A presentation made about Sickle cell disease by Yara Mostafa, Yasser Osama, Yaser Mostafa ,Ain shams university, Medicine faculty, first year students.
A presentation made about Sickle cell disease by Yara Mostafa, Yasser Osama, Yaser Mostafa ,Ain shams university, Medicine faculty, first year students.
Sickle cell anemia is a genetic diseases where red blood cells can take shape of a crescent or a sickle . And this allows them to be more easily destroyed – causing anemia and other complexities
This a series of notes on hematology useful for undergraduate and postgraduate medical and paramedical students. Notes are prepared from standard texts and are easy to reproduce in exams.
Megaloblastic Anaemia is an example of macrocytic anaemia. The impaired DNA synthesis due to lack of vitamin B 12 and folic acid.This presentation to learn about aetiology, causes, clinical features, lab diagnosis and treatment of Megaloblastic Anaemia
UAEU - CMHS - Hematology-Oncology Course - MMH 302 - HONC 320. Education material for medical students - It cover basic principles of hematology and oncology, including CAR-T and gene editing. It can be used for study and review. It illustrates main principles of hematology and oncology.
Sickle cell anemia is a genetic diseases where red blood cells can take shape of a crescent or a sickle . And this allows them to be more easily destroyed – causing anemia and other complexities
This a series of notes on hematology useful for undergraduate and postgraduate medical and paramedical students. Notes are prepared from standard texts and are easy to reproduce in exams.
Megaloblastic Anaemia is an example of macrocytic anaemia. The impaired DNA synthesis due to lack of vitamin B 12 and folic acid.This presentation to learn about aetiology, causes, clinical features, lab diagnosis and treatment of Megaloblastic Anaemia
UAEU - CMHS - Hematology-Oncology Course - MMH 302 - HONC 320. Education material for medical students - It cover basic principles of hematology and oncology, including CAR-T and gene editing. It can be used for study and review. It illustrates main principles of hematology and oncology.
This is slide regarding Thalassemia, prepared for Paediatric Posting. Introduction, clinical features and management of the condition (Paediatric Protocol of Malaysia).
Title: Understanding Anemia: Causes, Types, Clinical Features, and Diagnostic Investigations
Anemia is a condition characterized by a deficiency in the number or quality of red blood cells (RBCs) or hemoglobin in the blood, leading to reduced oxygen-carrying capacity. It is a prevalent global health issue affecting people of all ages, genders, and socioeconomic backgrounds. Understanding the causes, types, clinical features, and diagnostic investigations of anemia is crucial for effective management and treatment.
**Causes of Anemia:**
Anemia can result from various factors that disrupt the production, lifespan, or function of red blood cells. Some common causes include:
1. **Iron Deficiency:** Insufficient intake or absorption of iron, essential for hemoglobin synthesis, is a primary cause of anemia globally. It can stem from poor dietary intake, chronic blood loss (e.g., menstruation, gastrointestinal bleeding), or increased demand during pregnancy.
2. **Vitamin Deficiencies:** Deficiencies in vitamins such as vitamin B12 (cobalamin) or folate (vitamin B9) can impair RBC production, leading to megaloblastic anemia.
3. **Chronic Diseases:** Conditions like chronic kidney disease, inflammatory disorders (e.g., rheumatoid arthritis), and infections can disrupt erythropoiesis (RBC production) or accelerate RBC destruction, causing anemia.
4. **Hemolytic Disorders:** Inherited or acquired conditions that increase the breakdown (hemolysis) of red blood cells, such as sickle cell disease, thalassemia, or autoimmune hemolytic anemia, can result in anemia.
5. **Bone Marrow Disorders:** Diseases affecting the bone marrow, including leukemia, myelodysplastic syndromes, and aplastic anemia, can lead to decreased RBC production and anemia.
**Types of Anemia:**
Anemia is classified based on the underlying mechanism or etiology, leading to several types:
1. **Iron-Deficiency Anemia:** Characterized by low iron levels, resulting in decreased hemoglobin synthesis and microcytic (small-sized) RBCs.
2. **Megaloblastic Anemia:** Caused by impaired DNA synthesis in RBC precursors due to deficiencies in vitamin B12 or folate, leading to macrocytic (large-sized) RBCs.
3. **Hemolytic Anemia:** Occurs due to increased RBC destruction, either intravascularly (within blood vessels) or extravascularly (outside blood vessels), leading to various subtypes like autoimmune hemolytic anemia, hereditary spherocytosis, and sickle cell disease.
4. **Anemia of Chronic Disease:** Associated with chronic inflammation, infections, or malignancies, leading to impaired iron metabolism and decreased RBC production.
