Kidney disease Proteinurik Son

In the codes of conduct various kidney disease focal segmental proteinurik as glomerulosklerosis (GSFS), nephrotic syndrome survive steroids, and others have used kortiko-steroids, siklofosfamid, klorambusil, and given an oral siklosforin in a long time with different results . Despite many reports of success, but poor for a variety of things such as irregularity take medicine, take medicine that is not continuous, or high infection display, until researchers find a treatment with better results and side effects are rare. Puls therapy (pulse therapy is one treatment option for kidney disease this proteinurik. Puls usual therapy in children is metilprednisolon and siklofosfamid. To increase the number of success, then puls therapy is usually combined with oral corticosteroids or drugs alkilating.

Puls therapy is a high dose of medication at once by way of an intravenous bolus. Literature shows that puls therapy has been used against various diseases such as Rheumatoid Vasculitis, rheumatoid arthritis, multiple sclerosis, granulomatosis Wagener, eosinofilik lung disease, idiopathic pulmonary hemosi-derosis, leukemia, and some kidney disease. Some kidney diseases of children are not responsive to oral corticosteroids and imunosupresan puls terindikasi for therapy.

In several studies proved that siklofosfamid metilprednisolon puls puls and more effective than oral siklofosfamid and high dose oral steroids induces remission and prevention of terminal renal failure with lesser side effects.

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Puls Therapy Kidney Disease Proteinurik Son part 1

Guidance

Some kidney disease ditatalaksana proteinurik children with puls therapy, among others: class IV lupus nephritis or proliferative lupus nephritis difus, systematic lupus erythematosus temik Infantile, glomerulosklerosis focal segmental (GS-FS), membranoprolifetarif glomerulonephritis type I, Henoch-Schonlein purpura nephritis heavy, heavy glomerulonephritis, nephrotic syndrome and steroid survive.

Way
Medications are often used in therapy puls is metilprednisolon dose 15-30 mg / kgbb / day and cyclo-fosfamid 500-1000 mg / kgbb / day.

There are many ways giving puls therapy.

1. Metilprednisolon

a. Metilprednisolon with a dose of 30 mg / kgbb (maximum 1500 mg) was dissolved in 50-100 ml dekstrose 5%, given by infusion for 1 hour or can be for 6 hours. As long as the necessary monitoring of vital signs, especially blood pressure and heart frequency. Puls therapy is given every other day three times a week on weeks 1 and 2. During weeks 3 to 10 methyl-prednisolone 30 mg / kgbb given once a week, week 11 to 18 metilprednisolon 30 mg / kgbb given every 2 weeks, at week 19 to 50 metilprednisolon 30 mg / kgbb given every 4 weeks, and during the week 51 up to 82 metilprednisolon 30
mg / kgbb given every 8 weeks. Starting week three with oral doses given prednison 2 mg / kgbb / day every other day and week eleven prednison down slowly until week 82.

If the treatment is improvement of living there, so drugs are not given alkilating. Alkilating drug is given if there has been a persistent nephrotic proteinuri after 10 weeks of treatment metilprednisolon puls or if initially there is improvement, but accompanied by an increase proteinuri means. Cyclo-fosfamid usually given 2 to 2.5 mg / kgbb / day or klorambusil 0.2 mg / kgbb / day for 8-12 weeks.

b. Puls Metilprednisolon 1000 mg / 1.73 m2 LPB provided with the help of infusion pumps in 6 hours 3 times every other day. After it was given 30 mg/m2 prednison LPB every day for a month, then 30 mg / m2 per-day intervals LPB for 2 months, and 15 mg/m2 LPB every other day for 2 weeks. Empowerment series
puls metilprednisolon can be extended to 8 months to 4 years, depending on the clinical symptoms of disease.

c. Bergstein and Andreoli (1995) gave methyl-prednisolone puls 30 mg / kgbb / dose (maximum 1.5 g) infused intravenously in 50-100 ml of liquid dekstrose 5% for 30-60 minutes, is given every day 6 times followed by prednison 2 mg / kgbb / day (maximum 60 mg / day) single dose every other day for an average of 37 months (12-66 months) or prednison 2 mg / kgbb / day for 1 month and to then, slowly revealed. (8,10,17) Termination of treatment depends on the stability of the clinical disease.

