Search

The article describes the case of severe renal failure in a 36-week old baby girl whose mother had taken Olmesartan for hypertension during her last month of pregnancy. The baby died of cardio-circulatory failure 45 days after her birth with kidneys showing severe tubular dysgenesis. Also included is information on sartans as treatment for arterial hypertension and role of renin-angiotensin components in kidney development. The study concludes that exposure to sartans during the last month of pregnancy can cause congenital malformations, renal impairment, and fetal or neo-natal death.

Neonates face heightened susceptibility to drug toxicity, often exposed to off-label medications with dosages extrapolated from adult or pediatric studies. Premature infants in Neonatal Intensive Care Units (NICUs) are particularly at risk due to underdeveloped pharmacokinetics and exposure to multiple drugs. The study aimed to survey commonly used medications with a higher risk of ototoxicity and nephrotoxicity in Spanish and Italian neonatal units. A prospective cross-sectional study was conducted in Italian and Spanish neonatal units using a web-based survey with 43 questions. A modified Delphi method involved experts refining the survey through online consensus. Ethical approval was obtained, and responses were collected from January to July 2023. The survey covered various aspects, including drug-related ototoxic and nephrotoxic management, hearing screening, and therapeutic drug monitoring. Responses from 131 participants (35.9% from Spain and 64.1% from Italy) revealed awareness of drug toxicity risks. Varied practices were observed in hearing screening protocols, and a high prevalence of ototoxic and nephrotoxic drug use, including aminoglycosides (100%), vancomycin (70.2%), loop diuretics (63.4%), and ibuprofen (62.6%). Discrepancies existed in guideline availability and adherence, with differences between Italy and Spain in therapeutic drug monitoring practices., Conclusions: The study underscores the need for clinical guidelines and uniform practices in managing ototoxic and nephrotoxic drugs in neonatal units. Awareness is high, but inconsistencies in practices indicate a necessity for standardization, including the implementation of therapeutic drug monitoring and the involvement of clinical pharmacologists. Addressing these issues is crucial for optimizing neonatal care in Southern Europe., What Is Known: • Neonates in intensive care face a high risk of nephrotoxicity and ototoxicity from drugs like aminoglycosides, vancomycin, loop diuretics, and ibuprofen. • Therapeutic drug monitoring is key for managing these risks, optimizing dosing for efficacy and minimizing side effects., What Is New: • NICUs in Spain and Italy show high drug toxicity awareness but differ in ototoxic/nephrotoxic drug management. • Urgent need for standard guidelines and practices to address nephrotoxic risks from aminoglycosides, vancomycin, loop diuretics, and ibuprofen., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Objective: Aim of the study is to evaluate disorders related to positional plagiocephaly and introduce a new model of early intervention based on the osteopathic integrated approach., Methods: We review clinical experience of the "Program for Neurodevelopmental Follow-up and Pediatric Osteopathy", a service dedicated to newborns at risk for developmental disorders., Results: We present clinical data of 310 newborns followed during first years of life. Data analysis examines perinatal history, general features and disorders that could be related to plagiocephaly., Conclusions: The experience confirms that plagiocephaly is not only a problem regarding the shape of the head, it involves the functions. In our Service most babies (81%) with positional plagiocephaly showed isolated or associated disorders that had an impact on growth, behavior and development. The early intervention based on the osteopathic integrated approach is addressed not only to the cranial shape but consider the baby as a whole, and the environment where he lives., Competing Interests: Declaration of competing interest The authors have no conflicts of interest relevant to this article to disclose., (Copyright © 2020 The Authors. Published by Elsevier B.V. All rights reserved.)

