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  1. Nick Brown, Editor in Chief1,2,3
  1. 1 Department of Women’s and Children’s Health, International Maternal and Child Health (IMCH), Uppsala University, Uppsala, Sweden
  2. 2 Department of Paediatrics, Länssjukhuset Gävle-Sandviken, Gävle, Sweden
  3. 3 Department of Child Health, Aga Khan University, Karachi, Pakistan
  1. Correspondence to Dr Nick Brown, Department of Women’s and Children’s Health, International Maternal and Child Health (IMCH), Uppsala University, Uppsala, Sweden; nickjwbrown{at}gmail.com

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Reining in—October 2023

The hazel door swung ajar onto the penumbrally-lit corridor, the guttural scream of the hinges piercing the silence. The line of mute, quietly agitated applicants for the post uncomfortably positioned on the hard, lime green plastic chairs, reminiscent of museum pieces of 1960s furnishing, their survival having been secured wholly through their durability, providing the institution with a perfect excuse to ‘save resources for priority areas’.

“Please come in, doctor.” Grateful for the cursory introduction to the panel (mouth so dry as to glue tongue to palate) the interrogation began. The routine questions about qualities of a good doctor, lessons learnt, communication hurdles with the aplomb (a half smile maybe) hoped for given the advance rote learning. The benign-looking eminence-grise was the last to speak: the theme, research. In truth, the candidate would have acknowledged privately that the few citable case series perhaps weren’t world changing, but, the inner voice, somehow, took over. “This manuscript has already changed the way our specialty approaches the acutely unwell…” It’s as if someone else was doing the talking, the aspirant realising (but unable to stop) the harm the trumpet blowing, unfettered inner-self was causing.

What I love about this month’s selection is not just their ingenuity, but their modesty. A wonderful combination.

Spinal muscular atrophy: communication in the Smn1 modifier era

Though we now have a broad feel for the likely motor effect of the gene-modifying-therapy in SMA (both those diagnosed clinically and by newborn screening (NBS)), communication skills have been more under the radar. Difficulty with phonation (part coordination of oropharyngeal movement, part strength dependent) despite good comprehension is part of the classic phenotype, but harder to measure than the standard motor milestones on which most pre and post nusinersen and onasemnogene literature has been based. Biancha Buchignani at the University of Pisa, Italy and colleagues in multiple other centres (Rome, Florence, Milan, Genoa) take a closer look. Using the Bates Macarthur Developmental Communication Inventory (which quantifies comprehension, lexical understanding and gestural and expressive vocabulary skills) they tested changes from 8 to 18 months and 24–36 months. The majority of the clinically detected SMA group developed recognisable language, though unsurprisingly, lexical abilities were largely under the fifth centile. Perhaps intuitively, the communication development crudely paralleled motor progress, those with more severe movement restriction making the least vocal improvement. The smaller NBS group (many symptom free at diagnosis) performed better at baseline, though numbered too few to draw longer term inferences. See page 395

The operation went well…

I think it’s fair to say we all make assumptions about families’ priorities in terms of treatment. What we consider success, might be viewed as failure by parents… and vice versa. Oliver Rivero-Arias (Nuffield department of population health) and colleagues quantify the inherently hard-to-quantify using a normalised importance value of attribute in terms of operative results from the viewpoint of parents, public opinion and medical personnel. Using a complex weighting system, survival and quality of life were the highest rated priorities. Taking another angle, Bruce Jaffray’s (Great North Children’s Hospital, Newcastle, UK) editorial adds another nuance. If the reality that there is no guarantee that one procedure is curative then, is the handling of any complications (inevitable in a certain proportion) as good a measure? See pages 377 and 357

Blowing in the wind

We know the flags: the fragile asthmatic with recurrent acute admission; the high beta agonist to inhaled steroid ratio. Two papers (Robert Becket and colleagues and Prasad Nagakumar and colleague) flag the co-related issues of overestimating residual salbutamol content or earlier than necessarily disposal of still-usable MDI canisters. These include inhalation of the chloroflourocarbon based repellent (close to 90 bronchodilator effect free’ doses ’possible) or discarding the pollutant containers in the already overburdened environment in the devices without residual dose metres. The solution, so simple, so innovative is the use of the ubiquitous home digital weighing scales to assess the number of active doses left in the cylinder. In short, brilliant. See pages 444 and 446

In plain view

In non-acutely unwell children, a skeletal survey is pretty much a routine procedure as part of the assessment of an unsettling injury pattern including bruising and fracture phenotypes. A study within 72 hours has long been the standard recommendation. Rather ironically, though that many of these children require PICU level care, undertaking this forensic investigation on an immobile, ventilated child is more than a minor logistical problem. Gerard Manning (Great Ormond Street Hospital, London) and colleagues present findings from a national intensive care unit survey on radiology practice. See page 444

Reining in? November 2023

Having been distracted by and submerged in the autumn respiratory wave, the anonymous looking email appeared initially innocuous. In it, though, was the panel verdict—perfunctory, blunt: ‘clearly very competent, but behaves as if a recent Nobel winner—something to discuss with tutor.’

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Footnotes

  • Contributors -

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Commissioned; internally peer reviewed.

Linked Articles

  • Original research
    Oliver Rivero-Arias John Buckell Marian Knight B M Craig Rema Ramakrishnan Simon Kenny Benjamin Allin on behalf of the CSOR Collaborative Group Benjamin Allin Goher Ayman Timothy Bradnock Kirsty Brennan John Buckell Stefano Giuliani Claudia Grimaldo-Giraldo Nigel J Hall Lisa Hinton Crispin Jenkinson Ingo Jester Simon Kenny Marian Knight Karolina Kuberska Kokila Lakhoo Nick Lansdale Anna May Long Alexander Macdonald Charles Opondo Rema Ramakrishnan Oliver Rivero-Arias Joanne Shepherd Gregor Walker
  • Editorial
    Bruce Jaffray
  • PostScript
    Robert S Beckett Annapurna Jagadish Will Carroll Francis J Gilchrist
  • Original research
    Bianca Buchignani Gianpaolo Cicala Francesca Cumbo Martina Ricci Anna Capasso Chiara Ticci Sara Mazzanti Noemi Brolatti Michele Tosi Claudia Dosi Laura Antonaci Giorgia Coratti Maria Carmela Pera Daniela Leone Concetta Palermo Beatrice Berti Anna Lia Frongia Michele Sacchini Claudio Bruno Riccardo Masson Adele D’Amico Roberta Battini Marika Pane Eugenio Mercuri
  • PostScript
    Prasad Nagakumar Teresa Evans Susan Frost