Diced ear cartilage with perichondrial attachment in rhinoplasty: a new concept

Background: Diced cartilage is a valuable material that has recently been added to the graft options in rhinoplasty. Shaping, fixation, and resorption are the main concerns with this material. Perichondrially attached diced conchal cartilage may be a new possibility to solve some of these problems. Objectives: The authors evaluate the outcome of perichondrially attached diced cartilage in a rabbit model and compare the results with injectable cartilage grafting. Methods: Ear cartilage was removed from 1 auricle in each of the 16 rabbits included in this study; samples were divided in 2 pieces. After precise weighing, both segments were diced. The perichondrium was left attached to 1 of the pieces. Both segments were inserted in 2 separate pockets in the dorsum of the animal. After a 3-month period, both samples were removed and measured for growth/resorption. Results: At the beginning of this study, the difference in weight between groups was statistically insignificant (P = .213), but 3 months after insertion, significant growth was observed in the perichondrial group (P = .019). Conclusions: The vascularization and significant growth in weight of the perichondrially attached diced cartilage samples are evidence of the viability of this material. The structural integrity and solid framework afforded by this option suggest that the material should be used more frequently in nasoskeletal augmentation.     

Xanthogranulomatous osteomyelitis

Xanthogranulomatous osteomyelitis is a rare type of inflammatory process which is characterized by composition of immune cell aggregation on histological studies. Delayed-type hypersensitivity reaction of cell-mediated immunity may be implicated in its pathogenesis. Gross and radiological examination can mimic malignancy, and differentiation should be confirmed by histopathological evaluation. We describe the case of a 14-year-old Afghan boy presenting with pain in right shoulder and left leg with prior history of trauma. Fever, limitation in right shoulder range of motion, and tenderness in right shoulder and left thigh were detected following examination. Mild leukocytosis, elevated alkaline phosphatase, and increased erythrocyte sedimentation rate with negative C-reactive protein (CRP) were revealed. X-ray imaging showed mixed density, periosteal reaction, and cortical disruption. Computed tomography (CT) scan revealed lesions involving medulla and cortex, periosteal reaction with soft tissue component, and bone marrow infiltration in right humerus and left fibula. On magnetic resonance imaging (MRI), signal abnormalities in medulla, metaphysis, and diaphysis of the left fibula associated with cortical irregularity and diffuse soft tissue hypersignal areas were demonstrated. Finally, xanthogranulomatous osteomyelitis was confirmed by histological sample. The clinical manifestations and radiographic and laboratory findings of this rare condition are discussed.     

Conditioned Media of Choroid Plexus Epithelial Cells Induces Nrf2-Activated Phase II Antioxidant Response Proteins and Suppresses Oxidative Stress-Induced Apoptosis in PC12 Cells

Based on the critical role of the choroid plexus (CP) in detoxification processes in the central nervous system (CNS), herein we investigated the effect of choroid plexus epithelial cells conditioned media (CPECs-CM) under oxidative conditions. CPECs were isolated from rat brains, cultured, and the conditioned media were collected. Then pheochromocytoma neuron-like cells (PC12) were treated simultaneously with CPECs-CM and H₂O₂ as the oxidative stressor. Next, the effect of CPECs-CM on neurite outgrowth and cell differentiation in the presence of H₂O₂ was determined. Our results showed that CPECs-CM improved the expansion of neurites and differentiation in PC12 cells under oxidative stress conditions. Changes in apoptotic factors, nuclear factor erythroid 2-related factor 2 (Nrf2) and γ-glutamylcysteine synthetase as the highlighted pathway in the antioxidant defense system were determined by western blot. Also, the activity of antioxidant enzymes and lipid peroxidation level were determined. CPECs-CM-treated PC12 cells could survive after exposure to H₂O₂ by reduction of caspase-3 cleavage and Bax level and elevation of anti-apoptotic factor Bcl2. Our data also revealed that Nrf2 activation, and consequently its downstream protein levels, increased in the presence of CPECs-CM. Based on our data, we can conclude that CPECs-CM protects PC12 cells against oxidative stress and apoptosis. It seems that CPECs secrete antioxidative agents and neurotrophic factors that have a role in the health of the CNS.     

Adverse reactions of prophylactic intravenous immunoglobulin infusions in Iranian patients with primary immunodeficiency.

BACKGROUND: Although long-term intravenous immunoglobulin infusion is an effective treatment for children with antibody deficiencies, it can be complicated by systemic adverse reactions. OBJECTIVE: To evaluate the adverse reactions of intravenous immunoglobulin therapy in patients with primary immunodeficiency. METHODS: Seventy-one immunodeficient patients receiving intravenous immunoglobulin were evaluated during a 7-year period (1995-2002) at Children's Medical Center in Tehran, Iran. Immunological diagnoses were as follows: common variable immunodeficiency (31 patients), X-linked agammaglobulinemia (25 patients), IgG subclass deficiency (5 patients), hyper-IgM syndrome (2 patients), and ataxia-telangiectasia (8 patients). RESULTS: One hundred fifty-two cases (12.35%) of adverse reactions occurred following 1,231 infusions in 35 patients. The most frequent immediate adverse reactions were mild reactions (131 infusions), including chills, fever, flushing, muscle pains, nausea, headache, and anxiety. Moderate reactions, such as vomiting, chest pain, and wheezing, occurred in 19 infusions. Two patients experienced severe adverse reactions. The highest proportion (23.06%) of reaction to injection was in patients with common variable immunodeficiency. CONCLUSIONS: Intravenous immunoglobulin is a well tolerated medical agent for patients with antibody deficiency. However, to prevent occurrence of immediate adverse reactions during infusion in these patients, physicians should perform a detailed history and proper physical examination and check the titer of anti-IgA.     