2. Siklofosfamid

a. Siklofosfamid LPB 500 mg/m2 in 250 ml NaCl 0.9%, given by infusion slowly within 3-4 hours to reduce the risk of nausea and vomiting. In addition to being organized is also giving an oral liquid with a large number of 24 hours and often advisable to reduce the risk of new bladder hemoragik reefs. Puls Siklofosfamid given every month for six months. LPB Prednison 60 mg/m2 given every day for two months, followed by a grant every other day
(Alternate days) for 4 months, and a further dose 30 mg/m2 LPB every other day for 6 months. One year after the initial dose intravenous and prednison siklofosfamid, steroids down slowly 10 mg per month, and stopped in 2-6 months, depending on the initial dose.

b. Puls Siklofosfamid given in 100-150 ml dekstrose 5% for 1 hour followed by hydration with 5% in the infusion dekstrose NaCl 0.5% 2 l/m2 LPB given for 24 hours. After the initial grant, siklofosfamid puls given every month for 6 months and up to 7 doses and was continued every 3 months to 3 years. At the beginning of cyclo-fosfamid given dose of 500 mg/m2 LPB, LPB increased to 750 mg/m2 to 1000 mg / m2 LPB.

c. Puls Siklofosfamid dose of 750 mg/m2 given by LPB. After therapy siklofosfamid puls, patients received intravenous hydration for 24 hours and organized frequent bladder. Puls siklofosfamid Initially given every 1-2 months depending on disease activity. Dose reduced if klirens creatinine <40 ml / min. After a dose of a total of 6 times, the treatment stopped if there is no active form of the disease. This dose can be extended up to 2 or 3 times 6 doses. Prednison 1 mg / kgbb / day given every day for 6 weeks, then reduced to 40 mg and 20 mg every other day.

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Puls Therapy Kidney Disease Proteinurik Son part 2

Advanced part 1 ....

There are many ways giving puls therapy

d. Puls Siklofosfamid given by infusion for 1 hour with a dose of 500 mg-1, 000 mg/m2 LPB and done with organized adekuat hydration bladder frequently. Puls Siklofosfamid is given every 3 months for 4 years or until at least 18 months in remission.

e. Puls Siklofosfamid given every month for 12 months with a dose of 500 mg/m2 in the first month LPB, 750 mg/m2
LPB in the second, and a further 1000 mg / m2 LPB.

f. Siklofosfamid puls with the dose 750 mg-1, 000 mg/m2 given every month LPB to 6 months followed by a puls sikofosfamid the same dose every 3 months for 2 -3 years. Prednison given with a dose 0.5 mg / kgbb / day and then lowered slowly.

3. Metilprednisolon with siklofosfamid

a. Metilprednisolon puls 20 mg / kgbb / day given in 2 hours for 3 days. After giving intravenous metilprednisolon, carried out by intravenous hydration followed by a grant siklofosfamid LPB 500 mg/m2 plus Mesna (uromitexan) 360 mg/m2 in 100 ml LPB dekstrose 5% of each 2-hour infusion, followed by intravenous hydration. Mesna infusion given again after 2-4 hours, 6 hours and 10 hours. After metilprednisolon puls, prednison given dose 2 mg / kgbb / day and then lowered slowly. Siklofosfamid infusion given every month for six months with the dosage increased to 750-1000 mg/m2 LPB. After giving six times per month, puls siklofosfamid given every 3 months. This regimen is given in the codes of conduct lupus erythematosus Infantile systemic.

b. Metilprednisolon 30 mg / kgbb / dose given for 3 consecutive days followed by a dose of 0.5 siklofosfamid puls g/m2 LPB / dose once a month for 6 months. Prednison 2 mg / kgbb given every other day.

c. Puls cyclophosphamide at a dose of 500 mg LPB-750 mg/m2 given every month to 6 months; dose is increased to 1 g/m2 LPB for leukocytes> 3.000/mm3. Then given high doses of corticosteroids such as methylprednisolone iv Puls 1 g per day for 3 days or prednisone daily intervals; next dose lowered slowly. Patients received hydration for 24 hours before and after cyclophosphamide therapy. In addition to cyclophosphamide Puls, Puls one gives methylprednisolone with oral cyclophosphamide or chlorambucil. Puls methylprednisolone administered at a dose of 30 mg / kg / day for 3 consecutive days followed by prednisone 45 mg/m2 LPB / day and lowered slowly for 3 months, cyclophosphamide 2 mg / kg / day for 2 months, and dipiridamol 5 mg / kg / day for 6 months. Moroni et al. (1998) provide three times Puls methylprednisolone followed by oral prednisone 0.5 mg / kg / day at month 1,3, and 5 and chlorambucil 0.2 mg / kg / day in 2, 4, and 6. This regimen is given for the governance of lupus nephritis.

