The Rare HaJiRu

Bunga Harum Sundal malam

2009-08-21T01:42:00.012+08:00

Nama Tempatan:
Sundal malam
Nama Saintifik:
Polianthes tuberosa
Nama Lain:
tuberose
Famili:
Amaryllidaceae/Agavaceae
Lokasi dijumpai:
Asal:
keterangan:
Bunga sundal malam atau dikenali juga dengan nama tuberose berasal dari Amerika tengah. Nama saintifiknya ialah Polianthes tuberosa, tergolong dalam famili Amaryllidaceae/Agavaceae , sudal malam ialah jenis pokok berumpun yang dikategorikan sebagai lili sama seperti amaryllis dan pokok spider lily atai bakung.kebanyakan bunga yang berbau wangi akan kehilangan atau berkurangan bau wanginya selepas dipetik dari tangkainya, namun sundal malam mempunyai sifatnya berbeza.Sama seperti bunga melur, walaupun setelah dipetik, bunganya sendiri masih lagi menghasilkan bau wangi yang akan terus kekal hingga ianya kering dan layu.sesetengah ahli botani mengklaskan tumbuhan ini sebahagai tumbuhan semusim(annual) manakala yang lainya pula mengkelaskannya sebagai tumbuhan yang kekal lama (perennials).dalam keadaan yang optimum tumbuhan ini boleh kekal hidup dan menghasilkan bunga hingga bertahun-tahun.Sama seperti tumbuhan lili yang lain sundal malam membiak dengan ubi atau bebawang. bebawangnya boleh disimpan lama sebelum ditanam jika dikeringkan.Tumbuhan ini memerlukan pendedahan terus pada cahaya matahari.
kegunaan:
sundal malam digunakan diindia sebagai bunga untuk menumbulkan mood romantik terutamanya untuk pengantin baru.di india juga gadis2 yang belum berkahwin dilarang dari mencium harumannya pada waktu malam kerana dikhuatiri ini akan merangsang aktiviti seksual mereka.di sini juga bunga ini dikenali dengan nama ratkirani yang bermaksud mistress of the night.Ia dikatakan dapat menambah dan meransangkan tenaga chakra yang boleh membuatkan seseorang itu mempunyai kuasa psikik (tidak diketahui sejauh mana kebenarannya).Minyak pati dari bunga ini sangat mahal , ada tempat yang menjualnya dengan harga USD100 untuk 5ml dan digunakan untuk pemulihan emosi, ia mendatangkan ketenangan hati dan perasaan.Bunga ini banyak ditanam secara kormisial di mexico , hawaii , Us.

There are Two Type of Thalassemia

2009-08-20T02:41:00.003+08:00

Alpha Thalassemia

The alpha thalassemias are caused by a decrease in production of alpha globin chains due to a deletion or mutation of one or more of the four alpha globin genes located on chromosome 16. Alpha gene mapping can be obtained to determine the specific mutation. The alpha thalassemias can be generally categorized as: Silent Carrier, Alpha Thalassemia Trait, Hemoglobin H disease, Hemoglobin H-Constant Spring, and Alpha Thalassemia major. Frequently, the diagnosis of alpha thalassemia trait in a parent is discovered after the birth of an affected child.

The Silent Carrier status is characterized by three functional genes that code for the production of alpha globins (-a/aa). Outside the newborn period, it is not possible to make this diagnosis by conventional methods. There is overlap between the red blood cell indices of these individuals and normals, although the MCV may be slightly lower. The silent carrier will experience no health problems in his/her lifetime. This carrier state is diagnosed by deduction when a 'normal' individual has a child with Hgb H disease or with microcytic anemia consistent with alpha thalassemia trait. An unusual case of the silent carrier state is the individual who carries the Hemoglobin Constant Spring mutation [(a^csa/aa) or (aa^cs/aa)]. This is an elongated a-globin due to a termination codon mutation. Individuals who have this mutation have normal red blood cell indices, but can have children who have Hgb H-Constant Spring disease if the other parent has alpha thalassemia trait (--/aa). Generally, children with Hgb H-Constant Spring are more affected clinically than children who have classic Hgb H disease. Two Constant Spring carriers can also pass on their genes to have a child with Homozygous Constant Spring, a condition that has similar clinical implications as Hemoglobin H disease.

