Application of UKHCDO 2004 guidelines in type 1 von Willebrand Disease ‐ a single centre paediatric experience of the implications of altered or removed diagnosis

Summary. Diagnosis of type I von Willebrand Disease (VWD) can be challenging. In 2004, the United Kingdom Haemophilia Centre Doctors’ Organisation (UKHCDO) proposed more stringent diagnostic criteria to replace the 1995 guidelines. To determine the true number of cases of type 1 VWD in a single paediatric centre, the 2004 UKHCDO Guideline for the diagnosis of VWD was used to evaluate 114 patients on our type 1 VWD register. Clinical and laboratory data were collected and analysed to see whether they met the criteria for type 1 VWD. Only 8% remained on the type 1 VWD register. 18% have been classified as ‘possible type 1 VWD’. Twenty five surgical procedures have since been performed on patients from the group in which the diagnosis was removed without any haemostatic support or bleeding complications. Reaction to the removal of the VWD diagnosis or delivery of an alternative diagnosis was positive for most patients and families. This study is the first to assess the impact of the 2004 UKHCDO Guidelines on the diagnosis of VWD. It provides evidence that the prevalence of type 1 VWD may actually be closer to that of haemophilia instead of the previously reported 1–3% of the general population. We propose that all centres should review their patients with a diagnosis of VWD to revalidate this disease that claims to be our most common inherited bleeding disorder.     

Intercostal nerve blockade versus thoracic epidural analgesia for post thoracotomy pain relief

Background Post thoractomy pain is a major source of concern in the postoperative period. The purpose of this study was to evaluate the effectiveness of intraoperative temporary intercostal nerve blockade versus thoracic epidural analgesia for control of post thoracotomy pain. Methods 40 patients undergoing elective pulmonary resection through a postero lateral thoractomy were randomly allocated to receive epidural analgesia using 0.25% bupivicaine (Group A, n=20) or temporary intercostal nerve blockade using 0.25% bupivicaine (Group B, n=20). Adequacy of analgesia was assessed over a period of 24 hours using a visual analogue score and an observer verbal ranking scale. Results Pain scores were similar in both the groups for the first 4 hours after surgery. Thereafter, the pain scores were significantly higher (p<0.05) in Group B as compared to Group A for the remainder of the observation period. There was significantly higher (p<0.01) usage, of nonsteroidal analgesic consumption in Group B. No neurological complications were encountered, in both the study groups. Conclusion We conclude that in the early postoperative period there is no significant difference in pain relief in both the techniques but there after, epidural analgesia significantly reduces post thoracotomy pain.     

Thoracic epidural infusions for post thoracotomy pain relief: a clinical study to compare the efficacy of fentanyl — bupivicaine mixtures versus fentanyl alone

Background: Optimum pain relief following thoracotomy is essential for patient comfort and to reduce the incidence of postoperative pulmonary complications. Methods: A randomized clinical trial was conducted on 90 patients scheduled for pulmonary resection. The patients were randomly divided into three groups. Group 1 received 0.125% bupivicaine with fentanyl 10μg.ml⁻¹, Group 2 received 0.25% bupivicaine with fentanyl 10μg.ml⁻¹ and Group 3 received only fentanyl 10μg.ml⁻¹ in a calculated dose as a continuous thoracic epidural infusion. Adequacy of anglesia was assessed at rest and during movement over 24 hours. Analgesic efficacy was assessed using a visual analogue score and an observer verbal ranking scale. Results: Pain scores were significantly higher in Group 3 during the assessment period. (p<0.01) as compared to the other groups. The use of intraoperative vasopressors was significantly higher (p<0.05) in Group 2 as compared to the other groups. No neurological complications were encountered in any of the study groups. Conclusion: We conclude that in the early postoperative period, the use of 0.125% bupivicaine improves fentanyl epidural analgesia in patients undergoing lung resection.     

Impact of Body Mass Index on Left Ventricular Function

Background

Obesity is associated with increased cardiovascular morbidity and mortality. A direct effect of isolated obesity on cardiac function is not well established. The study was designed to determine the direct effect of various grades of isolated obesity on echocardiographic indices of systolic and diastolic left ventricular function.

Methods

Fifty one obese and 25 normal weight, serving personnel without any other pathological condition were studied. Group I (n=25) consisted of subjects with normal weight and body mass index (BMI <25kg/m²), Group II (n=34) of overweight subjects (BMI 25-29.9 kg/m²) and Group III (n=17) of obese subjects (BMI >30 kg/m²). Echocardiographic indices of systolic and diastolic function were obtained and dysfunction was assumed when at least two values differed by ≥ 2 SD from the normal weight group.

Result

Ejection fraction, fractional shortening were increased (p<0.05) in Group II and III. Left ventricular dimensions were increased (p< 0.001) but relative wall thickness was unchanged. Systolic dysfunction was not observed in any of the obese patients. The mitral valve pressure half time (p< 0.01), left atrial diameter (p < 0.01) and the deceleration time were increased (p< 0.01) in obese subjects, while other diastolic variables were unchanged. No difference were found between obesity subgroups. Subclinical diastolic dysfunction was more prevalent among obese subjects. BMI correlated significantly with indices of left ventricular systolic and diastolic function.

Conclusion

Subclinical left ventricular diastolic dysfunction was noted in all grades of obesity which correlates with BMI.Key Words: Obesity, Systolic function, Diastolic function, Echocardiography