The Effects of Colchicine on the Histopathological Findings of Celiac Disease During Familial Mediterranean Fever Treatment.

Familial mediterranean fever (FMF) and Celiac Disease (CD) are both autoinflammatory and autoimmune diseases with common clinical features affecting the gastrointestinal system. Familial mediterranean fever may coexist with CD. Inflammatory cells and cytokines are up-regulated within the intestinal mucosa of patients with CD. In this perspective, lymphocyte infiltration to the lamina propria of the small intestinal mucosa is a critical pathological sign. In the present study, we aimed to find out whether the treatment of an FMF patient with colchicine affects the pathological signs of concomitant developed CD on the same patient. We retrospectively reviewed the medical records of 147 patients with FMF who were followed up in our center between 2015 and 2020. Of the 147 patients with FMF, 3 patients also had CD. In this report, we presented these 3 cases. The findings obtained in this study suggest that pathological findings may be misinterpreted in autoimmune diseases, such as CD, when patients administer anti-inflammatory treatments, such as colchicine.

Vitamin D levels in children with COVID-19: a report from Turkey.

Several studies have demonstrated that higher levels of vitamin D are associated with better prognosis and outcomes in infectious diseases. We aimed to compare the vitamin D levels of paediatric patients with mild/moderate coronavirus disease 2019 (COVID-19) disease and a healthy control group. We retrospectively reviewed the medical records of patients who were hospitalised at our university hospital with the diagnosis of COVID-19 during the period between 25 May 2020 and 24 December 2020. The mean age of the COVID-19 patients was 10.7 ± 5.5 years (range 1-18 years); 43 (57.3%) COVID-19 patients were male. The mean serum vitamin D level was significantly lower in the COVID-19 group than the control group (21.5 ± 10.0 vs. 28.0 ± 11.0 IU, P < 0.001). The proportion of patients with vitamin D deficiency was significantly higher in the COVID-19 group than the control group (44% vs. 17.5%, P < 0.001). Patients with low vitamin D levels were older than the patients with normal vitamin D levels (11.6 ± 4.9 vs. 6.2 ± 1.8 years, P = 0.016). There was a significant male preponderance in the normal vitamin D group compared with the low vitamin D group (91.7% vs. 50.8%, P = 0.03). C-reactive protein level was higher in the low vitamin D group, although the difference did not reach statistical significance (9.6 ± 2.2 vs. 4.5 ± 1.6 mg/l, P = 0.074). Our study provides an insight into the relationship between vitamin D deficiency and COVID-19 for future studies. Empiric intervention with vitamin D can be justified by low serum vitamin D levels.

The comparison of the resistivity index values in the ultrasonographic evaluation of a unilateral atrophic/hypoplastic kidney.

Background: In the study, we aimed to determine the sensitivity of the renal resistivity index (RI) in differentiating hypoplastic and atrophic kidneys in patients with small-sized kidneys, and to evaluate its capacity to predict the renal involvement confirmed by the DMSA scintigraphy.Material and methods: We retrospectively reviewed the ultrasonography (US) and DMSA findings, and medical records of pediatric patients with unilateral diminutive kidneys followed between January 2017 and June 2018. The RI measurements were performed twice, and the mean RI was calculated for each kidney of all patients.Results: Sixty-three (male/female, m/f = 28/35) pediatric patients aged 107.2 ± 49.4 months (range 14-206 months) were included in this study. The DMSA scintigraphy revealed abnormal changes to atrophic kidneys in 38 patients and hypoplastic kidneys in 25. There were no differences between the groups with atrophy and hypoplasia by age, gender, urine density, and creatinine. The patient group with atrophic kidneys had a mean RI of 0.55 ± 0.21, and patients with hypoplastic kidneys had a mean RI of 0.67 ± 0.03. The mean RI and systolic/diastolic rates of the patients with atrophy were significantly lower than of the patients with hypoplastic kidneys (p = 0.042 and p = 0.048, respectively). There was a positive correlation between RI and DFR in the group with atrophy (r = 0.461, p = 0.016), but this was not the case for the group with hypoplastic kidneys (r= -0.066, p = 0.889).Conclusions: The resistivity index might be very useful for differentiating atrophy and hypoplasia in patients with unilateral small kidneys and can be used instead of scintigraphic evaluation.

