Progeria is an extremely rare genetic condition where children age rapidly, experiencing many signs of aging at a very early age. It affects about 1 in 4-8 million births. The main symptoms include baldness, wrinkled skin, joint stiffness, loss of body fat and muscle, and cardiovascular problems. Average life expectancy is 13 years. While there is no cure, treatments can help manage symptoms and slow progression, such as low-dose aspirin, physical therapy, and lifestyle modifications. Progeria is caused by a genetic mutation and provides insight into normal human aging processes.

1. Aging
Disorder

3. Many persons believe that they
lived an unhappy childhood.

4. Our childhood was stolen.
We’ve grown up before
our times.
We've been deprived of
our childhood.

6. What is Progeria?
• Progeria is an extremely rare, fatal genetic condition
that affects children and gives them an appearance of
accelerated aging.
• The word Progeria comes from the Greek
rogeros meaning 'prematurely old'.
• It was first described in an academic journal by Dr.
Jonathan Hutchinson in 1886, and Dr. Hastings Gilford
in 1897 - both in England.
• The condition was later named Hutchinson-Gilford
Progeria Syndrome (HGPS).

7. • It is currently hypothesized to result from a gene
mutation arising around the time of conception or shortly
thereafter.
• Although the term progeria applies to all diseases
characterized by premature aging symptoms, it is often
applied specifically in reference to Hutchinson-Gilford
Progeria Syndrome.
• Progeria disease provides medical researchers a window to
better understand how the body works and to explain some
of the mysteries of the aging process.
What is Progeria?

8. Prevalence
• Progeria is listed as a "rare disease" by the Office of Rare
Diseases (ORD) of the National Institutes of Health (NIH).
• HGPS prevalence is reported to be 1 in 8 million births.
• The true prevalence, however, has been suggested to be
closer to 1 in 4 million births because many cases likely go
undiagnosed or are misdiagnosed.

9. • Progeria affects all races with about 97% of the
children affected being Caucasian.
• Also, it affects both sexes almost equally with
slight male predilection; the male-to-female ratio
is 1.5:1.
• In the past 15 years, children with Progeria have
been reported all over the world.
Prevalence

10. Fig : Distribution of children with progeria around the world

11. Fertility
• In general, people with progeria have such severe failure to
thrive (poor growth from the time of childhood) that it
prevents fertility.
• The absence of complete sexual maturation has been
considered characteristic of the syndrome.
• However sexual maturity is rare in these patients.

12. Prognosis
• As there is no known cure, the average life expectancy for a
patient with HGPS is 13 years, with an age range of 7-27
years.
• At least 90% of patients die from complications of
atherosclerosis, such as heart attack or stroke.
• Mental development is not adversely affected; in fact,
intelligence tends to be above average.

13. • With respect to the features of aging that progeria appears
to manifest, the development of symptoms is comparable to
aging at a rate eight to ten times faster than normal.
• With respect to features of aging that progeria does not
exhibit, patients show no neurodegeneration or cancer
predisposition.
• They also do not develop the so-called "wear and tear"
conditions commonly associated with aging, such as
cataracts (caused by UV exposure) and osteoarthritis
(caused by mechanical wear).
Prognosis

14. Symptoms
• Although they are born looking healthy, children with
Progeria begin to display many characteristics of
accelerated aging at around 18-24 months of age.
• The children have a remarkably similar appearance,
despite differing ethnic backgrounds.
• Most of the following features are manifested after the age
of three years in children with Hutchinson-Gilford
progeria syndrome:

15. • Baldness
• Pinched nose
• Small, wrinkled face
• Head large for the size of the face
• Loss of eyebrows and eyelashes
• Prominent scalp veins
• Delayed tooth formation
• Loss of muscles and body fat
• Bulging eyes
• Wrinkled, scaly, dry skin
• High pitched voice
• Short stature
• Stiffness in joints
• Progressive cardiovascular diseases
• Progressive atherosclerosis
Symptoms

16. Fig: Dutch Patient at the age of 1 year, 1 year, 2 years, 6 years, 7 years, 8
years, 10 years, and 12 years.

17. Treatments
• There's no cure for progeria.
• Regular monitoring for cardiovascular disease may help
with managing the child's condition.
• Some children undergo coronary artery bypass surgery or
dilation of cardiac arteries (angioplasty) to slow the
progression of cardiovascular disease.

18. • Certain therapies may ease or delay some of the signs and
symptoms. They include:
• Low-dose aspirin. A daily dose may help prevent
heart attacks and stroke.
• Other medications. Depending on the child's
condition, doctor may prescribe other medications,
anticoagulants to help prevent blood clots. The use of
growth hormone may help increase height and
weight.
Treatments

19. • Physical and occupational therapy. These may help with
joint stiffness and hip problems and may allow the child to
remain active.
• Extraction of primary teeth. Extraction may help prevent
problems associated with the delayed loss of baby teeth.
Treatments

20. Lifestyle and Home
Remedies• Some steps you can take at home that may help progeria
child include:
• Make sure the child stays well hydrated. Dehydration can be
more serious in children with progeria. Be sure the child
gets enough to drink, especially during an illness or in hot
weather.
• Provide frequent, small meals. Because nutrition and
growth can be an issue for children with progeria, giving
the child smaller meals more often may help to increase his
or her caloric intake.

