Assessment of atherosclerosis in obese adolescents: positive correlation of mean platelet volume and carotid intima media thickness.

AIMS: This study aims to assess the correlation of mean platelet volume (MPV) and common carotid artery (CCA) thickness in a population of obese adolescents. METHODS: Sixty-eight patients and 23 controls were enrolled. Anthropometric measurements, triglyceride, total cholesterol, high-density lipoprotein cholesterol, low-density lipoprotein cholesterol, alanine aminotransferase, aspartate aminotransferase, γ-glutamyl transpeptidase, hemoglobin, white blood cell count, platelet count, MPV and insulin resistance by homeostasis model of assessment of insulin resistance were investigated. Furthermore, CCA thickness was measured by high-resolution ultrasound. RESULTS: MPV and the left CCA thickness were significantly higher in obese adolescents than the healthy controls. The association between MPV and left CCA was checked by linear regression analysis. MPV explained 19% of the variation in left CCA (P < 0.001). At multiple regression analysis, MPV maintained a positive association with the left CCA thickness (P = 0.002) independently of fatty liver grade, relative weight, total cholesterol and homeostasis model of assessment of insulin resistance. CONCLUSION: MPV is significantly correlated with CCA thickness in obese adolescents.

Vascular rings: presentation, imaging strategies, treatment, and outcome.

This study aimed to evaluate the presenting symptoms, the effectiveness of imaging methods, and the surgical treatment of vascular rings. Data for 44 patients (32 enrolled prospectively, 12 reviewed retrospectively) over a 10-year period in a tertiary referral center were analyzed. These patients comprised 25 patients with a left aortic arch and an aberrant right subclavian artery, 13 patients with a right aortic arch and a left subclavian artery originating from Kommerell's diverticulum, 1 patient with a right aortic arch and an aberrant left subclavian artery, 3 patients with a double aortic arch, and 2 patients with a pulmonary sling. Respiratory symptoms were found in 25 patients and dysphagia in 6 patients. Atypical symptoms such as reflex apnea, cyanosis, syncope episodes, and exercise-induced wheezing were noted in five patients. Associated congenital heart defects were detected in 41% of the patients. The diagnostic yield was 95.23% for barium esophagography, 54.54% for echocardiography, and 66.66% for computed tomography. The anatomy could be correctly identified by magnetic resonance imaging (MRI) in 97.43% and by angiography in 90.5% of the patients. Of the 30 patients who underwent surgery, 80% were completely relieved of symptoms during a mean follow-up period of 25 ± 33.5 months. Vascular rings should not be overlooked in infants with atypical symptoms. The authors' diagnostic procedure of choice is MRI because it is superior to angiography for delineating the relationship between abnormal vascular structures, trachea, and esophagus.

Soft tissue sarcomas and central nervous system tumors in children with neurofibromatosis type 1.

OBJECTS: We aim to evaluate the characteristics of pediatric patients with neurofibromatosis type 1 (NF1) who developed soft tissue sarcomas (STSs) and central nervous system (CNS) tumors that have been followed up in our center. MATERIALS AND METHODS: Medical records of children with NF1 were retrospectively analyzed. RESULTS: There were 78 patients who met at least two diagnostic criteria for NF1. The median age of patients was 10 years (0.5-18), and M/F ratio was 1.3. The prevalance of the optic glioma was 11.5% (n = 9), and one patient with optic glioma also had cystic astrocytoma, one patient had brain stem tumor, and one patient had a CNS tumor (without histopathologic diagnosis). Seven of nine children were ≥ 7 years old at the time of the diagnosis of optic glioma. Visual impairment developed in four patients, and two of them were treated with radiotherapy solely on the basis of evidence of clinical and radiological progression of the tumors. Four patients developed STSs. Two of them had malignant peripheral nerve sheath tumors (MPNST), and the remaining two had bladder rhabdomyosarcoma. Three of the four patients with STSs died with progressive disease. CONCLUSION: The clinical course of malignancy in NF1 is often different from that of similar tumor types in the general population. Careful follow-up in patients with NF1 is required to enable the early diagnosis of malignancies, and the developments of new targeted therapies are needed for improvement of the outcome for patients of this group, especially with MPNST.

A rare complication of intrathecal methotrexate in a child with acute lymphoblastic leukemia.