Role of Hemodialysis in the Management of Cyanide Intoxication From Apricot Kernels in a 3-Year-Old Child.

Cyanide (CN) is one among the most potent and rapidly acting lethal poisons, and it may cause death unless immediately diagnosed and treated. We report an unusual case of pediatric CN poisoning after ingestion of apricot kernels containing amygdalin, who survived with antidotal therapy and hemodialysis. A 3-year-old girl presented with respiratory distress and coma following tonic-clonic convulsions after ingestion of 3 apricot kernels. She had severe metabolic acidosis (pH 6.91, bicarbonate [HCO3] 5.6 mEq/L, base excess -26.0 mEq/L). Her blood CN level was measured 3.15 mg/L, 3 hours after ingestion. Hydroxocobalamin could not be administered immediately because it had to be brought from a medical center 4 hours apart. Therefore, a 3-hour hemodialysis session was carried out, following which she showed some clinical improvement. In addition, when hydroxocobalamin was obtained, it was then administered. During follow-up, she was completely asymptomatic with blood pressure, and other hemodynamic parameters normalized. This case presents hemodialysis as a way to correct metabolic derangements from CN poisoning and suggests that it may have a role in select cases of pediatric CN poisoning, especially when CN-scavenging antidotes may be unavailable.

Time-averaged hemoglobin values, not hemoglobin cycling, have an impact on outcomes in pediatric dialysis patients.

BACKGROUND: During erythropoietin-stimulating agent (ESA) treatment, hemoglobin (Hb) levels usually fluctuate; this phenomenon is known as "Hb cycling (HC)." In this study, we aimed to evaluate the predictors of HC and its impact on left ventricular hypertrophy (LVH) as a patient-important outcome parameter in pediatric dialysis patients. METHODS: Records of patients followed up in nine pediatric nephrology centers between 2008 and 2013 were reviewed. More than 1 g/dL decrease or increase in Hb level was considered as HC. Patients were divided into two groups according to 12-month Hb trajectory as rare cycling (RC) (≤ 3) and frequent cycling (FC) (> 3 fluctuation) as well as three groups based on T-A-Hb levels: < 10, 10-11, and > 11 g/dL. RESULTS: Two hundred forty-five dialysis (160 peritoneal dialysis (PD) and 85 hemodialysis (HD)) patients aged 12.3 ± 5.1 (range 0.5-21) years were enrolled in this study. Fifty-two percent of the patients had RC, 45% had FC, and only 3% had no cycling. There were no differences between HC groups with respect to age, dialysis modality, having anemia, hospitalization rate, residual urine volume, and mortality. Although left ventricular mass index (LVMI) tended to be higher in RC than FC group (65 ± 37 vs 52 ± 23 g/m2.7, p = 0.056), prevalence of LVH was not different between the groups (p = 0.920). In regression analysis, FC was not a risk factor for LVH, but low T-A Hb level (< 10 g/dL) was a significant risk for LVH (OR = 0.414, 95% CI 0.177-0.966, p = 0.04). The target Hb levels were more often achieved in PD patients, and the number of deaths was significantly lower in non-anemic patients (Hb level > 11 g/dL). CONCLUSION: Hb cycling is common among dialysis patients. Severity of anemia rather than its cycling has more significant impact on the prevalence of LVH and on inflammatory state.

Effect of the timing of dialysis initiation on left ventricular hypertrophy and inflammation in pediatric patients.

