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: Screening for asymptomatic urinary retention (AUR) in older adult men at hospital admission to the internal medicine department has never been studied.

Objectives: To assess the incidence of AUR in older adult men at hospital admission, its risk factors, and its outcome.

Methods: The study comprised 111 older adult men aged ≥ 75 years who were admitted to three internal medicine departments. All men underwent post-void residual (PVR) urine volume measurement on the morning following admission by using a portable ultrasound bladder scan. AUR was defined as a PVR urine volume of ≥ 200 ml without symptoms. Men with AUR had a follow-up phone call concerning symptoms and urinary catheter status30 days following hospitalization.

Results: Seven (6.3%) men had AUR. Relative to the 104 men without AUR, they had significantly higher prevalence of severe dependency (6/7 vs. 33/104, 85.7% vs. 31.7%, (P = 0.007), cognitive impairment (5/7 vs. 19/104, 71.4% vs. 18.3%, P = 0.005), and use of anticholinergic agents (4/7 vs. 19/104, 57.1% vs. 18.3%, P = 0.033). A urinary catheter was inserted in one man (14.3%), but it was removed later during hospitalization. No symptoms were reported and no urinary catheter was inserted following hospitalization in men with AUR.

Conclusions: AUR in older adult men at hospital admission is uncommon and has a favorable outcome. Hence, screening for AUR in all older adult men at admission is not recommended, but it may be considered in severely dependent older adult men with cognitive impairment who use anticholinergic agents

Background: Despite progress in medical and surgical care the mortality rate of congenital diaphragmatic hernia remains high. Assessment of short-term outcome is important for comparison between different medical centers.

Objectives: To evaluate the short-term outcome of infants born with symptomatic CDH[1] and to correlate demographic and clinical parameters with short-term outcome.

Methods: We performed a retrospective cohort study in which demographic, obstetric and perinatal characteristics were extracted from infants' files. For comparison of categorical variables chi-square test and Fisher's exact test were used and for continuous variables with categorical variables the Mann-Whitney test was used. Sensitivity and specificity were estimated by receiver operator curve.

Results: The study group comprised 54 infants with CDH, of whom 20 (37%) survived the neonatal period. Demographic characteristics were not associated with survival. Regarding antenatal characteristics, absence of polyhydramnion and postnatal diagnosis were correlated with better survival. Apgar scores (above 5 at 1 minute and 7 at 5 minutes), first arterial pH after delivery (above 7.135) and presence of pulmonary hypertension were significantly correlated with survival. Also, infants surviving up to 6 days were 10.71 times more likely to survive the neonatal period.

Conclusions: The survival rate of symptomatic newborns with CDH at our center was 37% for the period 1988–2006. Prenatal diagnosis, Apgar score at 5 minutes and first pH after delivery were found to be the most significant predictors of survival. Prospective work is needed to evaluate the long-term outcome of infants with CDH.