Multiple oral and cerebral relapses of a Granular cell tumor (Abrikossoff Tumor) in a young girl affected by congenital dyserythropoietic anemia type II.

CASE REPORT: A 14-year-old girl presented with 1 cm large whitened lesion on the ventral surface of the tongue, appeared from 1 month. Past history showed congenital dyserythropoietic anemia type II. The lesion was excised and microscopic and immunohistochemical analyses were compatible with benign Abrikossoff tumor. Total body MRI was negative. After six months the patient presented a second tongue lesion and four months later another large painful lesion in the soft palate, with the same istological diagnosis. In addition, she had other multiple lesions: two apperead at pharyngeal level (not biopsied) that remain stable over time, and one at the pituitary gland. CONCLUSION: Granular cell tumors, with or without multiple lesions, are rare in children. About 50% of cases involve the head and neck region, with the tongue being the most affected site. Therapy is based on the surgical excision of the lesions; however some tumor forms, although their histological aspect of benignity, often have an important infiltrative power, making the therapeutic approach difficult, as in our case.

Extramedullary hematopoiesis in the facial sinus in a patient homozygous for Hemoglobin Lepore: the first case in literature.

Extramedullary hematopoiesis (EMH) is a proliferation of hematopoietic tissue outside the bone marrow. The most affected areas are paravertebral ones. We report the case of a patient with homozygous Hb Lepore, not regularly transfused since the age of four years until the age of 29 years, when paravertebral heterotopic masses were first observed. After about 10 years she started reporting clinical signs suggestive of sinusitis. Computed tomography (CT) and Magnetic Resonance Imaging (MRI) showed heterotopic masses in the ethmoid and in the frontal sinuses. Involvement of the sinuses of the large facial area represents a rare localization of EMH. Various cases have been reported in patients with thalassemia intermedia, but no case has been reported with HbLepore. The diagnostic gold standard is MRI, which provides highly accurate and clear images. The treatment is based on hydroxyurea and/or an intensive transfusional regime and sometimes on surgery.

Pediatric urticaria in the Emergency Department: epidemiological characteristics and predictive factors for its persistence in children.

Summary: Introduction. Acute urticaria (AU) in children is a common clinical manifestation responsible for admission to the emergency department (ED). We aimed to analyze the epidemiological characteristics of AU in children and to identify predictors of both severity and progression. Material and methods. We evaluated 314 children admitted to the ED with a diagnosis of AU. We analyzed information concerning its onset, duration, severity, possible triggering factors, and the persistence of symptoms after 1, 3, and 6 months. Results. The most common etiological factors were infections (43.9%); in up to 32.4% of cases, AU was considered as idiopathic. AU was significantly most common in males and pre-school children. At the 6-month follow-up, 9.5% of children presented a persistence of urticaria, mainly those with contact (44.4%) or idiopathic (30.4%) forms. Conclusions. The AU etiology identified by history in the ED may be a significant predictor of persistence after a first attack of AU.

Retinal vein occlusion in child with rare mutations in genes for thrombophilia.

INTRODUCTION: Retinal vein occlusions (RVOs) are rare in the younger population. Hematological pro-thrombotic factors are thought to be important in a minority, amplifying an atherosclerotic anatomical predisposition. CASE REPORT: Anotherwise healthy 13-year-old girl presented two episodes of sudden decreased vision in few months.Ophthalmological exams pointed out post-thrombotic intra-retinal hemorrhage. All investigations were normal, thrombophilia screen showed factor XII deficiency and genetic mutations of methylenetetrahydrofolate reductase (MTHFR), angiotesin convertin enzyme (ACE) and angiotensinogen (AGT). Two intravitreal injection of bevacizumab was administered with improving visual acuity; subsequently the patient did not report further episodes. DISCUSSION: In addition to traditional factors with procoagulant activity, factor XII deficiency plays an important role in thrombosis's mechanism. Its deficiency causes a marked prolongation of the activated partial thromboplastin time in the laboratory examination. Moreover also MTHFR, ACE and AGT could have been involved in this case, so it is important to evaluate these parameters in the differential diagnosis of RVOs.

Do you remember scurvy?

Scurvy, a disease caused by a deficiency of ascorbic acid (vitamin C), is not very common disease especially in pediatric age. In the late nineteenth century, instead, we assisted to increase incidence of this problem for the use of heated milk and convenience foods. We report two cases of scurvy: a child of 3 years old came to our observation for an important gums' stomatitis, fever, widespread petechiae and ecchymosis on the skin of the lower limbs; in the second moment he had pain in upper and lower limbs with difficulty in walking; a second child of 4 years came to our observation for pain lower limbs and maintained the posture of the legs down, inability to walking, with reduced muscle tone and trophism in all limbs; at the instrumental examination he had an alveolar hemorrhage. The two boys had a history of a diet completely lacking in fruits and vegetables; they were drinking almost only milk. They carried various laboratory and instrumental tests, in particular X-ray of lower limbs that showed "a thick sclerotic metaphyseal line with beak-like excrescences of tibiae and femur". The characteristic radiological appearance, the particular clinical aspects, the dietary history and the dosage of vitamin C, have led us to the diagnosis of scurvy. They started therapy with vitamin C and they had a progressive improvement in general condition.

