Telemedicine and cystic fibrosis: Do we still need face-to-face clinics?

There has been growing interest in telemedicine for cystic fibrosis over recent years based largely on convenience for patients and/or increasing the frequency of surveillance and early detection which, it is assumed, could improve treatment outcomes. During 2020, the covid-19 pandemic catalysed the pace of development of this field, as CF patients were presumed to be at high risk of infection. Most clinics adapted to digital platforms with provision of lung function monitoring and sample collection systems. Here, we present the views of multidisciplinary team members at a large paediatric CF centre on what has worked well and what requires further optimisation in the future. In response to the question posed, 'Do we still need face to face clinics?' our answer is 'Yes, but not every time, and not for everyone'.

Communicating cystic fibrosis newborn screening results to parents.

The way results of cystic fibrosis (CF) newborn screening are communicated to parents is critical yet is done differently across the globe. We surveyed parents of 101 children in our tertiary London paediatric centre with a 48% response rate. Parental responses were as follows: 40/42 (95%) said the information could not have been given over the phone and 39/43 (91%) said they wanted both partners present; 27/42 (64%) said it was helpful having the health visitor also present; and 37/40 (92%) felt it was acceptable to wait until the next day for the sweat test. We have reduced the time from first contact to arriving in the home to 2-3 h.Conclusion: We believe that this survey backs up our approach of a home visit by a CF nurse specialist with the family's health visitor to break the news. This is challenging in the current COVID-19 pandemic. What is Known: • Breaking bad news can have a lasting impact on parents when not done the right way. • Giving results of cystic fibrosis (CF) newborn screening is done differently within the UK and around the world. What is New: • Our parental survey revealed that the majority (92%) believed this should be done face to face and not over the telephone. • There was a mixed response to whether the parents should be told the genotype (assuming the CF centre knew), and thus the CF diagnosis before the confirmatory sweat test was carried out.

Surgical treatment of femoral diaphyseal fractures in children using elastic stable intramedullary nailing by open reduction at Yopougon Teaching Hospital.

INTRODUCTION: Elastic stable intramedullary nailing (ESIN) has transformed children's femoral shaft fracture treatment, but this technique requires an image intensifier. Without it, open reduction is used to check fracture reduction and pin passage. The aim of this study was to describe our techniques and to evaluate our results at the middle term. HYPOTHESIS: The open reduction and ESIN technique provides satisfactory results with few major complications. PATIENTS AND METHODS: This was a retrospective study that focused on femoral diaphyseal fractures treated in the pediatric surgery unit at Yopougon Teaching Hospital (Abidjan, Côte d'Ivoire) between January 2007 and December 2013. Twenty children older than 6 years of age who underwent open reduction and ESIN without image intensifier assistance were included. Functional outcomes were assessed using Flynn's criteria. Postoperative complications and sequelae were recorded. RESULTS: At the 16-month follow-up, the results were excellent in 11 (55%) cases, good in eight (40%), and poor in one (5%) case. The mean duration of surgery was 71min (range, 57-103 min). The mean time for bone healing was 11.6 weeks (range, 7-15 weeks) and the average time to nail removal was 6 months. Complications included wood infection (n=3), skin irritation (n=3), knee stiffness (n=2), malunion (n=3), scar (n=5), and leg length discrepancy (n=3). DISCUSSION: Open reduction and ESIN yielded satisfactory results with few major complications. This method could be an alternative in low-income countries where the image intensifier is often unavailable. LEVEL OF EVIDENCE: Level IV retrospective study.

Surgery for children with Crohn's disease: indications, complications and outcome.

BACKGROUND: Outcome data for surgery in paediatric Crohn's disease are limited. We report 10 years' experience at a regional paediatric gastroenterology centre. METHODS: Children undergoing surgery for Crohn's disease (January 2002-December 2012) were identified from an onsite patient register. Patients were followed until transition to adult services. Data were obtained from medical records and are expressed as median (range). RESULTS: Sixty-nine children, aged 13.8 years (6.3-17.0) at diagnosis, were included. 42 were male (61%). Follow-up was 1.8 years (27 days-6 years). Surgery followed diagnosis by 9 months (0 days-7 years). 52 children (75%) received thiopurines and 5 (7%) anti-TNF (tumour necrosis factor) therapy preoperatively. 58 (84%) underwent intra-abdominal surgery (40 right hemicolectomy, 8 stoma formation, 2 subtotal colectomy, 2 small bowel resection, 6 other) and 10 (14%) underwent surgery for perianal disease. The commonest indications for intra-abdominal surgery were stricturing disease 35 (60%) and unresponsive luminal disease 13 (22%). There were 13 (22%) early, and 5 (8.6%) late, complications following intra-abdominal surgery. Nine children had disease relapse, five required further surgery. Height SD scores (SDS) did not increase between diagnosis; -0.5 (-3.4-2.1) and most recent follow up; -0.4 (-3.0-1.1). Body Mass Index (BMI) SDS increased from -1.0 (-6.3-1.5) to -0.3 (-3.3-2.0) (p<0.05). CONCLUSIONS: Surgery was associated with a 22% early complication rate and a 15% risk of relapse. 21% of patients required a second unplanned intra-abdominal procedure. Surgical intervention was associated with an increase in BMI SDS, but not in height SDS.

