Documented Reperfusion of the Retina on Fluorescein Angiography after Administration of Intravenous Prostaglandin E1 for Central Retinal Artery Occlusion: A Case Report.

Central retinal artery occlusion (CRAO) can result in devastating permanent vision loss. Presently, there is no evidence-based treatment for CRAO that is widely accepted. In the literature, multiple studies propose intravenous (IV) prostaglandin E1 (IV PGE1) as a potential treatment option for patients with CRAO. We illustrate 2 cases of CRAO successfully treated with IV PGE1. In both cases, our patients with vascular risk factors were diagnosed with CRAO of the left eye. They were started on twice daily IV 40 µg PGE1 in 100 mL normal saline, with each dose administered over 3 h. In the first case, we documented reperfusion of the retina on fluorescein angiography after administration of IV PGE1. In the second case, our patient improved from no light perception visual acuity (VA) to count fingers VA within 48 h of treatment with IV PGE1. Our study highlights the vasodilatory effect of IV PGE1. Due to its mechanism of action and safety profile, it should be considered a potential treatment option for CRAO. Further randomized controlled trials are necessary to determine the overall therapeutic effect of IV PGE1 for CRAO.

Case report: Blotchy skin in a puffy neonate: is there a new association?

Introduction: Purpura fulminans in the neonatal population is a rare but potentially life-threatening condition complicated by thrombosis, resultant vital organ necrosis, and gangrene of the extremities. Considering the rapid evolution of the pathogenetic mechanism, an index of suspicion, early identification, and prompt intervention are imperative for improved outcomes. The majority of purpura fulminans cases have an infectious etiology, but it is essential to consider other congenital and acquired causes. Case description: We present a clinical case of a female neonate to emphasize the correlation between purpura fulminans, congenital chylothorax, involvement of the PAK2 gene, and the occurrence of retinal detachment in both eyes. After draining the congenital chylothorax, the neonate developed purpura fulminans due to a loss of protein C, S, and antithrombin factors, previously not reported in the literature. The purpuric lesions resolved after the administration of fresh frozen plasma. Subsequently, no recurring purpura fulminans lesions were noted following the normalization of the antithrombotic factor levels in the serum. Subsequently, the child also developed retinal detachment in both eyes.

Structured Referral Call Handling Process Improves Neonatal Transport Dispatch Times.

OBJECTIVE: In 2019 the Southern Alberta Neonatal Transport Service adopted a transport call handling process change to expedite transport team mobilization. This study compares the impact of this change on neonatal transport decision to dispatch and mobilization times. STUDY DESIGN: This retrospective cohort study was conducted using a historical cohort of neonates referred for transportation between January 2017 and December 2021. The "dispatch time" (DT) was the time from the start of consultation to the time a decision to dispatch the transport team was made, whereas "mobilization time" (MT) referred to the time from start of consultation to the time the team departed the home base. In 2019, a DT target of <3 minutes was implemented to meet a target MT of <15 and <30 minutes for emergent and urgent high-risk transport referral calls, respectively. In 2021 use of the "Situation" component of the SBAR (Situation, Background, Assessment, Recommendation) communication tool was introduced with the transport team asking five questions to determine need for mobilization. Data between 2017 and 2018 represented the preintervention period, 2019, the "washout" period for implementation, and 2020 to 2021, the postintervention period. Data were analyzed to determine trends in DT and MT. RESULTS: The DT was reduced from a median of 5 to 3 minutes following intervention (p < 0.001). DT target goal of 3 minutes was achieved in 67.08% of calls compared with 26.24% in the preintervention period, (p < 0.001). The team achieved MT target goals in 42.71% of urgent and emergent transfers compared with 18.05% prior to intervention (p < 0.001). CONCLUSION: Introduction of a time-sensitive referral call handling process improved dispatch and mobilization time of the neonatal transport team. KEY POINTS: · Time-sensitive triaging of neonatal transport referrals improves dispatch and mobilization time.. · A structured referral call handling process improves the efficiency of neonatal transport decision-making.. · Dedicated neonatal transport vehicles are likely to improve neonatal transport mobilization time..

