[Granulomatous angiitis]. / Angiiti granulomatose.

Granulomatous angiitis are characterized by histologic feature of intravascular and extravascular formation of granulomas in association with necrotizing vasculitis of medium and small arteries. They are rare in childhood. The Churg-Strauss syndrome is associated with marked eosinophilia and asthma. Wegener's granulomatosis is characterized by the clinical triad of upper and lower respiratory tract diseases and renal involvement.

[Leukocytoclastic vasculitis]. / Le vasculiti leucocitoclastiche.

Leukocytoclastic vasculitis is characterized by infiltration of polymorphonuclear leucocytes with leukocytoclastic (presence of nuclear pigments) and fibrinoid necrosis affecting the small vessels. Henoch-Schönlein purpura is characterized by non thrombocytopenic purpura, arthritis and arthralgia, abdominal pain and renal involvement. Hypocomplementemic urticarial vasculitis and cryoglobulinemia are exceptional in childhood.

[Kawasaki syndrome]. / Sindrome di Kawasaki.

Kawasaki's syndrome is an acute multisystem vasculitis typical for childhood characterized by fever, conjunctivitis, stomatitis, swelling of cervical lymph-nodes, exanthema and desquamation of extremities. The etiology is so far unknown, the latest hypothesis formulated are reported. Cardiac involvement and particularly coronary aneurysmal dilatation is the most severe and characteristic SK complication.

[Giant cell arteritis]. / Arteriti a cellule giganti.

Giant cell arteritis are characterized by histologic disruption features of the lamina elastica of large arteries with mononuclear cells and giant cells infiltration. Systemic giant cell arteritis, temporal arteritis and Takayasu's arteritis are included in this group. Takayasu's arteritis predominantly affects the teenage girl and involves the aorta and its mayor branches. Early manifestations are systemic; later appears signs and symptoms of occlusion.

[Polyarteritis]. / Poliarteriti.

Polyarteritis is an uncommon childhood disease. The different forms of polyarteritis are described. Polyarteritis nodosa is a rare multisystemic disease characterized by high fever, calf pain and subcutaneous nodules. Infantile polyarteritis nodosa shows a peculiar predilection for the coronary arteries and has the worst prognosis. Polyarteritis cutaneous is the localized and mild form of polyarteritis. Hypersensitivity angiitis is described as a leukocytoclastic vasculitis identified by palpable purpura.

[Rheumatic fever from 1960's to 1990's. Case records in 2 hospitals of the Lombardian area]. / La malattia reumatica dagli anni '60 agli anni '90. Casistica rilevata in due ospedali dell'area lombarda.

An epidemiological study of rheumatic fever (RF) has been done. Incidence and prevalence of RF observed in Monza Hospital from 1964 to 1990 and in Como Hospital from 1980 to 1990 both show a progressive reduction. An exception to this trend was observed in 1986 in Monza in 1985 in Como. The percentage of carditis in pediatric age is high (60-70%), often associated with arthritis and chorea. However carditis clinical picture seems nowadays less severe, probably because in the past the diagnosis was delayed. Sice 1980 no death were observed due to RF or related cardiac involvement. Relapses and residual valvulopathies are significantly reduced only when penicillin prophylaxis was correctly performed.

[Echocardiographic and polycardiographic findings in multiple-transfused patients with thalassemia major]. / Indagine ecocardiografica e policardiografica in soggetti affetti da talassemia mayor politrasfusi.

Echocardiographic and policardiographic measurements were carried out in 11 thalassemic major patients between 2 and 24 years of age, kept at on average Hb level of 9,77 gr.% (+/- 1,13) and who underwent chelation therapy with Deferoxamine subcutaneous and Vit. C. The echocardiographic examination gave normal values in 7 patients. In 4 patients underwent enlargement of the left ventricle: only in the telediastolic diameter in one case, with progressive involvement of the aorta and the left atrium in another 3 cases. Such alterations, as have already been recorded by various researchers, are probably the result of a condition due to the anemia state of the younger patients, whereas in older and polytransfused patients may be attributed to iron overload. No parameters surveyed by the polycardiographic examination indicated significant alterations in cardiac efficiency, except of one patient. The importance of echocardiography should be stressed; it is a simple, non invasive way of recording early cardiac alterations in thalassemic major patients and enables long-term monitoring of cardiac function in the assessment of the effectiveness of the chelation therapy.

[Beta-pancreatic function in subjects with thalassemia. A 4-year follow-up]. / Funzionalità beta-pancreatica in soggetti affetti da talassemia Follow-up di quattro anni.

High Hb level transfusion scheme for treatment of thalassemia mayor has improved life prognosis but has increased also the incidence of Diabetes Mellitus. 10 patients with thalassemia major have been followed with OGTT for a period 4 years long (1979-1982). In 1979 we changed from low to high level transfusion regimen, and we began to use the pump for slow subcutaneous administration of desferrioxamine to treat iron overload. The results we obtained show a progressive increase of the average values in the insulinemic and glycemic plasma concentration from year to year. At the beginning of the follow-up period, insulinemic and glycemic values after OGTT showed a primitive pancreatic damage which evolved towards a better pancreatic function with the appearance of a peripheral insulin resistance. It is probable that both chronic hypoxia (low Hb level) and the iron overload (high Hb level) may cause, with different processes, an impairment of glucose metabolism.

[Chylothorax in a newborn infant]. / Chilotorace in neonato.

A case of chylothorax in newborn is described. A the fifteenth day a sudden respiratory distress for chylothorax has appeared. A continuous drainage, after thoracenteses, has been placed; the clinic course has been favourable and, at five day, we have started with MCT feeding.

[A case of stenosing ureteritis in Schoenlein-Henoch purpura]. / Su un caso di ureterite stenosante in porpora di Schönlein-Henoch.

A case of stenosing ureteritis in a 6 years old girl with Schönlein-Henoch purpura is described. In a careful review of the literature only a few cases results described. The authors discuss the problems concerning this rare and potentially serious complication of Schönlein-Henoch purpura.

[Schönlein-Henoch syndrome: clinical-epidemiological analysis of 98 cases]. / Sindrome di Schönlein-Henoch: analisi clinico-epidemiologica su 98 casi.

We analyzed the clinical and epidemiological data of 98 patients (50 males and 48 females) aged between 6 months and 16 years, hospitalized for Schönlein-Henoch syndrome in the last 20 years. The incidence was higher during spring time. Throat culture was positive for streptococcus pyogenes in 16% of patients. 14 of 60 (23%) had a positive allergometric response to various tests. The extrarenal manifestations were: purpura (100%), articular (68%) or gastrointestinal (32%) involvement and orchitis (10%). Renal symptoms were observed in 23% of the patients, but a clear nephropathy was documented just in 5% of the cases, with resolution within 2 years. Our data suggest, in agreement with the letterature, that renal involvement in Schönlein-Henoch syndrome has usually a benign course.

Reversal of haemochromatotic cardiomyopathy in beta thalassaemia by chelation therapy.

Haemochromatotic cardiomyopathy is the main cause of morbidity and mortality in patients with beta thalassaemia major. Once congestive heart failure develops most patients die in a few months. Congestive heart failure was reversed and echocardiographic findings were restored to normal in a 24 year old woman with beta thalassaemia who resumed treatment with chelation therapy (desferrioxamine).