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G Simonini

Publications and source records attributed to G Simonini.

29 records · Page 2Linked to original sources
PubMedDate not supplied

Recurrent monoarthritis in an 11-year-old boy with occult coeliac disease. Successful and stable remission after gluten-free diet.

A case of occult coeliac disease (CD) presenting with recurrent monoarthritis in a boy aged 11 years is reported. The case is unique due to the association of occult untreated CD and arthritis in childhood. Peripheral or axial arthritis as a first manifestation of occult CD has been described in adult patients, with an interval between the arthritis and CD of up to 15 years. In our case the interval between the appearance of arthritis and the diagnosis of CD was 2 years. The boy was asymptomatic for bowel disease and his nutritional status was normal. The diagnosis of CD was established using anti-gliadin (AGA) and anti-endomysium (EMA) antibody tests and was confirmed by small bowel biopsy. The introduction of a gluten-free diet resulted in the persistent remission of arthritis. As the treatment of CD-associated arthritis is based on dietary therapy, physicians should be alert to the possibility of occult CD in any child with arthritis of unclear origin.

Arthritis, Juvenile↗
PubMedDate not supplied

Bcl-2, p53 and c-myc expression in juvenile dermatomyositis.

OBJECTIVE: To investigate p53, bcl-2 and c-myc expression in muscle biopsies from children affected with juvenile dermatomyositis (JDM) and to verify a possible dysregulation of programmed cell death in this autoimmune disease. METHODS: Ten muscle biopsies of children affected with JDM were formalin fixed and paraffin embedded. After haematoxylin and eosin staining, immunohistochemistry was performed employing monoclonal antibodies, anti-p53, anti-bcl-2 and anti-myc. Two normal muscle biopsies were studied as controls. RESULTS: In the biopsies of JDM, two different patterns of myofibers damage were observed: the first, with zones characterised by necrosis; and the second, with zones where an apoptotic process was dominant. Immunoreactivity for bcl-2 was positive in 8 out of 10 biopsies. P53 and c-myc expression were not present in any case. No relationship between the degree of bcl-2 immunostaining and the disease course or outcome was observed. CONCLUSIONS: The over-expression of bcl-2 protein in JDM may suggest a dysregulation of apoptosis in myofibers. Further studies are required in order to better understand the role of our data in the pathogenetic pathways of the disease.

Apoptosis↗
PubMedDate not supplied

Acute febrile cholestasis as an inaugural manifestation of Kawasaki's disease.

We report a child who developed acute febrile cholestasis with jaundice and pruritus as the inaugural manifestation of Kawasaki's disease (KD). The severe obstructive icterus and hydrops of the gallbladder required cholecystectomy that was not followed by remission of the fever and cholestasis. KD was suspected after the exclusion of all infectious, metabolic and neoplastic conditions responsible for acute cholestasis. The administration of intravenous gammaglobulin (IVGG) promptly induced defervescence and improvement of the patient's general condition. Mucocutaneous alterations, peeling of the digits, right cervical lymph node enlargement and bilateral non-suppurative conjunctivitis supporting the diagnosis of KD developed 14 days after the appearance of jaundice. No coronary abnormalities had developed after 2 years of follow-up. We conclude that this syndrome should be suspected in any child with febrile cholestasis of unknown origin, in order that coronary involvement may be prevented by the administration of IVGG.

Acute Disease↗
PubMedDate not supplied

Severe abdominal involvement as the initial manifestation of cutaneous polyarteritis nodosa in a young girl.

We report a young girl who developed ingravescent intestinal symptoms as the first manifestation of cutaneous polyarteritis nodosa (PAN) while the typical skin nodules developed later during the disease course. Cutaneous PAN predominantly affects children and presents with crops of painful skin nodules in the medial aspect of the foot, often preceded by sore throat. Visceral manifestations including gut involvement are commonly associated with the classical form of PAN while they are rarely reported in the cutaneous form. In our patient the severity of the abdominal symptoms required a laparoscopy, which revealed diffuse erythematosus swelling of the intestine on the serosal side. The administration of penicillin and steroids was followed by a dramatic improvement in the disease course. Chronic anterior uveitis developed 4 months after the disease onset and responded to local treatment. At a 2-year follow-up the girl is in good condition under prophylaxis with benzathine-penicillin with no recurrence of the illness. Our case confirms that cutaneous PAN is often related to streptococcal infection, and suggests that ASO titers should be determined in children with vasculitides to ensure a timely diagnosis and treatment of the condition if present.

Abdominal Pain↗
PubMedDate not supplied

Kawasaki's disease in northern Italy: a multicenter retrospective study of 250 patients.

