Transient hypogammaglobulinaemia of infancy: many patients recover in adolescence and adulthood

Transient hypogammaglobulinaemia of infancy (THI) is a relatively rare disorder where there is an exaggeration of the physiological nadir of immunoglobulin (Ig)G between loss of transplacentally acquired maternal IgG and production by the infant. Patients may be vulnerable to infections during the period of hypogammaglobulinaemia. The precise time to recovery in all infants is currently unknown. We sought to determine the clinical features and time–course of recovery for patients with THI. We reviewed our experience with THI over the last three decades in order to describe clinical and laboratory features, as well as the time–course of recovery. Forty-seven patients were identified with THI. Only thirty-seven per cent remitted by 4 years of age, while some patients did not recover until the third or fourth decade. In keeping with previous studies, the majority (25 of 47) presented with recurrent infections, nine had a family history of immunodeficiency and 13 had adverse reactions to food as their dominant clinical manifestation. Chronic tonsillitis developed in 10 patients and symptoms improved following surgery. The group with food allergies recovered sooner than those presenting with infections or with a family history immunodeficiency. Eight patients failed to respond to at least one routine childhood vaccine. Two have IgA deficiency and four individuals recovering in adolescence and adulthood continue to have borderline/low IgG levels. None have progressed to common variable immunodeficiency disorders (CVID). THI is a misnomer, as the majority do not recover in infancy. Recovery from THI can extend into adulthood. THI must be considered in the differential diagnosis of adolescents or young adults presenting with primary hypogammaglobulinemia.     

The Feasibility of Extracorporeal Membrane Oxygenation in the Variant Airway Problems

Introduction: Extracorporeal membrane oxygenation (ECMO) is widely used to treat respiratory distress during cardiac or respiratory arrest; moreover, its use is being extended to a wide variety of clinical fields. In this study we assess the utility of ECMO in the management of airway obstruction.Patients and Methods: 15 patients underwent ECMO for airway obstruction. We retrospectively analyzed and evaluated the feasibility of ECMO in the treatment of airway problems.Results: Seven patients received ECMO to facilitate respiration and promote stability during trachea surgery. In six cases ECMO ceased immediately following the operation; in the remaining case ECMO cessation was delayed due to post-operative ARDS. In three cases emergency ECMO was used in response to respiratory arrest; two patients died. In five cases ECMO was emergently inserted to prevent death, following airway blockade by massive hemoptysis. One patient was not discharged from the intensive care unit. Another patient was transferred to a general ward but died from other causes.Conclusion: ECMO is useful during anesthesia in patients at high risk of airway blockade, for example due to endobronchial bleeding, and during complex thoracic surgery. ECMO confers a safer environment during airway surgery, and its complication rate is acceptable.     

Virtual implantations to transition from porcine to bovine animal models for a total artificial heart

Realheart total artificial heart (TAH) is a novel, pulsatile, four-chamber total artificial heart which had been successfully tested acutely in a porcine animal model. However, the bovine model is better suited for long-term testing and thus an evaluation of how the design would fit the bovine anatomy was required. Virtual implantation is a method that enables a computer simulated implantation based on anatomical 3D-models created from computer tomography images. This method is used clinically, but not yet adopted for animal studies. Herein, we evaluated its suitability in the redesign of the outer dimensions and vessel connections of Realheart TAH to transition from the porcine to the bovine animal model. Virtual implantations in combination with bovine cadaver studies enabled a series of successful acute bovine implantations. Virtual implantations are a useful tool to replace the use of animals in early device development and refine subsequent necessary in vivo experiments. The next steps are to carry out human virtual implantations and cadaver studies to ensure the design is optimized for all stages of testing as well as the final recipient.