Neurodevelopmental Outcomes of Preterm Infants Born <29 Weeks with Bronchopulmonary Dysplasia-Associated Pulmonary Hypertension: A Multicenter Study.

OBJECTIVE: This study aimed to determine neurodevelopmental outcomes of preterm infants born at <29 weeks' gestational age (GA) with bronchopulmonary dysplasia and pulmonary hypertension (BPD-PH) at 18 to 24 months' corrected age (CA). STUDY DESIGN: In this retrospective cohort study, preterm infants born at <29 weeks' GA between January 2016 and December 2019, admitted to level 3 neonatal intensive care units, who developed BPD and were evaluated at 18 to 24 months' CA in the neonatal follow-up clinics were included. We compared demographic characteristics and neurodevelopmental outcomes between the two groups: Group I: BPD with PH and Group II: BPD with no PH, using univariate and multivariate regression models. The primary outcome was a composite of death or neurodevelopmental impairment (NDI). NDI was defined as any Bayley-III score < 85 on one or more of the cognitive, motor, or language composite scores. RESULTS: Of 366 eligible infants, 116 (Group I [BPD-PH] =7, Group II [BPD with no PH] = 109) were lost to follow-up. Of the remaining 250 infants, 51 in Group I and 199 in Group II were followed at 18 to 24 months' CA. Group I and Group II had median (interquartile range [IQR]) birthweights of 705 (325) and 815 g (317; p = 0.003) and median GAs (IQR) were 25 (2) and 26 weeks (2; p = 0.015) respectively. Infants in the BPD-PH group (Group I) were more likely to have mortality or NDI (adjusted odds ratio: 3.82; bootstrap 95% confidence interval; 1.44-40.87). CONCLUSION: BPD-PH in infants born at <29 weeks' GA is associated with increased odds of the composite outcome of death or NDI at 18 to 24 months' CA. KEY POINTS: · Long-term neurodevelopmental follow-up of preterm infants born <29 weeks' GA.. · Association of neurodevelopmental outcomes with BPD-associated PH.. · Need for longitudinal follow-up of children with BPD-associated PH..

Advanced Systemic Mastocytosis with associated haematological neoplasm: Treatment with avapritinib can facilitate successful bridge to allogeneic haematopoietic cell transplant.

Advanced systemic mastocytosis (AdvSM) is a rare, life-limiting mast cell (MC) neoplasm, with approximately 70% patients having an associated haematological neoplasm (AHN). Avapritinib, a selective tyrosine kinase inhibitor targeting KIT D816V, has shown potent activity translating clinically into durable responses in the phase 1 EXPLORER (NCT02561988) and phase 2 PATHFINDER (NCT03580655) studies. We report three patients with AdvSM-AHN on avapritinib who achieved complete remission (CR) of SM and were successfully bridged to allogeneic haematopoietic cell transplant (allo-HCT). Two cases additionally highlight the risk of clonal evolution within the AHN component and requirement for close monitoring while on targeted therapy.

Cost effectiveness of using Faecal Immunochemical Testing (FIT) as an initial diagnostic investigation for patients with lower gastrointestinal symptoms suggestive of malignancy.

BACKGROUND: There has been an increase in the numbers of patients presenting to primary care with suspected colorectal malignancy and subsequently an increase in demand for endoscopy. This study aims to forecast the cost of faecal immunochemical testing (FIT) compared to conventional diagnostic tests as a primary investigation for patients with symptoms suggestive of colorectal malignancy. METHODS: Retrospectively, 1950 patients with symptoms suggestive of colorectal malignancy who were referred through primary care and underwent investigations through standard endoscopic evaluation were included. These patients were used to forecast the cost of faecal immunochemical testing creating theoretical data for sensitivity and specificity. Outcome measures included: the number of investigations under current protocol; cost of current investigations; number of predicted false negatives and false positives and positive/negative predictive values using current sensitivity data for FIT; the cost forecast of using FIT as the primary investigation for colorectal malignancy. RESULTS: Median age was 65 (IQR 47-82) with 43.7% male and 56.3% female. A total of 1950 investigations were carried out with a diagnostic yield of 26 cancers (18 colon, 8 rectal), 138 polyps and 29 high risk adenomas (HGD ± > 10 mm). In total, £713,948 was spent on the investigations. The commonest investigation was colonoscopy totalling £533,169. The total cost per cancer diagnosis was £27,459. Sensitivity (92.1% CI 86.9-95.3) and specificity (85.8% CI 78.3-90.1) for FIT in colorectal cancer was taken from NICE and was costed via the manufacturer(s). The projected total cost of FIT for the same population using a ≥ 4 µg haemoglobin cut off was £415,680 (£15,554 per cancer). The total cost of high-risk polyps using ≥ 4 µg cut off was £404,427 (sensitivity 71.2% CI 60.5-87.2, specificity 79.8%CI 76.1-83.7) or £13,945 per polyp. CONCLUSIONS: FIT is a cheaper and effective alternative test with the potential to replace current expensive methods. The forecast is based on the limited data available for sensitivity/specificity in the current literature. FIT has now been commenced for symptomatic patients in the UK and therefore sensitivity may change in the future.

