Acute Kidney Injury (AKI) tipe Failure Medical Presentation

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Acute Kidney Injury (AKI) tipe Failure Pada Bayi Usia 7 Bulan dengan Ginjal Polikistik Bilateral Program Pendidikan Dokter Spesialis Patologi Klinik Fakultas Kedokteran Universitas Sebelas Maret Surakarta 2018 Presentan : Tisha Patricia, dr. Pembimbing : Dian Ariningrum, dr., M.Kes, Sp.PK MK 006
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Learning Objectives Menjelaskan patofisiologi penyakit ginjal polikistik Menjelaskan presentasi klinis, diagnosis, komplikasi, manajemen tatalaksana ARPKD Menjelaskan mengenai prognosis dan indikasi transplantasi ginjal pada pasien ARPKD 2
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Laporan Kasus I. IDENTITAS PASIEN Nama : By. KLN Tanggal lahir : 21 Maret 2017 Umur : 7 bulan Jenis Kelamin : Perempuan Alamat : Tegal Asri, Bejen, Karanganyar, Jawa Tengah No.Rekam medik : 01395xxx Masuk Rumah Sakit : 17 Oktober 2017 Keluar Rumah Sakit : 26 Oktober 2017 3
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Anamnesis 4
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Riwayat penyakit 5
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Riwayat penyakit 6
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Riwayat penyakit Riwayat penyakit dahulu: Lahir prematur + BBLR  Ranap selama 1 bulan Atresia ani letak tinggi  PSARP (usia 2 hari) Riwayat penyakit keluarga: Status kehamilan ibu: Usia kehamilan 1 bulan perdarahan  bedrest Usia kehamilan 4 bulan  bayi kembar Usia kehamilan 7 bulan  KPD 7
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Riwayat penyakit Status kelahiran pasien Lahir secara SC, BBL 1700 gram BBL saudara kembar 1900 gram  ranap 1 minggu Riwayat imunisasi Hepatitis B 0 bulan BCG 1 bulan DPT, HB, HIB, 2,3,4 bulan Polio 0,2,3,4 bulan 8
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Pohon Keluarga 9
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Pemeriksaan fisik 10
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11 Diterbitkan oleh World Health Organization tahun 2005 Judul asli Pocket Book of Hospital Care for Children, Guidelines for the Management of Common Illnesses with Limited Resources, 2005 © World Health Organization 2005
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JVP R+2cm KGB tidak teraba membesar Kepala: Mesocephal Mata: Konj. anemis +/+, Sklera ikterik -/- Mulut: mucosa basah, sianosis (-) Paru depan: I. : Bentuk normochest Pengembangan dada kanan = dada kiri, P: fremitus raba kanan = kiri P: sonor //sonor A.: vesikuler + / +, RBH +/+ minimal, RBK -/- Ekstremitas inf : Akral dingin -/-, Oedem -/- Cor: I. IC tak tampak P. IC teraba tidak kuat angkat P. Batas jantung kesan tak melebar A. BJ I-II normal, regular, bising (-), gallop (-) Abdomen I. DP//DD, spider nevi (-) A. Bising usus (+) normal, 8x/menit P. Timpani, pekak alih (-) P. Supel, Nyeri tekan (-), hepar dan lien tidak teraba membesar Paru belakang: I. : Bentuk normochest Pengembangan dada kanan = dada kiri, P: fremitus raba kanan = kiri P: sonor //sonor A.: vesikuler + / + , RBH -/- , RBK -/- Ekstremitas sup : Akral dingin -/-, Oedem -/- Genital: Tidak ada kelainan 12 JVP tidak teraba KGB tidak teraba membesar
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Pemeriksaan Penunjang Tabel 1. Pemeriksaan Laboratorium CITO tanggal 17 Oktober 2017 13
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Hasil pemeriksaan penunjang dari RS luar 21
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Hasil pemeriksaan penunjang dari RS luar 22
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Pemeriksaan Penunjang Lanjutan Tabel 2. Pemeriksaan Laboratorium darah lanjutan tanggal 25-26 Oktober 2017 23
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PEMERIKSAAN LANJUTAN Tabel 3. Pemeriksaan Urinalisis 17 dan 26 Oktober 2017 26
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PEMERIKSAAN LANJUTAN 27
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Hasil pemeriksaan gambaran darah tepi (GDT) 18 Oktober 2017 Eritrosit : Hipokrom sebagian normokrom, mikrosit, polikromasi, eritroblas (+) Leukosit : Jumlah dalam batas normal, vakuolisasi neutrofil, limfosit teraktivasi, monosit teraktivasi, sel blast (-) IT ratio 0.05 Trombosit : Jumlah meningkat, makrotrombosit, clumping (+) di beberapa lapang pandang Simpulan : Anemia hipokromik mikrositik dengan trombositosis suspek ec proses kronik dd defisiensi besi disertai proses infeksi dan perdarahan Saran : CRP, ferritin, retic 28
