Refarat Nephrotic Syndrome Frequent Relaps

REFERAT

NEPHROTIC SYNDROM FREQENT RELAPS

Created by:Chriscahya Wibisana Candra0861050169

Pembimbing : Prof dr. Taralan Tambunan, SpA(K)

KEPANITERAAN KLINIK ILMU PENYAKIT ANAKPERIODE 6 AGUSTUS 2012 6 OKTOBER 2012FAKULTAS KEDOKTERAN UNIVERSITAS KRISTEN INDONESIAJAKARTA2012

BAB I

INTRODUCTION

Praise and gratitude i give to God upon the blessings and of his so writer can finish drafting a Referat with a title Nephrotic Syndrome Frequent Relapse. This Referat is arranging by the writer to completing task in Pediatric Department at RS FK-UKI.

Writer would give a million thanks to Prof. Dr. Taralan Tambunan, SpA (K), who has been guiding and teaching the writer in process of arranging referat, so the writer gets more understanding in Nephrotic Syndrome Frequent Relapse disease

Writer realizes there are still many deficiencies both on the content and format of this Referat. Therefore, writer accepts all criticism and input with open arms and apologizes if there are errors in the Referat task that writer created.

Writer hope that this referat could be a media for information and reference for the reader when facing a case that have a link to a kidney disease in general and especially in Nephrotic Syndrome

In final words writer would like to say a million thanks to all, may this referat can give more information for us all

Jakarta, Agustus 2012

Chriscahya Wibisana CandraBAB II

CONTENT

II.1Preliminary Syndrome Nephrotic is a chronical disease that usually found in child, with incident 2-4 cases in 100.000 children under 16 every year. histopathology deviation is most happen in syndrome nephrotic, syndrome nephrotic can attack every age, especially child in 2-6 years, man is get nephrotic syndrome more frequent than women in ratio 3:2 more than 90% case of syndrome nephrotic is idiopathic, and the rest is secondary syndrome nephrotic that causes by many kind of disease1Syndrome Nephrotic Frequent Relapse is when its happen more than 2 times in 6 month or relapse more than 4 times in 1 years, usually after initial treatments, 94 % cases get total remission of the disease, and the rest 60-70% will become relapse 30-40% will become frequent relapse2

II.2Etiology2 Nephrotic syndrome can be primary, as part of a systemic disease, or secondary due to several causes1. Primary causes:a. post infectionb. Collagen vascular disease (SLE, rheumatoid arthritis, polyarteritis nodosa)c. Henoch-Schnlein purpurad. Hereditary nephritise. Sickle cell diseasef. Diabetes Mellitusg. Amyloidosish. Malignancy (leukemia, lymphoma, Wilms Tumar, pheochromocytoma)i. Toxin (bee stings, snake venom) j. Drugs (probenecid, fenoprofen, catopril, lithium, wafarin, penicilamine, mercury, gold, trimethadione, the metadione, NSAIDs)

2. Secondary causesa. Group A beta-hemolytic streptococcusb. Syphilisc. Malariad. Tuberculosise. viral infection

Most (90%) of the children were suffering from nephrotic syndrome have some form of idiopathic nephrotic syndrome, including; disease lesions of at least about 85%, the proliferation of mesangium 5%, and 10% local sclerosis. In the remaining 10% of children suffer nephroses include. Nephrotic syndrome is largely mediated by some form of glomerulonephritis and most often affects the membranous and membranoproliferatif.

II.3Pathofisiology3Proteinuria is a major symptom of nephrotic syndrome, proteinuria occurred more severe than proteinuria in others kidney disease. The amount of protein in the urine can got 40mg/jam /m2 body surface area (1 gram / m2/day) or 2 to 3.5 grams / 24 hours. Proteinuria that occur due to changes in the selectivity of the protein, and changes in the glomerular filter.

Changes in selectivity to proteins and glomerular filtration rate changes will depend on the type of glomerular abnormalities. But the outline can be explained that, in the normal child, filtration of low molecular weight plasma protein negatively charged in glomerular basement membrane is normally maintained by the negative charge filtration barrier. The negative charge of the molecule consists of heparan sulfate proteoglycans. In people with nephrotic syndrome, the concentration of heparan sulfate saccharide mucopoly at basal membrane is very low. So many proteins can pass through the barrier. In addition there is also a change gap size (pores) on the barrier so that the neutral charge protein can pass through the barrier.

