Propranolol treatment for infantile hemangioma: a case series of sixty-two patients.

Infantile hemangiomas (IH) complicated by ulceration, disfigurement, functional impairment or life-threatening conditions need early, safe and effective treatment. This study explores the impact of propranolol on complicated IH. We report our experience of 62 patients treated with oral propranolol for complicated IH. The effect of propranolol was assessed using a score on a visual analogue scale integrated with echo, magnetic resonance or endoscopic findings. The average age at the beginning of the treatment was seven months [standard deviation (SD)±8.9], with a median of four months (range 1-53 months). The average age at the end of the treatment was 15 months (SD±8.4), with a median of 13 months (range 7-59 months). The mean treatment length was eight months (SD±3.2). Oral propranolol was successful in 95.2% of the patients in reducing the volume, the intensity of color and the elevation of IH. Statistically significant improvement of IH volume was observed in the first two months of therapy (P≤0.001), and between the second month and the end of the treatment (P<0.05). No significant bradycardia or hypotension occurred. Severe hypoglycemia occurred in one patient. Mild adverse effects were observed in seven patients. Our study demonstrates that propranolol administered orally at 2 to 3 mg/kg/day has a rapid therapeutic effect leading to remarkable shortening of the natural course of IH and it is safe in the majority of patients.

Severe hypokalemia and hypophosphatemia presenting with carpopedal spasm associated with rhabdomyolysis.

Background Severe hypokalemia, defined as serum potassium < 2.5 mEq/L, may lead to neuromuscular, gastrointestinal, and ECG abnormalities. Neuromuscular consequences of hypokalemia include weakness, cramps, rarely paralysis, eventually progressing to rhabdomyolysis. Case presentation We report a case of a 4-year-old girl presenting carpopedal spasm and rhabdomyolysis due to severe hypokalemia associated to hypophosphatemia and hypovolemia. At one month of age she underwent an ileal resection because of a neonatal necrotizing enterocolitis, and a bowel resection at two years of age, because of sub-occlusive episodes. The child had frequent episodes of diarrhoea and was treated with oral white clay (kaolin) and a restrictive diet. Three days prior the admission to the hospital she had numerous episodes of watery diarrhoea. Laboratory tests revealed severe hypokalemia, hypophosphatemia, normal calcium levels associated with marked dehydration. An ECG demonstrated sinus bradycardia, ST-segment depression, T-wave flattening, U-wave, and long-QTc. Symmetric carpal and pedal spasms were observed. A marked rise of creatinine phosphokinase and myoglobin associated to cola colored urine was observed. Intravenous supplementation of potassium phosphate as well as adequate volume repletion led to an improvement of the clinical condition, to the disappearance of carpal and pedal spasms, to normalisation of ECG.  Conclusions Careful electrolytes and volume supplementation led to the correction of potential life-threatening arrhythmias and obtained a complete recovery from carpopedal spasm and rhabdomyolysis. Dietary restriction and pharmacological preparations as kaolin have to be administered with caution to treat diarrhea in children and particularly in those who may present other pre-existing risk factors.

Pulmonary arterial hypertension in childhood: an unsual presentation with fibrosing mediastinitis.

Acquired stenosis of normally connected pulmonary veins is a rare condition in children, usually associated with mediastinal processes. It may present later with a less specific clinical picture, symptoms and signs mimicking chronic lung disease. Fibrosing mediastinitis is a rarer disorder of unknown etiology, although several suspected causes such as granulomatous diseases, characterized by fibrous tissue proliferation within the mediastinum, leading to respiratory and cardiac failure by bronchial obstruction or pulmonary hypertension.

Glycopyrrolate and theophylline for the treatment of severe pallid breath-holding spells.

Severe pallid breath-holding spells (BHSs) are based on parasympathetic hyperactivity, leading to cardiac asystole, pallor, brain ischemia, loss of consciousness, and reflex anoxic seizures. In recent years, an increasing number of patients with severe pallid BHSs have been successfully treated with pacemaker implantation. We present the case of a 13-month-old girl suffering from repeated severe pallid BHSs, causing asystole, loss of consciousness, and generalized anoxic seizures. She underwent treatment with oral glycopyrrolate, an anticholinergic drug, and an oral retard preparation of theophylline. The aim of the treatment was to decrease cardiac inhibition with glycopyrrolate and to bring about a positive chronotropic effect with theophylline. In our case, the combined therapy was effective in suppressing syncope and reflex anoxic seizures associated with BHSs This avoided the need for ventricular pacemaker implantation.

Adams-Stokes attack as the first symptom of acute rheumatic fever: report of an adolescent case and review of the literature.