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Kerangka pikir 32
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Resume Bayi usia 7 bulan, keluhan utama demam Rujukan dari RS luar dengan prolonged fever, pneumonia, hipokalemia, hipokalsemia, kista renal multiple. Demam ↑↓ sejak 15 hari SMRS, batuk (+), sesak minimal. Pemeriksaan fisik TD 100/70 mmHg, HR 142 x/menit, RR 68 x/menit, suhu 36,6ºC, saturasi O2 98%, konjungtiva anemis +/+, RBH +/+ minimal. Pemeriksaan laboratorium darah: anemia, azotemia, penurunan eGFR, penurunan saturasi transferin. Urinalisis: bakteriuria, kristaluria. Pemeriksaan penunjang: foto polos abdomen AP: hepatomegaly; foto thorak AP: pneumonia; USG abdomen: kista multipel pada ren bilateral. Kultur sputum: didapatkan kuman Esherichia coli ESBL (+). Kultur urin: didapatkan kuman Enterococcus faecalis. 35
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Simpulan 36
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Tinjauan pustaka 37
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ADPKD 38
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ARPKD 1:10.000 - 1:40.000 (Dell, 2011) polycystic kidney and hepatic disease 1 (PKHD1) mengkode kompleks protein fibrocystin/polyductin (FPC). In utero oligohidramnion 40
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41 Dell KM. 2011. The Spectrum of Polycystic Kidney Disease in Children. Adv Chronic Kidney Dis. 2011 September ; 18(5): 339–347. doi:10.1053/j.ackd.2011.05.001.
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Pembahasan Anamnesis: riwayat kelainan ginjal polikistik (+) USG abdomen (+) kista renal bilateral Orang tua (-)  kriteria modifikasi Zerres dkk, 2011  ARPKD 42
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Pembahasan 44
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Pneumonia pneumonia-masyarakat (community-acquired pneumonia/CAP) pneumonia nosokomial hospital-acquired pneumonia (HAP) Bakteri penyebab pneumonia: Streptococcus dan Mycoplasma pneumonia Virus adenoviruses, rhinovirus, influenza virus, respiratory syncytial virus (RSV) dan para influenza virus (Anwar, 2014) 45
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Pneumonia Bakteri yang sering menjadi penyebab pneumonia adalah Streptococcus dan Mycoplasma pneumonia, sedangkan Virus : pneumonia adalah adenovirus, rhinovirus, influenza virus, respiratory syncytial virus (RSV) dan para influenza virus (Anwar, 2014). 46
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Pneumonia yang didapat di rumah sakit atau Hospital-acquired pneumonia (HAP) Bakteri > resisten Staphylococcus aureus Klebsiella pneumoniae Pseudomonas aeruginosa Escherichia coli (American Thoracic Society, 1996). 47
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Pneumonia 48
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ISK Infeksi saluran kemih (ISK) Bakteri atau mikroba  saluran kemih dalam jumlah bermakna (Pardede, 2011). Escherichia coli (E.coli) merupakan kuman penyebab tersering (60-80%) pada ISK. Proteus mirabilis, Klebsiella pneumonia, Proteus vulgaris, Pseudomonas aeroginosa, Enterobakter aerogenes, dan Morganella morganii, Stafilokokus, dan Enterokokus (Kanellopoulos dkk, 2006). 51
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ISK Berdasarkan lokasi infeksi, ISK atas dan ISK bawah Berdasarkan kelainan saluran kemih ISK simpleks dan ISK kompleks (Pardede, 2011). 52
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AKI standar definisi AKI peningkatan kreatinin serum ≥0,3 mg/dL, 48 jam, atau peningkatan kreatinin serum ≥1,5 kali baseline, 7 hari, atau produksi urin <0,5 ml/kg/jam selama 6 jam. Definisi standar AKI pada anak-anak, sebagian besar didasarkan pada klasifikasi paediatric risk injury failure lost and end stage (pRIFLE), 53
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54 Nilawati GAP. 2012. Kejadian Acute Kidney Injury dengan Kriteria pRIFLE pada Unit Perawatan Intensif Anak Rumah Sakit Sanglah Denpasar. Sari Pediatri 2012;14(3):158-61.
