Clinical Therapeutic Outcomes Research Articles

The treatment of venous malformations with percutaneous sclerotherapy at a single academic medical center.

We report a retrospective analysis of venous malformation patients treated with percutaneous sclerotherapy, describing their clinical manifestations, therapeutic outcomes and procedural complications. We reviewed our Vascular Anomalies database for all patients who underwent percutaneous sclerotherapy for venous malformation between January 2005 and July 2011 and retrieved 186 patients, out of which 116 were included in the final analysis. The majority of patients were treated using 100% alcohol (72%) and the rest were treated with <100% alcohol, Sodium Tetradecyl Sulfate or combination of these therapies. The most common location was the lower extremity in 67 patients (58%), followed by the head and neck in 27 (23%) and the upper extremity in 11 (9%). Retrospective review of medical records was performed. Outcomes were classified on an improvement scale based on clinical therapeutic effects. Two-hundred and forty-five sclerotherapy procedures were performed in 116 patients, of which 52 patients (45%) underwent a single procedure, 32 (28%) had two procedures and 32 (28%) underwent ≥3 procedures. Median follow-up period from the last procedure was 2.5 months (interquartile range of 2.0 to 6.75 months). Significant improvement was seen in 37 patients (32%), moderate improvement in 31 (27%), mild improvement in 20 (17%), no improvement in 21 (18%) and worse than before in 7 (6%) patients. Major post-procedural complications were nerve injuries in 6 patients (5%), deep vein thrombosis in 5 (4%), muscle contracture in 2 (2%), infection in 3 (3%), skin necrosis in 4 (3%) and other complications in 3 (3%). Our study demonstrated that 76% of our patients with venous malformation had some level of improvement in symptoms with majority (72%) undergoing only one or two percutaneous sclerotherapy procedure/s. Although major complications occurred in 20% of the patients, majority (74%) of the complications either resolved spontaneously or were successfully treated.

Clinical features and prognosis of conjunctival lymphoid hyperplasia

Background Primary conjunctival lymphoid hyperplasia exhibit atypical clinical symptoms and easy to cause misdiagnosis and mistreatment.Understanding the clinical characteristics of primary conjunctival lymphoid hyperplasia is of an important clinical significance. Objective This study was to analyze the clinical characteristics and therapeutic outcome of patients with primary conjunctival lymphoid hyperplasia. Methods The medical records of 20 patients with primary conjunctival lymphoid hyperplasia who was treated in Beijing Tongren Hospital from January 2012 to December 2014 were retrospectively analyed.The age, symptoms, therapy, prognosis, histopathological features and immonochemistry results were reviewed, and the treating approach targeting to different types of conjunctival lymphoid hyperplasia was evaluated.The fellow-up duration was 1-2 years. Results Age of the patients ranged from 27 to 83 years old.The lesions were classified as benign lymphocytic hyperplasia, atypical lymphoid hyperplasia and lymphoma.Majority of lesions located in fornix conjunctiva tissue (90%). Follicle-like appearance was seen in the benign lymphocytic hyperplasia, and pink elavation was exhibited at the bulbar conjunctiva in the atypical lymphoid hyperplasia.A diffuse salmon fish like appearance in conjunctiva was the primary feature of conjunctival lymphoma.Histopathological examination showed that conjunctival benign lymphocytic hyperplasia had follicle-like tissue in lesions, atypical lymphoid hyperplasia exhibited diffuse lymphocyte filtration, and conjunctival lymphoma appeared monocyte filtration.The 3 tpyes of lesions also could be differentiated by immunochemistry.The medicine was applied in the eyes with benign lymphocytic hyperplasia, and the combination of resection of lesions with conjunctival tissue or amniotic membrane transplantation was used for the atypical lymphoid hyperplasia or lymphoma eyes.No recurrence of the lesions was found during fellow-up duration. Conclusions The clinical manifestations of conjunctival lymphoid hyperplasia lesions are varied, and it is easy to be confused with chronic ocular surface diseases.Pathology and immunochemistry are helpful for the differential diagnosis.The treating regimen is dependent on the lesion type.Most patients have a favorable prognosis with treatment. Key words: Conjunctival diseases/pathology; Conjunctival neoplasms/diagnosis & therapy; Hyperplasia; Lymphoid tissue/pathology; Lymphoma/pathology; Retrospective studies; Humans