BACKGROUND: Acquired complete heart block, in pediatric age is mainly the results of direct injury to conduction tissue during cardiac surgery or cardiac catheterisation. It can also be observed in different clinical settings as infectious diseases, neoplasia, and inflammatory diseases. It has a wide range of presentation and in some settings it can appear a dramatic event. Although a rare finding during acute rheumatic fever, with a transient course, it may need a specific and intensive treatment. CASE PRESENTATION: We report the case of an Adams-Stokes attack in an adolescent with acute rheumatic carditis and complete atrio-ventricular block. The attack was the first symptom of carditis.We reviewed the literature and could find 25 cases of complete atrio-ventricular block due to rheumatic fever. Ten of the 25 patients experienced an Adams-Stokes attack. Nineteen of the 25 patients were certainly in the pediatric age group. Seven of the 19 pediatric cases experienced an Adams-Stokes attack. In 16/25 cases, the duration of the atrio-ventricular block was reported: it lasted from a few minutes to ten days. Pacemaker implantation was necessary in 7 cases. CONCLUSION: Rheumatic fever must be kept in mind in the diagnostic work-up of patients with acquired complete atrio-ventricular block, particularly when it occurs in pediatric patients. The insertion of a temporary pacemaker should be considered when complete atrio-ventricular block determines Adams-Stokes attacks. Complete heart block during acute rheumatic fever is rare and is usually transient. Along with endocarditis, myocarditis and pericarditis, complete atrio-ventricular block has been recognized, rarely, during the course of acute rheumatic carditis.

Infective endocarditis following body piercing: Presentation of one case due to Gemella morbillorum and review of the literature.

BACKGROUND: In the last decade, body piercing has gained strongly in popularity. There are a few reports in the literature regarding infective endocarditis after body piercing. CASE REPORT: The case of an 18-year-old girl with heart failure and a six-month history of recurring fever is presented. Clinical and laboratory evaluation led to the diagnose of infective endocarditis due to Gemella morbillorum. Oral piercing was recognized to be the cause of bacteremia. Fourteen cases of infective endocarditis related to body piercing were found in the literature and the clinical, etiologic, pathogenic, and preventive aspects of this topic are presented. CONCLUSIONS: Infective endocarditis may be a complication of body piercing and has been observed in patients with heart lesions as well as in previous healthy people. Infective endocarditis can follow body piercing involving either mucous membranes or the skin. This patient is the first case of Gemella morbillorum endocarditis after body piercing reported in the literature. Careful observation for infection at the site of piercing and its treatment as well as professional training for operators seem to be the most effective way to minimize the risk of infective endocarditis after body piercing.

Transcatheter closure of patent ductus arteriosus: experience with a new device.

Transcatheter closure is the preferred method of treatment of patent ductus arteriosus (PDA). Detachable coils are widely used to close small ducts, while the Amplatzer duct occluder (ADO) is generally employed for moderate or large ducts. Recently a new device, the Amplatzer duct occluder II (ADO II), a nitinol flexible mesh, with a symmetrical design to provide high conformability for treatment of all types of PDA, has received the European Community mark approval. We report on one of the first experience, four cases (1 male, 3 female, age ranging from six months to seven years old) with different type and size of PDA treated with the new device. The use of this new Amplatzer duct occluder in our experience has the advantage of ease and safety of placement, conformability, stability, low profile catheters, adaptability for long ducts as in type E.

Syncope in children and adolescents: a two-year experience at the Department of Paediatrics in Parma.

BACKGROUND AND AIM OF THE WORK: Syncope occurs frequently in childhood and adolescence. It generally is benign, but may be a sign of pathology. The purpose of this study is to determine the incidence of syncope in children and adolescents in the Parma area, analyse the frequency of syncope due to cardiac, neurological or metabolic diseases, to identify a diagnostic procedure based on careful review, clinical features, and results of investigations. METHODS: The records of 156 children (age 1 to 18 years, mean age 10.5), who were referred to the Paediatric Department for syncope were reviewed. RESULTS: The incidence of syncope in the paediatric population of Parma during a 2-year period (2005-2006) resulted in 86.5/100.000 per year. The cause of syncope was discovered in 154/156 cases. Cardiovascular syncope was diagnosed in 121/156 cases (77.5%). Neurological syncope was diagnosed in 31/156 cases (20%), while metabolic syncope was diagnosed in 2/156 cases (1.25%). The most frequent type was the (benign) neurocardiogenic syncope, but 3 cases were due to arrhythmia, 9 cases were due to epilepsy, 1 case was due to subarachnoid hemorrage and 2 cases were due to hypoglicemia. CONCLUSIONS: Syncope frequently requires medical examination or admission to paediatric departments and, in the majority of cases, does not subtend serious diseases. Nevertheless, syncope always requires a complete medical evaluation in order to exclude underlying pathology. An electrocardiogram must complete the initial evaluation of all patients with syncope. Other investigations are required if clinical data and the ECG do not provide an etiological explanation of the episode.