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Saran Monitoring fungsi ginjal Ureum Creatinine eGFR Pemeriksaan elektrolit Monitoring fungsi hepar Pemeriksaan enzim transaminase Albumin 57
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Daftar pustaka Alves M, Fonseca T, de Almeida EAF. 2015. Differential Diagnosis of Autosomal Dominant Polycystic Kidney Disease. In: Li X, editor. Polycystic Kidney Disease [Internet]. Brisbane (AU): Codon Publications; 2015 Nov. Chapter 1. doi: 10.15586/codon.pkd.2015.ch1 American Thoracic Society. 1996. Hospital-acquired pneumonia in adults: diagnosis, assessment of severity, initial antimicrobial therapy, and preventive strategies. A consensus statement, American Thoracic Society, November 1995. Am J Respir Crit Care Med. 1996; 153:1711–25. [PubMed: 8630626] Anwar A, Dharmayanti I. 2014. Pneumonia pada Anak Balita. Jurnal Kesehatan Masyarakat Nasional Vol. 8, No. 8, Mei 2014 Baerton M, Bell Y, Thame M, Nicholson A, Trotman H. Urinary tract infection in neonates with serious bacterial infections admitted to the University Hospital of the West Indies. West Indian Med J 2008;57:. Diunduh dari: http://caribbean.scielo.org/scielo.php?script=sci_aettext&pid=S0043. Bergmann C. 2018. Genetics of Autosomal Recessive Polycystic Kidney Disease and Its Differential Diagnoses. Front. Pediatr. 5:221. doi: 10.3389/fped.2017.00221 Chawla LS1, Eggers PW, Star RA, Kimmel PL. 2014. Acute kidney injury and chronic kidney disease as interconnected syndromes. N Engl J Med. 2014 Jul 3;371(1):58-66. doi: 10.1056/NEJMra1214243. Choopa MS,van Biljona G. 2015. Acute kidney injury in children – not just for the nephrologist. South African Family Practice 2015; 57(6):30-33 58
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Davis ID, Ho M, Hupertz V, Avner ED. 2003. Survival of childhood polycystic kidney disease following renal transplantation: the impact of advanced hepatobiliary disease. Pediatr Transplant 7(5):364–369 Dell KM. 2011. The Spectrum of Polycystic Kidney Disease in Children. Adv Chronic Kidney Dis. 2011 September ; 18(5): 339–347. doi:10.1053/j.ackd.2011.05.001. Dell KM, Matheson M, Hartung EA, Warady BA, Furth SL; Chronic Kidney Disease in Children (CKiD) Study. 2016. Kidney Disease Progression in Autosomal Recessive Polycystic Kidney Disease. J Pediatr. 2016 Apr;171:196-201.e1. doi: 10.1016/j.jpeds.2015.12.079. Epub 2016 Jan 28. DewiSW, SubanadaIB, PurnitiPS, AriawatiK. 2012. Jurnal Ilmu Kesehatan Anak: JIKA, Vol. I, No. 1, Desember 2012 Garin EH, Olavarria F, Araya C, Broussain M, Barrera C, Young L. Diagnostic significance of clinical and laboratory findings to localize site of urinary infection. Pediatr Nephrol 2007;22:1002-6. Goldstein M, Carrillo M, Ghai S. 2013. Continuous ambulatory peritoneal dialysis—a guide to imaging appearances and complications. Insights into Imaging. 2013;4(1):85-92. doi:10.1007/s13244-012-0203-y. Guay-Woodford, LM, Bissler, JJ, Braun, MC, Bockenhauer, D, Cadnapaphornchai, MA, Dell, KM, Kerecuk, L. et al. 2014. Consensus expert recommendations for the diagnosis and management of autosomal recessive polycystic kidney disease: Report of an international conference. Journal of Pediatrics, vol. 165, no. 3, pp. 611-617. DOI: 10.1016/j.jpeds.2014.06.015 59
