Hemoglobinopties are disorders of blood cell structure and they may occur in many different ethnic groups, such as African, Asian, Mediterranean or Middle Eastern decent. There are two major types of Hemoglobinopaties:
1) Thalassemias : which are caused by quantitative deficiencies in the in the production of globulin chains which are used to produce hemoglobin. These defects generally produce anemia of varying degrees depending on severity.
2) Structural abnormalities of globin chain production causing a defect in the shape of the blood cells. Examples of this type of anemia would be an illness called Sickle Cell disease.
In this article we will talk about the Thalsemia syndromes and in particular about Hemoglobin H disease. In order for the human body to make normal hemoglobin, which is required to transport oxygen to all our vital organs, normal cells must have 4 alpha globin genes. In the Thalasemia disorders, there is a genetic defect or deletion in one to four of these alpha-globin genes on a chromosome #16. The severity of the syndrome is related to the number of gene deletions.
A) Silent Carrier: has one gene deletion. They are asymptomatic and have normal blood findings.
B) Alpha-Thalsemia Trait: 2 gene deletions, they have normal or slightly decreased hemoglobin (blood levels) causing a mild anemia.
C) Hemoglobin H disease: 3 gene deletions. They have mild to moderately severe anemia.
D) Hydrops fetalis: 4 gene deletions. They have severe intrauterine anemia, asphyxia, and usually expire shortly after birth.
blood In-patients with Hemoglobin H disease, or 3 gene deletions, these patents may be symptomatic and have secondary complications of hemolytic anemia. Patients with HbH disease can generally have moderate to severe anemia, with low hemoglobin levels of 7-10g/dl. Other common medical finding would be paleness, jaundice (or yellowness) large liver and spleen, increased susceptibility to infections, leg ulcers and pigment gallstones which occur after prolonged breakdown of red blood cells, can cause recurrent abdominal pain and may require surgery. Deficiency in Folic acid may also occur.
Complications that can occur in children with HbH disease are generally the result of intermittent exacerbation of their anemia, which may require repeated blood transfusions. These episodes of hemolytic anemia generally are precipitated by certain drugs or by a parvovirus infection. Usually patients with HbH disease can lead fairly normal lives with relatively few blood transfusions. Transfusion therapy is usually reserved for patients with severe anemia (usually less than 7g/dl) and with symptomatic anemia.
Another complication of this disease is the enlargement of the spleen with worsening of the anemia. Many times this requires the removal of the spleen itself, and then you have all the complications other than just that of surgery, which is the susceptibility to bacterial infections. Usually removal of the spleen is reserved for patients with symptoms of large spleen, as reflected by leukopenia (decreased white blood cell levels) thrombocytopenia (decreased platelet count) and worsening anemia or, in-patients who were previously stable and develop blood transfusion requirement.
What does the medical future of this child have in store for this adoptive family?
1) Usually patients with HbH disease can lead relatively normal lives.
2) They may require repeated blood transfusions, and after many years of therapy may result in a complication of iron overload.
3) Large spleen which may require its removal.
4) Susceptibility to bacterial infection after the removal of the spleen.
5) Many children are kept on prophylactic penicillin therapy to ward off infection.
6) Severe hemolytic anemia episodes which may easily be triggered in times of stress, infections with viral illness or may even by certain types of medications such as sulfur.
7) Gallstones and abdominal pain all secondary to pigment stone collection caused by prolonged destruction of red blood cells. Surgical removal of gallstones to alleviate the abdominal pain.
Now back to the original question that the parents say they are not carriers and are not ill. I believe that they are not ill but are more than likely both silent carriers of the disease or have the thalsemia trait or some combination of this. Because of the rules in genetics, it is possible to have three other children that are not ill with the severe form of the disease, but they may also be silent carriers or have the thalasemia trait. From the clinical description of this child's medical scenario, the diagnosis may be accurate. Good follow-up with a Pediatric Hematologist is essential to the management of this disease
Note: The information and advice provided is intended to be general information, NOT as advice on how to deal with a particular child's situation and or problem. If your child has a specific problem you need to ask your pediatrician about it -- only after a careful history and physical exam can a medical diagnosis and/or treatment plan be made.