BACKGROUND: The optimal time for dialysis initiation in adults and children with chronic kidney disease remains unclear. The aim of this study was to evaluate the impact of dialysis timing on different outcome parameters, in particular left ventricular (LV) morphology and inflammation, in pediatric patients receiving peritoneal dialysis and hemodialysis. METHODS: The medical records of pediatric dialysis patients who were followed-up in nine pediatric nephrology centers in Turkey between 2008 and 2013 were retrospectively reviewed. In addition to demographic data, we retrieved anthropometric measurements, data on dialysis treatment modalities, routine biochemical parameters, complete blood count, serum ferritin, parathormone, C-reactive protein (CRP), and albumin levels, as well as echocardiographic data and hospitalization records. The patients were divided into two groups based on their estimated glomerular filtration rate (eGFR) levels at dialysis initiation, namely, an early-start group, characterized by an eGFR of >10 ml/min/1.73 m2, and a late-start group, with an eGFR of < 7 ml/min/1.73 m2. The collected data were compared between these groups. RESULTS: A total of 245 pediatric dialysis patients (mean age ± standard deviation 12.3 ± 5.1 years, range 0.5-21 years) were enrolled in this study. Echocardiographic data were available for 137 patients, and the mean LV mass index (LVMI) was 58 ± 31 (range 21-215) g/m2.7. The LVMI was 75 ± 30 g/m2.7(n = 81) and 34 ± 6 g/m2.7(n = 56) in patients with or without LV hypertrophy (LVH) (p < 0.001). Early-start (eGFR >10 ml/min/1.73 m2) versus late-start dialysis (eGFR < 7 ml/min/1.73 m2) groups did not significantly differ in LVMI and LVH status (p > 0.05) nor in number of hospitalizations. Serum albumin levels were significantly higher in the early-dialysis group compared with the late-dialysis group (3.3 ± 0.7 vs. 3.1 ± 0.7 g/dl, respectively; p < 0.05). The early-start group had relatively higher time-averaged albumin levels (3.2 ± 0.5 vs. 3.1 ± 0.5 g/dl; p = > 0.05) and relatively lower CRP levels (3.64 ± 2.00 vs. 4.37 ± 3.28 mg/L, p > 0.05) than the late-start group, but these differences did not reach statistical significance. CONCLUSION: Although early dialysis initiation did not have a significant effect on important clinical outcome parameters, including LVH, inflammatory state, and hospitalization, in our pediatric dialysis patients, this area of study deserves further attention.

Results of native and transplant kidney biopsies of children in a single center over a 15 years period.

Renal biopsy is an important diagnostic procedure in pediatric nephrology. This study retrospectively investigates the indications, results and complications in a single tertiary children's hospital in Turkey. We evaluated the native and transplant kidney biopsies in Gazi University Pediatric Nephrology Department between 2001 and 2015. A total of 196 biopsies (144 natives and 52 transplants) were included into the study. The mean age of the patients was respectively 10.8 ± 3.5, 13.9 ± 1.5 years. The main indication for a biopsy was non-nephrotic proteinuria with or without hematuria (n= 35), followed by steroid-resistance nephrotic syndrome (SRNS) (n = 34) and Henoch-Schönlein purpura (HSP)-related proteinuria (n = 20) for native kidneys. We found that focal segmental glomerulosclerosis (FSGS) (11.7%) was the most common histopathologic diagnosis for native kidneys, but acute cellular rejection (7.6%) was the most common histopathologic diagnosis for transplant kidneys. The complication rate in our study was founded 6.6% totally. Different complication rates were found in other studies; however, we think that this difference comes from the inspecting methodology of the complications. Hence, we wanted to share our own experience in the context of other studies in the literature.

The effect of antidiuretic hormone on urine and serum electrolyte levelsin children with primary monosymptomatic nocturnal enuresis

Background/aim: The data concerning the effects of desmopressin on water/electrolyte disturbances of children with primary monosymptomatic nocturnal enuresis (PMNE) are limited. In the present study we aimed to evaluate the effect and tolerability of desmopressin on blood and urine electrolytes and osmolality in PMNE.Materials and methods: Thirty-five children with PMNE between the ages of 5 and 15 participated in the study. Patients collected urine during the daytime and acknowledged the night time fluid restriction before starting to use the desmopressin tablets. The medication was taken orally at least 1 h before bedtime. Blood and urine samples were collected before the introduction of the treatment (day 0) and on the third and seventh days of the administration of desmopressin to determine osmolality and electrolyte levels.Results: Thirty-five patients participated in the study. Twenty-one patients (60%) were male and 14 (40%) were female. The mean age was 9.6 - 2.7 years. There were no significant changes in serum osmolality, urine osmolality, and serum sodium concentration. Mean urine calcium/creatinine ratio was 0.03 - 0.01 mg/mg at the beginning, 0.06 - 0.02 mg/mg on the third day, and 0.04 - 0.01 mg/mg on the seventh day of the study. No significant changes were seen in urine calcium/creatinine ratio before and after treatment.Conclusion: Desmopressin appeared to be a well-tolerated drug and provided a safe and effective treatment for children who were following fluid intake restriction for PMNE.

