This document summarizes primary defects of antibody production and immunodeficiency. It discusses various types of primary immunodeficiencies including disorders of humoral immunity affecting B cell differentiation and antibody production. Specific disorders covered include X-linked agammaglobulinemia, common variable immunodeficiency, selective IgA deficiency, transient hypogammaglobulinemia of infancy, immunodeficiency with hyper-IgM, and X-linked lymphoproliferative disease. Treatment options for humoral defects such as immunoglobulin administration are also summarized.
Immunological Disorders can be classified into 3 distinct categories.They are Hypersensitivity, Autoimmunity and Immunodeficiency.Here in this presentation we talk about Immunodeficiency disorders.Get more on our blog : http://dentistryandmedicine.blogspot.com/
Immunological Disorders can be classified into 3 distinct categories.They are Hypersensitivity, Autoimmunity and Immunodeficiency.Here in this presentation we talk about Immunodeficiency disorders.Get more on our blog : http://dentistryandmedicine.blogspot.com/
This presentation is an overview of primary and secondary immunodeficiency disorders with highlights on the genetic basis of primary disorders and associated factors underlying secondary disorders, as well a management of these disorders
This presentation is an overview of primary and secondary immunodeficiency disorders with highlights on the genetic basis of primary disorders and associated factors underlying secondary disorders, as well a management of these disorders
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Are you curious about what’s new in cervical cancer research or unsure what the findings mean? Join Dr. Emily Ko, a gynecologic oncologist at Penn Medicine, to learn about the latest updates from the Society of Gynecologic Oncology (SGO) 2024 Annual Meeting on Women’s Cancer. Dr. Ko will discuss what the research presented at the conference means for you and answer your questions about the new developments.
Tom Selleck Health: A Comprehensive Look at the Iconic Actor’s Wellness Journeygreendigital
Tom Selleck, an enduring figure in Hollywood. has captivated audiences for decades with his rugged charm, iconic moustache. and memorable roles in television and film. From his breakout role as Thomas Magnum in Magnum P.I. to his current portrayal of Frank Reagan in Blue Bloods. Selleck's career has spanned over 50 years. But beyond his professional achievements. fans have often been curious about Tom Selleck Health. especially as he has aged in the public eye.
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Introduction
Many have been interested in Tom Selleck health. not only because of his enduring presence on screen but also because of the challenges. and lifestyle choices he has faced and made over the years. This article delves into the various aspects of Tom Selleck health. exploring his fitness regimen, diet, mental health. and the challenges he has encountered as he ages. We'll look at how he maintains his well-being. the health issues he has faced, and his approach to ageing .
Early Life and Career
Childhood and Athletic Beginnings
Tom Selleck was born on January 29, 1945, in Detroit, Michigan, and grew up in Sherman Oaks, California. From an early age, he was involved in sports, particularly basketball. which played a significant role in his physical development. His athletic pursuits continued into college. where he attended the University of Southern California (USC) on a basketball scholarship. This early involvement in sports laid a strong foundation for his physical health and disciplined lifestyle.
Transition to Acting
Selleck's transition from an athlete to an actor came with its physical demands. His first significant role in "Magnum P.I." required him to perform various stunts and maintain a fit appearance. This role, which he played from 1980 to 1988. necessitated a rigorous fitness routine to meet the show's demands. setting the stage for his long-term commitment to health and wellness.
Fitness Regimen
Workout Routine
Tom Selleck health and fitness regimen has evolved. adapting to his changing roles and age. During his "Magnum, P.I." days. Selleck's workouts were intense and focused on building and maintaining muscle mass. His routine included weightlifting, cardiovascular exercises. and specific training for the stunts he performed on the show.
Selleck adjusted his fitness routine as he aged to suit his body's needs. Today, his workouts focus on maintaining flexibility, strength, and cardiovascular health. He incorporates low-impact exercises such as swimming, walking, and light weightlifting. This balanced approach helps him stay fit without putting undue strain on his joints and muscles.
Importance of Flexibility and Mobility
In recent years, Selleck has emphasized the importance of flexibility and mobility in his fitness regimen. Understanding the natural decline in muscle mass and joint flexibility with age. he includes stretching and yoga in his routine. These practices help prevent injuries, improve posture, and maintain mobilit
micro teaching on communication m.sc nursing.pdfAnurag Sharma
Microteaching is a unique model of practice teaching. It is a viable instrument for the. desired change in the teaching behavior or the behavior potential which, in specified types of real. classroom situations, tends to facilitate the achievement of specified types of objectives.