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Seizures Fever in Children

A raised temperature or fever is the cause of most of the seizures in children; found at about 4.8% or 1 in 21 children, and is the largest difference among all the neurological diseases of children. What is referred to as spastic fever or "febrile convulsion"? Fever seizures are seizures that occur all the production on the increase in rectal temperature above 38 ° C without any sign of infection in the brain or other causes.

Cramps fever is generally considered harmless and often cause no symptoms remaining, but when seizures last a long time to cause hypoxia in the order of the Central Nervous network can cause the symptoms of waste at a later date. When temperature increases lead to the production strain on a child's healthy at all, referred to as spastic mild fever, as rising temperatures reduce excitatory threshold of the strain on each child.

In contrast to that is a difference in the composition of the Central Nervous is the basic cause of the seizures, while the temperature increased by only a factor only a trigger, for example in children with a variety of birth trauma and others. A lot of scholars who investigate the fever seizure is Livingston (1). It has made the criteria for the calls as "simple febrile conwlsion" mild fever or cramps, and is still used as a guide in sub-section Nervous Child, The Child Health Sciences, FKUI Jakarta.

Criteria and then made modifications are:

1. Child's age when seizures between 6 months and 4 years.
2. Seizures last only a little, not more than 15 minutes.
3. Seizures are common.
4. The incidence of seizures within 16 hours after the temperature begins to rise.
5. Neurological examination before and after the normal stiffness.
6. EEG examination made at least one week after the disease showed no abnormalities over.
7. The frequency of seizures in the first generation of not more than 4 times

All of the above criteria must be met to make the diagnosis mild fever cramps. Is the highest age for mild fever seizures are age where teijadi cramps the first time, when a child first cramped near that age, even with very high temperatures, mild fever usually is not stiff.

High temperature is a necessity in the medium fever cramps, seizures that are not the infection itself, but by the high temperature rise due to infection where Iain, for example in an acute inflammation of the middle ear and so on. When in the history of the age-age patients previously found periods in which children suffer very high temperature, but did not have seizures, so the spasms that occur later have to be careful, as this may be stiff
is the cause.

At a moderate fever cramps cramps usually occur when the temperature is increased rapidly, so parents often do not anticipate that children suffering from fever. Temperature rise suddenly is an important factor to cause seizures. Mild fever cramps cramps in the general form of always, usually tonic-clonic in nature, such as grand mal seizures; sometimes just general stiffness or spring mendelik moment. Seizures can also be repeated, but a little while, and still within 16 hours of rising temperatures, generally at a sharp temperature rise, in this case fever cramps medium is still possible.

Seizures more than 15 minutes, but some say more than 10 minutes, often creates unforeseen variations nerves settled. EEG at moderate fever cramps should be normal when done at least a week after the body temperature back to normal, because the fever itself can cause abnormalities are not specific on the EEG can be set to 1 week.

When all the criteria from Livingston met in a child suffering from fever cramps, general possibility of the child suffering from seizures without fever in later life is very small, even though sometimes cramps can recur when the child suffered a high fever once. Some writers add a few helper to the criteria above (2, 3), namely: family history on the stiffness mild fever often indicates a fever seizures in other family members, but no history of seizures is possible without fever and high temperature rise suddenly suddenly more important means of raising
seizures.

Cramps fever prognosis is not as mild fever seizures by Livingston as "Epilepsy-off triggered by fever" or epilepsy provoked by fever, a fever seizure that does not meet one or more of the seven criteria above Livingston. Seizures are the second group has a basis which gave rise to seizures, while the fever is only a trigger factor only. During seizures provoked by fever, high fever, the cramps may be repeated in the future, more and more to the point of low temperature and may occur without fever akhirnyakejang same
once. Livingston found that the occurrence of epileptic seizures idiopathic from mild fever at a later date is 2.9% of all patients (a number equal to the normal population), whereas from epilepsy provoked by fever, 78% may be changed to idiopathic epilepsy later in life.

