Shein R, du Toit J, Goeijenbier M
… +2 more, du Toit C, Verburgh E
Neth J Med
· 2020 Jul · PMID 32641545
Acquired haemophilia A is a rare coagulation disorder, which can lead to life-threatening haemorrhages if not identified and treated promptly. It is characterised by the presence of autoantibodies (inhibitors) to factor...Acquired haemophilia A is a rare coagulation disorder, which can lead to life-threatening haemorrhages if not identified and treated promptly. It is characterised by the presence of autoantibodies (inhibitors) to factor VIII. Acquired haemophilia A associated with HIV is a rare but well described phenomenon with limited directions to its management. We comparatively describe four patients - two with HIV and two without - that presented with unusual bleeding episodes with a prolonged activated partial thromboplastin time secondary to factor VIII inhibitors. An empiric observation is that the patients with acquired haemophilia A associated with HIV had higher antibody titres at presentation, that required more prolonged immunosuppressive therapy to induce remission.
Porphyrias are rare metabolic disorders. Lack of awareness and knowledge about the clinical features of porphyrias results in diagnostic and therapeutic delays for many patients. Delays in diagnosing and treating porphyr...Porphyrias are rare metabolic disorders. Lack of awareness and knowledge about the clinical features of porphyrias results in diagnostic and therapeutic delays for many patients. Delays in diagnosing and treating porphyrias can result in severe, progressive morbidity (and mortality) and psychological distress for patients. This review discusses the pathophysiology, diagnosis, treatment, and follow-up of the most prevalent porphyrias: acute intermittent porphyria, porphyria cutanea tarda, and erythropoietic protoporphyria.
Papaioannou TG, Georgiopoulos G, Stamatelopoulos KS
… +9 more, Protogerou AD, Alexandraki KI, Argyris A, Vrachatis DA, Soulis D, Papanikolaou A, Manolesou D, Daskalopoulou SS, Tousoulis D
Neth J Med
· 2020 Jul · PMID 32641542
BACKGROUND: Within-visit variability of repeated sequential readings of blood pressure (BP) is an important phenomenon that may affect precision of BP measurement and thus decision making concerning BP-related risk and h...BACKGROUND: Within-visit variability of repeated sequential readings of blood pressure (BP) is an important phenomenon that may affect precision of BP measurement and thus decision making concerning BP-related risk and hypertension management. However, limited data exist concerning predictive ability of within-visit BP variability for clinical outcomes. Therefore, we aimed to investigate the association between the variability of three repeated office BP measurements and the risk of all-cause mortality, independent of BP levels. METHODS: Data collected through the National Health and Nutrition Examination Survey (NHANES) were analysed. NHANES is a program of studies designed to assess health and nutritional status of adults and children in the United States. A complete set of three sequential BP measurements, together with survival status, were available for 24969 individuals (age 46.8±;19.3 years, 49% males). Multivariable logistic regression models were used to determine the prognostic ability of the examined demographic, clinical, and haemodynamic indices. RESULTS: Among various examined indices of variability of systolic (SBP) and diastolic (DBP) blood pressure measurements, the standard deviation of DBP (DBPSD) was the stronger independent predictor of mortality (odds ratio 1.064, 95% Confidence Interval: 1.011-1.12) after adjustment for age, sex, body mass index, smoking, SBP, heart rate, history of hypertension, diabetes mellitus, hypercholesterolaemia, and cardiovascular events. CONCLUSION: Within-visit variability of three sequential office DBP readings may allow for the identification of high-risk patients better than mean SBP and DBP levels. The predictive value of within-visit BP variability and methods to improve its clinical application are worthy of further research.
Grim CCA, Cornet AD, Kroner A
… +28 more, Meiners AJ, Brouwers AJBW, Reidinga AC, van Westerloo DJ, Bergmans DCJJ, Gommers D, Versluis D, Weller D, Christiaan Boerma E, van Driel E, de Jonge E, Schoonderbeek FJ, Helmerhorst HJF, Jongsma-van Netten HG, Weenink J, Woittiez KJ, Simons KS, van Ewelie L, Petjak M, Sigtermans MJ, van der Woude M, Cremer OL, Bijlstra P, van der Heiden P, So RKL, Vink R, Jansen T, de Ruijter W
Neth J Med
· 2020 Jul · PMID 32641541
BACKGROUND: Over the last decade, there has been an increasing awareness for the potential harm of the administration of too much oxygen. We aimed to describe self-reported attitudes towards oxygen therapy by clinicians...BACKGROUND: Over the last decade, there has been an increasing awareness for the potential harm of the administration of too much oxygen. We aimed to describe self-reported attitudes towards oxygen therapy by clinicians from a large representative sample of intensive care units (ICUs) in the Netherlands. METHODS: In April 2019, 36 ICUs in the Netherlands were approached and asked to send out a questionnaire (59 questions) to their nursing and medical staff (ICU clinicians) eliciting self-reported behaviour and attitudes towards oxygen therapy in general and in specific ICU case scenarios. RESULTS: In total, 1361 ICU clinicians (71% nurses, 24% physicians) from 28 ICUs returned the questionnaire. Of responding ICU clinicians, 64% considered oxygen-induced lung injury to be a major concern. The majority of respondents considered a partial pressure of oxygen (PaO2) of 6-10 kPa (45-75 mmHg) and an arterial saturation (SaO2) of 85-90% as acceptable for 15 minutes, and a PaO2 7-10 kPa (53-75 mmHg) and SaO2 90-95% as acceptable for 24-48 hours in an acute respiratory distress syndrome (ARDS) patient. In most case scenarios, respondents reported not to change the fraction of inspired oxygen (FiO2) if SaO2 was 90-95% or PaO2 was 12 kPa (90 mmHg). CONCLUSION: A representative sample of ICU clinicians from the Netherlands were concerned about oxygen-induced lung injury, and reported that they preferred PaO2 and SaO2 targets in the lower physiological range and would adjust ventilation settings accordingly.