Inactivating mutations in the SLC12A3 gene that encodes the thiazide-sensitive co-transporter causes Gitelman syndrome. The main features of this syndrome include normal or low blood pressure, hypokalemia, metabolic alkalosis, hypomagnesemia, hypocalciuria, and hyperreninemia. These patients are at low risk for preterm birth and do not present with symptoms before school age. As a consequence, the condition is usually diagnosed in late childhood or in adult life. We report on four patients, two pairs of prematurely born twins, in whom hypokalemia was demonstrated early in life. In these children, a tendency towards hypokalemia was first noted during the third week of life. Overt hypokalemia subsequently appeared associated with normal blood pressure, hypochloremia, hyperreninemia, and an inappropriately high fractional excretion of potassium and chloride. Molecular biology studies failed to detect mutations in the SLC12A1, KCNJ1, and CLCNKB genes responsible for the Bartter syndromes type I, II and III, respectively. Compound heterozygous mutations in the SLC12A3 gene were detected in both pairs of twins: a frameshift mutation in exon 10 (c.1196_1202dup7bp), leading to the truncated protein p.Ser402X, and a missense mutation in exon 11, p.Ser475Cys (c.1424C>G) in the first pair; two missense mutations, p.Thr392Ile (c.1175C>T) in exon 9 and p.Ser615Leu in exon 15 (c.1844C>T), in the second pair. In conclusion, the diagnosis of Gitelman syndrome deserves consideration in infants with unexplained hypokalemia.

Sartans are selective type 1 angiotensin II receptor antagonists that are used for treatment of arterial hypertension. We report a case of severe renal failure required dialysis after the use of olmesartan in the last month of pregnancy. Exposure to sartans during the last period of gestation seems to be associated with high risk of congenital malformations. It is important to stress that the use of these drugs during pregnancy must be avoided, especially in the third trimester.

Unlabelled: GM(1) gangliosidosis is a rare disorder characterized by deficiency of the ss-galactosidase enzyme, with the resulting accumulation of glycolipids, oligosaccharides and especially GM(1) ganglioside. It can be classified into three clinical types according to the time of onset: infantile, juvenile and adult form. We report a case of GM(1) gangliosidosis presenting with fetal hydrops at 24 wk of gestation. The parents were consanguineous; the baby, born at 35 wk of gestation, was dysmorphic and presented severe generalized oedema. The most common cause of fetal hydrops was excluded. A lysosomal storage disease was suspected, and GM(1) gangliosidosis was diagnosed. The child developed severe growth and mental retardation and died when she was 21 mo old., Conclusion: We suggest that the possible association between inborn errors of metabolism and antenatal ascites should be considered, in order to offer genetic counselling due to the high recurrence risk and the availability of early antenatal diagnosis.

Two infants on high-frequency oscillatory ventilation for chronic lung disease and severe respiratory failure, received a bolus of warmed and oxygenated perfluorodecalin up to residual functional capacity, followed by a continuous infusion of 6 ml/kg/hour. Our aim was to improve gas exchange without increasing ventilatory-induced lung injury. Heart rate, oxygen saturation, blood pressure, and TcPO(2)/TcPCO(2) were continuously monitored during treatment. Arterial blood gas was evaluated every 3 hours. Both patients showed improvement of gas exchange with a 13.6 and 12.5% reduction of oxygenation index, respectively. High-frequency partial liquid ventilation is an experimental ventilation technique that could be considered as rescue treatment, to improve oxygenation in subjects with critical respiratory failure. This method could probably produce less damage, than other ventilation modes, to severely injured lungs.

Objective: Pneumothorax (PNX) is a relatively common complication of nasal-CPAP (N-CPAP). Aim of the study was to identify prognostic factors of its onset., Methods: Seventy-seven newborns, admitted from January to December 2002 to the Neonatal Intensive Care Unit of Brescia, who were treated with N-CPAP with Infant Flow System as first intention, were included. Gestational age and birth weight were (mean +/- SD) 33.7 +/- 3.02 weeks and 2.047 +/- 684 grams, respectively. Infants were put on N-CPAP at 2.7 +/- 4.1 hours of life. The duration of treatment was 27.7 +/- 27.7 hours., Results: Fifty-one neonates improved and N-CPAP was discontinued, 26 worsened and required intubation and mechanical ventilation. Eight of them developed PNX (10,3%). No significant differences were found among the three groups (improved, worsened without PNX and worsened with PNX) concerning mode of delivery, gestational age, birth weight and blood gases. The patients with PNX needed a FiO2 28% higher than the initial value after 12 hours of treatment, and 46% higher at 24 hours (p = 0,017). At diagnosis, FiO2 was 53,5% higher than the initial value (p = 0,005)., Conclusion: A 40% increase of FiO2, during the first 24 hours of N-CPAP may represent an useful marker to identify the infants at high risk of developing a pneumothorax.

