Could serum pentraxin 3 levels and IgM deposition in skin biopsies predict subsequent renal involvement in children with Henoch-Schönlein purpura?

BACKGROUND: The aim of this study was to evaluate the potential of serum pentraxin 3 (PTX3) values as an early predictor of subsequent renal involvement in patients with Henoch-Schönlein purpura (HSP) with no abnormalities on urinary examination and in renal function tests at disease onset. METHODS: This was a prospective cohort study which included 60 pediatric patients with HSP (age range 3-15 years) who were diagnosed between February 2011 and October 2012 and 60 age- and sex-matched healthy controls. The patients were followed up for at least 18 months. Clinical findings were recorded for all patients at first examination, and blood samples for routine laboratory parameters and PTX3 value as well as skin biopsy specimens were obtained from each subject. RESULTS: Of the 60 patients with HSP, 29 (48.3 %) developed subsequent renal involvement, of whom four underwent kidney biopsy. The mean serum PTX3 level of patients with subsequent renal involvement was significantly higher than those of patients without renal involvement and of the controls (2.20 ± 1.30 vs. 1.36 ± 0.85 and 1.03 ± 0.7 ng/ml, respectively; p = 0.004). Immunofluorescence evaluation of skin biopsy revealed that in addition to immunoglobulin A (IgA) deposition, the IgM deposition was significantly associated with subsequent renal involvement (p = 0.008). CONCLUSIONS: A high PTX3 level and IgM staining in skin biopsies from HSP patients may be harbingers of subsequent renal involvement.

Relation of age and sex with carotid intima media thickness in healthy children.

BACKGROUND/AIM: To investigate the age- and sex-associated carotid intima media thickness (clMT) changes in healthy children to determine the age- and sex-specific normal range of values for childhood. MATERIALS AND METHODS: This study examined 91 healthy school-age children aged 7-15 years prospectively. Standardized sonographic cIMT measurements and analyses were performed. Body mass index and blood pressure were obtained, and atherosclerotic risk factors were investigated. Age- and sex-specific cIMT measurements for different age groups were calculated and the relation with sex was investigated. RESULTS: Regarding the total study group, mean cIMT measurements for age groups 7-9, 10-12, and 13-15 years were 4.1 + 0.5 mm, 4.4 ± 0.6 mm, and 4.6 ± 0.4 mm, respectively. cIMT did not differ between boys and girls in the same age group. Age related analyses showed significant variations among the age groups with positive correlation between cIMT and age. CONCLUSION: Our results suggest that age-related physiologic thickening of the carotid artery intima-media occurs not only in adults but also in children and that sex is not a factor for cIMT differences in childhood.

Primary hypogonadism in a case with XLAG syndrome.

Patients with X-linked lissencephaly with ambiguous genitalia (XLAG) syndrome present with lissencephaly, agenesis of the corpus callosum, refractory epilepsy of neonatal onset, microcephaly, and male genotype with ambiguous genitalia. The basis of the ambiguous genitalia in XLAG syndrome is not well-known. We report a case of the fourth child of healthy consanguineous parents who was presented to the hospital because of non-febrile seizures at 2 months of life. On physical examination, microcephaly, some dysmorphic face features, and ambiguous genitalia were determined. The cranial magnetic resonance imaging of the patient showed lissencephaly, agenesis of the corpus callosum, and enlarged ventricles. His karyotype was 46, XY. He had undetectable testosterone levels and elevated gonadotropins. Neither testicular tissue nor any testosterone response to human chorionic gonadotropin stimulation test was observed. These findings suggest that the hypogonadism in this patient with XLAG syndrome is primary hypogonadism due to gonadal agenesis or dysgenesis.