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

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 of abnormal hemoglobin molecules, thus causing anemia, the characteristic presenting symptom of the Thalassemia

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

 Inf Infection ection  Ir Iron o on over erload load  Bone def Bone defor ormities mities  Enlar Enlarged spleen ged spleen

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.

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.

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 oxymatrine (an extract from the sophora root) proponents to be helpful for hepatitis C

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 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.

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

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 or organ toxicity or genotoxicity.

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 sp. on thalassemic children, who are infected with Hepatitis C virus .

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.

120 94.3 100 Mean value of ALT (U/L) 74.1 80 56.9 60 40 20 0 At start After 3 months After 6 months Follow up intervals

25 at start 20 cases 18 cases = 80 % 17 cases after 3 months = 72 % 20 = 68 % after 6 months 15 Cases representation 8 cases 7 cases 10 = 32 % = 28 % 5 cases = 20 % 5 0 > 40 (U/L) ≤ 40 (U/L) ALT level ranges

100 89.9 90 71.2 80 Mean value of AST (U/L) 70 53.6 60 50 40 30 20 10 0 At start After 3 months After 6 months Follow up intervals

23 cases 25 21 cases = 92 % at start = 84 % after 3 months 20 after 6 months Cases representation 13 cases 12 cases 15 = 52 % = 48 % 10 4 cases = 16 % 2 cases 5 = 8 % 0 > 40 (U/L) ≤ 40 (U/L) AST level ranges

4.2 4.3 4.1 4.2 Mean value of Alb (g/100 ml) 4.1 4 3.8 3.9 3.8 3.7 3.6 3.5 At start After 3 months After 6 months Follow up intervals

2 1.8 1.7 Mean value of Bili-T (mg/100 ml) 1.8 1.6 1.4 1.4 1.2 1 0.8 0.6 0.4 0.2 0 At start After 3 months After 6 months Follow up intervals

0.6 0.55 0.49 0.5 Mean value of Creatinine 0.41 0.4 (mg/100 ml) 0.3 0.2 0.1 0 At start After 3 months After 6 months Follow up intervals

30 26.2 23.2 25 Mean value of Urea 17.1 20 (mg/100 ml) 15 10 5 0 At start After 3 months After 6 months Follow up intervals

3787 4000 Mean value of Ferritin (ng/L) 3500 2954 3000 2547 2500 2000 1500 1000 500 0 At start After 3 months After 6 months Follow up intervals

20 17 cases 16 cases = 68 % 18 At start = 64 % 14 cases 16 Cases representation After 3 months = 56 % 14 After 6 months 11 cases 12 = 44 % 7 cases 10 = 28 % 7 cases 8 = 28 % 6 2 cases 4 1 case = 8 % = 4 % 2 0 0 < 1000 (ng/L) 1000 - 2500 (ng/L) > 2500 (ng/L) Feritin level ranges

9.5 10 8.6 9 7.9 8 7 Mean value of Hg 6 (g/100 ml) 5 4 3 2 1 0 At start After 3 months After 6 months Follow up intervals

25 23 cases = 92 % At start 19 cases = 76 % After 3 months 20 Cases representation 15 cases After 6 months = 60 % 15 10 cases = 40 % 10 6 cases = 24 % 2 cases 5 = 4 % 0 ≤ 9 (g/100 ml) > 9 (g/100 ml) Haemoglobin level ranges

4 3.6 3.7 3.5 3.1 Mean value of RBCs x 106 3 2.5 (cells/µL) 2 1.5 1 0.5 0 At start After 3 months After 6 months Follow up intervals

25 22 cases = 88 % At start 17 cases After 3 months 20 = 68 % After 6 months Cases representation 14 cases = 56 % 15 11 cases = 44 % 8 cases 10 = 32 % 3 cases 5 = 12 % 0 ≤ 3000000 (cells/µL) > 3000000 (cells/µL) Red blood cells count ranges

18 15.4 Mean value of total leukocytes x 16 14.5 14 12 10.1 103 (cells/µL) 10 8 6 4 2 0 At start After 3 months After 6 months Follow up intervals

40 Mean value of CD4/CD8 absolute CD4 33 35 30 counts (x1000 cells/100ml) CD8 30 25 21 18 20 15 10 5 0 At start After 6 months Follow up intervals

400000 352300 Mean value of HCV count (IU/ml) 350000 300000 250000 200000 150000 67480 57900 100000 50000 0 At start After 3 months After 6 months Follow up intervals

20 18 cases 18 cases = 72 % 17 cases 18 At start = 72 % = 68 % 16 After 3 months No. of cases 14 After 6 months 12 10 5 cases = 20 % 6 cases 8 = 24 % 4 cases 6 3 cases = 16 % 2 cases = 12 % 2 cases 4 = 8 % = 8 % 2 0 0 0 0 Normal = 0.0 (IU/ml) Moderate < 100000 High = 100000 – Very High > 1000000 (IU/ml) 1000000 (IU/ml) (IU/ml) HCV level ranges

25 19 cases At start = 76 % 17 cases Cases representation 20 = 68 % After 6 months 15 8 cases 10 6 cases = 32 % = 24 % 5 0 ≤ 15 days > 15 days Blood transfusion intervals

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 out of 25 total treated 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

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