Post-traumatic tension pneumocephalus and cystic angiomatosis of the skull: case report

Background Tension pneumocephalus is an increasing air trapped intracranially. Either spontaneous, post-traumatic or iatrogenic in origin. Cystic angiomatosis is a benign vascular hamartoma of the skeleton, when acquired it is either due to trauma or infection. This is the second report in English literature of post-traumatic delayed tension pneumocephalus with the development of cystic angiomatosis of the skull bone. Case presentation A 55-year-old gentlemen, presented with scalp swelling of 6-month duration with history of head trauma 2 years back. The swelling was increasing and associated with progressive walking difficulties and left hearing loss. CT scan and MRI revealed extradural pneumocephalus, parietal and occipital pneumatocele, and multiple lytic bony lesions, left mastoid hyperpneumatization with inner table defect communicating with the extradural space. Diagnosis of delayed extradural tension pneumocephalus was made. Surgical exploration revealed multiple bony defects of parietal, temporal and squamous part of left temporal bones, confirmed extradural pneumocephalus with intact dura. Repair of mastoid defect of (0.5 × 0.5 cm), excision of pneumatocele and removal of lytic bones were performed. Defective bone “cribriform-like” was identified at occipital and parietal regions centrally with a defect of nearly 7 × 7 cm. Future cranioplasty was considered after 6 months. Histology of bony chips and surrounding soft tissues is recognized as cystic angiomatosis. Conclusions The present case developed two very rare complications, following trivial head trauma; the first complication was delayed extradural tension pneumocephalus with pneumatocele which presented 2 years after trauma, the origin of air was from a defect of the inner table of the mastoid, the second complication was cystic angiomatosis of the skull bones. Both complications were managed surgically in one operative session as a combined neurosurgery and otolaryngology teams approach.

Bone Deformity due to Rickets and Osteomalacia in Children and Adolescents

Rickets is a worldwide bone disease that is associated with disorders of calcium and phosphate homeostasis and can lead to short stature and joint deformities. Osteomalacia is a major metabolic bone disease that results from a chronic and severe deficiency of vitamin D or phosphate from any cause after growth has stopped. A deficiency of vitamin D or phosphate leads to defective bone mineralization and generalized or localized vague bone pain in various parts of the skeleton and / or proximal muscle weakness. Rickets and osteomalacia are two different clinical diseases with impaired bone mineralization. Rickets occurs throughout the growing skeleton in infants and children, while osteomalacia occurs in adults after fusion of the growth plates. Rickets and osteomalacia are increasingly common in Saudi Arabia, with vitamin D deficiency being the most common etiological cause. Early skeletal deformities can occur in infants, such as soft, thin skull bones, a condition known as craniotabes. In adults, as a result of demineralization, the bones become less rigid (soft bone) with pathological fractures. The diagnosis of both diseases is based on the medical history and physical examination, radiological characteristics, and biochemical tests. Management depends on the underlying etiology.

Diabetic oxidative stress and bone loss complications

Diabetes mellitus is a group of metabolic disorder characterize by and absolute or partial insulin deficiency. Diabetic hyperglycemia is produce by the effect of homeostasis between proteolytic enzymes, their inhibitors and the antioxidants defense that protect and repair vital tissues and molecular components. Bone consist of both component and trabecular bone tissue. Organic matrix and albumin form part of noncollagenous of bone .Initiation of mineralization and collagen fibrils form the phase of mineral matrix. Calcium flux into and out of bone depend of osteoclastic and osteoblastic activity. The remodeling is initiated by resorption and new bone formation at the resorption site. Diabetic complication is a critical factor for bone pathology and could start early inflammatory stage even before hyperglycemia. Diabetic produces bone loss from reduce osteoblast activity. Partly insulin deficiency produce defective bone remodeling indirect by oxidative stress. The current treatment for defective bone in diabetes state include biophosphonate and cinaciguat. Biphosphonate inhibit bone resorption, but may worsen bone quality. A novel type of activation of sGMP is cinaciguat an NO independent activator of oxidative GC, increase c GMP synthesis on diabetic and restore proliferation and survival of osteoblasts. Chronic hyperglycemia interferes with the oseointegration of implants in diabetics. Both diabetic and aging plays a role in abnormal differentiation of osteroblasts. In diabetic patients may improve the oral health to have a positive impact if optimal glycemic control is emphasized. However with cinaciguat present as a novel paradigm enhancing bone formation under hyperglycemia and protect bone implants.

