AZATHIOPRINE FOR THERAPY OF IBD IN CHILDHOOD

Key words: Crohn disease, Ulcerative recto-colitis, Azathioprine

28 children with IBD (mean age 14.7) received azathioprine (AZA) in addition to corticosteroids. Out of them, 17 are still receiving AZA, 11 had to discontinue the treatment because of adverse effects or other reasons. With reference to the Crohn disease, PCDAI decreased, from the beginning of treatment to the last follow-up, from 26.2 ± 11.4 to 6.5 ± 8.7 (p<0.0001); with reference to recto-colitis, the URC-score passed from 7.5 ± 1.6 to 3.0 ± 3.3 (p<0.05); the need of corticosteroids passed from 0.7 mg/kg ± 0.4 to 0.1 ± 0.2 mg/kg (p<0.0001).

PREVALENCE OF ANTI-SACCHAROMYCES ANTIBODIES IN IBD IN CHILDHOOD

Key words: Crohn disease (CD), Ulcerative recto-colitis (URC), Anti-Saccharomyces antibodies (ASCA)

We studied the serum of 28 children with IBD (15 URC, 13 CD). ASCAs were always negative in URC and positive in 7/13 CD (localisation: ileocecum and ascending colon). No correlation between ASCA-positivity, age and CD degree of activity was found. ASCAs are specific for CD diagnosis, but their sensitiveness is not sufficient for clinical screening.

CLINICAL VARIABILITY IN SCHWACHMAN DISEASE

Key words: Schwachman syndrome, Italian register, Clinical features

56 cases of Schwachman syndrome have been enrolled in the Italian register in order to better define the clinical spectrum. Almost all presented the hallmark of pancreatic insufficiency and hematologic cytopenia. 78% tipical and 41% atypical osseous impairement, 73% hepatic dysfunction (mostly non persistent), 50% short stature, 45% severe infections, 20% neurological or intellectual impairement, 12% renal disfunctions and 5% endocrine problems.

BREAST FEEDING IN MILANO

Key words: Breast-feeding, Epidemiology

Sampling study carried out on 368 interviewed mothers. Breastfeeding is started in 86% of cases. The risk factors for early discontinuance are: caesarean section (RR 0.55), twin delivery (RR 0.30), premature delivery (RR 0.42); administration of additions in hospital (RR 0.47), supply of milk samples at dismissal (RR 0.28), resumption of work. The protection factors are: first sucking within 6 hours (RR 1.31), rooming in (RR 1.34). Caesarean sections are 27% of deliveries. Additions in hospital are administrated in 72% of cases; rooming in is carried out in 19% of cases.

ANAPHYLAXIS BY FRESH FRUITS, TOLERANCE AGAINST HOMOGENATES

Key words: Anaphylaxis, Fruit, Homogenate, Prick-by-prick

2 children (6 years and 5 months) showed a severe anaphylaxis by apple and banana respectively: they tolerate those fruits in commercially available homogenates; prick-by-prick is positive for the relevant fruit, but negative for homogenates.

SPONDYLODISCITIS

Report of two cases with evocative symptoms (stiffness of column, refusal to sit and walk); easy diagnosis (scintigraphy + NMR), very ready response to antibiotics.

NEUROLOGIC NON NEUROGENIC BLADDER

Key words: Hinman syndrome, Neurologic non neurogenic bladder, Enuresis, Vescico-urinary reflux

A 5 year age boy, with pyelonephritis, hydronephrosis, vescico-urinary reflux, enuresis, parietal hypertrophy of the bladder, stipsis have been cured by simple mintional training.

CHRONIC SEVERE MULTI-DRUG RESISTANT THROMBOCYTOPENIA IN A GIRL WITH LYMPHOPROLIFERATIVE AUTOIMMUNE SYNDROME (ALPS). EFFICACY OF CYCLOPHOSPHAMIDE

Key words: ALPS, Canale-Schmidt syndrome, Apoptosis, Thrombocytopenia, Cyclophosphamide

Report of a very severe case of thrombocytopenia with lymphoproliferative autoimmune syndrome (hereditary ALPS) resistant to classical treatments, both in high doses or bolus, with prompt and durable response to cyclophosfamide.