A Comprehensive Review on the Predictive Performance of the Sheiner-Tozer and Derivative Equations for the Correction of Phenytoin Concentrations

In settings where free phenytoin concentrations are not available, the Sheiner-Tozer equation-Corrected total phenytoin concentration = Observed total phenytoin concentration/[(0.2 × Albumin) + 0.1]; phenytoin in µg/mL, albumin in g/dL-and its derivative equations are commonly used to correct for altered phenytoin binding to albumin. The objective of this article was to provide a comprehensive and updated review on the predictive performance of these equations in various patient populations. A literature search of PubMed, EMBASE, and Google Scholar was conducted using combinations of the following terms: Sheiner-Tozer, Winter-Tozer, phenytoin, predictive equation, precision, bias, free fraction. All English-language articles up to November 2015 (excluding abstracts) were evaluated. This review shows the Sheiner-Tozer equation to be biased and imprecise in various critical care, head trauma, and general neurology patient populations. Factors contributing to bias and imprecision include the following: albumin concentration, free phenytoin assay temperature, experimental conditions (eg, timing of concentration sampling, steady-state dosing conditions), renal function, age, concomitant medications, and patient type. Although derivative equations using varying albumin coefficients have improved accuracy (without much improvement in precision) in intensive care and elderly patients, these equations still require further validation. Further experiments are also needed to yield derivative equations with good predictive performance in all populations as well as to validate the equations' impact on actual patient efficacy and toxicity outcomes. More complex, multivariate predictive equations may be required to capture all variables that can potentially affect phenytoin pharmacokinetics and clinical therapeutic outcomes.

THU-040 - Clinical Features, Diagnosis and Therapeutic Associated Outcomes in Clear Cell Hepatocellular Carcinoma

THU-040 - Clinical Features, Diagnosis and Therapeutic Associated Outcomes in Clear Cell Hepatocellular Carcinoma

Markedly High Plasma Thrombopoietin (TPO) Level is a Predictor of Poor Response to Immunosuppressive Therapy in Children With Acquired Severe Aplastic Anemia.

Immunosuppressive therapy (IST) is commonly used for patients with acquired severe aplastic anemia (SAA). Because the clinical response rate and therapeutic outcome for individual patients to IST varies, an in vitro test that identifies potential responders would be desirable. We evaluated the relationship between thrombopoietin (TPO) levels at the time of diagnosis and the response to IST at 6 months in 85 children (median age, 9.0 years; range, 1.0-15.5 years) with acquired SAA using enzyme-linked immunosorbent assay. Thirty-one age-matched healthy individuals were used as controls. All patients received antithymocyte globulin and cyclosporine. Overall, 39 patients (45.9%) responded to IST at 6 months. TPO plasma levels were significantly higher in nonresponders than in responders (1,555.8 vs. 1,284.7 pg/ml, respectively; P = 0.031). Multivariate analysis identified the TPO levels of >1,796.7 pg/ml (TPO-high group, 20 patients; odds ratio (OR), 8.285; 95% confidence interval (CI), 2.114-32.904; P = 0.002) as independent poor predictors of IST response at 6 months. Moreover, the TPO-high group was associated with lower 5-year failure-free survival rates (30% vs. 68%, P = 0.012) compared with the TPO-low group. The measurement of TPO levels at diagnosis is useful for predicting the response to IST in children with SAA and may help in decision making.

