Mother's psychopathological conditions and parenting characteristics according to smoke exposure.

The study aims to investigate the relationship between maternal smoking and smoke exposure with the mother's parenting attitude and psychopathological conditions in the absence of any health problems in the mother-child pairs. A descriptive form consisting of 27 questions, a "Parental Attitude Scale", and a "Depression, Anxiety, and Stress Scale" was applied by mothers with children aged 2 to 6 years. Of the 450 voluntary mothers included in the study, 107 (23.8%) had environmental smoke exposure and 69 (15.0%) were smokers. The highest quartile of democratic subscores was associated with maternal smoking and smoke exposure when confounding factors were controlled. Compared to non-smokers and unexposed, the adjusted odds ratio of having abnormal anxiety scale subscores was 3.90 [95% CI: 1.69-8.97] for smokers. When parenting types were included, mothers' smoking status and overprotective subscores were found to be associated with anxiety. There is an interaction among mothers' smoking, parenting types, and anxiety scores.

Liver magnetic resonance elastography and fat fraction in pediatric patients with cystic fibrosis versus healthy children.

BACKGROUND: Liver involvement is an important cause of morbidity and mortality in patients with cystic fibrosis (CF). While liver biopsy is the gold standard for demonstrating involvement, its invasiveness prompts a search for noninvasive alternatives. OBJECTIVE: To evaluate liver involvement in pediatric patients with CF (versus healthy controls) using magnetic resonance (MR) elastography/spectroscopy and to correlate the imaging findings with clinical/laboratory characteristics. MATERIALS AND METHODS: This was a single-center, prospective cross-sectional study conducted between April 2020 and March 2022 in patients with CF versus healthy controls. Patients with CF were divided into two subgroups: those with CF-related liver disease and those without. MR images were acquired on a 1.5-tesla machine. Kilopascal (kPa) values were derived from processing MR elastography images. MR spectroscopy was used to measure liver fat fraction, as an indication of hepatosteatosis. Groups were compared using either the Student's t test or the Mann‒Whitney U test. The chi-square test or Fisher's exact test were used to compare qualitative variables. RESULTS: Fifty-one patients with CF (12 ± 3.3 years, 32 boys) and 24 healthy volunteers (11.1 ± 2.4 years, 15 boys) were included in the study. Median liver stiffness (P=0.003) and fat fraction (P=0.03) were higher in the CF patients than in the controls. Median liver stiffness values were higher in CF patients with CF-related liver disease than in those without CF-related liver disease (P=0.002). Liver stiffness values of CF patients with high alanine aminotransferase (ALT), high gamma-glutamyl transferase, and thrombocytopenia were found to be higher than those without (P=0.004, P<0.001, P<0.001, respectively). Only the high ALT group showed a high fat fraction (P=0.002). CONCLUSIONS: Patients with CF had higher liver stiffness than the control group, and patients with CF-related liver disease had higher liver stiffness than both the CF patients without CF-related liver disease and the control group. Patients with CF had a higher fat fraction than the control group. Noninvasive assessment of liver involvement using MR elastography/spectroscopy can support the diagnosis of CF-related liver disease and the follow-up of patients with CF.

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.

Features of Terra Firma-Forme Dermatosis.

Terra-firma-forme dermatosis (TFFD) is a little known hyperpigmented skin disease that is more common than expected. We examined retrospectively the medical records of 79 patients: 88.6% were children, the mean age was 10.4 years (SD = 7.5 years), and 64.6% were female. The dermatoses were found on the trunk in 27.8%, extremities in 26.6%, fold zones in 8.9%, and head in 2.5% of the patients. The lesions appeared in more than 1 location in 34.2% of the patients and were symmetrically located 77.2% of the time. Swabbing with alcohol is sufficient for diagnosis and treatment. TFFD, mostly seen in children and female patients, should be kept in mind to avoid unnecessary diagnostic testing and treatment.

Home accident or physical abuse: Evaluation of younger children presenting with trauma, burn and poisoning in the Pediatric Emergency Department.