Steroids administrated antenatally to the mothers improve postnatal outcomes of the newborns with pleiotropic effects. Furthermore steroids have been used in preterm infants to prevent or treat chronic lung disease. Synthetical glucocorticoids readily cross placental barrier and reach significant pharmacologic levels in the fetus: besides their well known pulmonary effects they have a concomitant maturational effect of postnatal renal function in preterm infants both with a direct and indirect effect. Endogenous and exogenous glucocorticoids play a role in the maintenance of glomerular filtration (GFR). The antenatal administration of steroids increases the GFR, in association to the maturation of the tubular function. According to different studies the improvement of renal function, expressed by the increase of GFR, is only partially referable to the increase of MAP and the improvement of the cardiovascular status, while it was imputable to a direct renal effect of the steroids, especially on the renal blood flow, on functional glomerular surface area available for filtration and on the glomerular filtrate of the single cortical nephron. However debate remains about the mechanism through which steroids would act on the renal vascular smooth muscolature. The increase the GFR observed after the antenatal administration of glucocorticoids in premature fetuses is also accompanied by an increase of urinary flow and of fractional excretion of sodium. Glucocorticoids would increase the proximal reabsorption of sodium increasing directly the function and the expression of the sodium transporters and both indirectly and directly increasing the activity of Na-K-ATPase. In extremely low weight antenatal administration of betamethasone or dexamethasone was associated with lower estimated insensible water loss, secondary to a direct maturational effect in the skin epithelial barrier, as well as an increased reabsorption of the fetal lung fluid. Moreover antenatal glucocorticoid administration was associated, at birth, to a significant suppression of plasma renin activity and angiotensin II in comparison to the controls. Despite the wide use of the steroidal therapy in the prevention of the bronchopulmonary dysplasia, only few articles, in literature, analyse the effects of glucocorticoids on postnatal renal function, such as the increase in urinary flow. The authors think that steroids contribute in a meaningful way to the clinical improvement observed in children with BPD through the maturative action on the premature kidney with effect both at glomerular and tubular level.

Patient hospitalization was compared in 207 pediatric patients (age < or = 15 years at the start of dialysis) on chronic peritoneal dialysis (CPD) (127 patients) or center hemodialysis (HD) (80 patients), treated in 17 dialysis centers during the period 1989 to 1994, and followed up for at least three months. The hospitalization rate was expressed as hospital days per patient-month, and was calculated on the overall period of treatment and separately for the first and second year. Since the age at start of dialysis markedly differed between CPD (8.2 +/- 4.7 years) and HD (11.2 +/- 2.9 years) patients (with no HD patient younger than five years), results are separately presented in three patient groups: CPD patients aged < 5 years (A); CPD patients aged five to 15 years (B); HD patients (C). The duration of hospitalization was subdivided according to the following different causes: routine (monitoring of dialysis adequacy), complications of the modality, patient primary renal disease, and other causes. The results are presented in Table 1. A statistically significant difference in total days hospitalized was found between each of the two groups of CPD patients and the HD patients; the results for hospitalization for dialysis-related complications were higher in the group of younger children on CPD, while the difference between the two age-matched groups of patients on CPD and HD was not significant.

