The Evaluation of Neutropenia in X-linked Agammaglobulinemia Patients

2021 ◽  
Author(s):  
Molood Safarirad ◽  
Ali Abbaszadeh Ganji ◽  
Ahmad Vosughi Motlagh
Keyword(s):  
Mortality Rate ◽  
Infectious Diseases ◽  
Rare Complication ◽  
Clinical Manifestations ◽  
Primary Immunodeficiency Disease ◽  
Infectious Complications ◽  
Significant Difference ◽  
Neutropenic Patients ◽  
The Mean ◽  
Immunodeficiency Disease

Objectives: X-linked Agammaglobulinemia (XLA) is a primary immunodeficiency disease, characterized by severe hypogammaglobulinemia and the low numbers of peripheral B cells. Neutropenia is a rare complication among the XLA patients, which may lead to a higher rate of infections and morbidity. The aim of the authors is to assess the correctness of this issue. Methods: In this study, we compared demographic, clinical and laboratorial data between two groups of XLA patients, with and without neutropenia. Results: Frequency of neutropenia was 15% in our population. Infectious complications were the most prevalent clinical manifestations, regardless of the presence of neutropenia. However, Lymphoproliferative complication was significantly higher in the neutropenic patients (p = 0.001). No significant difference in mortality rate was observed between the groups. Conclusion: Neutropenia is a rare complication among the XLA patients, and significantly decreases the mean age of XLA diagnosis in the patients. But it is not related to the higher frequency of infectious diseases in the neutropenic patients compared to non-neutropenic ones.

Clinical and molecular epidemic characterization of rotavirus infection among children under five years old in Shandong province, China.

2021 ◽  
Author(s):  
Shixiao Dong ◽  
Deyu Huang ◽  
Zheng Wang ◽  
Guanyou Zhang ◽  
Fengjuan Zhang ◽  
...  
Keyword(s):  
Lower Incidence ◽  
Age Groups ◽  
Clinical Manifestations ◽  
Shandong Province ◽  
Rotavirus Infection ◽  
National Immunization ◽  
Significant Difference ◽  
The Mean ◽  
And Control

Abstract Rotavirus was the an important causative agent of acute gastroenteritis in children. In China, rotavirus was positive in approximately 30% of the diarrhea children and become a serious public problem. This study was carried out to investigate the clinical and molecular epidemic characterization of rotavirus infection among children under 5 years old with acute diarrhea in Shandong province, China. From July 2017 to June 2018, a total of 1211 fecal specimens were detected and the prevalence of rotavirus infection was 32.12%. The mean age of positive children was 12.2 ± 10.9 months and the highest infection rate was observed in children aged 7–12 months with a rate of 41.64%. G9P[8] (76.61%) was the most prevalent combinations followed by G2P[4] (7.20%), G3P[8] (3.60%) and G9P[4] (2.06%). In addition to diarrhea, vomiting, fever and dehydration were the most common accompanied symptoms. In general, there was no significant difference in clinical manifestations among different age groups. However, the clinical manifestations between vaccinated and unvaccinated children were significantly different. Vaccinated children showed lower incidence and frequency of vomiting, lower incidence and degree of dehydration, lower incidence of severe cases than unvaccinated children. The findings suggested necessary to continue rotavirus strains surveillance in order to monitor the change of prevalent genotype. Moreover, introducing vaccine into national immunization program to prevent and control rotavirus infections is needed in China.

scholarly journals Indications of admitting patients in internal ICU and the rate of mortality in Emam Khomeini hospital in 2013

2019 ◽  
Vol 6 (6) ◽  
pp. 2335
Author(s):  
Hasan Ghobadi ◽  
Shahram Habibzadeh ◽  
Bita Shahbazzadegan ◽  
Mohsen Mirzanezhadasl ◽  
Mahsa Kamranimoghaddam
Keyword(s):  
Mortality Rate ◽  
Continuous Monitoring ◽  
Good Condition ◽  
Vital Signs ◽  
The Public ◽  
Special Services ◽  
Disease Prognosis ◽  
Significant Difference ◽  
The Mean ◽  
End Stage