Common consensus held by the National Health Institute in the United States in 1980 (4) produce a number of factors in patients with fever cramps higher risk for seizures without fever attacks in the future, is: when there are at least two of the risk factors as follows:
1. Seizures without fever hospital on one or both parents or siblings,
2. Variations in development or neurological abnormalities before the child cramped fever, and
3. Seizures that last a long time or focal seizures. When the above risk factors studied, it is not much different from the criteria mentioned previously Livingston

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Cramps FIGHTING FEVER

Reduction of long-term febrile seizures carried out by two ways:

A. Prophylaxis "Intermittent"

From previous research it appears to be a fever seizure relapses at 30 to 40% of patients, especially in children under 18 months. To prevent recurrent seizures in the future, patients suffering from mild fever seizure medication prophylaxis "Intermittent", ie a mixture of medication and Anticonvulsants antipiretika as drug preparations in elderly patients, who should be given to children suffering from fever so it was palpable in the future . Anticonvulsants are given drugs usually phenobarbital, which is known to have the fewest side effects compared with other drugs Anticonvulsants. Rates therapeutic levels in the blood in order to prevent the occurrence of seizures is between 15 to 20 micrograms%. In giving oral phenobarbital, the normal dose, the therapeutic can be achieved within 24 hours, but then would fall back under the line of therapeutic rates.

Included in the rate achieved therapeutic longer and faster, use double doses every 12 hours, then a stable therapeutic dose would be achieved in 24-36 hours. Usual dose is 4-5 mg / kg of body weight per day, and double doses of 80-10 mg / kg / day every 12 hours. This double dose usually given for at least 2 x 24 hours, then continued with the usual dose for the following days. When given the normal dose of phenobarbital with 4-5 mg / kg per day, a steady rate of new therapeutics is achieved only after 10-14 days. Anticonvulsants and antipyretic actually giving as it is considered less accurate, because the cramps are usually mild fever cramps thus arises in the first 16 hours after the child is getting the temperature rise. But in the research of children suffering from mild fever cramps, giving antipiretika without Anticonvulsants than those given antipiretika by Anticonvulsants, it appears the two seizures could be prevented with meaningful results (P <0.02) to obtain better results actually need a higher dose that is 10-15 mg / kg / day, but with this dose of course give side effects like drowsiness, emphasis on the respiratory center. etc..

Drugs which are now widely used to prevent a recurrence of mild fever seizures is rectal diazepam. Diazepam rectal is presumably to help prevent seizures when given as children palpable heat, and even better results with giving oral. How long prophylaxis "Intermittent" is given?

Generally, prophylaxis is given up to the age where the possibility of the child to suffer from mild fever cramps are very small, so until the maximum age limit of the criteria Livingst on.

B. Long-term prophylaxis

Long-term or continuous prophylaxis given to patients who do not meet the stiff one or more criteria from Livingston or suffering from epilepsy provoked by fever. Long-term prophylaxis guarantee a stable therapeutic dose there was an adequate d in the blood of patients to prevent recurrent seizures in the future.

The way the grant is the usual dose. given in two doses per day, given continuously for at least three years are not cramps, kernudian down slowly ("taperring off) for 3-6 months. Consensus with (4) produce some criterion to be used as a guide for medication Anticonvulsants in all seizures fever, which continued prophylaxis is given if there are conditions as follows:

1. There is a nerve growth disorders such as cerebral palsy, retardasi development, or microcephaly.
2. When cramps fever lasts more than 15 minutes, to be focal, or followed by a temporary neurological abnormalities or settled.
3. When there is history of a seizure without any heat on the genetic parents or relatives home.

When observed by one, the third fact is not inconsistent with the standard criteria from Livingston. Drugs used for long-term prophylaxis:

• phenobarbital to prevent the risk of recurrence of seizures likely good enough, although sometimes - sometimes in a provoked epileptic seizures fever fever is sometimes though rarely, is still repeated by provision Anticonvulsants but in this case must be considered is the possibility of the elderly
forget or get bored give Anticonvulsants drugs continuously. Side effects of long-term fenobarbital is changing the nature of children to be hyper active, sleep cycle changes (difficulty sleeping) and sometimes disorders, cognitive function or functions noble.

• Sodium valproat / valproat acid (Epilin, Epanutin, Depakin), may reduce the risk of recurrence of seizures with fever in this group is satisfactory, even more satisfying than with phenobarbital. Given in doses of 20 mg / kg / day in 3 divided doses. Lack of this drug is its price much more expensive compared to phenobarbital, and many report symptoms of nausea foksis form in the stomach, liver damage,
pancreatitis.

• phenytoin (Dilantin) is sometimes given to kids, with his kick to the grant of the nature of phenobarbital hiperaktifitas fear will increase the symptoms, but the results are often unsatisfactory. In general, the prognosis cramps fever prophylactic treatment satisfactory. We recommend that parents be given an adequate explanation to understand all the risks that may occur due to fever, cramps, and are briefed on how the grant of the right drug and the first penangpenanggulangan when children cramped in the house.

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