English Summary
MITOCHONDIRIAL MYOPATHY
Santosa, Soenarto*, Suyanto Hadi**
Resident, *Professor, **Head of
Rheumatology subdivision, Dept. of
Internal Medicine, Faculty of Me-
dicine, Diponegoro University,
Semarang, Central Java, Indonesia
Mitochondria are unique or-
ganella because they have DNA in
self called mitochondria DNA with
specific characteristics.
The term mitochondria myo-
pathy refers to various syndromes
with diverse pathology, histo-
chemistry and biochemistry cha-
racteristics. These syndromes are
often multisystemic with varying
signs and symptoms affecting
many organ system; and were
under exotic names such as CPEO
(chronic progressive external
ophthalmoplegia), MELAS (mito-
chondrial encephalomyopathy,
lactic acidosis, and stroke-like
episodes), MERRF (myoclonic
epilepsy with ragged-red fibers),
MNGIE (myoneurogastrointestinal
encephalopathy), NARP (neuro-
genic weakness, ataxia, retinitis
pigmentosa).
The main function of mito-
chondria is to produce chemical
energy in the form of ATP molecule
that is used by body cells. When a
key component of respiration
chain is missing or defective, the
result is like the aftermath of a train
derailment. Mutation and deletion
of mitochondria DNA results many
mitochondrial syndromes.
The common approaches are
to give drug(s) that stimulate
enzymatic activity for transporting
electron or to give artificial elec-
tron acceptor. Gene therapy may
be used in the future.
Cermin Dunia Kedokt.2004; 147; 13-22
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CLINICAL EXPERIENCE ON HEART
TRANSPLANTATION
Yanto Sandy Tjang, Gero Tenderich,
Lech Hornik, Michiel Morshuis,
Kazutomo Minami, Richardus Budi-
man, Reiner Korfer
Dept. of Cardiovascular and Thora-
cic Surgery, Cardiac and Diabetes
Center, Nordrhein Westfalen/ Ruhr
Bochum University Teaching
Hospital, Bad Oeynhausen, Germany
I
mproved longevity, advances
in prevention, diagnosis and treat-
ment of cardiovascular diseases
have led to the rapidly growing
number of patients with heart
failure. The prevalence of heart
failure is increasing with age,
ranging from <1% in patients
under 50 years of age to 5% in
patients 50 to 70 years and 10% in
patients over 70 years of age.
Prognosis of chronic heart failure is
still very bad if the underlying
causes of disease are untreated.
Almost 50% of patients suffered
from chronic heart failure will die
within 4 years, whereas 50% of
end stage patients will die within 1
year. Despite different novel treat-
ment modalities either non
pharmacologic, pharmacologic
or surgical procedures which have
recently emerged, heart transplan-
tation is still well accepted as
treatment of choice for these
patients. On December 3
th
, 1967;
Christian Barnard successfully per-
formed the first human orthotopic
heart transplantation in South
Africa. This success was then rapid-
ly followed by other cardiac trans-
plant centers around the world.
The improvement in actuarial
survival following cardiac trans-
plantation is related not only to
better postoperative care but also
to improved patient selection.
Futhermore, the selection of donor
hearts for cardiac transplantation
also have impacts on success.
Since the initiation of the trans-
plantation programmes in the
Heart & Diabetes Center NRW in
Bad Oeynhausen, Germany on
March the 13
th
, 1989; 1406 ortho-
topic heart transplantation have
been successfully perfomed. The
actuarial survival rates range
between 80%, 69%, 54%, and
39% in the first, fifth, tenth and
fifteenth year respectively.
Cermin Dunia Kedokt.2004; 147; 41-4
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NITROGEN OXIDES ESSENTIAL OR
POLLUTANT FOR LIFE ?
Jansen Silalahi
Dept. of Pharmacies, Faculty of
Mathematics and Physical Sciences,
Sumatra Utara University, Medan,
North Sumatra, Indonesia
Nitrogen oxide is generated
from amino acid L-arginine by
nitric oxide synthase enzymes in
endothelial mammalian cells in-
cluding humans. It functions as
biological mediator allowing cells
to communicate each other.
Nitrogen oxide has an import-
ant role in controlling blood vessel
tone, blood flow and regulating
platelet function, gastrointestinal
motility, and reactivity of certain
airways as well as urinary bladder.
Nitrogen oxide also contributes to
host defense and pathophysio-
logical changes such as in life
threatening hypotension and also
might cause tissue damage. By
understanding the physiological
roles, new drug development and
therapeutic application may be
developed by selectively enhance
or inhibit the production of nitro-
gen oxide from nitrogen oxideargi-
nine pathway in biological system.
Cermin Dunia Kedokt.2004; 147; 36-40
jsi
Cermin Dunia Kedokteran No. 147, 2005
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