In Nephrotic syndrome occurs hipoproteinemia mainly albumin, this is caused by increased excretion of albumin in the urine and increased degradation in the renal tubules that exceed power synthesis of the liver. Disruption of protein in plasma was decreased -1 globulin. While 2globulin -, -globulin and fibrinogen increased relative or absolute. -2globulin increased due to the selective retention of high molecular weight proteins by the kidney, while the rate of synthesis is relatively normal.

There are two theories regarding the pathophysiology of edema in nephrotic syndrome; theory underfilled and the theory overfille. In underfill theory described edema formation occurs because of decreased albumin (hipoalbuninemia), due to loss of protein in the urine. Hypoalbuminemia causes a decrease in plasma oncotic pressure, which allows the transudation of fluid from the inervaskular to interstisial space. Intravakular volume decrease causes a decrease in renal perfusion pressure, resulting in the activation of the renin-angiotensin-aldosterone system, which stimulate sodium reabsorption distal tubulus. Decrease in intravascular volume underfilled also stimulates the release of hormones that enhance antideuritik water absorption in the collecting ducts. Due to the lack of oncotic pressure fluid and sodium that has been reabsorbed back into the interstitial space so that aggravate edema.

In theory described overfill sodium and water retention caused by intra-renal mechanisms of primary and independent of systemic peripheral stimulation. And the presence of agents that increase the permeability of the capillary circulation throughout the body and kidneys. Primary sodium retention caused by intra-renal defect is causing the expansion of the plasma and extracellular fluid. Edema that occurs due to overfilling of fluid into the interstitial space.

II.4Diagnosis1 Syndrome Nephrotic can be diagnose based on 4 specific symptomps11. Masif Proteinuria or Proteinuria NephroticUrine contain protein 40 mg/m2lpb/jam or > 50mg/kgBB/24hours, ratio albumin/kreatinin in urine > 2 mg/mg, or dipstik 2 . 2. HipoalbuminemiaAlbumin serum < 2,5 g/dl. Normal albumin serum in child is 3,6-4,4g/dl. In syndrom nephrotic water retension and swelling can be seen if albumin under 2,5-3,0 g/dl 3. Swelling4. HiperlipidemiaPasien syndrome nephrotic mengalami hiperkolesterolemia ( cholesterol more than 200mg/dl

II.5Examination1,31. Urinalisis and Urine Culture2. Quantitative protein urin, can be 24 hours urine, or ratio protein/kreatini in first urine in the morning3. Blooda. Peripheral Blood (Hemoglobin, White Blood Cell,Diff Count, Trombosit, Hematokritb. Levels of albunin and plasma cholestrol c. Ureum and Creatinine and Creatinine Clearanced. ASO titer and levels of complement C3 if have microscopic hematuria presisten4. Kidney BiopsyKidney biopsy usually not needed in most of child with nephrotic syndrome, more than 80% child with nephrotic syndrome, is a nephrotic syndrome with minimal lesion with typical characteristic when see in light microscope the normal histological kidney, the indication for kidney biospy is

II.6Treatment1Initial treatment for nephrotic syndrom according to ISKDC (International Study on Kidney Disease in Children), initial prednisone start with full dose 2mg/kgBB/day or 60mg/m2LPB/day (maximum dose 8o mg/hari) that divided into 3 times of administration, for induce remision, full dosage of prednisone is administrate for 4 weeks, if there are remition after 4 weeks of treatment , the dosage of steroid for further 4 weeks is 40 mg/m2LPB/day (2/3 of ful l dose), 1 times a day after breakfast, if 4 weeks early treatment not giving a remition patient will catogorize as resisten steroid

Today there are 4 option for treatment in syndrom nephrotic frequent relaps, and that is :1. Long Term Steroidmany eksperiment show that long term steroid can be try at first before giveing siklofosfamid (CPA), knowing the side effect of steroid is small. If it has been diagnose as syndrom nephrotic frequent relaps, after get to remision with prednison full dosage, the threatment continued with altrenating steroid that tamprering off slowly 0,2 mg/kgBB until it reach smallest dosage that no causeing relaps 0,1-0,5 mg/kgBB , this dosage called, threshold dosage and can be continued for 6 12 month, then tried to stop. In most cases schoolchild can tolerate prednison 0,5 mg/kgBB and preschoolers can tolerate until 1 mg/kgBB/day in alternating1