The secreted protein DEL-1 activates a β3 integrin–FAK–ERK1/2–RUNX2 pathway and promotes osteogenic differentiation and bone regeneration

The integrin-binding secreted protein developmental endothelial locus-1 (DEL-1) is involved in the regulation of both the initiation and resolution of inflammation in different diseases, including periodontitis, an oral disorder characterized by inflammatory bone loss. Here, using a mouse model of bone regeneration and in vitro cell-based mechanistic studies, we investigated whether and how DEL-1 can promote alveolar bone regeneration during resolution of experimental periodontitis. Compared with WT mice, mice lacking DEL-1 or expressing a DEL-1 variant with an Asp-to-Glu substitution in the RGD motif (“RGE point mutant”), which does not interact with RGD-dependent integrins, exhibited defective bone regeneration. Local administration of DEL-1 or of its N-terminal segment containing the integrin-binding RGD motif, but not of the RGE point mutant, reversed the defective bone regeneration in the DEL-1–deficient mice. Moreover, DEL-1 (but not the RGE point mutant) promoted osteogenic differentiation of MC3T3-E1 osteoprogenitor cells or of primary calvarial osteoblastic cells in a β3 integrin–dependent manner. The ability of DEL-1 to promote in vitro osteogenesis, indicated by induction of osteogenic genes such as the master transcription factor Runt-related transcription factor-2 (Runx2) and by mineralized nodule formation, depended on its capacity to induce the phosphorylation of focal adhesion kinase (FAK) and of extracellular signal-regulated kinase 1/2 (ERK1/2). We conclude that DEL-1 can activate a β3 integrin–FAK–ERK1/2–RUNX2 pathway in osteoprogenitors and promote new bone formation in mice. These findings suggest that DEL-1 may be therapeutically exploited to restore bone lost due to periodontitis and perhaps other osteolytic conditions.

EXPERIMENTAL STUDY OF INTEGRATION FEATURES OF POROUS PERMEABLE AND MESH TITANIUM NICKELIDE IN VIVO

Objective: Morphological assessment of the integration features of the combined use of porous permeable and textile mesh titanium nickelide with bone structures of the recipient zone. Methods: The study was carried out on 20 Chinchilla rabbits of both sexes at the age of 1-1.5 years with a bodyweight of 2500-4000 g. In animals, the artificial defect was created in one of the areas of the lower jaw, where a porous permeable titanium nickelide was installed, wrapped in a textile mesh version of it was installed. Animals were removed from the experiment for 30, 90, 180, and 360 days of research via intraperitoneal injection of mortal dose 1% solution thiopental sodium. The material for histological research was fixed in 10% buffered formalin during 12 hours after then washed with water and decalcified. The prepared sections were stained with hematoxylin-eosin. Microscopic examination of the stained preparations and photography were carried out using a light microscope Axioscope 40 (Zeiss, Germany) via a digital camera (Canon, Japan). Results: Studies have shown that with the combined use of porous permeable and textile mesh titanium nickelide, the artificially created defects of the mandibula between both contacting surfaces are formed directly, which leads to stable fixing. The porous structure of the material, its hysteresis behavior with organism tissues, provides widespread regeneration of cells and formed a homogeneous mature bone tissue bone tissue, both in the pores and around the structure. Conclusions: These and other facts indicate the high integration properties of the material that is investigated, which ensures optimal growth and population of osteogenic cells in the vicinity of defective bone areas in a growing organism. Keywords: Bone defect, osteointegration, regeneration, titanium nickelide, porous permeable implant.

Clinical application experience of asfotase alfa for a young patient with childhood hypophosphatasia

Hypophosphatasia (HPP) is a rare hereditary metabolic disease characterized by defective bone and dental mineralization, systemic complications that lead to disability of patients. HPP is classified into six forms according to the age of onset and severity of its clinical picture. The disease is caused by a reduced activity of the tissue nonspecific alkaline phosphatase (TNSALP) and elevated concentrations of pyrophosphates. Asfotase alfa is the only pathogenetic enzyme replacement therapy with recombinant human bone-targeted TNSALP approved for treatment of patients with perinatal, infantile and juvenile‐onset HPP. This treatment is associated with improved skeletal mineralization, respiratory function and overall survival in infants and young children with life-threatening hypophosphatasia. The world experience in application of recombinant alkaline phosphatase in adults is very limited. We present a clinical case that describes the first Russian experience in the use of asfotase alfa in an 18-year-old patient with late diagnosis of childhood-onset HPP.

Rare cause of pathological fracture in adults as hypophosphatemic rickets

Hypophosphatemic rickets is a disorder of defective bone minerlization due to defect in renal phosphate handling process. It is characterised by increased phosphate excretion accompanied by increased phosphatonins like fibroblast growth factor 23. It can be hereditary form of X linked, autosomal dominant, autosomal recessive type of hypophosphatemic rickets. It is associated with low serum phosphorus, normal serum calcium, inappropriately low to normal vitamin D level. Correct identification of these disorders is important for determining therapy. Early diagnosis and management prevent subsequent complication of the disease.