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Transcript of 2. Rds Ue Perlu 3
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Uremic Encephalopathy (UE)
Rusdidjas, Rafita Ramayati, Oke Rina dan Rosmayanti
Divisi Nefrologi Anak, Dept. Ilmu Kes. Anak FK-USU/ RSHAM
Medan
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INTRODUCTION / PENDAHULUAN
Uremia (= Urine in the blood) the final stage of progressive Renal failure the resultant multiorgan failure accumulating metabolites of proteins and amino acids and
concomitant failure of renal catabolic, metabolic, andendocrinologic processes
Uremic encephalopathy (UE) is one of many manifestationsof renal failure (RF).
Uremia: = ada urin dlm darah
Hasil akhir dari Renal failure
Merupakan resultante dari gagal MultiorganPenumpukan hasil Metab. Protein, Asam amino dan
Kegagalan klatabolik renal.
UE. Salah satu manifestasi Kegagalan ginjal
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AIMS of this paper:
Talking about:
Etiology
Patophysiology
Clinical features
Diagnosis Treatment
TUJUAN, membicarakan : Penyebab, Patofisiologi,Gejala Klinis, Diagnosis dan Pengobatan UE.
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UE
USUALLY due to
ARF (most difficult to treat / PALING SULIT DIOBATI)
CRF (Chronic Renal Failure)
ESRD (End Stage Renal Diseases= Gagal Ginjal Thp Akhir)
SEMUA RENAL FAILURE MENGSAHILKAN
-SISA-SISA METAB. PROTEIN mis UREUM, CREATININ,
URIC ACID, DLL SUBSTANS MEMBUAT KELAINANOTAK (= UE).
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Tabel 1. Beberapa substans lainnya sebagai Uremic Toxins,yang implikasinya merupakan toxisitas dari Uremic.1
---------------------------------------------------------------------------------1. Organic metabolit
Urea (cyanide)
CreatinineGuanethidine
Aliphatic aminesPhenol dan aromatic amineUric acid
Oxalic acidMyoinositol
2.3 Buthylene glycol2. Peptides dan Protein Degradasi productsMiddle moleculeAmono acidBeta1 mucroglubulines
3. EnzymesRenin
RibonucleaseLysozymes4. Hormones
Parathyroid hormoneGlucagonGrowth hormonesCalcitonineNariuretic hormones
---------------------------------------------------------------------------
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Neurological complications in renal failure
The incidence and severity of uremic encephalopathy,atherosclerosis, neuropathy and myopathy havedeclinedbut many patients fail to fully respond todialytic therapy.
Dialytic therapy or kidney transplantation induceneurological complications.
Insidens dan keparahan UE, atherosclerosis, Neuropati
dan Myopati, sudah menurun, tetapi banyak pasien yggagal dgn terapi dialysis.
Terapi dialysis dan Tranplantasi meng-induksi kom-plikasi neurologi.
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Pathophysiology of Uremic Encephalopathy..1
Complex and poorly understood 1. Accumulation of metabolites 2. Disturbance of the intermediary metabolism 3. Imbalance in excitatory and inhibitory neurotransmitters
4. Hormonal disturbance
PATOFISIOLOGI UE: komplek dan sedikit ygdiketahui.
1.Ada penumpukan hasil metabolisme 2.Ada gangguan dari intermediari metabolisme 3.Imbalans antara perangsangan dan penghambatan
neuro transmiter.4.Ada ganggaun Hormon
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Pathophysiology of uremic encephalopathy..2
.Accumulation of numerous organic substances, e.g. 5. Uremic neurotoxins ,urea, guanidino compounds, uric acid,
hippuric acid, various amino acids, polypeptides,polyamines, phenols
6. and conjugates of phenol, phenolic and indolic acids,
acetone, glucuronic acid, carnitine, myoinositol, sulphates,phosphates and middle molecules.
PENUMPUKAN Organik substans yg banyak:
5. Uremic neurotoxins, uric acid, hipurric acid, ber
bagai Amino acid, polypeptides, polyamines,phenol. dan
6. conjugate phenols, phenolic dan indolic acidacetone, glucoronic acid, carnitine, myoinositol,
sulfat, phosphate, dan molekul2 sedang.
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Pathophysiology of uremic encephalopathy..3
7. Guanidino compounds guanidinosuccinic acid, methylguanidine, guanidine andcreatinine : highly increased in serum, CSF and brain
Inhibited responses to GABA and glycine (inhibitory aminoacids)
Guanidinosuccinic acid : inhibits transketolase, a thiamine-dependent enzyme of the pentose phosphate pathway
Inhibition of transketolase : demyelinative changes to bothcentral and peripheral nervous system
Methylguanidine : seizures and uremic twitch-convulsivesyndrome
7. GUANIDINO COMPUNDS, meningkat dlm serum, penghambat GABA (Gamma Amino Butyric Acid) dan glycine
Penghambat jalur transketolase dan menghambatperobahan demyelinasi dari syaraf sentral dan perifer.Methylguanine kejang, twiching.
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Pathophysiology of uremic encephalopathy..4
8.Decrease in brain metabolic function
Increased levels of creatine phosphate, adenosine triphosphat(ATP) and glucose Decreased levels of monophosphate(AMP), adenosine diphosphate(ADP) and
lactate
9.Activation of the excitatory N-methyl-d-aspartate receptors and concomitant inhibitionof inhibitoryGABA(A)ergic neurotransmission
8. Fungsi Brain Metabolik berkurangmeningkat; creatin phosp. ; ATP; Glukosa,
Berkurang : monophosphate AMP; ADP dan Lactose
9. Aktifasi reseptor N-methyl-d-aspartat dan bersamaan dgn
inhibisi GABA [Gamma Amino Butyric Acid) (A)lergeic
neurotransmission
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Pathophysiology of uremic encephalopathy..5
10. Hormonal disturbances
parathyroid hormone, insulin, growth hormone, glucagon, thyrotropin, prolactin, luteinizinghormone and gastrin are elevated
PTH : promote the entry of calcium into neurons
Calcium : essential mediator of neurotransmitter release and plays a major role inintracellular metabolic and enzymatic processes
disrupt cerebral function by interfering with any of these processes.