Abstract 19031: Prediction and Incidence of Non-PV Trigger Foci of Atrial Fibrillation With Regard to Clinical Profile

Introduction: Non-PV triggers play significant roles in initiating atrial fibrillation (Af). We hypothesized that location of non-PV Af triggers is predictable to some extent from clinical background and may have influence on clinical outcome after ablation. Methods: We analyzed consecutive 109 patients (76 men) with Af who underwent catheter ablation and investigation of Af triggers. Af was induced under recordings of intra-cardiac electrograms before ablation procedure in the following ways (1) watching spontaneous firing, (2) induction with intravenous infusion of isoproterenol and adenosine, (3) burst atrial pacing and/or intentional defibrillation to watch immediate recurrence of Af. Af triggers were analyzed and compared to clinical profiles and therapeutic outcome. Results: Eighty-four were paroxysmal Af and 25 were persistent. Any ectopic trigger of Af was identified in 73 patients (66%) with 99 foci. Seventy-eight foci were PVs (79%) whereas 21 (21%) were non-PV triggers. In general, non-PV foci were notably identified in female (P&lt;0.01) whereas age, LA diameter or Af type was not significantly relevant to the presence of non-PV foci. Among non-PV foci, superior vena cava (SVC, n=5), crista terminals (CT, n=5) and left atrium (LA, n=5) were prevalent sites. CT was prevalently noted in younger females (P&lt;0.01), whereas SVC was regardless of age. Non-PV foci in the LA were preferably noted in patients with persistent Af (P&lt;0.05). Importantly, multiple or non-PV foci were not significantly correlated to Af recurrence after ablation, whereas LA diameter was weakly correlated. Conclusion: Presence and sites of non-PV foci are rather predictable by simple clinical profiles such as gender, age and type of Af. Multiple or non-PV foci may not be associated with worse clinical outcome as long as they can be successfully targeted and ablated.

Minimally invasive percutaneous compression plating versus dynamic hip screw for intertrochanteric fractures: a randomized control trial

ObjectiveIntertrochanteric femur fracture is a common injury in elderly patients. The dynamic hip screw (DHS) has served as the standard choice for fixation; however it has several drawbacks. Studies of the percutaneous compression plate (PCCP) are still inconclusive in regards to its efficacy and safety. By comparing the two methods, we assessed their clinical therapeutic outcome. MethodsA total of 121 elderly patients with intertrochanteric femur fractures (type AO/OTA 31.A1-A2, Evans type 1) were divided randomly into two groups undergoing either a minimally invasive PCCP procedure or a conventional DHS fixation. ResultsThe mean operation duration was significantly shorter in the PCCP group (55.2min versus 88.5min, P<0.01). The blood loss was 156.5ml±18.3ml in the PCCP group and 513.2ml±66.2ml in the DHS group (P<0.01). Among the patients treated with PCCP, 3.1% needed blood transfusions, compared with 44.6% of those that had DHS surgery (P<0.01). The PCCP group displayed less postoperative complications (P<0.05). The mean American Society of Anesthesiologists score and Harris hip score in the PCCP group were better than those in the DHS group. There were no significant differences in the mean hospital stay, mortality rates, or fracture healing. ConclusionDue to several advantages, PCCP has the potential to become the ideal choice for treating intertrochanteric fractures (type AO/OTA 31.A1-A2, Evans type 1), particularly in the elderly.

Pharmacist-led implementation of a vancomycin guideline across medical and surgical units: impact on clinical behavior and therapeutic drug monitoring outcomes.