Modulation of mitochondrial function by the microbiome metabolite propionic acid in autism and control cell lines.

Propionic acid (PPA) is a ubiquitous short-chain fatty acid, which is a major fermentation product of the enteric microbiome. PPA is a normal intermediate of metabolism and is found in foods, either naturally or as a preservative. PPA and its derivatives have been implicated in both health and disease. Whereas PPA is an energy substrate and has many proposed beneficial effects, it is also associated with human disorders involving mitochondrial dysfunction, including propionic acidemia and autism spectrum disorders (ASDs). We aimed to investigate the dichotomy between the health and disease effects of PPA by measuring mitochondrial function in ASD and age- and gender-matched control lymphoblastoid cell lines (LCLs) following incubation with PPA at several concentrations and durations both with and without an in vitro increase in reactive oxygen species (ROS). Mitochondrial function was optimally increased at particular exposure durations and concentrations of PPA with ASD LCLs, demonstrating a greater enhancement. In contrast, increasing ROS negated the positive PPA effect with the ASD LCLs, showing a greater detriment. These data demonstrate that enteric microbiome metabolites such as PPA can have both beneficial and toxic effects on mitochondrial function, depending on concentration, exposure duration and microenvironment redox state with these effects amplified in LCLs derived from individuals with ASD. As PPA, as well as enteric bacteria, which produce PPA, have been implicated in a wide variety of diseases, including ASD, diabetes, obesity and inflammatory diseases, insight into this metabolic modulator from the host microbiome may have wide applications for both health and disease.

Review of Giant cell arteritis.

Giant-cell arteritis (GCA) is a systemic autoimmune disease affecting primarily the elderly. Giant cell arteritis can cause sudden and potentially bilateral sequential vision loss in the elderly. Therefore, it is considered a medical emergency in ophthalmology and a significant cause of morbidity in an increasingly aging population. Ophthalmologists need to be able to recognize the classic symptoms and signs of this disease, and then be able to work-up and treat these patients in an efficient manner. An in-depth review of GCA from the literature as well as personal clinical experience follows.

Real-time ultrasound guided percutaneous dilatational tracheostomy with and without bronchoscopic control: an observational study.

BACKGROUND: Real-time ultrasound guidance may enable precise introducer needle and guidewire insertion during percutaneous dilatational tracheostomy (PDT). However, the safety of PDT done solely under ultrasound guidance has not been compared to additional bronchoscopic confirmation. We aimed to compare the efficacy, incidence of complications and procedural times of real-time ultrasound guided PDTs done with and without bronchoscopic confirmation. METHODS: We analysed all bedside PDTs performed over an 18 month period. On transverse view, the introducer needle was inserted under real time guidance into the chosen interspace. The interspace of guidewire entry was confirmed on longitudinal view. In the bronchoscope group, needle and guidewire position within the tracheal lumen were confirmed by bronchoscopy. RESULTS: We performed 177 PDTs under ultrasound guidance during the study period - 95 with and 82 without bronchoscopic confirmation. The introducer needle was inserted at the desired level on the first attempt in 81.4% of instances with no difference between groups (77.9% vs. 85.4%, P=0.6). Introducer needle entry between the first and third tracheal cartilages did not differ between groups (90.5% vs. 92.7%, P=0.6). Procedural times were longer in the bronchoscope group (13.9 min vs. 10.7 min, P<0.0001). There were more episodes of fall in oxygen saturation below 90% in the bronchoscope group (16.8% vs. 3.7%, P=0.006). Minor bleeding occurred in 6.2% of cases and did not differ between groups. CONCLUSION: Ultrasound guidance for PDT may be equally safe, even without bronchoscopic confirmation; the incidence of hypoxic episodes may be less and the procedure can be performed in less time.