Alpha thalassemia trait is characterized by two functional genes that code for the production of alpha globins [(-a/-a) or (--/aa)]. The two genes can either occur on the same chromosome (cis-type) or on each of the pair (trans-type). Cis-type a-thalassemia trait tends to be found in individuals of Asian descent, while trans-type tends to run in individuals of African descent. Cis-type can be co-inherited with another cis-type or hemoglobin H disease to result in alpha thalassemia major, or hydrops fetalis. Individuals who have alpha thalassemia trait are identified by microcytosis,erythrocytosis, hypochromia, and mild anemia. The diagnosis is made by a combination of family studies and the ruling out of both iron deficiency anemia and beta thalassemia trait. In the neonatal period, when hemoglobin Bart's (g⁴) is present, the diagnosis can be strongly suspected. In children, there are no markers such as Hgb A₂ and Hgb F to make the diagnosis. (One exception is the case where both of the deletions occur on the same chromosome and zeta [z] globin is expressed in carriers. This is most common in Southeast Asians.) The diagnosis is one of exclusion. The clinician should be satisfied with the presumed diagnosis if the above criteria are met. During pregnancy, the microcytic anemia can be mistaken for anemia of pregnancy.

The individual with a thalassemia trait will experience no significant health problems except a possible slight anemia which cannot be treated with iron.

Hemoglobin H disease is characterized by one functional gene that codes for the production of alpha globins (--/-a). Hgb H disease should be considered in the case of a neonate in whom all of the red blood cells are very hypochromic. These neonates have a high percentage of hemoglobin Bart's on the their newborn screening results. In older children, this hemoglobinopathy is characterized by moderate anemia with a hemoglobin in the 8 to 10 gm/dL range, hypochromia, microcytosis, red cell fragmentation, and a fast migrating hemoglobin (Hgb H) on electrophoresis.

Hemoglobin H does not function as a normal hemoglobin and has a high oxygen affinity(holds onto oxygen longer making it unavaible for use by the body), so the measured hemoglobin in these children is misleading. Individuals who have Hgb H generally have a persistent stable state of anemia, which may be accentuated by increased hemolysis during viral infections and by exposure to oxidant medications, chemicals and foods such as sulfa drugs, benzene, and fava beans (similar to individuals who have G6PD deficiency). As the red cells mature they loose their ability to withstand oxidant stress and Hgb H precipitates, leading to hemolysis. Therapy for individuals who have Hgb H disease includes folate, avoidance of oxidant drugs and foods, genetic counseling education and frequent medical care. Uncommon occurrences in a child with Hgb H would be severe anemia, cholelithiasis, skin ulceration, and splenomegaly requiring splenectomy. Unlike individuals who have beta thalassemia, hemosiderosis is rare in Hgb H disease.

Children with Hemoglobin H-Constant Spring (--/a^csa) have a more severe course than children who have Hgb H. They have a more severe anemia, with a steady state hemoglobin ranging between 7 and 8 gm/dl. They more frequently have splenomegaly and severe anemia with febrile illnesses and viral infections, often requiring transfusion. If anemia is chronically severe and the child has splenomegaly, a splenectomy may be performed. If splenectomy is anticipated, the complication of severe post-splenectomythrombocytosis with hypercoagulability can occur, leading to thrombosis of the splenic vein or hepatic veins. This complication has also been reported as recurrent pulmonary emboli and clotting diathesis. At CHO, children who are scheduled to have surgery are treated pre-surgically with low molecular weight heparin, followed by low dose aspirin, continued indefinitely.

