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ORAL MANIFESTATION OF

SYSTEMIC DISEASES:
Diabetic mellitus:
Groups of metabolic disorders
Characterized by chronic hyperglycaemia
due to low insulin or resistance or both
The constellation of abnormalities
caused by insulin v is called DM
The fundamental defects to which most
of the abnormalities can be traced are:
-v entry of glucose into various
peripheral tissues
- ^ liberation of glucose into the
circulation from the liver
*extra cellular glucose excess
*intracellular glucose deficiency
There's is also v entry ( aa) into muscle & ^
^ in lipolysis.
Aetiology of DM
Pancreatitis
Hormonal abnormalities
Pheochromocytoma
Acromegally
Cushing syndrome
During injury
Drugs
Insulin receptor abnormalities
Genetic
Major consumers of glucose

Brain
Muscles
Fat
* In the absence of insulin the entry of
glucose in these tissues v.
Glucose
Amino acids
Fatty acids
Ketane bodies

Promote the secretion of insulin


Insulin deficiency
< ( & glucagon excess)-------------------v
Degresed V |
Glucose V |
Uptake V V
I
I I
v ^Protein catabolismI
HPERGYCAEMIA I
Glucosuria ^Plasma aa,nitrogen loss
Electrolyte v < in urine
^ lipolysis
Osmotic diuresis v
I ^ plasma
FFA,
I Ketogenesis,
Ketonuria
V ketonemia
V I
|
------ Dehydration <--------------------
Acidosis
v
coma
Cellular actions of insulin

Regulations of glucose transport


Regulation of glucose metabolism
Regulation of gene transcription
DM

Hyperglycaemia
Altered metabolism of lipids
Altered and metabolism CH2O
Altered metabolism of proteins
High risk of complication from vascular
disease.
Oral manifestation

Oral Ulcerations
Oral Candidiosis
Periodontal disease
dehydration
Different types of DM

IDDM type I
NIDDM Type II
Generally all forms of diabetes is due to
- Insulin deficiency
- Insulin resistance
Insulin decreased concentration
of blood sugar by:
Inhibit hepatic glucose production
Stimulating uptake and metabolism of
glucose in muscle adipose tissue
Treatment of DM

Insulin therapy
Oral hypoglycaemic agents
Sulfonylureas
Biguanides
Somatostatin
Sulfonyliureas

Tolbutamide
Acetohexamide
Tolazomide
Chlorpropamide
Glibenclamide
Mechanism of action

Stimulate insulin release from pancraetic


(-cells)
Increase insulin levels by decreasing
hepatic clearance of the hormone
Biguanides

Metformin
Phenformin
Buformin
Mechanisms of action

Increase insulin action in the peripheral


tissue
Inhibit gluconeogenesis
Decrease absorption of glucose from
intestine
Normal value of B/Sugar

3-6.7 mmol/l (fasting sugar)


Postprandial 5-11 mmol/l
Addisons Disease

Primary adrenal cortical insufficiency


May result from adrenal gland infection
(Tuberculosis)
Autoimmune disease (60%)
Idiopathic
Pernicious Anaemia
metastatic -Ca
Characterised by

Low cortisol production


High ACTH
High MSH (diffuse pigmentation of the skin)
Weakness
Wt loss
Nausea
Vomiting
Hyperkalaemia
Age of occurence

3rd and 4th decade


Treatment -

Hydrocortisone with fludrocortisone


I/V fluid and maintan BP
Chemoehterapy for TB
Ix the cause
Absence of steroid response to
stress
Hypertension
Collapse
Death
Hyperparathyroidism

Due to hyperfunction of parathyroid


gland
Can be primary or secondary
Clinically presents with
High PTH
High serum Ca2+
Low serum phosphate
Primary Hyperparathyroidism
causes:
Parathyroid adenoma
Hyperplasia [MEN Type II a and b]
Carcinoma of the gland
Secondary
Hyperparathyroidism
Occurs in patient with some form of:
Osteomalacia
Pseudohypoparathyroidism (resistant to end
organ response to PTH)
Chronic renal failure
Malabsoption syndrome
Peak age 20-60 yrs
Nausea, vomiting, peptic ulcer, pancreatitis,
necrosis.
Investigation:

Isitope scanning (90%)


US
MRi (64%)
Serum Ca2+, phosphate and alkaline
phosphate
ORAL MANIFESTATION

Brown tumours (Jaws)


Pathological fractures
Treatment

Surgical removal of parathyroids


Steroids to suppress level of
hypercalcaemia
Rehydration
Biphosphanate therapy
Leukemias

Primary neoplastic colonal disorders of


the bone marrow.
Can affect
Erythroid precusor
Myeloid precursor
Lymphoid precursor
Types