A case of spontaneous gastric rupture in a 5 years girl.

We describe a case of spontaneous gastric rupture in a child of 5 years old. The patient reached us in a serious condition; the anamnesis was negative for traumatic events or gastrointestinal disorders. An abdominal X-ray and CT scan revealed free air and fluid in the abdominal cavity, leading to the diagnosis of gastro-intestinal perforation. Submitted to urgent surgery, a rupture of the posterior wall of the stomach was found that was treated with gastrectomy "à la demande". The surgery follow-up was regular. Morphological and immunohistochemical study showed some muscular abnormalities of the muscular gastric wall.

[Diet and lifesyle of a cohort of primary school children]. / Abitudini alimentari e comportamentali di una coorte di bambini della scuola primaria.

INTRODUCTION: The obesity is the disease of the new millennium, because it affects about 300 million people in the world, and especially it has a high prevalence in children. Obesity is a significant risk factor for cardiovascular disease, diabetes mellitus type II, hypertension, problems of adaptation and relationship with other, lower self-esteem and depression. AIMS: The objective of our study is to identify children at risk of overweight/obesity in order to primary prevention. We have organized meetings with children, families and school's members where we discussed the results of our investigation about the importance of healthy diet and lifestyle. PATIENTS-METHODS: The study was carried out on 545 children (282F, 263M), age 6.-10 years, of two primary schools in Catanzaro, from 2008 to 2010. The valuation parameters were: gender, age, weight, height, blood pressure and waist circumference. To children were also administered a questionnaire about dietary habits and lifestyle. STATISTICAL ANALYSIS: Fisher's test. RESULTS: We had that 62% of children was normal weight, 27% overweight, 11% obese. A particularly relevant datum is that the percentage of overweight-obese boys of 8 and 9 years old was higher (56%) than that of normal weight. We found cases of hypertension only in obese children. 98% of obese, 80% of overweight and 24% of normal weight children had a high waist circumference. We did not find differences in food quality among normal weight and overweight/obese children. Instead, we found significant differences in behavior between children: 90% of obese, 64% of overweight and 53% of normal weight children passed more than 2 hours in the afternoon watching television, playing computer and video games. 70% of normal weight, 82% of overweight and 95% of obese children practiced physical activity. DISCUSSION/CONCLUSION: Our study shows a alarming fact about the increase of the obesity in children. In particular the most important problem is that this condition could predispose to cardio-metabolic, endocrine, respiratory, musculoskeletal and psychological consequence. So it is important that everybody who lives with children, especially parents and school's members, educates children to have healthy lifestyles. These attentions may slow the worryng epidemic of obesity.

[Evaluation of the varus-valgus knee in a group of calabrian children]. / Valutazione del varismo-valgismo del ginocchio in un gruppo di bambini calabresi.

INTRODUCTION: In the early years of life, the angular deviation of knee causes anxiety in parents and alert to paediatricians. The problem is often an expression of physiological development of the lower limbs and correction occurs spontaneously. A small number of children has a true pathology, which can lead to functional problems, modification of the knee's axis and muscular-skeletal disorders. We analyzed the prevalence of pathological forms of varus-valgus knee and their association with lower limb dysmetria and scoliotic attitude. MATERIALS AND METHODS: We evaluated radiographies of the lower limbs of 60 calabrian children; we analyzed the mechanical axis of knee and articular spacing angles. We evaluated the X-ray of the spine with study of Cobb's angle and the gradient of the femoral head. RESULTS: 61.6% of children had a deflection angle of the knee: 6.7% physiological varus and 28.3% physiological valgus, 8.3% pathological varus and 18.3% pathological valgus. Mechanical axis deviation was: +15.6 degrees with articular spacing angle of about +2.4 degrees in pathological varus and +1.95 degrees with articular spacing angle of about -2.09 degrees in pathological valgus. 15% of children had pathological deviation and femoral head dysmetria (7.7 mm). 10% of children had association between pathological valgus/varus knee, dysmetria and scoliotic attitude ( 8.3% sacral-lumbar scoliotic attitude with Cobb's angle <20 degrees ). CONCLUSIONS: Without alarm we must pay attention to varus/valgus knee, because probably it will be a physiological deviation. In the pathological condition, it is important to consider the association with the limbs dysmetria that can lead to irreversible scoliotic postural deviation, if ignored for a long time. Unlikely early diagnosis and a subsequent correction of these defects could assurance a good development of skeletal structure.