Background: ICU is the costly part of the hospital that has functional approach for patients who have reversible conditions so it needs mechanical ventilation and other special services. Some patients are not really in need of special care only the continuous monitoring of vital signs needs of the public sector. Patients with good condition or End-Stage were not candidate to admitting in ICU. The aim of this study was to evaluate indications of admitting patients in internal ICU and the rate of mortality in Emam Khomeini hospital in 2013.Methods: The study was conducted retrospectively evaluated the records of patients hospitalized in ICU and disease prognosis and treatment of disease and APACHE2 criteria was analyses.Results: The mean age of patients in the study was 61.05±19.81. Of 118 patients, 70 (59.3%) survived and 48 (40.7%) patients died. APACHE2 mean in the study was 21.46±7.5. GCS average was 9.83±4.27. There was correlation between mortality of patients and type of disease. In this study in APACHE2 score between 25-29 and >35 in mortality rate we are higher than standard average and in 10-14 and 20-24 we are lower than standard average.Conclusions: This study shows that GCS is not a good measure for the evaluation of patients hospitalized in internal ICU. In the present study, patients with higher APACHE2 score of 35 died. That show hospitalization that patient in ICU has no difference in the prognosis of them. As regards mortality rate in ICU patients in this study has no significant difference with predicted APACHE values, indications of ICU admition in Emam Khomeini hospital observed exactly.

scholarly journals Correlation between Clinical Symptoms of Coeliac Disease, Serum IgA Anti TTG and Biopsy in Pediatric Population of Northern India

2020 ◽  
Vol 8 (1) ◽  
pp. 65-68
Author(s):  
Sumit Jeena ◽  
Jaswinder Kaur ◽  
Nishant Wadhwa
Keyword(s):  
Coeliac Disease ◽  
Clinical Symptoms ◽  
Tissue Transglutaminase ◽  
Pediatric Population ◽  
Clinical Manifestations ◽  
North India ◽  
P Value ◽  
Serum Iga ◽  
Significant Difference ◽  
The Mean

Background: Celiac disease is basically an immune-mediated enteropathic condition produced by permanent sensitivity to gluten in genetically susceptible subjects. There is paucity of data in north India regarding clinical symptoms of coeliac disease, Serum IgA Anti TTG and Biopsy in pediatric population. The present study was conducted with the aim to determine the correlation between clinical symptoms of coeliac disease, Serum IgA Anti TTG and Biopsy in pediatric population of northern India.Materials and Methods: The present study was conducted in prospective including 73 pediatric patients at Department of Pediatric Gastroenterology, Institute of Child Health, Sir Gangaram Hospital, New Delhi, India. Esophagogastroduodenoendoscopy and serum anti Ig A tissue transglutaminase were performed. The characteristic scalloping of the folds were looked for in endoscopy followed by four duodenal biopsies performed from second part of duodenum and histological grading was performed as per modified marsh system. Patients with Serum IgA anti tTG>20 U/ml were confirmed to be at risk. Complete histological work up was done including hemoglobin, RBC indices and peripheral blood smear examination. The association of clinical manifestations with disease grade was also established with correlation coefficient. All the data thus obtained was arranged in a tabulated form and analyzed using SPSS software. Probability value of less than 0.05 was regarded as significant.Results: There were 4 males and 16 females with marsh grade 1 and 2 and mean age of 7.3±1.9 years. There were 5 males and 8 females with marsh grade 3a and mean age of 6.8±2.3 years. The mean weight of 18.11±3.89, height of 103.17±8.73 and BMI of 16.26±3.78 was observed amongst subjects with Marsh grade 1 and 2. The mean weight of 15.12±3.17, height of 99.28±9.19 and BMI of 15.02±3.20was observed amongst subjects with Marsh grade 3a. Diarrhoea was maximum amongst subjects with grade 3c and 4(70%) and minimum amongst Grade 1 and 2 (40%). There was a significant difference between the frequency of anemia amongst different grades as the p value was less than 0.05.Conclusion: The most common presenting signs and symptoms were diarrhea and abdominal pain. The study also concluded that the incidence of anemia increases with higher marsh grades.

