Bacillus Calmette-Guérin (BCG) is a live vaccine and has the potential to cause local disease and systemic dissemination in immunocompromised hosts, including infants who are infected with human immunodeficiency virus (HIV) through vertical transmission, and patients with primary immunodeficiencies (PID) such as severe combined immunodeficiency (SCID), chronic granulomatous disease (CGD), hyper-IgM syndrome, and defects of the IL12- IFNγ axis (Mendelian susceptibility to mycobacterial diseases, MSMD). Disseminated BCG is extremely difficult to treat. The chance of complete eradication is low unless functional immune response is restored by haematopoietic stem cell transplant. Prolonged use of anti-mycobacterial drugs often causes organ toxicities and drug resistance. Inflammatory complications which develop upon immunoreconstitution post-transplant may necessitate immunosuppressive treatment, which adversely affect immune recovery and increases risks of opportunistic infections. Multiple BCG reactivations can occur in patients with CGD and MSMD, and BCG can remain latent until reactivations take place in adulthood and manifest as disease. It is important for neonatologists, general practitioners, primary care clinicians and nurses working in maternal and child care centres to be aware of BCG-related complications, which may be the first sign of an underlying immunodeficiency. As neonatal BCG is included in standard vaccination schedule in many countries, it is a challenge to identify and avoid administration of BCG to infants who potentially have PIDs. Deferring BCG vaccination is recently advocated to protect highly vulnerable populations, but the appropriate strategy is yet to be determined. Newborn screening for SCID offers a potential to avoid this complication, if an integrated system of screening and vaccination can be organised.
Adjuvants, Immunologic ; adverse effects ; therapeutic use ; BCG Vaccine ; adverse effects ; immunology ; therapeutic use ; Humans ; Immunologic Deficiency Syndromes ; diagnosis ; immunology ; Infant, Newborn ; Mycobacterium Infections ; prevention & control ; Mycobacterium bovis ; drug effects ; Neonatal Screening ; methods ; Risk Assessment ; Vaccination ; adverse effects ; methods

Objective To investigate the relationship between NP involvement and clinical factors and the potential predictors for NPSLE in children. Methods Sixty-two Chinese children with SLE diagnosed between 1990 and 2006 were retrospectively reviewed. Clinical characteristics and potential predictors for NPSLE were analyzed in patients with NP vs those without NP, early-onset NP vs late-onset NP, SLE-onset vs NP-onset in late-onset NP group. Results Ratio of lupus nephritis(LN) at SLE onset was less common in patients with NP disorders than those without NP; the mean age for the early-onset group was significantly younger and the SLEDAI score was higher than those of late-onset group. There was no difference in all the clinical and serological factors for SLE-onset vs NP-onset in the late-onset NPSLE group. Conclusion NP development is negatively associated with renal involvement at SLE diagnosis. Early-onset NPSLE usually happens in young patients with high disease activity scores. There are no clinical factors that can predict the development of NPSLE.

Objective To define the clinical characteristics of NPSLE in Chinese children by analyzing their demographic data,clinical manifestations,laboratory parameters and treatment.Methods Sixtytwo children with SLE who were diagnosed between 1990 and 2006 were retrospectively reviewed.The demographic data,clinical manifestations,laboratory parameters,imaging and treatment were analyzed.The syndromes were compared with those reported in other studies.Results Nineteen (31%) SLE patients with 21 NP events were identified.The most common NP manifestations were cognitive dysfunction (48%),seizure disorder (43%) and headache (29%).The abnormality percentage of SPECT was 80%.86% patients received MP pulses therapy.Most (74%) patients recovered without long-term neurological complications.Conclusi NP symptoms are common in Chinese children with SLE,however,the prese-ntations are different from that reported in other studies.SPECT and MR are sensitive tests for NPSLE.The prognosis of NPSLE in our series is favorable.

Aged ; Aortic Aneurysm ; diagnostic imaging ; etiology ; Aortic Valve Insufficiency ; diagnostic imaging ; Echocardiography ; Giant Cell Arteritis ; complications ; diagnostic imaging ; Humans ; Male

Background: Increased maternal anxiety level has been reported to have detrimental effects on the physical outcome of pregnancies such as not achieving vaginal births. This study thus aims to determine the level and factors affecting mental preparedness among mothers with normal pregnancies and its correlation with birth outcomes. Methods: Three hundred healthy mothers above 37 weeks of gestation in the early stage of labour were assessed for their level of mental preparation before birth process and outcomes after births which include general feeling (euphoria), ability to withstand labour pain and bonding with the new born. The successfulness of vaginal birth and other data on factors affecting mental preparation were also collected. Results: The level of mental preparedness was found good in 78% of the mothers, mainly determined by their socioeconomic status, family support and personal ability to adjust to changes. Age (p= 0.048), parity (0.00) and income (0.01) were found to influence mental preparedness significantly. Race, occupation, education level and marital status are however not significantly related. Poor mental preparedness is associated with greater pain during labour. A correlation analysis also found a positive relationship between the level of mental preparation and mental outcomes following birth in these mothers but it did not significantly influence the mode of delivery. Conclusion: Mental preparation before birth seems to have an effect on mental outcomes of mothers following birth process. It is vital that mothers of the younger age group with no previous obstetric experience be given more attention in preparing them mentally before they face the painful birth process.

