Acute onset of bilateral visual loss during sildenafil therapy in a young infant with congenital heart disease

We report a case of posterior non-arteritic ischaemic optic neuropathy (NAION) causing bilateral visual loss in a 7-month-old female infant, after a therapeutic course with sildenafil, a phosphodiesterase type 5 inhibitors (PDE5i). The patient was affected by a complex cyanotic congenital heart defect and had undergone cavopulmonary anastomosis (Glenn operation) 3 months ago. After 2 months of recurring chylothorax, a course of oral sildenafil was administered, with the hypothesis that pulmonary vascular resistances were increased. Approximately 4 weeks later the acute onset of visual worsening and poor pupillary light reflex prompted the diagnosis of posterior NAION. Despite a rapid cessation of PDE5i and systemic treatment with corticosteroids, no visual recovery was noticed at 2-year follow-up. NAION has been associated with PDE5i therapy in adults, but to the best of our knowledge it is almost unheard of in children. We suggest close monitoring of visual function in children undergoing treatment with sildenafil

Oxygenation response to NO in newborns with severe pulmonary hypertension

Oxygenation Response to NO in Newborns With Severe Pulmonary Hypertensio

How much and for how long does the neonatal myocardium suffer from mild perinatal asphyxia?

Cardiac troponins can be useful in monitoring cardiac injury following perinatal distress. We report here an increase of cardiac troponin I (cTnI) to 2.84 microg/l at 3 weeks (age-related median: 0.07 microg/l) followed by normalization in a newborn with an uneventful clinical course after resuscitation at birth. Serial echocardiographs showed normal cardiac function. Such a time course of cTnI, not previously reported, could be due to either a greater sensitivity of biochemical markers than of instrumental tools or birth asphyxia. Larger studies are neede

A pediatric case of Miller Fisher syndrome with central involvement.

no abstract availabl

Unexpected death due to refractory metabolic acidosis and massive hemolysis in a young infant with Prader-Willi

To our knowledge, massive acute hemolysis has not yet reported among the catastrophic events related to the demise of patients with PWS. We believe that this phenomenon might have played a decisive role in the terminal course of our patient, even though the causative mechanism remains unclear. While further studies are needed in order to better clarify this issue, we suggest a close surveillance of these patients during acute diseases such as gastroenteritis and upper respiratory tract infections or when anticonvulsant treatment must be provide

Familial fetal-type rhabdomyoma of the tricuspid valve in the neonate: Malignant course for a benign disease

Familial fetal-type rhabdomyoma of the tricuspid valve in the neonate: malignant course for a benign disease

Diagnostic pitfalls in the assessment of congenital hypopituitarism.

BACKGROUND: The diagnosis of congenital hypopituitarism is difficult and oftendelayed because its symptoms are nonspecific.AIM: To describe the different clinical presentations of children with congenitalhypopituitarism to reduce the time for diagnosis and to begin a precocious andappropriate treatment.STUDY DESIGN: We analyzed a cohort of five children with congenitalhypopituitarism, describing their clinical, biochemical and radiologicalcharacteristics from the birth to diagnosis.RESULTS: As first sign of the disease, all of five patients presented a neonatal hypoglycemia, associated in four cases with jaundice. In all these four cases,the clinicians hypothesized a metabolic disease delaying the diagnosis, which wasperformed in only two cases within the neonatal period. In the other three cases,the diagnosis was formulated at 2, 5 and 8 years of life because there was severeand precocious growth impairment.CONCLUSIONS: It is important to suspect congenital hypopituitarism in thepresence of persistent neonatal hypoglycemia associated with jaundice and of aprecocious and severe reduction of the growth velocity in childhood. In all thesecases, it is necessary to undertake a hypothalamic-pituitary magnetic resonanceimaging scan as soon as possible, and to start appropriate treatment

Effect of atmospheric nitric oxide (NO) on measurements of exhaled NO in asthmatic children

The measurement of exhaled nitric oxide concentrations [NO] may provide a simple, noninvasive means for measuring airway inflammation. However, several measurement conditions may influence exhaled NO levels, and ambient NO may be one of these. We measured exhaled NO levels in 47 stable asthmatic children age 5 to 17 years and in 47 healthy children, gender and age matched. Exhaled [NO] in expired air was measured by a tidal breathing method with a chemiluminescence analyzer, sampling at the expiratory side of the mouthpiece. NO steady\u2010state levels were recorded. In order to keep the soft palate closed and avoid nasal contamination, the breathing circuit had a restrictor providing an expiratory pressure of 3\u20134 cm H2O at the mouthpiece. To evaluate the effect of [NO] in ambient air, measurements were randomly performed by breathing ambient air or NO\u2010free air from a closed circuit. Breathing NO\u2010free air, exhaled [NO] in asthmatics (mean \ub1 SEM) was 23.7 \ub1 1.4 ppb, significantly higher (P < 0.001) than in healthy controls (8.7 \ub1 0.4 ppb). Exhaled NO concentrations measured during ambient air breathing were higher (49 \ub1 4.6 ppb, P < 0.001) than when breathing NO\u2010free air (23.7 \ub1 1.4 ppb) and were significantly correlated (r = 0.89, P < 0.001) with atmospheric concentrations of NO (range 3\u2013430 ppb). These findings show that (1) exhaled [NO] values of asthmatic children are significantly higher than in healthy controls, and (2) atmospheric NO levels critically influence the measurement of exhaled [NO]. Therefore, using a tidal breathing method the inhalation of NO\u2010free air during the test is recommended. Pediatr Pulmonol. 1998; 26:30\u201334. \ua9 1998 Wiley\u2010Liss, Inc

Beneficial effect of sodium dichloroacetate in muscle cytochrome C oxidase deficiency

Beneficial Effect of Sodium Dichloroacetate in Muscle Cytochrome C Oxidase Deficienc

The collateral impact of COVID-19 emergency on neonatal intensive care units and family-centered care: Challenges and opportunities

The ongoing Coronavirus disease 2019 (COVID-19) pandemic is disrupting most specialized healthcare services worldwide, including those for high-risk newborns and their families. Due to the risk of contagion, critically ill infants, relatives and professionals attending neonatal intensive care units (NICUs) are undergoing a profound remodeling of the organization and quality care. In particular, mitigation strategies adopted to combact the COVID-19 pandemic may hinder the implementation of family-centered care within the NICU. This may put newborns at risk for several adverse effects, e.g., less weight gain, more nosocomial infections, increased length of NICU stay as well as long-term worse cognitive, emotional, and social development. This article aims to contribute to deepening the knowledge on the psychological impact of the COVID-19 on parents and NICU staff members based on empirical data from the literature. We also provided evidence-based indications on how to safely empower families and support NICU staffs facing such a threatening emergency, while preserving the crucial role of family-centered developmental care practices