Limited value of zinc protoporphyrin as a marker of iron status in chronic hemodialysis patients

BACKGROUND: In an attempt to find new parameters able to evaluate the actual iron availability by bone marrow cells, zinc protoporphyrin (ZnPP), a metabolic intermediate generated in the red blood cell by the incorporation of zinc instead of iron, has been proposed. ZnPP is a good marker of iron-deficiency anemia in non-uremic people, as red blood cell ZnPP concentration rises specifically (except for lead intoxication) in this condition. Existing data on ZnPP as a marker of iron deficiency in uremic patients comes mainly from cross sectional studies on chronic hemodialysis and has produced conflicting results. SUBJECTS AND METHODS: Therefore, we prospectively studied 42 HID patients, 28-88 years old, 13-346 months of dialysis age, beginning from a period of maximal iron deficiency, due to the lack of parenteral iron compounds (T0) up to the end of more than one year of follow-up with continuous parenteral iron supplementation (T4). ZnPP, hemoglobin, transferrin saturation and ferritin were serially determined before and after six weeks (T1), four months (T2), seven months (T3) and 14 months (T4) of parenteral iron supplementation at a maintenance dose of 0.5-1 mg/kg/week. RESULTS: In comparison with baseline values (95+/-37 micromol/mol heme) there were no significant changes in ZnPP levels at T1 and T2 despite a continuous increase in both transferrin saturation and ferritin values, while ZnPP significantly decreased at T4 (63+/-37 micromol/mol heme, p<0.001). There was no correlation between ZnPP and both transferrin saturation and ferritin at any time during the study, the same was true for ZnPP and zinc and lead serum concentration, fibrinogen and reactive C protein levels at T1 and T4, respectively. At T4, only 2/10 patients who still showed ZnPP levels >80 micromol/mol heme had absolute or functional iron deficiency, when the percentage of hypochromic red cells were measured. CONCLUSION: We conclude that ZnPP untimely parallels a change in iron balance in only a proportion of uremic people, in as much as confounding factors, such as chronic inflammation and uremia in itself may obscure its relationship with iron status. Therefore, ZnPP cannot be assumed to be a first-line diagnostic marker of iron balance in uremic patients.     

Die kongenitale Tibiapseudarthrose

Congenital pseudarthrosis of the tibia (CPT) is one of the most challenging conditions in paediatric orthopaedics. The history of CPT treatment is characterised by repeatedly ineffective surgical interventions with the inevitable outcome of severe disability. Most procedures failed to account for the complexity of the problem. The past two decades have witnessed more experience having been gained with the Ilizarov technique. This method provides a comprehensive approach to all aspects of CPT problem, allowing the surgeon to simultaneously address the problems of union as well as those of deformity, length discrepancy joint function, ankle valgus and weight bearing. It appears that the goal to achieve stable fusion before the child begins school is attainable. In the pre-pseudoarthrotic stage, bracing should be preferred to surgery until the patient is in an older age group. This study reports on the results of 19 patients: 15 have finished treatment, and 14 of them with a stable fusion at follow-up. The results were highly encouraging in reaching the ultimate goal of avoiding amputation and achieving a well-functioning limb.     

Long term follow up of 69 patients treated for optic pathway tumours before the chemotherapy era

AIM—To analyse the long term results of conservative management with radiotherapy in patients with optic pathway tumours.DESIGN—All 69 patients were symptomatic at diagnosis and most neoplasms involved the optic chiasm and hypothalamus.RESULTS—At 10 years, overall survival and progression free survival were 83% and 65.5%, respectively. After radiotherapy, vision improved in 18 patients and remained stable in 29 other patients. Cerebrovascular complications occurred in nine of 53 patients treated with radiotherapy after a median interval of two and a half years. These complications were five times more frequent in patients with neurofibromatosis type 1 (NF1). Severe intellectual disabilities were present in 18 children, most of whom underwent irradiation at a very young age (median age, 4years).IMPLICATIONS—Radiotherapy is a valuable treatment in terms of tumour response, visual outcome, and progression free survival. However, in young children and in patients with NF1, major sequelae are encountered and new treatment strategies should be proposed for these patients.     

Patterns of neuropsychological deficits in children with medulloblastoma according to craniospatial irradiation doses

This study aimed to analyse the relationship between supratentorial irradiation dose and the intellectual outcome in 36 children (aged between 5 and 15 years) treated for medulloblastoma. The supratentorial radiation dose was reduced to 25 Gy in 23 children and given at the standard dose, 35 Gy, in 13 other children. Neuropsychological evaluation was performed at a mean of 4.3 years (SD 4.7 years) after radiotherapy. The supratentorial radiation dose was the principal risk factor associated with impaired intellectual outcome. Verbal fluency, immediate word list recall, block design, and fine motricity of the dominant hand were significantly lower in children irradiated at the standard doses than in those irradiated at reduced doses. These findings suggest that the dose of radiotherapy applied to the brain strongly influences later verbal and non-verbal skills in children with medulloblastoma. This should be taken into account in treatment planning and in rehabilitation programs.     

Long-term effects on feeding and body weight after stimulation of forebrain or hindbrain CRH receptors with urocortin

Research on the contribution of CRH receptor stimulation to energy homeostasis has focused on forebrain substrates. In this study, we explored the effects of caudal brainstem administration of the CRH receptor agonist, urocortin, on food intake and body weight, and on plasma glucose and corticosterone (CORT) in non-deprived rats. Urocortin (0, 0.3, 1, 3 microg) delivered, respectively, to the fourth and lateral ventricles yielded substantial suppression of food intake measured 2, 4 and 24 h later. A significant but more modest anorexia was observed between 24 and 48 h after injection. Intake responses did not differ between the injection sites, but body weight loss measured 24 h after lateral-i.c.v. injection was substantially greater than that after fourth-i.c.v. injection. Fourth-i.c.v. urocortin administration (3 microg) produced substantial elevations in plasma glucose and CORT that were not distinguishable in magnitude and duration from responses to lateral-i.c.v. delivery. Unilateral microinjection of urocortin into the dorsal vagal complex significantly reduced 24-h food intake at a dose (0.1 microg) that was subthreshold for the response to ventricular administration, suggesting that fourth-i.c.v. effects are mediated in part by stimulation of CRH receptors in this region of the caudal brainstem. The results indicate that similar effects can be obtained from stimulation of anatomically disparate populations of CRH receptors, and that interactions between forebrain and hindbrain structures should be considered in the evaluation of CRH contributions to food intake and body weight control.     

Paravaginal defect: A new classification of fascial and muscle tears in the paravaginal region

The lateral support of the vaginal wall depends on the integrity of the paravaginal section of the visceral pelvic fascia, levator ani, and their connection. Various defects of the muscle and fascia can result in identical clinical findings—ie, the descent of the lateral vaginal sulcus. In this study, we created a realistic scheme for classifying paravaginal defects, based on the complex relationship of the pelvic fascia with the levator ani. Surgical observations, cadaver examinations, and a complex magnetic resonance imaging (MRI)‐based 3‐dimensional (3D) model were used to analyze the spatial relationships of normal and defective anatomy of the female pelvic floor. Descent of the lateral vaginal sulcus can result from a defect in the paravaginal visceral pelvic fascia, levator ani, or both. The fascial defect can be partial or complete, and the muscle defect can vary in location. A detailed illustrated classification is presented. We present a new model of the pathology that underlies a common clinical finding. Clin. Anat. 29:524–529, 2016. © 2016 Wiley Periodicals, Inc.