OBJECTIVE: To evaluate (i) the clinical course and outcome in a cohort of 250 children affected with Kawasaki disease (KD) in northern Italy; (ii) the prevalence of coronary aneurysms and their correlation with treatment; and (iii) the prevalence of incomplete and atypical KD in the series as a whole. RESULTS: The male to female ratio was 1.8 to 1 and the median age at diagnosis 37 months. The majority of cases occurred in children under five years, with the youngest patient aged 51 days. Median duration offever was 8 days; median erythrocyte sedimentation rate (ESR) and C Reactive protein (CRP) values, performed before any treatment, were 77 mm/1st h and 10.4 mg/dl respectively. Median haemoglobin (Hb) levels was 9.2 g/dl and median platelet (PTL) count 408,000/mm3. Out of 250 patients, 209 (83.6%) fulfilled all criteria for the diagnosis of KD whilst 41/250 (16.4%) had an incomplete disease and 11/250 (4.4%) had an atypical onset. Fifty-four children (21.6%) were treated with aspirin alone (50-80 mg/kg/day, during the acute phase), while 196/250 (78.4%) were given aspirin (50-80 mg/kg day, during the acute phase and then 3-5 mg/Kg/day) and high-dose intravenous gammaglobulin (IVIG, 400 mg/Kg/day or 2 g/Kg/day). Coronary artery abnormalities, including aneurysms and dilatation, were reported in 7/54 patients (5 males and 2 females) who had not received IVIG and 53/196 patients (27 males and 26 females) who were treated with the IVIG in addition to aspirin. Other clinical manifestations included abdominal pain/diarrhoea (11), arthritis (10), hydrops of the gallbladder (8), disseminated intravascular coagulation (3), and hemiparesis (1). All patients except one infant are still alive. In most of the patients coronary alterations normalised, while in 20 patients they persisted for more than one year; in 12/20 patients they were permanent. CONCLUSION: Pediatricians should be aware of the increasing number of children with atypical and incomplete KD, in order to diagnose and treat the disease even in the absence of all typical manifestations and to prevent coronary complications.

Adolescent↗
PubMedDate not supplied

[Kawasaki disease: clinical characteristics and cardiovascular involvement in a cohort of 121 patients].

Kawasaki disease is an acute generalized self-limiting vasculitis of small and medium size arteries of unknown ethiology, mostly affecting children younger then 5 years of age. Coronary arteries may be involved with aneurysms lesions, thrombotic occlusion, acute coronary syndrome and sudden death. The mortality rate has been significantly reduced by combined therapy of intravenous immunoglobuline (IVIG) and acetil salicylic acid (ASA) from 3% to 0.2%. From February 1986 to November 2001, 121 children, 76 male and 45 females with Kawasaki disease, have been observed at the Paediatric Department, University of Florence. Coronary changes (i.e. coronary ectasia and coronary aneurysms) have been observed in 22.3%. In only one 3-months old child, giant aneurysms of coronary arteries and myocardial necrosis have been detected. In 26 of 27 children at 2D echo a remodelling of the coronary changes within 12 months from the onset of the illness was observed. The mean follow-up has been five years (range 6-138 months) and in no patient further cardiac manifestations have been observed; however, as little is known about the progression of vascular damage, a more protracted follow-up is needed.

Adolescent↗
PubMedDate not supplied

Juvenile psoriatic arthritis and acquired sensorineural hearing loss in a teenager: is there an association?

Autoimmune inner ear disease is a cause of sensorineural hearing loss, first described in 1979 by McCabe. The occurrence during rheumatic diseases is already documented in adults, but to our knowledge, this evidence is still lacking in children. A 13-yr-old girl affected by juvenile psoriatic arthritis, treated with etanercept, developed a bilateral and asymmetric sensorineural deafness. The patient significantly improved after steroid administration. Once ruled out the principal causes of sensorineural hearing loss, we also considered the hypothesis of an anti-TNF side effect. However, the clinical presentation, the efficacy on steroid treatment and the presence of inner ear auto-antibodies prompt us to consider autoimmune-SNHL as the most plausible diagnosis. The young age of our patient seems to suggest a genetic susceptibility to autoimmunity and supports the concept of associated autoimmune diseases.

Antirheumatic Agents↗
PubMedDate not supplied

Bilateral renal artery stenosis in Kawasaki disease: a report of two cases.

We report two cases, a boy and a girl, who developed severe renal hypertension over sub-acute phase of Kawasaki Disease (KD). Paediatricians should be alert to consider preceding KD as potential source of secondary hypertension in young infants, and raised blood pressure should be regarded as a life-threatening complication in all KD children.

Female↗