Hypoxic-ischemic brain injury is a very important neurological problem of the perinatal period and a major cause of chronic disability later in childhood. The subsequent neurological deficits are a variety of motor defects-especially spasticity but also choreoathetosis, dystonia and ataxia, often grouped together as "cerebral palsy," mental retardation, and seizures. The gestational age determines the neuropathology of the brain injury. One of the patterns of hypoxic-ischemic encephalopathy, typically affecting full-term infants, consists of parasagittal lesions and ulegyria. The aim of this study is to describe the magnetic resonance imaging (MRI) features and discuss the "suggested" pathogenetic mechanisms of this pattern, which affects the cortex and the white matter in a mainly parasagittal distribution; in this type of brain injury, the damage usually involves the deeper sulcal portion while sparing the apex, thus resulting in the so-called mushroom gyri characteristic ulegyric pattern. We discuss the MRI findings of parasagittal lesions and ulegyria in the brain examinations of 14 patients with a clinical history of perinatal hypoxia/anoxia presenting with mental retardation, seizures, and cerebral palsy. Differential diagnosis from polymicrogyria is discussed.

The position and morphology of the head of the epididymis were abnormal in all boys with acute testicular torsion but with preserved testicular flow.

We report a case of a 13-year-old girl with chronic recurrent multifocal osteomyelitis (CRMO) who developed severe arthritis in four different joints within the first year from the onset of the disease. Her multiple vertebrae lesions showed significant amelioration after a 2-month treatment with prednisolone. In parallel, the initial severe symmetrical arthritis of both knees showing overt synovitis and joint effusion, in the absence of lesions in the metaphyses of the femur or the tibia, responded remarkably well in intra-articular triamcinolone hexacetonide injections. However, upon discontinuation of prednisolone, the patient developed severe arthritis of her right ankle and the proximal interphalangeal joint of her right middle finger. Thus, prednisolone was reinitiated combined with methotrexate, and the patient went into remission, which persists one year after prednisolone tapering. The appearance of arthritis in both knees in the absence of bone lesions and the emergence of severe arthritis of the ankle after remission of spinal bone lesions suggest that CRMO and juvenile idiopathic arthritis may coexist and be causally related.

Gender assignment of cases with frank genital ambiguity is often difficult to be determined, because several factors have to be taken into consideration, such as genital appearance, anticipated urological and sexual function, capacity for future fertility, gonadal malignancy risk, and psychosocial factors. A multidisciplinary approach is definitely needed in the management of such cases.

IntroductionPreclinical work and studies in adults have shown that endogenous regeneration efforts that involve mobilization of progenitor cells take place after brain injury. However, kinetics of endogenous circulating progenitor cells (CPCs) in preterm neonates is not well described, particularly their possible role regarding brain injury and regeneration. We aimed to assess the kinetics of CPCs in neonates with encephalopathy of prematurity in relation to brain injury biomarkers, chemoattractants and relevant antenatal and postanal clinical factors, in an effort to outline the related pathophysiology.Materials and methods47 preterm neonates (of 28–33 weeks GA) were enrolled: 31 newborns with no or minimal brain injury (grade I IVH) and 16 prematures with encephalopathy (grade III or IV IVH, PVL or infarct). Peripheral blood samples obtained on days 1, 3, 9, 18 and 45 after birth were analyzed using flow cytometry, focusing on EPCs (early and late Endothelial Progenitor Cells), HSCs (Hematopoietic Stem Cells) and VSELs (Very Small Embryonic-Like Stem Cells). At the same time-points serum levels of S100B, Neuron-specific Enolase (NSE), Erythropoietin (EPO), Insulin-like growth factor-1 (IGF-1) and SDF-1 were also measured. Neonates were assessed postnatally with brain MRI, and with Bayley III developmental test at 2 years of corrected age.ResultsPreterms with brain injury proved to have significant increase of S100B and NSE, followed by increase of EPO and enhanced mobilization mainly of HSCs, eEPCs and lEPCs. IGF-1 was rather decreased in this group of neonates. IGF-1 and most CPCs were intense decreased in cases of antenatal or postnatal inflammation. S100B and NSE correlated with neuroimaging and language scale in Bayley III test, providing good prognostic ability.ConclusionThe observed pattern of CPCs’ mobilization and its association with neurotrophic factors following preterm brain injury indicate the existence of an endogenous brain regeneration process. Kinetics of different biomarkers and associations with clinical factors contribute to the understanding of the related pathophysiology and might help to early discriminate neonates with adverse outcome. Timely appropriate enhancement of the endogenous regeneration effort, when it is suppressed and insufficient, using neurotrophic factors and exogenous progenitor cells might be a powerful therapeutic strategy in the future to restore brain damage and improve the neurodevelopmental outcome in premature infants with brain injury.

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A boy born to healthy, young, nonconsanguineous, white parents was born at 40 weeks of gestation by vaginal delivery after an uneventful pregnancy to a gravida I mother. The Apgar score was 9 and 10 at 1 and 5 min, respectively. Anthropometric parameters including birth weight (3480 g), length (52 cm), and head circumference (35.5 cm) were all normal (25th-50th centiles). On physical examination, the baby presented a constriction ring of the left arm, hypoplastic left upper limb (Fig. 1a), wrist, and fingers of the left hand in contracture (Fig. 1b), a skin-covered mass extending asymmetrically into the buttock, and a 7 cm caudal appendage of a soft, boneless, well-circumscribed tissue located at the sacrococcygeal region (Fig. 1c). Ultrasonography and MRI indicated a closed spinal dysraphism with nonossified posterior elements of superior sacral vertebrae and a lipomyelocele. The spinal cord was tethered at the level of L4-L5 tracted by the large lipoma, whereas fatty tissue was projecting out of the spinal canal inside the caudal appendage (Fig. 2). Liver microcalcifications and a bilateral grade I hydronephrosis were also observed on abdominal ultrasound. On ophthalmic examination, the retinal epithelium was hypopigmented, whereas the optic nerves and the fovea were intact. Heart anatomy and function were normal.The affected left upper limb was treated by multiple Z-plasty combined with removal of fibrous groove and fasciotomy. Neurosurgical excision of the caudal appendage was also performed. On last examination, at the age Fig. 1Left arm (a) and hand deformities (b) and the caudal appendage (c).