This website does not constitute a physician patient relationship
Thalasemia
8:25 AM Diposkan oleh arfa
Article Source: International Adoption Articles Directory
Obama's Indonesian Classmates: We Are Proud of You, Barry
10:33 PM Diposkan oleh arfa
Barack Obama: The 44th U.S. President
By Mulyanda Djohan
JAKARTA. Jan. 21 (Xinhua) -- Old friends and teachers of U.S. newly-elected President Barack Obama in Indonesia celebrated his inauguration on Wednesday, expressing happiness and pride of him.These people reunited in the primary school of SDN Menteng Besuki 01 Jakarta in down town area for their memory with Barry, the way they called small Obama at that time, and to celebrate the success of their friend.
Obama has made friends with his classmates and teachers in Indonesia, who know his personality and character years ago and put hopes and trust on him to bring peace to the world and an end to the global economic crisis.
Several sets of pictures showing small Obama studying in the school were displayed at the entrance of the school. The photos showed Obama, with short curly hair and a little bit fat and taller body, cheerfully sat among his classmates. It seems that Obama had been socially and culturally united with his Indonesian friends with various ethnic backgrounds.
With cheer and happiness, the attendees of the celebration enjoyed very much the moment which was enlivened by performance of music, singing and reading of the history of Obama's childhood in Jakarta.
Obama's tremendous career has let the people whom he knew and was acquainted with in the primary school in Jakarta admire him very much.
"For us, as friends of Barry, it has been a happiness, because we can not imagine that one of our classmates can become a president. We are proud of his achievement," said Bernaldi Hadi, one of Obama's old friends.
"To Obama, all your friends and teachers in primary school of SDN Menteng Jakarta congratulate for your success to become the U.S. president," said Tina Hayari, the head master of the primary school when Obama studied there.
During his four years in Indonesia, Obama made friends with various cultural and ethnic backgrounds, as the country is comprised of hundreds of ethnic groups, which then contributed to his personality.
In the photo display, Obama was seen among his friends wearing various colorful ethnic uniforms to celebrate the Kartini Day, an event to remember the heroic deeds of an Indonesian woman, named Kartini.
"Barry experienced such diversity," said Rully Saad, one of Obama's friends.
"He is easy to adapt, cares a lot with environment, and has a strong capability to learn new things," said Dewi Asara Oetojo, another Obama's friend.
Effendi, one of Obama's teachers, said that his pupil was easy going and had often raised questions during the class session.
"He was very polite with people around him, including his seniors. And he was enjoying raising questions to teachers," said Effendi, who goes with a single name like many Indonesians.
But, Effendi said that Obama was a little bit fond of teasing girl classmates.
"He has a good heart but often teased girls," said Effendi.
"Mrs. Ati Kisjanto's long hair used to be pulled by Barry," he said. Kisjanto was also present at the celebration.
Still, the teacher expected very mush that his brilliant student could terminate the war, violence and global recession as well as bring progress to the relations between Indonesia and the United States.
"I hope after Barry becomes president, he can create world peace, end the economic crisis and build a better diplomatic ties with Indonesia," said Effendi.
Ati Kisjanto, one of Obama's girl classmates, said that Obama's position has been a good bridge that should be well used by Indonesia.
"Obama is a good bridge as he understands more about the Indonesian culture. And we must catch this opportunity," she said.
The Indonesia-U.S. relation was once hampered by a human rights issue, as the U.S. forbid Indonesia to purchase spare parts of military planes due to poor human rights record before and after the referendum in Timor-Leste in 1999. However, it has started to recover.
Ahmad Solihin, vice principle of the SDN Menteng Jakarta Primary School, is optimistic that the new U.S. president could be better than his predecessors in addressing the global problems.
"I expect that Obama would be different from the previous presidents of the United States. He would be better than them," said Solihin.
On current issues of the Middle East conflict, the school's Principle Hashimah expected that Obama could stop such violence faster in the future.
"I hope Obama can take wise decision and actions as he used to live in Indonesia, a country with strong tolerance and understanding of each other," she said.
U.S. Ambassador to Indonesia Cameron R. Hume said that President Obama had learned social skills in the primary school.
"The importance for President Obama is the years he was here and the years he learned his social skills as much from the faculty and his friends in his class," he said.
Posted: www.chinaview.cn
Jakarta Biennale: City Gives a Little, Takes a Little
8:22 AM Diposkan oleh arfa
Evi Mariani, The Jakarta Post, Jakarta
How much does it cost to place art at public spaces?