5. **Aplastic Anemia:** Results from bone marrow failure, leading to decreased production of all blood cell types, including RBCs.
**Clinical Features of Anemia:**
The clinical presentation of anemia can vary depending on its severity, underlying cause, and individual factors. Common clinical features include:
Mechanical Ventilation Cheat Book for Internal Medicine ResidentsThe Medical Post
This short cheat book talks about basic concepts and physiology of artificial ventilation and also elaborates on point guided approach in maneuvering different modes of mechanical ventilation. Consider this as a basic overview and is intended for all internal medicine residents.
Salient features of the book are -
- The book provides a shortcut to understand and remember certain specific formulae and points you require to interpret the 12-lead ECG.
- Treatment protocols (in green boxes) for most of the important conditions are also included.
- View sample ECGs as you read along the topics.
- The content is explained in a very simple language to provide good conceptions, written from a student’s point of view.
- People can gain their belief in the book after going through sample ECGs which would be available at www.themedicalpost.net/ecg
- The book competes with the other books available in the market in simplicity, summaries, treatment protocols, live diagrams and regularly updated sample ECGs on the website.
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 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
Ethanol (CH3CH2OH), or beverage alcohol, is a two-carbon alcohol
that is rapidly distributed in the body and brain. Ethanol alters many
neurochemical systems and has rewarding and addictive properties. It
is the oldest recreational drug and likely contributes to more morbidity,
mortality, and public health costs than all illicit drugs combined. The
5th edition of the Diagnostic and Statistical Manual of Mental Disorders
(DSM-5) integrates alcohol abuse and alcohol dependence into a single
disorder called alcohol use disorder (AUD), with mild, moderate,
and severe subclassifications (American Psychiatric Association, 2013).
In the DSM-5, all types of substance abuse and dependence have been
combined into a single substance use disorder (SUD) on a continuum
from mild to severe. A diagnosis of AUD requires that at least two of
the 11 DSM-5 behaviors be present within a 12-month period (mild
AUD: 2–3 criteria; moderate AUD: 4–5 criteria; severe AUD: 6–11 criteria).
The four main behavioral effects of AUD are impaired control over
drinking, negative social consequences, risky use, and altered physiological
effects (tolerance, withdrawal). This chapter presents an overview
of the prevalence and harmful consequences of AUD in the U.S.,
the systemic nature of the disease, neurocircuitry and stages of AUD,
comorbidities, fetal alcohol spectrum disorders, genetic risk factors, and
pharmacotherapies for AUD.
- Video recording of this lecture in English language: https://youtu.be/lK81BzxMqdo
- Video recording of this lecture in Arabic language: https://youtu.be/Ve4P0COk9OI
- Link to download the book free: https://nephrotube.blogspot.com/p/nephrotube-nephrology-books.html
- Link to NephroTube website: www.NephroTube.com
- Link to NephroTube social media accounts: https://nephrotube.blogspot.com/p/join-nephrotube-on-social-media.html
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.
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
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.
HOT NEW PRODUCT! BIG SALES FAST SHIPPING NOW FROM CHINA!! EU KU DB BK substit...GL Anaacs
Contact us if you are interested:
Email / Skype : kefaya1771@gmail.com
Threema: PXHY5PDH
New BATCH Ku !!! MUCH IN DEMAND FAST SALE EVERY BATCH HAPPY GOOD EFFECT BIG BATCH !
Contact me on Threema or skype to start big business!!
Hot-sale products:
NEW HOT EUTYLONE WHITE CRYSTAL!!
5cl-adba precursor (semi finished )
5cl-adba raw materials
ADBB precursor (semi finished )
ADBB raw materials
APVP powder
5fadb/4f-adb
Jwh018 / Jwh210
Eutylone crystal
Protonitazene (hydrochloride) CAS: 119276-01-6
Flubrotizolam CAS: 57801-95-3
Metonitazene CAS: 14680-51-4
Payment terms: Western Union,MoneyGram,Bitcoin or USDT.
Deliver Time: Usually 7-15days
Shipping method: FedEx, TNT, DHL,UPS etc.Our deliveries are 100% safe, fast, reliable and discreet.
Samples will be sent for your evaluation!If you are interested in, please contact me, let's talk details.
We specializes in exporting high quality Research chemical, medical intermediate, Pharmaceutical chemicals and so on. Products are exported to USA, Canada, France, Korea, Japan,Russia, Southeast Asia and other countries.
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
1. Sickle cell anemia
Dr. Kalpana Malla
MD Pediatrics
Manipal Teaching Hospital
Download more documents and slide shows on The Medical Post [ www.themedicalpost.net ]
2. Introduction:
• Abnormal haemoglobin (HbS) which has a
propensity for sickling and rapid haemolysis
under adverse conditions
• Valine, a hydrophobic amino acid replaces
glutamic, a hydrophilic amino acid at the sixth
amino acid position in the beta polypeptide
chain.
