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Cappadocian fšáx 'child' and Pharasiot fšáxi 'boy' are traditionally derived from Turkish uşak, assuming a hitherto unexplained fricativization of [u] to [f] and of word‐final [k] to [x] after the borrowing process. The latter cannot be attributed to Cappadocian or Pharasiot, however, as it is a common feature of Anatolian Turkish. In order to understand the former sound change, we have to assume an isolated case of high vowel fricativization in the articulated plural ta ušáxja > ta fšáxja → sg. to fšáx(i) by metanalysis, comparable to the generally acknowledged case of ta otjá > ta utjá > ta ftjá → sg. to ftí 'the ear'. We argue that fšáx(i) is an archaism in light of the parallel use of uşak → ušák reported in nineteenth‐century sources and the preservation of word‐final [x] even in dialects which seem to have borrowed words ending in [k] from Standard instead of Anatolian Turkish. The irregular inflection of Cappadocian fšáx suggests that it was borrowed as an adjective from Old Anatolian Turkish before it was substantivized in Ottoman Turkish, perhaps even from Old Turkish uvşak 'little': ta ufšáxa ta peðjá 'the little children' > ta fšáxa 'the little ones' by apheresis. [ABSTRACT FROM AUTHOR]

Evidence-based medicine (EBM) is gaining importance in the current paediatric healthcare landscape. Improvement of paediatric health status is its major aim. However, for EBM to be successful, all stakeholders involved should understand what EBM really is, why and how EBM should or should not be practiced, and have the necessary skills to distinguish methodologically sound papers from biased opinion papers, and understand how and why guidelines are different from systematic reviews. Improving patient outcome requires attention to high-quality evidence and understanding of the processes of medical decision-making. Rigorous methodology is the cornerstone of guideline production, but in cases where quality evidence cannot be produced, as is often the case in paediatric nephrology because of low patient numbers, consensus-based guidance may be suitable to assist the practitioner at the bedside, as long as the underlying process is transparent. Most importantly, EBM should support patient involvement in a shared decision-making process. The more consistent and accurately predictable the effect of certain interventions is, clinically relevant to patients rather than affecting surrogate outcomes, and a priority for patients and other stakeholders, the more likely it is that adherence to the guidance provided will improve the outcome of patients. [ABSTRACT FROM AUTHOR]

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Purpose: Complement regulators control the activated complement system. Defects in this homeostasis can result in tissue damage and autoimmune diseases with a heterogeneity in clinical presentation. Complement factor I (FI), a serine protease, is an important regulator of alternative pathway activation. We report a diagnostic work-up of a patient with relapsing inflammatory mediated meningo-encephalitis. Our work-up revealed a rare genetic factor I (FI) deficiency. So far, all cases of reported complete factor I deficiency have absent serum levels of FI. We present here a unique case of a complete factor I deficiency based on a functional FI defect. Methods: Complement assays and measurement of FI activity were performed in the patient, her family, factor H-deficient patients, a patient with C3-nephritic factor and 11 healthy controls. Genetic sequencing of the FI coding regions in the patient and her parents was performed. Results: The patient had absent alternative pathway activity with low levels of C3 and normal serum level of FI. The patient’s plasma FI did not degrade C3b, with normalisation of C3b degradation after adding purified FI. Mutation analysis of the complement factor I gene revealed two heterozygous mutations (I322T and D506V). Conclusion: To our knowledge, this paper describes a complete FI deficiency caused by a defect of FI activity for the first time. Normal FI concentration does not exclude a complete FI defect, additional functional analysis of FI is required in any patient with a defect of complement activation. Recurrent aseptic meningo-encephalitis is a rare clinical presentation of complete FI deficiency. [ABSTRACT FROM AUTHOR]

This guideline for the investigation and initial treatment of atypical hemolytic uremic syndrome (HUS) is intended to offer an approach based on opinion, as evidence is lacking. It builds on the current ability to identify the etiology of specific diagnostic sub-groups of HUS. HUS in children is mostly due to infection, enterohemorrhagic Escherichia coli (EHEC), Shigella dysenteriae type 1 in some geographic regions, and invasive Streptococcus pneumoniae. These sub-groups are relatively straightforward to diagnose. Their management, which is outside the remit of this guideline, is related to control of infection where that is necessary and supportive measures for the anemia and acute renal failure. A thorough investigation of the remainder of childhood HUS cases, commonly referred to as “atypical” HUS, will reveal a risk factor for the syndrome in approximately 60% of cases. Disorders of complement regulation are, numerically, the most important. The outcome for children with atypical HUS is poor, and, because of the rarity of these disorders, clinical experience is scanty. Some cases of complement dysfunction appear to respond to plasma therapy. The therapeutic part of this guideline is the consensus of the contributing authors and is based on limited information from uncontrolled studies. The guideline proposes urgent and empirical plasmapheresis replacement with whole plasma fraction for the first month after diagnosis. This should only be undertaken in specialized pediatric nephrology centers where appropriate medical and nursing skills are available. The guideline includes defined terminology and audit points so that the early clinical effectiveness of the strategy can be evaluated. [ABSTRACT FROM AUTHOR]

Beecham Dugout is situated on the lower slopes of the Passchendaele Ridge, to the north-east of the Belgian town of Ypres (Ieper). Fears over the stability of the structure created the need for emergency archaeological investigations. The dugout is shallow (2 m deep) and was constructed in a basic 'T' shape, with accommodation for 66 men and four officers. It is cut within silty-sand levels of the Passchendaele Ridge. The dug-out was surveyed, recorded and removed. Archival studies, coupled with artefacts recovered, suggest that this is a dugout of German construction, but one that had been occupied by British troops after capture of the ridge in 1917. Prior to this discovery, the presence of German dugouts was largely unknown. [ABSTRACT FROM AUTHOR]

Background: Nocturia is common and associated with multiple disease states. Many potential mechanisms have been proposed for nocturia, which also remains challenging to manage. Purpose: To use multivariate analysis to determine which combinations of factors can accurately discriminate clinically significant nocturia in patients to facilitate clinical management and treatment decisions. Patients and methods: Data analysis was based on frequency volume charts from three randomized controlled trials. There were 1479 patients included, of which 215 patients had no/mild nocturia and 1264 had clinically significant nocturia with at least two voids per night. Factors studied that may influence nocturia were demographics, sleep duration, functional bladder capacity, 24 h urine volume and literature-suggested definitions of nocturnal polyuria. We used univariate analysis and cross-validated multivariate modelling to assess association between factors and nocturia status, redundancy between factors and whether the combined use of factors could explain patients′ nocturia status. Results: The multivariate analyses showed that the most useful definitions of nocturia are 'Nocturia Index' (NI) and 'Nocturnal Urine Production per hour' (NUPh) in combination with functional bladder capacity and sleep duration. Published definitions providing binary nocturnal polyuria outcomes had lower performance than continuous indices. These analyses also showed that NI was not specific to nocturnal polyuria as it also captured nocturia due to low functional bladder capacity. By contrast, NUPh was demonstrated to be specific to nocturnal polyuria. Conclusion: NUPh has previously been shown among elderly males to be essential in nocturia and a very valid measure of nocturnal polyuria. However, the current, large and independent dataset now confirms that it can be applied in an adult population with a complaint of nocturia covering both males and females. [ABSTRACT FROM AUTHOR]