Familial Mediterranean fever gene mutation frequencies in a sample Turkish population.

OBJECTIVES: Our knowledge about the frequencies of mutations in the Turkish population is based on the studies on the affected patients and hospital-based control groups. We aimed to determine the frequencies of MEFV gene mutations in a population-based field study in Turkey. METHODS: Turkish citizens aged between 5 and 65 years were included in the study. Cities from seven regions of Turkey were studied. Blood samples were obtained from individuals who gave permission for laboratory experiments, and they were analysed for 10 MEFV gene mutations. RESULTS: Among 500 participants, MEFV mutations were found in 74 (14.8%). Sixty four (12.8%), 7 (1.4%), and 3 (0.6%) participants were heterozygous, compound heterozygous, and homozygous, respectively. Among inhabitants with heterozygous mutations, the most common heterozygous mutations were E148Q/- and M694V/-. Sixteen participants were found to be heterozygous for M694V, 2 were compound heterozygous for M694V/E148Q, and one was homozygous for M694V/M694V mutation; in total, the frequency of M694V allele was 4% (n=20). Twenty-three (4.6%) individuals were heterozygous for common mutations (M694V, M680I, V726A). Total allelic frequency was 8.4%. CONCLUSIONS: Our study, which describes the MEFV mutational spectrum and distribution in a healthy Turkish population, found a carrier rate that is much higher than expected.

Evaluation of urinary KIM-1, NGAL, and IL-18 levels in determining early renal injury in pediatric cases with hypercalciuria and/or renal calculi.

AIM: One has to measure urinary enzymes and proteins to determine renal dysfunction and tubular injury in earlier stage during the course of disease. The aim of this study was to investigate the role of urinary NGAL (neutrophil gelatinase-associated lipocalin), IL-18 (Interleukin 18), and KIM-1 (kidney injury molecule-1) levels in determining early renal injury in pediatric patients with hypercalciuria (HC) and/or nephrolithiasis (NL) and to compare the levels of these markers between the sick and healthy subjects. MATERIAL AND METHODS: Urinary NGAL, KIM- 1, and IL- 18 levels were measured in 40 pediatric patients diagnosed with NL, 23 patients with HC, and 20 healthy controls. RESULTS: A significant difference was found between patient groups (NL and HC) and healthy children with respect to urinary NGAL/cr ratio (p < 0.001). There were no significant differences between patient and control groups with respect to urinary IL18/cr and KIM1/cr ratios. CONCLUSIONS: We found that urinary NGAL is a useful marker for determining renal tubular damage in NL and HC. To our knowledge, this is the first study to report significantly increased urinary NGAL levels in NL and HC.

Familial Mediterranean fever with a single MEFV mutation: comparison of rare and common mutations in a Turkish paediatric cohort.

OBJECTIVES: Presence of common MEFV gene mutations strengthened the diagnosis of FMF in addition to the typical clinical characteristics of FMF. However, there are also rare mutations. P369S, A744S, R761H, K695R, F479L are the main rare mutations in Turkish population. We aimed to evaluate FMF patients with a single allele MEFV mutation and to compare patients with common and rare mutations. METHODS: We retrospectively reviewed the medical records of FMF patients with a single allele mutation who were followed up between 2008 and 2013 in six centres. We compared the patients with rare and common mutations for disease severity score, frequent exacerbations ( >1 attack per month), long attack period (>3 day), symptoms, age at the onset of symptoms, gender, consanguinity, and family history. RESULTS: Two hundred and seventeen patients (M/F=101/116) with the diagnosis of FMF and single mutation were included. Heterozygote mutations were defined as common (M694V, V726A, M68OI) and rare mutations (A744S, P369S, K695R, R761H, F479L). Sixty-seven patients (27 males, 40 females) had one single rare mutation and 150 (74 males, 76 females) had one single common mutation. No difference was found between the rare and common mutations with respect to the disease severity score. There was no significant difference between common and rare heterozygote form of mutations in terms of disease severity. CONCLUSIONS: Patients with typical characteristics of FMF, with some rare mutations (A744S, P369S) should be treated in the same manner as patients with a common mutation.

