The Operative Incidence of Syndactyly in Northern Ireland. A 10-Year Review.

BACKGROUND: Syndactyly is a common congenital condition that can present sporadically or in relation to an underlying genetic condition. Little contemporary published data exists detailing specific rates of presentation and surgical intervention, especially in Western European population. This is the first published review of operative intervention rates for the condition over time in Northern Ireland. METHODS: A ten-year retrospective review of electronic operative records from January 2007 - October 2017 was carried out within Northern Ireland's regional tertiary centre Royal Belfast Hospital for Sick Children (RBHSC). All congenital hand surgery in the country was performed here during the period reviewed, by a single surgeon. Patient age at surgical intervention, their sex, digits involved and clinical grade of syndactyly was recorded. RESULTS: One hundred and twenty four cases were returned following the review. On individual analysis 22 cases were excluded as they were not primary congenital syndactyly. The remaining 102 cases were all Caucasian. Six cases were toe syndactyly while 96 cases involved the upper limb digits. The group consisted of 70 males and 32 female infants. Age range at time of surgical intervention was 8 months to 14 years with a median age of 26 months. For clinical grade of upper limb syndactyly; 35 cases in the data set were classed as simple incomplete, 34 cases as simple complete, 17 as complex and 5 cases as complicated syndactyly. The remaining 5 cases lacked clear documentation. The most common site of syndactyly was between the ring and middle finger (40/102). Annual frequency of operative intervention has trended upwards in the period studied. CONCLUSION: This case review adds epidemiological data on the operative incidence of syndactyly cases in Northern Ireland - a relatively isolated genetic population. Overall rates of incidence have increased over the past 10 years. It remains unclear if this is due to new environmental influences on the developing population or increased referral for surgical intervention over time.Levels of evidence - IV (Case Series).

Early clinical experiences with nintedanib in three UK tertiary interstitial lung disease centres.

BACKGROUND: Nintedanib has been shown to slow disease progression in patients with idiopathic pulmonary fibrosis (IPF). It was approved by the National Institute for Health and Care Excellence (NICE) in January 2016 for IPF patients with a forced vital capacity (FVC) of 50-80% in the United Kingdom (UK). AIM: To report real world data about our early clinical experience using nintedanib in 187 patients with a multi-disciplinary (MDT) diagnosis of IPF in a manufacturer funded patient in need scheme (three UK centres) prior to NICE approval. METHODS: All patients with a MDT diagnosis of IPF from December 2014 to January 2016 commenced on nintedanib were included. Demographic details, adverse events (AEs) and where available lung function results were retrospectively collected from clinical letters. RESULTS: 187 patients (76% males) with a median age of 72 years (49-89) were treated with nintedanib. The average pre-treatment FVC was 81.1 ± 19.8% and diffusion capacity of the lungs for carbon monoxide was 43.9 ± 15% (n = 82). Fifty percent of patients started nintedanib because they were ineligible for pirfenidone due to an FVC > 80%. The median treatment course was 8 ± 4 months. The majority of patients experienced 1-3 AEs with nintedanib (52%, n = 97). The most frequent AEs were diarrhoea (50%), nausea (36%), reduced appetite (24%), tiredness (20%) and gastro-oesophageal reflux (18%). The majority of AEs resulted in no change in treatment (64%, n = 461). 21% (n = 150) of AEs resulted in a dose reduction and 13% (n = 94) necessitated discontinuation of treatment. 1 in 5 patients discontinued treatment either temporarily or on a permanent basis during the monitoring period. In a select cohort of patients, a statistically significant greater proportion of patients remained stable or improved and a lower proportion declined, as depicted by FVC changes of > 5% after nintedanib commencement (P < 0.05 using Chi squared test). CONCLUSIONS: Nintedanib is well tolerated and has an acceptable safety profile. Only 8% of those reporting diarrhoea discontinued treatment either on a temporary or permanent basis. There were no signals with respect to increased cardiovascular morbidity or major bleeding risk. This is in keeping with the INPULSIS clinical trial findings but in a real world cohort.

