Acute lymphoblastic leukemia (ALL) is the most common malignancy of childhood.Central nervous system is the most frequent place of both at the onset and relapse of the disease, but orbital involvement is rarely seen.A seven-year-old girl diagnosed with high risk ALL and Turkish ALL-Berlin Frankfurt Münster (BFM)-2000 high risk group treatment was given.However, after induction and consolidation treatments, before the maintenance treatment she had blindness in her right eye.Ophthalmologic examination her visual acuity was fingers to count in two meters.In her right eye there was exudative retinal detachment and her orbital magnetic resonance imaging (MRI) showed that, there was a mass lesion in the posterior of right orbital globe, on the neighbourhood of optic disc, consistent with metastasis.After ALL-REZ-BFM-2002 relapse protocol treatments and radiotherapy her visual acuity was increased to 6/10.In control orbital MRI regression of mass lesion size was established.Bone marrow transplantation was planned.Orbital involvement is rarely seen in leukemia, but it is responsive to chemotherapy and radiotherapy combination.
Purpose:To investigate the demographic characteristics, clinical features, and metabolic risk factors of children with urolithiasis.Materials and methods:This retrospective study included 98 patients (48 boys, 50 girls) with urolithiasis diagnosed by ultrasonography.The mean age at presentation was 59.8 (1-192) months, and the mean followup period was 5.5 (1-27) months.Clinical and laboratory data including gender, age at diagnosis, presence of family history of renal stone, follow-up duration, presenting symptoms, the history of urinary tract infection (UTI), stone localization, presence of anatomical abnormalities of the urinary tract, presence of microscopic or macroscopic hematuria, pyuria, urinary metabolic examinations, blood tests, analysis of stone composition, treatment modality, and prognosis were assessed.Results: The most common symptom was restlessness in infants (<1year) and abdominal or flank pain in older children.Microcalculi (stone diameter <3mm) and calculi (>3mm) were found in 29.6% and in 70.4% of the patients, respectively.Hypercalciuria was the most common abnormality, followed by hypomagnesiuria.Nearly half of the hypercalciuria cases were <1 year old.Recurrent urinary tract infection was detected in half of the patients.Four patients underwent extracorporeal shock-wave lithotripsy, four underwent open surgery, and the other 90 were treated with conservative treatment.Spontaneous passage occurred in 17 patients.Stone analysis revealed calcium-oxalate in 82.4% of the 17patients.At the time of their last visit, 70% of the patients that were treated with conservative treatment, either had stones that disappeared or diminished in size with appropriate therapy. Conclusion:In terms of stones disappearance or decrease in the size of the stones, it seems that children younger than 1 year have more disadvantages than older children.Microcalculi in children should be taken into consideration because we found that 3/5 of these cases had urinary metabolic abnormalities.
Objectives: This study aims to investigate if cardiac involvement may occur in children with familial Mediterranean fever (FMF) without cardiovascular symptoms by using heart rate recovery (HRR) and systolic blood pressure recovery (SBPR) parameters.Patients and methods: A total of 50 FMF patients (26 males, 24 females; mean age 151±33.4month; range 60 to 216 month) and 30 healthy controls (18 males, 12 females; mean age 143±43.9month; range 84 to 228 month) were included in the study.All patients were evaluated by echocardiography.All patients underwent a maximal graded exercise stress test.HRR and SBPR parameters were calculated.Results: There was a significant decrease in HRR1 value in FMF group (p=0.03).SBPR1 and SPBR2 values were higher in FMF group compared to control group (0.96±0.12 vs 0.88±0.12and 0.95±0.09vs 0.91±0.11,respectively); and the high SBPR1 value was statistically significant (p=0.02).FMF presence had a negative correlation with HRR1 (r= -0.26, p=0.03) and a positive correlation with SBPR1 (r=0.29,p=0.02).There was a negative correlation of M694V homozygous mutation with HRR1 and HRR2 values (r= -0.43, p=0.004, r=-0.42,p=0.005).Conclusion: Cardiac involvement may occur in FMF patients without cardiovascular symptoms.Impaired SBPR and decreased HRR response may indicate increased cardiovascular risk in these patients despite normal exercise stress test results.
The study aims to present the incidence of COVID-19 in pediatric patients undergoing renal replacement therapy (RRT) and to compare the severity and outcomes of the disease between the dialysis and kidney transplant (KTx) groups. This multicenter observational study was conducted between 1 April and 31 December 2020 in Istanbul. Members of the Istanbul branch of the Turkish Pediatric Nephrology Association were asked to report all confirmed cases of COVID-19 who were on RRT, as well as the number of prevalent RRT patients under the age of 20. A total of 46 confirmed cases of COVID-19 were reported from 12 centers, of which 17 were dialysis patients, and 29 were KTx recipients. Thus, the incidence rate of COVID-19 was 9.3% among dialysis patients and 9.2% among KTx recipients over a 9-month period in Istanbul. Twelve KTx recipients and three dialysis patients were asymptomatic (p = 0.12). Most of the symptomatic patients in both the dialysis and KTx groups had a mild respiratory illness. Only two patients, one in each group, experienced a severe disease course, and only one hemodialysis patient had a critical illness that required mechanical ventilation. In the entire cohort, one hemodialysis patient with multiple comorbidities died.Conclusion: While most cases are asymptomatic or have a mild disease course, pediatric patients undergoing dialysis and a kidney transplant are at increased risk for COVID-19. What is Known: • In adult population, both dialysis patients and kidney transplant recipients are at increased risk for severe illness of COVID-19 and have higher mortality rate. • Children with kidney transplantation are not at increased risk for COVID-19 and most have mild disease course. • Data on children on dialysis are scarce. What is New: • Pediatric patients undergoing dialysis and kidney transplantation have an increased risk for COVID-19. • Most patients undergoing renal replacement therapy either on dialysis or transplanted develop asymptomatic or mild COVID-19 disease with a favorable outcome.
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