PURPOSE: To determine whether the use of salicylates is a predictive factor for detecting explanatory pathology during gastroscopy or colonoscopy procedures in patients with iron deficiency anaemia (IDA). METHODS: This r...PURPOSE: To determine whether the use of salicylates is a predictive factor for detecting explanatory pathology during gastroscopy or colonoscopy procedures in patients with iron deficiency anaemia (IDA). METHODS: This retrospective study included patients who underwent a gastroscopy and/or a colonoscopyto determine the cause of IDA at Treant Healthcare, hospital location Scheper in Emmen, the Netherlands, between 2010 and 2016. The study compared two groups. The first group consisted of patients who were not taking antithrombotics at the time of, and during the last six months prior to, the endoscopy. The second group consisted of patients who used salicylates at the time of, and during the last six months prior, to the endoscopy. Data were collected on whether and which explanatory pathology was found in the endoscopic evaluation. RESULTS: In total, 464 patients were included, of whom, 174 were using a salicylate and 290 were not. In 41.2% of the patients, explanatory pathology was found, which was not significantly different between the two groups with univariate analysis (p = 0.207). However, the patients in the group of salicylate users were significantly older and more often male. When correcting for these differences in group characteristics during multivariate analysis, the use of salicylates was found to be a negative predictive factor for finding explanatory pathology (p < 0.001; OR 2.307). CONCLUSION: When determining the chance of finding explanatory pathology during endoscopic evaluation in patients with IDA, the use of salicylates should be taken into account as a negative predictive factor for finding explanatory pathology during endoscopic evaluation.
Khairoun M, Meynen F, Vercoutere W
… +1 more, Leavis HL
Neth J Med
· 2020 Apr · PMID 32332189
Macrophage activation syndrome (MAS) is a secondary form of haemophagocytic lymphohistiocytosis (HLH). MAS-HLH is an underrecognised and life-threatening condition associated with a heterogeneous group of diseases includ...Macrophage activation syndrome (MAS) is a secondary form of haemophagocytic lymphohistiocytosis (HLH). MAS-HLH is an underrecognised and life-threatening condition associated with a heterogeneous group of diseases including connective tissue disease and inflammatory disorders. Here, we report three cases of adult patients with MAS-HLH triggered by different entities, including systemic lupus erythematosus, Griscelli syndrome type 2, and Adult onset Still's disease.
We present a case with five auto-immune phenomena, including Sjögren's syndrome, for which we also diagnosed a tip lesion of focal segmental glomerulosclerosis (FSGS).v About one-third of Sjögren's syndrome patients have...We present a case with five auto-immune phenomena, including Sjögren's syndrome, for which we also diagnosed a tip lesion of focal segmental glomerulosclerosis (FSGS).v About one-third of Sjögren's syndrome patients have renal involvement, but FSGS is rarely reported. FSGS is thought to involve T-cell dysfunction and in this patient with multiple auto-immune phenomena, it may reflect a severe dysregulation of cellular immunity.