The evaluation of bone metabolism in children with renal transplantation.

This study aims to evaluate BMD and bone biomarkers and to investigate the effects of immunosuppressives on bone disease after RTx. Thirty-three RTR aged 16.7 ± 3.7 yr and healthy controls (n = 32) were enrolled. There was no difference between pre-RTx BMD and BMD at the time of study (45.9 ± 30.9 months after RTx), while both values were lower than controls (p < 0.01 and p < 0.05, respectively). Worst BMD scores were obtained at sixth month after RTx (-0.2 ± 0.9) and best at fourth year (1.4 ± 1.3). 25-hydroxy-(OH) vitamin D and OPG were higher in RTR (p < 0.001). BMD z scores negatively correlated with OPG and cumulative CS doses at the time of study (r = -0.344, p < 0.05 and r = -0.371, p < 0.05, respectively). Regression analysis revealed OPG as the only predictor of BMD (ß -0.78, 95% CI -0.004 to -0.013, p < 0.001). The increase in OPG, a significant predictor of BMD, could either be secondary to graft dysfunction or for protection against bone loss. CS doses should be minimized to avoid their untoward effects on bone metabolism.

Clinical and Cytokine Profile of Children With COVID-19: A Report From Turkey.

Background We aimed to analyze the expression of infection-related biomarkers and inflammatory cytokines in laboratory-confirmed cases and compare the differences between clinically severe and non-severe ones. Method We randomly selected 35 patients who were hospitalized with the diagnosis of coronavirus disease 2019 (COVID-19). Blood serum was obtained at the time of admission to the hospital, on the third to the fifth day, and at the time of discharge. Result The median age of our patients was 56.5±69.7 months (range: 1-205 months). The mean pro-B-type natriuretic peptide (pro-BNP) was significantly higher at the time of admission than on the third to the fifth day of illness. The mean pro-B-type natriuretic peptide levels at three time points were significantly higher in patients with severe cases than in mild-moderate cases. However, there was no significant difference between the clinical severity with regard to the cytokine levels at disease onset and recovery. Conclusion In the study, it was shown that cytokines play an important role in the pathogenesis of COVID-19. Therefore, it may be beneficial to use agents such as tocilizumab in the treatment.

Clinical significance of MEFV gene variation R202Q.

BACKGROUND: The aim of this study was to evaluate the phenotypic features and the clinical significance of the R202Q mutation of the MEFV gene. METHODS: We retrospectively reviewed the medical records of Familial Mediterranean Fever patients with M694V/- and M694V/R202Q mutations. We compared the patients regarding disease severity, symptoms, age at the onset of symptoms, gender, consanguinity, and family history. RESULTS: Twenty-one patients (9 males, 12 females) had compound heterozygote mutation (M694V/R202Q), and 37 patients (23 males, 14 females) had M694V/- mutation. The mean age of the patients at the time of diagnosis was 7.3 ± 4.3 and 9.2 ± 3.7 years. The rate of arthritis was significantly higher in patients with M694V/R202Q heterozygote mutation than those with M694V/- heterozygote mutation (76.2% vs 32.4%; p = < 0.001). The mean severity score was higher in M694V/R202Q heterozygote group although it did not reach statistical significance (8.43 ± 1.69 vs 7.49 ± 1.50; p = 0.082). However, the rate of having a high severity score was significantly higher in the M694V/R202Q mutation group than in the other group (47.6% vs 21.6%, respectively; p = 0.039). The rate of arthritis was significantly higher in patients with M694V/R202Q heterozygote mutation than those with M694V/- heterozygote mutation (76.2% vs 32.4%; p = < 0.001). CONCLUSION: Our finding supports the possibility that R202Q may be pathogenic rather than a variation. We found that the R202Q mutation is associated with the inflammatory phenotype of FMF; hence, the typical clinical findings of FMF especially arthritis can be observed in patients with compound mutation including R202Q. Key Points • We found that the R202Q mutation is associated with the inflammatory phenotype of FMF • The patients with the R202Q mutation had a greater rate of arthritis symptoms.