The most severe form of alpha thalassemia is Alpha Thalassemia Major or hydrops fetalis, characterized by a deletion of all four genes that code for alpha globins (--/--). This diagnosis is frequently made in the last months of pregnancy when fetal ultrasound indicates a hydropic fetus. The mother frequently exhibits toxemia and can develop severe postpartum hemorrhage. These infants are usually stillborn. There can be other congenital anomalies, though none are pathognomonic for alpha thalassemia major. Since alpha globins are required for production of fetal and adult hemoglobin, the fetus suffers from signifiant in utero hypoxia. The only hemoglobins found in these infants are: Hgb Portland (d²g²), Hgb H (b⁴), and Hgb Bart's (g⁴), and no Hgb A or A₂. These babies can have other complications associated with hydrops, such as heart failure andpulmonary edema.

If the diagnosis is made early, intrauterine transfusions can be performed. There are reports of survival and chronic transfusion in these infants; CHO cares for one alpha thalassemia major baby with chronic transfusion therapy. Undoubtedly, more of these infants could be saved if the diagnosis is anticipated by prenatal diagnosis and treatment provided.

Beta Thalassemia

There are hundreds of mutations within the beta globin gene, but approximately 20 different alleles comprise 80% of the mutations found world wide. Within each geographic population there are unique mutations. Individuals who have beta thalassemia major are usually homozygous for one of the common mutations, or heterozygous for one of the common mutations and one of the geographically-unique mutations. Both lead to absence of beta globin chain production.

The beta thalassemia syndromes are much more diverse than the alpha thalassemia syndromes due to the diversity of the mutations that produce the defects in the beta globin gene. Unlike the deletions that constitute most of the alpha thalassemia syndromes, beta thalassemias are caused by mutations on chromosome 11 that affect all aspects of beta globin production: transcription, translation, and the stability of the beta globin product. Most hematologists feel there are three general categories of beta thalassemia: beta thalassemia trait, beta thalassemia intermedia and beta thalassemia major.

Splice site mutations also occur and are of clinical consequence, when combined with a thalassemia mutation. Three splice site mutations occur in exon 1 of the beta globin gene. These mutations result in three different abnormal hemoglobins: Malay, E, and Knossos. Hemoglobin E is a very common abnormal hemoglobin in the Southeast Asian population, and when paired with a b⁰ thalassemia mutation, can produce severe transfusion-dependent (Eb⁰) thalassemia. Hemoglobin E is described in the next section on variant hemoglobins.

Individuals who have Beta thalassemia trait have microcytosis and hypochromia; there may be targetting, elliptocytosis, though some individuals have an almost normal smear. Hemoglobins A₂ and F will be elevated on hemogram results. These hematologic features can be accentuated in women with trait who are pregnant and in individuals who are folate or iron deficient. If iron deficiency is concurrent with beta thalassemia trait there may be a normal Hgb A₂. Iron deficiency causes decreased hemoglobin production, and folate or vitamin B₁₂ deficiency can lead to megaloblastic anemia with increased Hgb A₂. Both of these deficiencies need to treated prior to evaluation for thalassemia trait. In iron, B₁₂, and folate replete individuals, the Hgb A₂ can be as high as 3.5 to 8% and the Hgb F as high as 1 to 5%. Generally, beta thalassemia trait is milder in African-Americans (who frequently have a promoter gene mutation) but has a similar presentation in individuals of Chinese, Southeast Asian, Greek, Italian, and Middle Eastern heritage.

Infants born in 42 of the 50 states in the United States with newborn screening programs will be diagnosed as having a hemoglobin disorder. In states without newborn screening for hemoglobinopathies and in recent immigrants to this country, affected children are frequently identified outside the newborn period, and the evaluation of their microcytic anemia includes differentiation between iron deficiency and beta thalassemia trait. The red blood cell indices can be helpful in this differentiation, as the hemoglobin concentration and the red cell count will generally be lower in iron deficiency. The distinguishing finding in beta thalassemia is a hemoglobin electrophoresis with the finding of elevated Hgb A₂ and F. Both will be increased in beta thalassemia trait without iron deficiency, and will be normal or decreased in alpha thalassemia and isolated iron deficiency anemia. There are several formulas to help in office screening, but they are also based on the assumption that the child is not iron deficient. Usually iron deficiency can be ruled out using free erythrocyte protoporphyrin (FEP), transferrin saturation or ferritin as a screening test in children who have a hypochromic microcytic anemia. The least expensive test is a trial of iron and a repeated hemogram after a month. A lead level should be obtained if there is an index of suspicion for lead toxicity.