Isiyel E, Teksam Ö, Foto-Özdemir D, Özmert E, Tümer AR, Kale G. Home accident or physical abuse: Evaluation of younger children presenting with trauma, burn and poisoning in the Pediatric Emergency Department. Turk J Pediatr 2018; 60: 625-632. Most cases of physical abuse and neglect refer to pediatric emergency clinics, and these patients are diagnosed as only home accidents. Cases that cannot be diagnosed and managed correctly are again exposed to severe trauma and consequently, they may die. The aim of this study is to evaluate the physical abuse and neglect in children younger than three years of age, admitted to the pediatric emergency department with the complaints of trauma, burn, drug poisoning and/or caustic ingestion. This prospective study included 132 patients who were admitted to the pediatric emergency department. Children were classified into three groups as `no abuse`, `suspected abuse` and `abused` after being evaluated with a standard form. Additionally, suspected abuse and abused cases were evaluated once again by `The Team of Child Abuse and Neglect Evaluation, Research and Treatment` in Hacettepe University, Ihsan Dogramaci Children's Hospital. The frequency of the suspected abuse or abused cases in all burn, trauma and poisoning cases was found to be 7.5%. It was noticed that 65 of the patients (49.2%) were physically neglected considering the mechanism of occurrence of events. Unplanned pregnancy, absence of prenatal follow-up, high number of siblings, previous physical abuse in the family, absence of witness during the event, and hospitalization were statistically significant differences between no abuse and suspected abuse or abused cases (p < 0.05). Moreover, age group and income were significant risk factors in the logistic regression model for the patients who were thought to be suspected abuse/abused in univariate analyses (age group: Odds ratio (OR) 0.279, 95% confidence interval (CI) 0.085-0.723, p=0.0049; income: OR 2.323, 95% CI 1.052-6.198, p=0.0345). In conclusion, most cases of physical abuse and neglect are referred to the pediatric emergency clinics, and these patients are misdiagnosed as home accidents. The physicians working in the emergency department should be informed and trained in recognizing the cases of abuse and neglect, making the differential diagnosis, identifying the high-risk families and appropriate physical and psychological treatment for the abused and neglected children.

Successful treatment of a childhood synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome with subcutaneous methotrexate: A case report.

Akçaboy M, Bakkaloglu-Ezgü SA, Büyükkaragöz B, Isiyel E, Kandur Y, Hasanoglu E, Buyan N. Successful treatment of a childhood synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome with subcutaneous methotrexate: A case report. Turk J Pediatr 2017; 59: 184-188. SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis and osteitis) is defined as a syndrome that is related to various osteoarticular manifestations and chronic dermatological conditions especially severe acne. SAPHO syndrome is a rare and unusual clinical entity in childhood and treatment choices are variable. We report an 11-year-old girl who suffered from SAPHO syndrome and successfully treated with subcutaneous methotrexate. We report our case in order to take attention to this rare clinical condition in evaluating patients and also to point out that treatment options beyond biologic agents should be the first line treatment in childhood.

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.

Cerebral Vasculitis in Henoch-Schönlein Purpura: A Case Report.

Henoch-Schönlein purpura is a common form of systemic small vessel vasculitis in childhood. Although headache and behavioral changes have been described in a significant proportion of children with Henoch-Schönlein purpura, severe neurological complications are rare. In this article, we report a case of central vasculitis in a four-year-old boy who presented with hemiplegia and aphasia. The treatment options for cerebral vasculitis of Henoch-Schönlein purpura are numerous but controversial in pediatric patients. Our patient was successfully treated by pulse methylprednisolone and pulse cyclophosphamide. The patient was followed-up for four years without any sequel.

Molecular analysis of the AGXT gene in patients suspected with hyperoxaluria type 1 and three novel mutations from Turkey.

Primary hyperoxaluria type 1 (PH1) is a rare, autosomal recessive disease, caused by the defect of AGXT gene encoding hepatic peroxisomal alanine glyoxylateaminotransferase (AGT). This enzyme is responsible for the conversion of glyoxylate to glycine. The diagnosis of PH1 should be suspected in infants and children with nephrocalcinosis or nephrolithiasis. Early diagnosis and treatment is crucial in preventing disease progression to end stage kidney disease (ESKD). In this study, AGXT gene sequence analyses were performed in 82 patients who were clinically suspected (hyperoxaluria and nephrolithiasis or nephrocalcinosis with or without renal impairment) to have PH1. Disease causing mutations have been found in fifteen patients from thirteen families (18%). Novel mutations have been found (c.458T>A (p.L153X), c.733_734delAA (p.Lys245Valfs*11), c.52 C>T (p.L18F)) in three of 13 families. There were 3-year lag time between initial symptoms and the time of PH1 is suspected; additionally, 5.5-year lag time between initial symptoms and definitive diagnosis. Consanguinity was detected in 77% of the patients with mutation. After genetic diagnosis, one patient received combined kidney and liver transplantation. AGXT gene sequencing is now the choice of diagnosis of PH1 due to its non-invasive nature compared to liver enzyme assay. Early diagnosis and accurate treatment in PH1 is important for better patient outcomes.