4. EPIDEMIOLOGY
• Endemic malarial areas
• Central India, Sub-Saharan Africa,
Mediterranean, North Africa, Middle East
• South America, Carribean, US: African
descent…slave trade
• Incidence 1:625 in African Americans
5.
6. The Proteins of Hemoglobin A
• There are 4 protein subunits of Hemoglobin A
Hemoglobin
A
Alpha Alpha Beta Beta
There will be different forms of Hemoglobin when there is a
mutation in the beta subunit.
7. A carrier for Sickle Cell Will
have…
•An S mutation
in one copy of Hemoglobin
the hemoglobin
beta gene.
•Half of the beta Alpha Alpha
Beta Beta S
subunits are
replaced with
Beta S.
•This person has
the Sickle Cell
trait.
8. When a person is a carrier, they will have:
25 % normal HbA 50 % HbS
25 % HbSS
9. Sickle Cell Disease:
•This results
HbSS
when both
copies of the Hemoglobin
hemoglobin beta
gene have an S
mutation. Alpha
Alpha Beta S Beta S
•All of this
person’s beta
subunits are
replaced by beta
S.
10. 1. It is Unknown why people with the Sickle Cell Trait are
RELATION TO MALARIA
resistant to Malaria, but there are several theories…
2. The carriers of Sickle Cell have some abnormal
Hemoglobin, and when they come in contact with the
Malaria parasite they become sickled. Then those cells
go through the spleen, which eliminates the cells
because of their sickle shape, so the Malaria would be
eliminated as well.
3. The Sickle Cell trait causes the malaria to stay in the
body for an extended period of time, so it is able to
build up a defense to it.
11. 3. Because oxygen concentration is low in the
spleen, and because infected cells often get
trapped in the spleen, it is possible that they
are destroyed in the spleen
• 4. The Malaria parasite produces an acid when
it is inside of the red blood cells. This causes
the red blood cells to polymerize, and the cells
will sickle. These sickled cells are then
destroyed when the blood cells go through the
spleen.
12. RELATION TO MALARIA
Sickle cell “trait” (heterozygote Hb AS) have:
• 1. lower levels of Pl. falciparum parasitemia
• 2. higher hemoglobin counts
• 3. less severe reinfections than normal ppl
Why?
• 1. confounding factors in these areas that confer immunity/
resistance:
Duffy chemokine factor
G6PD
HLA-B53, HLA-DRB1
• 2. actual mechanism not known
13. TYPES
• Hb SS (Homozygous S)- Sickle Cell Anemia
• Hb AS (Heterozygous S) - Sickle Cell Trait
• Sickle β0 Thalassemia (functions exactly like
Sickle Cell Anemia)
• Sickle β+ Thalassemia
• Hb SC ( mutation for Hb C occurs same site as
HbS. Instead of valine, lysine is exchanged for
glutamine)
14. SEVERITY OF DISEASE
Hb SS ≥ Sickle β 0 Thal >>
Hb SC >> Sickle β + Thal>> HbAS
15. PATHOPHYSIOLOGY
• Single base pair exchange
• Thymine for adenine in 6th
codon of β gene
• Encodes Valine instead of
Glutamine
• Charge at that site altered
• Allows polymerization of Hb
under hypoxia and acidosis
• Inc 2,3-DPG and dec pH
increases O2 affinity ->
hypoxia
16. PATHOPHYSIOLOGY (CONTD…)
• Amount of polymerisation is dependant upon the other Hb (extent of
homology with HbS)
• Copolymerization in this desc.order (S, C, D, O...so on and so forth)
• Polymerization -> RBC membrane alters-> sickling -> incr.adhesiveness ->
capillaries blocked -> local anoxia -> infarction of organs
• Infarction of liver, kidney, spleen, brain, bones, joints
• Thrombotic coagulopathy
• If resp infections -> hypoxia worsens -> more sickling
• Sickle cells seq in spleen. So splenomegaly
• Paradoxically spleen undergoes rptd infarction -> autosplenectomy
• Long standing hemolysis -> biliary pigment stones
• Long standidng hypoxia -> clubbing, leg ulcers, growth retardation
• Long standing anemia -> compensatory hyperactivity of BM
17.
18.
19. CLINICAL FEATURES
• Newborns: do not manifest as there is high HbF
• By 5-6mo: many have functional asplenia.