Diagnostic challenges can still arise: if both alpha and beta thalassemia coexist, the changes in Hgb A₂ and F will not be apparent, and as noted above, there are instances of normal or elevated levels of Hgb A₂ and F in beta thalassemia trait. Family studies and, if warranted, DNA analysis can be used to make a definitive diagnosis.

Children who are diagnosed with Thalassemia Intermedia have a homozygous orheterozygous beta globin mutation that causes a decrease in beta chain production, but not to the degree that chronic transfusion therapy is required. The phenotype can also occur in children who have a mutation that increases production of c-globin, in children who have co-inherited alpha thalassemia and beta thalassemia, and in other rarer mutations. Children who have thalassemia intermedia are able to maintain a hemoglobin of 7 gm/dl or slightly higher with a greatly expanded erythron and may manifest bony deformities, pathologic fractures and growth retardation. Children who have thalassemia intermedia can also have delayed pubescence, exercise intolerance, leg ulcers, inflammatory arthritis and extramedullary hematopoiesis causing spinal cord compression, a medical emergency requiring radiation therapy and transfusion. They can also have iron overload due to increased absorption of iron from the gastrointestinal tract and intermittent transfusion. They are at risk for the cardiac and endocrine complications of hemosiderosis, but usually at an older age than chronically transfused children. Chelation therapy is indicated for increasing ferritin and elevated liver iron.

Children who can not maintain a hemoglobin between 6 and 7 gm/dl should have an alternative diagnosis considered. If thalassemia is the cause of the anemia, transfusion and/or splenectomy should be considered. Frequently, adolescents and adults are unable to tolerate the degree of anemia that is seen in thalassemia intermedia.Hypersplenism, splenic pain, congestive heart failure secondary to anemia, severe exercise intolerance, thrombocytopenia and leukopenia should be considered indications for beginning transfusion therapy or for splenectomy in the child who has severe hemolytic anemia.

Beta thalassemia major was first described by a Detroit pediatrician, Thomas Cooley, in 1925. The clinical picture he described is prevalent today in countries without the necessary resources to provide patients with chronic transfusions and desferoxamine therapy. Children who have untreated thalassemia major have ineffective erythropoiesis, decreased red cell deformability, and enhanced clearance of defective red cells by macrophages (immune system cells). The result is a very hypermetabolic bone marrow with thrombocytosis, leukocytosis and microcytic anemia in the young child prior to the enlargement of their spleen. At presentation they have almost 100% percent Hgb F (these cells have a longer life span due to a balanced globin ratio, as c rather than b, globin is present Hgb F). These children have little or no Hgb A₂ and a low reticulocytecount. The diagnosis can be confirmed by demonstrating thalassemia trait in both parents, by globin biosynthetic ratios, or by beta gene screening. Beta gene screening identifies the most common and some uncommon mutations, but not all mutations. An electrophoresis showing only Hgb F, a complete blood count and a smear will generally be diagnostic. In most states, these children will be discovered by state screening or occasionally by the obstetrician who makes a diagnosis of thalassemia trait in the mother and obtains a family history of thalassemia or anemia in both parents prior to the birth of the baby.

Children who have untreated thalassemia generally die in the first decade of life from anemia and septicemia, and may suffer from pathologic fractures. When palliative transfusions are introduced, children live into their late teens, but eventually succumb to heart failure if iron overload is not treated. But with the introduction of frequent chronic transfusion therapy and the use of subcutaneous desferoxamine, children are now surviving into adulthood. The longevity of patients who are compliant with their Desferal therapy or who have received bone marrow transplantation is not known.

Calcium and Thalassemia: What can be done to prevent problems related to bone strength and fractures?