Cardiac involvement in Henoch-Schönlein purpura.

Cardiac involvement is not regarded as a feature of Henoch-Schönlein purpura (HSP). We report a patient with HSP who developed significant cardiac symptoms, which resolved with immunosuppression. We review previous cases of HSP with cardiac features, none of which showed a response to treatment, and discuss evidence that cardiac involvement is more common than recognized. The implications for investigation and treatment of this condition are discussed.

Intensive bedside teaching of physical examination to medical undergraduates: evaluation including the effect of group size.

The effectiveness of intensive bedside teaching of techniques of physical examination of the alimentary system to students commencing their clinical training was assessed. All students viewed a videotape demonstrating these techniques and then participated in a morning practice examination session but only half of the class received afternoon bedside teaching of physical examination in addition. Teaching in history taking was used as a 'placebo' for the control group, and half the students in each of the study and control groups were taught in units of four, the other half in units of eight. Thus the effect of group size could also be estimated. After 1 week, the students' performance of a physical examination was assessed using a standardized scoring chart which demonstrated good reliability. The study group performed significantly better than the control and within the study group there was a trend for the students taught in smaller units to perform better.

Videotape demonstration of physical examination: evaluation of its use in medical undergraduate teaching.

A videotape demonstration of the physical examination of the alimentary system was used, in place of a traditional lecture, in the first week of a teaching programme for half of a group of commencing, clinical students. At the end of the week, these students obtained higher scores than the matched controls in a clinical assessment which utilized an objective scoring-chart especially for this study.

Retrospective analysis of 158 cases of adult acute leukaemia: factors influencing prognosis and treatment response.

Discriminant and regression analyses were employed to determine the influence of a large number of clinical and laboratory indices on outcome of treatment in a series of 158 adults presenting with acute leukaemia between 1970 and 1977. Induction therapy had been most commonly cytosine arabinoside plus daunorubicin, vincristine and prednisolone. This induced complete remission in 42% of non-lymphoblastic, and in 58% of lymphoblastic plus undifferentiated leukaemias. Some induction failures who had non-lymphoblastic leukaemia were treated with cytosine arabinoside plus 6-thioguanine, which induced remissions in 46%. Advanced age was the factor most strongly associated with failure either to achieve or to sustain complete remission. Thrombocytopenia, promyelocytic leukaemia, high percentage of marrow blasts, and absence of metaphases in marrow cytogenetic preparations were also associated with poor survival. A number of other factors which appeared to be associated with poor prognosis were found by the analysis to lack significance as independent variables.

Heparin-associated thrombocytopenia: case report and prospective study.

After observing a patient with heparin-induced thrombocytopenia we prospectively recorded the incidence of thrombocytopenia associated with heparin treatment by measuring the platelet count every second day in 166 patients given therapeutic heparin for various thromboembolic disorders, and 51 patients given low-dose heparin prophylaxis. A platelet count below 100 x 10(9)/litre developed in nine patients (5.4%) during or soon after full-dose heparin therapy, and in one patient given low-dose heparin. Careful clinical review suggested that heparin was either the most likely cause of a contributing cause of thrombocytopenia in 5/166 patients (3.0%) receiving therapeutic heparin and none of the patients who received prophylactic heparin. Associated laboratory studies suggest that heparin-initiated platelet aggregation in vivo is a useful marker for heparin-induced thrombocytopenia.

Detection of deep venous thrombosis by scanning of 99mtechnetium-labelled red-cell venous pool.

A comparative study of 32 patients with suspected deep venous thrombosis was carried out using blood-pool radionuclide scanning and conventional x-ray phlebography. Results of the two methods showed close agreement, the sensitivity (positive correlation) of the scan being 100% and its specificity 89%. We conclude that a patient's red cells labelled with 99mtechnetium (99mTc) provide an excellent medium for this type of scanning. The technique has particular advantages in visualising the whole venous system, giving a persisting image, and obviating the need to inject into a vein of the affected limb. In view of the inherent disadvantages of contrast phlebography, 99mTc-red-cell scanning is clearly an acceptable alternative.