BackgroundVancomycin is the antibiotic of choice for the treatment of serious infections such as methicillin-resistant Staphylococcus aureus (MRSA). Inappropriate prescribing of vancomycin can lead to therapeutic failure, antibiotic resistance, and drug toxicity.ObjectiveTo examine the effectiveness of pharmacist-led implementation of a clinical practice guideline for vancomycin dosing and monitoring in a teaching hospital.MethodsAn observational pre–post study design was undertaken to evaluate the implementation of the vancomycin guideline. The implementation strategy principally involved education, clinical vignettes, and provision of pocket guidelines to accompany release of the guideline to the hospital Intranet. The target cohort for clinical behavioral change was junior medical officers, as they perform the majority of prescribing and monitoring of vancomycin in hospitals. Assessment measures were recorded for vancomycin prescribing, therapeutic drug monitoring, and patient outcomes.ResultsNinety-nine patients, 53 pre- and 46 post-implementation, were included in the study. Prescribing of a loading dose increased from 9% to 28% (P=0.02), and guideline adherence to starting maintenance dosing increased from 53% to 63% (P=0.32). Dose adjustment by doctors when blood concentrations were outside target increased from 53% to 71% (P=0.12), and correct timing of initial concentration measurement increased from 43% to 57% (P=0.23). Appropriately timed trough concentrations improved from 73% to 81% (P=0.08). Pre-dose (trough) concentrations in target range rose from 33% to 44% (P=0.10), while potentially toxic concentrations decreased from 32% to 21% (P=0.05) post-implementation. Infection cure rates for patients increased from 85% to 96% (P=0.11) after the guideline was implemented.ConclusionThe implementation strategy employed in this study demonstrated potential effectiveness, and should prompt additional larger studies to optimize strategies that will translate into improved clinical practice using vancomycin.

Rhino-orbital Mucormycosis: Clinical Findings and Treatment Outcomes of Four Cases.

In this case report, we present the clinical findings and therapeutic outcomes of four rhino-orbital mucormycosis patients. The four patients (1 female, 3 male; age range, 55-77 years) all had diabetes mellitus and two also had chronic renal failure. All patients exhibited proptosis, sinusitis, and dark-colored lesions on the nasopharynx and/or hard palate; three patients had ipsilateral peripheral facial paralysis. Visual acuity was no light perception in the two patients with severe orbital involvement and 0.8 in two patients with limited orbital involvement. Histopathological examination of the hard palate, nasopharynx or sinus biopsy revealed typical Mucor hyphae. Systemic liposomal amphotericin B was initiated in all patients. The patients with limited ocular involvement received amphotericin B both intravenously and by local irrigation; both patients had complete recovery. The other two patients underwent orbital exenteration; one patient died after declining systemic treatment postoperatively. Rapid diagnosis and treatment are important for the survival of rhino-orbital mucormycosis patients. With orbital involvement, surgical debridement and systemic and local treatment with antifungal agents may help avoid mutilating surgery like exenteration.

Forecasting Accuracy of the Hollow Fiber Model of Tuberculosis for Clinical Therapeutic Outcomes.

The hollow fiber system model of tuberculosis (HFS-TB), in tandem with Monte Carlo experiments, represents a drug development tool (DDT) with the potential for use to develop tuberculosis treatment regimens. However, the predictive accuracy of the HFS-TB, or any other nonclinical DDT such as an animal model, has yet to be robustly evaluated. To avoid hindsight bias, a literature search was performed to identify clinical studies published at least 6 months after HFS-TB experiments' quantitative predictions. Steps to minimize bias and for reporting systematic reviews were applied as outlined in the Preferred Reporting Items for Systematic Reviews and Meta-Analyses. Publications were scored for quality of evidence. Accuracy was calculated using the mean absolute percentage error, then summated with weighting assigned by sample size and quality-of-evidence score. Given the lack of a gold-standard tuberculosis DDT, the forecasting accuracy of a completely unreliable tool was also calculated from 1000 simulated experiments for a random or "total guesswork" model. The quantitative forecasting accuracy (95% confidence interval [CI]) for the "total guesswork" model was 15.6% (95% CI, 8.7%-22.5%); bias was -0.1% (95% CI, -2.5% to 2.2%). Twenty clinical studies were published after HFS-TB experiments predicted optimal drug exposures and doses, susceptibility breakpoints, and optimal combination regimens. Based on these clinical studies, the predictive accuracy of the HFS-TB was 94.4% (95% CI, 84.3%-99.9%), and bias was 1.8% (95% CI, -13.7% to 6.2%). The HFS-TB model is highly accurate at forecasting optimal drug exposures, doses, and dosing schedules for use in the clinic.