Delayed treatment of supracondylar elbow fractures in children.

BACKGROUND: Supracondylar fractures are the most common elbow fractures in children and are usually treated on an emergency basis, using percutaneous pinning. However, the treatment is often delayed in areas where healthcare resources are scarce. HYPOTHESIS: Delaying treatment does not influence the perioperative complication rate. MATERIALS AND METHOD: We retrospectively reviewed the medical charts of 89 children aged 2 to 15 years in whom surgery for extension-type supracondylar elbow fractures was delayed by more than 48 hours. The 53 boys and 36 girls with a mean age of 6 years 9 months had severe fracture displacement (28 stage III and 61 stage IV according to Lagrange and Rigault classification scheme). Mean time to treatment was 4.5 days (range: 2-17 days). Open reduction and crossed K-wire fixation via the posterior approach were performed in all 89 patients. Postoperative complications and sequelae were collected. Functional outcomes were evaluated using Flynn's criteria. RESULTS: Outcomes were satisfactory in 74 (83.2%) of patients. Postoperative complications occurred in 13 (14.6%) patients and consisted of surgical site infection (n=7, 7.8%), iatrogenic nerve injury (n=3, 3.4%), and reoperation (n=3, 3.4%). At last follow-up after a mean of 5 months, three (3.4%) patients had cubitus varus and one had a recurrent fracture due to massaging. Elbow motion was limited in 11 (12.4%) patients. No case of compartment syndrome was recorded. DISCUSSION: Despite an average time to surgery of 4.5 days, the outcome was satisfactory in 83% of cases. Delayed treatment was not associated with an increased rate of perioperative complications. LEVEL OF EVIDENCE: Level IV, retrospective study.

Elastic stable intramedullary nailing in paediatric traumatology at Yopougon Teaching Hospital (Abidjan).

CONTEXT: Elastic stable intramedullary nailing has revolutionised the treatment of fractures in children. AIM: To report our experience with this current technique of management of fractures in children. METHODS AND MATERIALS: A retrospective study of all children with fractures treated by this method from November 2003 to June 2006 at the Paediatric Surgery Department of Yopougon Teaching Hospital, Abidjan, Côte d'Ivoire. Data were recorded from their medical charts regarding demographics, fracture patterns, associated injuries, morbidity, and outcome. RESULTS: A total of 38 children (14 boys; mean age, 11.7 years; range, 9-15 years) with 41 fractures, sited at femur (n=15), humerus (n=8), tibia and fibula (n=6), forearm (n=7), and radial neck (n=5), associated with other injuries in 7 cases, were enrolled in the study. The mean interval for surgery was 6.8 days. Open reduction was required 5 times (12.1%). The hospital stay was in the range 5-12 days. Minor adverse events were 1 (2.4%) early tibial loss of reduction, 3 (7.3%) skin irritations and 2 (4.8%) transient knee limitations. Complications (17.3%) included 2 axial (4.8%) and 1 rotational (2.4%) femoral malunions below 15°; 3 elbow (7.3%) and 1 knee stiffness (2.4%) in openly reduced cases. Solid consolidation was achieved in all cases, with no disturbance in standard union times. At a mean follow-up period of 16 months (range, 9-28 months), all but 3 patients with opened joints recovered full range of motion, and none showed either limp and gait anomalies or re-fracture after removal of wires. CONCLUSION: Elastic stable intramedullary nailing in children fractures is easy to perform and has little complications if a proper technique is used.

Operative treatment of proximal humeral fractures in children: indications and results.

BACKGROUND: In most children proximal humeral fractures are treated non-operatively with generally good results. This review discusses the indications of operative treatment and assesses the treatment results. MATERIALS AND METHODS: The charts of 20 patients (14 girls; mean age: 12.3+/- 2.8 years; range: 7-16 years) with proximal humeral fractures who were operated on at our institution were reviewed from 1992 to 2002. RESULTS: There were five metaphyseal fractures and 15 physeal injuries which were angulated according to Neer-Horowitz score as grade III in four cases and grade IV in 16 cases with a mean angulation of 47.8+/-39.1 degrees (range: 6-148 degrees). Associated lesions comprised open fracture and head trauma in two cases each. Patients with associated injuries were operated on primarily and the 16 others by secondary intention. All but one were reduced via an anterior approach with internal fixation. They were assessed for clinical and radiological healing at a mean follow up of 3.6 years ranging from 1.2 to 7.8 years. CONCLUSION: Based on our study, surgical option is indicated for severely displaced and unstable fractures in older children and adolescents.

LFA-1 binding site in ICAM-3 contains a conserved motif and non-contiguous amino acids.

