METHODS: Data from the Malaysia Primary Immunodeficiency Network (MyPIN) with cases of CGD diagnosed from 1991 until 2016 were collated and analysed.
RESULTS: Twenty patients were diagnosed as CGD. Males (N = 13, 65%) outnumber females (N = 7, 35%). CGD is commonest amongst the Malays (65%) followed by the Chinese (15.0%), Indians (10.0%) and natives of Borneo (10.0%), reflecting the ethnic composition of the country. The mean age of diagnosis was 3.7 years. There was a positive family history in 40% of the cases. Abscess was the main presenting feature in 16 patients (80%) with one involving the brain. Pneumonia occurred in 10 (50%) and one with complicated bronchiectasis. Catalase-positive bacteria were the most commonly isolated pathogen with Chromobacterium violaceum predominating (N = 5, 25%) with consequent high mortality (N = 4, 80%). All CGD patients with C. violaceum infection displayed CD4 + (T helper cells) lymphopenia.
CONCLUSION: This study has shown CGD occurs in the major ethnic groups of Malaysia. To the best of our knowledge, this is the first and the largest series of chronic granulomatous disease in South East Asia which may be reflective of similar clinical pattern in the region. C. violaceum infection is associated with a higher mortality in CGD patients in Malaysia. All the CGD patients with C. violaceum infection in this patient series displayed CD4 + (T helper) lymphopenia. We recorded rare clinical manifestation of CGD viz. brain abscess and bronchiectasis.
METHODS: We followed up 240 participants (112 cognitively unimpaired [CU], 78 amnestic mild cognitive impairment [aMCI], and 50 Alzheimer's disease (AD) dementia [ADD]) for 2 years from 9 referral centers in South Korea. Participants were assessed with neuropsychological tests and 18F-flutemetamol (FMM) positron emission tomography (PET). Ten regions (frontal, precuneus/posterior cingulate (PPC), lateral temporal, parietal, and striatum of each hemisphere) were visually examined in the FMM scan, and participants were divided into three groups: No-FMM, Focal-FMM (FMM uptake in 1-9 regions), and Diffuse-FMM. We used mixed-effects model to investigate the speed of cognitive decline in the Focal-FMM group according to the cognitive level, extent, and location of Aß involvement, in comparison with the No- or Diffuse-FMM group.
RESULTS: Forty-five of 240 (18.8%) individuals were categorized as Focal-FMM. The rate of cognitive decline in the Focal-FMM group was faster than the No-FMM group (especially in the CU and aMCI stage) and slower than the Diffuse-FMM group (in particular in the CU stage). Within the Focal-FMM group, participants with FMM uptake to a larger extent (7-9 regions) showed faster cognitive decline compared to those with uptake to a smaller extent (1-3 or 4-6 regions). The Focal-FMM group was found to have faster cognitive decline in comparison with the No-FMM when there was uptake in the PPC, striatum, and frontal cortex.
CONCLUSIONS: When predicting cognitive decline of patients with focal Aß deposition, the patients' cognitive level, extent, and location of the focal involvement are important.
Method: Quasi-experimental and repeated-measures study designs were used in this study. Twenty-six adults with normal hearing (17 females, 9 males) participated. ABRs were acquired from the study participants at 3 intensity levels (80, 60, and 40 dB nHL), 3 frequencies (500, 1000, and 2000 Hz), 2 electrode montages (ipsilateral and vertical), and 2 stimuli (NB LS CE-Chirp and tone-burst) using 2 stopping criteria (fixed averages at 4,000 sweeps and F test at multiple points = 3.1).
Results: Wave V amplitudes were only 19%-26% larger for the vertical recordings than the ipsilateral recordings in both the ABRs obtained from the NB LS CE-Chirp and tone-burst stimuli. The mean differences in the F test at multiple points values and the residual noise levels between the ABRs obtained from the vertical and ipsilateral montages were statistically not significant. In addition, the ABR elicited from the NB LS CE-Chirp was significantly larger (up to 69%) than those from the tone-burst, except at the lower intensity level.
Conclusion: Both the ipsilateral and vertical montages can be used to record ABR to the NB LS CE-Chirp because of the small enhancement in the wave V amplitude provided by the vertical montage.
METHODS: We obtained random urine samples from 9,275 cases of acute first stroke and 9,726 matched controls from 27 countries and estimated the 24-hour sodium and potassium excretion, a surrogate for intake, using the Tanaka formula. Using multivariable conditional logistic regression, we determined the associations of estimated 24-hour urinary sodium and potassium excretion with stroke and its subtypes.
