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SPORADIC DISEASES IN INDIA

Dr Mohin M Sakre.III Year P.G Student.

(MD) Community Medicine.KBNIMS, Kalaburgi.

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2 Contents…I. What are Sporadic Diseases?II. Types of Sporadic Diseases.III. Genetic or Prion Sporadic Diseases.IV. Infectious Sporadic Diseases.V. Other Sporadic Diseases.VI. Hepatitis A.VII.Hepatitis E.VIII.Alzheimer’s disease.IX. Conclusion.X. References.

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3 Introduction What are Sporadic Diseases?

Occurring Occasionally, Singly or in scattered instances.

Arising or occurring randomly with no known cause.

Occurring haphazardly and irregularly.

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Medically…….

Not denoting a temporal pattern of disease occurrence in an animal or human population in which the disease occurs only rarely and without regularity.

In the genetic context, sporadic denotes a singleton or sport.

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5 Types of Sporadic DiseasesSporadic diseases

Genetic or Prion disease Infectious others

A disease that is sporadic to one region

doesn’t have to be sporadic to another

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6 Genetic or Prion Sporadic Diseases Several quite different and disparate phenomena are covered by this

term, including a new mutation; occult non-paternity. the chance outcome for a recessive trait in two carrier parents with a

small family. extreme variability in the expression of a gene. an environmental phenocopy. a multilocal genocopy, etc.

No useful properties can be predicated of all members of this class; and the term is notionally useless.

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7 Examples:

Creutzfeldt-Jakob disease.

Kuru. Alzheimer’s

disease. Fatal Insomnia. Transmissible

spongiform encephalopathy.

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8Infectious

Occurs occasionally. A disease sporadic to one geographical region doesn’t have to be

sporadic to another, for example: Typhoid in US is sporadic where as it is a common occurrence in India.

In terms of sporadic infection, the difference is that the cases are very few and far apart.

It is different from low endemic diseases. Key word being no “temporal or spatial” relation with each other.

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9 Examples:

Chikungunya Sporadic

campylobacter infection.

Typhoid in the Americas.

Salmonellosis in the sub-continent.

Hepatitis A and E. Sporadic colorectal

cancer. Legionellosis. Diphtheria. Meningococcal

infections.

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Other Sporadic Diseases:

Sporadic goitrous cretinism. Sporadic hemiplegic migraine. Amyloidosis. Sporadic porphyria cutanea tarda. Sporadic primary pulmonary hypertension. Sporadic amyotrophic lateral sclerosis.

Even breast cancer was considered sporadic initially until it became a relatively commonly occurring disease.

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11 The Most commonly occurring sporadic diseases in India Hepatitis E and A. Salmonellosis. Chikungunya. Alzheimer's. Anthrax and Brucellosis in Animals. Diphtheria. Meningococcal diseases.

We’ll be discussing about Hepatitis A, E and Alzheimer’s disease.

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12 Hepatitis A

Sub Acute disease occurring in mainly children and young adults.

Caused by Hepatitis A Virus which is a Non enveloped 27 nm heat resistant, acid and ether resistant RNA virus in the hepatovirus genus of the picornavirus family.

Virus enters by Oral and Orofeacal route.

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13 It has an incubation period of around 2 to 6 weeks. The virus multiplies in the Intestinal Epithelium and

reaches the liver via haematogenous route. Epidemiology: The Infection spreads from case to case

through contaminated food, water or milk. It does not show a chronic carrier state and does not lead to hepatic carcinoma.

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14 Clinical Features:

Fever. Malaise. Anorexia. Nausea. Vomiting. Liver tenderness. Icterus.

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15The Antigen demonstrated in the blood is HAV antigen (during Late

Incubation and Pre Icteric phase), to counter which Anti-HAV antibodies (IgM - initially around the time of the onset of Jaundice and detectable in blood

for around 6 months and IgG which appears around the same time and persists for years) are produced.

Laboratory Diagnosis

Demonstration of Virus

IEM, ELISA, Isolation

Demonstration of Antibody

IgM<IgG

Biochemical tests

ALT, Bilirubin

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17 Treatment: No specific antiviral therapy is required. The treatment is only

symptomatic.

Prevention: Improved sanitation and prevention of contamination of food an

water. Passive prophylaxis with Normal Human Pooled Immunoglobulin

may prevent apparent clinical innless. A formalin deactivated, alum conjugated vaccine grown in Human

Diploid cell culture can be administered. Dose: 0.5 ml (12 months to 18 Years) and 1.0 ml (19 Years and

older). 2 IM injections, 6 months apart. Protection lasts for around 10-20 years.