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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Recomendações da OMS sobre cuidados maternos e neonatais para uma experiência pós-natal positiva.
Em consonância com os ODS – Objetivos do Desenvolvimento Sustentável e a Estratégia Global para a Saúde das Mulheres, Crianças e Adolescentes, e aplicando uma abordagem baseada nos direitos humanos, os esforços de cuidados pós-natais devem expandir-se para além da cobertura e da simples sobrevivência, de modo a incluir cuidados de qualidade.
Estas diretrizes visam melhorar a qualidade dos cuidados pós-natais essenciais e de rotina prestados às mulheres e aos recém-nascidos, com o objetivo final de melhorar a saúde e o bem-estar materno e neonatal.
Uma “experiência pós-natal positiva” é um resultado importante para todas as mulheres que dão à luz e para os seus recém-nascidos, estabelecendo as bases para a melhoria da saúde e do bem-estar a curto e longo prazo. Uma experiência pós-natal positiva é definida como aquela em que as mulheres, pessoas que gestam, os recém-nascidos, os casais, os pais, os cuidadores e as famílias recebem informação consistente, garantia e apoio de profissionais de saúde motivados; e onde um sistema de saúde flexível e com recursos reconheça as necessidades das mulheres e dos bebês e respeite o seu contexto cultural.
Estas diretrizes consolidadas apresentam algumas recomendações novas e já bem fundamentadas sobre cuidados pós-natais de rotina para mulheres e neonatos que recebem cuidados no pós-parto em unidades de saúde ou na comunidade, independentemente dos recursos disponíveis.
É fornecido um conjunto abrangente de recomendações para cuidados durante o período puerperal, com ênfase nos cuidados essenciais que todas as mulheres e recém-nascidos devem receber, e com a devida atenção à qualidade dos cuidados; isto é, a entrega e a experiência do cuidado recebido. Estas diretrizes atualizam e ampliam as recomendações da OMS de 2014 sobre cuidados pós-natais da mãe e do recém-nascido e complementam as atuais diretrizes da OMS sobre a gestão de complicações pós-natais.
O estabelecimento da amamentação e o manejo das principais intercorrências é contemplada.
Recomendamos muito.
Vamos discutir essas recomendações no nosso curso de pós-graduação em Aleitamento no Instituto Ciclos.
Esta publicação só está disponível em inglês até o momento.
Prof. Marcus Renato de Carvalho
www.agostodourado.com
Explore natural remedies for syphilis treatment in Singapore. Discover alternative therapies, herbal remedies, and lifestyle changes that may complement conventional treatments. Learn about holistic approaches to managing syphilis symptoms and supporting overall health.
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
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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.
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
1. Primary Defects of Antibody
Production
PRESENTER : DR. IRA KC
MODERATOR: DR. SANTOSH GAUTAM
2. IMMUNE SYSTEM:
complex network of organs, cells and proteins that
defends the body against infection, whilst
protecting the body's own cells
Protects against pathogen
Check on auto-immunity
Cancer surveillance
IMMUNODEFICIENCY:
Inability to produce an adequate immune response
because of an insufficiency or absence of antibodies,
immune cells, or both
Increased susceptibility to infection
Increased risk of autoimmunity/rheumatologic
disorder
Increased predisposition to malignancy
6. IMMUNODEFICIENCY
Primary
Abnormalities in development of immune
mechanisms
Disorders of Humoral Immunity-affecting B
cell differentiation and antibody production
Disorders of Cellular Immunity-T cell
defects
Combined T cell and B cell defects
Phagocytic Defects
Complement Deficiencies
Secondary
Consequences of disease, drugs, nutritional
inadequacies & other processes interfere with
proper functioning of mature immune system
EG : HIV
: Nephrotic syndrome
7. December
21, 2023
Disorders of Humoral Immunity
(Antibody production)
1) X-linked Agamma - globulinemia ( Bruton’s Agamma-globulinemia, BTK deficiency, X-LAG)
2) Common Variable Immunodeficiency (CVID)
3) Selective IgA deficiency
4) Transient Hypo-gammaglobulinemia of Infancy
5) Immunodeficiency with hyper-IgM
6) X- linked lymphoproliferative disease
8. X-linked (Bruton’s) Agammaglobulinemia
1:200,000 live births,
1st immunodeficiency disease recognised. Described by Bruton (
1952 )
Genetic features: x-linked recessive inheritance, males.
