Case number 4 : chronic granulomatous disease (CGD)

An 8-year old girl presented with a 2-year history of recurrent nasal skin infections. In this context of a prolonged history of facial and nasal infections, multiple perianal abscesses and a non-specific chronic colitis, an underlying immunodeficiency was suspected. She was referred for further immunological assessment and after ruling out other haematological disorders, the diagnosis of Chronic Granulomatous Disease (CGD) was made following an abnormal Nitroblue Tetrazolium (NBT) test. The patient was started on long-term prophylactic antibiotics and genetic testing is currently being carried out on her family. Bone marrow transplantation is the optimum treatment but will only be considered when the benefits outweigh the risks.peer-reviewe

Sustainability assessment of wastewater reuse in a Portuguese military airbase

Funding Information: This research was funded by School for International Training, World Learning, Vermont, United States. The authors acknowledge the Portuguese Foundation for Science and Technology (FCT) for the support given to CENSE through the strategic project UIDB/04085/2020. The authors would also like to thank to the Air Force members, namely the Air Base No.5 Commander João Vicente and his team, for the availability to support this work. Publisher Copyright: © 2022 Elsevier B.V.The current water-scarcity crisis that is being felt in Europe, namely in the southern region, has leveraged the development and implementation of national and regional water management plans. These policies aim to promote efficient wastewater reuse in industrial and urban sectors. Thus, stakeholders are now seeking strategies to enhance the sustainability of their wastewater treatment processes. The present work details the evaluation of the wastewater treatment methods used at an Air Force Base located in Portugal. In addition, this study also intended to determine how wastewater reuse can be implemented and add value to the environmental protection mission of the military airbase. Hence, an assessment of wastewater treatment practices was carried out, considering primary and secondary treatments. The chemical, physical, and biological indicators of samples collected over two consecutive years were analyzed to determine trends and fluctuations. The results revealed that the overall effectiveness of nutrient removal is low due to the oversized nature of the treatment plant, the age of the facility, and the composition of the wastewater. The effluent produced meets standards for non-potable reuse and could be used on base for aircraft maintenance and the cleaning of facilities. Nonetheless, the effectiveness of the plant could be improved by implementing a more advanced tertiary wastewater treatment to decrease the concentration of undesired compounds (e.g., total nitrogen), enabling the reuse of water in a broader range of activities.publishersversionpublishe

An unusual cause of chest pain in a young man : bronchogenic cysts and their cardiac manifestations

We report a case of a 24-year-old man who presented with chest pain and electrocardiographic evidence of myocardial ischaemia. An abnormal structure located behind the heart on the urgent transthoracic echocardiogram and a computed tomography scan of the mediastinum led to prompt surgery with eventual resection of the lesion. The histology revealed fragments of connective tissue covered by squamous epithelium and ciliated epithelium, consistent with a bronchogenic cyst. The case study is accompanied by a literature review of the pathogenesis, diagnosis and management of bronchogenic cysts and their association with cardiac symptoms.peer-reviewe

Causes and outcomes of hyponatraemia at Mater Dei Hospital, Malta

CONTEXT: Hyponatraemia is the most common electrolyte balance disorder in clinical practice, amounting to 15–20% of casualty visits. While there is general agreement that associated mortality rates are high, most studies are uncontrolled.OBJECTIVE: To determine the characteristics, causes and outcome of severe hyponatraemia (<125 mmol/l) in hospitalised patients and to indentify mortality predictors.DESIGN: This is a retrospective case-controlled study of all medical admissions in the months of February, June and November, who at any point during the index admission developed a serum sodium <125 mmol/l. For each case, an age- and gender-matched control was identified.RESULTS: A total of 5195 medical admissions were reviewed. Of these, 193 patients had a sodium level <125 mmol/. 26 patients were excluded from the case group leaving a total of 167 cases and 193 controls. Length of hospital stay was more prolonged in the case group (12 vs 8 days, P<0.001). There was a highly significant excess mortality, both during the index admission (25% in cases vs 7% in controls (P < 0.001)) as well as till the end of the follow-up period (52% in cases vs 22% in controls (P<0.001)). Mortality was unrelated to severity of hyponatraemia. Patients who developed the lowest serum sodium later on during their admission (ie sodium levels continued falling during the admission or fell de novo), had a higher rate of mortality than patients whose lowest serum sodium was on the day of admission (64.3% vs 45%, P 0.019). A cox regression analysis showed that hyponatraemia (P<0.001), male gender (P 0.033), age (P 0.021), and serum creatinine level (P 0.008) were independent risk factors for mortality. There was no statistically significant difference between the rates of ITU admission at different levels of hyponatraemia <125 mmol/l (P=0.497). Thus, serum sodium levels of <125 mmol/l should be used to identify patients who need more intensive monitoring and therapy irrespective of the degree of hyponatraemia. Only 41% of cases developed neurological symptoms, of these, confusion and altered level of consciousness were the more prevalent at 12% each, followed by falls (9%), unsteady gait (4%) and seizures (4%). The cause for hyponatraemia was frequently poorly evaluated and in 23% of cases no definite diagnosis was made.CONCLUSION: Data on assessment, investigation and management of hyponatraemia illustrates variability and shortcomings in clinical practice. The question remains whether the relationship between hyponatraemia and increased mortality is causal or associative.peer-reviewe

The role of attachment in body weight gain and weight loss in bariatric patients

Purpose: To explore the role of attachment styles in obesity. Material and methods: The present study explored differences in insecure attachment styles between an obese sample waiting for bariatric surgery (n=195) and an age, sex and height matched normal weight control group (n=195). It then explored the role of attachment styles in predicting change in BMI one year post bariatric surgery (n=143). Results: The bariatric group reported significantly higher levels of anxious attachment and lower levels of avoidant attachment than the control non obese group. Baseline attachment styles did not, however, predict change in BMI post-surgery. Conclusion: Attachment style is different in those that are already obese from those who are not. Attachment was not related to weight loss post-surgery