Valvular heart disease in Antiphospholipid antibody syndrome: Isolated Tricuspid stenosis

Antiphospholipid antibody syndrome (APLS) is a rare disorder characterized by a hypercoagulable state. Manifestations include arterial or venous thrombosis, recurrent fetal wastage, coronary artery disease, valvular heart disease, dilated cardiomyopathy, pulmonary artery hypertension, and intracardiac thrombus. Most commonly mitral valve is affected followed by aortic and then tricuspid valve. In this report, a rare case of spontaneous aortic thrombosis with tricuspid stenosis uncomplicated by other valve lesions is presented with clinical and echocardiographic studies and computed tomographic images.     

Widespread cutaneous necrosis associated with antiphospholipid syndrome

Summary Widespread cutaneous necrosis as the catastrophic manifestation in antiphospholipid syndrome (APLS) is rather uncommon. Even in the few documented cases, the extensive skin necrosis lesions have been stabilized and regressed with eventual healing utilizing pulse steroid therapy alone or in conjunction with further treatment with plasmapheresis.We describe one case of APLS associated with lupus-like disease. The young female suffered from widespread cutaneous necrosis of the lower legs and gangrene change in the digital end of hands and feet. This condition necessitated amputation of major lower extremities and digital amputation of both hands.     

Prevalence and reversibility of pulmonary dysfunction in refractory systemic lupus: improvement correlates with disease remission following hematopoietic stem cell transplantation

AIM: To report the prevalence and reversibility of pulmonary function test (PFT) abnormalities among systemic lupus erythematosus (SLE) patients, refractory to therapy, undergoing hematopoietic stem cell transplantation (HSCT). METHODS: Thirty-four SLE patients received 200 mg/kg cyclophosphamide and 90 mg/kg equine antithymocyte globulin followed by HSCT. PFTs were performed prior to, at 6 months, and yearly following HSCT. RESULTS: The prevalence of significant PFT abnormalities was high (97%). Low FEV(1) and FVC occurred in 26 of 34 patients (76%). A significant abnormality in diffusion capacity of the lung for carbon monoxide (Dlco) occurred in 26 of 32 individuals able to complete Dlco testing (81%). Dlco 18 months after HSCT. Five of 28 patients had a normal entry FVC; for each, the FVC remains normal. Of the 23 patients with an abnormal baseline FVC, 18 have improved, 15 completely and 3 partially. Eight of these 18 patients also have improved Dlco. The two patients with a diagnosis of SLS and one patient with SLE-related pulmonary hypertension improved in both parameters. Only 5 of 23 patients with an abnormal FVC did not improve. Each of these five patients retained active lupus in spite of HSCT. CONCLUSION: The prevalence of lung impairment among SLE patients requiring long-term immune suppression is high. Following HSCT, pulmonary impairments can improve, which is sustained if disease control is sustained.     

Resuscitation competencies in paediatric specialist registrars

Background

Despite the increased availability of resuscitation courses and guidelines requiring optimal training for acute situations, little is known on the actual qualification of house staff with in‐hospital on‐call duties for critically ill newborns and children.

Objective

To assess (1) the characteristics of education and training; (2) the level of experience; and (3) factors that may hamper optimal performance of paediatric specialist registrars caring for acute critically ill newborns and children.

Methods

A structured questionnaire was completed by a national cohort of all paediatric specialist registrars in their fourth year (ie pre‐final) of training.

Results

Important shortcomings in training and assessment of actual qualifications of resuscitation competencies were identified in paediatric specialist registrars. In 17 of 57 (30%) specialist registrars, competencies in acute care had never been assessed or reconfirmed when starting on‐call duties while in the others, 40 (70%), substantial heterogeneity was found regarding the type of assessment of qualifications for on‐call duties. In acute situations, occasionally untrained and unsupervised resuscitation procedures were performed. Individual responsibility was the most important stressor that may hamper optimal performance. Despite these findings, adequate reported levels of self‐confidence were found; self confidence was higher in newborns as compared to children (7.8 and 7.0 respectively on scale 1‐10, p<0.05).