Hence bacterial sepsis
• By 5yr: 95 % functional asplenia
• Universally anemia and jaundice
• Thereafter CRISES EPISODES
21. HAND FOOT SYNDROME
• Acute sickle dactylitis
• Painful, symmetric swelling of hand and feet
• Ischemic necrosis of small bones
• Blood supply choked off due to rapidly
expanding bone marrow
• Xray: extensive bone destr. and repair
22. ACUTE PAINFUL CRISIS
• Young children: mostly extremities
• Older patients: abdomen, back, chest
• Assoc with intercurrent illness
• Vaso occlusive event
• Abdominal organ infarctions
24. ACUTE CHEST SYNDROME
• Pulmonary infarction
• Associated with pneumonia or microscopic fat
emboli from BM infarction
• Acute chest pain
• Hemopytsis
• Cyanosis
• May lead to death
25. ACUTE SPLENIC SEQUESTRATION
• In infants and young children (6mo-3yrs)
• Distinct and episodic event
• Foll acute febrile illness
• For unknown reasons large amounts of blood
become acutely pooled in spleen
• Massive enlargement of spleen
• Circulatory collapse
26. STROKE
• Catastrophic event
• >5yrs
• Hemiplegia
• 10% of patients affected
• 25-30% have occult strokes-
school performance affected
• Trancranial doppler studies
27. OTHER MANIFESTATIONS
RENAL
Progressive deterioration
Glomerular and tubular fibrosis
Polyuria >5yrs
Renal papillary necrosis -> hematuria
Nephrotic syndrome
PRIAPISM
Pooling of blood into corpora cavernosa obstructing
venous bloodflow
30. INVESTIGATIONS
• Mild to moderate anemia (5-9gm/dl)
• Inc. retics
• Inc. TLC with sometimes Inc neutrophils
• Normal to inc. platelets
• Normal MCV (unless thalassemic Hb)
• PS: sickle cells, nucl RBCS, target cells, HJ bodies
• BM: hyperplastic marrow with erythroid predominance
• Xray:Osteoporosis, sclerosis of long bones
• Renal conc capacity decreased
• Hb electrophoresis
• Genetic / mutational analysis
• Prenatal diagnosis
• Transcranial doppler (TCD). If blood velocity >200cm/sec in brain, then
transfuse to keep Hb S <30%
32. SICKLE THALASSEMIA
• Presentation is same as Hb SS (SCA) if Sickle β0
Thalassemia
• If Sickle β+ Thalassemia, then C/F are blunted
• Doubt if Hb electrophoresis shows HbSS
• But MCV <78fl or microcytosis in PS
33. SICKLE CELL TRAIT
• Assoc with α thalassemia
• Life span normal
• Complications rare
• Sudden death due to rigorous exercise
• Splenic infarcts at high altitude
• Hematuria
• Hyphema with IO bleed and blindness
• Renal medullary Ca predisposition
34. MANAGEMENT OF SCD
• General
• Prophylaxis
• Febrile episodes
• Analgesia
• Blood transfusion
• Drugs
• Bone marrow transplant
• Surgeries – splenectomy, cholecystectomy
35. GENERAL MANAGEMENT
• Vaccines
• Penicillin V prophylaxis
• Caretakers taught : temp, assess
illness, spleen, anemia, stroke…and bring to
hosp urgently
• Folic acid supplementation due to high red cell
turnover
36. MANAGEMENT OF FEBRILE EPISODES
• Hospitalise. Not OPD management <3yrs
• Look for occult bacteremia
• CBC, Blood culture, Urine culture, CXR
• Monitor for Acute splenic sequestration crisis
• Empirical Ceftriaxone (75-100mg/kg)
37. MANAGEMENT OF PAIN
• Paracetamol intially
• Later if relenteless pain : limited opiates –
codeine
• Wean off opiates in 3-4days
• Avoid masking of disease progression with
opiates
• >7-10 yr PCA (patient controlled analgesia)
pump
• Look for cause of pain
38. BLOOD TRANSFUSION
• Indications:
acute chest syndrome
stroke
severe anemia
acute splenic sequestration
• BT doesn’t reverse ischemic damage
• Can continue to have CVD and AVN
• Preferably give leuco-poor packed cell
39. DRUG THERAPY
Hydroxyurea : 15-30 mg/kg/day
Increases Hb F
BONE MARROW TRANSPLANT
<16 yr with HLA matched sibling
Only cure
40. Thank you
Download more documents and slide shows on The
Medical Post [ www.themedicalpost.net ]
![Sickle cell anemia
Dr. Kalpana Malla
MD Pediatrics
Manipal Teaching Hospital
Download more documents and slide shows on The Medical Post [ www.themedicalpost.net ]](https://image.slidesharecdn.com/hematology-sicklecellanemia-120108093300-phpapp02/85/Siickle-cell-anemia-1-320.jpg)
