Growth Flashcards

Question

What is the cytology of malignant tumours?

Answer 1

``` variation in cell shape and size • variation in nuclear shape and size • odd mitoses • altered differentiation ```

Answer 2

Benign ...

Answer 3

``` melanoma: melanocytes malignant • lymphoma • leukaemia bone marrow precursors • teratoma germ cell tumours most in testes, most malignant (ovarian tend to be benign) can mimic ANY tissue ```

Answer 4

• benign tumours little known, many be inherited factors genetic susceptibility to cancer • inherited cancer syndromes • single mutant genes, often tumour suppressor genes • retinoblastoma, some colon cancers.

Answer 5

Invasion - unconfined growth into CT

Answer 6

Surface apposition (bone added on surface by osteoblasts) and resorption (Bone being removed by osteoclasts)

Answer 7

Sutures in membranous bones are growth centres and expression of growth at these sites changes shape

Answer 8

Cartilages are primary factor in craniofacial growth - growing cartilage replaced by bone Suggested that this growth is genetically determined Examples - spheno-occipital and spheno-ethmoidal synchondroses, nasal septum, condyle

Answer 9

Bone growth takes place in response to growth of surrounding tissues. Craniofacial growth as a response to functional needs that is mediated by adjacent soft tissue growth 2 matrices: Periosteal matrix Capsular matrix Function of jaw changed to influence growth - orthodontics

Answer 10

Growth of the brain

Answer 11

Synchondroses

Answer 12

Surface and sutural deposition

Answer 13

Cartilage replacement in the secondary growth cartilage at the condylar head

Answer 14

Resorption from the anterior surface and deposition on the posterior surface

Answer 15

Mandibular canal Retromoalr region Mandibular symphysis

Answer 16

Matrix rotation - around the condyle | Intra-matrix rotation - within body of mandible

Answer 17

Gets longer with time, matrix rotation centred on the condyle

Answer 18

Mandible growing downwards and backwards

Answer 19

Becomes shorter with time

Answer 20

Increases in lower and total face height, narrow upper arches, retroclined incisors, difficulties in nasal breathing etc

Answer 21

Phase 1 ..

Answer 22

``` Measurement of change in height. Secondary sexual characteristics Hand-wrist radiographs Radiographic assessment of cervical spine maturation Average growth increment s ```

Answer 23

Shows growth patterns of most systems of the body including the skeleton and muscle mass - height

Answer 24

Graph of the growth of the brain, nervous system and associate structures

Answer 25

Ulnar sesamoid bone ossified at the start of pubertal growth spurt - looking for this bone on hand-wrist radiograph - comapred with the reference atlas

Answer 26

x ray of the craniofacial area Assesses growth spurt in body height and mandibular size 3 cervical vertebrae examined on radiograph

Answer 27

Rapid foetal growth In infancy deceleration of the foetal growth rate nutrition is determinant

Answer 28

Slow deceleration except mid childhood adrenal spurt Growth hormone is determinant

Answer 29

Pubertal growth spurt sex steroids and growth hormone is determinant

Answer 30

- Growth charts - height/weight - Bmi - Bone age

Answer 31

Mass (kg)/ height (m))^2

Answer 32

Uses standardised x-rays to measure the maturity of each epiphyseal centre of the left hand wrist to derive a score the age at which the score is on the 50th centile is the bone age of the individual

Answer 33

Tanner and Whitehouse - quantify how much growth has occurred and how much is to come

Answer 34

under nutrition, malabsorption - intestinal infection, CF, Crohns, Coeliac individual and familial short stature damage by alcohol, drugs, genetic e.g. downs systemic disease - heart, lung, renal, haematological, diabetes mellitus, endocrine - hypothyroid/hypopituitary iatrogenic - steroid excess inherited - acondroplasia, hypophosphatasia, Noonan's syndrome

Answer 35

Drugs/Chemicals - Thalidomide, Epanutin, Warfarin, Alcohol, Fluoride (teeth) ``` Radiation Infection Metabolic defects Hyperthermia Vascular Amniotic bands ```

Answer 36

Associations Sequences Field complexes Syndromes

Answer 37

Single system defect e.g. abnormal failed completion of embryonic process

Answer 38

Combinations of anomalies which are associated | statistically but underling mechanism not clear

Answer 39

``` V Verterbral anomalies A Anal atresia T Tracheo – E Esophageal atresia R Radial and Renal anomalies ```

Answer 40

Abscence or abnormal narrowing of a normal opening or passage in the body?

Answer 41

atypical physical appearance of a baby due to oligohydramnios experienced when in the uterus. It includes clubbed feet, bowed limbs, joint contractures, squashed faces. pulmonary hypoplasia and cranial anomalies related to the oligohydramnios (deficiency of amniotic fluid).

Answer 42

a set of abnormalities affecting the head and face, consisting of a small lower jaw (micrognathia), failed palatal closure, a tongue that is placed further back than normal (glossoptosis), and blockage (obstruction) of the airways

Answer 43

a series of ordered consequences due to a single cause. It differs from a syndrome in that seriality is more predictable: if A causes B, and B causes C, and C causes D, then D would not be seen if C is not seen

Answer 44

``` Breathing difficulties Feeding difficulties Cleft Palate Speech therapy Dental care Possible learning disability ```

Answer 45

Insult to a localised part of an embryo resulting in abnormalities in adjacent structures of disparate embryonic origin

Answer 46

Eg Stapedial artery interruption in rodents hemifacial microsomia

Answer 47

Trisomy 21 - Downs'

Answer 48

``` Learning delay Cardiac Defects (70%) Relatively short neck, unstable atlanto-axial joint Short stature Increased risk of Leukaemia Hypotonia Hypothyroidism Epilepsy, Alzheimers Large, fissured tongue Periodontal disease Hypodontia Microdontia Enamel Defects Class 3 malocclusion due to maxillary retrognathia ```

Answer 49

``` - deletion of genes chromosome 7 Fault in calcium metabolism leading to xs calcium Failure to thrive in infancy Highly verbal and overly sociable Characteristic facial appearance “Elfin” Short stature Delayed growth Variable learning delay & behavioural problems Heart defects Susceptible to loud noise Renal calculi ```

Answer 50

``` Osteogenesis imperfecta Down’s syndrome X-linked Vitamin D resistant rickets Cleidocranial dysostosis Achondroplasia Gigantism Acromegaly ```

Answer 51

Dentinogenesis imperfecta | amelogenesis imperfecta

Answer 52

Osteogenesis imperfecta

Answer 53

1) Mutation in Type I collagen. Associated with OI, Chromosome 7 and 17 2) Mutation in Dentine Sialophosphoprotein I Gene, Chromosome 4 3) Brandywine Isolate, Maryland Chromosome 4 DSPP mutation affecting non-collagenous proteins (DSP, DPP, DGP); variation in severity of presentation

Answer 54

dentin dysplasia

Answer 55

Amber, grey to purplish discolouration Pulpal obliteration relatively bulbous crowns, short narrow roots enamel may be lost following tooth eruption, exposing soft dentine which wears rapidly normal mantle dentine affects primary> permanent

Answer 56

``` Bone fragility Multiple fractures Unstable vertebral column Blue sclera Progressive hearing loss Bisphosphonates- risk of osteonecrosis Dentinal changes ```

Answer 57

