Spinal cord central echo complex: histoanatomic correlation

Real-time sonography consistently demonstrates an echo complex centrally located in the spinal cord. This echogenic focus is currently attributed to the central canal. The authors sought the source of this central echo with ultrasound (US) and histoanatomic study of both fresh and fixed cadaver spinal cords. Correlative transverse sections of the spinal cord were stained with hematoxylin-eosin and Luxol fast blue for cells and myelin and with Holzer stain for glial fibrils. The central echo complex is produced by the interface between the myelinated ventral white commissure and the central end of the anterior median fissure. Variations in the shape of the central complex seem to reflect varying degrees of flaring of the central end of the anterior median fissure. The inconstant residual central canal and islands of residual ependymal cells are clearly not the source of the central echo complex.     

The angiographic diagnosis of acute hemorrhagic renal cortical necrosis

The authors report a case of acute renal failure resulting from acute cortical necrosis. A selective renal angiogram defined a very unusual, abrupt termination of vascularity at the interlobular and arcuate levels. Nephro-angiography is an important tool in the diagnosis of acute cortical necrosis in the initial phase of acute renal failure.     

Differential modulation of the immune response by breast- or formula-feeding of infants

Spontaneous integrin expression on CD4+, CD8+ and CD19+ lymphocytes at 6 months was significantly lower in breastfed than formula-fed infants ( p < 0.05). In another study of 59 formula-fed and 64 breastfed 12-month-old children blast transformation and cytokine production by lymphocytes, and T cell changes were measured before and after measles-mumps-rubella vaccination (MMR). Before vaccination, lymphocytes of breastfed children had lower levels of blast transformation without antigen ( p < 0.001), with tetanus toxoid ( p < 0.02) or Candida ( p < 0.04), and lower interferon-γ production ( p < 0.03). Fourteen days after the live viral vaccination, only the breastfed children had increased production of interferon-γ ( p < 0.02) and increased percentages of CD56+ ( p < 0.022) and CD8+ cells ( p < 0.004). These findings are consistent with a Thl type response by breastfed children, not evident in formula-fed children. Feeding mode has an important long-term immunomodulating effect on infants beyond weaning.     

Social skills training for primary school children: A 1-year follow-up study

Objective: To evaluate the effectiveness of the Rochester Social Problem Solving Program to reduce emotional and behavioural problems amongst primary school children.
Methodology: Children in years 3 and 4 at primary school were assessed prior to receiving the program, immediately after the program and 1 year after the program. At each assessment, the functioning of the children who received the program was compared to the functioning of children enrolled in years 3 and 4 at a comparable school who did not receive the program.
Results: The program improved the ability of children to cope with potentially difficult social situations. However, the program did not reduce the prevalence of teacher-reported or mother-reported childhood emotional and behavioural problems.
Conclusions: School-based social skills programs may be more effective in reducing childhood emotional and behavioural problems if they include components which focus specifically on childhood behaviour problems as well as components focusing on social skills and peer relationships.     

Incidence, population, and survival of cystic fibrosis in the UK, 1968-95. UK Cystic Fibrosis Survey Management Committee

The UK Cystic Fibrosis Survey holds data on all people resident in the UK who were diagnosed as having cystic fibrosis and born either since 1968 or before 1968 and alive in 1977. Thus, incidence may be reported from 1968 and prevalence from 1977. The previous estimates are updated to the end of 1995 from data held in the database on 23 August 1996. The incidence is now calculated as one in 2415 live births. The 1992 mid-year population was 6500 people with 65% aged under 16 years. Births outnumber deaths by 160 per year, which suggests a population of 7750 by the year 2000, with all the increase being in the adult age range. The survival of successive cohorts continues to be better than earlier cohorts, the linear descent of the curves is still evident. The infant mortality rate for cystic fibrosis is now under 20 per thousand per year and early childhood mortality is under five per thousand per year. The crude mortality rate for 1995 was 21 per thousand per year, but the standardised mortality ratio was about 3300.     

The clinical phenotype of succinic semialdehyde dehydrogenase deficiency (4-hydroxybutyric aciduria): case reports of 23 new patients

OBJECTIVES: To further define the clinical spectrum of the disease for pediatric and metabolic specialists, and to suggest that the general pediatrician and pediatric neurologist consider succinic semialdehyde dehydrogenase (SSADH) deficiency in the differential diagnosis of patients with (idiopathic) mental retardation and emphasize the need for accurate, quantitative organic acid analysis in such patients. PATIENTS: The clinical features of 23 patients (20 families) with SSADH deficiency (4-hydroxybutyric acid-uria) are presented. The age at diagnosis ranged from 3 months to 25 years in the 11 male and 12 female patients; consanguinity was noted in 39% of families. OUTCOME MEASUREMENTS: The following abnormalities were observed (frequency in 23 patients): motor delay, including fine-motor skills, 78%; language delay, 78%; hypotonia, 74%; mental delay, 74%; seizures, 48%; decreased or absent reflexes, 39%; ataxia, 30%; behavioral problems, 30%; hyperkinesis, 30%; neonatal problems, 26%; and electroencephalographic abnormalities, 26%. Associated findings included psychoses, cranial magnetic resonance or computed tomographic abnormalities, and ocular problems in 22% or less of patients. Therapy with vigabatrin proved beneficial to varying degrees in 35% of the patients. Normal early development was noted in 30% of patients. CONCLUSIONS: Our data imply that two groups of patients with SSADH deficiency exist, differentiated by the course of early development. Our recommendation would be that accurate, quantitative organic acid analysis in an appropriate specialist laboratory be requested for any patients presenting with two or more features of mental, motor, or language delay and hypotonia of unknown cause. Such analyses are the only definitive way to diagnose SSADH deficiency; the diagnosis can be confirmed by determination of enzyme activity in white cells from whole blood. We think that increased use of organic acid determination will lead to increased diagnosis of SSADH deficiency and a more accurate representation of disease frequency. As additional patients are identified, we should have a better understanding of both the metabolic and clinical profiles of SSADH deficiency.