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Abstract Background This study aimed to determine incidence of kidney complications in pediatric allogeneic hematopoietic stem cell transplantation (HSCT) patients.MethodsPediatric allogeneic HSCT patients were included. Post-transplantation urinary system complications were collected from medical records and glomerular filtration rates at last visit compared with clinical parameters. Additionally, 24-h ambulatory blood pressure monitoring was performed.ResultsThe study included 165 pediatric patients. Acute kidney injury (AKI) developed in 125 (75.8%) patients of whom 54 (43.2%) had stage 1, 36 (28.8%) stage 2, and 35 (28%) stage 3 AKI. Primary malignant disease and viral infection post-HSCT were associated with increased risk of AKI (OR: 4; 95%CI: 1.2–13, p = 0.022 and OR: 2.9; 95%CI: 1.2–6.8, p = 0.014, respectively). Mean duration of post-HSCT follow-up was 4.4 ± 2.5 years, during which time 8 patients had chronic kidney disease (CKD) (stage 1, 4 patients; stage 2, 3 patients; stage 3, 1 patient). CKD incidence was higher in patients in whom stem cell product was bone marrow + cord blood and mobilized peripheral blood, compared to bone marrow alone (40–37.5% versus 5.1%, p = 0.002). Based on 24-h ABPM, 14.7% and 7.4% of patients with normal office blood pressure had pre-hypertension and hypertension, respectively. In patients with albuminuria/severe albuminuria, daytime and nighttime systolic SDS scores were higher than those without albuminuria/severe albuminuria (p = 0.010 and p = 0.004, respectively).ConclusionsIncidence of AKI is higher in pediatric HSCT patients with primary malignant disease and those with documented viral infection. Our study highlights the beneficial role of 24-h ABPM as a routine part of standard care of pediatric HSCT recipients.

The prognostic factors and outcomes of Turkish children with newly diagnosed acute lymphoblastic leukemia (ALL), treated with the Modified St. Jude Total XV Protocol, which was adjusted by adding high-dose methylprednisolone (HDMP) before induction in the original protocol, were assessed in this study. The Modified St. Jude Total XV Protocol was administered to 183 newly diagnosed ALL patients, aged 1-18 years, between 1 January 2008 and 30 January 2016. HDMP was applied at doses of either 10 mg/kg/day (Group A) or 20 mg/kg/day (Group B) for 7 days before induction and then tapered over the next 7 days to 5 or 10 mg/kg/day, and continued at 2 mg/kg/day for 2 weeks during the induction phase. Absolute blast count (ABC) in peripheral blood and minimal residual disease (MRD) in bone marrow were assessed at the end of the initial 7-day HDMP treatment. MRD in the bone marrow was evaluated on day 15 and at the end of the induction period. The follow-up for these patients ended on 15 July 2019. The 5-year event-free (EFS) and overall survival (OS) rates for all patients were 85.6±2.6% and 89.2±2.3%, respectively. The rate of good response to steroids (defined as ABC in peripheral blood of less than 1000/mm3 on day 7) was 88% and 97% of children achieved complete remission after induction. The survival rate and infection frequency did not show statistically significant differences between Group A and B. EFS and OS correlated with initial leukocyte count, age of 10-18 years at diagnosis, CD20 positivity at diagnosis, and gram-negative bacterial infection during remission induction. The remarkable response rates on days 7 and 15, along with the promising EFS and OS results in childhood ALL patients treated with the Modified St. Jude Total XV Protocol, highlight the early and substantial response effect of HDMP. At the onset of induction, short-term HDMP can be initiated, preferably at 10 mg/kg/day for the first 7 days, to minimize potential side effects.

Fanconi anemia (FA) is an inherited disease, characterized by congenital malformations, short stature, progressive bone marrow failure and predisposition to leukemia and solid tumors. The aim of this study was to evaluate the clinical and prognostic features of FA patients followed in a single center. The charts of FA patients were reviewed 35 years retrospectively and a total of 175 patients were included in the study in which 51.4% of patients were male. The mean age at diagnosis was 6.3±4.1 years. The incidence of microcephaly was 92.6%, skin findings were 88.0%, eye abnormality was 74.3%, thumb and radius abnormality was 53.1%, urinary system abnormality was 30.9%, skeletal system abnormality other than thumb and radius was 18.9%, genital system abnormality was 11.4%, cardiovascular system abnormality was 11.4%, ear and hearing abnormalities were 9.7% and gastrointestinal system abnormality was 5.7%. Short stature was present in 75.4% of the patients. Of the 175 patients 167 (95.4%) developed bone marrow failure during follow-up and the mean age of bone marrow failure was 7.1 ±3.7 years (1 month-old-19.8 years). The first clinical symptom was thrombocytopenia in 83.4% of patients. Malignancy developed in a total of 23 (13.1 %) patients (20 leukemia, 3 solid tumors) during follow-up. Of 175 patients, 35 (20%) underwent hematopoietic stem cell transplantation. Fatality rate among patients who underwent hematopoietic stem cell transplantation was 31.4% (11/35) and fatality rate among other patients was 63.4% (83/131; p < 0.05). Of 94 patients who deceased, death was due to bleeding in 44.7%, infection in 34%, leukemia progression in 16.0% and graft versus host disease in 5.3%. In terms of the number of patients included, this study is one of the largest cohorts with a remarkable duration of follow-up time. Hematopoietic stem cell transplantation was found to have a good impact on sur vival of patients.