``` Aka Hypophosphatemic rickets Rachitic changes in long bones Failure of distal tubular phosphate reabsorption Short stature Bowing of legs ↓Serum phosphate, ↑Alkaline phosphatase Large pulp chambers and elongated pulp horns Abscesses in the absence of caries ```

Answer 58

defect in FGFR3 gene Sporadic mutation in 75% and AD

Answer 59

Shortened arms and legs. Upper arms & thighs > than forearms and lower legs Large head size, frontal bossing Flattened nasal bridge Crowding due to small jaws especially maxilla

Answer 60

``` Defective development of intramembraneous ossification Short in stature Delayed closure of sutures Absent clavicles - can touch each shoulder together Frontal & parietal bossing Hypoplastic maxilla & zygoma Multiple unerupted teeth Multiple supernumeraries ```

Answer 61

Overproduction of pituitary growth hormone | Usually due to adenoma - a benign tumour formed from glandular structures in epithelial tissue

Answer 62

results in gigantism of whole skeleton after - results in acromegaly

Answer 63

Acromegaly

Answer 64

``` Continued growth at the mandibular condyle Gross prognathism Macroglossia Spacing of the dentition Thickening of the facial soft tissues Overgrowth of hands and feet ```

Answer 65

section of a chromosome that is an imperfect copy of a functional gene

Answer 66

used to produce noncoding RNA components such as transfer RNA, regulatory RNA and ribosomal RNA. Landing spots for proteins that influence gene activity Strands of RNA with myriad roles Places where chemical modifications silence stretches of chromosome

Answer 67

Short sequence of bases upstream of the start of each gene where RNA polymerase recognises and binds to

Answer 68

Polymerase I - nucleolar region of nucleus, transcribes large ribosomal RNA Polymerase II - mRNA precursors Polymerase III - small RNAs (tRNA), 5S ribosomal RNA and other small DNA sequences

Answer 69

TFIID is the first protein to bind to DNA during the formation of the pre-initiation transcription complex of RNA polymerase II (RNA Pol II). Binding of TFIID to the TATA box in the promoter region of the gene initiates the recruitment of other factors required for RNA Pol II to begin transcription

Answer 70

25 – 35 bases upstream | of initiation site

Answer 71

basal promoter

Answer 72

DNA sequences which can control efficiency and rate of transcription. Regulate expression of genes in specific cell type and control timing of gene expression

Answer 73

they are on the same molecule of DNA as the gene they regulate

Answer 74

Tumour promoting viruses transform healthy cells by inserting strong promoters in vicinity of growth-stimulating genes, while translocations in some cancer cells place genes that should be “turned off” in the proximity of strong promoters and enhancers

Answer 75

Looping of the DNA due to interactions between proteins bound to the enhancer and those bound to the promoter these proteins are called activators and the proteins that inhibit the looping are called repressors

Answer 76

binding site sequences for transcription factors (TF) and enhance/upregulate transcription. Active enhancers are bound by activating TF and brought into proximity of target promoters by looping.

Answer 77

bind to promoter and enhancer sequences and recruit RNA polymerase. Basal transcription factors are required at every promoter site for RNA polymerase interaction (TFIID).

Answer 78

they are encoded by a different gene to that they are regulating

Answer 79

the sugar-phosphate bond between 3’-OH of ribose and 5’ PO4

Answer 80

The ability to fold into complex three-dimensional shapes

Answer 81

Termination of pol I genes by a termination factor = hair pin loop

Answer 82

The sequence AAUAAA is found near the polyadenylation site of eucaryotic mRNAs. This sequence is required for accurate and efficient cleavage (needed for release of polymerase from DNA template) and polyadenylation of pre-mRNAs in vivo

Answer 83

1. CAP on 5’ end - to stabilize the pre-mRNA, essential for transport of RNA out of nucleus 2. Alternative splicing 3. Poly(A) tail on 3’ end - cleavage at AAUAAA (stop codon) by endonuclease then multiple adenosine added, up to 250. This step needed for release of polymerase from DNA template

Answer 84

Protects from degradation Serves as assembly point for proteins needed to recruit small subunit of ribosome to begin translation

Answer 85

A mechanism by which different forms of mature mRNAs (so different proteins) (messengers RNAs) are generated from the same gene - different splicing sites are selected - splicesomes - plasticity allows for diseae development

Answer 86

a segment of a DNA or RNA molecule containing information coding for a protein or peptide sequence.

Answer 87

Major – removes 99.5% of introns | Minor – removes remaining 0.5%

Answer 88

1. Recognition of intron/exon boundaries 2. Catalysis of cut and paste reactions which remove non-coding introns and stitch flanking exons back together spliceosome contains both proteins and RNA's

Answer 89

Mis-splicing = rapid degeneration of mRNA | Mis-regulation of splicing factor levels = cancer

Answer 90

1. mRNA 2. rRNA 3. tRNA 4. Non-coding RNA (ncRNA) 5. Small nuclear RNA (snRNA) 6. small nucleolar RNA (snoRNA) 7. micro RNA (miRNA)

Answer 91

Build ribosomes

Answer 92

18S - one of these along with other proteins make the small subunit of the ribosome. 28S, 5.8S and 5S - one each of these, along with 45 other proteins used to make the large subunit of the ribosome. S = (Svedberg unit) sedimentation rate, related to mass and shape

Answer 93

Each kind carries, at 3’ end, one of 20 amino acids | i.e. most amino acids have more than one tRNA

Answer 94

RNA biogenesis and processing several are part of spliceosomes

Answer 95

Participate in making ribosomes by helping to cut large precursor of 28S, 18S and 5.8S. Modify many nucleotides in rRNA, tRNA and snRNA e.g. can add methyl groups to ribose Implicated in alternative splicing of pre-mRNA Template for synthesis of telomeres

Answer 96

Tiny RNA molecules regulate gene function post-transcriptionally - estimated more than one third of protein 18-25 nucelotides long Bind to mRNA and cause degradation – inhibits protein synthesis

Answer 97

variable number of tandem repeats (VNTRs) ``` Repeating nucleotides (15 -100) 1kb to 20kb. Number of repeats and loci are inherited from your parents. Large variation of number of these regions between individuals ``` repeat regions bounded by specific restriction enzyme sites - restriction enzymes can cut the DNA at specific sequences - cuts the VNTR's out

Answer 98

RNA is used to make cDNA from mRNA, then cDNA is multiplied by PCR , DNA can then be detected

Answer 99

``` template - DNA/cDNA nucelotides - A,C, G, T primers Taq buffer ```

Answer 100

1. - denaturation to break the cDNA double bonds - 94 degrees 2. annealing - temperature lowered to 54 degrees to allow the primers to bond to the DNA template (45 seconds) 3. Extending - when the temperature is raised and the new strand of DNA is made by the Taq polymerase enzyme. (2 mins 72 degrees)

Answer 101

Different amounts of DNA in starting sample and if measurements taken at plateau the data would not truly represent the starting material. Exponential phase is optimal point for analyzing data.

Answer 102

Any double stranded DNA

Answer 103

Traditional measure at end-point and real-time collects data in exponential growth phase Increase in reporter fluorescence directly proportional to number of amplicons generated Increased dynamic range of detection No post-PCR processing Detection down to 2-fold change

Answer 104

localization technique for the detection of a gene product (RNA) in tissues

Answer 105

The transcriptome is the set of all RNA molecules in one cell or a population of cells.

Answer 106

– large scale study of proteins, particularly their structures and functions, includes post-translational modifications

Answer 107

comprehensive characterization of small molecule metabolites in biological systems

Answer 108

DNA sequence variations when single nucleotide in genome sequence is altered Many have no effect on cell function, others predispose to disease, influence response to drugs or environmental insults (bacteria, viruses, toxins, chemicals).

Answer 109

An increase in a specific genotype of IL-1 gene

Answer 110

IL-1RA - interleukin-1 receptor antagonist is similar in structure to IL-1α and IL-1β and so competes with these for the surface receptor. It doesn’t cause ”normal” signaling ie acts as competitive inhibitor and could control the inflammatory response.

Answer 111