Conclusions

Successful completion of a resuscitation course does not ensure adequate qualifications by on‐call residents, unless regular refresher sessions are provided. Teaching hospitals should establish and implement uniform guidelines for training and assessment of competencies regarding acute care for critically ill children and newborns.     

Mid-arm circumference can be used to estimate children's weights

Introduction

Accurate measurement of children's weight is rarely possible in paediatric resuscitation, and rapid estimates are made to ensure appropriate drug and fluid doses and equipment selection. Weight is commonly estimated from formulae based on children's age, or from their height using the Broselow tape. Foot-length and mid-arm circumference have also been suggested as the basis of weight-estimation formulae.

Objectives

To determine which of age, height, foot-length or mid-arm circumference had the strongest relationship with weight in healthy children, to derive a simple weight-estimation formula from the strongest correlate, and to compare its performance with existing weight-estimation tools.

Methods

This was a population-based prospective observational study of Hong Kong Chinese children aged 1-11 years old last birthday. Weight was measured to the nearest 0.2 kg; height, foot-length and mid-arm circumference to the nearest 0.1 cm. Multiple regression analysis was used to determine the strongest independent relationships with weight, and linear regression analysis derived a weight-estimation formula. Accuracy and precision of this formula were compared with standard age-based and height-based weight-estimation methods.

Results

Mid-arm circumference had the strongest relationship with weight, and this relationship grew stronger with age. The formula, weight [kg] = (mid-arm circumference [cm] − 10) × 3, was at least as accurate and precise as the Broselow method and outperformed the age-based rule in school-age children, but was inadequate in pre-school children.

Conclusion

This weight-estimation formula based on mid-arm circumference is reliable for use in school-age children, and an arm-tape could be considered as an alternative to the Broselow tape in this population.     

Validation of the APLS age-based vital signs reference ranges in a Chinese population

Reference ranges for vital signs may differ significantly among children of different ethnic origins.

Aim

(1) To validate the Advanced Paediatric Life Support (APLS) age-based vital signs reference ranges in Chinese children in Hong Kong. (2) To derive age-based centile curves for systolic blood pressure, heart rate and respiratory rate for Chinese children. (3) To summarize the reference ranges in a table format appropriate for applying APLS to ethnic Chinese patients.

Method

A cross-sectional study was performed on a population of healthy Chinese children recruited from 8 kindergartens and 6 primary schools in Hong Kong. Trained operators visit the sites to obtain measurements. Age-groups: small toddlers (12-23 months); pre-school (24-59 months); and school (60-143 months). Z-test was used to assess statistical significance for proportions of each parameter falling outside the APLS reference range. One-sample t-test was used for comparison with APLS means according to age-groups. LMS Chartmaker Pro v2.3 software was used to describe the data in centile curves.

Results

A total of 1353 patients (55.1% boys) were included. For heart rate, systolic blood pressure and respiratory rate respectively, 34.1%, 55.9% and 55.7% of corresponding measurements were outside the APLS age-based reference ranges. In the ‘pre-school’ and ‘school’ age-groups, the mean value for blood pressure is significantly higher, and the mean values for heart rate and respiratory rate significantly lower, in comparison to APLS mean values.

Conclusion

Independently derived vital signs reference ranges are more appropriate for use when applying APLS to Chinese patients in Hong Kong.     

Refractory macrophage activation syndrome in a patient with SLE and APLA syndrome – Successful use of PET- CT and Anakinra in its diagnosis and treatment

A patient with antiphospholipid syndrome, SLE and refractory fever is described. The cause for the fever was macrophage activation syndrome (MAS). The diagnosis of MAS was made with the help of PET-CT. Since the syndrome was refractory to conventional therapy with high-dose steroids and cyclosporin, anakinra was administered with complete recovery of the patient.

The present case illustrates the difficulties in diagnosing MAS when multiple bone marrow biopsies fail to show hemophagocytosis. It emphasizes the significance of PET-CT in the diagnosis and evaluation of treatment of MAS. Finally, it describes the important role of Anakinra in treating refractory cases of MAS.