Management of acute myocarditis in children.

Myocarditis is defined as the inflammation of the myocardium. It continues to be a significant cause of morbidity and mortality in the paediatric population and is the commonest cause of cardiac failure in a healthy child. Some studies estimate the incidence of myocarditis to be around 1 per 100 000. PubMed search was performed using the term 'myocarditis.' The search was limited to age 0-19 years. A total of 50 articles were identified between 1966 to date and reviewed. Myocarditis is a challenging diagnosis to make on clinical grounds and requires high index of suspicion. The cornerstone of treatment remains supportive though therapeutic modalities such as immunosuppressive and intravenous immunoglobulin therapies are being studied extensively. The overall prognosis of the disease is good with survival rates up to 80%.

Comparison of enteral versus intravenous potassium supplementation in hypokalaemia in paediatric patients in intensive care post cardiac surgery: open-label randomised equivalence trial (EIPS).

OBJECTIVES: The primary objective was to compare the efficacy of enteral potassium replacement (EPR) and intravenous potassium replacement (IVPR) as first-line therapy. Secondary objectives included comparison of adverse effects and number of doses required to resolve the episode of hypokalaemia. TRIAL DESIGN: The EIPS trial is designed as a randomised, equivalence trial between two treatment arms. STUDY SETTING: The study was conducted at the paediatric cardiac intensive care unit (PCICU) at Aga Khan University Hospital, Karachi. PARTICIPANTS: 41 patients (aged 1 month to 15 years) who were admitted to PCICU post cardiac surgery were recruited (23 IVPR arm and 18 EPR arm). INTERVENTION: Intervention arms were block randomised on alternate weeks for IVPR and EPR. OUTCOME MEASURE: Change in serum potassium levels in (mmol/L) and percentage change after each event of potassium replacement by the intravenous or enteral route. RESULTS: Both groups (41 patients) had similar baseline characteristics. Mean age was 4.7 (SD±4) years while the most common surgical procedure was ventricular septal defect repair (12 patients, 29.3%). No mortality was observed in either arm. Four episodes of vomiting and one arrhythmia were seen in the EPR group. After adjusting for age, potassium level at the beginning of the episode, average urine output, inotropic score and diuretic dose, it was found that there was no statistically significant difference in change in potassium levels after EPR and IVPR: 0.86 mmol/L (±0.8) and 0.82 mmol/L (±0.7) respectively (p=0.86, 95% CI -0.08 to 1.10), or percentage change in potassium level after enteral and intravenous replacement: 26% (±30) and 24% (±20) (95% CI -3.42 to 4.03, p=0.87). CONCLUSION: EPR may be an equally efficacious alternative first-line therapy in treating hypokalaemia after surgery in selective patients with congenital heart disease. ETHICS AND DISSEMINATION: This study has been approved by Ethics Review Committee at AKU. TRIAL REGISTRATION NUMBER: NCT02015962.

Comparison of Enteral versus Intravenous Potassium Supplementation in hypokalaemia in postcardiac surgery paediatric cardiac intensive care patients: prospective open label randomised control trial (EIPS).

BACKGROUND: Hypokalaemia is frequently encountered in the daily clinical practices of a paediatric cardiac intensive care unit (PCICU). It is a strong independent predictor of mortality in patients with heart failure. Thus, prompt potassium replacement therapy holds pivotal importance in therapy for hypokalaemia. Although intravenous potassium replacement (IVPR) in hypokalaemia is the preferred route in most intensive care settings, it is associated with known safety risks and can lead to arrhythmias, cardiac arrest and death if inappropriately administered. Enteral potassium replacement (EPR), with its superior safety profile, may be a better alternative to IVPR. OUTCOME: Primary outcome To compare the efficacy EPR and IVPR for treatment of hypokalaemia. Secondary outcome measures include a comparison of adverse effects (hyperkalaemia, diarrhoea, gastrointestinal bleeds, nausea and vomiting) after EPR and IVPR and a comparison of the number of dose/s required to achieve resolution of hypokalaemia for each episode of hypokalaemia. METHODS AND ANALYSIS: The Enteral Versus Intravenous Potassium Supplementation trial is designed as a randomised, controlled, non-blinded trial with two arms. Intervention arms will be block randomised on alternate weeks for IVPR and EPR. Recruited patients will receive treatment accordingly. For analysis, the percentage change in serum potassium levels in mEq/L after each event of potassium replacement in both arms will be used as an end point to compare the efficacy EPR and IVPR for treatment of hypokalaemia. STUDY SETTING: The study will be conducted at the PCICU at the Aga Khan University Hospital, Karachi. ETHICS AND DISSEMINATION: This study has been approved by the Ethics Review Committee and Clinical Trials Unit at The Aga Khan University with respect to scientific content and compliance with applicable research and human subjects regulations. TRIAL REGISTRATION NUMBER: This trial is registered at Clinical Trials.Gov. REGISTRATION NUMBER: NCT02015962.

New developments in the management of congenital Factor XIII deficiency.

Congenital Factor XIII (FXIII) deficiency is a rare, inherited, autosomal recessive coagulation disorder. Most mutations of this condition are found in the A-subunit with almost half these being missense mutations. Globally, approximately one in three million people suffer from this deficiency. Factor XIII deficiency is associated with severe life threatening bleeding, intracranial hemorrhage, impaired wound healing, and recurrent pregnancy losses. FXIII is known to have a potential role in mediating inflammatory processes, insulin resistance, bone metabolism, neoplasia, and angiogenesis. The algorithm provided for FXIII diagnosis and classification will enable prompt identification and early intervention for controlling potential life threatening complications. Prophylactic replacement therapy using blood products containing FXIII such as fresh frozen plasma, cryoprecipitate, or using FXIII concentrate remains the mainstay for the management of FXIII deficiency. In most parts of the world, cryoprecipitate and plasma transfusions are the only treatments available. Management developments have revealed the effectiveness and safety of recombinant FXIII concentrate for prophylaxis and treatment. The aim of this review is to provide an overview of advancements made in the management of FXIII deficiency from the time it was first detected, highlighting novel developments made in recent years. Greater research is warranted in identifying novel approaches to manage FXIII deficiency in light of its underlying pathophysiology.