Chronic peritoneal dialysis (CPD) is the first treatment modality for most infants with end-stage renal failure; this group of patients shows peculiar clinical and technical problems. We present the data from a National Registry on 22 children starting CPD under one year of age, representing 11.6% of the total population of the Registry (189 patients). Mean weight at start of CPD was 6.1 +/- 1.8 kg and duration of dialysis was 22.1 +/- 15.5 months. During the follow-up period, 9 patients were transplanted, 1 was shifted to hemodialysis, and 4 died. Patient survival was 89.1% and 82.2% at 1 and 2 years (97.9% and 96.5% in the group of 167 older children); technique survival results were 89.1% at 1 year and 77.1% at 2 years (vs 92.5% and 85.7%, respectively). The incidence of peritonitis was 1 episode every 15.6 CPD-months (1:16.1 in the older children). Catheter-related complications occurred more frequently in infants (1:11.8 vs 1:17 episode:CPD-months), even if this difference was not statistically significant. Statural growth was on average -0.29 +/- 0.66 SD/year with a significant improvement between the first (-0.50 +/- 0.79) and the second (+0.23 +/- 0.77) year of CPD. Our data confirm that infants represent a higher risk group and that they can be treated satisfactorily with CPD while awaiting renal transplantation.

Anemia, through a hyperkinetic state, is an important contributor to myocardial function impairment. To determine the cardiovascular effects of recombinant human erythropoietin (rHuEPO) therapy, 10 chronic peritoneal dialysis (CPD)-treated anemic children were studied before and during 18 months of treatment. The following parameters were recorded: hemoglobin (Hb) [percent of target level (TL) = x-2 standard deviations of normal Hb values for age and sex], heart rate (HR, beats/minute), mean arterial pressure (MAP, mmHg), end-diastolic left ventricular diameter (EDLVD, mm/sm BSA), shortening fraction (SF, percent), and interventricular septal thickness (IVS, mm/sm BSA). Student's t-test for paired data showed (vs time before treatment, T0) a progressive increase in Hb, a progressive decrease in HR, and a progressive increase in MAP. EDLVD progressively decreased, while SF and IVS remained unchanged throughout the study. Regression analysis showed a close correlation between anemia correction and decrease of HR (p < 0.01), while no correlation was found between Hb and EDLVD or SF, IVS, or MAP. Our data indicate that anemia correction in these patients is mainly associated with a decrease in hyperkinetic state (HR reduction with SF unvaried), while left ventricular function and dimensions remain normal, despite an increase in MAP.

Nightly intermittent peritoneal dialysis (NIPD) is an automated form of intermittent peritoneal dialysis which has potential medical and psychosocial advantages in comparison with CAPD/CCPD due to the lack of daytime exchanges. Data on solute/water removal in children on NIPD are nevertheless scarce, so that no clear indications for NIPD can yet be formulated in pediatric age. For this reason, 12 patients, mean age 10.49 +/- 5.81, mean body weight 23.73 +/- 10.92, with a residual creatinine clearance 1.70 +/- 2.30 ml/min/1.73 sqm, on NIPD for 14.7 +/- 5.4 months, underwent clearance studies over 3 days. Mean dialysis infusion volume was 460.08 +/- 196.30 ml/kg/day, with 10.33 +/- 1.22 h dialysis time. Peritoneal creatinine and urea clearances were 6.36 +/- 2.96 and 8.49 +/- 3.35 1/day/1.73 sqm, respectively. Combined creatinine and urea clearances averaged 6.12 +/- 2.21 and 6.96 +/- 2.16 ml/min/1.73 sqm, resulting in serum creatinine and urea values of 7.78 +/- 1.90 and 115.58 +/- 29.93 mg/dl, respectively. Ultrafiltration rate was 16.94 +/- 16.34 ml/g glucose absorbed. NIPD provided similar or improved solute and water clearances compared with those reported in children and adults on CAPD/CCPD, without inconvenient long periods in bed. These data indicate that NIPD is a suitable treatment in pediatric end-stage renal disease.