Surgical Outcomes of Patients With Stage III Thymoma in the Japanese Nationwide Database

To investigate the clinical characteristics and therapeutic outcomes of patients who underwent surgery for stage III thymoma in Japan. Using the Japanese nationwide database, which contains the records of 2,835 patients with thymic epithelial tumors who underwent treatment between 1991 and 2010, we extracted and analyzed the records of those who underwent surgery for stage III thymoma. A total of 310 patients (170 males, 140 females; median age, 58 years) were analyzed. Involved sites were the lung in 194 (62.6%), the pericardium in 151 (48.7%), the great vessels in 126 (40.6%), the phrenic nerve in 84 (27.1%), and the chest wall in 7 (2.3%). Complete resection (R0) was achieved in 247 (79.7%) cases. Induction therapies were administered to 42 (13.5%) patients, and postoperative therapies were administered to 147 (47.4%). In R0 cases, 68 (27.5%) experienced recurrence. The pleural space was the most frequent site of recurrence (46; 18.6%). The 10-year overall and disease-free (in R0) survival rates were 80.2% and 51.6%, respectively. Multivariate analyses revealed that age (p= 0.002), male sex (p= 0.017), induction therapy (p < 0.001), and type B histology (p= 0.037) were independent adverse predictors for overall survival. Chest wall invasion was the only independent adverse predictor for disease-free survival, although the factor analysis was marginal for overall survival. The outcomes of surgery for patients with stage III thymoma were favorable unless chest wall invasion was present; however, the role of complete resection and appropriate multimodal treatment plan require further investigation.

Isolation of Haemophilus influenzae and Haemophilus parainfluenzae in urethral exudates from men with acute urethritis: a descriptive study of 52 cases

ObjectivesTo describe the clinical characteristics and therapeutic outcomes from male patients diagnosed of Haemophilus spp urethritis.MethodsA chart review of patients who presented to our hospital from January 2013 to December...

Long-Term Follow-Up of the Therapeutic Outcomes for Papillary Thyroid Carcinoma With Distant Metastasis.

Papillary thyroid carcinoma (PTC) patients with distant metastasis (DM) have variable clinical courses and therapeutic outcomes. Survival time after diagnosis of DM may be several months to years. Long-term follow-up is necessary to determine prognostic factors for survival in PTC with DM. The purpose of this study was to investigate the clinical features and therapeutic outcomes of PTC with DM after 10 years of follow-up. The study population consisted of 70 patients who underwent initial thyroidectomy before 2004 and had DM beyond the locoregional neck area. Of these 70 patients, 40 patients were diagnosed with DM before or within 9 months after initial thyroidectomy in first radioactive iodide (131I) whole-body scintigraphy (group A), and 30 patients were diagnosed with DM during the follow-up period (group B). Patients with DM underwent 3.7 to 7.4 GBq 131I therapy every 6 to 12 months. After a mean follow-up period of 10.1 ± 0.9 years, the disease-specific mortality and remission rates were 70.0% (49/70) and 10% (7/70), respectively. The survival rates for patients in groups A and B were 72.5% and 96.7% at 1 year, 47.5% and 90.0% at 5 years, 40.0% and 70.0% at 10 years, 36.4% and 41.1% at 15 years, and 35.0% and 8.0% at 20 years, respectively. The percentage of male and older patients and patients with larger tumor size was higher in the mortality group than in the survival group, whereas the percentage of patients with 131I avid metastatic lesions (first DM) was lower in the mortality group. The percentage of patients with secondary primary cancers was higher in group B than in group A. In the multiple regression analysis, age and male gender were independently associated with disease-specific mortality. In conclusion, after a mean follow-up of 10.1 years, the disease-specific mortality rate for PTC with DM was 70.0%. Older patients and male PTC patients with DM need more aggressive treatment. The timing of DM diagnosis did not influence disease-specific mortality.

