Publication: Hemolitik Üremik Sendromlu Pediatrik Hastaların Klinik ve Laboratuvar Bulgularının Akut ve Uzun Dönem Sonuçları Üzerine Etkisi
Abstract
ÖZET Amaç: Ondokuz Mayıs Üniversitesi Tıp Fakültesi Çocuk Sağlığı Hastalıkları ABD, Çocuk Nefroloji BD'da Hemolitik Üremik Sendrom (HÜS) tanısı alan olguların klinik ve laboratuvar bulgularının akut ve uzun dönem sonuçları üzerine etkisini incelemek amacıyla yapıldı. Gereç ve Yöntem: Çalışmamızda Temmuz 2009 ile Temmuz 2023 tarihleri arasında kliniğimizde Hemolitik Üremik Sendrom tanısı alan 18 yaş altı 45 olguyu inceledik. Olguların başvuru anında ve klinik takibindeki bulguları, laboratuvar parametreleri, tedavi ve prognozlarını inceledik. Bulgular: Çalışmamızda 33 hasta (%73,3) ishal ilişkili HÜS, 12 hasta (%27,7) ise atipik HÜS olarak değerlendirilmiştir. Vakaların yaş ortalaması 3,3 ±3,6 yıl olup (0-16 yıl arası), 22'si (%48,9) kız, 23'ü (%51,1) erkekti. Prodromal ishal süresi 2-10 gün arasında değişmekle birlikte ortalama 5 gün, STEC HÜS olgularında ise ortalama 3,7 gündür (2-5 gün arası ). Vakaların %73,3'ünde prodromal ishal, %66,7'sinde ise kanlı ishal (tüm vakaların %48'i) tespit edilmiştir. Oligoanüri oranı genel olarak %88,9 iken, tipik HÜS olgularında %88, atipik HÜS olgularında %91,7 olarak bulunmuştur. Oligoanüri süresi tipik HÜS olgularında 2-26 gün, atipik HÜS olgularında ise 3-14 gün arasında değişmektedir. Dışkı kültürü alınan 34 hastada üreme saptanmamış, STEC tayini yapılan 15 hastadan 5'inde (%33,3) pozitiflik bulunmuştur. Nörolojik bulgular, tipik HÜS olgularının 9'unda (%27,3) nöbet ve bilinç değişikliği olarak gözlenmiştir. Hipertansiyon, tipik HÜS olgularının 14'ünde (%42,4), atipik HÜS olgularının ise 6'sında (%50) saptanmıştır. Hastaların tedavisinde, 31 hastada (%68,9) diyaliz, 8 hastada (%17,8) destek tedavisi ve 6 hastada (%13,3) taze donmuş plazma uygulanmıştır. Toplam 33 hastaya (%73,3) diyaliz tedavisi uygulanmış, bunların 20'si (%60,6) periton diyalizi, 13'ü (%39,4) ise hemodiyaliz almıştır. Ayrıca 7 hastaya (%15,6) plazma değişimi, 22 hastaya (%48,9) taze donmuş plazma, 14 hastaya (%31,1) ise eculizumab tedavisi verilmiştir. Eculizumab, tipik HÜS tanılı 9 (%27,3) ve atipik HÜS tanılı 5 (%41,7) hastada kullanılmıştır. Takiplerde bir hastaya KBY nedeniyle böbrek nakli yapılmış, iki hasta halen eculizumab tedavisi almaktadır. Bir hastamız, akut dönemde gelişen intrakraniyal kanama sonucu kaybedilmiştir. Hastaların hiçbirinde antihipertansif kullanımına ihtiyaç duyulmamış ve izlem sırasında proteinüri tespit edilmemiştir. Sonuç: HÜS olgularımızın demografik ve klinik özellikleri, tipik ve atipik formlar arasında farklılık göstermektedir. Tipik HÜS daha çok küçük yaşlarda, atipik HÜS ise daha geniş bir yaş aralığında izlenmiştir. Tipik HÜS'te prodromal kanlı ishal ve nörolojik tutulum daha sık görülürken, atipik HÜS'te anüri süresi daha uzundur. Olguların üçte birinde STEC pozitifliği tespit edilmiştir; ancak antibiyotik kullanımı bu oranı etkileyebilmektedir. Sonuç olarak, HÜS'ün erken tanı ve tedavisi, komplikasyonları önlemek için kritik olup, atipik vakalarda uzun dönem izlem önemlidir. Anahtar Kelimeler: Hemolitik Üremik Sendrom, Prodromal İshal, Oligoanüri , STEC HÜS , Eculizumab.