Pathogenesis: abnormal gene XLA maps to q22 on the long
arm of the X chromosome, encodes the B-cell protein tyrosine
kinase Btk (Bruton tyrosine kinase).
Helps in signaling and maturation of pre B cell.
No/few B cells (pan hypogammaglobinemia), normal T cells
9. Clinical Features
Boys, remain well till first 6-9 months of life
Infections with extracellular pyogenic organisms , Mycoplasma
Chronic fungal infections are seen; Pneumocystis jiroveci pneumonia rarely occurs
Viral infections handled normally, except hepatitis viruses and enteroviruses
Paralysis after live polio vaccine
Failure to thrive
A boy with recurrent pneumonia absent tonsils/ no palpable Lymph
Nodes + positive family history
Order serum immunoglobulin level
10. Diagnosis
Serum concentrations of IgG, IgA, IgM, and IgE are far below 95% confidence
limits for appropriate age- and race-matched controls
Total Immunoglobulins :<100mg/dl
Flow cytometry: Absence of circulating B cells
Mature B cells (CD19,CD20,BCR) absent in peripheral blood
Under developed germinal centers of lymph nodes
11. Common Variable Immunodeficiency
Hypogammaglobinemia with phenotypically normal B cells
Age : 2nd -3rd decade of life
Sex: M=F
Recurrent pneumonia /sinusitis/ chronic diarrhea
Phenotypic diagnosis with a polygenic inheritance
Mutated (< 10 % case) ICOS, SH2DIA, CD19, CD20, CD21, CD81, BAFF-R, TACI
similar clinically with XLA
(except that enterovirus meningoencephalitis is rare )
Association with other autoimmune disease : high
14. Transient Hypogammaglobulinemia of Infancy
Represents developmental delay in the production of immunoglobulin
Most infants begin to produce IgG in the 1st 3 months, increases throughout infancy
Small number either begin late or do not increase their production as expected
Characterized by
Frequent sinopulmonary infections
Low serum immunoglobulins
Condition may last into pre-school years
Diagnosis can only be made definitively in retrospect
key distinction : responses to vaccines preserved.
Source of Diagnostic confusion
15. 15
Care and Treatment of the Child with Suspected
Transient Hypogammaglobinemia
Serial monitoring of immunoglobulins and antibody titers
Re-immunization may be necessary
Awareness that infections may require longer courses of antibiotics for treatment than
usually prescribed
Gamma globulin is seldom necessary
Any fever of 101.5F or greater requires that the child have a CBC, blood culture and
physical exam
Live viral vaccines should be held until immuno-competency is demonstrated
16. Selective IgA Deficiency
Most Common in this group
incidence 0.33% in normal populations.
Immunological features:
Serum IgA<5mg/dl but normal IgM and IgG
Genetic features : IgA Deficiency and genetic
factors: associated with HLA-A2, B8 and DW3 or A1 and B8
Pathogenesis: failure in terminal differentiation of B cells due to intrinsic B cell defect or abnormal T
cell help (TGF-B,IL-5) or in B cell responses to these cytokines
17. Selective IgA Deficiency
Clinical Features:
Majority are asymptomatic
Anaphylactic transfusion reactions
Atopic disorders
Recurrent infections in respiratory tract, Gastrointestinal tract, urogenital tract.
Diagnosis cannot be made until about 4 years of age, when IgA levels should be matured to adult levels
Treatment:
Washed erythrocytes (frozen blood would have this done routinely) or blood products from other IgA-
deficient individuals should be administered to patients with IgA deficiency.
IVIG not recommended
Not very severe
life threatening
18. Hyper IgM syndrome
Genetically heterogeneous
Characterized by Normal/Elevated serum IgM levels associated with Low/Absent IgG, IgA, and IgE
serum levels defect in the class switch recombination (CSR) process
Causative mutations identified in the CD40 ligand gene ( X chromosome) and 3 genes on autosomal
chromosomes: AID(Activation induced cytidine deaminase), UNG(Uracil DNA glycosylase) and
CD40 gene on chromosome 20.