A Decade of Experience in Developing Preclinical Models of Advanced- or Early-Stage Spontaneous Metastasis to Study Antiangiogenic Drugs, Metronomic Chemotherapy, and the Tumor Microenvironment.

The clinical circumstance of treating spontaneous metastatic disease, after resection of primary tumors, whether advanced/overt or microscopic in nature, is seldom modeled in mice and may be a major factor in explaining the frequent discordance between preclinical and clinical therapeutic outcomes where the trend is "overprediction" of positive results in preclinical mouse model studies. To evaluate this hypothesis, a research program was initiated a decade ago to develop multiple models of metastasis in mice, using variants of human tumor cell lines selected in vivo for enhanced spontaneous metastatic aggressiveness after surgical resection of established orthotopic primary tumors. These models have included breast, renal, and colorectal carcinomas; ovarian cancer (but without prior surgery); and malignant melanoma. They have been used primarily for experimental therapeutic investigations involving various antiangiogenic drugs alone or with chemotherapy, especially "metronomic" low-dose chemotherapy. The various translational studies undertaken have revealed a number of clinically relevant findings. These include the following: (i) the potential of metronomic chemotherapy, especially when combined with a vascular endothelial growth factor pathway targeting drug to successfully treat advanced metastatic disease; (ii) the development of relapsed spontaneous brain metastases in mice with melanoma or breast cancer whose systemic metastatic disease is successfully controlled for a period with a given therapy; (iii) foreshadowing the failure of adjuvant antiangiogenic drug-based phase III trials; (iv) recapitulating the failure of oral antiangiogenic tyrosine kinase inhibitors plus standard chemotherapy in contrast to the modest successes of antiangiogenic antibodies plus chemotherapy in metastatic breast cancer; and (v) revealing "vessel co-option" and absence of angiogenesis as a determinant of intrinsic resistance or minimal responsiveness to antiangiogenic therapy in lung metastases. Developing similar models of metastatic disease but involving mouse tumors grown in syngeneic immunocompetent mice may also prove useful for future translational studies of immune therapy-based treatments.

Diffuse Large B-Cell Lymphoma in adults aged 65 years and older: A retrospective multicenter analysis of clinical characteristics and therapeutic outcomes

S218 506 Diffuse Large B-Cell Lymphoma in adults aged 65 years and older: A retrospective multicenter analysis of clinical characteristics and therapeutic outcomes Mehmet Hilmi Dogu, Muzaffer Keklik, Hatice Terzi, Serdal Korkmaz, Mehmet Ali Erkurt, Gulsum Akgun Cagliyan, Leylagul Kaynar, Serdar Sivgin, Ismail Sari, Ali Keskin, Mehmet Sencan, Fevzi Altuntas, Osman Ilhan Department of Hematology, Pamukkale University Faculty of Medicine; Department of Hematology, Erciyes University Faculty of Medicine; Department of Hematology, Cumhuriyet University Faculty of Medicine; Department of Hematology, Inonu University Faculty of Medicine; Department of Hematology, Bozyaka Training and Research Hospital; Department of Hematology, Yildirim Beyazit University, Ankara Oncology Hospital; Department of Hematology, Ankara University, Ibni Sina Hospital

Cyclin-dependent kinase inhibitor dinaciclib potently synergizes with cisplatin in preclinical models of ovarian cancer.

