Issues to resolve with the use of extracorporeal membrane oxygenation during interfacility transportation. / Transporte interhospitalario con membrana de oxigenación extracorpórea: cuestiones a resolver.

Extracorporeal membrane oxygenation (ECMO) support is indicated in patients who are refractory to treatment, those with cardiogenic shock or respiratory failure and those with exacerbations eligible for heart and lung transplantation. Physician experience and quantity of necessary resources are reasons why regionalization could benefit patients of this kind, establishing ECMO reference centers and integrating a transportation network specialized in ECMO. This type of transportation is a challenge for healthcare systems and physicians, given its greater complexity, requiring a multidisciplinary and inter-territorial approach. ECMO transportation is safer than without mechanical support, though there are currently no criteria for starting such therapy in patients being transferred. Criteria of lesser severity might be necessary for these patients. The training and specialization of the team in extracorporeal support therapies, interfacility transport and the systemization of transfer can improve the outcomes. There are no studies on the conditions that must be met by the transportation media, although space and stability are important characteristics. Air transfer with ECMO is an increasingly frequent option. Although there are data on its safety, there are none on the physiology of patients undergoing ECMO at high altitudes. Such information could be of help in the indication and management of this type of transportation.

Eighteen-year follow up after Hancock II bioprosthesis insertion.

BACKGROUND AND AIM OF THE STUDY: The long-term (18 years) results after aortic (AVR), mitral (MVR) and double (aortic/mitral, DVR) valve replacement with Hancock II bioprosthesis were investigated. METHODS: Between 1978 and 1996, 279 Hancock II bioprostheses were implanted in 269 patients (166 males, 113 females; mean age 61.8+/-13.3 years). There were 135 AVR (48.4%), 122 MVR (43.8%) and 22 DVR (7.8%). Preoperatively, 208 patients (77.3%) were in NYHA functional class III/IV, 53 (19.7%) had previous cardiac surgery, and 19 (7.1%) underwent concomitant coronary artery bypass. Follow up (mean seven years) was 96% complete, with a total of 1,857 patient-years. RESULTS: There were 20 early (7.3%), and 78 (29.0%) late deaths. At the last follow up, 68.3% of patients were in NYHA functional class I/II. The actuarial survival rate of patients at 10 and 18 years after discharge was 67.7+/-5.0% and 44.7+/-8.8% after AVR and 64.5+/-5.6% and 32.7+/-11.5% after MVR, respectively; survival after DVR was 74.0+/-11.2% at 12 years. At 10 and 18 years, actuarial freedom from thromboembolism was 83.5+/-4.5% and 73.1+/-10.5% after AVR and 82.1+/-4.3% and 73.2+/-7.3% after MVR; it was 78.4+/-15.0% after DVR at 12 years. At these times, actuarial freedom from hemorrhage was 88.7+/-3.8% and 83.5+/-6.2% after AVR and 79.0+/-4.9% and 32.6+/-23.3% after MVR; freedom after DVR was 36.2+/-26.6%. Probability of freedom from endocarditis at 10 and >15 years was 93.4+/-3.5% and 85.9+/-7.8% after AVR and 97.0+/-2.1% and 97.0+/-2.1% for MVR, respectively; freedom at 10 years after DVR was 75.0+/-21.6%. Freedom from structural deterioration at 10 and 18 years was 77.9+/-5.3% and 18.7+/-14.6% after AVR and 78.3+/-6.0% and 32.1+/-10.2% after MVR; freedom at 10 and 12 years after DVR was 64.0+/-17.5% and 32.0+/-24.2%. A low incidence of structural valve deterioration was found in AVR patients aged >65 years (p = 0.0478). Hemorrhage and paravalvular leak were more frequent in MVR (p = 0.0296 and 0.0309, respectively). No difference was seen in thromboembolism after anticoagulation for one or three months after AVR. Actuarial freedom from explantation at 10 and 18 years was 73.1+/-5.9% and 15.9+/-13.5% after AVR and 77.1+/-6.1% and 37.3+/-9.7% after MVR; freedom at 10 and 12 years after DVR was 72.0+/-17.8% and 24.0+/-20.4%. CONCLUSION: Over an 18-year follow up, the Hancock II bioprosthesis has shown satisfactory results, with a low incidence of valve-related complications, especially in elderly patients in the aortic position.

Prenatal growth retardation, pelvic hypoplasia, and arthrogrypotic changes of lower limbs: a distinct autosomal-recessive disorder.

We report on 5 sibs (4 males, 1 female) with growth retardation, severe pelvic hypoplasia, arthrogrypotic changes and muscular hypotrophy of the lower limbs, and mild vertebral changes of prenatal onset. To our knowledge, this syndrome has not yet been reported. The family history suggests autosomal-recessive inheritance.

Hereditary multiple benign cystic epithelioma (multiple trichoepithelioma) with onset at early age.

A mother and a daughter affected with multiple trichoepithelioma were studied. The age of onset of the symptomatology in both was 7-years-old, the daughter being more severely affected than the mother at this age. This early age of onset is an exceptional observation which could be explained by maternal imprinting.

Autosomal dominant congenital epiphyseal dysplasia limited to the femoral heads.

A father and son with unusual congenital skeletal symptoms, mainly characterized by short stature and epiphyseal dysplasia limited to the femoral heads are described. The comparative analysis with other spondyloepiphyseal dysplasias supports the notion that the syndrome reported here is a distinct autosomal dominant connective tissue disorder.

Generalized osteoporosis in a patient with oculocutaneous hypopigmentation syndrome (OOCHS), without cerebral defects. A new syndrome?

An 8-year-old male patient presented a unique pattern of congenital anomalies. Prominent findings included a combination of severe osteoporosis and congenital oculocutaneous hypopigmentation. The patient may represent a hitherto undescribed syndrome of unknown etiology.

Autosomal dominant congenital macroglossia: further delineation of the syndrome.

We describe seven patients from two Mexican families with congenital macroglossia. Comparative analysis of these cases and the patients previously described in literature, allows to confirm the notion that this isolated primary macroglossia is a distinct dominant condition.

Osteopoikilosis: report of a familial case.

We describe four members of a family in which the clinical and radiological findings lead to consider the diagnosis of osteopoikilosis. The symptoms in all affected members were only those referred to as typical radiological features; these features became more extensive with older age. None of the subjects showed the skin lesions reported in the Buschke-Ollendorff syndrome. The importance of a suitable differential diagnosis is emphasized in order to avoid dangerous and unnecessary treatments.