ALL (Lymphocytes)
ANLL (myeloid, erythroid,
megakaryoctye)
CLL (Lymphocytes)
CGL (Granulocytes)
Acute involved proliferation of
immature cells
Chronic involve proliferation of mature
cells
With exception of ALL 50% of
leukemia occurs in elderly
Aetiology

Hereditary factors
Ionizing radiation
Chemical and drugs (benzene ring)
Cytotoxic drugs [alkylating agents]
Chromosomal abnomaliting [ALL or ANLL]
t (9 : 20)
t ( 4: 11)
t (9 : 14)
t (9 : 22)
Leukemic virus [[HILV-1] (retrovirus)
Down syndrome
Acute Leukemias

ALL (80%) childhood


ANLL (85%) > 15 yrs
Clinical features

Attribute to failure of normal haemopoiasis


Anema
Recurrent infection
Bleeding from the gum
Petechial haemorrhages into the skin
LNDS enlargement
Spleen enlargement
Blood picture

High leucocyte count


Blast cells N/CP-R
Thrombocytopenia
Bone Marrow aspiration

Increased cellularity with primitive blast


cells
[Netrophills and megakeryotes are
sparse]
Dx

Clinical features
Blood picture
Morphology of bone marrow
Rx

VAP ( vincristine, adriamycine,


predinsolone )
Chronic Leukemias

CLL
CGL
CLL

Rare below 40 years


Age onset [50 60 yrs)
Account for 50% of leukaemia's which
occur after 60 years
Characterized by high number of
leukocytes in circulation & on other
tissues
Blood Picture

Increased leukocytes 100x109/l


Pancytopenia
Haemolytic anaemia
Thrombocytopenia
IgM low
B cell 95%
Tcell 5%
Bone marrow

Increased lymphocytes
Generalized LNDS enlargement
Liver enlargement
Treatment

Splenectomy
CGL

Common in adult but can occurs at any


age
Clinically:
Dominated by gross enlargement of liver
and spleen
Anaemia, thrombocytopenia
After several years ANLL or ALL
Blood picture

Leukocytosis > 300x109/l [ increased


mature neutrophils, granulocytes,
myelocytes]
Increased basophils signification
diagnosis
Bone marrow aspiration

Increased cellulority of marrow [ soft pale


pink or greenish tissue extending into log
bones]
Treatment

Mostly palliating
Glivac (signal transduction inhibitor)
Bone marrow transplation
Hydroxyurea
Bulsulphan
IRON DEFICIENCY ANAEMIA

Occurs when the supply of iron to bone marrow


falls short of that required for the production of
red blood cells
Stem cells Erythroid promoting hormone
(erythroposeting)
Pronomoblast
Posses specific receptor for iron transferring
complex (enable to incorporate sufficient iron
for haeomoglobin production)
Definition

Anaemia
- Decreased red cell mass
- Decreased 02 carring capacity of the
blood
- But blood volume constant
Etiology

Increased requirement
Inadequate diet intake
Decreased intestinal absorption
Blood loss
Decreased intake occurs:

Following partial or total gastrectomy


Achlorhydria
Chronic diarrhoea
Intestinal malapsorption
Blood loss

Peptic ulcer
Cancer
Hemmorhoids
Salicylate ingestion
Platelate disorder
Von Willebrands disease
Clinically
Weakness
Fatigue
Palpitation
Dysphagia [Plumer-Vinson-Syndrome]
Nausea
Constipation
Pallor
Angular stomatitis
Glossitis
Enlarged spleed
Evaluation [Lab)

Low serum iron


High serum iron-binding capacity
MCHC High
% reticulocyte normal
RBC Microcytic and hypichromic
Bone marrow aspiration
Displaced erythroid hyperplasia
[ineffective erythropoiesis]
DDX

Hypochronic microcytic anaemia


Iron deficiency
Thalassemia
Anaemia of chronic inflammation
Sideroblastic anaemia
Treatment
Iron therapy [ferrous sulphate 300 mg x tds for six
months]
Parenterally iron dextran 500 mg infused slowly
Transfusion of blood is seldom indicated unless there is
evidence of cardiovascular compromise
Haemoglobinopathies

HbA1 (2 2) 97%
HbA2 (2 S2) 3%
HbF (2 x2) less than 1% of total HB
Haemoglobinopathias associated
with sickling
HbSS
SThal
SC
About 90% of these abnomal
haemoglobins are single amino acid
susbtitution
Substitution valine for glutanic acid at the
sixth position of -chain HbS
Haemoglobinopathias associated
with sickling (cont.)
Substitution Lysine for glutamic acid at
the same position HbC
Substitution glycine for flumatic acid at
121 position HbD
Individual with sickle cell trait usually
have
60% HbA
35% HbS
In a disease state sickle cell trait HbS
is more than 50%
Sickle Cell anaemia

