Ivemark syndrome with agenesis of the corpus callosum: a case report with a review of the literature

Asplenia associated with situs ambiguus, symmetric liver, bilateral trilobulated lungs, and a complex heart defect was diagnosed on autopsy in a 14-day-old infant. Furthermore, examination of the brain displayed agenesis of the corpus callosum (ACC) with pachygyria and hydrocephalus. The characteristic association of asplenia with visceroatrial heterotaxia is traditionally named after the Swedish pediatrician, Ivemark. Although exceptional, association of Ivemark syndrome with callosal agenesis has been reported recently. The concept of ‘developmental fields’ describes morphogenetically reactive units of the embryo determining and controlling the development of complex structures in a hierarchical manner. Lateralization defects such as situs inversus, asplenia or polysplenia due to defective left–right axis development, as well as decussation defects such as ACC, are considered as defects of the primary developmental field. Therefore, additional callosal agenesis in Ivemark syndrome may be a coherent and synchronic defect in the primary developmental field rather than a causally independent malformation. Copyright © 2002 John Wiley & Sons, Ltd.     

Deletion 15q24-26 in prenatally detected diaphragmatic hernia: increasing evidence of a candidate region for diaphragmatic development

Survival of children with congenital diaphragmatic hernia (CDH) is mainly dependent on the extent of lung hypoplasia and the presence of additional congenital anomalies or chromosomal aberrations. A chromosomal deletion 15q25-q26.2 in a fetus with prenatally diagnosed CDH and growth retardation is reported. Despite optimal pre- and neonatal management the baby died shortly after birth. There is increasing evidence that the long arm of chromosome 15, and especially the region 15q24 to 15q26, plays a crucial role in the development of the diaphragm. The finding of a deletion within 15q24-26 in a fetus with CDH has to be considered a predictor of poor prognosis. It is of utmost interest for proper parental counselling to search in fetuses with CDH for subtle chromosomal lesions paying special attention to chromosome 15q. Copyright © 2001 John Wiley & Sons, Ltd.     

Egg production of Calanus finmarchicus : effect of temperature, food and season

The use of the egg production rate of herbivorous copepods as an important parameter for understanding population dynamics and as an index of secondary production requires knowledge of the regulatory mechanisms involved and of the response to changes in food concentrations and temperature. Furthermore, the effects of season and generation on egg production have to be studied. In this context data are presented for Calanus finmarchicus from the northern North Atlantic. Prefed and prestarved females were exposed to different concentrations of the diatom Thalassiosira antarctica over 1 to 2 wk at 0 or 5 °C, and egg deposition was controlled daily. Egg production increased with higher food concentrations, but much less when prestarved. The effect of temperatures between −1.5 and 8 °C on egg production was studied in females maintained at optimum feeding conditions. Egg production rate increased exponentially over the whole temperature range by a factor of 5.2, from 14.2 to 73.4 eggs female⁻¹ d⁻¹, and carbon-specific egg production by 4, from 2.1 to 8.5% body C d⁻¹. The response to starvation was also temperature dependent. In both the temperature and feeding experiments egg production rate was regulated mainly by changes of the spawning interval, while changes of clutch size were independent of experimental conditions. Different responses to optimum feeding conditions were observed in females collected in monthly intervals on three occasions between March and May. The March females deposited more clutches than the April and May females. In May, >50% of the females did not spawn at all. Maximum egg production rates were never >25% of the rate expected at 5 °C, indicating endogenous control of egg production in addition to food and temperature effects. Received: 4 August 1996 / Accepted: 11 September 1996