Woudstra J, de Boer MP, Hempenius L
… +1 more, van Roon EN
Neth J Med
· 2020 Apr · PMID 32332187
BACKGROUND: Hyponatraemia due to the syndrome of inappropriate antidiuretic hormone secretion (SIADH) can pose a therapeutic challenge. After fluid restriction, urea is recommended as a second-line treatment by Dutch and...BACKGROUND: Hyponatraemia due to the syndrome of inappropriate antidiuretic hormone secretion (SIADH) can pose a therapeutic challenge. After fluid restriction, urea is recommended as a second-line treatment by Dutch and European treatment guidelines. Data on this practice are still scarce. We introduced urea for the treatment of SIADH in our hospital and prospectively collected data on its effectiveness and tolerability. METHODS: In hospitalised patients with a serum sodium level ≤ 129 mmol/l due to SIADH, urea in a dosage of 0.25-0.50 g/kg/day was indicated if prescribed fluid restriction had no effect or could not be applied. Measurement of serum sodium was performed at baseline, after the first and second day of urea therapy and at the end of the first inpatient treatment episode (EIT). The primary outcomes were normonatraemia (serum sodium level 135-145 mmol/l) at EIT and discontinuation of urea due to side effects. RESULTS: Thirteen patients were treated with urea over a median of 5 days (range 2-10 days). The median serum sodium level at baseline was 124 mmol/l (IQR 122-128), which increased to 128 mmol/l (IQR 123-130) (p = 0.003) after the first dose of urea and to 130 mmol/l (IQR 127-133) (p = 0.002) after the second dose of urea. Normonatraemia at EIT was observed in 8 (62%) patients. Seven (54%) patients reported distaste. In one of these patients, urea was discontinued because of nausea. Overcorrection was not observed. CONCLUSION: Our data show that urea is an effective treatment for hospitalised patients with SIADH. Distaste was a frequent side effect, but usually did not lead to early treatment discontinuation.
Blans MJ, Pijl MEJ, van de Water JM
… +2 more, Poppe HJ, Bosch FH
Neth J Med
· 2020 Apr · PMID 32332186
Point-of-care ultrasound (POCUS) is gaining interest in intensive care medicine and good reviews and guidelines on POCUS are available. Unfortunately, how to implement POCUS and practical examples how to train staff and...Point-of-care ultrasound (POCUS) is gaining interest in intensive care medicine and good reviews and guidelines on POCUS are available. Unfortunately, how to implement POCUS and practical examples how to train staff and junior doctors is not well described in literature. We discuss the process of POCUS implementation, and a POCUS training program for residents prior to their intensive care rotation in a Dutch teaching hospital intensive care unit. The described four-day basic POCUS course consists of short tutorials and ample time for hands-on practice. Theoretical tests are taken shortly before, on the last day of the course, and after three months to assess learning retention. Practical tests are taken on the last day of the course and after three months. We stress the importance of POCUS for intensive care and hope that our experiences will help colleagues who also want to go forward with POCUS.
van Halem K, Cox J, Messiaen P
… +6 more, Pat K, Declercq C, Meersman A, Ramaekers D, Cartuyvels R, van der Hilst J
Neth J Med
· 2020 Apr · PMID 32332185
The current Covid-19 outbreak poses many challenges on hospital organisation and patient care. Our hospital lies at the epicentre of the Belgian epidemic. On April 1st, a total of 235 Covid-19 patients had been admitted...The current Covid-19 outbreak poses many challenges on hospital organisation and patient care. Our hospital lies at the epicentre of the Belgian epidemic. On April 1st, a total of 235 Covid-19 patients had been admitted to our hospital. This demanded an unprecedented adaptation of our hospital organisation, and we have met many clinical issues in the care for Covid-19 patients. In this article, we share our experience in the handling of some of the practical and organisational issues in the care for Covid-19 patients.
Anaemia is a common diagnosis for clinicians. This mini-review summarises criteria for diagnosing the cause of anaemia. Within the microcytic anaemias, iron-deficient anaemia is most common. In addition, we would like to...Anaemia is a common diagnosis for clinicians. This mini-review summarises criteria for diagnosing the cause of anaemia. Within the microcytic anaemias, iron-deficient anaemia is most common. In addition, we would like to raise awareness of thalassaemia as a differential diagnosis. A normocytic anaemia, such as anaemia of chronic disease, is a diagnosis of exclusion. A macrocytic anaemia scheme is provided and differentiates based on reticulocyte count. We aim to provide the readers a clear overview of anaemia and when to refer to haematologists.
Goto NA, van Loon IN, Boereboom FTJ
… +1 more, Hamaker ME
Neth J Med
· 2020 Apr · PMID 32332183
Decision-making in older persons with end-stagebkidney disease (ESKD) regarding dialysis initiation is highly complex. While some older persons improve with dialysis and maintain a good quality of life, others experience...Decision-making in older persons with end-stagebkidney disease (ESKD) regarding dialysis initiation is highly complex. While some older persons improve with dialysis and maintain a good quality of life, others experience less benefit and multiple complications due to a high morbidity burden and (early) mortality. Geriatric impairments are highly prevalent among this population and these impairments may complicate the care of an older person with ESKD. Knowledge of these impairments can potentially help improve care and decision-making regarding dialysis initiation and advance care planning. Therefore, the aim of this review is to give healthcare providers an insight into the existing literature on geriatric impairments in older persons with ESKD. Furthermore, specific areas of concern will be discussed, in combination with some practical advice.