Long-term Effects of Kidney Transplantation Compared With Dialysis on Intima-media Thickness in Children-Results From the 4C-T Study.

Background: Children requiring kidney replacement therapy experience high burden of cardiovascular (CV) disease leading to increased mortality. Intima-media thickness (IMT) indicating atherosclerosis is a validated surrogate marker for future CV events.

Methods: We investigated the effect of different treatment modalities (dialysis, preemptive kidney transplantation (KTx), late KTx after dialysis) on IMT by multivariable linear mixed-effect modeling.

Empirical cefepime+vancomycin versus ceftazidime+vancomycin versus meropenem+vancomycin in the treatment of healthcare-associated meningitis: results of the multicenter ephesus study.

Background: Herein, we analyzed the efficacy of main antibiotic therapy regimens in the treatment of healthcare-associated meningitis (HCAM).

Materials/methods: This retrospective cohort study was conducted in 18 tertiary-care academic hospitals Turkey, India, Egypt and Romania. We extracted data and outcomes of all patients with post-neurosurgical meningitis cases fulfilling the study inclusion criteria and treated with empirical therapy between December 2006-September 2018.

Serum indoxyl sulfate concentrations associate with progression of chronic kidney disease in children.

The uremic toxins indoxyl sulfate (IS) and p-cresyl sulfate (pCS) accumulate in patients with chronic kidney disease (CKD) as a consequence of altered gut microbiota metabolism and a decline in renal excretion. Despite of solid experimental evidence for nephrotoxic effects, the impact of uremic toxins on the progression of CKD has not been investigated in representative patient cohorts. In this analysis, IS and pCS serum concentrations were measured in 604 pediatric participants (mean eGFR of 27 ± 11 ml/min/1.

Is Vitamin D Deficiency Associated With Metabolic Syndrome in Renal Transplant Recipients?

Renal transplantation is the gold standard method for the treatment of end-stage renal failure. The main causes of graft loss are chronic allograft dysfunction and death with functioning graft due to cardiovascular diseases. Metabolic syndrome (MetS) is common in renal transplant recipients (RTRs).

Masked Hypertension and Obesity in Renal Transplant Recipients.

The diagnosis of arterial hypertension has traditionally been based on measurements of blood pressure (BP) in the clinic. However, home or ambulatory BP monitoring (ABPM) is reported to correlate better with target organ damage than clinic BP readings. The other potential advantage of out-of-clinic BP measurement is the detection of both white-coat hypertension (WCHT) and masked hypertension (MHT).

Body Shape Index Predicts Metabolic Syndrome and Insulin Resistance in Renal Transplant Recipients.

Obesity is one of the most important metabolic diseases around the world. There are no reliable and inexpensive methods to evaluate obesity. All of the anthropometric measurements used have some limitations.

Mutational analysis of PHEX, FGF23 and CLCN5 in patients with hypophosphataemic rickets.

Context: Hypophosphataemic rickets (HR) is a group of rare hereditary renal phosphate wasting disorders caused by mutations in PHEX, FGF23, DMP1, ENPP1, CLCN5 or SLC34A3.

Objective: To investigate underlying genetic defects in patients with hypophosphataemic rickets.

Methods: We analysed genomic DNA from nine unrelated families for mutations in the entire coding region of PHEX, FGF23, DMP1, ENPP1, CLCN5 or SLC34A3 by PCR sequencing and copy number analysis.

Integrase Strand Transfer Inhibitors (INSTIs) Resistance Mutations in HIV-1 Infected Turkish Patients.

Objectives: Integrase strand transfer inhibitor (INSTI) is a new class of antiretroviral (ARV) drugs designed to block the action of the integrase viral enzyme, which is responsible for insertation of the HIV-1 genome into the host DNA. The aim of this study was to evaluate for the first time INSTI resistance mutations in Turkish patients.

Methods: This study was conducted in Turkey, between April 2013 and April 2015 using 169 HIV-1-infected patients (78 ARV naive patients and 91 ARV-experienced patients).

Spondyloenchondrodysplasia Due to Mutations in ACP5: A Comprehensive Survey.

Purpose: Spondyloenchondrodysplasia is a rare immuno-osseous dysplasia caused by biallelic mutations in ACP5. We aimed to provide a survey of the skeletal, neurological and immune manifestations of this disease in a cohort of molecularly confirmed cases.

Methods: We compiled clinical, genetic and serological data from a total of 26 patients from 18 pedigrees, all with biallelic ACP5 mutations.

Rare Causes of Primary Adrenal Insufficiency: Genetic and Clinical Characterization of a Large Nationwide Cohort.

Context: Primary adrenal insufficiency (PAI) is a life-threatening condition that is often due to monogenic causes in children. Although congenital adrenal hyperplasia occurs commonly, several other important molecular causes have been reported, often with overlapping clinical and biochemical features. The relative prevalence of these conditions is not known, but making a specific diagnosis can have important implications for management.

HIV-1 Transmitted Drug Resistance Mutations in Newly Diagnosed Antiretroviral-Naive Patients in Turkey.

HIV-1 replication is rapid and highly error-prone. Transmission of a drug-resistant HIV-1 strain is possible and occurs within the HIV-1-infected population. In this study, we aimed to determine the prevalence of transmitted drug resistance mutations (TDRMs) in 1,306 newly diagnosed untreated HIV-1-infected patients from 21 cities across six regions of Turkey between 2010 and 2015.

Quality of life survey following laparoscopic and open radical nephrectomy.

Purpose: To compare the quality of life (QoL) of renal cancer patients following laparoscopic and open radical nephrectomy.

Materials And Methods: Seventy-two (64.9%) patients who were treated with open radical nephrectomy (ORN group) and 39 (35.