Paradoxical emboli in children and young adults: role of atrial septal defect and patent foramen ovale device closure.

OBJECTIVE: To describe a multicenter experience with patent foramen ovale (PFO) and atrial septal defect (ASD) device closure for presumed paradoxical emboli in children and young adults (<35 years old). PATIENTS AND METHODS: Medical records were reviewed of patients who had device closure of an ASD or PFO, who were younger than 35 years, and who had a history of presumed paradoxical embolus between January 1999 and August 2005 at Mayo Clinic, Rochester, Minn, University of Parma, Parma, Italy, and Loyola University Medical Center, Maywood, Ill. RESULTS: Forty-five patients fulfilled the inclusion criteria. Median patient age was 29.0 years (range, 5.0-34.9 years), and 23 patients (51%) were male. Clinical diagnoses included the following: stroke, 30 (67%); transient ischemic attack, 13 (29%); myocardial infarction, 1 (2%); and renal infarct, 1 (2%). Overall, 42 patients (93%) had a PFO, and 3 (7%) had an ASD. Seventeen patients had known cardiovascular disease risk factors: tobacco use (10 patients), hypercoagulable states (7 patients), systemic hypertension (3 patients), and hyperilpidemia (2 patients). No major procedural complications occurred. Median follow-up evaluation was performed at 5.3 months (range, 2.5-40.0 months). Forty-four patients (98%) had no recurrent neurologic events and no residual atrial shunt by contrast transthoracic echocardiography. CONCLUSIONS: Cryptogenic ischemic events occur in young patients and have serious sequelae. The potential for paradoxical embolization through a PFO or an ASD should be assessed in all such patients. In our short-term follow-up, device closure was a safe alternative therapeutic option for children and young adults with presumed paradoxical emboli.

Atypical subacute thyroiditis caused by Epstein-Barr virus infection in a three-year-old girl.

Subacute thyroiditis is a rare disease in childhood and is considered postviral in origin. Epstein-Barr virus (EBV) involvement has been suspected, but just once demonstrated in an adult female. We report a case of subacute thyroiditis during infectious mononucleosis in a 3-year-old girl. The diagnosis of infectious mononucleosis was supported, in addition to the typical clinical symptoms, by presence of EBV DNA both in plasma and leukocytes. Subacute thyroiditis was diagnosed based on elevated thyroid hormone levels with thyrotropin (TSH) suppression, high inflammation markers and almost absent (99)TC uptake by thyroid. Moreover after 3 months from diagnosis thyroid function went back to normal as well as the (99)TC uptake.

Acute myocardial infarction in a child: possible pathogenic role of patent foramen ovale associated with heritable thrombophilia.

We report an 8-year-old girl who presented with clinical features of an acute myocardial infarction. The angiographic appearance of the coronary arteries was normal. A thrombophilic state caused by a homozygote genotype for the prothrombin G20210A mutation was detected, and a patent foramen ovale (PFO) with right-to-left shunting after Valsalva maneuver was demonstrated by transesophageal contrast echocardiography. No other embolic source was identified. We suggest that paradoxical embolization through a PFO resulted in a myocardial infarction in this young patient with hereditary thrombophilia. We closed the patient's PFO with a 25-mm PFO occluder. She was anticoagulated with warfarin for 6 months. After 6 months, a contrast echocardiogram showed no evidence of residual atrial shunt. There has been no evident recurrent paradoxical embolization.

Descending thoracic aorta to left pulmonary artery fistula after stent implantation for acquired left pulmonary artery stenosis.

This is a case report of a Fontan patient with previous Potts anastomosis who underwent stent implantation for left pulmonary artery stenosis. At follow-up the patient developed a fistula between the descending thoracic aorta and the left pulmonary artery at the site of the stent. This represents a late complication of stent placement.

[Transcatheter treatment of patent ductus arteriosus]. / Trattamento transcatetere del dotto arterioso pervio.

Isolated patent ductus arteriosus accounts for 10 to 18% of all cardiovascular malformations. Transcatheter closure of the patent ductus arteriosus has now become established practice with a high success rate and a low incidence of complications. Many devices have been proposed during the last 20 years. The use of some of these devices has largely been abandoned for different reasons: high costs, the need of large transvenous sheaths and the high incidence of late residual shunts. The devices currently available (Amplatzer duct occluder, buttoned device, DuctOcclud coil, Gianturco-Grifka vascular occlusion device, detachable coils) render the embolization of a patent ductus arteriosus relatively easy, with a short hospital stay. We feel that this is the treatment of choice for the majority of patients with a patent ductus arteriosus. The aim of this article was to review our experience and report the state-of-the-art in transcatheter patent ductus arteriosus therapy.