Associations of Adipocyte-derived Versican and Macrophage-derived Biglycan with Body Adipose Tissue and Hepatosteatosis in Obese Children

Objective: In animal models of obesity, adipocyte-derived versican, and macrophage-derived biglycan play a crucial role in mediating adipose tissue inflammation. The aim was to investigate levels of versican and biglycan in obese children and any potential association with body adipose tissue and hepatosteatosis. Methods: Serum levels of versican, biglycan, interleukin-6 (IL-6), and high sensitivity C-reactive protein (hsCRP) were measured by ELISA. Fat deposition in the liver, spleen, and subcutaneous adipose tissue was calculated using the IDEAL-IQ sequences in magnetic resonance images. Bioimpedance analysis was performed using the Tanita BC 418 MA device. Results: The study included 36 obese and 30 healthy children. The age of obese children was 13.6 (7.5-17.9) years, while the age of normal weight children was 13.0 (7.2-17.9) years (p=0.693). Serum levels of versican, hsCRP, and IL-6 were higher in the obese group (p=0.044, p=0.039, p=0.024, respectively), while no significant difference was found in biglycan levels between the groups. There was a positive correlation between versican, biglycan, hsCRP, and IL-6 (r=0.381 p=0.002, r=0.281 p=0.036, rho=0.426 p=0.001, r=0.424 p=0.001, rho=0.305 p=0.017, rho=0.748 p<0.001, respectively). Magnetic resonance imaging revealed higher segmental and global hepatic steatosis in obese children. There was no relationship between hepatic fat content and versican, biglycan, IL-6, and hsCRP. Versican, biglycan, hsCRP, and IL-6 were not predictive of hepatosteatosis. Body fat percentage >32% provided a predictive sensitivity of 81.8% and a specificity of 70.5% for hepatosteatosis [area under the curve (AUC): 0.819, p<0.001]. Similarly, a body mass index standard deviation score >1.75 yielded a predictive sensitivity of 81.8% and a specificity of 69.8% for predicting hepatosteatosis (AUC: 0.789, p<0.001). Conclusion: Obese children have higher levels of versican, hsCRP, and IL-6, and more fatty liver than their healthy peers.

Cooccurring Type 1 Diabetes Mellitus and Autoimmune Thyroiditis in a Girl with Craniofrontonasal Syndrome: Are EFNB1 Variants Associated with Autoimmunity?

Craniofrontonasal syndrome (CFNS), also known as craniofrontonasal dysplasia, is an X-linked inherited developmental malformation caused by mutations in the ephrin B1 (EFNB1) gene. The main phenotypic features of the syndrome are coronal synostosis, hypertelorism, bifid nasal tip, dry and curly hair, and longitudinal splitting of nails. A 9-year-and-11-month-old girl with CFNS was admitted due to polyuria, polydipsia, fatigue, and abdominal pain. On physical examination, she had the classical phenotypical features of CFNS. Genetic tests revealed a c.429_430insT (p.Gly144TrpfsTer31) heterozygote variant in the EFNB1 coding region. The patient was diagnosed with type 1 diabetes mellitus (T1DM) and autoimmune thyroiditis based on laboratory findings and symptoms. The mother of the patient, who had the same CFNS phenotype and EFNB1 variant, was screened for autoimmune diseases and was also with autoimmune thyroiditis. This is the first report describing the association of CFNS with T1DM and autoimmune thyroiditis in patients with EFNB1 mutation.

Cutaneous manifestations of obesity in Turkish children: A comparative study.

BACKGROUND: Cutaneous changes in obese adults have been investigated in numerous studies, but this issue has not been adequately investigated in children. OBJECTIVES: We aimed to determine the prevalence of skin manifestations in children with obesity by comparing them to normal-weight children. METHODS: A cross-sectional study was conducted between June 2017 and January 2019 in which 82 children with obesity and 86 normal-weight healthy control children were enrolled. Study participants had detailed full-body skin examinations performed by the same dermatologist; mycological and Wood's lamp examinations were performed when necessary. Sociodemographic and anthropometric measurements of the participants were recorded. RESULTS: Striae distensae were the most common skin manifestation in children with obesity; striae were significantly higher in the obese than in the control group (72% vs. 26.7%, p < .001). The anatomical distribution of the striae distensae in the groups differed significantly. Striae distensae were most commonly observed on the buttocks in the control group, while the thighs were the most common site in the obese group. Acanthosis nigricans (63.4% vs. 3.5%, p < .001), acrochordons (17.1% vs. 1.2%, p < .001), keratosis pilaris (32.9% vs. 17.4%, p = .021), intertrigo (11% vs. 0%, p = .001), folliculitis (31.7% vs. 5.8%, p < .001), seborrheic dermatitis (12.2% vs. 3.5%, p = .035) and facial erythema (7.3% vs. 0%, p = .012) were found to be statistically significantly more common in the obese group compared to the control group. CONCLUSIONS: Obesity in children is associated with numerous cutaneous manifestations. Further study is needed to identify whether weight loss can reduce skin manifestations in obese children.

