(1973). Archives It is concluded that the overall results of selective early operation compare favourably with those of purely conservative management on the one hand, and with routine early operation on the other. It is suggested that this policy offers the best prospects of independence for the less severely affected child, and the least distress and suffering for the grossly afflicted.Routine early closure of myelomeningocele was advocated in Sheffield a decade ago (Sharrard et al., 1963) and has since become standard practice in many centres in Great Britain and abroad. The only detailed report of the results of unselective early operation is that of Lorber (1971a). The end results of an unparalleled expenditure of resources on myelomeningocele were so disappointing that he went on to advocate a policy of selection based on certain major adverse criteria at birth, which would have excluded just over half from operation. As yet, however, there are no published reports on the actual effectiveness of such a policy.Since the selection criteria employed at the Royal Hospital for Sick Children, Edinburgh, where routine early operation has never been accepted, accord closely with those now defined by Lorber, it is of interest to compare our results with those of the nonselective policy employed in Sheffield.Received 12 February 1973. Patients and methods of study Between April 1965 and March 1971, 163 unselected infants with open myelomeningoceles were admitted to the spina bifida unit. More than 98% were under 24 hours of age on admission, the range being 1 to 64 hours. On admission every child was subjected to a detailed paediatric and neurological examination by one of us, as previously described (Stark, 1971), and detailed records kept as part of a prospective study. No predefined criteria for selection were employed, but each case was considered on its merits-social as well as medical and surgical factors being taken into consideration.Patients fell into two groups. In group A 78 infants (48%) were subjected to early operation, associated problems were dealt with systematically, and complications treated vigorously. The 85 patients (52%) in group B were not subjected to operation. They were retained in the unit where they received good nursing care, were fed normally, and kept comfortable. In the event of life-threatening situations, such as ventriculitis, antibiotics were withheld and therapy confined to analgesics, such as heroin. In infants in this group 676 on 12 May 2018 by guest. Protected by copyright.
SUMMARY The neurological lesions which occur in the lower limbs of infants suffering from myelomeningocele are described. In a series of 30 consecutive newborn patients evidence of function in isolated distal spinal cord was found on the first examination in 50 per cent and later in 66.6 per cent. While there was a tendency for reflex function to diminish, rapid deterioration of ‘voluntary’ function was exceptional. The clinical and pathogenetic significance of these findings is discussed. RÉSUMÉ La complication neurologique des membres inférieurs dans le myeloméningocele Les auteurs décrivent les lésions neurologiques qui apparaissent dans les membres inférieurs des jeunes enfants atteints de myeloméningocèle. Dans une série de trente patients nouveau‐nés consécutifs on a mis en évidence une fonction réflexe dans la moelle épinière distale isolée au premier examen dans 50 pour cent des cas et plus tard dans 66.6 pour cent. Il y avait une tendance à la diminution de la fonction réflexe, mais la détérioration rapide de la fonction volontaire était exceptionelle. On discute de la signification clinique et pathogènétique de ces observations. RESUMEN Afecciones neurólogicas de los miembros inferiores en casos de mielomeningocele Se describen las lesiones neurológicas que ocurren en los miembros inferiores de niños afectos de mielomeningocele. En una serie de 30 recién nacidos, enfermos consecutivos, se halló evidencia de función en la medula espinal distal aislada, al primer examen en un 50 por ciento y más tarde en un 66.6 por ciento. Mientras que la función refleja disminuía generalmente, se veía solamente en casos excepcionales un deterioro rápido de la función ‘voluntaria’. Se discute la importancia cliníca y patogénica de estos hallazgos.
SUMMARY Four children are described from three Saudi Arabian families suffering from osteopetrosis, distal renal tubular acidosis and cerebral calcification associated clinically with mental retardation, stunted growth, abnormal teeth and a similar facial appearance. The syndrome is inherited as an autosomal recessive. The growth retardation is attributed to renal tubular acidosis but no metabolic link is evident between its other major features, and the fundamental defect is unknown. The disorder is radiologically indistinguishable from the classical recessive (malignant) and dominant (benign) forms of osteopetrosis but its other characteristics establish it as a separate disease entity. RÉSUMÉ Maladie du cerveau marmoréen: ostéopétrose récessive, aeidose rénale tubulaire et calcification cérébrate dans trois families 'Arabie Séoudite L'article décrit les enfants de trois families 'Arabie Séoudite atteints 'ostéopétrose, d'acidose rénale tubulaire distale et de calcification cérébrale associées cliniquement à un retard mental, un arrêt de croissance, des anomalies dentaires et une apparence faciale commune. Le retard de croissance peut être reliéà 'acidose rénale tubulaire mais aucun lien métabolique n'est évident entre les autres caractéristiques principals du syndrome et le défaut fondamental est inconnu. Radiologiquement, le trouble ne peut être distingué des formes classiques 'ostéopétrose, récessive (maligne) et dominant (bénigne), mais les autres caractéristiques conduisent à identifier une entité morbide particulière. ZUSAMMENFASSUNG Marmorknochenkrankheit mit Gehirnbefall: Autosomal recessiv vererbte Osteopetrose, renale tubuläre Azidose und cerebrale Verkalkung bei drei Saudi Arabischen Familien Von drei Saudi Arabischen Familien werden Kinder beschrieben, die eine Osteopetrose, distale tubuläre Azidose und cerebrale Verkalkung hatten, verbunden mit dem klinischen Hinweis auf geistige Retardierung, Wachstumsverzögerung, Zahnanomalien und einem ähnlichen Gesichtsausdruck. Das Syndrom wird autosomal recessiv vererbt. Die Wachstumsverzögerung wird durch die renale tubulare Azidose verursacht, für die anderen Hauptmerkmale des Syndroms konnte jedoch kein ursächlicher metabolischer Defekt gefunden werden. Die eigentliche Ursache ist unbekannt. Die Erkrankung kann radiologisch nicht von der klassischen recessiven (malignen) und der dominanten (benignen) Form der Osteopetrose unterschieden werden, jedoch ist sie durch thre anderen Charakteristika als abgegrenzte, eigenständige Krankheit anzusehen. RESUMEN Enfermedad del cerebro de mármol: osteopetrosis progresiva, acidosis renal tubolar y calcificación cerebral en ties familias de Arabia Saudita Se describen niños de tres familias de Arabia Saudita que surfían osteopetrosis, acidosis tubolar renal distal y calcificación cerebral, asociados clínicamente con retraso mental, detención del crecimiento, dientes anómalos y apariencia facial similar. El retraso en el crecimiento se atribuye a la acidosis renal tubolar pero no había evidencia de ningúna relación metab...
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