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18 Hepatitis E

A disease resembling Hepatitis A caused by a spherical non-enveloped virus 27-38 nm in diameter belonging to the family hepeviridae and genus hepevirus.

Occurs in sporadic mostly, but also endemic and epidemic forms mainly in developing countries.

Mainly associated with contaminated water. Incubation period of 2-8 weeks with an average of 6 weeks. Occurs predominantly in middle aged adults.

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Clinical Features: Fever. Malaise. Anorexia. Nausea. Vomiting. Liver tenderness. Icterus.

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20 PCRLaboratory Diagnosis 1

• HEV RNA can be detected in the feces in the acute phase of the disease. The gold standard for diagnosis of Hepatitis E.

IMMUNOELECTRON MICROSCOPYLaboratory Diagnosis 2

• Examination of feces under an electron microscope.

ELISA AND WESTERN BLOT ASSAYLaboratory Diagnosis 3

• IgG and IgM antibodies.

Hepatitis E can also be diagnosed by ruling out Hepatitis A and B. Hepatitis A can be ruled out by IgM detection and Hepatitis B by

demonstration of HBsAg antibodies.

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21 Treatment: No specific antiviral therapy is required. The treatment is

only symptomatic.

Prevention:

Improved standards of Sanitation. Chlorinated Water. No vaccine is available.

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22Complications:

Relapsing Hepatitis. Cholestatic Hepatitis. Fulminant Hepatitis (Hepatic Necrosis). Persistent Anorexia and Weight Loss. Does Not cause Cirrhosis and Cancer.

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23 Alzheimer’s Disease Alzheimer’s is the most common form of dementia.

It’s a neurodegenerative disorder that occurs in the brain resulting in decline of cognitive function with decline in activities of daily living and behavioural disturbances.

It causes the deterioration of nerve cells in the brain. Most commonly between the age of 60 to 80 Years.

The deterioration of the brain cells in the brain leads to the reduction in responses of other brain cells.

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247 Stages of Alzheimer’s

Normal. Normal Aged Forgetfulness. Mild cognitive impairment. Mild Alzheimer’s. Moderate. Moderately Severe. Severe.

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25 Pathology:

Neuronal loss in the temporopareital and frontal cortex. Senile plaques and Neurofibrillary tangles are the

pathological hallmark of diagnosis. Other pathological changes include:

1. Synaptic degeneration.2. Accumulation of abnormal lysosomes.3. Neuronal loss.4. Glia mediated inflammation.

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26 Aetiology:

Ageing. Family history of Alzheimer’s. Reduced reserve capacity of the brain. Diet. Head Injury. Associated with risk factors associated with vascular

diseases.

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27 Clinical features:

Insidious in onset. Decreased ADL leading to profound disability. Disturbances in memory, language and visual skills. Nominal and comprehensive dysplasia. Dyspraxia. Agnosia. Behavioural problems, depression and psychotic problems.

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28 Laboratory Investigations:

These are carried out to rule other causes of dementia. CBC. VDRL. B12 and Thyroid functions. CT head. MRI. PET and SPECT.

Criteria for diagnosis: Episodic memory deficit + impairment of at least one other cognitive

domain.

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Management:

Cholinesterase inhibitors: Donepezil (5mg HS OD > 10 mg HS OD after 4 weeks) , Rivastigmine (1.5 mg BD > 6mg BD over 6 months), Galantamine (4 mg BD > 12 mg BD with step-ups every 4 weeks) etc..

Treatment of Co-morbid conditions.

Treatment of Behavioural problems.

Health Education.

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31 Conclusion In summary, sporadic diseases are the ones that occur periodically and in a

scattered fashion with no particular disposition of any kind. A disease sporadic to one area can be endemic to another and vice-versa.

Even though no specific measures of prevention can be applied to all sporadic diseases, maintenance of good health and hygiene status can go a long way to try and prevent the occurrence of sporadic diseases.

As in the case of the diseases discussed today, Hepatitis A and E can be prevented by clean water and food and good hygiene practices. The vaccine for prevention of Hepatitis A provides a success rate of around 95%. Studies have recently shown that changes in lifestyle, cognitive function improvement, the use of bio marker and genetic inclusion criteria and intervention with anti-hypertensives provide a positive step towards the prevention of Alzheimer’s.

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32 References www.references.com/Health/Conditions&Diseases. www.ncbi.nlm.nih.gov. www.thefreedictionary.com. www.cdc.gov/CSELS/OPHSS. www.medicinenet.com. www.Britannica.com. www.medilexicon.com. Textbook of Microbiology by C.P Baweja. Textbook of Medicine by K George Matthew.

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