosis.
32. Differential Diagnosis of Noncaseating
Granulomas
• TB
• Fungal infections
• Lymphoma
• Epithelioid tumors of the breast
• Lung cancer
33. Treatment
• Observation
• Initiating corticosteroid therapy when
appropriate
• Monitoring response to therapy
• Discontinuing corticosteroids when clinically or
physiologically indicated.
34. Treatment
• Topical therapy for cutaneous or ophthalmic
disease.
• Systemic corticosteroids for patients with
unresponsive ophthalmic manifestations,
cardiac, neurologic and progressive pulmonary
involvement.
• Systemic therapy for patients with
hypercalcemia.
35. Treatment
• Prednisone, 20 to 40 mg/d in divided doses or
alternate-day dosing is used for organ
involvement that is not life threatening.
• Higher dosage is used off-label for potentially life
threatening disease.
• High-dose inhaled corticosteroids may be useful
in patients with symptomatic pulmonary disease.
36. Treatment
• Clinical improvement should be assessed after 3
months of corticosteroids.
• If no improvement is found, further treatment is
unlikely to be beneficial.
• Long term adverse affects of therapy include
weight gain, mood swings, cataracts, GERD,
osteoporosis
37.
38.
39.
40. Prognosis
Many patients do not require therapy, and their
conditions will spontaneously improve.
Markers for a poor prognosis include :
• Advanced CXR stage.
• Extrapulmonary disease (predominantly cardiac and
neurologic)
• Evidence of pulmonary hypertension.
• Multiple studies have demonstrated that the
most important marker for prognosis is the initial
CXR stage.
41.
42. Remission
• 2/3 of patients with sarcoidosis generally have a
remission within a decade after diagnosis, with
few or no consequences; remission occurs for
more than half of patients within 3 years.
• Unfortunately, up to 1/3 of patients have
progressive disease, leading to clinically significant
organ impairment.
• A recurrence after 1 or more years of remission is
uncommon (affecting <5% of patients), but
recurrent disease may develop at any age and in
any organ.
43. Death
• Less than 5% of patients die from
sarcoidosis.
• death is usually the result of pulmonary
fibrosis with respiratory failure or of
cardiac or neurologic involvement.