ABSTRACT Purpose: The aim of this study was to investigate the effect of clinical and laboratory findings on acute and long-term outcome of patients diagnosed with haemolytic uremic syndrome (HUS) in Ondokuz Mayıs University Faculty of Medicine, Department of Paediatrics, Division of Paediatric Nephrology. Materials and Methods: In our study, we examined 45 patients under the age of 18 who were diagnosed with Haemolytic Uremic Syndrome in our clinic between July 2009 and July 2023. We examined the findings, laboratory parameters, treatment and prognosis of the patients at the time of presentation and during clinical follow-up. Results: In our study, 33 patients (73.3%) were diagnosed with diarrhea-associated HUS (D+ HUS), and 12 patients (27.7%) with atypical HUS (aHUS). The mean age of the patients was 3.3 ± 3.6 years (range: 0–16 years), with 22 (48.9%) female and 23 (51.1%) male patients. The duration of the prodromal diarrhea ranged from 2 to 10 days, with an average of 5 days; in STEC-HUS cases, the average duration was 3.7 days (range: 2–5 days). Among all patients, 73.3% had prodromal diarrhea, and 66.7% presented with bloody diarrhea, which accounted for 48% of the total cases. The overall rate of oliguria/anuria was 88.9%, with 88% of patients with D+ HUS and 91.7% of those with aHUS experiencing oliguria/anuria. The duration of oliguria ranged from 2 to 26 days in D+ HUS patients and from 3 to 14 days in aHUS patients. Stool cultures were performed in 34 patients, with no pathogens isolated. STEC identification was conducted in 15 patients, and 5 (33.3%) tested positive. Neurological symptoms, including seizures and altered mental status, were observed in 9 (27.3%) patients with D+ HUS. Hypertension was detected in 14 (42.4%) of the D+ HUS cases and 6 (50%) of the aHUS cases. Regarding treatment, dialysis was initiated as the first-line therapy in 31 patients (68.9%), supportive therapy in 8 patients (17.8%), and fresh frozen plasma (FFP) therapy in 6 patients (13.3%). In total, 33 patients (73.3%) underwent dialysis, including 20 (60.6%) who received peritoneal dialysis and 13 (39.4%) treated with hemodialysis. Plasma exchange was performed in 7 patients (15.6%), FFP was administered to 22 patients (48.9%), and eculizumab therapy was initiated in 14 patients (31.1%). Specifically, eculizumab was administered to 9 (27.3%) patients with D+ HUS and 5 (41.7%) patients with aHUS. During follow-up, one patient underwent renal transplantation due to end-stage kidney disease, and two patients remain on eculizumab therapy. One patient died due to intracranial hemorrhage in the acute phase, resulting in a deterioration of the general condition. None of the patients required antihypertensive therapy, and no proteinuria was observed during follow-up. Conclusıon: The demographic and clinical characteristics of our HUS cases differ between typical and atypical forms. Typical HUS predominantly occurs in younger children, whereas atypical HUS is observed across a broader age range. Prodromal bloody diarrhea and neurological involvement are more common in typical HUS, while the duration of anuria is longer in atypical HUS. STEC positivity was identified in one-third of the cases; however, prior antibiotic use may have affected this rate. In conclusion, early diagnosis and treatment of HUS are crucial for preventing complications, with long-term follow-up being important in atypical cases. Keywords: Haemolytic uremic syndrome, prodromal diarrhoea, oligoanuria, STEC HUS, Eculizumab.
ABSTRACT Purpose: The aim of this study was to investigate the effect of clinical and laboratory findings on acute and long-term outcome of patients diagnosed with haemolytic uremic syndrome (HUS) in Ondokuz Mayıs University Faculty of Medicine, Department of Paediatrics, Division of Paediatric Nephrology. Materials and Methods: In our study, we examined 45 patients under the age of 18 who were diagnosed with Haemolytic Uremic Syndrome in our clinic between July 2009 and July 2023. We examined the findings, laboratory parameters, treatment and prognosis of the patients at the time of presentation and during clinical follow-up. Results: In our study, 33 patients (73.3%) were diagnosed with diarrhea-associated HUS (D+ HUS), and 12 patients (27.7%) with atypical HUS (aHUS). The mean age of the patients was 3.3 ± 3.6 years (range: 0–16 years), with 22 (48.9%) female and 23 (51.1%) male patients. The duration of the prodromal diarrhea ranged from 2 to 10 days, with an average of 5 days; in STEC-HUS cases, the average duration was 3.7 days (range: 2–5 days). Among all patients, 73.3% had prodromal diarrhea, and 66.7% presented with bloody diarrhea, which accounted for 48% of the total cases. The overall rate of oliguria/anuria was 88.9%, with 88% of patients with D+ HUS and 91.7% of those with aHUS experiencing oliguria/anuria. The duration of oliguria ranged from 2 to 26 days in D+ HUS patients and from 3 to 14 days in aHUS patients. Stool cultures were performed in 34 patients, with no pathogens isolated. STEC identification was conducted in 15 patients, and 5 (33.3%) tested positive. Neurological symptoms, including seizures and altered mental status, were observed in 9 (27.3%) patients with D+ HUS. Hypertension was detected in 14 (42.4%) of the D+ HUS cases and 6 (50%) of the aHUS cases. Regarding treatment, dialysis was initiated as the first-line therapy in 31 patients (68.9%), supportive therapy in 8 patients (17.8%), and fresh frozen plasma (FFP) therapy in 6 patients (13.3%). In total, 33 patients (73.3%) underwent dialysis, including 20 (60.6%) who received peritoneal dialysis and 13 (39.4%) treated with hemodialysis. Plasma exchange was performed in 7 patients (15.6%), FFP was administered to 22 patients (48.9%), and eculizumab therapy was initiated in 14 patients (31.1%). Specifically, eculizumab was administered to 9 (27.3%) patients with D+ HUS and 5 (41.7%) patients with aHUS. During follow-up, one patient underwent renal transplantation due to end-stage kidney disease, and two patients remain on eculizumab therapy. One patient died due to intracranial hemorrhage in the acute phase, resulting in a deterioration of the general condition. None of the patients required antihypertensive therapy, and no proteinuria was observed during follow-up. Conclusıon: The demographic and clinical characteristics of our HUS cases differ between typical and atypical forms. Typical HUS predominantly occurs in younger children, whereas atypical HUS is observed across a broader age range. Prodromal bloody diarrhea and neurological involvement are more common in typical HUS, while the duration of anuria is longer in atypical HUS. STEC positivity was identified in one-third of the cases; however, prior antibiotic use may have affected this rate. In conclusion, early diagnosis and treatment of HUS are crucial for preventing complications, with long-term follow-up being important in atypical cases. Keywords: Haemolytic uremic syndrome, prodromal diarrhoea, oligoanuria, STEC HUS, Eculizumab.
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