OBJECTIVE: To evaluate whether computed tomography (CT) reconstruction algorithms affect the CT texture features of the liver parenchyma. MATERIALS AND METHODS: This retrospective study comprised 58 patients (normal liver, n = 34; chronic liver disease [CLD], n = 24) who underwent liver CT scans using a single CT scanner. All CT images were reconstructed using filtered back projection (FBP), hybrid iterative reconstruction (IR) (iDOSE4), and model-based IR (IMR). On arterial phase (AP) and portal venous phase (PVP) CT imaging, quantitative texture analysis of the liver parenchyma using a single-slice region of interest was performed at the level of the hepatic hilum using a filtration-histogram statistic-based method with different filter values. Texture features were compared among the three reconstruction methods and between normal livers and those from CLD patients. Additionally, we evaluated the inter- and intra-observer reliability of the CT texture analysis by calculating intraclass correlation coefficients (ICCs). RESULTS: IR techniques affect various CT texture features of the liver parenchyma. In particular, model-based IR frequently showed significant differences compared to FBP or hybrid IR on both AP and PVP CT imaging. Significant variation in entropy was observed between the three reconstruction algorithms on PVP imaging (p < 0.05). Comparison between normal livers and those from CLD patients revealed that AP images depend more strongly on the reconstruction method used than PVP images. For both inter- and intra-observer reliability, ICCs were acceptable (> 0.75) for CT imaging without filtration. CONCLUSION: CT texture features of the liver parenchyma evaluated using the filtration-histogram method were significantly affected by the CT reconstruction algorithm used.
Entropy ; Filtration ; Humans ; Liver Diseases ; Liver ; Methods ; Retrospective Studies ; Tomography, X-Ray Computed

Alcohol withdrawal syndrome (AWS) is a common condition occurring after intentional or unintentional abrupt cessation of alcohol in an alcohol-dependent individual. AWS represents a major problem in our society and alcohol withdrawal seizure is the major cause of seizures encountered by neurology residents in the emergency department. Patients with AWS present with mild symptoms of tremulousness and agitation or more severe symptoms including withdrawal seizures and delirium tremens. Particularly, severe AWS can produce significant rates of the morbidity (complications) and mortality. When diagnosed and managed insufficiently, the morbidity and mortality rates increase. Nevertheless, patients with AWS may be neglected and are often marginalized and the teaching about AWS to neurology residents is usually minimal. Also, attending neurologists are often poorly informed on the topic. Although there is insufficient consensus about the optimal investigation and management, the purpose of this review is to serve as a summary of the appropriate identification and management of this important condition in a neurological setting.
Alcohol Withdrawal Delirium ; Alcohol Withdrawal Seizures* ; Consensus ; Dihydroergotamine ; Disease Management ; Emergency Service, Hospital ; Humans ; Mortality ; Neurology ; Seizures

Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Chemotherapy, Adjuvant ; Clinical Trials as Topic ; Disease-Free Survival ; Dose Fractionation ; Humans ; Nasopharyngeal Neoplasms ; drug therapy ; pathology ; radiotherapy ; Neoplasm Staging ; Radiotherapy, Intensity-Modulated ; Randomized Controlled Trials as Topic ; Survival Rate

Antineoplastic Agents ; administration & dosage ; Carcinoma, Non-Small-Cell Lung ; drug therapy ; Cost-Benefit Analysis ; Humans ; Lung Neoplasms ; drug therapy ; Meta-Analysis as Topic

Objective:To summarize the clinical characteristics, common images, pathogens, and gene mutation types of chronic granulomatosis disease (CGD) in 19 children.Methods:The clinical manifestations, laboratory findings, treatment, and prognosis of 19 patients diagnosed with CGD in Hong Kong University-Shenzhen Hospital from December 2012 to December 2018 were analyzed.Results:The 19 patients were all males and confirmed as CGD by the dihydrorhodamine test and gene sequencing.The age of the first infection was mostly 1 month after birth(13 cases), and the age of clinical diagnosis ranged from 2 months to 10 years.Sixteen mothers were carriers.The patients presented with pulmonary fungal infection (19/19 cases), Bacillus Calmette Guerin (BCG)-osis (14/19 cases), lymphadenitis (14/19 cases), perianal abscess (9/19 cases), skin abscess (5/19 cases) and ulcerative colitis (2/19 cases). There were 59 positive cultures.Pathogens included fungi (9 cases), Klebsiella pneumonia (8 cases), mycobacteria (7 cases), Streptococcus Viridans (5 cases), Escherichia coli (3 cases), gram-positive bacteria (3 cases), Staphylococcus aureus (3 cases), and Burkholderia cenocepacia (2 cases). Gene mutations were found in all 19 patients, including 17 cases of CYBB, 1 case of CYBA and 1 case of NCF2.The type of mutations included nonsense mutations (6 cases), deletion mutations (5 cases, including 2 large fragment deletions), splice mutations (3 cases) and missense mutations (5 cases). Five mutations were novel.Splice mutations in 3 cases often led to skin abscess, perianal abscess and lymphadenitis.Two patients with large deletion mutations had more serious infection than other patients. Conclusions:In China, CGD is characterized with pulmonary infection and disseminated BCG-osis.Mycobacteria are common pathogens of CGD, and fungi are dominant pathogens of CGD.The most common infection is respiratory infection. Klebsiella pneumonia and Escherichia coli often lead to perianal abscess.The relationship between gene mutation types and clinical phenotypes requires further verification by big data.