This study sought to determine delta granule normal ranges for children and to validate methodology for the appropriate diagnosis of delta granule deficiency (storage pool disease) by using the whole-mount technique in electron microscopy. Specimens obtained from 40 healthy volunteers (2 months of age through 21 years old, 21 females and 19 males) were tested. Results showed dense granules/platelet (DG/Plt) ranged from 1.78 to 5.25. The 5th percentile was 1.96 DG/Plt with an overall mean ± SEM 3.07 ± 0.12 DG/Plt. In comparison, a previously published lower cutoff value, 3.68 DG/Plt, was significantly higher than the mean from our volunteers (P < 0.0001). We found no variability in dense granules/platelet based on race or sex and no significant variation by age subgroup. Pending wider studies, the value of 2 DG/Plt is a more appropriate lower limit of normal. In the absence of wider studies (in healthy volunteers and patients), laboratories should consider establishing their own reference ranges.
The rarity of glucagonoma imposes a challenge with most patients being diagnosed after a long period of treatment for their skin rash (months-years). Awareness of physicians and dermatologists of the characteristic necrolytic migratory erythema often leads to early diagnosis. Early diagnosis of glucagonoma even in the presence of resectable liver metastases may allow curative resection. Herein, we present a typical case of glucagonoma treated at our center and review the literature pertinent to its management.
Xanthogranulomatous inflammation is a well-described inflammatory process, which may involve any organ but is most frequently encountered in the gall bladder and the kidney. There are rare reports of xanthogranulomatous appendicitis (XA) in the adult population, but only one brief mention of such a diagnosis in a child. In this report, we describe the case of an 11-year-old boy who presented with clinical signs and symptoms of acute appendicitis necessitating appendectomy. Upon microscopic examination, the appendix showed the typical features of XA. To the best of our knowledge, this is the first well-described case XA in a noninterval appendix in a child. We also reviewed the limited medical literature on the subject.
Objectives: We report our experiences of brain tumors management by surgery and radiotherapyWhether It has been used as adjuvant, radical or palliative therapy belonging to benign or malignant tumors over 2 years period at ENT, neurosurgery and radiotherapy departments royal medical services.
Methods: We used a retrospective study design to review all brain tumor patients(benign, malignant or metastatic) operated in neurosurgical department and referred to radiotherapy after discussing each case in multidisciplinary clinic in 2018 and 2019. Patient files, radiological images computed tomography (CT) or magnetic resonance imaging (MRI) scans), histo-pathological reports and radiotherapy management plan were reviewed for patients.
Results: In total, 137 patients with brain tumor managed byradiotherapy 64 patients were metastatic, 37 patients high grad glioma, 12 patients atypical meningioma, 7 patients medulloblastoma, rest of cases discussed in the study .
Conclusions: Radiotherapy has been fulfilling crucial part in the treatment of CNS tumors, where it has been implemented as adjuvant therapy or even being the solitary resort where surgery is inapplicable or used as palliative therapy in different regimens according to histopathology, performance status and different sites.
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