Thalassemia Thalassemia is an inherited autosomal recessive blood - - PowerPoint PPT Presentation

▶

Oct 08, 2022 246 likes •641 views

Thalassemia Thalassemia is an inherited autosomal recessive blood disease that originated in the Mediterranean region. In Thalassemia, the genetic defect which could be either mutation or deletion, results in reduced rate of synthesis or no

SLIDE 1

SLIDE 2

Thalassemia

Thalassemia is an inherited autosomal recessive blood disease that originated in the Mediterranean region. In Thalassemia, the genetic defect which could be either mutation or deletion, results in reduced rate of synthesis or no synthesis of one of the globin chains that make up

hemoglobin. This can cause the formation
f abnormal hemoglobin molecules, thus

causing anemia, the characteristic presenting symptom of the Thalassemia

SLIDE 3

two major forms of the disease, α and β are prevalent in discrete geographical clusters around the world.

SLIDE 4

 Inf

Infection ection

 Ir

Iron o

n over

erload load

 Bone def

Bone defor

rmities

mities

 Enlar

Enlarged spleen ged spleen

SLIDE 5

The hepatitis C virus is a small (50 nm) in size, enveloped, single-stranded, positive sense RNA virus. .An infectious disease primarily affecting the liver, caused by the hepatitis C virus (HCV). The infection is often asymptomatic, but chronic infection can lead to scarring of the liver and ultimately to cirrhosis. An estimated 180 million people worldwide are infected with hepatitis C.

SLIDE 6

Treatment

Generally recommended for patients with proven hepatitis C virus infection and persistently abnormal liver function tests. Current treatment is a combination of pegylated interferon-alpha-2a or pegylated interferon-alpha-2b (brand names Pegasys or PEG-Intron) and the antiviral drug ribavirin for a period of 24 or 48 weeks, depending on hepatitis C virus genotype.

SLIDE 7

Several alternative therapies are claimed by their milk thistle, ginseng, colloidal silver, licorice root (or its extract glycyrrhizin), lactoferrin, TJ-108 (a mixture of herbs used in Japanese Kampo medicine), schisandra, and

xymatrine (an extract from the sophora

root) proponents to be helpful for hepatitis C

SLIDE 8

Spirulina

Is the common name for human and animal food supplements. Spirulina comes from two different species of Cyanobacterium: Arthrospira platensis, and Arthrospira maxima.

SLIDE 9

Spirulina contains an unusually high

amount of protein, containing all essential amino acids. Spirulina is also rich in gamma-linolenic acid (GLA), and also provides alpha-linolenic acid (ALA), and Arachidonic acid (AA). Spirulina contains vitamin B1 (thiamine), B2 (riboflavin), B3 (nicotinamide), B6 (pyridoxine), B9 (folic acid), vitamin C, vitamin D, and vitamin E. Spirulina is a rich source of potassium, and also contains calcium, chromium, copper, iron, magnesium, manganese, phosphorus, selenium, sodium, and zinc.

SLIDE 10

 Antiviral.

 Immune-enhancer.  Anti-anemic.  Anti-oxidant.  Anti-mutagenic.  Cardio-protective.  Anti-inflammatory.

SLIDE 11

The proteins, lipids, and carbohydrates in Spirulina are digested, absorbed, and metabolized by the humans upon oral

consumption. Spirulina can be consumed

at a dose of 3 – 20 g/day without manifestation of any adverse effects. Studies on the acute, sub-chronic, and chronic toxicity and mutagenicity of Spirulina have revealed no specific body

r organ toxicity or genotoxicity.

SLIDE 12

To date, there are no drug interactions reported with Spirulina. The United Nations Organization (UNO) has recommended Spirulina as the ideal food and the World Health Organization (WHO) has also declared Spirulina as a safe food with excellent nutritional value

SLIDE 13

Aim of the Work

This study will aim to evaluate the effect of the Cyanobacterium Spirulina

sp. on thalassemic children,

who are infected with Hepatitis C virus.

SLIDE 14

Follow up of 25 Egyptian thalassemic children infected with HCV, aged from 3 to 8 years was performed, who were 12 male, and 13 female

children. Volunteers were supplied with dried

Spirulina (250 mg/Kg/day) by oral uptake.

