Saving, investment, government finance and the current account: The Dutch experience

Economic Policy;Current Account

Buffercapaciteit : bedrijfsstijlen in de melkveehouderij, volatiele markten en kengetallen

Dit rapport doet verslag van een statistische analyse uitgevoerd op een bestand van Nederlandse melkveehouderijbedrijven. Het doel van deze analyse was na te gaan wat de effecten waren van de melkprijsdaling, die zich voordeed in de tweede helft van 2008 en de eerste helft van 2009. De toenmalige prijsdaling beschouwen we als een voorbode van de situatie die steeds meer de zuivelmarkt zal typeren: een volatiele markt met scherpe prijswisselingen. Het onderzoek toont aan dat de effecten van een melkprijsdaling differentieel van aard zijn, ze variëren al naar gelang het type bedrijf. Om deze verschillen te vatten is een bedrijfsstijlenanalyse uitgevoerd die 4 strategische grondpatronen naar voren brengt: schaalvergroting, fijnregulering, kostenreductie en arbeidsbesparing. In ‘normale’ jaren kan via elk strategisch grondpatroon een goed inkomen worden verworven. Naarmate men meer ‘stijlspecifiek’ is, is het inkomen beter. In ‘slechte’ jaren ligt dit anders: dan blijken strategieën, die draaien om kostenreductie en fijnregulering, tot de beste resultaten te leiden. Ook de balans van vreemd en eigen vermogen speelt in slechte jaren een grote rol. Op haar beurt hangt die balans ook weer op significante wijze samen met de verschillende stijlen. Daarmee brengt de analyse een verschil naar voren dat in de komende jaren wellicht van groot belang zal worden: dat is het verschil tussen boerenlandbouw en ondernemerslandbouw. Met dit onderscheid wordt hier geen moreel of maatschappelijk oordeel gegeven. Waar het om gaat is de vraag welke vorm van landbouwbeoefening het best moeilijke marktomstandigheden weet te weerstaan. In de tweede helft van dit verslag staat de vraag centraal: welk kengetal biedt de melkveehouder het meeste houvast om na te gaan of zijn bedrijf over voldoende veerkracht beschikt om periodes van lage prijzen het hoofd te bieden? Er wordt een nieuw kengetal voorgesteld: buffercapaciteit. Daarnaast kijken we bij welke bedrijfsopzet (stijl) de buffercapaciteit het hoogst is en hoe deze verder is op te voere

Cardiovascular disease in non-classic Pompe disease: A systematic review

Pompe disease is a rare inherited metabolic and neuromuscular disorder, presenting as a spectrum, with the classic infantile form on one end and the more slowly progressive non-classic form on the other end. While being a hallmark in classic infantile Pompe disease, cardiac involvement in non-classic Pompe disease seems rare. Vascular abnormalities, such as aneurysms and arterial dolichoectasia, likely caused by glycogen accumulation in arterial walls, have been reported in non-classic Pompe patients. With this first systematic review on cardiovascular disease in non-classic Pompe disease, we aim to gain insight in the prevalence and etiology of cardiovascular disease in these patients. Forty-eight studies (eight case-control studies, 15 cohort studies and 25 case reports/series) were included. Fourteen studies reported cardiac findings, 25 studies described vascular findings, and nine studies reported both cardiac and vascular findings. Severe cardiac involvement in non-classic Pompe disease patients has rarely been reported, particularly in adult-onset patients carrying the common IVS1 mutation. There are indications that intracranial dolichoectasia and aneurysms are more prevalent in non-classic Pompe patients compared to the general population. To further investigate the prevalence of cardiovascular disease in non-classic Pompe patients, larger case-control studies that also study established cardiovascular risk factors should be conducted

Retrospective evaluation of the Dutch pre-newborn screening cohort for propionic acidemia and isolated methylmalonic acidemia: What to aim, expect, and evaluate from newborn screening?

Evidence for effectiveness of newborn screening (NBS) for propionic acidemia (PA) and isolated methylmalonic acidemia (MMA) is scarce. Prior to implementation in the Netherlands, we aim to estimate the expected health gain of NBS for PA and MMA. In this national retrospective cohort study, the clinical course of 76/83 Dutch PA and MMA patients, diagnosed between January 1979 and July 2019, was evaluated. Five clinical outcome parameters were defined: adverse outcome of the first symptomatic phase, frequency of acute metabolic decompensations (AMD), cognitive function, mitochondrial complications, and treatment-related complications. Outcomes of patients identified by family testing were compared with the outcomes of their index siblings. An adverse outcome due to the first symptomatic phase was recorded in 46% of the clinically diagnosed patients. Outcome of the first symptomatic phase was similar in 5/9 sibling pairs and better in 4/9 pairs. Based on the day of diagnosis of the clinically diagnosed patients and sibling pair analysis, a preliminary estimated reduction of adverse outcome due to the first symptomatic phase from 46% to 36%-38% was calculated. Among the sibling pairs, AMD frequency, cognitive function, mitochondrial, and treatment-related complications were comparable. These results suggest that the health gain of NBS for PA and MMA in overall outcome may be limited, as only a modest decrease of adverse outcomes due to the first symptomatic phase is expected. With current clinical practice, no reduced AMD frequency, improved cognitive function, or reduced frequency of mitochondrial or treatment-related complications can be expected

Distal muscle weakness is a common and early feature in long-term enzyme-treated classic infantile Pompe patients

Background: Enzyme replacement therapy (ERT; alglucosidase alfa) has improved the prospects for patients with classic infantile Pompe disease considerably. However, over time we noticed that many of these children exhibit distal muscle weakness at an early age, which is in contrast to the primarily proximal and axial muscle weakness in patients with late-onset Pompe disease. This was reason to study the prevalence and severity of distal muscle weakness, and the sequence of muscle involvement over time in patients that had learned to walk under ERT. Methods: In this prospective, single-center cohort study, we studied 16 classic infantile patients. We used video recordings that were made during regular standardized assessments to investigate distal muscle function (active dorsiflexion of the feet during walking; ability to use a pincer grasp/actively extend the fingers) and proximal muscle function (standing up from a supine position; raising the arms above the head). Results: Median age at start of ERT was 3.2 months (0.1–5.8 months), median age at study end was 5.6 years (2.9– 18.2 years). Six patients (6/16, 38%) initially had no evident signs of distal muscle weakness and developed a gait with active dorsiflexion of the feet. The other 10 patients never exhibited active dorsiflexion of the feet during walking. At study-end two patients showed no loss of distal muscle function. A subset of five patients (5/16, 31%) developed also weakness of the hands, particularly of the extensors of the 3rd and 4th digit. Conclusions: We found that the majority (14/16, 88%) of patients who had learned to walk exhibited distal muscle weakness of the lower extremities, while a subset (5/16, 31%) also developed weakness of the hands. The distal muscle weakness was often more serious than, and preceded the development of, the proximal muscle weakness