``` S phase - replication of DNA G2 - cell prepares to divide M phase - cell division and cytokineses G0 - cells leave cell cycle that cease division G1 - cell grows S phase ```

Answer 112

Prophase - condensation of chromatin and disappearance of nucleolus prometaphase - the nuclear membrane breaks apart into numerous "membrane vesicles", and the chromosomes inside form protein structures called kinetochores. Kinetochore microtubules emerging from the centrosomes at the poles (ends) of the spindle reach the chromosomes and attach to the kinetochores, throwing the chromosomes into agitated motion. metaphase - These chromosomes, carrying genetic information, align in the equator of the cell anaphase - replicated chromosomes are split and the daughter chromatids are moved to opposite poles of the cell. telophase -During telophase, the effects of prophase and prometaphase (the nuclear membrane and nucleolus disintegrating) are reversed. As chromosomes reach the cell poles, a nuclear envelope is re-assembled around each set of chromatids, the nucleoli reappear, and chromosomes begin to decondense back into the expanded chromatin that is present during interphase. The mitotic spindle is disassembled and remaining spindle microtubules are depolymerized

Answer 113

Extra & Intracellular signals G1 checkpoint – growth & environment G2 checkpoint – DNA replication M checkpoint – chromosome alignment on spindle

Answer 114

Proteins that control the cell cycle

Answer 115

G1 - cdk 4 and 6

Answer 116

Cyclin dependent kinases - phosphorylate cyclins to activate them activity controlled by cdk inhibitors

Answer 117

Neuron, epithelial cell, red blood cells - specialised cells

Answer 118

Active repression of genes

Answer 119

p53 (mutated in approx 50% of cancers including oral- hence tumour forms as cell cycle continues)

Answer 120

p27 - less of an aid to cyclins if reduced levels

Answer 121

Tumour suppressor genes

Answer 122

Destroys cells that may be a threat - virus infected, immune after inflammation has been dealt with, DNA damaged cells

Answer 123

Withdraw of positive signals such as growth factors, hormones or if the cell receives negative signals e.g. UV, death activators, hypoxia

Answer 124

``` Mild convulution chromatin compaction and margination condensation of cytoplasm breakup of nuclear envelope blebbing cell fragentation apoptotic bodies are phagocytosed ```

Answer 125

``` Apoptosis Controlled Energy dependent cells shrink Membrane intact Non-inflammatory No scarring Indiv. or small cell groups Nuclear fragmentation Physiological (or Pathol.) ``` ``` Necrosis Uncontrolled No ATP required Cells swell No membrane integrity Inflammatory Scarring Large cell groups Nuclear dissolution Pathological ```

Answer 126

Mitchondria senses various apoptotic stimuli releases cytochrome C - involved in electron transport chain binds to Apaf-1 protein forms complex that initiates a cascade of proteolytic enzyme reactions - carry out degradation processes = apoptosis

Answer 127

oncogene that binds to Apaf-1 protein - prevents complex formation with cytochrome C, which prevents apoptotic cells dying, this allows DNA damaged cells etc to stay alive

Answer 128

Ligand binds to membrane receptor e.g. FasL to Fas intiates cascade of phosphorylation reactions within cell transducing signal to activate enzyme cascade that carry out apoptosis

Answer 129

Family of proteolytic enzymes - affectors of apoptosis (do the digesting) - present as inactive proenzymes as don't need them all the time activated by proteolytic cleavage which activates another enzyme etc = cascade (all enzymes have different substrates and controlled by different mechanisms)

Answer 130

caspase 2, 3, 6, 7, 8, 10

Answer 131

Treacher Collin's syndrome, cleft palate/lip

Answer 132

Epstein Barr Virus protein - nasopharyngeal cancer

Answer 133

Fas antagonists

Answer 134

SLE, rheumatoid arthiritis

Answer 135

Neurodegenerative | Hiv

Answer 136

Lichen Planus

Answer 137

DNA methylation, histone modification, nucleosome location or non-coding RNA (micro RNA)

Answer 138

transcription factor – activates its own transcription so epigenetic state is self-sustaining

Answer 139

DNA methylation or | changes in histones

Answer 140

Blocks the bind of transcription factors to the promoter region

Answer 141

DNA methylation inhibitor

Answer 142

Oligodontia

Answer 143

Isolated tooth agenesis, microdontia, short tooth roots, taurodontism, sparse and slow growing hair, dry and itchy skin

Answer 144

Antagonistic Actions of Msx1 and Osr2 Pattern

Answer 145

Hypodontia with malformations of anterior chamber of the eye

Answer 146

abnormalities in forebrain cleavage and midface development

Answer 147

Amelogenin

Answer 148

Well-encapsulated, limiting undesirable spread of vector. Luminal membranes of virtually every epithelial cell in SGs are easy to access in a relatively non-invasive manner. Ductal access of SGs uses a limited fluid volume that is not diluted or disseminated following delivery, enabling use of low vector doses. Salivary epithelial cells are well differentiated and very slowly dividing, providing a relatively stable cell population for non-integrating vectors. SGs normally make large amounts of protein for export, both exocrine and endocrine A single SG is not crucial for life and can be removed in event of unexpected adverse effect with relatively little morbidity, cf liver or lung!!!

Answer 149

Predominant leading to saliva (mucosal; across apical membrane). Constitutive leading mainly towards interstitium and bloodstream (serosal; across basolateral membrane).

Answer 150

human aquaporin-1 (cDNA)

Answer 151

durable, cell-specific lipid microvesicles can migrate through the vasculature reside in a number of biofluids, eg urine, blood, breast milk, bronchial lavage fluid, CSF and saliva true functions not known but suggested could range from immune response regulators to tumour invasion promoters.

Answer 152

Breast cancer-derived exosomes could activate transcription in salivary gland cells and alters the salivary gland-derived exosomes in terms of their proteome and transcriptome This suggests that tumour-derived exosomes could function as a shuttle between distal tumour and oral cavity – resulting in discriminatory salivary biomarkers. Pancreatic tumour exosomes function in this way too.

Answer 153

increase in cell number

Answer 154

the abnormal transformation of one differentiated cell type to another differentiated cell type within the same germinal layer- not cancer

Answer 155

the presence of cells of an abnormal type within a tissue, which may signify a stage preceding the development of cancer.

Answer 156

Labile e.g. epidermis, GI tract epithelium, stable (G0)e.g. hepatocytes, permanent cell e.g. neurons, cardiac myocytes

Answer 157

mostly benign, focal malformation that resembles a neoplasm in the tissue of its origin. Tissues are normal for site but excessive e.g. moles, haemangioma - collection of small blood vessels under the skin.