Clinical Significance of Cryofibrinogenemia: Possible Pathophysiological Link with Raynaud’s Phenomenon

2011 ◽  
Vol 39 (1) ◽  
pp. 119-124 ◽  
Author(s):  
MUHAMMAD S. SOYFOO ◽  
AHMED GOUBELLA ◽  
ELIE COGAN ◽  
JEAN-CLAUDE WAUTRECHT ◽  
ANNICK OCMANT ◽  
...  
Keyword(s):  
Raynaud’S Phenomenon ◽  
Clinical Signs ◽  
Clinical Manifestations ◽  
Raynaud's Phenomenon ◽  
Clinical Findings ◽  
University Hospital ◽  
Significant Difference ◽  
Primary Raynaud’S Phenomenon ◽  
The Mean ◽  
Mean Concentration

Objective.To describe the clinical findings and prevalence of patients with cryofibrinogenemia (CF) and to determine whether CF is associated with primary Raynaud’s phenomenon.Methods.Between June 2006 and December 2009, 227 patients were tested for CF in a single university hospital. Forty-five patients with primary Raynaud’s phenomenon were tested for CF.Results.A total of 117 patients with CF without cryoglobulinemia were included. The main clinical manifestations included skin manifestations (50%) and arthralgia (35%). There were 67 patients with primary CF and 50 patients with secondary CF. There was no significant difference in the mean concentration of the cryoprecipitate in primary CF as compared to the secondary form (172 ± 18.6 vs 192 ± 20.9 mg/dl, respectively; p = 0.41). Highest concentrations of cryoprecipitate were observed in those containing fibrinogen only as compared to cryoprecipitates containing fibrinogen and fibronectin (301 ± 43.5 vs 125 ± 10.6 mg/dl; p < 0.001). Patients having skin necrosis (n = 3) had significantly higher values of cryofibrinogen compared to those without necrosis (638 ± 105 vs 160 ± 10.2 mg/dl; p = 0.0046). Among the 45 patients with primary Raynaud’s phenomenon, 36 had associated CF. There was no significant difference in the mean concentration of the cryoprecipitate in these patients compared to those with primary CF.Conclusion.There seems to be a significant correlation between cryofibrinogen concentration and the severity of the clinical signs, particularly when cryoprecipitate is composed of fibrinogen alone. CF might have a possible pathophysiological role in primary Raynaud’s phenomenon.

Clinical manifestations of canine babesiosis in Hungary (63 cases)

2006 ◽  
Vol 54 (3) ◽  
pp. 367-385 ◽  
Author(s):  
Á. Máthé ◽  
K. Vörös ◽  
L. Papp ◽  
J. Reiczigel
Keyword(s):  
Distress Syndrome ◽  
Clinical Manifestations ◽  
Multiple Organ ◽  
Multiple Organ Dysfunction ◽  
Canine Babesiosis ◽  
Clinical Observations ◽  
Immune Mediated ◽  
Significant Difference ◽  
Organ Manifestations ◽  
The Mean