10. Gangguan Hormonal.
Meningkat : PTH; Insulin; Growth Hormon; Glucagon; Thyrotoxin;
Prolactin; Luteinizing hormon; dan Gastrin.
PTH : Mendesak masuknya Ca kedalam Neuron. Ca adalah mediato
essential utk neurotransmitter.
merusak fungsi CEREBRUM oleh karena salah satuproses tersebut diatas (1-10)
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Incidence
The prevalence of UE is difficult to determine. Depends on the number of ESRD patients
In the 1990s : > 165,000 people
In the 1980s : 158,000
In the 1970s : 40,000
As the number of patients with ESRD increased,presumably so did the number of cases of UE.
Sex: Incidences are equal in men and woman.
Age: People of all ages can be affected
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Symptoms and signs
Symptoms begin insidiously. (tiba-tiba)
Progress slowly or rapidly. Changes in sensorium : loss of memory, impaired concentration, depression,
delusions, lethargy, irritability, fatigue, insomnia, psychosis, stupor, catatonia, andcoma.
Slurred speech, pruritus, muscle twitches, or restless legs.
SYMPTOM DAN SIGN
Mulai mendadak / tiba-tiba
Berlanjut Lambat atau Cepat
Timbul perubahan Sensorium, kehilangan memory, gangguan consentrasi
depressi, dilusi, letargi, irritabel, lelah, insomia, psychosis, stupor, catatonia
dan Coma. Sukar Berbicara, Pruritus, muscle twiching, atau kaki restlesness.
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Findings include the following;
Myoclonic jerks, twitches, or fasciculations (ie, uremic twitch-convulsivesyndrome postulated by Adams et al in 1997)
Dysarthria Agitation
Tetany
Seizures, usually generalized tonic-clonic
Confusion, stupor, and coma
TEMUAN YANG LAIN:
Myoclonik Jerk, Twiching, atau faciculation
( Uremic-Twiching-convulsion)
Dysarthria
Agitasi Tetany
Kejang, biasanya Tonik-Klonik
Confusi, Stupor dan Coma
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Imaging studies Brain imaging : limited value.
CT and MRI :cerebral atrophy and secondary ventricular dilatation.
ExcludingICH (Intra Cranial Hemorrhage) and
SDH (Sub Dural Hemorrhage(
Increased signal intensity in the cortical and subcortical areas of theparietal and occipital lobes
resolved after dialysis (Sembuh sesudah Dialysis)
PENCITRAAN:
Pencitraan otak, hasilnya terbatas
CT Scan dan MRI, terlihat atrophy Cerebral, dilatasi sekunder Ventricle
Exclusi : ICH ( Intra Cranial Hemorrhage) danSDH ( Sub Dural Hemorrhage)
Intensitas signal meningkat pada area Cortex dan Subcortex dari
Lobus Parietal dan Occipital.
Sembuh sesudah Dialysis
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EEG Serial EEG : useful in assessing patients and in monitoring their progress
Generalized slow wave : more severe as the condition worsens In acute uremia,
irregular low voltage with slowing of the posterior dominant alpha rhythm and occasional theta bursts.
prolonged bursts of bilateral, synchronous slow and sharp waves or spike waves
Bilateral spike discharge : myoclonic jerks
After dialysis begins, EEG may worsen for up to 6 months before slowly normalizing as renal functionimproves
EEG sangat bermanfaat utk penderita,dan utk monitor progresifitas.
Umumnya: Gelombang lambat, makin lambat makin parah
Pada akut uremia:
Ada Low voltage yang irreguler dgn gelombang lambat diposterior,rythme alfa dan kadang gelombang theta yg kacau, kalau bilateraltwiching
Sesudah dialysis dimulai, EEG masih jelek6 mgg mulai normal sesudah
Fungsi ginjal membaik
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EEG In chronic uremia,
the EEG stabilizes during long-term dialysis treatment. Deterioration corresponding to fluctuations in blood urea
levels : diffuse delta and theta activity, generalized spike-wave activity, and heightened sensitivity to photicstimulation.
PADA KRONIK UREMIA. EEG stabil selama dilysis
Kekacauan sesuai dgn kadar ureum dlm darah,
Umumnya ada aktifitas Spike waves dan sensitif ter
hadap stimulasi cahaya
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Brain histologic findings in UE
Meningeal fibrosis, glial changes, edema, vascular degeneration,
focal and diffuse neuronal degeneration, and focal
demyelination.
Small infarcts : due to hypertension or focal necrosis.
Cerebellar acute granule cell necrosis
GAMBARAN HISTOLOGI OTAK PADA UE
Meningeal fibrosis, Gial berobah, Vascular degenerasi,
fokal dan diffuse neuronal dan fokal demyelinsasi Dan infact kecil, ok. Hipertensi dan fokal necrosis
Cereberal akut granule sel necrosis.
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Neuroimage. 2007;34(2):694-701
ATROPHY OTAK, dan KELAINAN VASCULAR pd UE
Ada small necrosis, ada demyelinisasi ada fibrosis19.
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DIAGNOSIS
History
Clinical symptom and sign
EEG
IMAGING STUDIES
ANAMNESIS
GEJALA KLINIS
EEG
PENCITRAAN
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Thank you
Terima Kasih
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