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Halvorson CR, Bremmer MS, Jacobs SC. 2010. Polycystic kidney disease: inheritance, pathophysiology, prognosis, and treatment. International Journal of Nephrology and Renovascular Disease. 2010;3:69-83. Harris PC. 2008. 2008 Homer W. Smith Award: insights into the pathogenesis of polycystic kidney disease from gene discovery. J Am Soc Nephrol. 2009 Jun;20(6):1188-98. doi: 10.1681/ASN.2009010014. Epub 2009 May 7. Harris PC, Torres VE. Polycystic Kidney Disease, Autosomal Dominant. 2002 Jan 10 [Updated 2018 Jul 19]. In: Adam MP, Ardinger HH, Pagon RA, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2018. Hartung, EA, Guay-Woodford, LM. 2014. Autosomal recessive polycystic kidney disease: a hepatorenal fibrocystic disorder with pleiotropic effects. Pediatrics, 134(3), e833-e845. Kanellopoulos TA, Salakos C, Spiliopoulou I, Ellina A, Nikolakopoulou NM, Papanastasiou DM. First urinary tract infection in neonate, infants, and young hildren: a comparative study. Pediatr Nephrol 2006;21;1131-7. Kidney Disease: Improving Global Outcomes (KDIGO) Acute Kidney Injury Work Group. 2012. KDIGO Clinical Practice Guideline for Acute Kidney Injury. Kidney inter., Suppl. 2012; 2: 1–138. Lin CW, Chiou YH, Chen YY, Huang YF, Hsieh KS, Sung PK. Urinaty tract infection in neonates. Clin Neonatol 1999;6:1-4. Lonergan GJ, Rice RR, Suarez ES. 2000. Autosomal recessive polycystic kidney disease: radiologic-pathologic correlation. Radiographics. 2000 May-Jun; 20(3): 837–855. doi: 10.1148/radiographics.20.3.g00ma20837 60
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Nahm AM, Ritz E, Henriquez DE. 2002. Renal cystic disease (ADPKD and ARPKD), Nephrology Dialysis Transplantation, Volume 17, Issue 2, 1 February 2002, Pages 311–314, https://doi.org/10.1093/ndt/17.2.311 Pardede SO., Tambunan T., Alatas H., Trihono PP., Hidayati EL. 2011. Konsensus Infeksi Saluran Kemih Pada Anak. Bandung : Badan Penerbit Ikatan Dokter Anak Indonesia. Patil A, Sweeney WE Jr., Avner ED, Pan C. 2015. Childhood Polycystic Kidney Disease. In: Li X, editor. Polycystic Kidney Disease [Internet]. Brisbane (AU): Codon Publications; 2015 Nov. Chapter 2. doi: 10.15586/codon.pkd.2015.ch2 Sweeney WE, Avner ED. 2016. Polycystic Kidney Disease, Autosomal Recessive. 2001 Jul 19 [Updated 2016 Sep 15]. In: Adam MP, Ardinger HH, Pagon RA, et al., editors. GeneReviews® [Internet]. Seattle (WA): University of Washington, Seattle; 1993-2018. diunduh dari: https://www.ncbi.nlm.nih.gov/sites/books/NBK1326/ 61
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Takiar V, Caplan MJ. 2011. Polycystic kidney disease: pathogenesis and potential therapies. Biochim Biophys Acta. 2011 Oct; .1812(10):1337-43. doi: 10.1016/j.bbadis.2010.11.014. Epub 2010 Dec 10 Telega G, Cronin D, Avner ED. 2013. New approaches to the autosomal recessive polycystic kidney disease patient with dual kidney–liver complications. Pediatr Transplantation 2013: 17: 328–335. doi: 10.1111/petr.12076. Epub 2013 Apr 17. Unicef/WHO. 2006 Pneumonia: the forgotten killer of children. Geneva: The United Nations Children’s Fund/World Health Organization. Wilson PD. 2004. Polycystic Kidney Disease. N Engl J Med 2004; 350:151-164 DOI: 10.1056/NEJMra022161 Zerres K, Mücher G, Becker J, Steinkamm C, Rudnik-Schöneborn S, Heikkilä P, et al. Prenatal diagnosis of autosomal recessive polycystic kidney disease (ARPKD): molecular genetics, clinical experience, and fetal morphology. Am J Med Genet 1998;76:137-44. 62
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