Israel Medical Association Journal

Carbamoyl phosphate synthetase 1 (CPS1; MIM *608307; E.C. 6.3.4.16) is the first rate-limiting enzyme of the urea cycle, an essential metabolic pathway for ammonia detoxification. CPS1 deficiency (CPS1-D) is characterized by severe hyperammonemia during disease exacerbations. During a metabolic crisis, children with CPS1-D are admitted with vomiting, altered mental status, and high serum levels of ammonia. Rapid normalization of ammonia level ameliorates neurological outcome [1,2]. The first-line treatment for hyperammonemia in these patients is ammonia scavengers in combination with citrulline or arginine and high-calorie supplementation while controlling protein intake [1].

Background: Temporary abdominal closure (TAC) surgical technique relates to a procedure in which the post-surgical abdominal wall remains open in certain indications. The Bogota bag (BB) technique is a tension-free TAC method that covers the abdominal contents with a sterilized fluid bag. There are very few reports of pediatric patients treated with this technique.

Objectives: To describe our institution’s 15 years of experience using the BB technique on pediatric patients.

Methods: A retrospective cohort study describing our experience treating patients with BB was conducted. The medical files of 17 pediatric patients aged 0–18 years were reviewed.

Results: Between January 2000 and December 2014, 17 patients were treated with BB at our medical center (6 females, median age 12 years). Indications for BB were a need for a surgical site re-exploration, mechanical inability for primary abdominal closure, and high risk for ACS development. Median BB duration was 5 days and median bag replacement was 2 days. Median ICU length of stay (LOS) was 10 days and hospital LOS was 27 days. The ICU admission and BB procedure was tolerated well by 6 patients who were discharged home without complications. Of the remaining 11 patients, 6 patients died during the admission (35%) and the others presented with major complications not related to the BB but to the patient's primary disease.

Conclusions: This report represents the largest series of children treated with BB. The technique is simple to perform, inexpensive, and has very few complications.

Background: Little is known of the outcome of pregnant patients with previously diagnosed dilated cardiomyopathy. These patients are usually firmly advised against continuation of the pregnancy.

Objectives: To examine the usefulness of serial echocardiographic follow-up and plasma N-terminal pro-B type natriuretic peptide levels in the management of pregnant women with preexisting DCM[1].

Methods: We prospectively enrolled pregnant women with DCM either known or diagnosed in the first trimester. Clinical examination and serial echocardiography studies at baseline, 30 weeks gestation, peripartum, and 3 and 18 months postpartum were performed. Blinded NTproBNP[2] levels were obtained at 30 weeks, delivery and 3 months postpartum.

Results: Between June 2005 and October 2006 we enrolled seven women who fulfilled the study criteria. Delivery and postpartum were complicated in 3 patients (42%): 2 with acute heart failure, which resolved conservatively, and 1 with major pulmonary embolism. The left ventricular ejection fraction was stable throughout the pregnancy (35% ± 2.8 at baseline, 33% ± 2.9 at 30 weeks) and postpartum (35% ± 2.8 at 1 day, 34% ± 3.1 at 90 days). Similar stable behavior was observed regarding left ventricular dimensions: LV[3] end-systolic diameters 43.3 ± 2.7 mm and LV end-diastolic diameters 57.3 ± 3.3 mm at baseline compared with 44.1 ± 3.1 mm and 58.7 ± 3.1 mm postpartum, respectively. The NT-ProBNP levels rose significantly peripartum in all three patients with complications.

Conclusions: Serial NT-proBNP levels, as compared to echocardiography, may be a better clinical tool in monitoring and management of pregnant women with preexisting DCM. An early rise in NT-ProBNP level appears to predict the occurrence of adverse events.