Evaluation of oxidative stress biomarkers in acute mercury intoxication.

INTRODUCTION: Very few studies have evaluated the association between mercury exposure and oxidative stress in humans, particularly in children. AIM: This is the first report where we aimed to determine the oxidative stress status of children who were accidentally exposed to elemental mercury. MATERIALS AND METHODS: In the present study, the study group was composed of 86 randomly selected children poisoned by mercury; the control group was composed of 78 children who had no history of mercury exposure. At admission, blood samples were collected. Blood superoxide dismutase activity, catalase enzyme activity, and glutathione peroxidase activity were measured by Fridovich, Beutler, and Lawrence Burk methods respectively, and the results were given as U/g Hb. Malondialdehyde level was measured by Ohkawa methods, and the results were given as mmol/ml. RESULTS: Catalase activity was significantly lower in the patient group compared to the control group (1.28±0.62 vs. 3.90±0.86 U/g Hb, p=0.010). In exposed children, SOD activity was significantly higher than the controls (5936±810 vs. 2226±464 U/g Hb, p=0.03), while the GSH-Px activity was significantly lower (13.01±3.21 vs. 34.97±7.32 U/g Hb, p=0.013). The MDA levels of the mercury group were significantly higher than the MDA levels of the control group (2.85±0.84 vs. 2.05±0.79 mmol/ml, p=0.04). CONCLUSIONS: The results of the present study showed that acute mercury poisoning causes an alteration of oxidative stress status in children exposed to elemental mercury.

Evaluation of Oxidative Stress Biomarkers in Patients with Henoch-Schönlein Purpura.

INTRODUCTION: Henoch-Schönlein Purpura (HSP) is a systemic vasculitic syndrome characterized by non-thrombocytopenic purpura, arthritis/arthralgia, abdominal pain, and glomerulonephritis. The pathogenesis of HSP has not been clearly identified. Oxidative damage has a role in the pathogenesis of most cases. AIM: This study aimed to evaluate changes of oxidative stress by studying parameters like superoxide dismutase (SOD), catalase (CAT), and malondialdehyde (MDA) in an attempt to identify the role of oxidative stress in HSP from another perspective. MATERIALS AND METHODS: This study enrolled 23 pediatric patients (ten girls and thirteen boys) diagnosed with HSP who were under follow-up at Sutcu Imam University School of Medicine Department of Pediatrics between 2014 and 2016 and twenty healthy children as the control group. The parents of all subjects gave informed consent to participate in the study. In the HSP group, the beginning season of the illness and the systemic involvement during follow-up were determined. Blood specimens were obtained at presentation before any treatment was started. SOD, CAT activities, and MDA values in erythrocyte and plasma samples were compared between the patient group and the healthy children. RESULTS: Twenty-three patients with HSP (13 males, 10 females) and 20 healthy children participated in this study. The mean age of the HSP cases was 8.21±3.78 years (range 2-16 years) and of the controls was 8.6±4.2 (range 3-14 years). The mean MDA value was 2.95±0.71 nmol/ml in the patient group and 2.67±0.66 nmol/ml in the control group (p=0.787). The mean level of the CAT enzyme was 1.32±0.35 U/g Hb in the patient group and 7.8±1.74 U/g Hb in the control group (p=0.001). The mean levels of the SOD enzyme were 3.06±0.85 U/g Hb in the patient group and 0.97±0.36 U/g Hb in the control group (p=0.001). CONCLUSIONS: Although high MDA levels support the role of lipid peroxidation in the pathogenesis of HSP, statistical significance was not reached owing to a limited number of our patients. The reduced CAT enzyme activity is consistent with the findings of previous reports. This finding supports the notion that oxidative stress can play a role in the pathogenesis of HSP. KEYPOINTS: Our findings support the notion that oxidative stress can play a role in the pathogenesis of HSP.

