Current Pediatric Reviews - Volume 10, Issue 3, 2014

Congenital nephrotic syndrome (CNS) refers to a disease presenting with massive proteinuria in association with hypoalbuminemia, hyperlipidemia, and edema at birth or within the first three months of life. In the past, most children with CNS had extremely poor prognosis and succumbed to various complications, usually within the first 6 months. Recent advancements in protein supplementation and nutritional support, renal replacement therapy and renal transplantation in infancy, render these patients to have much better outcomes [1-5]. However, there are still many hurdles in the management of this disease. Thromboembolism is an uncommon, yet important complication which the healthcare givers must be aware of. This article reviews the challenges in the management of the thrombotic complications with special emphasis on the unique characteristics of the newborn hemostasis system and anti-thrombin (AT) depletion in nephrotic syndrome. Due to the relatively low incidence of CNS in children and scarce information in the literature on the optimal management of the thromboembolic complications, most of the recommendations are based on the authors’ experience.

Iron deficiency remains the most common nutritional deficiency worldwide despite the fact that global prevention is a high priority. Recent guidelines suggest intake of red meat both in infants and toddlers to prevent iron deficiency. However frequent consumption of red and processed meat may be associated with an increased risk for cancer, cardiovascular disease and diabetes. Evidence also suggests that even in vegetarian diets or diets with little consumption of white or red meat, iron status may not be adversely affected. The Eastern Orthodox Christian Church dietary recommendations which is a type of periodic vegetarian diet, has proved beneficial for the prevention of iron deficiency and avoidance of excess iron intake. This paper aims to provide examples of meals for children and adolescents that may be sufficient to meet age specific iron requirements without consumption of red meat beyond the recommended consumption which is once or twice per month.

The quest for achieving optimal therapeutic outcomes in pediatric patients has evaded the healthcare professionals for long and often lack of child specific dosage forms and the associated events that follow with it have been considered to be major contributor towards suboptimal outcomes. Consequently, there have been sustained efforts over the years to address this issue with the enactment of legislations like Best Pharmaceutical for Children Act (BPCA), Pediatric Research Equity Act (PREA) and Pediatric Regulation by European Union (EU) to incentivise the participation of pharmaceutical industry towards development of child friendly dosage forms. Initiatives taken in past by organisations like World Health Organisation (WHO) and Drugs for Neglected Diseases Initiative (DNDi) to spur the development of child friendly dosage forms has helped to address issues pertaining to management of Human Immunodeficiency Virus (HIV) and malaria in pediatric patients. Present efforts aimed at developing child friendly dosage forms include oro-dispersible platforms including thin films and mini-tablets. Despite these leaps and advancements in developing better dosage forms for children, lower therapeutic outcomes in pediatric patients continue to remain an unresolved issue because of detrimental effects of additional factors such as parents understanding of label instructions and complexities involved in executing pediatric clinical studies thus requiring a concerted effort from pharmaceutical companies, academic researchers, parents and healthcare providers to work for better treatment outcomes in children.

Chvostek’s Sign was first described in 1876, as a clinical clue associated with patients who suffered from latent tetany, and is induced by percussion of the angle of the jaw. However, over the years many clinicians have called into question the strength of the association with latent tetany, particularly in paediatric practice. This review examines the variation in techniques used to elicit the sign in studies conducted on this phenomenon in children as well as how differences in the classification of a positive Chvostek’s sign have lead to varied reports on the strength of the association. Furthermore, an appraisal of the literature regarding the proposed mechanism of Chvostek’s sign is reported alongside analysing other diseases which have been associated with Chvostek’s sign to uncover any unifying mechanism for the presence of this clinical sign in children.

Cystic fibrosis (CF) is a common autosomal recessive genetic disorder where a deletion mutation and subsequent downstream alteration in transmembrane regulator proteins results in increased mucus viscosity. CF manifests clinically with chronic multisystem inflammation and recurrent infections. Nearly all children with CF have chronic sinusitis, and a large majority will have concurrent sinonasal polyposis. Chronic sinusitis and sinonasal polyposis in pediatric patients with CF can be managed conservatively initially, though most will fail medical management and require surgical intervention. Unfortunately, symptom resolution is marginal and polyp recurrence rates are high. Currently, no cure exists for CF and the mainstay of treatment is to provide symptomatic relief, and minimize disease morbidity.