All patients should undergo molecular analysis to ascertain the affected gene for purposes of genetic
counseling, carrier detection, and decisions regarding definitive therapy.
19.
20.
21. X-linked Autosomal Recessive
Gene mutation CD40 ligand gene AID, UNG, CD40
Lymph node Abortive germinal centers with with
depletion of follicular dendritic cells
cells
Giant germinal centers filled with
highly proliferating B cells
Susceptibility to P.jiroveci
pneumonia
marked No
Treatment HLA identical Hematopoietic Stem
Cell Transplantation, Granulocyte-
CSF
IVIg infusion, Antibiotics
22. X-linked Lymphoproliferative Disease
Also known as Duncan Disease
Two types namely ; XLP Type I AND XLP Type II
XLP Type I:
mutation in SH2D1A gene (encoding SAP)
Can lead to uncontrolled cytotoxic T-cell response to EBV
XLP Type II:
mutation in XIAP gene
23. Clinical Presentation
Fulminant Infectious Mononucleosis:
Excessive proliferation of EBV-infected B cells, cytotoxic T cells and macrophage in various tissues
including hematopoietic cells, brain, liver and heart massive infiltration of these cells into lymphoid
and other organs extensive parenchymal damage including hepatic necrosis and bone marrow failure
Thrombocytopenia, Anemia, hepatic dysfunction, meningoencephalitis, fulminant hepatitis
Dysgammaglobulinemia:
Decreased levels of IgG1 and IgG3 with elevated levels of IgM and IgA progress over time to include
deficiencies of all isotypes of Immunoglobulins
Lymphoma:
Due to impaired immune surveillance and elimination by T cells
24. Mean year of presentation: <5 yrs
Affected males are usually healthy until they acquire EBV infection
Asymptomatic until onset of complications
Unfavorable Prognosis
25. Treatment of humoral defects
Immunoglobulin administration (IVIG or SCIG)
at a dose of 400 mg/kg per month
EXCEPT FOR THE CD40 LIGAND DEFECT AND XLP stem cell transplantation
Judicious use of antibiotics to treat documented infections
26. Subcutaneous Immunoglobulin Replacement Therapy in Patients with Primary
Immunodeficiency in Routine Clinical Practice: The VISPO Prospective Multicenter Study
Alessandra Vultaggio, Chiara Azzari, Cinzia Milito, Andrea Finocchi, Claudia Toppino, Giuseppe Spadaro, Antonino Trizzino, Martire Baldassarr
Aim
To evaluate the shifting from intravenous immunoglobulins (IVIGs) replacement therapy to SCIG in patients with
primary immunodeficiency (PID) in a routine real-life situation.
Methods
In a multicenter prospective observational study, we enrolled 50 patients suffering from PID who were monitored for
24 months; 44 patients switched from IVIG and six from different SCIG preparations. The study preparation (human
IgG 16 %, Vivaglobin®, CSL Behring GmbH, Germany) was subcutaneously infused weekly (maximum volume
15 mL/site; maximum infusion rate 22 mL/h). The study endpoints were: annual rate of severe bacterial infections
(SBIs), local adverse reactions, quality of life, days off school/work, and days of hospitalization
Results
Thirty-three of 39 (84.6 %) patients who completed the study experienced an infection or signs thereof. Only five
SBIs were observed, corresponding to an annual rate of 0.056 episodes per patient in 44 subjects [intention-to-treat
(ITT) population]. A significant decrease in both days of hospitalization (1.93 ± 4.08 vs. 0.64 ± 2.94) and days off
school/work (15.27 ± 23.17 vs. 2.26 ± 4.45) was recorded at 24 months.
27. Local reactions were observed in 14/50 (28 %) patients, mainly consisting of skin manifestations at the injection site.
Only three (6.8 %) patients discontinued due to infusion site reactions. In patients shifting from IVIG to SCIG, the
total mean score of Life Quality Index (LQI) improved from 76.9 ± 16.8 to 90.7 ± 11.6 (P < 0.01) at 6 months; there
was an improvement also in the overall patients’ evaluation.
Conclusions
A total of 93.2 % patients tolerated the new route of administration and reported a significant improvement in their
LQI. Our results from a routine clinical practice in a real-life population are consistent with those of phase III
clinical studies.
