Newborn screening for inborn errors of metabolism: a systematic review. Health Technol Assessment 1997; 1(11). NHS R&D HTA Programme T he overall aim of the NHS R&D Health Technology Assessment (HTA) programme is to ensure that high-quality research information on the costs, effectiveness and broader impact of health technologies is produced in the most efficient way for those who use, manage and work in the NHS. Research is undertaken in those areas where the evidence will lead to the greatest benefits to patients, either through improved patient outcomes or the most efficient use of NHS resources. The Standing Group on Health Technology advises on national priorities for health technology assessment. Six advisory panels assist the Standing Group in identifying and prioritising projects. These priorities are then considered by the HTA Commissioning Board supported by the National Coordinating Centre for HTA (NCCHTA). This report is one of a series covering acute care, diagnostics and imaging, methodology, pharmaceuticals, population screening, and primary and community care. It was identified as a priority by the Population Screening Panel (see inside back cover). The views expressed in this publication are those of the authors and not necessarily those of the Standing Group, the Commissioning Board, the Panel members or the Department of Health.
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Nulla est alia pro certo noscendi via, nisi quam plurimas et morborum et dissectionum historias, tam aliorllm proprias, collectas habere et inter se comparare.-MORGAGNI. De Sed. et Caus. Morb.. lib. 14. Proœmium.WITH the view of continuing our illustrations of diseases of the spleen, which were commenced in previous " Mirrors," we ' ' now place upon record a few clear and uncomplicated exam-' I ples of intermittent fever, all (excepting in one instance) accompanied by well-marked enlargement of the organ in i question. On the 7th of January (see page 9 of the present , volume) we gave three characteristic examples of leucocythemia splenica-the blood possessing a preponderance of white cellsassociated with hypertrophy of the spleen and other symptoms then detailed, which taken together form a well-recognised affection in which the spleen is involved. A second splenic disease is the anaemia lymphatica, wherein some of the lymphatic glands of the body are enlarged, with deposits in the spleen, and simple anæmia. These two forms of disease are now looked for, and can be recognised without much difficulty by the careful physician. A third malady is the intermittent fever, in which we usually expect to find what is popularly called the ague cake, the result of repeated congestions. The causes of this form of fever, its symptoms and varieties, are so well known and understood, that it would be superfluous to enter into their consideration here. We shall confine ourselves solely to the condition of the spleen, so that when an enlargement is met with, the knowledge of the fact of there having once been ague present will help us in our diagnosis.The true pathology of the condition of the spleen in ague is not such a particularly essential feature in this latter respect, although we understand pretty well that its enlargement is due, firstly, to the mechanical congestion, which sets up some irritative action, as has been supposed, and deposits some of the peculiar elements of the blood within its structure. Whether the blood undergoes disintegration in this organ, and the hasmatine is set free, in certain severe examples, we shall not stop to inquire. The point we desire to note is the enlargement. This, as it is witnessed in England, is generally moderate, although it is sometimes pretty extensive, and occupies a large portion of the left side of the abdomen. We have seen it in hot climates attain the bulk of fully twenty times its natural size; and we recollect an instance, which ended fatally, wherein the organ was the size of a large liver, and contained an abscess. In rare cases the hypertrophy has been found but partial in ague. Usually, however, in examples that have succumbed, the organ is found enlarged and indurated, but presenting, in other respects, no features to the naked eye differing from those in the healthy gland, except the occasional presence of numerous transparent little grains, which are the Malpighian bodies apparently enlarged.A patient who shall have recovered from an ague of a chronic ...
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