Syndrome of encephalopathy and fatty degeneration of viscera in New Zealand children.
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Adipose Tissue↗
Biomedical subjects
D M Becroft
Publications and source records attributed to D M Becroft.
Goat's milk and megaloblastic anaemia of infancy: A report of three cases and a survey of the folic acid activity of some New Zealand milks.
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Anemia, Macrocytic↗
Idiopathic infantile arterial calcification with survival to adult life.
Moran, in an excellent review, defined idiopathic infantile arterial calcification as a rare disorder of undetermined etiology, characterized by deposition of calcific material along the internal elastic membrane of large, medium, and small arteries. Fibrous proliferation in the intima occurs and the arterial lumen is narrowed. The constellation of affected tissues varies but the coronary arteries are almost always involved whereas the arteries of the central nervous system are spared. Death from myocardial infarction usually results within the first six months of life. To date there have been only three survivors. We present the case history of a fourth survivor, a 22-year-old woman who, at the age of three months, was diagnosed as having idiopathic infantile arterial calcification.
Adolescent↗
Epidemiology of intrathoracic petechial hemorrhages in sudden infant death syndrome.
The possible effects of a wide range of sociodemographic and environmental factors on the incidence and distribution of petechiae were investigated in 485 sudden infant death syndrome (SIDS) cases from the New Zealand Cot Death Study. The number (nil, few, many) of macroscopic petechial hemorrhages in the visceral pleura, capsule of thymus, and epicardium was recorded in 458 of 474 autopsied SIDS cases. Other information was obtained from parental interview and obstetric records. Univariate analysis showed highly significant relationships (P < or = 0.005) between the frequency of petechiae at one or more sites and socioeconomic status, parity, breast feeding, age at death, time of death, sleep position, and head covering at death and lesser but significant relationships (P < or = 0.05) with Maori ethnicity, birth weight, gestation, pacifier use, and bed sharing. After multivariate analysis, significant associations remained between increased frequencies of thymic petechiae and parity (P = 0.0001), age at death (P = 0.0003), Maori ethnicity (P = 0.0019), pacifier use (P = 0.0001), and head covering at death (P = 0.0032); between increased frequencies of epicardial petechiae and head covering at death (P = 0.008) and an estimated time of death between 00:00 and 05:59 h (P = 0.056); and between increased frequencies of pleural petechiae and maternal smoking (P = 0.058) and parity (P = 0.022). There was a decreased frequency of pleural petechiae in infants placed prone for their final sleep (P = 0.058). The distribution and frequency of petechiae are affected by environmental factors, including known risk factors for SIDS, but these factors occur inconsistently across the three sites. The findings imply differences in the pathogenesis at each site but do not provide consistent support for previous theories of causation of petechiae.
Analysis of Variance↗
Fetal megakaryocytic dyshemopoiesis in Down syndrome: association with hepatic and pancreatic fibrosis.
Trisomy 21 was diagnosed by prenatal blood sampling at 30 and 31 weeks of gestation, respectively, in two fetuses with hepatosplenomegaly. In both, the fetal blood contained blast cells and cells showing megakaryocytic differentiation. Case 1 died neonatally 1 week later and the cellular infiltration causing enlargement of liver and spleen had a megakaryocytic/megakaryoblastic component staining positively for von Willebrand factor and binding to Ulex europaeus 1. Case 2, when stillborn 4 weeks later, had remarkably severe hepatic and pancreatic fibrosis. Cells in pulmonary vessels had morphology and immunohistochemical reactions consistent with megakaryocytic/megakaryoblastic differentiation. Comparison of the two cases suggests that the visceral fibrosis of perinatal Down syndrome may progress very rapidly in utero. They demonstrate further the association of the fibrosis with a dyshemopoiesis in which there is proliferation of cells of megakaryocytic lineage and a close relationship to the transient leukemia of neonatal Down syndrome.
Adult↗
Good prognosis of cellular mesoblastic nephroma with hyperdiploidy and relaxation of imprinting of the maternal IGF2 gene.
A large congenital mesoblastic nephroma (CMN) of combined classical and and cellular histological structure was removed from a 1-month-old female infant. The tumor extended extrarenally and may have been incompletely excised. Tumor tissue showed a mosaic hyperdiploidy with 54 chromosomes in the hyperdiploid line. No other antitumor therapy was given and there has been no recurrence after 4 years. Genomic imprinting normally prevents transcription of the maternal gene for insulin-like growth factor 2 (IGF2). Relaxation of IGF2 imprinting leading to abnormal transcription of the maternal gene is found in a majority of Wilms' tumors and in other malignant neoplasms. The biallelic transcription of IGF2 demonstrated in the CMN from this case is consistent with abnormal transcription of the maternal allele. Relaxation of imprinting of the maternal IGF2 gene or abnormal expression of the gene through other mechanisms may have a role in the genesis of CMN or the cellular subtype.
Diploidy↗