Clinical observations of Babesia canis infection in 63 dogs during a 1-year period are summarised, demonstrating the pathogenicity of the Babesia strain endemic in Hungary. Most patients had babesiosis in the spring and autumn, correlating with the seasonal activity of ticks. Male animals appeared in higher numbers, probably due to an overrepresentation of outdoor dogs. Uncomplicated babesiosis was diagnosed in 32 cases. The disease affected dogs of any age in this study. Symptoms were similar to those published from other parts of the world: lethargy, fever, splenomegaly, pallor, icterus, haemoglobinuria and presence of ticks were the most common observations. Thrombocytopenia, lymphopenia and neutropenia were frequent haemogram changes. Imidocarb appeared to be highly effective in eliminating the Babesia infection. Thirty-one animals demonstrated babesiosis with complications. Most Rottweilers (7/9) developed complicated disease. Old age was a risk factor for multiple complications. Multiple organ manifestations had poor prognosis. Hepatopathy (44%), pancreatitis (33%), acute renal failure (ARF; 31%) and disseminated intravascular coagulation (DIC; 24%) were frequent complications, while immune-mediated haemolytic anaemia (IMHA; 10%), acute respiratory distress syndrome (ARDS; 6%) and cerebral babesiosis (3%) were rarely observed. There was a significant difference between the mean age of dogs having uncomplicated disease, babesiosis with a single complication and babesiosis with multiple complications (3.4, 4.8 and 8.6 years, respectively, p < 0.001). The recovery rate (78, 68 and 25%, respectively, p = 0.005) and mortality rate (3, 21 and 67%, respectively, p < 0.001) also tended to differ significantly in these groups. Systemic inflammatory response syndrome (SIRS) and DIC are two possible pathways leading to multiple organ dysfunction syndrome (MODS) in babesiosis. DIC was found to predict MODS more sensitively in this study than SIRS: there were 6 animals developing MODS out of 11 identified with DIC, while only 5 dogs developed MODS out of 22 having SIRS.

scholarly journals Novel mutations of TYK2 leading to divergent clinical phenotypes

2021 ◽  
Author(s):  
Ge Lv ◽  
Gan Sun ◽  
Peilin Wu ◽  
Xiao Du ◽  
Ting Zeng ◽  
...  
Keyword(s):  
Flow Cytometry ◽  
Western Blot ◽  
Mononuclear Cells ◽  
Clinical Manifestations ◽  
Primary Immunodeficiency Disease ◽  
Loss Of Function ◽  
Human Immune System ◽  
Peripheral Blood Mononuclear ◽  
Qrt Pcr ◽  
Immunodeficiency Disease

Background: TYK2 deficiency is a rare Primary immunodeficiency disease caused by loss of function mutations of TYK2 gene, which is initially proposed as a subset of Hyper IgE syndrome (HIES). However, accumulating evidence suggest TYK2 deficient patients do not necessarily present with HIES characteristics, indicating a vacuum of knowledge on the exact roles of TYK2 in human immune system. Method: Pathogenic effects of patients were confirmed by qRT-PCR, western blot and protein stability assays. The responses to cytokines including IFN-α/β/γ, IL-6, IL-10, IL12 and IL-23 of peripheral blood mononuclear cells (PBMCs) from these patients were detected by western blot, qRT-PCR and flow cytometry. The differentiation of T and B cells were detected by flow cytometry. Results: We describe five more TYK2 deficient cases presenting with or without hyper IgE levels, atopy and distinct pathogen infection profile, which are caused by novel TYK2 mutations. These mutations were all found by high throughout sequencing and confirmed by Sanger sequencing. The patients showed heterogenous responses to various cytokine treatments, including IFN-α/β/γ, IL-6, IL-10, IL12 and IL-23. The homeostasis of lymphocytes is also disrupted. Conclusion: Based on our findings, we propose that TYK2 works as a multi-tasker in orchestrating various cytokines signaling pathways, differentially combined defects of which account for the expressed clinical manifestations.

scholarly journals Evaluation of Iodine in Distributed Salts in Abarkouh City in 2017-2018

2018 ◽  
pp. 659-665
Author(s):  
Mahrokh Mahrokh Jalili ◽  
Ali Asghar Ebrahimi ◽  
Mohammad Hassan Ehrampoush ◽  
Fariba Abbasi ◽  
Eslami Hadi Eslami ◽  
...  
Keyword(s):  
Iodine Deficiency ◽  
Random Sampling ◽  
Clinical Manifestations ◽  
Iodine Concentration ◽  
T Test ◽  
British Pharmacopoeia ◽  
Wide Range ◽  
Significant Difference ◽  
The Mean ◽  
The City