Rituximab therapy for primary Sjögren's syndrome: an open-label clinical trial and mechanistic analysis.

OBJECTIVE: To study the safety and clinical efficacy of rituximab therapy for primary Sjögren's syndrome, as well as to investigate its mechanisms. METHODS: Patients with primary Sjögren's syndrome were enrolled in an open-label trial, were given rituximab (1 gm) infusions on days 1 and 15, and were monitored through week 52. The primary end point was safety, with secondary end points evaluating clinical and biologic efficacy. Blood was obtained for enumeration of lymphocyte subsets, measurement of serum autoantibody and BAFF levels, and analysis of gene expression. RESULTS: Twelve female patients with primary Sjögren's syndrome were administered rituximab. They had a median age of 51 years (range 34-69 years) and a median disease duration of 8.0 years (range 2-18 years). We observed no unexpected toxicities from the rituximab therapy. Modest improvements were observed at week 26 in patient-reported symptoms of fatigue and oral dryness, with no significant improvement in the objective measures of lacrimal and salivary gland function. The recovery of blood B cells following the nadir from rituximab therapy was characterized by a predominance of transitional B cells and a lack of memory B cells. While blood B cell depletion was associated with an increase in serum BAFF levels, no significant changes were observed in the levels of serum anti-Ro/SSA, anti-La/SSB, and anti-type 3 muscarinic acetylcholine receptor autoantibodies or in the blood interferon signature. CONCLUSION: In patients with primary Sjögren's syndrome, a single treatment course of rituximab was not associated with any unexpected toxicities and led to only modest clinical benefits despite effective depletion of blood B cells.

Clinical, neuroimaging and pathological features of 5-nitroimidazole-induced encephalo-neuropathy in two patients: Insights into possible pathogenesis.

We report two patients manifesting with involvement of central and peripheral nervous system with brain magnetic resonance imaging (MRI) changes and pathological features of neuropathy possibly due to harmful and chronic use of various nitroimidazole group of medications for recurrent diarrheal illness. Patient 1, a 21-year-old man with obsessive-compulsive disorder, impulsive behavior and harmful use of substance (tinidazole), had developed encephalopathy and biopsy-proven neuropathy with partial remission. The MRI of brain showed involvement of bilateral caudate, lentiform and dentate nuclei, and splenium, with contrast enhancement of the caudate and putaminal lesions and restricted diffusion of the splenial lesion. Patient 2 was a 50-year-old woman with irritable bowel syndrome and was on harmful use of tinidazole and metronidazole. She manifested with encephalopathy, ataxia, and neuropathy. Her MRI of brain revealed involvement of bilateral putamen, dentate nuclei and periventricular white matter with restricted diffusion. Sural nerve biopsy revealed evidence of vasculitic neuropathy. At follow-up, there was definite, though incomplete, recovery in both the patients. The MRI alterations improved completely in patient 2 and substantially in patient 1. Increasing awareness among the physicians may enable early recognition of potentially reversible neurotoxicity and avoid unwarranted prescription of such medications.

Critically ill patients with 2009 H1N1 infection in an Indian ICU.