Answer 158

* increase in cell numbers * response to stimulus * regression once stimulus removed * increased size and function

Answer 159

normal growth and development | puberty and pregnancy

Answer 160

parathyroids and thyroid

Answer 161

bone marrow, lymphoid tissue | • chronic irritation/inflammation

Answer 162

* increase in cell size | * often occurs with hyperplasia

Answer 163

• muscle – mechanical stimulus skeletal - exercise smooth - pregnancy cardiac – LVH in hypertension/MI/endocrine disorder

Answer 164

the failure of an organ to develop during embryonic growth and development due to the absence of primordial tissue

Answer 165

• fails to develop normal structure

Answer 166

less tissue formed

Answer 167

Enamel hypoplasia | Hypoplastic mandible and malocclusion

Answer 168

Decrease in size and number of cells usually by apoptosis, or reduction in structural components of the cell especially proteins not necrosis (mostly)

Answer 169

* nutritional – e.g. in starvation * senile * endocrine organs * bone - osteoporosis

Answer 170

* ischaemic * pressure * disuse * neuropathic/denervation * immune mediated (autoimmune) * idiopathic

Answer 171

Romberg's disease

Answer 172

changes in environmental demands e.g. smoking causes changes in from columnar to metaplastic squamous cell in bronchi

Answer 173

a displacement or malposition of an organ or other body part to another part of the body, which is then referred to as ectopic e.g. ectopic pregnancy normal tissue, abnormal site

Answer 174

• an abnormal mass of tissue • growth of which is excessive • and is unco-ordinated with that of normal tissues • and persists after the provoking stimulus is removed

Answer 175

unconfined growth into CT –

Answer 176

features of individual cells

Answer 177

* pressure e.g. in the brain * obstruction * function – esp hormone secretion

Answer 178

the loss of the mature or specialized features of a cell or tissue, as in malignant tumours. They don't resemble any particular tissue

Answer 179

the supportive tissue of an epithelial organ, tumour, gonad, etc., consisting of connective tissues and blood vessels.

Answer 180

Large number of irregularly shaped dividing cells large variably shaped nuclei small, cytoplasmic volume relative to nuclei variation in cell size and shape loss of normal specialised cell features disorganised arrangement of cells poorly defined tumour boundary odd mitoses

Answer 181

Epithelium - benign (in the lining = papilloma, or in glands = adenoma) mesenchymal/ connective tissue tumours -

Answer 182

a tumour composed of tissues not normally present at the site (the site being typically in the gonads - testes, ovaries).

Answer 183

Initiation - carcinogen causes genetic change - permanent DNA damage Promotion - cell multiplication Transformation - genetic changes to malignant cell Progression - to malignant tumour

Answer 184

Tumour suppressor genes

Answer 185

BRCA1 - 65% BRCA2- 45% onset around age 41-43

Answer 186

On basis of their tissue of origin epithelial - then benign or malignant mesenchymal - then benign or malignant

Answer 187

Wisdom tooth germs | Further studies need to identify tooth-inducible stem cells in the elderly

Answer 188

multipotent, capable of differentiating into neural cells, odontoblasts

Answer 189

Growing teeth repair/regeneration of dental tissue e.g. pulp/PDL craniomaxillofacial bone repair - DPSC and craniofacil osteoblasts both derived from neural crest cells

Answer 190

a) Cementoblast b) Adipocyte c) Odontoblast d) Neuronal cells e) Myoblast f) Chondrocyte g) Pulp cells h) Hepatocyte i) Endothelial cell j) Osteoblast k) Melanocyte not ameloblast

Answer 191

SHED improves cognitive function in model of Alzheimer's Tissue engineered sheet of DPSC to reconstruct cornea SHED muscle rengeration in muscular dystrophy DPSC enhances bone mineral density and enhanced vascular invasion similar intramembranous ossification

Answer 192

Rejection/ Immune reaction Legal & Ethical & Political, especially embryonic stem cells & human cloning Adult stem cells difficult to isolate & purify May be the cells that produce cancer

Answer 193

Cells Scaffold - ECM Signals

Answer 194

A technique for correcting defective genes responsible for disease development. Normal gene inserted into genome to replace defective one. Vectors deliver gene to patient’s target cells Vectors commonly viruses (Retro, Adeno or Adeno associated)

Answer 195

``` Encapsulated & accessible. Stable cell population which export large amounts of protein. Can be removed if there is a problem Sjogren’s syndrome / Radiation damage. Could be used for systemic conditions. ```

Answer 196

Immune response/ Virus vectors (Jesse Gelsinger 1999) Gene therapy causes cancer: leukaemia in 2 children treated for X-SCID Multi-gene disorders e.g. heart disease, most cancers not well suited to gene therapy Short lived – integration of DNA into genome, rapidly dividing cells Ethics & Regulation - germ cell therapy

Answer 197

recognise specific DNA sequences - specific genes cut DNA - restriction enzyme/nuclease insert new gene via vector

Answer 198

Clustered, Regularly Interspaced, Short Palindromic Repeat (CRISPR) technology, an important new approach for generating RNA-guided nucleases, such as Cas9

Answer 199

Genetically engineer donor immune cells to attack cancer TALEN used to add CAR19 gene which will recognise CD19 Also disabled a receptor on donor cells that the body would recognise as ‘foreign

Answer 200

Technology is in a relatively early stage and needs to be further developed. Can enough cells be edited to have therapeutic impact? Will the editing be exquisitely specific, or will other regions of the genome aside from the target be affected? ‘Designer babies’!

Answer 201

Microarray analysis – Gene chips Expression of 61,000 genes analysed simultaneously & rapidly Could use saliva ``` Identify patients with: disease (before symptoms) ↑ risk of recurrence responders to therapy customise treatment ```

Answer 202

``` • benign tumours little known, many be inherited factors • malignant tumours inherited factors environmental factors chemical agents physical agents viruses ```

Answer 203

time from promotion to clinical tumour

Answer 204

a precursor to a carcinogen. One example is nitrites when taken in by the diet. They are not carcinogenic themselves, but turn into nitrosamines in the body, which can be carcinogenic

Answer 205

a chemical that promotes the effects of a carcinogen in the production of cancere

Answer 206

tumour arises at the site of carcinogen application | • e.g. smoking and lung cancer

Answer 207

tumour arises at different site from carcinogen application • e.g. aromatic amines – industrial exposure: inhaled - lungs metabolised in liver excreted in kidney bladder enzymes release aminophenol which results in bladder carcinoma

Answer 208

Smoking - polycyclic hydrocarbons including tar diet - burnt hydrocarbons asbestos - fibrous silicates, when inhaled = fibrosis or mesothelioma ( type of cancer that develops from the thin layer of tissue that covers many of the internal organs (known as the mesothelium). The most common area affected is the lining of the lungs and chest wall.)

Answer 209

ionising radiation damages DNA and causes mutations source- x rays, radioactive metals and gases UV light damages DNA causes squamous/basal cell carcinoma and malingant melanom

Answer 210

those where the cells are rapidly renewed. ``` most to least: embryonic tissues haematopoietic organs (spleen, bone marrow) the gonads the epidermis the intestinal mucous membranes (variable) connective tissue muscle tissue and nerve tissue ```

Answer 211

genetic disorder (autosomal recessive) in which there is a decreased ability to repair DNA damage such as that caused by ultraviolet (UV) light.