Introduction: Iodine deficiency and associated disorders, which lead to a wide range of clinical manifestations, have been raised as one of the health and nutritional problems in Iran. Therefore, this study was aimed to investigate the amount of iodine in distributed salts in Abarkouh city in 2017-2018. Materials and Methods: Random sampling was done by census on all 30 distributed brands in Abarkouh city in the different stores across the city. Sixty samples (30 samples after one month from the production date and 30 samples after 6 months from the production date) were collected and transferred to the laboratory. The measurement of iodine was done according to the British Pharmacopoeia recommended method. T-test was used to analyze the data. Results: The Mean iodine concentration in the salts supplied in the Abarkouh was 29.83 ± 19.9 ppm, which was 50% less than the Iran's standard, with a statistically significant difference (P ≤ 0.001). 66.6% of the studied brands did not contain adequate iodine. Six months after production date, the average iodine amount in salts was reduced to 24.26 ± 16.94 ppm, but the reduction was not statistically significant (P = 0.224). Conclusion: Average iodine amount in most of the salts supplied across the Abarkouh city was not acceptable and some salts lacked iodine. Therefore, careful and continuous supervision and monitoring the process of iodized salts production in factories is recommended.

Urinary tract infections in obstetrics and gynecology

2015 ◽  
Vol 64 (6) ◽  
pp. 91-104 ◽  
Author(s):  
Elena Vasilyevna Shipitsyna ◽  
Tatyana Alekseyevna Khusnutdinova ◽  
Alevtina Mikhailovna Savicheva ◽  
Tatyana Aykovna Ayvazyan
Keyword(s):  
Urinary Tract ◽  
Antimicrobial Resistance ◽  
Infectious Diseases ◽  
Urinary Tract Infections ◽  
Clinical Manifestations ◽  
Clinical Importance ◽  
Infectious Complications ◽  
Obstetrics And Gynecology ◽  
Tract Infections ◽  
In Pregnancy

Urinary tract infections (UTIs) are among the most common infectious diseases in women, and are the most frequent cause of infectious complications of pregnancy. This paper reviews current scientific and methodical literature on UTIs in obstetrics and gynecology. Aspects of clinical importance of UTIs (epidemiology, clinical manifestations, complications), their etiology, antimicrobial resistance of UTIs agents were discussed, and current recommendations on diagnostics and treatment of UTIs were summarized. Special attention was paid to UTIs in pregnancy.

scholarly journals P1225PREVALENCE AND PROGNOSTIC SIGNIFICANCE OF DIABETES IN PERITONEAL DIALYSIS: TECHNIQUE AND PATIENT SURVIVAL

2020 ◽  
Vol 35 (Supplement_3) ◽  
Author(s):  
Amel Ayed ◽  
Meriem Ben salem ◽  
Faouzi Haouala ◽  
Ayed Sinda ◽  
Imen Chemli ◽  
...  
Keyword(s):  
Peritoneal Dialysis ◽  
Median Survival ◽  
Patient Survival ◽  
Infectious Complications ◽  
Diabetic Group ◽  
The Other ◽  
Diabetic Patients ◽  
Ultrafiltration Failure ◽  
Significant Difference ◽  
The Mean

