Marianna Fabi

Are nasal decongestants safer than rhinitis? A case of oxymetazoline-induced syncope.

Derivatives of Imidazoline usually act to stimulate peripheral alpha2 receptors causing vasoconstriction. In young children, however, they can also stimulate alpha2receptors in the cardiovascular and central nervous systems, possibly causing cardiovascular, neurological, and respiratory depression. These medications do not require medical prescriptions, so often parents use them, bypassing paediatricians. We report here a case of cardiovascular and neurological depression induced by oxymetalzoline in a toddler.

An Unusual Cardiomyopathy after Physical Stress in a Child

Takotsubo cardiomyopathy, or broken heart syndrome, is characterized by transient left ventricular dysfunction associated to chest pain, elevation of cardiac enzymes, and electrocardiographic changes, mimicking an acute coronary syndrome, especially in older women after a physical or emotional stress. It is extremely infrequent in children as well as after infective stress. We described a celiac 4-year-old girl, following a gluten-free diet, who developed features of cardiac failure few days after episodes of acute diarrhea with fever. The patient was treated with oral anticongestive therapy and intravenous immunoglobulins, and she had a dramatic and rapid improvement; echocardiographic features normalized in 48 hours.

Dilated cardiomyopathy due to hypocalcaemic rickets: is it always a reversible condition?

Nutritional rickets is still occasionally found in high-income countries, especially in populations at risk, and induced hypocalcaemia is a rare but possible cause of dilated cardiomyopathy. Although rare, physicians need to consider nutritional rickets in the differential diagnosis of hypocalcaemia cardiac failure, especially in high-risk populations such as immigrants. Despite being a reversible condition, the prognosis depends on the severity and time of diagnosis. We report two cases of exclusively breastfed infants with congestive cardiac failure due to hypokinetic dilated cardiomyopathy who had completely different outcomes. This report supports the need for prevention of this deficiency and underlies the role of vitamin D supplementation.

Persistent fever with chills and an endocardial mass in a child: an unusual presentation of Hughes-Stovin syndrome.

A 12-year-old boy with a right atrium endocardial mass was initially diagnosed as having Lemierres syndrome on the basis of previous mastoiditis and jugular vein and cerebral venous thrombosis. Lack of response to antibiotics, persistent high fever with chills, acute-phase reactants, and peripheral arterial pseudoaneurysms made us reconsider the diagnosis. Only after the late appearance of radiological pulmonary lesions and recognition of pulmonary artery aneurysms, Hughes-Stovin syndrome was diagnosed. Hughes-Stovin syndrome is an exceedingly rare vasculitis, especially in childhood, consisting of multiple pulmonary artery aneurysms and deep venous thromboses. The lack of formal diagnostic criteria and the rarity of the disease make the diagnosis very challenging, especially when respiratory complaints are not present at onset, as in the presented case. The treatment aims to reduce inflammation, although there is debate about anticoagulation therapy because of the risk of pulmonary haemorrhage.

A 74-year-old unoperated univentricular heart: the oldest reported survival.

Univentricular heart is a rare congenital heart malformation. Nowadays prognosis is considered to be strictly linked to surgical intervention, and survival into late adulthood is unusual. In some patients native haemodynamic circulation balances pulmonary and systemic blood flow, allowing long-term survival without the need for surgery. We report the case of a 74-year-old man with a univentricular heart in natural history, and we discuss the factors that might contribute to his extraordinary long-term survival.

Gastrointestinal presentation of Kawasaki disease: A red flag for severe disease?

Background Kawasaki disease (KD) is a febrile systemic vasculitis of unknown etiology and the main cause of acquired heart disease among children in the developed world. To date, abdominal involvement at presentation is not recognized as a risk factor for a more severe form of the disease. Objective To evaluate whether presenting abdominal manifestations identify a group at major risk for Intravenous immunoglobulin (IVIG)-resistance and coronary lesions. Methods Retrospective study of KD patients diagnosed between 2000 and 2015 in 13 pediatric units in Italy. Patients were divided into 2 groups according to the presence or absence of abdominal manifestations at onset. We compared their demographic and clinical data, IVIG-responsiveness, coronary ectasia/aneurysms, laboratory findings from the acute and subacute phases. Results 302 patients (181 boys) were enrolled: 106 patients with, and 196 patients without presenting abdominal features. Seasonality was different between the groups (p = 0.034). Patients with abdominal manifestations were younger (p = 0.006) and more frequently underwent delayed treatment (p = 0.014). In the acute phase, patients with abdominal presentation had higher platelet counts (PLT) (p = 0.042) and lower albuminemia (p = 0.009), while, in the subacute phase, they had higher white blood cell counts (WBC) and PLT (p = 0.002 and p < 0.005, respectively) and lower red blood cell counts (RBC) and hemoglobin (Hb) (p = 0.031 and p 0.009). Moreover, the above mentioned group was more likely to be IVIG-resistant (p < 0.005) and have coronary aneurysms (p = 0.007). In the multivariate analysis, presenting abdominal manifestations, age younger than 6 months, IVIG- resistance, delayed treatment and albumin concentration in the acute phase were independent risk factors for coronary aneurysms (respectively p<0.005, <0.005, = 0.005 and 0.009). Conclusions This is the first multicenter report demonstrating that presenting gastrointestinal features in KD identify patients at higher risk for IVIG-resistance and for the development of coronary aneurysms in a predominantly Caucasian population. Clinical trial registration 8/20014/O/OssN.

Upside-down position for the out of hospital management of children with supraventricular tachycardia

BACKGROUND The upside-down position is a little known modified Valsalva manoeuvre (VM). The aim of this study was to investigate the safety and the efficacy of the upside-down position for the treatment of paroxysmal SVT in children. METHODS Twenty-four paediatric patients followed for SVT were enrolled. The patients were assigned (1:1) to a standard VM or to an upside-down position at the first episode of SVT at home. If no cardioversion occurred, a second attempt was undertaken with the other VM. At the patients first relapse, the intervention protocol was applied in the opposite order at home. RESULTS The upside-down position compared to standard VM reached 67% vs 33% rate of cardioversion at a first attempt, followed by 50% vs 0% rate of cardioversion in patients who had failed the first attempt. After having reversed the order of intervention in case of SVT recurrence, we recorded 67% vs 25% and 71% vs 42% success rates in favour of the upside-down position. There were no adverse events. CONCLUSION The upside-down position was safe and tended to be more effective than standard VM for out of hospital SVT treatment. Doctors and parents should be more aware of this effective but overlooked manoeuvre.

Pulmonary embolism in complex corrected congenital heart disease

Pulmonary embolism is an uncommon but potentially fatal disease in children, especially with congenital heart disease where pulmonary perfusion depends on caval flow. Anticoagulation is the mainstay of therapy for children with pulmonary embolism. However, thrombolytic therapy can also be considered. The outcome of pediatric pulmonary embolism is uncertain and needs further investigation. We suggest increased awareness and a high index of suspicion in the presence of suggestive clinical signs in high-risk patients in order to initiate prompt diagnostic imaging and treatment.