Necrotizing enterocolitis (NEC) is a devastating and common disease of very low birth weight (VLBW) infants with a mortality rate of 10% to 50% and a significant cause of morbidity in survivors. The incidence of NEC has increased from 5% to 7% in the last decades and this rate is likely to rise because of the increased survival of infants born at 24 weeks gestation, which are at high risk of developing NEC. NEC etiology is multifactorial: ischemia, infections, cytokines, enteral feeding and reactive oxygen species or free radicals (FRs) may contribute to the disruption of the immature gut barrier. In particular, ischemia, hypoxia-reperfusion, infection and inflammation are mechanisms capable of producing high levels of FRs, perturbing the normal redox balance and shifting cells to a state of oxidative stress (OS). Despite advances in neonatal medicine, the early diagnosis of NEC remains a major challenge. Early clinical signs are non specific and the laboratory findings are not fully reliable. Therefore, its delayed occurrence after birth, its rapid onset, the highly fulminant nature, and its severe morbidity, as well as the possibility of progression to death, strongly require the identification of new prospective biomarkers specific for high NEC risk. There is evidences that OS biomarkers in cord blood allow the early identification of infants at risk for NEC and thereby can be used to develop novel therapies for this devastating disease which predominantly occurs in premature infants.

The experience of early adversity can increase one’s risk of psychopathology later in life. Extremely low birth weight (ELBW) provides a unique model of early adversity that affords us the opportunity to understand how prenatal and early postnatal stressors can affect the development of emotional, biological, and behavioural systems. Since the neuroendocrine system and emotion regulation can both be negatively affected by exposure to early adversity, and dysregulation in these regulatory systems has been linked to various forms of psychopathology, it is possible that these systems could mediate and/or moderate associations between early adversity, specifically ELBW, and later internalizing disorders. In this review, we discuss evidence of an early programming hypothesis underlying psychopathology and the identification of neuroendocrine markers of early adversity that may mediate/moderate the development of psychopathology.

Background: Ibuprofen and indomethacin are potent non-selective cyclo-oxygenase inhibitors and inhibit prostaglandin E2 synthesis. The patent ductus arteriosus (PDA) occurs in more than 70% of preterm infants weighing <1500 g. Prostaglandin E2 relaxes smooth muscle, tends to inhibit the closure of PDA, yields vasodilatation of the afferent renal arterioles and maintains glomerular filtration rate (GFR). Ibuprofen and indomethacin inhibiting prostaglandin E2 synthesis close PDA and reduce GFR with consequent decrease of urine output and increase of serum creatinine concentrations. Aims: The aims of this study are to give the definitive estimates of PDA closure rate following ibuprofen or indomethacin treatment and to evaluate the extent of renal side effects following the administration of these drugs to preterm infants. Other aims are to review the metabolism and the pharmacokinetics of ibuprofen and indomethacin in preterm infants with PDA. Methods: The bibliographic search was performed using PubMed and EMBASE databases as search engines, January 2013 was the cutoff point. Results: The %PDA closed by ibuprofen (n=24) and indomethacin (n=24) is 77.7±14.1 and 77.3±11.0, respectively. For ibuprofen, the gestational age of the infants included in the study ranged from 25.0 to 39.0 weeks (mean±SD=29.3±3.1 weeks). The %PDA did not correlate with the gestational age (p=0.2516). For indomethacin, the gestational age of infants included in the study ranged from 25.0 and 39.0 weeks (mean±SD=29.4±2.9 weeks). The %PDA did not correlate with the gestational age (p=0.3742). The treatment with ibuprofen reduces the urine output and increases the serum creatinine concentrations less extensively than indomethacin. The half-life (t1/2) of ibuprofen and indomethacin is lengthened and the clearance is reduced in preterm infants as compared with fullterm infants. Conclusions. Ibuprofen and indomethacin are equally effective in closing PDA. Treatment with ibuprofen decreases the risk of renal failure. Ibuprofen has the most favourable risk/benefit ratio. The rate of metabolism is reduced and t1/2 is lengthened in prematures as compared with term infants.

Introduction: Lymphangioma is a rare benign cyst caused by congenital malformation of the lymphatic systems that often occurs in the cervicofacial region. There is no consensus on its management: Observation, aspiration, injection, cryotherapy, electrocautery, radiation, laser, ligation and excision. Methods: We performed a literature search with the keywords “cystic hygroma”, “lymphangioma”, “management”, “OK 432” and “picibanil” from Medline, Embase and PubMed databases. Results: We present a review of the history, signs and symptoms, diagnosis, histology, classification and management options of cystic hygroma. Conclusion: There is no consensus on the treatment options. It should be individualised depending on the size of the lesion, anatomic localisation and complications.