Its due to molecular lesion


HbSS clinical signs appear after 6
months of life when more of HbF
replaced by HbS
Substitution of valine for glutamic acid at
the sixth position of the B-chain
Sickled cell becomes rigid and abstract
capillary vessels flow
Sickle Cell anaemia (Cont)

Tissue hypaxia
Infarction
Loss of K+ and water form RBCs
Distracted in the spleen
Haemolytic anaemia
High 2 3 DPG Lower oxygen affinity of
RBCS
Hypertonic environment of renal medulla
Local sickling
Clinically
Impairment growth and failure to thrive
Infections (Pnemococci)
Spleen become constracted Low clearing of circulating
bacteria
Infarction of organs
Anaemia
Pain crisis (vasoocclusive due to infection)
Pleuric chest pain
Icterus
Haematuria
Expansion of bone marrow
IX

Solubility test
Electrophoresis
Treatment

Rx Infection early
Give malaria prophylaxis (proguanil1)
Hydrate the patient
Give analgesias
Administer O2 during acute pain crisis
Vit. B. Deficiency

Vit B deficiencies may involve one or


several of the water-soluble B complex
vitamins
Aetiology

Malnutrition associated with alcoholism


Starvation
GIT disease (V absorption)
Hyper parathyroidism (increased
demand)
B-complex Vitamins

Biolin
Nicotinamide
Pantothenic acid
Thiamine
Are involved in intercellular metabolism of
carbohydrates, fats and proteins
Others
- Vit B12 (are involved in erythrocyte
- Follic acid development)
B-Complex Vitamins (cont.)

Folate in cell B12 Polyglutarute


(maintain cell folate)
When Vit B12 is deficient follate
metabolism is derraged defect in DNA
Vit B12 necessary for DNA synsthesis
esp. in rapidly dividing cells such as
those found in bone marrow and GIT.
Clinically

Cheilitis
Glossitis
Crackling and fissuring of the lips
(angular chelitis)
Reddened tongue
Atrophy papillae
Burning pain of the tongue
Clinically (cont.)

Keratitis of the eyes


Scaly dermatitis on the nasolabial area
and genitalia
Diarrhoea
Dementia
Death
Dermatitis
Folic acid deficiency results

Megaloblastic RBC
Megaloblastic enaemia
Diarrhoea
Oral lesions
Vitamin B deficiency

Pernicious anaemia
Lack of intrinsic factor
Rx- Replacement Vit B12
[cyanocobalamines]
Cherubism

Hereditary condition of maxilla and


mandible (autosound dominant)
Found in children by 5 years of age
Presents with marked fullness of the
jaws and cheeks and upward gazing
eyes
Clinically

Painless expansion of jaws


Premature exfoliation of dentition (3
years)
Ectopic eruption
Missing permanent or malfomed (2nd and
3rd mandibular mollars)
Lymphodenopathy
Normal serum Ca2+ and phosphate but
increased alkaline phosphate
Radiographically

Well defined multicocular radiolucencies


Thinning of cortical plate with occasional
perforation
Displacement of inferior alveolar nerve
Un erupted teeth often displaced and
appear to be floating in a cystic like
spaces
Histologically

Resemble central giant cell granuloma


High vasculense high vascularised
Numerous fibroblasts
Multinuclear giant cells
Eosinophilic perivascular cuffing of
collagen
DDX

Hyperparathyroidism
Multiple odontogenic keratocysts
Treatment

Self limiting at puberty


Surgery to improve function and
esthetic (conservative curettage with
bone recontouring)
Albright syndrome

Polyostatic type of FD
Usually affect children
Characterized by:

Polyoslolic FD with a tendency to


unilateral distribution
Abnormal pigmentation of skin and
occasionally of the mucous membrane
Sexual precocity especially in females
due to endocrine dysfunction
Skin pigmentation

Caf an-Lait type


Scattered over the back, thigh, buttocks
and occasionally oral mucosa
Endocrine

Precocious puberty with menarch from


1-5 years in 50% or 6-10 years in 30%
Breast development
Pubic and auxiliary hair appear after the
menarch
In-male it may accompanied by
gynaecomarlia
Bone lesion
Polyoslolic FD
Aetiology

Development defect
Treatment

Cosmetic reasons shaving but be


caseful with haemorrhage in young
patients (high vasularised)
Posp op Review [important]
Malignant change
OM [adult] decreased vasculenty at the
area
Infection? [ decreased vascularity]
Clinically presents:

Tense, non-pitting oedema of the face,


neck, heads, feet genitalia and GIT
Episode of painless swelling which
subside in 24-48 hrs
Oedema may extend to the glottis
airway obstruction
IX

Diagnosis confirmed by the assay of C,


esterase inhibitor and C4
MX

Fresh frozen plasma


I/V traxanemic acid (antifbrolytic agent)
prior surgery
Long term prophylaxis with anabolic
steroid stanazol (raises C1 esterase
inhibitor)