Then clinical parameters were recorded at regular intervals (0, 3, and 6 months) of treatment by the analysis of ALT, AST, creatinine, urea, serum albumin, total bilirubin, ferritin, hemoglobin, RBCs, WBCs counts, percentage of segmented neutrophils, absolute differential neutrophil count, CD4, and CD8 concentrations. Mainly HCV quantification was performed by real time PCR; and blood transfusion intervals were observed.

SLIDE 15

94.3 74.1 56.9

20 40 60 80 100 120

At start After 3 months After 6 months

Mean value of ALT (U/L) Follow up intervals

SLIDE 16

5 cases = 20 % 20 cases = 80 % 7 cases = 28 % 18 cases = 72 %

8 cases = 32 % 17 cases = 68 %

5 10 15 20 25

≤ 40 (U/L) > 40 (U/L)

Cases representation ALT level ranges at start after 3 months after 6 months

SLIDE 17

89.9 71.2 53.6

10 20 30 40 50 60 70 80 90 100 At start After 3 months After 6 months Mean value of AST (U/L)

Follow up intervals

SLIDE 18

2 cases = 8 % 23 cases = 92 % 4 cases = 16 % 21 cases = 84 % 12 cases = 48 % 13 cases = 52 % 5 10 15 20 25

≤ 40 (U/L) > 40 (U/L) Cases representation

AST level ranges at start after 3 months after 6 months

SLIDE 19

3.8 4.1 4.2

3.5 3.6 3.7 3.8 3.9 4 4.1 4.2 4.3

At start After 3 months After 6 months

Mean value of Alb (g/100 ml)

Follow up intervals

SLIDE 20

1.8 1.7 1.4

0.2 0.4 0.6 0.8 1 1.2 1.4 1.6 1.8 2

At start After 3 months After 6 months

Mean value of Bili-T (mg/100 ml)

Follow up intervals

SLIDE 21

0.55 0.49 0.41

0.1 0.2 0.3 0.4 0.5 0.6

At start After 3 months After 6 months

Mean value of Creatinine (mg/100 ml)

Follow up intervals

SLIDE 22

26.2 23.2 17.1

5 10 15 20 25 30

At start After 3 months After 6 months

Mean value of Urea (mg/100 ml)

Follow up intervals

SLIDE 23

3787 2954 2547

500 1000 1500 2000 2500 3000 3500 4000

At start After 3 months After 6 months

Mean value of Ferritin (ng/L)

Follow up intervals

SLIDE 24

14 cases = 56 % 11 cases = 44 % 1 case = 4 % 17 cases = 68 % 7 cases = 28 % 2 cases = 8 % 16 cases = 64 % 7 cases = 28 %

2 4 6 8 10 12 14 16 18 20

< 1000 (ng/L) 1000 - 2500 (ng/L) > 2500 (ng/L) Cases representation

Feritin level ranges

At start After 3 months After 6 months

SLIDE 25

7.9 8.6 9.5

1 2 3 4 5 6 7 8 9 10

At start After 3 months After 6 months

Mean value of Hg (g/100 ml) Follow up intervals

SLIDE 26

23 cases = 92 % 2 cases = 4 % 19 cases = 76 % 6 cases = 24 % 10 cases = 40 % 15 cases = 60 %

5 10 15 20 25

≤ 9 (g/100 ml) > 9 (g/100 ml)

Cases representation Haemoglobin level ranges At start After 3 months After 6 months

SLIDE 27

3.1 3.6 3.7

0.5 1 1.5 2 2.5 3 3.5 4

At start After 3 months After 6 months

Mean value of RBCs x 106 (cells/µL) Follow up intervals

SLIDE 28

11 cases = 44 % 14 cases = 56 % 8 cases = 32 % 17 cases = 68 % 3 cases = 12 % 22 cases = 88 %

5 10 15 20 25

≤ 3000000 (cells/µL) > 3000000 (cells/µL)

Cases representation Red blood cells count ranges At start After 3 months After 6 months