Ovarian cancer is one of the most lethal of woman cancers, and its clinical therapeutic outcome currently is unsatisfied. Dinaciclib, a novel small molecule inhibitor of CDK1, CDK2, CDK5 and CDK9, is assessed in clinical trials for the treatment of several types of cancers. In this study, we investigated the anticancer effects and mechanisms of dinaciclib alone or combined with cisplatin in ovarian cancer. Dinaciclib alone actively induced cell growth inhibition, cell cycle arrest and apoptosis with the increased intracellular ROS levels, which were accompanied by obvious alterations of related proteins such as CDKs, Cyclins, Mcl-1, XIAP and survivin. Pretreatment with N-acety-L-cysteine significantly blocked ROS generation but only partially rescued apoptosis triggered by dinaciclib. Moreover, the combination of dinaciclib with cisplatin synergistically promoted cell cycle arrest and apoptosis, and inhibited the subcutaneous xenograft growth of ovarian cancer in nude mice. Altogether, dinaciclib potently synergizes with cisplatin in preclinical models of ovarian cancer, indicating this beneficial combinational therapy may be a promising strategy for treatment of ovarian cancer.

Clinical presentation and therapeutic outcomes of anti-N-methyl-D-aspartate receptor encephalitis in children

Objective To explore the clinical characteristics, diagnosis, treatment and outcomes of anti-N-methyl-D-aspartate receptor(anti-NMDAR) encephalitis in children. Methods Six children diagnosed as anti-NMDAR encephalitis were recruited at the Department of Neurology, Capital Institute of Pediatrics, from December 2011 to April 2013.The data of clinical characteristics and laboratory examinations were retrospectively analyzed.All the children had long-term follow-ups and the prognosis was assessed. Results (1)Age and course of the disease at the time of the admission: the mean age of the 6 patients(2 female) was 3 years and 5 months, ranging from 2 years and 2 months to 6 years and 8 months.The course of the disease at the time of the admission ranged from 15 to 80 days, with a mean time of 39 days.(2)Clinical characteristics: 5 cases had afebrile convulsion and 1 case had speech impairment at the onset of disease.Convulsion occurred in all the 6 cases, 4 cases of whom had persistent convulsion, and 5 cases had impaired consciousness.All the 6 cases exhibited aphasia, and complicated with mental or emotional abnormalities, irritability or shouting.Five cases developed into sleep disorders such as sleep deprivation.Five cases had limb and facial involuntary movement, in which 2 cases had stereotyped action.Prominent autonomic nervous dysfunction including hidrosis was found in 1 case.(3)Laboratory examination: cerebrospinal fluid test was normal in 6 cases, and 1 case had slightly increased white blood cell level.Specific anti-NMDAR antibody was positive in serum and/or cerebrospinal fluid in the 6 cases.Electroencephalograph of the 6 cases showed slow wave background during lucid interval, and 5 cases had interictal epileptiform discharges.The skull MRI showed cerebral atrophy 4 cases, and 2 cases of them were complicated with encephalomalacia.No tumor was found in the patients.(4)Treatment and follow-ups: 6 cases received gamma globulin or methylprednisolone or other immunotherapy.Three cases received combined therapy with Rituximab, 1 case received plasmapheresis, and 1 case received Cyclophosphamide.Follow-ups lasted for 2 to 31 months.Three patients had clinical recovery, and varying degrees of neurological complications were found in 3 cases. Conclusions (1)Anti-NMDAR encephalitis is common in children.(2)The specificity of its clinical symptoms is not strong.The incidence of convulsion is high, and different degrees of consciousness disorders may occur in some of the severe patients.Degeneration of language function and emotional changes can be observed.Most pediatric patients have abnormal movement, and the symptoms of automatic nervous system are not prominent.(3)The disease can be confirmed by the specific anti-NMDAR antibody in the spinal fluid or plasma.(4)The time of clinical recovery is long, and an early immunotherapy is associated with a better prognosis. Key words: Anti-N-methyl-D-aspartate receptor; Child; Encephalitis

Animal allergens: common protein characteristics featuring their allergenicity.