Changing trends in pediatric renal biopsies: analysis of pediatric renal biopsies in national nephrology registry data.

Renal biopsy is the gold standard method for determining the diagnosis, treatment, and prognosis in children with renal disease. This study aims to evaluate the histopathological features of pediatric renal biopsies obtained from the national nephrology registry in the last two decades. Data recorded in the Turkish Society of Nephrology Registry System (TSNRS) in 1991 as well as in between 2001 and 2010 were analyzed. A total of 3892 biopsies were recorded; with the least number in 1991 (total 103 biopsies from 17 centers) and the highest number in 2008 (total 654 biopsies from 23 centers). Glomerular diseases constituted the main group in the registry (62.64%), followed by systemic diseases (20.06%). Focal and segmental glomerulosclerosis (FSGS) and Henoch-Schönlein purpura (HSP) nephritis (IgA vasculitis) were the most common glomerular and systemic diseases, respectively. Overall prevalence of renal amyloidosis and membranous nephropathy (MN) was quite low (1.87% and 1.56%, respectively) in all periods. Compared to 1991, there was an increasing trend in the frequencies of certain disorders including hemolytic uremic syndrome (HUS), IgA nephropathy, and HSP nephritis; and there was a decrease in acute proliferative glomerulonephritis (GN) in 2008. As well as demonstrating the etiologies of renal diseases which can only be identified by renal biopsies, this study provides important information regarding the changing patterns of histopathological findings due to better management of pediatric renal diseases over the years in Turkey.

Febrile seizures in children with familial Mediterranean fever: Coincidence or association?

BACKGROUND: Familial Mediterranean fever (FMF) is an inherited disease characterized by recurrent bouts of fever and polyserositis and caused by MEditerranean FeVer gene (MEFV) mutations. Given the febrile characteristics of the disease one would expect higher frequency of febrile seizure in this group of pediatric patients. OBJECTIVES: To evaluate the frequency of febrile seizure and related factors in patients with FMF. METHODS: The children with the diagnosis of FMF were enrolled in the study. Information including clinical features, type of mutation and the history of febrile seizure were all noted. RESULTS: A total of 97 patients, 43 (44.3%) girls with a median age of 7.93 ± 4.05 years (2-16) and a median follow-up period of 20.65 ± 24.33 months (6-135) were included in the study. The frequency of febrile seizure in children with FMF was found as 13.4%, which is higher than the general population [p = 0.04, OR: 2.9 (95% CI: 1.0-8.5)]. The allele frequency of exon 2 mutations in MEFV genes was higher in the patients with febrile seizure (p = 0.03). Frequency of FMF related clinical findings (fever, abdominal pain, arthralgia/myalgia, arthritis, chest pain and erysipelas-like erythema) was similar between the two groups. However, frequency of headache was higher in the patients with febrile seizure (p = 0.014). CONCLUSION: The frequency of febrile seizure in children with FMF was found to be higher than the general population. Although this finding may be related to high fever during FMF attacks in individuals with genetic propensity of febrile seizure, it may also be a neurologic complication of FMF.

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.

Hemolytic uremic syndrome outbreak in Turkey in 2011.

The aim of this retrospective multicenter study was to define the epidemiological and clinical features and prognostic factors of the first diarrhea-related hemolytic uremic syndrome (D+HUS) outbreak in Turkey in 2011. All pediatric nephrology centers in Turkey were asked about D+HUS patients via e-mail. Seventy D+HUS patients (median age: 5.7 years) participated. The seasonal peak was around the 7th, 8th and 9th months with 44 cases, centered in the east Marmara region. No causative agent could be identified. The rate of neurological complications and mortality was 21.4% and 4.2%, respectively. Eculizumab was used in four cases. Two of them had severe neurological complications despite plasma exchange. Elevated polymorphonuclear leukocyte count during hospital admission was the predictor of both severe disease and poor outcome. Duration of prodrome was the predictor of poor outcome (p<0.05). In conclusion, the median age of the affected children was greater than in the previous reports, while clinical features and outcome were similar.