The committee of this year Jakarta Biennale's Battle Zone has the answer: Hundreds of millions of rupiah, dozens of official letters, several trips to the city hall and a lot of headaches.
The cost was incurred even though the event was organized by the city's own Jakarta Arts Council and was included in the city budget and program.
One of the artists commissioned for the international art event, Ari Dina Krestyawan, wanted to run text around a crossing bridge on Jl. Thamrin about the people who used the space every day. The bridge has equipment to display text for advertising.
The Battle Zone committee asked for a time slot to use the equipment for Ari's project.
"We were first told by the billboard association that the time slots were being used by [a cellular phone provider]," Ardi Yunanto, the curator for the Battle Zone, told The Jakarta Post on Monday.
"But, the city administration has some slots there. We were led to the general bureau at the administration."
Three officers and several letters later, Ardi's team got nothing except an instruction to write more letters and wait longer.
"Time was running out. It was January and the art work needed to be on display early February. Finally, I told the artist to make an audio visual simulation about what would happen if we're allowed to run the texts there," Ardi said.
Battle Zone has brought artworks to several public spaces: Menteng Park in Central Jakarta; a wall along Jl. TB Simatupang toll road in South Jakarta; several roads in East Jakarta; four billboards in Central, West, and South Jakarta; two train stations; National Monument (Monas) Park in Central Jakarta; and the space under a flyover in Tebet, South Jakarta.
"The mural along the toll road and exhibitions at train stations received permits with relative ease.
"We met with an officer of PT Kereta Api at station city [in West Jakarta] and he gave us a permit for the exhibition at city and Tanah Abang stations," Ardi said.
"The toll road operator was also quick to give us a go ahead."
For the exhibition of Daniel Kampua's work at Monas, the committee only met twice before receiving the green light. Kampua could display his photo exhibition about Monas' mobile photographers without any charge.
Menteng Park was a whole different story.
Artist Cecil Mariani planned to have three billboards at three spots in the park. The billboards would bear pictures of Persija stadium, which previously stood at the park.
After several meetings and clinching the permit, the committee was asked to pay a fee of Rp 100,000 (US$8.9) per day for each board.
"That's on top of `wisdom' money asked for by the park attendants. One of them told me, `to ease closer ties between us here and your products', they wanted Rp 200,000 per day," said Ardi. The closer ties he was talking about included fixing the position of the board should they fall, Ardi said.
He said for each set of letters, his team attached a letter from the city's tourism and cultural agency, saying the event was a city program. But he said many officials from other agencies just ignored it.
Many of them said they would were complying with a gubernatorial decree, Ardi said.
Money wise, the four billboards cost Jakarta Biennale the most. The owners of the billboard, who happened to know some committee members, let the artists use them free of charge. The advertisement tax, however, was not waived.
"For each billboard, the tax is Rp 150 million. Meaning we should pay Rp 600 million for all. But we got 80 percent discount from the city's tax agency, so we were told to pay Rp 120 million," Ade Darmawan, Jakarta Biennale's program director said.
"The city administration gave us Rp 2 billion for the whole event. Our expenses reached Rp 3 billion," he said.
He said Governor Fauzi Bowo had verbally lent his support to Jakarta Biennale, citing the need to improve the city's creative industry.
In his speech read by his assistant for people welfare to city secretary Effendi Anas during the opening of the event on Feb. 6, Fauzi mentioned the relationship between the creative industry and potential tourism.
Head of the city's tourism and cultural agency, Arie Budhiman, recognized the need to support cultural events in Jakarta to increase tourist numbers.
"We are listing international events in Jakarta which will attract tourists -Jakarta's Java Jazz, Jakarta Fashion Week, Jakarta Great Sales, Jakarta Biennale as well as Jakarta International Film Festival," he said on Wednesday.
He said he believed such events would help increase the number of tourists to the city. Thus it was reasonable, he said, for the events to receive tax exemptions for publication, for example.
"But for total exemption, we haven't got the regulation yet, so we need a new bylaw. Right now, the most we can do is give tax discounts," Arie said.
"From the top officials, we get support," Ade said. "But it should become a policy, not only verbal support.
The policy should also be translated into operational rules.
"This year, Jakarta Biennale received international attention. We welcomed several international press members here. And arts, we have potential big spenders who are willing to fly to see the event," Ade said.
"And Indonesia, given our interesting social and political history, we are the next big thing among international arts events."
