Anjioödem İle Karıştırılan Nefrotik Sendrom: Olgu Sunumu

Abstract

Nefrotik sendrom yaygın ödem, masif proteinüri ve hipoalbuminemi ile karakterize, sıklıkla 2-6 yaşlarında oluşan bir sendromdur. Genellikle gün içinde azalan periorbital ödem nedeniyle bu hastalar yanlışlıkla alerjik hastalık tanısı almaktadır. Daha önceden bilinen takipli hastalığı olmayan, bilateral hidrosel ve inguinal herni nedeniyle ameliyatı planlanan, operasyon öncesi değerlendirmede gözlerinde bilateral şişlik farkedilen 4 yaş 9 aylık erkek hasta, anjioödem ön tanısı ile çocuk immünoloji alerji bölümüne konsülte edildi. Göz kapakları ödemli, bilateral pretibial gode bırakan ödem ve skrotumda bilateral translüminasyon gösteren şişlik olan hastanın diğer muayeneleri normaldi. Laboratuvar incelemesinde: Albumin: 1.6g/dl, Sedimentasyon:76 mm/saat, C3:1,23 mg/dl, ve C4: 0,24 mg/dl. İdrarda: dansite: 1051, protein: +++, protein/kreatinin 17,8 mg/mg idi. Hem nefrotik sendrom hem de anjioödem göz kapaklarında, genital bölgede ciddi ödem oluşturur. Bu nedenle laboratuvar tetkikleri, klinik bulgular, dikkatli muayene ayırıcı tanı için yeterlidir. Çalışmamızda alerjik anjioödem ön tanılı nefrotik sendrom olgusunu sunmayı amaçladık.

Keywords

• Anjioödem
• hipoalbuminemi
• nefrotik sendrom

Angioedema mimicking nephrotic syndrome.: A case report

Abstract

Abstract Nephrotic syndrome is characterized by diffuse edema, massive proteinuria and hypoalbuminemia. It usually occurs between the ages of 2-6. Due to periorbital edema these patients are mistakenly diagnosed with allergic disease. A 4-year-old 9-month-old male patient, who had no known disease under follow-up, was admitted to the pediatric surgery service due to bilateral hydrocele and inguinal hernia, was planned to be operated on, and bilateral swelling in his eyes was noticed in the preoperative evaluation, was consulted to the pediatric immunology-allergy department. His general condition was good, his skin turgor was normal, his eyelids were edematous. There was bilateral pretibial pitting edema and bilateral transluminated swelling in the scrotum. In laboratory examination: Albumin: 1.6g/dl, Sedimentation:76 mm/hr, C3:1.23 mg/dl, and C4: 0.24 mg/dl. In urine: density: 1051, protein: +++, protein/creatinine was 17.8 mg/mg. Both nephrotic syndrome and angioedema cause severe edema of the eyelids and genital area. Therefore, laboratory tests, clinical findings and careful examination are sufficient for differential diagnosis. In our study, we aimed to present a case of nephrotic syndrome with a prediagnosis of allergic angioedema.

Keywords

• Angioedema
• hypoalbuminemia
• nephrotic syndrome

References

1. 1. Downie ML, Gallibois C, Parekh RS, Noone DG. Nephrotic syndrome in infants and children: pathophysiology and management. Paediatr Int Child Health, 2017; 37(4): 248–58.
2. 2. Pasini A, Benetti E, Conti G, Ghio L, Lepore M, Massella L, et al. The Italian Society for Pediatric Nephrology (SINePe) consensus document on the management of nephrotic syndrome in children: Part I – Diagnosis and treatment of the first episode and the first relapse. Ital J Pediatr , 2017; 43(1), 41.
3. 3. Özdemir Ö. Acil Serviste Herediter Anjioödem’e Güncel Yaklaşım. Sakarya Tıp Dergisi / Sakarya Med J, 2020; 10(1):152-61.
4. 4. Özdemir Ö. Current and future therapy of hereditary angioedema. South Clin Ist Euras 2020; 31(3): 301-307.
5. 5. Norris M, Ashoor Z, Craig T. Management of pediatric hereditary angioedema types 1 and 2: A search for international consensus. Allergy Asthma Proc. 2022; 43(5):388- 396.
6. 6. Özdemir Ö. Diagnosis and differential diagnosis in hereditary angioedema. Türkiye Klinikleri J Allergy -Special Topics. 2011;4(2):21-35.
7. 7. Özdemir Ö. Tekrarlayan Anjiyoödem Atakları ile Başvuran Hastaya Akılcı Yaklaşım. Klinik tıp pediatri dergisi 2019; 11(2):64-73
8. 8. Zamora G, Pearson-Shaver AL. Minimal Change Disease. 2021 Aug 1. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan–. PMID: 32809474.

9. 9. Vivarelli M, Massella L, Ruggiero B, Emma F. Minimal Change Disease. Clin J Am Soc Nephrol. 2017;12(2):332- 345.
10. 10. Willows J, Wood K, Bourne H, Sayer JA. Acquired C1-inhibitor deficiency presenting with nephrotic syndrome. BMJ Case Rep. 2019;12(7):e230388.
11. 11. Robertson JI, Tillman DM, Ball SG, Lever AF. Angiotensin converting enzyme inhibition in hypertension. J Hypertens Suppl. 1987; 5(3):S19-25.
12. 12. Majoni SW, Smith SR. Membranous nephropathy in a patient with hereditary angioedema: a case report. J Med Case Rep. 2008; 2:328.