Answer 212

DNA viruses • more common • viral DNA inserted into host DNA RNA viruses • reverse transcribed and then inserted • may contain “oncogenes”

Answer 213

Epstein-Barr virus - Burkitt's lymphoma, nasopharyngeal carcinoma

Answer 214

hepatocellular carcinoma

Answer 215

• Viral protein binds to and | inactivates the tumoursuppressor, p53

Answer 216

``` • hormones breast cancer hormonal dependence ovary, adrenal - prostate cancer testosterone ``` * drugs, inc alcohol * inflammation

Answer 217

Smooth muscle

Answer 218

Glandular epithelium

Answer 219

Surface epitheliu,

Answer 220

* 90% carcinoma | * 10% lymphoma or sarcoma (more in young)

Answer 221

Breast, lung, prostate, oral, bowel, malignant melanoma, kidney, liver

Answer 222

Higher incidence of oral cancer in more deprived people

Answer 223

de novo - from new via a benign tumour via a premalignant lesion - HNSCC some cases

Answer 224

``` some of the changes in cells and tissue architecture are seen before invasion occurs • this disorganisation of the tissue is called DYSPLASIA ``` forms the basis of cancer screening - detection of premalignancy e.g. cervical smears or oral leukoplakia and dysplasia in the mouth

Answer 225

at the peak height velocity

Answer 226

During adolescence

Answer 227

The lymphoid tissues grow rapidly in childhood and by twelve years of age represent about twice the size of their relative final size. The tonsils in particular reduce in size during adolescence

Answer 228

facial growth occurs at more or less the same time as growth in height (with the exception of the orbits and part of the upper face). Height change over time measured as standing height might therefore be used as a proxy for growth in facial structures.

Answer 229

a measure device for height

Answer 230

Age on the x axis | cm/year on y axis

Answer 231

Post-natal growth is characterized by a rapid, then rapidly declining growth, in the early years. This then levels off in the period four to ten years, and increases again during the pubertal growth spurt. Growth then tails off again to zero by the age of sixteen for girls and eighteen for boys.

Answer 232

it is possible to predict PHV with an error of about six months using standing height.

Answer 233

Tell us current status and doesn't predict the future necessarily

Answer 234

Standing height measurement

Answer 235

the simple observation that when a young child is entering adulthood these changes occur and generally herald an increase in growth velocity can be valuable when assessing a patient with a dental deformity. Such information can usually be elicited by tactful history taking.

Answer 236

Houston concluded that information from hand-wrist radiographs is of only limited value in predicting the time of peak height velocity (PHV) and is no more accurate than standing height measurement Since they use potentially damaging ionising radiation hand-wrist radiographs are now considered an inappropriate tool in orthodontic investigation

Answer 237

the analysis can be carried out on a lateral cephalogram which is frequently taken by orthodontists for other clinical reasons. not using extra radiation

Answer 238

They suggested that peak mandibular growth velocity would occur within one year of CVMS II. (second stage out of 5 references stages)

Answer 239

This uses average growth increments for each chronological age to give expected growth. It produces an output from a computer system. studies have shown that much of the data produced does not reflect the final outcome as there are great variations in growth and treatment response.

Answer 240

use existing data from a patient such as a cephalogram and add average growth increments Unfortunately the assumption that an individual’s future growth pattern will be the same as average is least appropriate in those patients whose facial growth differs significantly from the average. These are the patients where a good prediction would be most useful.

Answer 241

There is an increase in all facial dimensions which continues throughout life. The size and shape of the craniofacial complex changes with time Vertical changes are most prominent and antero-posterior changes are less prominent. Little change in width occurs Female growth decelerates in the teens and then resumes in their twenties The magnitude of these growth changes is small but cumulative over years Growth rotation continues and on average there is a small decrease in the face height with a reduction in the Frankfurt mandibular plane angle Compensatory changes occur in the dentition Greater changes occur in the soft tissues rather than the hard tissues Lip incompetency decreases with age Lower lip grows more, and combined lip growth exceeds, lower face height (LFH)

Answer 242

Trabecular/cancellous bone - 20% of skeleton, porous meshwork, mainly in axial skeleton Cortical/ compact/laminar bone - approx 80% of the sekelton, dense, strong, outer layer Woven bone - forms quickly during periods of repair or rapid growth and is remodelled into lamellar bone.

Answer 243

Bone is 30% organic material (majority is collagen), 45% inorganic [hydroxyapatite (HA)-a hydrated crystalline material of calcium and phosphate].

Answer 244

Scaffold of interwoven collagen fibres (>95 % type I collagen, <5% type V). Between fibres are small, uniform, plate-like crystals of carbonated hydroxyapatite Small amounts of non-collagenous proteins, some which are unique to calcified tissue (eg osteocalcin).

Answer 245

Support the body and protect internal organs allows movement - bones provide attachment for muscles so allowing leverage Haematopoiesis - bone marrow is the major producer of blood cells including cells of the immune system calcium homeostasis - serum calcium levels maintained by intestinal absorption, renal excretion and skeletal mobilisation or uptake

Answer 246

Endosteum (inner) and the periosteum (outer)

Answer 247

Osteoclast (Oc): Large multinuceated cells. Bone resorbing cells. Osteoblast (Ob): Bone forming cells. Osteocyte: Originate from osteoblasts which have become embeded in the bone matrix. Involved in sensing mechanical loads and Ca homeostasis. Bone lining cells. Originate from osteoblasts. Line quiescent periosteoal and endosteal surfaces of bone . ``` Osteoprogenitor cells (stromal cells). Precursors of osteoblastic lineage. ```

Answer 248

Cell processes lie in cannaliculi and link to each other and blood vessels for passage of nutrients

Answer 249

release calcium or | alter architecture of cancellous bone to meet new stresses.

Answer 250

Because bone can only grow by appositional growth

Answer 251

when the cartilage model also grows in thickness (diameter) due to the addition of more extracellular matrix on the peripheral cartilage surface, which is accompanied by new chondroblasts that develop from the perichondrium. happens in bone remodelling

Answer 252

bone develops by replacing hyaline cartilage. Activity in the epiphyseal plate enables bones to grow in length (this is interstitial growth)

Answer 253

Starts with resorption phase - bone ECM destroyed and removed = 3 weeks bone formation phase - new ECM formed and mineralised = 3-4 months

Answer 254

1. Bone resorption initiated by recruitment of osteoclast precursors to remodeling site 2. Osteoclast precursors mature into osteoclasts. 3. Bone lining cells erode a little ECM and leave the remodelling site. 4. Osteoclasts bind to the ECM exposed by bone lining cells and digest the bone matrix with enzymes to form a resorption pit. 5. Osteoclasts stop digesting matrix and die (apoptosis). 6. Osteoblast precursors recruited to the remodeling site. 7. Osteoblast precursors develop into mature osteoblasts. 8. Osteoblasts make new ECM to fill the resorption pit made by the osteoclasts. 9. When the synthesis of matrix is complete, the new bone surface becomes covered in bone lining cells. 10. Osteoblasts trapped within the ECM become osteocytes

Answer 255

during formation of the skeleton during fracture repair during tooth socket healing after extraction

Answer 256

woven bone - but the trabeculae are disorganised and cannot bear weight until remodelling occurs

Answer 257

1. Bone is formed directly from condensed mesenchyme or ectomesenchyme = intramembranous ossification (IMO) (osteoblasts formed) 2. A cartilaginous precursor of the bone is formed and replaced by bone as it grows = endochondral ossification (ECO) (chondrocytes formed)

Answer 258

Interstitial growth occurs in hyaline cartilage of epiphyseal plate, increases length of growing bone. Appositional growth occurs at endosteal and periosteal surfaces, increases width of growing bones. Interstitial growth only occurs as long as hyaline is present, cannot occur after epiphyseal plate closes.