Methotrexate (MTX) is an essential component of chemotherapy for childhood acute lymphoblastic leukemia (ALL). Both intravenous and most commonly intrathecal routes of MTX have been implicated in acute, subacute, and chronic neurotoxicity syndromes. Subacute MTX neurotoxicity occurs within days to weeks after the intravenous or intrathecal therapy and characterized by a distinct presentation with remarkable clinical resemblance to stroke, including hemiparesis, hemisensory deficits, aphasia, dysarthria, dysphagia, and diplopia. Herein the authors describe the clinical and typical neuroimaging features of a female patient with ALL who presented with subacute MTX neurotoxicity that rapidly progressed to a severe clinical condition in a few hours but eventually resolved completely with dexamethasone and folinic acid. Subacute MTX neurotoxicity is a transient neurological dysfunction that should be considered in patients presenting with stroke-like and various neurological symptoms 10 to 14 days after intrathecal therapy and diffusion-weighted magnetic resonance imaging should be undertaken for the correct diagnosis and exclusion of possible ischemic infarct. Discontinuation of subsequent intrathecal MTX therapies should be considered in severe cases and treatment with dexamethasone and folinic acid may help to resolve the symptoms.

Challenges of radiological assessment in an infant with giant calcified hepatoblastoma.

Hepatoblastoma is a rare neoplasm of all pediatric cancers. The goal of treatment is to remove the tumor completely because cure without complete resection is extremely unusual. Accurate assessment of tumor resectability following preoperative chemotherapy is of crucial importance. It is sometimes difficult, especially when the tumor is as large and calcified as in the described case. Detailed radiological imaging such as computed tomography angiography or magnetic resonance angiography is the key for selecting the proper treatment method in hepatoblastoma during the preoperative period. In this article, we report a successfully treated giant calcified hepatoblastoma despite radiological assessment complexity.

Fibromuscular dysplasia as a cause of stroke in a 9-year-old girl.

Fibromuscular dysplasia is a rare, idiopathic and nonatheromatous disease. It is rarely encountered as a cause of stroke in children. We report a nine-year-old girl with stroke in whom extensive fibromuscular dysplasia of intracranial vessels was established. She also had familial combined hyperlipidemia as an additional risk factor. This case suggests that additional risk factors like hyperlipidemia in cases with fibromuscular dystrophy may facilitate the occurrence of stroke at early ages.

Pulmonary tuberculosis in infants under one year of age.

This study describes the clinical and radiological features of tuberculosis in infants under one year of age. Medical records were reviewed for infants aged 12 months or less with proven tuberculosis. Six patients' data were evaluated. Cough and tachypnea were the major symptom and sign, respectively. Contact with an adult case of tuberculosis was present in five of the cases. Tuberculin skin test was positive in only one case. Mycobacterium tuberculosis was cultured from gastric aspirates of four of five infants and from cerebrospinal fluid in one case. Consolidation was the most common parenchymal lung lesion occurring in four of the patients. Mediastinal or hilar lymphadenopathies were also detected in four of the patients and calcifications were seen within the enlarged nodes in two of them. Antituberculous treatment appeared to be well tolerated without significant adverse effects. Significant radiological improvement was noted after a mean period of 4.6 months.

Two cases with megalencephalic leukoencephalopathy with subcortical cysts and MLC1 mutations in the Turkish population.

Megalencephalic leukoencephalopathy with subcortical cysts is a rare leukodystrophy that is characterized by macrocephaly and a slowly progressive clinical course. It is one of the most commonly reported leukoencephalopathies in Turkey. Mutations in the MLC1 gene are the main cause of the disease. We report two patients with megalencephalic leukoencephalopathy with subcortical cysts with confirmed mutations in the MLC1 gene. The mutation in the second patient was novel. We also review identified mutations in the Turkish population.

Chronic recurrent multifocal osteomyelitis in a patient with selective immunoglobulin M deficiency.

Chronic recurrent multifocal osteomyelitis is an unusual inflammatory process of unknown origin involving multiple osseous sites, often recurrently. Selective immunoglobulin M (IgM) deficiency is a rare primary immunodeficiency disease, which can be associated with autoimmune diseases such as systemic lupus erythematosus, Hashimoto's disease, or hemolytic anemia. Here we report a case of a chronic recurrent multifocal osteomyelitis coexisting with selective IgM deficiency.

Subscapular abscess after blunt trauma.

Infection around the shoulder girdle is an infrequent and difficult diagnosis requiring a high index of suspicion and early evaluation by the physician. An infection within the area of the subscapularis muscle and the chest wall is extremely rare. To the best of our knowledge, only 4 cases of subscapular abscess have been reported. In this article, we present a 7-year-old boy with a subscapular abscess after blunt trauma.

Chlamydia pneumonia mimicking miliary tuberculosis.

