Mental retardation in young children is often missed by clinicians. The condition is present in 2 to 3 percent of the population, either as an isolated finding or as part of a syndrome or broader disorder. Causes of mental retardation are numerous and include genetic and environmental factors. In at least 30 to 50 percent of cases, physicians are unable to determine etiology despite thorough evaluation. Diagnosis is highly dependent on a comprehensive personal and family medical history, a complete physical examination and a careful developmental assessment of the child. These will guide appropriate evaluations and referrals to provide genetic counseling, resources for the family and early intervention programs for the child. The family physician is encouraged to continue regular follow-up visits with the child to facilitate a smooth transition to adolescence and young adulthood.

OBJECTIVE To identify factors associated with very long survival among all cancer cases diagnosed at age 19 years or younger registered by the Cancer Data Service at the University of Kansas Medical Center in Kansas City, Kansas, U.S.A. in the 40-year period between 1944 and 1983, with follow-up to 1993. METHODS There were 2720 pediatric patients with 2750 cancers who were studied. Forty-four types of cancer were grouped into 11 diagnostic categories. Diagnosis years spanned four eras: 1944-1953, 1954-1963, 1964-1973, and 1974-1983. Cases were compared using specific characteristics and were divided into short-term and long-term survivors with the division generously set at seven years. The proportions of the long-term survivors were compared by specific characteristics. RESULTS Among the diagnostic categories, leukemias were the most common (29.8%), followed by CNS tumors (15.2%), and Hodgkin's disease (9.0%). Male to female ratio was 4:3; average age at diagnosis was 8.83 +/- 6.08 years. Long-term survivors totaled 1148 (41.7%). Prognosis was better in cases diagnosed in earlier stages and in later eras. Proportion of long-term survivors increased from 18.7% in era I to 52.6% in era IV. Improvement of survival was statistically significant in most diagnostic categories. CONCLUSIONS This study shows continuing improvement of survival during four consecutive eras for childhood and adolescent cancer. Early diagnosis was associated with better survival. Unstaged cases decreased over time reflecting progress in diagnostic techniques. Many patients died before seven years after diagnosis. Those who survived more than seven years had excellent survival. Pediatricians can expect to participate in the care of these patients long after the original dianosis and treatment.

There is little research into the extent to which Hospice Ethics Committees exist or how they operate (Ballentine & Dalinis, 2014). In the wider health system clinical and research Ethics Committees are more commonplace. In some countries other than the UK, Hospice Ethics Committees are more common. The role of an advisory ethics committee is one that has emerged, particularly in the USA. However, other than in relation to research, there is little evidence of the emergence of Ethics Committees in the UK. The aim of the project was to establish an Ethics Advisory Committee and identify if it could add value to staff, patients and family members. An Ethics Advisory Committee was established through open recruitment. Terms of reference and an induction programme were developed. Committee members have considered ethical issues relating to clinical service, fundraising, purchasing and the use of volunteers. In order to raise awareness of its existence, the Committee initiated an online survey with hospice staff who were asked to identify the ethical issues they faced. In order to ‘launch’ the Committee, a seminar was held on ‘The Ethics of Hospice Admissions’ attended by staff members, external partners (referrers) and patient/carer representatives. The online survey was completed by 11 members of the clinical team. This confirmed that clinical staff (across the hospice: in-patient care, day care, and community work) are facing ethical dilemmas at least once a month. The evaluation of the ethics seminar showed that staff and external stakeholders valued the access to an Ethics Committee created by the hospice. The project suggests that hospices should consider establishing an Ethics Advisory Committee to support the resilience of staff and add value to key stakeholders.

We present two male sibs with a series of malformations including microcephaly, mental retardation, congenital heart disease, skeletal abnormalities, micropenis, and mild hypothyroidism. Both have had seizures. While the pattern of abnormalities is similar to that previously reported in this journal as an unknown syndrome, the facies is clearly distinct, the hypothyroidism is mild, micropenis is present, and there are additional minor skeletal abnormalities.

Minor abnormalities reflect problems in prenatal development and can provide useful clues to the presence of major anomalies or developmental problems. In a comparative study, children with Down's syndrome, as expected, had significantly more minor abnormalities than those with other syndromes or major anomalies and those with developmental delay and/or cerebral palsy of unknown etiology. These two groups were not significantly different from each other in the number of minor abnormalities, but both had significantly more abnormalities than the group of normal children.

The authors identified 100 adults who survived cancer who were diagnosed and treated in childhood between 1945 and 1975. Using standardized interviews, each survivor and matched same-sex sibling was asked about life and health including questions about insurance. Compared with their same-sex siblings, childhood cancer survivors had significantly more difficulty in securing life insurance (P less than 0.001), in having life insurance in force (P less than 0.004), and in obtaining health insurance because of health reasons (P less than 0.001). Survivors were significantly less likely than siblings to be covered by health insurance (P less than 0.04). Cure of childhood cancer has become more common, allowing thousands of survivors to enter adult life. This study suggests that childhood cancer survivors have an unmet need in respect to life and health insurance.

Normal reproduction prospects for Hodgkin's disease patients are unknown. This study compares the outcome of 93 pregnancies in 48 patients with 228 pregnancies in 69 sibling controls. No statistically significant differences for spontaneous abortions or abnormal offspring were noted comparing all patients with all controls or 35 irradiated patients with all controls. Pregnancy outcome of 13 patients who received both irradiation and chemotherapy prior to pregnancy appeared to be compromised compared to controls. Wives of male patients in this category were more likely to have spontaneous abortions than wives of male controls; female patients in this category were significantly more likely to produce abnormal offspring than were female controls (p = 0.047). In this series of patients therapeutic irradiation alone did not appear to jeopardize posttreatment reproduction in fertile Hodgkin's disease patients but in the smaller group of patients who received both irradiation and chemotherapy the reproduction picture was statistically not as good.

The clinical, anatomical and biochemical variations in this disease are well demonstrated in the family here described. The transmission of the syndrome in this family seems to be by means of an X-linked gene. The presence of lenticular opaci ties in all mothers of affected males may indicate an X-linked, partially dominant, or intermediate transmission pattern. The observed delay in the appearance of hyperaminoaciduria in one youngster is suggestive of a progressive renal tubular lesion.

ASSOCIATION of marked bilirubinemia in the early neonatal period with kernicterus is well established. The possibility has occurred to many that lesser degrees of bilirubinemia may be related to less severe cerebral injury, too mild to be detected during the newborn period, but recognizable in later years as mild to moderate impairment of intelligence, partial nerve deafness, and other neurological deficits. A closely analogous concept is that of a continuum of reproductive casualty developed by Pasamanick and Knobloch. 1 It was the purpose of the present study to investigate the application of such a hypothesis to children with mild to moderate neonatal bilirubinemia. In preparation for this study, the world literature was reviewed. The most significant reports are summarized in Table 1. This illustrates the conflicting results and complexities of relating moderate bilirubinemia to subsequent cerebral difficulties in surviving children. Examination of Table 1 reveals that a number of factors

A RECENT report1 described the simultaneous occurrence of aseptic meningitis in a woman at term and fatal meningoencephalitis with myocarditis in her newborn infant. It is the purpose of this report to provide details pertinent to the laboratory diagnosis of this infant's disease, which were not included in the original report, and to document relative immunologic maturity in this newborn infant.Clinical AbstractsThe clinical records presented in detail elsewhere1 are briefly summarized. A twenty-two-year-old woman was admitted to the hospital in Honolulu, Hawaii, at term with chills, fever and severe frontal headache. Nasal congestion and a nonproductive cough had . . .