Thrombotic Microangiopathy in a Severe Pediatric Case of COVID-19.

In this case report, we report a pediatric patient with COVID-19 and atypical hemolytic uremic syndrome (aHUS). A 3-year-old girl with fever and respiratory distress was admitted to the hospital. The patient tested positive for COVID-19 by a PCR test. As her respiratory distress increased and blood gas indicated deep respiratory acidosis on the third day of the pediatric intensive care unit follow-up, the patient was intubated and ventilated. Thorax computerized tomography (CT) showed bilateral effusion and atelectasis. During her follow-up, the pleural effusion resolved but there were marked consolidation areas and ground glass opacities compatible with COVID-19 on the follow-up CT. On the 10th day, she became anuric and developed progressive thrombocytopenia and persistent microangiopathic hemolytic anemia, which were suggestive of HUS given a high creatinine level (1.9 mg/dl), an undetectable haptoglobin level, reticulocytosis (8%), and an LDH level of 2540 U/l. Direct Coombs test returned negative. Examination of a peripheral blood smear revealed schistocytes. Disseminated intravascular coagulation was ruled out by normal INR and PTT. In the light of the available findings, we considered the patient to have thrombotic microangiopathy (TMA) triggered by COVID-19. It was not a TTP-form of TMA but rather an aHUS type, based on an ADAMTS13 activity level >5%. Hence, plasmapheresis was performed with fresh frozen plasma (FFP). After 4 weeks, she became completely asymptomatic, and her hemodynamic parameters normalized. COVID-19 induced uncontrolled complement activation leading to the development of aHUS. Early diagnosis and treatment may reduce morbidity and mortality since its treatment options.

Assessment of Children Exposed to Maras Powder Intoxication.

INTRODUCTION: A smokeless tobacco known as Maras powder (MP) is commonly consumed in the southern region of Turkey. To the extent of our knowledge, no previous study in literature has reported acute MP intoxication in children. AIM: Our aim was to determine the clinical effects and treatment strategies of MP poisoning in children.Materials and methods: We retrospectively reviewed the medical records of children <18 years of age with MP intoxication who were followed up in the Intensive Care Unit between January 2016 and April 2018 in our center. RESULTS: Forty-one patients (M/F= 25/16) were included in this study. The mean age was 13.2±22.4 months (age range: 7 to 30 months). The patients presented with vomiting (n=23, 56%), cough (n=17, 41.5%), loss of consciousness (n=11, 26.8%), respiratory distress (n=6, 14.6%), convulsion (n=7, 17%), cyanosis (n=1, 2.4%), and abdominal pain (n=1, 2.4%) following oral ingestion of the substance. On their physical examination all patients with convulsion were in a comatose state. Thirty-two patients (78%) had tachycardia; 15 patients (36.5%) had pharyngeal hyperemia; and three (7.3%) had hiccups. Although the female patients had a lesser rate of symptoms than male patients, the difference was not statistically significant. CONCLUSIONS: Our aim was to inform the doctors about the clinical picture that develops after taking this substance and contribute to the understanding of the treatment approach.

An adolescent case of extensive Behçet`s disease successfully treated with Infliximab.

Isiyel E, Bakkaloglu S, Oguz D, Yenicesu I, Boyunaga Ö, Özdemir Y, Damar Ç, Kandur Y, Akçaboy M, Aslan AT, Sismanlar T, Hasanoglan E, Buyan N. An adolescent case of extensive Behçet`s disease successfully treated with Infliximab. Turk J Pediatr 2019; 61: 585-588. Cardiac involvement is an uncommon and life-threatening complication of Behçet`s Disease. We present a 14-year-old boy, admitted to our hospital for recurrent hemoptysis. In his radiologic evaluation, a right ventricular thrombus and pulmonary arterial aneurysm were identified. He was diagnosed with Behçet`s Disease, and then he received prednisone and cyclophosphamide. However, his cardiac thrombus enlargened. After his treatment was replaced with infliximab, the pulmonary aneurysms regressed, and the cardiac thrombus disappeared. In conclusion, infliximab should be considered as a reliable option for vascular Behçet`s Disease resistant to conventional treatment.