Chlamydia trachomatis is a common cause of subacute, afebrile pneumonia with onset from 1 to 3 months of age. On physical examination, crepitant inspiratory rales are commonly heard. Infiltration is usually bilateral and interstitial; reticulonodular pattern and atelectasis have also been described, which distinct the disease from miliary tuberculosis. We report an infant who had a disseminated miliary pattern in the chest radiograph and computed tomographic scan of the thorax that was diagnosed as Chlamydia pneumonia with serologic investigations. We emphasized that Chlamydia trachomatis can cause a miliary reticulonodular pattern in radiological examinations of infants who were admitted with respiratory symptoms. We suggest that pneumonia due to C. trachomatis should be kept in mind in the differential diagnosis of infants examined because of a diffuse miliary pattern.

Aneurysmal bone cyst of rib presenting as a huge chest wall mass.

Aneurysmal bone cyst is a rare benign tumor of the bone that can be difficult to distinguish from malignant tumors, especially when it presents in an unusual location. Herein, we report a six-year-old girl with a primary aneurysmal bone cyst in an uncommon location. It originated from the 4th rib and she presented with a huge chest wall mass. Despite the large size of the cyst, the only symptom was persistent cough. She was successfully treated with total excision of tumor without any complication. The patient has been followed up for 56 months without any recurrence.

Acute cerebellitis with cerebellar swelling successfully treated with standard dexamethasone treatment.

Although cerebellitis is common in childhood but cerebellitis with cerebellar swelling is rarely reported. Pulsed high dose methylprednisolone treatment is the choice of treatment for cases who have non-progressive symptoms. An 8-year-old girl presented acutely with vertigo, headache, and vomiting. Brain magnetic resonance imaging showed marked bilateral cerebellar swelling with increased signal on T2-weighted imaging. Following treatment with standard dexamethasone dose, the clinical and radiological signs resolved in 1 week. We conclude that standard dexamethasone treatment should be used in mild cases of acute cerebellitis in order to avoid adverse reactions of pulsed high dose methylprednisolone treatment.

Intima-media thickness of carotid artery and susceptibility to atherosclerosis in obese children with nonalcoholic fatty liver disease.

OBJECTIVE: To test, via prospective case-control study, whether measurements of carotid artery intima-media thickness (cIMT) reflect the susceptibility to atherosclerosis in obese children with nonalcoholic fatty liver disease. PATIENTS AND METHODS: In all, 80 obese asymptomatic normotensive adolescents (body mass index [BMI], 29.12 +/- 5.13; 8.5-17.5 years) and 30 age- and sex-matched nonobese healthy children (BMI 18.55 +/- 2.24). Children were analysed in 4 groups: Controls formed group 1, 26 children with obesity without hepatosteatosis formed group 2, 32 children with grade 1 hepatosteatosis formed group 3, and 22 children with grade 2 or 3 hepatosteatosis formed group 4. The key parameters were BMI, liver function tests, lipid profiles, glucose, hepatic ultrasonography, and IMT of common carotid artery (CCA), left carotid bulbus (CB), and left internal carotid artery (ICA). RESULTS: Comparisons of IMT measurements of left CCA, left CB, and left ICA between controls and obesity group revealed statistically significant differences (each segment P < 0.0001). There was an increase in the mean of IMT of each segment with the increase in hepatosteatosis grade (each segment P < 0.0001). The relation between the mean of IMT of left CCA, left CB, and left ICA with BMI (P = 0.001, r = 0.318), alanine aminotransferase (P = 0.007, r = 0.256), and hepatosteatosis (P < 0.0001, r = 0.470) was significant. Multiple regression analysis between left CCA, left CB, and left ICA, and age, sex, relative weight, alanine aminotransferase, total cholesterol, obesity, and hepatosteatosis grade was significant for only hepatosteatosis grade. CONCLUSIONS: The cIMT is significantly higher, increasing susceptibility to atherosclerosis, in obese children with hepatosteatosis than in healthy children or obese children without hepatosteatosis.

Vertebral artery dissection in a patient with Wildervanck syndrome.

Vertebral artery dissection as a cause of stroke is rarely reported in children. The association between vertebral artery dissection and Klippel-Feil syndrome is also very rare. We report on a case of vertebral artery dissection with posterior circulation involvement in a child with Klippel-Feil syndrome after a hard physical-training lesson. She was also diagnosed with Wildervanck syndrome, with additional clinical findings. Vertebral artery dissection should be considered in patients with Klippel-Feil syndrome who present with acute-onset neurologic signs. Movements such as hyperextension with rotation of the neck should be avoided in these cases.

