[Distribution of new cases of insulin-dependent diabetes mellitus (IDDM) by age, sex, seasonality, and clinical characteristics at onset in youngsters from the Friuli Venezia Giulia region from 1987 to 1990]. / Distribuzione dei nuovi casi di diabete mellito insulinodipendente (DID) per età, sesso, stagionalità e caratteristiche cliniche all'esordio nei giovani della Regione Friuli Venezia Giulia durante il quadriennio 1987-'90.

A retrospective study was undertaken to estimate the incidence of insulin-dependent diabetes mellitus (IDDM) in youngs (< 18 years) in a North-East Province of Italy: Friuli Venezia Giulia (total population: 1,211,320; under age 18: 185,860). On the assumption that all children with newly detected IDDM are referred to a hospital, data were collected from all pediatric departments (14) and from all diabetologic services (14) of the Province. A central register for all patients receiving drug reimbursement was used as the secondary source for validation of case ascertainment, that was 98%. All IDDM cases diagnosed between 1987 and 1990, with age onset < 18 years, and using insulin at discharge from hospital, were included. 73 new IDDM cases under 18 years (47 M, 26 F) were observed during the considered period. The average annual incidence of the disease (based on first hospital admission) was estimated to be 9.8 per 100,000 children under 18 years. There was a significantly higher incidence for boys (12.6/100,000) than for girls (6.9/100,000). The highest incidence (12.5/100,000) was in the age group 10-15, and the lowest (7.2/100,000) in the aged 0-5 years. First admission rates showed seasonal variations, with peaks in Oct/Nov and Jan/Feb. History of an infection during the month before the first admission was present in 17/73 cases. Duration of symptoms < 15 days, 15-30 days, > 30 days, was present in 43, 18, 12 patients respectively. Weight loss < 5% was present in 31/73 cases. Ketoacidosis at onset (pH < 7.36) was present in 30/73 cases.(ABSTRACT TRUNCATED AT 250 WORDS)

[Experience with gamma globulins per os in the therapy and prevention of infectious diarrhea]. / Esperienze con le gammaglobuline per os nella terapia e prevenzione della diarrea infettiva.

The efficacy of oral gammaglobulin in the treatment of acute infectious diarrhea in immunocompetent children has been evaluated in an open placebo controlled trial. Moreover the efficacy of oral gammaglobulin has been tested also for treatment of chronic diarrhea in IgA deficient infants and for prevention of rotavirus infection during an epidemic in the ward. 54 infants (aged 1-36 months) with acute diarrhea (30 rotavirus +) were enrolled in the study. 24 out of 54 were assigned with randomised method to group a receiving gammaglobulin 150 mg/kg x 2 in the first day of admission to hospital and the remaining 30 infants were assigned to group b receiving placebo. Diarrhea cleared up in 2.57 +/- 1.4 days without a significant difference between group a and b (2.6 +/- 1.6 and 2.46 +/- 1.1 days respectively). The diarrhea's duration in Rotavirus+infants was 2.78 +/- 1.4 days in group a and 3 +/- 1.4 days in group b again without a significant difference. The excretion time of rotavirus in the stools was significantly shorter in rotavirus+group a (2.6 +/- 1.3) than in rotavirus group b (3.9 +/- 1.6) with p < 0.04. Three infants (4.9 months) with chronic post-infectious diarrhea and IgA deficiency (< 5 mg%ml) received gammaglobulin 300 mg/kg/die for 3 days. The diarrhea recovered in 2-3 days. Out of 16 infants hospitalized during an epidemic rotavirus diarrhea 6 infants received oral gammaglobulin at the dose of 150 mg/kg/die during the hospitalization period (that was at least 5 days). No one became ill.(ABSTRACT TRUNCATED AT 250 WORDS)

Chronic non-specific diarrhoea in childhood: how often is it iatrogenic?

Epidemiological data, the appropriateness of the medical approach, the effectiveness of unrestricted diet and the subsequent course of chronic non-specific diarrhoea (CNSD) were evaluated in 20 children, mean age at diagnosis 4.7 y, and mean duration of diarrhoea 24 months. A mean of 2.4 previous hospital admissions and a mean of 2.5 diagnoses other than CNSD were recorded per child before admission. On admission, 14/20 were following an elimination diet and 8/20 had an inadequate caloric intake; 16/20 had a weight/height ratio below the 50th percentile. In all cases a normal diet prescribed during hospitalization improved the diarrhoea and increased weight. A telephone interview performed 5.6 y after discharge revealed that in 10/20 of the cases, the parents were disappointed with the unrestricted diet prescribed during hospitalization. Nevertheless they reported that CNSD stopped spontaneously in a mean time of 1.7 y.

Antiendomysium antibodies and coeliac disease: solved and unsolved questions. An Italian multicentre study.

A total of 3783 subjects were enrolled to compare IgA and IgG gliadin antibodies (AGA) with IgA endomysium antibodies (EMA) in coeliac disease (CD). Among 688 children with untreated CD EMA were positive in 93.8%, IgA AGA in 84.9% and IgG AGA in 90.2%. AGA, but not EMA, sensitivity decreased with age. EMA were present in 3.8% of control subjects, IgA AGA in 14.9% and IgG AGA in 34.3%. Follow-up of 5 of 39 EMA-positive controls showed flat mucosa. Combined determination of EMA and AGA showed an increased predictive value: if EMA and AGA were both positive, the mucosa was flat in 99.1%, if both were negative, the mucosa was normal in 99.1%. After a gluten-free diet (GFD), IgA-AGA disappeared first. Among 21 patients not on a strict GFD and in 194 coeliac patients after challenge, EMA, but not AGA, were always positive. Among 67 first-degree relatives of coeliacs, the positive predictive value of EMA was 90.6%, IgA AGA 74.3% and IgG AGA 44.6%. In conclusion, EMA screening is an excellent test for the diagnosis and follow-up of CD, and for identification of its silent and latent forms.

Aminohydroxypropylidene-biphosphonate in the treatment of bone lesions in a case of Gaucher's disease type 3.

Gaucher disease is the most prevalent lysosomal storage disorder. It is characterized by an autosomal recessive inheritance of a deficiency of lysosomal acid glucocerebrosidase. Three clinical phenotypes are recognized: type 1 (non-neuronopathic), type 2 (acute neuronopathic), type 3 (subacute neuronopathic). Bone lesions are associated with type 1 and type 3 Gaucher disease. Skeletal involvement is secondary to the progressive accumulation of histiocytes and macrophages laden with glucosylceramide in bone marrow. Our patient was a female type 3 Gaucher patient who was referred to us at the age of 3 years with a neurological symptomatology and severe bone lesions (bilateral fracture of the femur heads, lytic process of the bone matrix of the femurs and distal flask deformity, kyphoskoliosis and chest deformity). The baby was constrained to a wheel-chair. The use of (3-amino-1-hydroxypropylidene)-1,1-biphosphonate (APD) was described in a case of Gaucher disease with very severe bone lesions. We used periodic iv infusions of APD (10 mg every 3 weeks) in our patient for a period of 20 months; after that, enzyme replacement therapy (alglucerase) was commenced. APD treatment showed normalization of bone density, formation of bone callus at the femural heads, positive calcium balance. The urinary Ca/Cr ratio and TRP were consistently normal during therapy. After 9 months of alglucerase therapy the patient was able to walk again. The data indicate that APD therapy can find an indication in Gaucher patients with severe bone involvement.