SLIDE 29

10.1 15.4 14.5

2 4 6 8 10 12 14 16 18

At start After 3 months After 6 months

Mean value of total leukocytes x 103 (cells/µL) Follow up intervals

SLIDE 30

21 33 18 30

5 10 15 20 25 30 35 40

At start After 6 months

Mean value of CD4/CD8 absolute counts (x1000 cells/100ml) Follow up intervals CD4 CD8

SLIDE 31

352300 67480 57900

50000 100000 150000 200000 250000 300000 350000 400000

At start After 3 months After 6 months

Mean value of HCV count (IU/ml) Follow up intervals

SLIDE 32

17 cases = 68 % 6 cases = 24 % 2 cases = 8 % 2 cases = 8 % 18 cases = 72 % 5 cases = 20 % 3 cases = 12 % 18 cases = 72 % 4 cases = 16 %

2 4 6 8 10 12 14 16 18 20

Normal = 0.0 (IU/ml) Moderate < 100000 (IU/ml) High = 100000 – 1000000 (IU/ml) Very High > 1000000 (IU/ml)

No. of cases

HCV level ranges At start After 3 months After 6 months

SLIDE 33

17 cases = 68 % 8 cases = 32 % 6 cases = 24 % 19 cases = 76 %

5 10 15 20 25

≤ 15 days > 15 days

Cases representation Blood transfusion intervals At start After 6 months

SLIDE 34

The studied parameters gave significantly improved LIVER FUNCUTIONS values, such as the lowering of ALT, and AST less than 40 U/L in 8 cases, and 12 cases, respectively.

SLIDE 35

Ferritin, hemoglobin, and RBCs count possessed a significant improvement, as 16 cases recorded ferritin values between 1000-2500 ng/L at the end of treatment; 15 cases recorded hemoglobin values more than 9 g/100 ml; and 22 cases

ut of 25 total treated cases

recorded RBCs count more than 3 x 106 cells/µL after 6 months of treatment.

SLIDE 36

The main parameters of healing for these complicated cases were the promising

appearance of 3 cases with – ve (0.0 IU/ml) HCV count in patient serum by real time PCR and the prolonged blood transfusion intervals more than 15 days in 19 cases after 6 months of treatment with Spirulina.

SLIDE 37

This work should be extended on a wider scale of patients with more complete follow up for longer time to throw more light on the prognostic significance of Spirulina on HCV patients and Thalassemic children

SLIDE 38

Thalassemia

two major forms of the disease, α and β are prevalent in discrete geographical clusters around the world.

Infection ection

Iron o

erload load

Bone defor

mities

Enlarged spleen ged spleen

Treatment

Spirulina

Is the common name for human and animal food supplements. Spirulina comes from two different species of Cyanobacterium: Arthrospira platensis, and Arthrospira maxima.

Spirulina contains an unusually high

To date, there are no drug interactions reported with Spirulina. The United Nations Organization (UNO) has recommended Spirulina as the ideal food and the World Health Organization (WHO) has also declared Spirulina as a safe food with excellent nutritional value

Aim of the Work

This study will aim to evaluate the effect of the Cyanobacterium Spirulina

who are infected with Hepatitis C virus.

The studied parameters gave significantly improved LIVER FUNCUTIONS values, such as the lowering of ALT, and AST less than 40 U/L in 8 cases, and 12 cases, respectively.

Ferritin, hemoglobin, and RBCs count possessed a significant improvement, as 16 cases recorded ferritin values between 1000-2500 ng/L at the end of treatment; 15 cases recorded hemoglobin values more than 9 g/100 ml; and 22 cases

recorded RBCs count more than 3 x 106 cells/µL after 6 months of treatment.

The main parameters of healing for these complicated cases were the promising

appearance of 3 cases with – ve (0.0 IU/ml) HCV count in patient serum by real time PCR and the prolonged blood transfusion intervals more than 15 days in 19 cases after 6 months of treatment with Spirulina.

This work should be extended on a wider scale of patients with more complete follow up for longer time to throw more light on the prognostic significance of Spirulina on HCV patients and Thalassemic children

TH THANK ANK YO YOU