Mycoplasma pneumoniae: nervous system complications in childhood and review of the literature.

Mycoplasma pneumoniae is an important pathogen which causes nervous system disorders during or after the course of a respiratory tract infection. The exact pathogenic mechanism which causes neurological disorders still remains unknown. Although meningoencephalitis and acute disseminated encephalomyelitis are common complications, there are few cases of acute transverse myelitis and isolated abducens nerve palsy associated with M. pneumoniae infection in childhood. The association between ocular myasthenia gravis and M. pneumoniae infection has not been described before. Here, we describe five patients with different nervous system complications associated with M. pneumoniae infection and discuss the pathological features of central nervous system involvement.

Stromal-predominant mesenchymal hamartoma of the liver with elevated serum alpha-fetoprotein level.

Mesenchymal hamartoma of the liver (MHL) is an uncommon, benign, tumor-like lesion and is usually diagnosed in the first 2 years of life. Its pathogenesis remains unclear. Treatment of choice is radical excision. The authors report a case of solid stromal predominant MHL in a 12-month-old male infant who also had an elevated serum alpha-fetoprotein level. He also had hypospadias, which might represent a spectrum of developmental anomalies. It usually presents as an asymptomatic mass, however, as in the reported case, it may cause several complications due to the compression of surrounding structures. He was successfully treated with total excision of the pedunculated large tumor without any complication.

Changes of primary headache related white matter lesions in pediatric patients.

Bayram E, Yis U, Paketçi C, Okur D, Polat I, Çakmakci H, Hiz S, Anlar B. Changes of primary headache related white matter lesions in pediatric patients. Turk J Pediatr 2018; 60: 380-384. We aimed to describe the long-term prognosis of white matter lesions detected on magnetic resonance imaging in children with primary headache. Children who were admitted with the complaint of headache and had nonspecific white matter lesions on magnetic resonance imaging were included in the study. The clinical findings of the patients were reinvestigated using the same magnetic resonance imaging scanner and acquisition protocol after at least a two year period. Magnetic resonance imaging results of the patients were documented in detail. Findings of the baseline and follow-up studies were compared with each other by the same radiologist. Among the 11 patients, 8 ( 72.7%) were male and 3 (27.3%) were female. Mean age of patients at the time of second imaging was 12.9±2.3 years. Eight (72.7%) had migraine without aura, 1 (9.1%) had tension-type headache and 2 (18.2%) had migraine with aura. The mean clinical follow-up period of the patients was 4.31±1.31 years. All patients had low headache frequency on the last control visit when compared to the first clinical findings. The follow-up magnetic resonance imaging studies showed two newly developed white matter lesions in two patients who had migraine without aura and the white matter lesions disappeared in the patient who had tension-type headache, compared to the baseline neuroimaging. Findings of the baseline and long-term follow-up magnetic resonance imaging studies of the patients with primary headache showed no significant changes in terms of the location, size and laterality. Repeated neuro-imaging studies are not warranted in the absence of the progression in clinical findings.

The correlation of seizure characteristics and hippocampal volumetric magnetic resonance imaging findings in children with idiopathic partial epilepsy.

Cerebral volumetric measurements based on magnetic resonance imaging have been established as advanced morphometric techniques with anatomic and clinical utility in adults and children with epilepsy. This study investigated the cerebral and hippocampal volumes in children with idiopathic partial epilepsy to detect the factors correlated with volume reduction. Magnetic resonance imaging volumetric measurements were performed of the total cerebral and hippocampal formation volumes in 30 patients with idiopathic partial epilepsy between 3 to 18 years old. The cerebral and the total, right, and left hippocampal volumes of the study and control patients were detected using volumetric magnetic resonance imaging, and the volumes were compared between the 2 groups. In study patients, the correlation between volumetric findings and seizure characteristics was evaluated. The results suggested that children with idiopathic partial epilepsy had significant hippocampal volume reduction that was not influenced by the age of onset and the duration of epilepsy.

Basilar artery thrombosis in a child heterozygous for prothrombin gene G20210A mutation.

Prothrombin G20210A mutation is an important prothrombotic condition for venous thrombosis. Recently, some studies have also considered it to be a risk factor for arterial ischemic stroke in children. A 10-year-old boy with basilar artery thrombosis who was heterozygous for prothrombin G20210A mutation is described. In concordance with the previous literature, the present case suggests that prothrombin G20210A mutation may be a risk factor for arterial ischemic stroke in childhood.