Annual Review of Medicine - Volume 34, 1983

Type I diabetes is a heterogeneous disorder and the causes of pancreatic beta-cell destruction are unknown. In 1-2% of all cases, viruses (e.g. coxsackie, rubella, mumps, or beta-cell poisons) have been implicated. Twin studies suggest at most 50% of genetic predisposition.

In this review we describe the autoimmune components which, in association with inheritance of HLA-haplotypes in susceptible families, allow the future selection of predisposed sibs for possible preventive therapy to retard loss of insulin secretion. The known association of the endocrine autoimmune organ-specific disorders in 10% of Type I diabetics is the extreme expression of the other main genetic ingredient in the development of insulitis in this disease, irrespective of the triggering environmental components. In this “polyendocrine” subgroup and in the “juvenile-onset” cases there is a prolonged latency period during which pancreatic autoimmunity markers are present before clinical expression of the disease.

The strategy of management in childhood renal failure is to integrate available therapeutic modalities with the natural history of the disease in such a manner that linear growth is preserved. Attention to psychosocial growth is necessary for full rehabilitation. Treatment is ordered to provide hope and confidence to family and patient. Conservative medical therapy provides chemical stabilization and prophylaxis against bone disease and growth retardation. Planned progression to replacement of renal function by dialysis or renal transplant is made when growth or control of bone disease is no longer possible, but before complications occur. Therapy is effective; 90% of patients survive 5-10 years with 65% retaining a functioning allograft. Full rehabilitation is attained in 85-90% of post-transplant and 50% of dialysis patients. Technical advances now allow safe dialysis for all age groups. Although growth maintenance remains a major problem, it is possible that earlier metabolic control and transplantation to protect growth in the critical early years, together with improved control of rejection, may allow close approximation to optimal growth potential.

The cognitive functions are defined and discussed. Factors that place cancer patients at high risk for disorders of cognition are presented along with the problem of semantic confusion in this area. The cognitive impairment found in cancer patients receiving chemotherapy is reviewed, as is the importance of the mental status examination and the treatment of delirium in cancer patients. Several illustrative case reports are presented.

The complement system, phagocytic leukocytes, and antibodies constitute the “department of defense” in the battle against infections caused by pyogenie microorganisms. Complement components are capable of directly killing (by lysis) certain susceptible bacteria and are the source of chemotactic peptides and heat-labile opsonin, which facilitate recognition and killing of microbes by phagocytic cells. When these vital functions are compromised, as in individuals with inherited deficiencies of complement components, the result is unusual susceptibility to severe infections.

The rate of absorption of iron is adjusted according to body iron requirements, but the virtual absence of heme and the poor bioavailability of the nonheme iron in the diets of many people, especially in developing countries, means that the amount that can be absorbed is limited. Those whose requirements are increased by growth, menstruation, or pregnancy frequently cannot absorb enough. Sufficient is now known about the factors in food that increase or diminish the bioavailability of nonheme iron to permit the effective fortification of dietary staples, although the application of this information has proved difficult particularly in the Third World where nutritional iron deficiency is most prevalent. Effective fortification may lead to iron overload in those whose control of iron absorption is genetically defective, and recent evidence that the HLA-linked recessive gene for idiopathic hemochromatosis may occur much more commonly than hitherto suspected makes it imperative that an effective monitoring system should form a part of every fortification program.

Hemapheresis is the selective collection of any blood component. With the use of automated equipment, hemapheresis has become practical both for procuring specific components for transfusion and for removing specific components considered pathogenic factors in clinical disease. This review considers all aspects of hemapheresis, including its role in blood component procurement and its potential therapeutic applications. Attention is focused on the clinical indications for the use of apheresis-harvested components, and on the rationale for the effectiveness of therapeutic cytapheresis and plasma exchange.

The identification of markers of human hematopoietic cell differentiation and function has revealed the nature of the proliferating cell in many lymphoid neoplasms and demonstrated marked phenotypie heterogeneity in traditional subgroups. Selected markers are described that define common subgroups of lymphoid leukemia and non-Hodgkin’s lymphoma. The identification of cell markers has significant implications for accuracy of diagnosis, for predicting clinical characteristics, and for understanding the biology of these diseases.

Heart failure seems to occur in adult-onset diabetics with a greater frequency than in the nondiabetic population, particularly in women. A number of such patients do not have significant occlusive disease of the major coronary arteries, or convincing small-vessel disease. A subclinical abnormality of myocardium in experimental diabetes and asymptomatic human diabetics supports the concept of a diabetic cardiomyopathy.

The authors review the evidence supporting the hypothesis of similarity or identity of prepubertal and adult major depressive disorders and give a state-of-the-art account of current assessment techniques, diagnosis, and treatment methods of prepubertal major depression. This is an area of child psychiatry where recent psychobiological research advances are fueling profound changes in traditional nosology and therapeutics. In addition, further understanding of early onset major depression is likely to throw considerable light on the nature and pathophysiology of depressive illness across ages.