Prophylactic therapeutic plasma exchange in pregnant woman with Familial Chylomicronemia Syndrome - A case report.

CONTEXT: Familial Chylomicronemia Syndrome (FCS) is an inherited disease where lack of lipoprotein lipase results in severe hypertriglyceridemia that frequently leads to recurrent acute pancreatitis. Pregnancy in patients with familial chylomicronemia syndrome (FCS) post a risk for mother and baby with potential complications (pancreatitis, miscarriage and death). Therapeutic approach includes strict dietary measures and plasma exchange. Despite the development of new drugs for FCS, their safety in pregnancy has not yet been confirmed. CASE DESCRIPTION: We present a case of a young, pregnant female with FCS who had miscarriage in the past during one episode of acute pancreatitis. Due to the inability to achieve lower TG levels with current therapy, from 27-th week of pregnancy we have started prophylactic therapeutic plasma exchange (two times per week). Patient was followed up until the delivery of a healthy baby boy and did not experience an episode of acute pancreatitis. CONCLUSIONS: With adequate supervision and monitoring therapeutic plasma exchange represents a safe approach in pregnant women with FCS in order to reduce TGs and prevent pancreatitis. Therefore, we prevented potential complications for both mother and child.

Cerebral palsy: early diagnosis, intervention and risk factors.

Early diagnosis and intervention intensity were suggested to be crucial factor in cerebral palsy (CP) treatment. Herein we observed 347 children diagnosed for CP in Clinical Hospital Mostar, Bosnia and Herzegovina, and studied the relationship between (a) intervention start point and the final motor outcome, (b) intensity of treatment and final outcome, and (c) relationship between documented risk factors and early diagnosis of the CP. Our study suggests that it is possible to relatively accurately diagnose the CP in the first trimester. Previous miscarriages, sepsis and intracerebral haemorrhage were significantly related to early diagnosis, while delivery outcome, RDS, premature birth, intracerebral haemorrhage, sepsis, meningitis, hydrocephalus and convulsions were found as significantly related to final motor CP outcome. We have found no significant influence of the intervention intensity and final diagnosis. Our results support the idea that the intervention start point has to be considered as one of the most important factors for the effective intervention program. In future studies dealing with the CP interventions and risk factors, special attention should be paid to homogeneity and size of the sample, as well as necessity of including the non-treated controls in the investigation.