RESULTS: Compared with an estimated urinary sodium excretion of 2.8-3.5 g/day (reference), higher (>4.26 g/day) (odds ratio [OR] 1.81; 95% confidence interval [CI], 1.65-2.00) and lower (<2.8 g/day) sodium excretion (OR 1.39; 95% CI, 1.26-1.53) were significantly associated with increased risk of stroke. The stroke risk associated with the highest quartile of sodium intake (sodium excretion >4.26 g/day) was significantly greater (P < 0.001) for intracerebral hemorrhage (ICH) (OR 2.38; 95% CI, 1.93-2.92) than for ischemic stroke (OR 1.67; 95% CI, 1.50-1.87). Urinary potassium was inversely and linearly associated with risk of stroke, and stronger for ischemic stroke than ICH (P = 0.026). In an analysis of combined sodium and potassium excretion, the combination of high potassium intake (>1.58 g/day) and moderate sodium intake (2.8-3.5 g/day) was associated with the lowest risk of stroke.
CONCLUSIONS: The association of sodium intake and stroke is J-shaped, with high sodium intake a stronger risk factor for ICH than ischemic stroke. Our data suggest that moderate sodium intake-rather than low sodium intake-combined with high potassium intake may be associated with the lowest risk of stroke and expected to be a more feasible combined dietary target.
SUBJECTS AND METHODS: The pure-tone audiometry (PTA) and auditory brainstem responses (ABRs) from 22 patients (44 ears) with diagnosed noise-induced permanent hearing loss were studied. Three indices of PTA were average thresholds of 0.5 kHz/, /1 kHz, and 2 kHz (PTA1); 2 kHz and 4 kHz (PTA2); and 4 kHz (PTA3) were subdivided into 3 thresholds of hearing. Their relationships with ABR results were analysed. The patterns of PTA from various groups of ABR wave patterns were studied.
RESULTS: In this study, the abnormal ABR wave patterns were detected in 72.7% of the ears. The ears with prolonged ABR wave latency, absent early waves, prolong interpeak wave I-V latency was 20.5%, 18.2%, and 21.1%, respectively. Normal ABRs were recorded in 27.3% of the ears despite marked thresholds elevation of the PTA at high frequencies. Other relationships between PTA results and ABR wave results were discussed.
CONCLUSION: There were relationships between severity of noise-induced hearing loss indicated by PTA and the patterns of ABR wave abnormalities among workers with noise-induced permanent hearing loss.
Objective: To determine whether preoperative NT-proBNP has additional predictive value beyond a clinical risk score for the composite of vascular death and myocardial injury after noncardiac surgery (MINS) within 30 days after surgery.
Design: Prospective cohort study.
Setting: 16 hospitals in 9 countries.
Patients: 10 402 patients aged 45 years or older having inpatient noncardiac surgery.
Measurements: All patients had NT-proBNP levels measured before surgery and troponin T levels measured daily for up to 3 days after surgery.
Results: In multivariable analyses, compared with preoperative NT-proBNP values less than 100 pg/mL (the reference group), those of 100 to less than 200 pg/mL, 200 to less than 1500 pg/mL, and 1500 pg/mL or greater were associated with adjusted hazard ratios of 2.27 (95% CI, 1.90 to 2.70), 3.63 (CI, 3.13 to 4.21), and 5.82 (CI, 4.81 to 7.05) and corresponding incidences of the primary outcome of 12.3% (226 of 1843), 20.8% (542 of 2608), and 37.5% (223 of 595), respectively. Adding NT-proBNP thresholds to clinical stratification (that is, the Revised Cardiac Risk Index [RCRI]) resulted in a net absolute reclassification improvement of 258 per 1000 patients. Preoperative NT-proBNP values were also statistically significantly associated with 30-day all-cause mortality (less than 100 pg/mL [incidence, 0.3%], 100 to less than 200 pg/mL [incidence, 0.7%], 200 to less than 1500 pg/mL [incidence, 1.4%], and 1500 pg/mL or greater [incidence, 4.0%]).
Limitation: External validation of the identified NT-proBNP thresholds in other cohorts would reinforce our findings.
Conclusion: Preoperative NT-proBNP is strongly associated with vascular death and MINS within 30 days after noncardiac surgery and improves cardiac risk prediction in addition to the RCRI.
Primary Funding Source: Canadian Institutes of Health Research.
Case presentation: We report a case 41 years old female presented with lesion on the scalp and sternal mass, increasing in size with itchiness and erythematous for 6 months duration. Further CECT scan of brain and neck shows features of malignant left frontal scalp lesion with poor plane with overlying skin and underlying skull bone and CECT of thorax shows a large, irregular heterogeneously enhancing mass with necrotic center noted at right hilar within superior segment of right lower lobe, encasing right middle and lower lobe bronchi. Wedge biopsy of scalp lesion showed an intradermal lesion extensively infiltrating by malignant gland accompanied by desmoplasia and the tumor cells are seen extending into the surgical margins suggestive of ductal eccrine carcinoma.Clinical Discussion:This case highlights the importance and challenges in achieving early diagnosis coupled with the scarcity of information on these leads to difficulty in managing this patient.
Conclusion: In managing Ductal Eccrine Carcinoma tumor, standard method of treatment for has not been established. However, wide surgical excision is the treatment of choice for localized lesions. Regarding prognosis, there is conflicting data published which we describe in this article.