Pediatric data about early or long-term pulmonary complications of hematopoietic stem cell transplantation (HSCT) are limited. Here we aimed to evaluate children who were treated with HSCT in the last 10 years and developed pulmonary complications following HSCT and to determine their risk factors associated with pulmonary complications. In this retrospective study, we evaluated 195 patients for the development of pulmonary complications after HSCT within a 10 years of period. Pulmonary complications developed in 71 (36.4%) patients. Of the 71 patients who had pulmonary complications, 60 had one pulmonary complication, 11 had two pulmonary complications. Pulmonary complications were diagnosed as early in 42 (51.2%) and late in 40 (48.8%) episodes. Pulmonary complications were infectious in 28 (34.1%), noninfectious in 20 (24.4%) and both infectious and nonfectious in 34 (41.5%) episodes. Pulmonary complications developed significantly more frequently in patients with malignancy, congenital immune deficiency and with at least one pulmonary disease before HSCT. The number of patients who had myeloablative conditioning regimen was significantly higher in the group of patients without pulmonary complications than the group with pulmonary complications. However, in multivariate analysis, none of these variables were shown to be effective in predicting pulmonary complications after HSCT (p > 0.05). During follow up, 54 (65.8%) episodes recovered, 20 (24.3%) episodes resulted with death due to pulmonary complications, 6 (7.3%) episodes had chronic pulmonary disease (bronchiolitis obliterans (BO) and bronchiolitis obliterans organizing pneumonia (BOOP)); 2 patients (each patient with one episode) were lost to follow up. In conclusion; identifying children who are at risk for severe pulmonary complications and close longitudinal follow-up after HSCT by pediatric pulmonologists is mandatory to increase survival and life quality of these patients.

Uric acid (UA), a known danger signal released from injured cells, is a valuable sign of inflammation. We aimed to evaluate the association of serum UA levels before the start of conditioning regimens with the risk of hepatic sinusoidal obstruction syndrome (SOS) development after hematopoietic stem cell transplantation (HSCT). Two hundred and twenty-two children who underwent allogeneic HSCT at the Pediatric BMT Unit of Hacettepe University between 2000 and 2014 were included in this retrospective study. Serum UA levels were measured before conditioning as an indicator of the pre-transplant inflammatory status of the patients. Patients with and without a diagnosis of SOS were compared regarding primary diagnosis, previously described risk factors for SOS, and pre-conditioning serum UA. SOS was diagnosed in 42 patients who had higher pre-conditioning serum UA levels compared to those who did not. Pre-transplant serum creatinine, gamma-glutamyl transferase, bilirubin, ferritin, and C-reactive protein levels did not differ significantly among patients with and without SOS; however, serum albumin was lower in the patients who developed SOS. Receiver operating characteristic analysis revealed that a pre-conditioning UA level higher than 3.32 mg/dL was predictive of SOS. When applied to a multivariate model, only pre-conditioning UA and albumin levels remained significant risk factors for SOS (UA: odds ratio [OR], 2.54; 95% confidence interval [CI], 1.26-5.12, p=0.009; albumin: OR, 0.45, 95% CI, 0.22-0.95, p=0.037). Our results suggest that pre-conditioning serum UA is an independent risk factor for SOS, and it might be used as an early predictor of hepatic SOS together with previously described clinical and laboratory parameters.

Background The aim of this study was to describe the incidence and contributory risk factors for thromboembolic complications in children with nephrotic syndrome (NS) and thrombosis. Methods Among 188 children with the diagnosis of NS (80 girls; mean age: 12.6 ± 5.4 years) followed up in our hospital for the last 5 years, 17 (9.0 %) children (16 boys) identified as having thromboembolic complications. All 17 children with NS and thrombosis were screened for laboratory risk factors for thrombosis. The diagnosis was confirmed by cranial magnetic resonance imaging, doppler ultrasonography, and echocardiography. Results Among 17 children with thrombosis, 14 (82.3 %) were found to have focal segmental glomerulosclerosis (FSGS) as underlying pathology by renal biopsy. The mean age of the thrombotic children was 4.5 ± 3.2 years at the diagnosis of NS and that was 7.1 ± 4.9 years at the time of thrombosis. The mean time from NS diagnosis to the first thrombosis development was 2.6 ± 2.3 years. Thrombosis occurred during the first year of NS in 9/17 (52.9 %) children. Most of the children (88.2 %) had venous thrombosis. Among the screened risk factors, high factor VIII level (64.7 %) was the leading factor followed by decreased antithrombin III level (29.4 %). Furthermore, 4 children had central venous catheters and 2 had infection as clinical risk factors for thrombosis. Conclusion In this case series, subtype of FSGS, active disease state of NS, central venous catheters, and some inherited and acquired thrombotic risk factors have been identified as contributory factors for the development of thrombosis in children with NS.

Hematopoietic stem cell transplantation (HSCT) is the only curative treatment for many hematological disorders, primary immunodeficiencies, and metabolic disorders. Thyroid dysfunction is one of the frequently seen complications of HSCT. However, hyperthyroidism due to Graves' disease, autoimmune thyroiditis, and thyrotoxicosis are rare. Herein, we report a series of 4 patients who were euthyroid before HSCT but developed hyperthyroidism (3 of them developed autoimmune thyroid disease) after transplantation.