2009-08-20T02:31:00.001+08:00

The major problems can be delayed or prevented by the adequate use of Desferal to prevent damage to the ovaries and testes by iron. In addition, everyone, including children and young adults with either transfusion dependent or non-transfusion dependent thalassemia, should have an adequate intake of calcium, either by eating foods high in calcium or by taking calcium supplements. Exercise in moderate amounts also increases bone strength. It is hard to determine how much calcium is in your diet without knowing how much calcium is contained in foods. At an annual comprehensive clinic visit, each patient can visit a nutritionist to help him or her determine whether enough calcium is coming from the diet. Most people need about 1.5 grams of calcium daily to build strong bones. Children need less: about 1 gram per day. Adolescents need more during this time of increased bone growth: 1.5 to 2 grams per day. Adults need about 1.5 grams per day. These levels of calcium can be achieved by eating foods high in calcium:

High calcium foods
(one serving = 400 mg or .400 g of calcium)

One cup of milk
yogurt
milk based pudding or custard
6 sardines with bones

Medium calcium foods
one serving = 125 mg or .125 g of calcium)

One cup tofu
One cup of beans* or peas
One cup broccoli, kale, mustard greens*
One half cup of bok choy or turnip greens*
One half cup cottage cheese, frozen yogurt, cream soup, or ice cream
One quarter cup almonds
2 ounces canned fish with bones

Remember that if you are on a low-iron diet, try to find alternate sources of calcium, as these tend to be high in iron as well

Many foods are fortified with calcium: orange juice, breads, soy milk. Look on the labels for both the iron content (avoid iron) and the calcium content of prepared foods. Also important is vitamin C for calcium absorption. If you think about calcium and how important it is for your health, you can easily increase the calcium you receive everyday. If you want more information about your diet, call your health care provider or a nutritionist for a consultation.

Foods to Avoid for Thalassemia Patient

2009-08-20T02:08:00.000+08:00

Low Iron Diet for the Chronically-Transfused Patient

Very high iron sources are found in the following foods; they should be avoided or eliminated from the diet:

Proteins that should be avoided or eliminated from the diet:

oysters
liver
pork
beans
beef
peanut butter
tofu

Grains that should be avoided or eliminated from the diet:

flour tortillas
infant cereal
cream of wheat
Malt-O-Meal
cereals, such as Most, Product 19, Total, Kix, All Bran, Life, Raisin Bran, Special K, 100% Bran, Rice Chex, Rice Krispies, Cornflakes, Wheaties

Fruits/Vegetables that should be avoided or eliminated from the diet:

prune juice
prunes
watermelon
spinach
leafy green vegetables
dates
raisins
broccoli
peas
Fava beans

Nutrition & Diet for Thalassemia Patient

2009-08-20T01:59:00.001+08:00

Diet for the Non-Transfused Patient

Non-transfused thalassemia intermedia patients are encouraged to avoid high-iron and iron-supplemented foods, and encouraged to drink tea with meals, which decreases iron absorption. Serum ferritin is evaluated in adolescents. Desferrioxamine is instituted early in the development of hemosiderosis. Iron overloaded individuals receive a liver biopsy. Early cardiac evaluation with Holter monitoring and stress ECHO cardiogram is done in individuals with significant hemosiderosis. In addition, folic acid deficiency appears to be more common in these individuals.

Low Iron Diet for the Chronically-Transfused Patient

Regular blood transfusions can lead to iron overload in the body. Extra iron from chronic transfusions is stored in the liver. Once the liver stores are full, the iron begins to accumulate in places like the heart and pituitary, where it can do damage. Iron overload can also result from increased absorption of iron from the gut, as can be the case with thalassemia intermedia.
To help keep the iron stores from building up too fast, a medication called Desferal will be used in conjunction with a low iron diet. Keep the iron under 10 mg/day for those children under 10 years old, and under 18 mg/day for those who are 11 years old and older.

Children who have thalassemia and are transfused are still relatively anemic, so their bodies might still crave iron. As it may be difficult to watch their diets closely, they should develop good habits early. Remind children to definitely avoid very high iron foods such as dried beef and other high iron beef products, even if they are craving it. Remember that the iron found in meat is much more easliy absorbed than other sources of iron, such as cereals and breads.