BACKGROUND AND AIMS: The 2009 pandemic influenza A (H1N1) has taken its toll across most parts of India. We aimed to study its epidemiology, clinical characteristics and outcomes from an Indian multidisciplinary intensive care unit (ICU). MATERIALS AND METHODS: All patients admitted to our ICU with a flu-like illness and who tested positive for the 2009 H1N1 by reverse -transcriptase polymerase- chain -reaction assay during a 3 month period were prospectively studied. RESULTS: Thirty one patients were admitted to the ICU during the study period. Patients were in the younger age group with a median age of 35 years (IQR: 28.2-42.8). Obesity was the commonest risk factor. Twenty six patients (83.9%) required ventilator support; the median duration of ventilator support was 10 days (IQR: 4-22). Severe hypoxemia was the predominant feature in all patients. Circulatory failure requiring vasopressors occurred in 18 (58.1%) patients and acute kidney injury in 6 (3.2%) patients. Twenty six patients were alive at the end of 28 days; subsequently all except one were discharged. The median duration of hospital stay was 15 (IQR: 8-22.5) days. Increasing APACHE II scores were associated with an increased risk of death (Hazard Ratio: 1.1; CI: 1.08 -1.2; P = 0.04). Mean tidal volumes in non-survivors were significantly lower; this was related to poor lung compliance in this group. CONCLUSIONS: 2009 H1N1 infection caused severe disease in relatively young patients without significant co-morbidities, characterized by severe hypoxemia and the requirement for prolonged mechanical ventilation. Extra-pulmonary organ failure included circulatory and renal failure.

Ruptured hydatid cyst masquerading as tension pneumothorax.

We report a 35-year-old man who presented to us with features suggestive of a tension pneumothorax. However insertion of a chest tube did not result in any signs of improvement. Further investigation demonstrated that he had a ruptured hydatid cyst. He underwent an emergency thoractomy and excision of the cyst. Ruptured hydatid cyst should be considered as a possibility in patients who present with sudden onset respiratory distress and hypoxia in geographical areas where the disease may be prevalent.

Factors affecting the nasal carriage of methicillin-resistant Staphylococcus aureus in human immunodeficiency virus-infected patients.

Human immunodeficiency virus-infected patients attending skin outpatient department were studied for nasal carriage of methicillin-resistant Staphylococcus aureus (MRSA) and associated factors affecting nasal colonization. Nasal swabs were used for isolation of S. aureus. MRSA were detected by agar screen and agar dilution methods. Careful examination for dermatoses was carried out. Forty-six of the 60 (76.67%) outpatients with HIV infection were colonized with S. aureus in the anterior nares. Significant number of S. aureus carriers were in the 31-40 year age group. Methicillin resistance was found in eight (17.39%) isolates. Of the 46 S. aureus strains, 29 (63%) were resistant to erythromycin, 69.5% to co-trimoxazole and 41.3% to ciprofloxacin. Co-trimoxazole use was found to be a risk factor for S. aureus carriage ( P = 0.0214) but not for methicillin resistance. Hospital stay for more than 10 days was a risk factor for methicillin resistance whereas stay for more than 25 days was found to be a highly significant risk factor. Dermatophytosis and herpes simplex virus infection were other risk factors for nasal carriage of S. aureus.

Jaggery: an avoidable cause of severe, deadly pediatric burns.

BACKGROUND: Jaggery is the non-industrial refinement of sugar cane into a sugar product. Sugar cane cultivation, harvest and refinement are central aspects of rural Indian life. METHODS: We present a retrospective review of pediatric burns at a single institution in Southern India, drawing special attention to scald burns incurred when young children fall into the cauldron of boiling jaggery. Descriptive statistics comparing children burned by jaggery and children burned by other mechanisms were performed. Multivariable logistic regression including burn size and mechanism of burn (jaggery and non-jaggery) was performed to determine the increased risk of death when burned by jaggery. RESULTS: Children burned by jaggery immersions are older, more likely male, and have larger burns. They have longer hospital stays, more operations, and are more likely to die. When controlling for age, gender, size of burn, and mechanism, jaggery exposure was associated with a higher mortality. DISCUSSION: Jaggery burns are deadly, devastating burns which could be prevented. While jaggery and sugar cane production can lead to economic independence for rural Indian villages, the cost it exacts from burns and death to the youngest and most vulnerable children must be addressed and prevented.

Fatal aluminium phosphide poisoning due to myocardial depression refractory to high dose inotropic support and intra-aortic balloon counterpulsation.

We report a case of aluminium phosphide poisoning that presented to us with refractory myocardial depression. This patient developed worsening circulatory failure that did not respond to inotropic or vasopressor support and intra-aortic balloon counterpulsation. She went on to develop progressive metabolic acidosis, renal failure and died within 48 hours of admission to the intensive care unit.