Abstract Background and Aims Diabetes in recent years is climbing up as the number one cause of chronic kidney disease (CKD). Clinical statistics suggest that Diabetics on peritoneal dialysis (PD) tend to have a poorer prognosis than others. Thus, the aim of this study is to determine the clinical outcomes and to evaluate the survival rates as well as the predictors of mortality among this group. Method It‘s a retrospective study carried out in the nephrology Department of Fattouma Bourguiba Hospital (Monastir, Tunisia) from 1990 to 2017 including 304 PD patients. We compared two groups: diabetic and non diabetic patients in term of survival and factors in correlation. Results A total of 110 diabetic versus 194 non diabetic patients were reported. The mean age of diabetic group was 55.14 ± 15 years with a sex ratio 2.33 (men/women). In addition to diabetes, comorbid diseases included hypertension (47.3%) and cardiopathy (32.7%). The mean Charlson score of diabetic group was 5.22±1.5 versus 2.72±1.23 in the other group. A total of 62.7% (n=69) of the patients performed PD with the help of another person mostly a member of the family. The PD modality often used was CAPD (71%). Only six patients made a compulsory choice to begin PD due to vascular access while sixty diabetics chose PD to maintain autonomy (54.5%). During the follow-up period, transfers to hemodialysis (40.9%) and death (53.6%) were the most common cause of withdrawal from PD. In total, 45 patients were transferred to hemodialysis because of infectious complications (31%), Ultrafiltration failure (31%), catheter dysfunction (27%), and psychological intolerance (11%). The death was unrelated to PD in 83% of cases mostly due to considerable burden of cardiovascular events (23 patients). The median survival of the diabetic patients was 15.8 years versus 20.8 years in non diabetic ones with significant difference between the 2 groups (p=0.0001). Diabetes was associated with worse prognosis (OR:147, p=0.0001). The median survival adjusted to the diabetes group was 180 months. Kaplan–Meier analysis showed that diabetes was associated with a significant increase in mortality (p=0.006). Global median survival of the technique was estimated to 68 months (95%, IC [47 ,90]), and it was correlated to the presence or the absence of diabetes. In fact, the technique survival among diabetic patients was estimated to 80% after 12 years and 25% after 20 years whereas, in the other group it approaches 90% and 35% respectively. Conclusion This study confirms the pejorative impact of diabetes in the technique and patient survival in DP. So, it stresses the importance of organizing appropriate care upstream to prevent the development of cardiovascular morbidities and infectious complications in DP.

Prognostic factors in methanol poisoning

2007 ◽  
Vol 26 (7) ◽  
pp. 583-586 ◽  
Author(s):  
H. Hassanian-Moghaddam ◽  
A. Pajoumand ◽  
S.M. Dadgar ◽  
Sh. Shadnia
Keyword(s):  
Mortality Rate ◽  
Cross Sectional Study ◽  
Time Interval ◽  
Poison Center ◽  
Cross Sectional ◽  
Arterial Blood Gas ◽  
Arterial Blood ◽  
Significant Difference ◽  
The Mean ◽  
Mean Time

The aim of this study was to assess the clinical and laboratory factors in methanol poisoned patients to determine the prognosis of their toxicity. This survey was done as a prospective cross-sectional study in methanol-poisoned patients in Loghman-Hakim hospital poison center during 9 months from October 1999—June 2000. During this time 25 methanol-poisoned patients were admitted. The mortality rate was 12 (48%). Amongst survivors, three (23%) of the patients developed blindness due to their poisoning and the other 10 (77%) fully recovered without any complication. The mortality rate in comatose patients was nine (90%) while in non-comatose patients it was three (20%) ( P < 0.001). There was a significant difference in mean pH in the first arterial blood gas of patients who subsequently died (6.82 ± 0.03) and survivors (7.15 ± 0.06) ( P < 0.001, M-W). The mean time interval between poisoning and ED presentation in deceased patients were (46 ± 15.7) hours, in survived with sequelae were (16.7 ± 6.7) and in survived without sequelae were (10.3 ± 7.2) hours ( P < 0.002, K-W). We found no significant difference between the survivors versus the patients who died regarding methanol. Simultaneous presence of ethanol and opium affected the outcome of the treatment for methanol intoxication favourably and unfavourably, respectively. In our study, poor prognosis was associated with pH < 7, coma on admission and >24 hours delay from intake to admission. Human & Experimental Toxicology (2007) 26: 583—586.

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