Answer 259

mesenchymal stem cells migrate to the site of eventual bone formation condense, align differentiate into osteoblasts and secrete an organic framework of extracellular matrix (osteoid) is laid down in long strands. Others MSCs differentiate to form blood vessels Osteoblasts line the osteoid and begin to deposit calcium salts, mineralization, forming the bone matrix, forming trabecula - cycles of secretion and mineralisation (appositional growth) areas that completely fill with mineralised osteoid = compact bone areas that don't completely fill in and contain lattice structures = priary cancellous bones skull, clavicle, part of the mandible, facial bones, basicranium

Answer 260

the process of converting the cartilage in embryonic skeletons into bone. Cartilage is deposited early in development into shapes resembling the bones-to-be. Cells inside this cartilage grow and begin depositing minerals. The spongy bone forms, and osteoblasts (build bone, produce proteins, etc.) attach and lay down the mineral portions of spongy bone. Osteoclasts (erode bone to free up calcium) remove material from the center of the bone, forming the central cavity of the long bones. The perichondrium, a connective tissue, forms around the cartilage and begins forming compact bone while the above changes are occurring. Blood vessels form and grow into the perichondrium, transporting stem cells into the interior. Two bands of cartilage remain as the bone develops (epiphyseal plates), one at each end of the bone. During childhood, this cartilage allows for growth and changes in the shape of bones - all bones except skull, clavicle 1. A miniature cartilage replica of a bone is formed by differentiation of mesenchymal or ectomesenchymal cells into chondroblasts which mature into chondrocytes 2. Cartilage grows in a specific direction – by interstitial and appositional growth + invasion of blood vessels 3. Cartilage is converted into bone

Answer 261

the fundamental functional unit of much compact bone

Answer 262

a rare genetic disorder that interferes with intramembranous ossification (skeletal dysplasia): Autosomal dominant-defect in the RUNX2 gene Neurocranium underdeveloped - delay in closure of sutures Viscerocranium (facial skeleton) underdeveloped with severe dental malocclusion, delayed formation/ eruption, narrow high arched palate with increased incidence of cleft palette Large head and frontal bossing Clavicles reduced or absent-characteristic feature - can touch shoulders together

Answer 263

Proliferation zone - parallel columns of dividing cells - formatin of ECM hypertrophic zone - significant increase in cell size - matrix reduced - down regulation of ecm - production of factors to stimulate blood vessel ingrowth cartilage/ bone interface - cartilage matrix mineralisation - chondrocyte apoptosis (cell death) - blood vessels grow - production of new bone on mineralised cartilage matrix after fusion of the plates, EO only happens during fracture repair

Answer 264

a autosomal dominant genetic condition that affects bone formation via ECO. = dwarfism genetic defect in gene for FGFR-3 a membrane receptor is important in response of chondrocytes to growth factor (FGF-18) during development of cartilage template. ``` all bones formed by ECO reduced in length - trunk and limbs short basicranium short - middle 1/3 of face sunken producing a dish face profile with class III malocclusion ```

Answer 265

1kg in bone, 99% in bone Blood and ECF total 2.2mM = 60% Calcium Intracellular 100nM basal

Answer 266

reduced blood flow Neuromuscular irritability hypocalcaemia (low calcium) causes sodium influx in nerves - hence triggers uncontrolled contractions tetanic contraction - muscles twitching

Answer 267

1300mg adults

Answer 268

In the small intestine via transcellular (through the cell) - happens in low intake, active process via paracellular (between cells)- happens in high intake, passive process

Answer 269

Gut takes up dietary calcium - plasma absorbs some from the gut kidney excretes excess calcium - some reabsorption from the kidney with the aid of vitamin D from the gut Bone resorption and mineralisation balances levels in the plasma Parathyroid glands - chief cells release PT hormone when calcium levels are low, this causes osteolytic osteolysis (breaks down bone, stimulates osteoclasts - releases calcium ions in the circulation which maintains ION POTENTIAL) release causes hydroxylation of vitamin D in the kidney - activates vitamin D calcitonin released from parafollicular cells when calcium is high, inhibits bone resorption, not a significant role as removal of thyroid glands doesn't affect calcium levels much

Answer 270

Bone pain, insomnia, high blood pressure, asthma, cramps, anxiety, arrythmias, soft tissue calcification, constipation, increase in urination

Answer 271

Vitamin D Allows SI absorption upregulates carriers (transcellular) and changes junctions to be more porous to movement- paracellular

Answer 272

pro-vitamin to vitamin D3 and then hydroxylated in the liver and kidney (PT hormone) giving active vit D - 1,25-(OH)2D not really a vitamin as it can be synthesised in the body

Answer 273

``` 6-24 months of age enamel hypoplasia enlarged pulp horns delayed eruption caries risk prevention : vitamin D and fluoride ```

Answer 274

Effect on OB Osteoblasts release RANK ligand osteoclast differentation osteoclast activation

Answer 275

``` Cancer autoimmune disease hypertension and CVS disease Diabetes muscle strength schizophrenia and depression ```

Answer 276

``` lack of remineralisation in bones pathological amount of callus matrix causes pseudofractures including mandible bone pain muscle weakness periodontitis ``` can see areas of radiolucencies and pseudofractures on x rays (not proper fractures, poor mineralisation)

Answer 277

``` Bone becomes more porous/meshwork like Bones more likely to break with a fall reduced uptake of dietary calcium imbalance of bone turnover - less oestrogren as you age, so reduces osteoclast inhibition, so more bone is broken down reduced bone mineral density ``` vitamin D and calcium mixed tablets Alendronic acid tablets load bearing exercise in children and not adults

Answer 278

* Invasion (local spread) * pressure/obstruction * destruction & loss of function * metastasis * non-metastatic effects * 25% will die from cancer-related cachexia (weakness and wasting of the body due to severe chronic illness)

Answer 279

Invasion: local spread • path of least resistance • tissue destruction • perineural spread Metastasis: lymphatic spread • invasion of vessels – embolism or permeation • spread to draining lymph node(s) Metastasis: haematogenous spread • invasion of blood vessels– mainly veins tumour may enter the vascular system from the lymphatics via the thoracic duct • organs liver, lung, bone and brain Metastasis: transcoelomic spread • spread across serous cavities - pleural, pericardial, peritoneal • e.g. abdominal cavity colon cancer, ovarian cancer

Answer 280

• tumour cells interact with cells and molecules in the local environment ``` tumour cells gain new abilities • motility is enhanced • alter adhesion molecules • make poor basement membrane • increase protease production or reduce inhibitors • alter ECM ``` “metastatic cascade

Answer 281

Lymphatic | Blood (often later)

Answer 282

• blood (lymphatic spread rare)

Answer 283

local nodes, liver, bone and | brain

Answer 284

neck nodes, later lung | and spine

Answer 285

* pressure and obstruction * destruction * haemorrhage * infection * pain * anaemia * starvation & cachexia

Answer 286

often caused by biochemical substances released by tumour cells, e.g. TNFα • Fever, anorexia and weight loss/cachexia • endocrine syndromes • Cushings syndrome • metabolic effects e.g. hypocalcaemia • neurological problems e.g. neuropathy • haematological syndromes e.g. erythrocytosis

Answer 287

a syndrome (a set of signs and symptoms) that is the consequence of cancer in the body, but unlike mass effect, is not due to the local presence of cancer cells.