2. LevamisolLevamisol is drugs with effect immunomodulating selT. Consumptin of levamisol on nephrotic syndrom still limited becasue the effect is till doubtful . in Jakarta study show giving levamisol show bad result, side effect of levamisol is nausea, regurgutate, amd reversible neutropenia, the dosage of levamisol is 2,5 mg/kgBB single dosage for 4-12 month

3. SitostatikSitostatik drugs that usually used in treatment nephrotic syndrom in child is siklofosfamid (CPA) with dosage 2-3mg/kgBB for 8 weeks. Stiostaika can reduce relaps more than 50%, that is 67-93% in first years, and 36-66% for 5 years. APN report that giving CPA for 12 weeks can keep the remition longer than giving CPA for 8 weeks, but this statment not confirmation by other researcher Administration of CPA on keeping remition more effectiveon nephrotic syndrom frequent relaps (70%) than sindrom nephrotic steroid dependen (30%). Side effect of sitosatika is bone marrow depressio, aloplesia, sistisis hemorage, azospremia and on long trem can cause malignacy

4. Siklosporin (CyA)

On Nephrotic Syndrome that not responsif in steroid and sitostatik treatment its recommended to giving siklosporin, that inhibit the calcineurin with dosage 5-6mg/kgBB/day to keep the consentration in blood. CyA can make and keep the remition, so the administration of steroid can lowerer or stop, but if CyA is stoped, usually will become relaps (dependen siklosporin)

II.7Prognosis1,4

The majority of children with steroid-responsive nephrotic syndrome have repeated relapses, which generally decrease in frequency as the child grows older. Although there is no proven way to predict an individual child's course, those children who respond to steroids rapidly and those who have no relapses during the first 6 mounth after diagnosis are likely to follow an infrequently relapsing course. It is important to indicate to the family that the child with steroid-responsive nephrotic syndrome is unlikely to develop chronic kidney disease, that the disease is generally not hereditary, and that the child (in the absence of prolonged cyclophosphamide therapy) will remain fertile. To minimize the psychological effects of the condition, the physician should emphasize that the child should be considered normal when in remission and may have unrestricted diet and activity, without the need for urine testing for protein.

Children with steroid-resistant nephrotic syndrome, most often caused by FSGS, generally have a much poorer prognosis. These children develop progressive renal insufficiency, ultimately leading to end-stage renal disease requiring dialysis or renal transplantation. Recurrent nephrotic syndrome develops in 3050% of transplant recipients with FSGS. Plasmapheresis, plasma protein absorption onto protein Abased columns, high-dose cyclosporine or tacrolimus, and ACE inhibitors may reduce proteinuria in these patients.

II.7Conclusion

Syndrome Nephrotic is a chronical disease that usually found in child, with incident 2-4 cases in 100.000 children under 16 every year. Syndrome Nephrotic Frequent Relapse is when its happen more than 2 times in 6 month or relapse more than 4 times in 1 years, usually after initial treatments, 94 % cases get total remission of the disease, and the rest 60-70% will become relapse 30-40% will become frequent relapse.

Syndrome Nephrotic can be diagnose based on 4 specific symptomps that is Masif Proteinuria or Proteinuria Nephrotic is when urine contain protein 40 mg/m2lpb/jam or > 50mg/kgBB/24hours, ratio albumin/kreatinin in urine > 2 mg/mg, or dipstik 2 .hipoalbuminemia is when albumin serum < 2,5 g/dl. Normal albumin serum in child is 3,6-4,4g/dl. In syndrom nephrotic water retension and swelling can be seen if albumin under 2,5-3,0 g/dl , swelling, and hyperlipidemia patien with nephrotic syndrome have hiperkolesterolemia ( cholesterol more than 200mg/dl.

Today there are 4 option for treatment in syndrom nephrotic frequent relaps, and that is, Long Term Steroid, Levamisol, Sitostatik and Siklosporin (CyA)

DAFTAR PUSTAKA

1. Noer M Sjaifullah, 2011. Sindrom Nefrotik Idioatik. In Noer M Sjaifullah, Soemyarso Ninik, Subandiyah Krisni, dkk. Kompendium Nefrologi Anak. Jakarta : Badan Penerbit Ikatan Do; 142-163.

2. Lane C Jerome, Pediatric Nefrotik Syndrome. www.emedicine.com Last Update: November 11, 2011..

3. Eddy A Allison, Symons M Jordan, 2003. Nephrotic Syndrome in Childhood. Lancet. 2003. 362:629-639

4. Kliegman RM, et al. Nelson textbook of pediatrics. 18th ed Philadelphia : Saunders Elsevier, 2007 : 1533-4.