21. • Provide opportunities for regular physical activity. Check
with the child's doctor to learn which activities are right
for the child.
• Get cushioned shoes or shoe inserts for the child. The loss of
body fat in the feet can cause discomfort.
Lifestyle and Home
Remedies

22. • Make sure the child is up to date on childhood
immunizations. A child with progeria isn't at increased risk
of infection, but like all children is at risk if exposed to
infectious diseases.
• Provide learning opportunities. Progeria won't affect the
child's intellect, so he or she can attend school at an age-
appropriate level.
Lifestyle and Home
Remedies

23. ORTHOPEDICS
• Specialty of medicine that deals with
diseases and injuries of the locomotor
system
• Literally means “straight children”

24. ORTHOPEDICS DISORDERS

25. CLUB FOOT

26. DEFINITION
Club Foot is a complex deformity of foot, result of
complicated inter- relationships between bone
ligaments and muscles.

27. CAUSES
 Defective gene – Hereditary
 Excessive pressure of amniotic fluid
 Uterine compression
 Arrested fetal development
 Circulatory failure
 Irradiation (maternal)

28. MEDICAL MANAGEMENT
1. CORRECTION
a) Stretching and casting (PONSETI METHOD)

29. b) Stretching and Taping ( French Method)

30. c) Dennis - Brown Brace

31. SURGERY

33. DEFINITION
Juvenile rheumatoid arthritis ( JRA) is a chronic
systemic inflammatory disease involving connective
tissue of one or more joints.

34. SIGNS
1. Involvement of both small and large joints
2. Joints are tender, swollen and warm
3. Reduced mobility
4. Prolonged fever
5. Muscle aches

35. SIGNS
6. Weight loss
7. Subcutaneous nodules
8. Hepatosplenomegaly
9.Pericarditis, myocarditis
10. Pneumonia

36. HIP DISPLACEMENT

38. DEFINITION
Hip displacement is referred to a condition in which
the femoral head and the acetabulum are improperly
aligned in which the ball of the femur is outside the
hip socket.

39. SIGNS
Laxity of ligaments
Asymmetry of gluteal fold
Limited ROM
Apparent shorter femur on affected side

40. SIGNS
Variation in gait
Extra fold at thighs and groins
Difference in length of limbs
Wading gait

41. MEDICAL MANAGEMENT
Pavlik harness

42. 2. SKIN TRACTION

43. 3. SURGERYAND CAST

44. FRACTURES

45. DEFINITION
A fracture is defined as a break in the continuity of a
bone. This can occur either in the long bones as is
most common or in flat bones such as skulls or pelvis.

47. SIGNS
Pain
Pallor
Paralysis
Generalized Swelling
Bruising
Tenderness
Deformity
Severe muscle rigidity

48. MEDICAL MANAGEMENT
1. CLOSED REDUCTION

49. 2. OPEN REDUCTION

50. 3. TRACTION

51. KYPHOSIS

52. DEFINITION
It is the deformity of the spine.
The term kyphosis refers to an enhanced angulations
in the thoracic or thoraco-lumbar spine in the sagittal
plane or a round back deformity.

53. CAUSES
It can develop due to defective posture.
Rickets
May be congenital or secondary to
compression fracture.
Malignancy
Juvenile Rheumatoid Arthritis

54. MEDICAL MANAGEMENT
Orthotic devices
Orthopedic surgery
Pre and post operative care
Psychological support

55. SCOLIOSIS

56. DEFINITION
It is a lateral or side curvature of spine due to
abnormality in alignment of spine.
Scoliosis develops two curves – one is original
abnormal curve and second a compensatory curve in
the opposite direction.

57. CAUSES
Idiopathic
Defective embryonic development of the spine.
Neuromuscular paralysis
Cerebral palsy
Post polio
Rickets
Fractures
Disease of hip and spine.

58. SIGNS
Leg length discrepancy
Shoulder asymmetry in breast size
Uneven waist line
Scapular prominence
Bone pain
Poor posture

59. MEDICAL MANAGEMENT
Orthotic devices
Surgery
Pre and post op casting
Psychological support
Health education
Exercise
Counseling of parents

60. BOW LEG (Genu
Varum)
the term bow leg refers lateral angulation of knee
joints because of inward deviation of longitudinal
axis of tibia and femur.

61. CAUSES
Rickets
Traumatic
Developmental anomalies
Physiological

62. MEDICAL MANAGEMENT
Early detection of condition.
Orthopedic intervention
Surgical corrections

63. KNOCK KNEE ( Genu
Valgum)
Knock knee is abnormal convergent of knee with
divergent ankles.
This occurs due to outward deviation of longitudinal
axis of both tibia and femur

64. CAUSES
Pathological : rickets, JRA.
Cerebral palsy
Fractures
Neoplastic disease

65. MEDICAL MANAGEMENT
Early detection
Orthopedic intervention
Correction using orthopedic devices.

66. POLYDACTYL
It is defined as an extra finger or toe.
Usually occurs at metatarsophalangeal joints of little
finger or thumb.

67. CAUSES
Carpenter syndrome
Trisomy 13
Mekhel gruber syndrome
Isolated trait

68. SYNDACTYL
It is fusion of digits and toes.

69. CAUSES
Isolated trait
Compound of certain syndrome
a) carpenter syndrome
b) trisomy 21, 13 & 18