The intercellular adhesion molecule-3 (ICAM-3) is a counter receptor for the integrin LFA-1 that supports cell-cell adhesion dependent functions. ICAM-3 is a member of the immunoglobulin superfamily possessing five immunoglobulin-like domains. Here, we characterize the overall shape of ICAM-3 and the amino acid residues involved in binding LFA-1 and monoclonal antibodies (Mab). Electron microscopic observations show that ICAM-3 is predominantly a straight rod of 15 nm in length, suggesting a head to tail arrangement of the immunoglobulin-like domains. Six out of nine ICAM-3 Mab described blocked the interaction with LFA-1 to varying degrees. Domain assignment of blocking Mab epitopes and characterization of LFA-1-dependent cell adhesion to ICAM-3 mutants demonstrate that the amino-terminal domain of ICAM-3 interacts with LFA-1. A conserved amino acid motif including residues E37 and T38 form an integrin binding site (IBS) in ICAM-3. This motif has also been shown to function as an IBS in ICAM-3 and VCAM-1 and hence many form a common site of contact in all CAMs of this type. Other ICAM-3 residues critical to adhesive interactions, such as Q75, conserved in ICAM-1 and ICAM-2, but not VCAM-1, may confer specificity to LFA-1 binding. This residue, Q75, is predicted to locate in a model of ICAM-3 to the same site as RGD in the immunoglobulin-like domain of fibronectin that binds several integrins. This suggest an evolutionary relationship between ICAMs and fibronectin interactions with integrins.

Chromatic aberration and accommodation: their role in emmetropization in the chick.

The roles of chromatic aberration and accommodation as cues to emmetropization in the chick were investigated. Myopia was induced monocularly by lid suture for a period of 1-2 weeks from hatching, after which eyes were reopened and the recovery process followed. Monochromatic light (ML) rearing conditions and ciliary nerve section surgery were used to eliminate chromatic aberration and accommodative activity respectively. Control animals were reared in white light (WL). When accommodation was left intact, chickens reared under monochromatic light were able to recover normally. However, ciliary nerve section produced hyperopia, deepening of the anterior chamber and a tendency towards axial lens thinning, irrespective of the light conditions used. Hyperopic refractive errors peaked at 4 weeks (mean refractive errors: +5.7 D, +4.21 D for ML, WL groups respectively, 4 weeks), with the ML group still exhibiting significant hyperopia at 7 weeks. Ciliary nerve section did not prevent the myopic response to lid suture (mean refractive errors: -22.65 D; -25 D for ML, WL groups respectively, 1 week) nor the elimination of myopia when eyes were reopened. These data indicate that neither accommodation nor chromatic aberration are fundamental to the gross operation of the emmetropization process although they may be essential for the fine tuning of refraction.

Selection and characterization of a variant of human melanoma cell line, A375 resistant to growth inhibitory effects of oncostatin M (OM): coresistant to interleukin 6 (IL-6).

Human melanoma cell line A375 is extremely sensitive to growth inhibitory effects of oncostatin M (OM). A375 cells resistant to the antiproliferative effect of OM were isolated by exposing OM-sensitive cells to ethyl methane sulfonate (EMS) for 24 h followed by continuous exposure to OM. An A375 subline resistant to OM-induced growth inhibition was selected by a limiting dilution technique and designated 4-1.10". The resistant cells were completely refractory to OM even up to a concentration of 500 ng/ml. Interestingly, the resistant cells were also nonresponsive to the growth inhibitory effects of interleukin-6 (IL-6). Other cytokines such as transforming growth factor beta (TGF-beta), interleukin-1 (IL-1), and tumor necrosis factor alpha and beta (TNF-alpha and TNF-beta) exhibited similar growth inhibitory effects on OM-sensitive or -resistant cells. OM-resistant cells were found to possess approximately 20% of OM receptors with the same affinities as compared to the parental OM-sensitive cells. However, the affinities and number of receptors for IL-6 were the same on both cell types. The OM treatment did not alter the cyclic AMP (cAMP) level of either the parental or the resistant cells. The OM-resistant cell line will be very useful in elucidating the mechanism of OM-elicited growth inhibition.

Amphiregulin-associated protein: complete amino acid sequence of a protein produced by the 12-0-tetradecanoylphorbol-13-acetate-treated human breast adenocarcinoma cell line MCF-7.

Amphiregulin-associated protein (ARAP) was purified from serum-free conditioned medium of MCF-7, human breast carcinoma cells, treated with 12-0-tetradecanoylphorbol-13-acetate (TPA). ARAP is a single-chain, extremely hydrophilic, heparin-binding protein. Its apparent molecular weight is approximately 21,500 as assessed by gel chromatography and approximately 15,500 as determined by polyacrylamide gel electrophoresis. The complete amino acid sequence of ARAP was determined. The larger form contains 123 amino acids, whereas a shorter form is missing two amino acids at the amino-terminal. ARAP contains 10 cysteines and 30 basic amino acids (23 lysines and 7 arginines). ARP sequence has been found to be identical to protein encoded by human MK gene.