Animal allergens: common protein characteristics featuring their allergenicity.

Congenital hyperinsulinism in Chinese patients: 5-yr treatment outcome of 95 clinical cases with genetic analysis of 55 cases.

The aim of this study is to investigate the clinical features, therapeutic outcomes, and genetic mutations of congenital hyperinsulinism (CHI) in Chinese patients. The clinical features and therapeutic outcomes of 95 CHI cases were recorded, and genetic analyses were conducted to identify mutations in ABCC8 and KCNJ11 in 55 cases. Direct sequencing was carried out in 25 of the cases with ABCC8 and KCNJ11 mutations. Additionally, 16 samples with no mutations and the remaining 30 samples were sequenced using Ion Torrent platform. Clinical misdiagnosis occurred in 36/95 (38%) of the cases. Most (82/95; 84%) of the patients were given diazoxide therapy combined with age-dependent frequent feeding, which was effective in 54/95 (66%) cases. The side effects of diazoxide included sodium and water retention, gastrointestinal reactions, polytrichia, and thrombocytopenia. Five patients were treated with octreotide for 1-4 months, of which 80% (4/5) showed a positive response. Non-surgical therapy was effective in 71/95 (75%) cases. Of the four children who received subtotal pancreatectomy, only one had a good outcome. The remission rate of hypoglycemia was 59% for children over 2-yr-old. The CHI-related gene mutation rate was 38% for potassium channel-related genes. Early onset of CHI and a lower diazoxide response rate were associated with potassium-ATP channel gene mutations. Age-dependent frequent feeding is an acceptable therapy for CHI. Non-surgical therapy may be highly effective, in part, due to the low rate of potassium channel gene mutations. Surgical outcomes are unreliable without 18F-fluoro-L-DOPA positron emission tomography. Therefore, we do not recommend operation without definitive identification of the pathologic type.

Clinical features and therapeutic outcome of 30 patients diagnosed with primary myelofibrosis at the national center of hematology

Background: Primary myelofibrosis is characterized by clonal expansion of hematopoietic stem cell with a non-reactive clonal proliferation of fibroblasts and bone marrow fibrosis, which occurs at an extramedullary hematopoiesis. The clinical features of Primary myelofibrosis include anemia, marked splenomegaly and constitutional symptoms. Ineffective erythropoiesis and extra-medullary hematopoiesis are the main causes of anemia and organomegaly, respectively Objectives : the aim of this study was to evaluate the clinical features, diagnostic tools and the treatment outcome of patients with primary myelofibrosis.Patients and methods: this is a prospective study conducted at the national center of hematology in Baghdad during the period between October 2009 and October 2013. It includes 30 adult patients diagnosed with primary myelofibrosis. Diagnosis was based primarily on bone marrow aspirate and biopsy. The patients were followed up and assessed monthly with complete blood picture and liver function test and renal function test with serum lactate dehydrogenase level.Results: thirty patients were included in this study. There were 16 male and 14 female, the median age was 58 year with range (26-75) years. The most frequent presentation was in the group older than 60 years. Twenty four (80 %) patients had symptoms at diagnosis. The most frequent presentation were the fatigue, left upper quadrant swelling, night sweats, weight loss and fever. The main clinical features were huge splenomegaly which is found in 29 (97%) of patients, while the main finding in blood count at presentation were anemia followed by leukocytosis with leukoerythroblastic blood picture in 22 (73 %) of cases. The level of LDH was high in about 23 (77 %). The bone marrow biopsy showed fibrosis stage 2 in 24 (80 %).Patients who died were in the highest risk groups. The median survival was approximately 28 months.Conclusion: Primary myelofibrosis is part of myeloproliferative neoplasia; it usually runs chronic course. Till now none of the therapeutic modalities approved for treatment of primary myelofibrosis proved to be superior to others, yet the 5 year survival of this disease considered good.