Answer 288

``` histological assessment • often related to differentiation • linked to prognosis • various methods • numerical grades (1,2,3 etc) • low, intermediate, high ```

Answer 289

``` • T = tumour • N =nodes (regional) • M =metastases - distant • specific staging systems for tissue/tumour ```

Answer 290

* biopsy * cytology (FNA) * imaging – CT and MR scanning * molecular analysis

Answer 291

* Surgery * Radiotherapy * Chemotherapy * Biological (immune) therapy * Supportive care and palliative cre

Answer 292

* Tiredness * Feeling sick * Difficulty eating and drinking * Skin reaction * Hair loss * Haematological changes * Possible long-term side effects

Answer 293

Intensity-modulated radiation therapy (IMRT) is an advanced type of radiation therapy used to treat cancer and noncancerous tumors. IMRT uses advanced technology to manipulate photon and proton beams of radiation to conform to the shape of a tumor - delivers less dose to normal tissue and can save important structures being affected

Answer 294

Convention chemotherapy agents - cytotoxic (targeting DNA) Targeted agents - Targeted therapies act on specific molecular targets that are associated with cancer, whereas most standard chemotherapies act on all rapidly dividing normal and cancerous cells Hormonal therapies - e.g. blocks oestrogen in breast cancer Biologic therapies - repair, stimulate, or enhance the immune response.

Answer 295

block tumor cell proliferation

Answer 296

``` Alopecia Pulmonary fibrosis cardiotoxicity local reactions to the injection renal failure neuropathy myalgia sterility cystitis diarrhoea nausea/ vomiting ```

Answer 297

``` • Oral mucosal disease • Dental disease • Discomfort • Social embarrassment Dry mouth immuno-compromised difficulty in maintain oral hygiene ```

Answer 298

``` Is it likely to develop this cancer what type of cancer is it is this the optimal drug for my cancer what's the optimal dose for my body will the cancer return? ```

Answer 299

a collection of signs and symptoms due to prolonged exposure to glucocorticoids such as cortisol. ... Cushing's syndrome is caused by either excessive cortisol-like medication such as prednisone or a tumor that either produces or results in the production of excessive cortisol by the adrenal glands. causes: - taking too much steroid - a growth (tumour) in the pituitary gland in the brain - a tumour in one of the adrenal glands above the kidneys

Answer 300

``` Pituitary gland Thyroid gland Thymus gland Adrenal glands Pancreas Ovary glands Testis ```

Answer 301

Stimulates the pituitary to release hormones which leave from the anterior pituitary

Answer 302

a hormonal disorder that develops when your pituitary gland produces too much growth hormone during adulthood. When this happens, your bones increase in size, including those of your hands, feet and face. Acromegaly usually affects middle-aged adults - not increasing in height in middle age

Answer 303

As its name implies, gigantism causes the individual to grow taller than average. This condition, which most often affects children, occurs due to a noncancerous tumor on the pituitary gland that creates too much growth hormone.

Answer 304

Hypothalamus releases growth hormone releasing hormone which stimulates pituitary to release growth hormone which stimulates the liver to release insulin-like growth factor 1 which has a negative feedback effect on the pituitary and hypothalamus

Answer 305

Increases their recruitment, proliferation and matrix - hence bones get fatter and longer in acromegaly/gigantism

Answer 306

Increases GH secretion | Induces epiphyseal plate closure - so becomes insensitive to GH, won't grow any taller

Answer 307

testosterone and other androgens can be converted to oestrogen via enzymatic pathways hence why males grow bigger - extra oestrogen pathway androgen receptor in bone too oestrogen acting on growth plate whereas androgen acting on radial bone?

Answer 308

GH affects via IGF1 for myofibril prolliferation usually proportionate to body size but little evidence that GH promotes hypertrophy Pararcrine IGF1 has a rolr ein load-induced hypertrophy

Answer 309

Oestrogen inhibits condylar growth and testosterone increases

Answer 310

Increases GH secretion Affects sst and GHRH release chronic exposure reduces GH release treatment of children with corticosteroids likely to reduce growth in adults risk of osteoporosis, protein loss and raised serum lipids chronic glucocorticoid use mitigated by rGH leads to glucose intolerance

Answer 311

Promotes gluconeogenesis, glucogenolysis, lipolysis - increases blood glucose levels (diabetogenic) insulin is then released to compensate long term this can lead to insulin resistance

Answer 312

T3 affects chrondrocytes and osteoblasts in growth plate drives IGF1 synthesis prepubertal Stimulated GH synthesis Hypothydroidism reduces adult height

Answer 313

TH dependent - prepuberty GH dependent - puberty both drop off after puberty

Answer 314

``` Social model Individual model (includes the medical model) medical model biopsychosocial model ```

Answer 315

 Views disability as functional limitation which is biologically or physiologically determined ```  Medical-biological diagnosis which emphasises:  Individual pathology,  Individual (personal) deficit,  Individual medical treatment ```

Answer 316

 Disability is viewed as a tragic problem for isolated, unfortunate individuals.  The focus is on what the individual can’t do, or what’s wrong with them  The individual model incorporates the medical model

Answer 317

it was not our impairments that were the main cause of our problems as disabled people, but that it was the way society responded to us as an oppressed minority

Answer 318

the permanent loss or limitation of physical, cognitive or sensory functioning

Answer 319

the loss or limitation of opportunities to take part in the everyday life of any given community on an equal basis with others, resulting from structural and social barriers which take little or no account of people with disabilities, and excludes them from the mainstream of social activities.

Answer 320

``` Disability is seen as oppression either institutional or societal or both  We live in a disabling society  Society disables (through barriers; attitudinal or structural) those who have impairments ```

Answer 321

For some disabled people health care workers (doctors, dentists, nurses etc.) are ‘part of the problem’.  Health assessments often emphasise individual deficit in an attempt to ensure that disabled people receive the resources and services they need

Answer 322

 Views disability as arising from a combination of factors at the physical, emotional and environmental levels Consistent with the WHO’s revised definitions of disability Recognizes that impairments are often due to illness or injury and does not dismiss the importance of the impact of biological, emotional and environmental issues on health, well-being, and function in society

Answer 323

 Mental Capacity Act (fully enacted November 2007) - Any preconceptions and prejudicial assumptions held by a person making the assessment of capacity must therefore have no input into the assessment of capacity  Human Rights Act 1998 (in force 2004)  Equality Act Oct 2010 (supersedes DDA)