Pons ratio as a potential diagnostic biomarker for the detection of growth hormone deficiency in children.

INTRODUCTION: Pituitary insufficiency (PI) needs further research to optimize treatment. Growth hormone deficiency (GHD) is a subtype of PI. The purpose of the present study is to investigate the possible relationship between GHD and the anatomical position of the pons as revealed by magnetic resonance imaging (MRI) in the pediatric age group. In the current study, we developed a novel and simple index using MRI that could provide an alternative to other indexes in the classical literature. PATIENTS AND METHODS: Brain MRI and clinical data of 48 children with suspected PI (22 females, 26 males; mean age 11.6 ± 2.2 years) were examined retrospectively. To estimate the location of the pons, the ratio of pons height over the axis between the dorsum sellae and the fourth ventricular hill in the sagittal plane (A) to the total height of the pons (B) was calculated (A/B). It was termed the pons ratio (PR). The PRs of children with or without a diagnosis of GHD were then compared statistically. RESULTS: Fifteen children were diagnosed with GHD and treated with growth hormone (GH), whereas the remaining 33 were reported normal. The mean PRs of the children diagnosed with GHD and given GH treatment (0.31 ± 0.07, range: 0.18-0.42) differed significantly from those without PI (0.26 ± 0.06, range: 0.17-0.44; P = 0.018). CONCLUSION: There appears to be an association between GHD and decreased PR in children receiving GH treatment. Spatial measurements of posterior fossa in radiological examinations may provide additional information that is helpful in the diagnosis of suspected cases of GHD.

Prolonged mechanical ventilation associated with hypothyroidism after paediatric cardiac surgery.

Hypothyroidism in patients undergoing congenital heart defect surgery is known to be possible. This generally temporary condition can progress as it involves yet other factors, increasing the patients' time to heal. The case presented here is that of a 5-month-old girl who was dependent in the long term on mechanical ventilation following cardiac surgery. After having been diagnosed with hypothyroidism, she was extubated on the fourth day of her hormone replacement therapy, and discharged from hospital on the tenth day.

Tamoxifen as first-line treatment in a premenarchal girl with juvenile breast hypertrophy.

BACKGROUND: Juvenile breast hypertrophy is uncommon and is characterized by excessive breast enlargement in the peripubertal period. The clinical entity is thought to result from increased sensitivity of mammary tissue to normal levels of circulating hormones. CASE: Here, we report a female patient, aged 12 years and 6 months, suffering from juvenile breast hypertrophy, who presented at the third month of symptoms and benefited from tamoxifen treatment. COMMENTS: In experienced clinics, use of tamoxifen in the treatment of juvenile breast hypertrophy during the brisk growth period may become a medical alternative to reconstructive surgery.

Nutritional rickets and vitamin D deficiency in infants, children and adolescents.

Nutritional rickets continues to be a public health problem in many countries despite the presence of cheap and effective means of preventing the disease. Deficiency of vitamin D is associated with rickets in growing children and osteomalacia in adults. Vitamin D deficiency is attributed to a variety of causes including diet, atmospheric pollution, religious practices that restrict sunlight exposure (clothing), geographic latitude and altitude, season, and time of the day. The clinical findings of rickets can vary among stages of the disease. It is recommended that healthy infants, children and adolescents take at least 400 IU vitamin D per day to prevent rickets and vitamin D deficiency. Pediatricians and other healthcare professionals should try to ensure that children and adolescents receive daily vitamin D requirements appropriate for their risk factors, traditions, and customs. Additionally, it is important to use every opportunity to ensure that effective preventive strategies are put in practice.

Hepatic glycogenosis: a rare cause of hepatomegaly in Type 1 diabetes mellitus.

Hepatomegaly, with or without abnormal liver function tests, was a common feature of both pediatric and adult patients with diabetes mellitus. We are reporting a case of a 16-year-old diabetic boy in whom we found hepatomegaly, mildly elevated transaminases and elevated serum lipids never noticed before. Abdominal ultrasound confirmed hepatomegaly; liver biopsy pointed out a picture compatible with glycogenosis. The patient's abnormal liver function tests, elevated serum lipids and hepatomegaly decreased over a period of 4 weeks with tight metabolic control. This situation was due to overinsulinization because the patient assumed an excessive quantity of food and therefore took an excessive quantity of insulin. In conclusion, hepatomegaly may be seen in diabetic patients due to hepatic glycogen accumulation as a result of excessive food and insulin consumption. In hepatic glycogenosis, the pathological findings improve in 4 weeks when good metabolic control is provided. Therefore, the other reasons must be investigated when hepatomegaly persists for a longer period.