Early puberty in end stage renal failure and renal transplant recipients.

Background Delayed puberty and hypogonadism are common in children with chronic kidney disease and in renal transplant recipients, but precocious puberty has rarely been reported in these populations. We describe six girls with precocious and/or early-onset, rapidly progressive puberty before and following renal transplantation. Methods Of 112 children under the age of 18 years (67 boys, 45 girls) who received renal transplants between 2010 and 2018, six girls presented with precocious or rapidly progressive early puberty at ages 6-7/12, 7-2/12, 7-4/12, 8, 8-8/12 and 8-11/12 years. Clinical evaluation included measurements of height, weight, body mass index (BMI), Tanner staging and bone age assessment. Gonadotropin responses to intravenous gonadotropin releasing hormone (GnRH) or intramuscular triptorelin acetate were determined. Results Tanner breast stage 3 was noted at 2-6 years following renal transplantation in five girls, four with preserved kidney function. One girl began puberty before renal transplantation. Peak luteinizing hormone (LH) and follicular stimulating hormone (FSH) levels were 6.5, 20.2, 7.83, 19.1, 9 and 2.2 mIU/mL and 13, 8.3, 8.01, 7.5, 8.1 and 7.7 mIU/mL, respectively. Treatment with an intramuscular slow-release formulation of triptorelin acetate every 4 weeks slowed progression of breast development. Conclusions Although delayed puberty is more common in children with renal disease, precocious puberty can also be seen. Evaluation of growth and puberty by a pediatric endocrinologist should be part of the routine care for all children following kidney transplantation.

Early neonatal hypoglycemia: incidence of and risk factors. A cohort study using universal point of care screening.

OBJECTIVE: The objective of this study is to determine the true incidence of early neonatal hypoglycemia and to confirm potential risk factors. STUDY DESIGN: The study was conducted at tertiary Medical Center in Israel, between June and September 2014. First blood glucose concentrations of all infants admitted to the nursery were measured using a "point of care" analyzer (Accu-Chek). We recorded risk factors for hypoglycemia such as birth weight, gestational age, maternal diabetes and demographics and analyzed their association with two hypoglycemia cutoffs: 40 and 47 mg/dl. RESULTS: Of 4000 newborns admitted during that period, 3595 were analyzed after excluding 405 who had missing data. Glucose level was obtained at a mean age of 74 ± 30 min. One hundred and twenty-four newborns (3.4%) had blood glucose levels below 40 mg/dl and 435 (12.1%) below 47 mg/dl. Univariate analyses revealed that gestational age, maternal diabetes, low birth weight (<2500 g), and twin delivery were associated with early neonatal hypoglycemia. Other risk factors (e.g. large or small for gestational age, birth weight >3800 g) were not. In multivariate analysis, gestational age remained the strongest association, while maternal diabetes and low birth weight became non-significant. CONCLUSIONS: We showed a high occurrence of early hypoglycemia in normal newborns using universal screening. The strongest risk factor was early gestational age. Surprisingly, incidence of early hypoglycemia in the presence of other classical risk factors was like that of the general population.

Octreotide-associated cholestasis and hepatitis in an infant with congenital hyperinsulinism.

Congenital hyperinsulinism (CHI) is the most common cause of prolonged hypoglycemia in the neonate. It is caused by several genetic mutations that interfere with the cascade of normal insulin secretion from pancreatic beta cells. Octreotide, a somatostatin analog, suppresses insulin secretion from pancreatic beta cells, and is an effective therapy used for both short and long term in the treatment of CHI. It is well tolerated in most patients; however, several adverse effects have been reported, most of them mild and transient. Impaired liver function has been described previously in few children. Here, we describe about a child with CHI treated with continuous intravenous octreotide who developed cholestasis and hepatitis after a short period of treatment. This combination of liver effects with a short duration of treatment has not been reported previously with octreotide use in this population.

Effect of eutectic mixture of local anesthetics (EMLA) for pain relief during suprapubic aspiration in young infants: a randomized, controlled trial.

OBJECTIVES: The purpose of this study was to determine whether a eutectic mixture of local anesthetics (EMLA) cream reduce the pain experienced by newborns and young infants undergoing suprapubic aspiration (SPA). METHODS: A single-center, randomized, placebo-controlled, double-blind, prospective study including 52 infants 1 to 60 days of age undergoing SPA to obtain urine for culture. Twenty-four infants were randomly assigned to receive 1-g EMLA cream 1 hour before the procedure, whereas 28 were randomly assigned to receive placebo cream 1 hour before the planned procedure. Pain symptoms were measured using the Premature Infant Pain Profile (PIPP). RESULTS: The mean PIPP scores were significantly lower in the EMLA group compared with the placebo group (8.90+/-3.04 vs. 10.46+/-2.29; P=0.038). There were significantly fewer patients in the EMLA group who were scored as having some degree of pain (defined as PIPP score >6) in comparison with the placebo group (70% vs. 96%; P=0.025). DISCUSSION: Newborns and infants undergoing SPA experience pain during the procedure. Application of EMLA cream 1 hour before SPA is effective in reducing the pain associated with SPA. In situations in which an SPA is not emergent, strong consideration should be given to the use of EMLA.