X-Linked Agammaglobulinemia: A Survey of 33 Iranian Patients

In order to determine the clinical and laboratory features of X-linked agammaglobulinemia, the records of 33 male patients with XLA were reviewed during 22 years (1980-2002) in the Iranian referral center of primary immunodeficiency disorders. The patients' ages ranged from 20 to 360 months (median 113 months). The median age at the onset of the disease was 8 months and the median age of diagnosis was 48 months, with a median diagnosis delay of 33 months. Almost all of the patients presented common infectious diseases, which were: pneumonia, otitis, diarrhea, sinusitis, and arthritis. During the course of illness, infections in the respiratory tract, gastrointestinal tract, central nervous system, and musculoskeletal system were seen in 93.9%, 75.8%, 33.3%, and 21.2% of XLA patients, respectively. The most common complications of these patients were chronic infections in 75.8% of them, including: chronic otitis media, chronic sinusitis, chronic diarrhea, and bronchiectasis.     

Wheat anaphylaxis in children

Food anaphylaxis is now the leading known cause of anaphylactic reactions treated in emergency departments, and wheat is one of the most common causes of anaphylaxis. Wheat is an important source of food worldwide. Wheat anaphylaxis is increasingly observed in our clinic. The purpose of this study was to describe the clinical features of wheat-induced anaphylaxis in 19 children for better elucidation of this disease. Children with severe reactions after ingestion of small amounts of wheat were referred to our clinic during a 4-year period. A detailed clinical history was recorded for each of the patients and a skin prick test was performed with wheat allergen extracts. The wheat-specific IgE and total IgE were measured. Grading of anaphylaxis episodes was performed according to a specific grading system. We identified 36 episodes of wheat anaphylaxis in 19 patients. All of the first attacks of wheat anaphylaxis occurred in the first-time ingestion. The most frequent manifestations of the reactions were skin and respiratory symptoms. In this study 78.9% of reactions were moderate and 21.1% of them were severe. All of our patients had positive skin prick tests to wheat. Mean total IgE level was 853.4 ± 455.27 IU/ml, and mean wheat-specific IgE was 70 ± 14.61 Ucs/ml. We conclude that wheat-induced anaphylaxis is a disease that is sufficiently severe, and. prevention of first wheat-induced anaphylaxis episodes is almost impossible. It would, however, probably be good practice to educate physicians to recognize the common clinical manifestations of this disease for early management.     

Use of D11S2179 and D11S1343 as markers for prenatal diagnosis of ataxia telangiectasia in Iranian patients

Ataxia telangiectasia (AT) is an autosomal recessive disorder with an estimated prevalence of 1/40,000 to 1/100,000 in reported populations. There is a 25% possibility for having an affected child when parents are carriers for the ATM gene mutation. There is no cure available for this disease and prenatal testing is strongly recommended for prevention of this disease. Although the preferred method is the direct mutation analysis of the ATM gene, the large size of the ATM gene with 63 exons and the large number of possible mutations in patients considerably limit efficiency of mutation analysis as a diagnostic choice. Indirect method is a better tool when parents are not carriers of founder mutation and pass different mutations to their children. Indirect molecular diagnosis using ATM-related molecular markers facilitates prenatal diagnosis of AT children. In this study, four molecular markers: D11S2179, D11S1787, D11S535, D11S1343 are genotyped in 19 unrelated families from different regions of Iran. Those markers are amplified using extracted sequence primers from the Gene Bank with their described PCR conditions. Amplified products were separated using denaturing PAGE gels, and data were analyzed to detect their pattern of inheritance in each family. In all families, segregation of alleles was according to Mendelian inheritance, and affected chromosomes were distinguishable from unaffected ones. All carriers and affected patients were diagnosed accurately. Thus, this method is effectively useful in prenatal diagnosis of AT.     

Cyclooxygenase (COX)-1 Activity Precedes the COX-2 Induction in Aβ-Induced Neuroinflammation

Two different isoforms of cyclooxygenases, COX-1 and COX-2, are constitutively expressed under normal physiological conditions of the central nervous system, and accumulating data indicate that both isoforms may be involved in different pathological conditions. However, the distinct role of COX-1 and COX-2 and the probable interaction between them in neuroinflammatory conditions associated with Alzheimer’s disease are conflicting issues. The aim of this study was to elucidate the comparable role of each COX isoform in neuroinflammatory response induced by β-amyloid peptide (Aβ). Using histological and biochemical methods, 13 days after stereotaxic injection of Aβ into the rat prefrontal cortex, hippocampal neuroinflammation and neuronal injury were confirmed by increased expression of tumor necrosis factor-alpha (TNF-α) and COX-2, elevated levels of prostaglandin E2 (PGE2), astrogliosis, activation of caspase-3, and neuronal cell loss. Selective COX-1 or COX-2 inhibitors, SC560 and NS398, respectively, were chronically used to explore the role of COX-1 and COX-2. Treatment with either COX-1 or COX-2 selective inhibitor or their combination equally decreased the level of TNF-α, PGE2, and cleaved caspase-3 and attenuated astrogliosis and neuronal cell loss. Interestingly, treatment with COX-1 selective inhibitor or the combined COX inhibitors prevented the induction of COX-2. These results indicate that the activity of both isoforms is detrimental in neuroinflammatory conditions associated with Aβ, but COX-1 activity is necessary for COX-2 induction and COX-2 activity seems to be the main source of PGE2 increment.