Chloroquine and hydroxychloroquine retinopathy-related risk factors in a Turkish cohort.

To evaluate the role of previously reported risk factors on developing retinopathy in patients treated with chloroquine (CQ) and hydroxychloroquine (HCQ). Ophthalmologic examination, visual field testing, and spectral domain-optical coherence tomography were performed in 31 patients treated with CQ and HCQ. Toxicity diagnosis was proven by electrophysiological assessment.

Clinical features and management of diabetic ketoacidosis in different age groups of children: children less than 5 years of age are at higher risk of metabolic decompensation.

Objective: To characterize the clinical features and biochemical status at presentation of diabetic ketoacidosis (DKA) in different age groups of children, and to analyze the outcomes of a certain treatment protocol.

Methods: We reviewed records of patients with DKA who were admitted to our hospital between January 2007 and December 2010. Patients were divided into three subgroups according to age, and the results were compared between these groups.

False positivity of magnetic resonance imaging under the effect of granulocyte-colony stimulating factor in a child with leukemia.

Granulocyte-colony stimulating factor (G-CSF) increases the proliferation and maturation of committed polymorphonuclear leukocyte precursors, as well as the function of mature polymorphonuclear leukocytes. It has previously been shown in pediatric patients that G-CSF induces reconversion of fatty bone marrow to hematopoietic bone marrow in the pelvis and lower extremities that is detectable by magnetic resonance imaging (MRI). Here, we report a 13-year-old Burkitt leukemia patient with bone pain while he was in remission.

Warburg Micro syndrome.

Micro syndrome is an autosomal recessive disorder characterized by severe intellectual disability, microcephaly, congenital cataract, microcornea, microphthalmia, agenesis, or hypoplasia of the corpus callosum and hypogenitalism. We report an 11-month-old boy who was referred for assessment of micropenis and cryptorchidism. Sequence analysis of exon 8 of the RAB3GAP1 gene confirmed the presence of a splice donor mutation (748+1G>A) in the homozygous state.

Delirium in diabetic ketoacidosis: a case report.

A 15-year-old female patient with known type 1 diabetes mellitus was referred because of abdominal pain. On admission, she was alert but dehydrated with marked Kussmaul breathing. Blood glucose was 414 mg/dL (23 mmol/L).

Multiple pterygium syndrome: mimicking the findings of Turner syndrome.

Multiple pterygium syndrome (MPS or Escobar syndrome) is a rare, generally autosomal recessive disorder characterized by multiple congenital joint contractures and multiple skin webs. An 11.5-year-old girl with a working diagnosis of Turner syndrome (TS) was referred for her phenotypic features and growth retardation.

3M syndrome: a report of four cases in two families.

3M syndrome is a rare entity characterized by severe growth retardation, dysmorphic features and skeletal changes as its major components. It is differentiated from other types of dwarfism by its clinical features and by the typical slender long bones and foreshortened vertebral bodies that can be visualized radiographically. 3M syndrome has an autosomal recessive mode of inheritance.

Association between the corrected QT interval and carotid artery intima-media thickness in obese children.

Objective: Sudden death has been reported in asymptomatic obese adults and the mechanism is unclear. In recent years, obesity has shown a dramatic increase in children and this enhances the risk factors for the development of cardiovascular disease. The aim of this study was to investigate whether there is repolarization abnormality and any potential risk factor such as increase in intima-media thickness (IMT) of carotid artery for corrected QT (QTc) prolongation among obese children.

Mitochondrial neurogastrointestinal encephalomyopathy: case report.

Mitochondrial neurogastrointestinal encephalomyopathy is an autosomal recessive multisystem disorder caused by thymidine phosphorylase deficiency. The disease is characterized clinically by ptosis, progressive external ophthalmoparesis, severe gastrointestinal dysmotility, peripheral neuropathy, leukoencephalopathy, and mitochondrial abnormalities. Herein, we describe a patient with mitochondrial neurogastrointestinal encephalomyopathy who presented intestinal pseudoobstruction.

Optic neuritis following hepatitis B vaccination in a 9-year-old girl.

Hepatitis B vaccination can prevent hepatitis B virus infection and its serious consequences, including liver cancer and cirrhosis. Serious side effects reported after receiving hepatitis B vaccination are very uncommon. A majority of the ophthalmological complications seen following hepatitis B vaccination consist of vision loss, optic neuritis, papillary edema, uveitis, acute placoid pigment epitheliopathy and central vein occlusion.

Spontaneous remission of acute lymphoblastic leukemia with mediastinal mass.

Spontaneous remission/regression of cancer is defined as partial or complete disappearance of malignant disease temporarily or permanently in the absence of medical treatment. This event is named as spontaneous regression for solid tumors and spontaneous remission for leukemia. The authors report the case of a girl aged 4 years and 3 months, who presented with mediastinal mass and leukemic findings in the bone marrow both of which reappeared after spontaneous regression and remission, respectively.

Transvaginal sonography in the diagnosis of adenomyosis: which findings are most accurate?

Objectives: To evaluate the accuracy of various transvaginal sonographic findings in adenomyosis by comparing them with histopathological results and to determine the most valuable sonographic feature in the diagnosis of adenomyosis.

Methods: In this prospective study, 70 consecutive patients scheduled for hysterectomy underwent preoperative transvaginal sonography. If at least one of the following sonographic features was present, a diagnosis of adenomyosis was made: heterogeneous myometrial echotexture, globular-appearing uterus, asymmetrical thickness of the anteroposterior wall of the myometrium, subendometrial myometrial cysts, subendometrial echogenic linear striations or poor definition of the endometrial-myometrial junction.