Do not cook with cast iron cookware (e.g. a wok) because iron from the cookware can transfer onto the food. Some foods, such as orange juice, can enhance iron absorption, while others, like tea, dairy and coffee, can decrease absorption. If you are using Desferal, however, it is recommended that you take 250 mg or less of vitamin C after beginning infusion to help increase output of iron.

On food labels, the percentage of iron in one serving of that food is usually listed. This is based on the U.S. Recommended Daily Allowance of 18 mg/day. If the label says the food contains 8% of the daily recommended iron, multiply .08 by 18mg to get the mg iron from a serving of that food. Don't forget to check candy bars and snacks!

Thalassemia

2009-08-20T01:49:00.002+08:00

Also called: Cooley's anemia, Mediterranean anemia

If you have thalassemia, your body has problems making hemoglobin, the protein in red blood cells that carries oxygen through your body. When your blood does not carry enough oxygen to the rest of your body, you have anemia.

Thalassemia, a genetic disease, can be mild or severe. Some carriers of the gene have no symptoms. The most common severe form in the United States is a type called Cooley's anemia. It mainly affects people of Mediterranean or Asian ancestry. It usually appears during the first two years of life. Severe thalassemia is treated with blood transfusions and treatment to remove excess iron in the blood.

Yasmin Ahmad

2009-08-18T04:23:00.005+08:00

al-fatihah,

buat Yasmin Ahmad semoga roh beliau dicucuri rahmat

dan ditempatkan di

kalangan orang2 yang beriman..

Hasil kerja beliau memang hebat dan memukau...

bak kata orang memang berkualiti tinggi...

Nama Tempatan:	Sundal malam
Nama Saintifik:	Polianthes tuberosa
Nama Lain:	tuberose
Famili:	Amaryllidaceae/Agavaceae
Lokasi dijumpai:
Asal:

keterangan:
Bunga sundal malam atau dikenali juga dengan nama tuberose berasal dari Amerika tengah. Nama saintifiknya ialah Polianthes tuberosa, tergolong dalam famili Amaryllidaceae/Agavaceae , sudal malam ialah jenis pokok berumpun yang dikategorikan sebagai lili sama seperti amaryllis dan pokok spider lily atai bakung.kebanyakan bunga yang berbau wangi akan kehilangan atau berkurangan bau wanginya selepas dipetik dari tangkainya, namun sundal malam mempunyai sifatnya berbeza.Sama seperti bunga melur, walaupun setelah dipetik, bunganya sendiri masih lagi menghasilkan bau wangi yang akan terus kekal hingga ianya kering dan layu.sesetengah ahli botani mengklaskan tumbuhan ini sebahagai tumbuhan semusim(annual) manakala yang lainya pula mengkelaskannya sebagai tumbuhan yang kekal lama (perennials).dalam keadaan yang optimum tumbuhan ini boleh kekal hidup dan menghasilkan bunga hingga bertahun-tahun.Sama seperti tumbuhan lili yang lain sundal malam membiak dengan ubi atau bebawang. bebawangnya boleh disimpan lama sebelum ditanam jika dikeringkan.Tumbuhan ini memerlukan pendedahan terus pada cahaya matahari.
kegunaan:
sundal malam digunakan diindia sebagai bunga untuk menumbulkan mood romantik terutamanya untuk pengantin baru.di india juga gadis2 yang belum berkahwin dilarang dari mencium harumannya pada waktu malam kerana dikhuatiri ini akan merangsang aktiviti seksual mereka.di sini juga bunga ini dikenali dengan nama ratkirani yang bermaksud mistress of the night.Ia dikatakan dapat menambah dan meransangkan tenaga chakra yang boleh membuatkan seseorang itu mempunyai kuasa psikik (tidak diketahui sejauh mana kebenarannya).Minyak pati dari bunga ini sangat mahal , ada tempat yang menjualnya dengan harga USD100 untuk 5ml dan digunakan untuk pemulihan emosi, ia mendatangkan ketenangan hati dan perasaan.Bunga ini banyak ditanam secara kormisial di mexico , hawaii , Us.