Answer 324

the person cannot comprehend the information relevant to the decision the person can't retain the information for long enough to make the decision the person cannot use the information to weight it up and arrive at a decision the person cannot communication the decision in any way e.g. not even by blinking to say yes or no

Answer 325

Capacity in the past does not mean the person has capacity now however take into consideration past as well as present wishes consider written statements whilst capacity was present consider values and beliefs of the person seen in progressive or acquired impairments ```  Talk to the person  Include them in choices and decisions about their care  Find out how each individual communicates  Use non-medical language to explain  Do not assume they cannot understand ```

Answer 326

Embryological defects - evident at birth - more severe anomlies may be incompatible with life Developmental defects - may not be immediately apparant - presentation often worsens with growth

Answer 327

Facial clefts - cleft lip and/or palate, mid face clefts first arch anomalies - hemi facial microsomia, treacher collins syndrome

Answer 328

Forme frustre - almsot there failure of fusion of the last little section causes gap in the vermillion border and a minor degree of asymmetry affects later incisor/canine - canine guidance

Answer 329

``` Facial appearance Hearing difficulties speech difficulties dental anomalies, crowding oronasal fistula growth and scarring from surgery skeletal III pattern ```

Answer 330

Abnormal palatal function affects drainage of the inner ear | build up of fluid in the middle ear which can be intermittent or cause a long term hearing deficit

Answer 331

Backing issue - can’t say d sounds - G instead because hard to place tongue against upper front teeth normally Nasality - palate doesn’t seal off the back of the mouth well - surgery can make the palate longer

Answer 332

Upper permanent lateral incisor

Answer 333

Restriction of maxillary development | adverse effects of facial growth

Answer 334

Because of the connection to the nose (oronasal fistula), strong tastes aren’t preferred so toothpaste is too strong for them and toothbrush bristles are hard and dont want it to go near the sensitive area. Prefer soft food that can just be swallowed which are usually heavily sugar laden quality of their enamel - pitted hypoplastic enamel makes it hard to maintain poor oral hygiene

Answer 335

Failure of the brain to dividie into left and right hemispheres causes median facial clefting all midline features affected to variable extent range of presentations pituitary gland develops in the midline

Answer 336

Embryological defect at 4 weeks - interrupted blood supply to branchial arch causing restricted facial development range of presentations one side of the face doesn't grow as well as the other - affects the development of the lower half of the face, most commonly the ears, the mouth and the mandible.

Answer 337

Autosomal dominant mutation of gene controlling TREACLE protein which affects amount and low of mesenchyme in first and second pharyngeal arches

Answer 338

``` Hypoplastic maxilla (espec zygomatic arches) and mandible Ear anomalies often with atresia of auditory canals Ocular anomalies - coloboma Cleft palate/high arched palate Airway problems affects zygomatic bone- very little support for the cheekbones usually have a bone achored hearing aid ```

Answer 339

Craniosynostoses e.g Crouzon’s syndrome Apert’s syndrome Achondroplasia

Answer 340

Premature fusion of 1 or more fibrous sutures resulting in distortion/abnormal cranial development and facial features. 20% are linked to a specific syndrome Aetiology- autosomal dominant/spontaneous mutation

Answer 341

Type of craniosynostosis ``` •Autosomal dominant/spontaneous mutation •Variable expression •Premature fusion of sutures results in abnormal skull development and raised intra cranial pressure (often needs early surgical release). ```

Answer 342

``` •Facial appearance •Increased intra cranial pressure – often require early and subsequent surgery. •Hydro cephalus. •Restricted mid face development: Choanal atresia (blocking of nasal air passages),Obstructive Sleep Apnoea •High arched palate •Speech difficulties •Hearing difficulties •Class III skeletal pattern ```

Answer 343

incidence 1 in 50,000 Similar facial presentation to Crouzon but also has associated syndactyly Aperts is also associated with learning difficulties whereas crouzon's is not

Answer 344

Dwarfism autosomal dominant a disorder of bone growth that prevents the changing of cartilage (particularly in the long bones of the arms and legs) to bone. It is characterized by dwarfism, limited range of motion at the elbows, large head size (macrocephaly), small fingers, and normal intelligence.

Answer 345

•Understand the multidisciplinary nature of care for these individuals •Prevention as a high priority. •Ensure routine care is accessable and a crucial part of a complex overall treatment strategy

Answer 346

Pouches that correspond to clefts

Answer 347

Outer surface covered by ectoderm Inner surface covered by endoderm mesoderm between the endoderm and ectoderm Packed with ectomesenchyme Each arch has an arch artery, vein and cranial nerve, skeletal element and muscle block (from mesoderm so migrated into the arch).

Answer 348

``` • 1st arch, trigeminal • 2nd arch, facial • 3rd arch, glossopharyngeal • 4th arch, vagus ```

Answer 349

``` • Patterns of Hox gene expression within the midbrain and hindbrain set up identity • As cells migrate from the neural tube they keep this pattern • Regulates movement and differentiation of the cells ```

Answer 350

muscles of mastication • some suprahyoids • tensor veli palatini • Supplied by third division of CNV

Answer 351

muscles of facial expression • some suprahyoids • stapedius • All supplied by CNVII

Answer 352

* one trivial muscle (stylopharyngeus) | * Supplied by CNIX

Answer 353

* pharyngeal constrictors * muscles of soft palate and larynx * All supplied by CNX

Answer 354

``` muscles of mastication • some suprahyoids • tensor veli palatini digastric anterior belly tensor tympani • Supplied by third division of CNV ```

Answer 355

``` muscles of facial expression • some suprahyoids • stapedius posterior belly of digastric • All supplied by CNVII ```

Answer 356

``` meckel's cartilage - malleus and incus of ear maxilla and mandible spine of sphenoid bone sphenomandibular ligament palatine bone squamous part of temporal bone anterior ligament of malleus ```

Answer 357

Stapes, styloid process, Stylohyoid ligament, and Lesser cornu of the hyoid bone.

Answer 358

Large proportion of the hyoid bone (neural crest)

Answer 359

laryngeal cartilages and epiglottis

Answer 360

1st cleft all others only form temporary cervical sinuses – which are then obliterated by the rapidly proliferating 2nd pharyngeal arch.

Answer 361

1st Eustachian tube and middle ear cavity 2nd Lining of the palatine tonsils 3rd Dorsal – Inferior parathyroid glands Ventral – Thymus 4th Dorsal – Superior parathyroid glands Ventral – Ultimobranchial body (C cells)

Answer 362

cells that migrate into the thyroid gland and secrete thyrocalcitonin

Answer 363

1st arch - Lateral lingual swellings and tuberculum impar from 1st arch which produces the anterior portion of tongue (body) – innervated by lingual nerve (from trigeminal nerve) Arch 3 swellings overgrows arch 2 so doesn’t contribute to development of tongue really Arch 3 – posterior 1/3 of tongue receives innervation from glossopharyngeal nerve 4th arch - epiglottis - vagus

Answer 364

Muscles of tongue are innervated by hypoglossal nerve Muscle migrates from paraxial mesoderm and drag their nervous supply with them – hypoglossal nerve

Answer 365

1st ``` Clearest impact on skeletal structures • Hypotrophic mandible • Conductive hearing loss (incus and malleus) • Malformed external ear • Can be part of a syndrome arch ```