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A case of Henoch-Schönlein purpura (HSP) characterized by several unusual complications and exceedingly prolonged course is reported. A 6-year-old boy, hospitalized with a typical clinical picture of HSP, developed after a few days a severe gastrointestinal vasculitis leading to digestive hemorrhages and food intolerance. The treatment included continuous gastrointestinal infusion, parenteral nutrition, and corticosteroids during the exacerbations of symptoms. Intestinal ultrasonography showed diffuse submucous edema and widespread mural hematomas; hydrops of the gallbladder and edematous swelling of the pancreas were also detected. A gastro-duodenoscopy revealed diffuse and severe mucosal vasculitis with ulcers and petechial lesions. One week after admission the occurrence of weight gain, hyponatremia, and hypoprotidemia in the absence of proteinuria suggested a protein-losing enteropathy. The elevation of serum and urine amylases was consistent with pancreatic involvement. The course was characterized by recurrent exacerbations of gastrointestinal manifestations; symptoms subsided slowly to such a degree that oral feeding could be gradually restarted only seven weeks after admission. The use of high-frequency intestinal ultrasonography proved useful and sensitive in monitoring the evolution of intestinal involvement. During hospitalization the child complained of acute painful scrotal swelling. Ultrasonography showed scrotal hemorrhage and testicular swelling but no signs of testicular torsion, thus helping to avoid surgical exploration. In the recovery phase the child complained of colicky abdominal pain with vomiting. A plain abdominal roentgenogram showed bilateral staghorn pelvic urolithiasis. This finding was confirmed by an intravenous pyelography which also revealed a bilateral pyeloureteritis with edema of the ureteral wall and partial stenosis of the lumen.(ABSTRACT TRUNCATED AT 250 WORDS)

Exocrine pancreatic function was evaluated in 21 diabetic children on the basis of a p-aminobenzoic acid (PABA) test and a determination of fasting serum amylase, pancreatic isoamylase, lipase, trypsin and elastase levels. Fecal chymotrypsin was also measured. Compared to the controls, the diabetic children had significantly lower levels of trypsin (P less than 0.001) and elastase (P less than 0.02). Fecal chymotrypsin appeared to be significantly lower (P less than 0.01) in diabetic children than in controls but in all patients fecal chymotrypsin values registered above the limit considered to be normal. No significant correlation was observed between pancreatic enzyme concentrations, serum and urinary PABA values, and chronologic age, HbA1 and insulin requirement. Only for serum PABA a significant negative correlation with duration of disease (P less than 0.01) has been observed. These data show that exocrine pancreatic function may be abnormal in children with IDDM.

To the Editor: We read with great interest the comments by Capristo et al. They wonder whether our results concerning body mass index (BMI) of early-diagnosed celiac patients (which were higher than in controls) could be caused by an unusually low BMI in the control group (i.e., lower than that reported in the literature). Moreover, they suggest that the different body composition of the early-diagnosed patients could be related to a significant presence of subclinical forms of celiac disease and/or to a wide dispersion of data because of the age range (2.3–18.9 yr).

Cell-mediated immune response was evaluated in 14 children with long-lasting intra- or extrahepatic cholestasis. Cell-mediated immunity was clearly depressed in children with intrahepatic cholestasis while children with extrahepatic biliary obstruction had a more modest and variable degree of impairment. This finding may be related to the longer duration of cholestasis and the higher total bile acid level in the intrahepatic compared to the extrahepatic group. In particular, in children with Byler disease, long-lasting, severe intrahepatic cholestasis was associated with depressed cell-mediated immunity and recurrent severe infections.

The diagnosis of Cow's Milk Protein Allergy was considered in 303 infants aged less than 1 year, who presented with one or more of the following symptoms: acute reaction related to cow's milk proteins (CMP) ingestion, severe colics, persisting vomiting, protracted diarrhea with or without blood and mucus, failure to thrive, eczema, respiratory symptoms, such as chronic rhinitis and wheezing. A diagnosis of CMPA was confirmed in 148 cases (60%): 125 relapsed on milk challenge, 23 were not challenged because of acute reactions at onset, presence of specific IgE (RAST and prick), and improvement on milk free diet. Familial atopy, familial history of CMPA and previous acute gastroenteritis were significantly more frequent in cases than in 191 age matched controls. Breast feeding was not more common or of longer duration in controls, compared to cases. Mean IgE serum levels were higher (46.3 U/ml) in cases than in controls (17 U/ml), while specific Cow's Milk Protein IgE were found in 71/148 cases (48%). 15 infants entered the study while on breast milk, because of the confirmed relation between their symptoms and CMP on the maternal diet. These infants had a higher prevalence of IgE mediated problems. All cases improved on a milk free diet but in 26 (17.8%) a further modification of the diet was required after the first prescription. Milk challenge was monitored by simple laboratory tests: all cases who had symptoms on challenge showed at least one test modification. Six infants, with no history of acute reaction, showed severe self-limited clinical symptoms at challenge.

Clinical, biochemical and histological features of chronic hepatitis type B were studied in 29 children aged 8 months to 13 years. On entry into the study, all were known to have had hepatitis B surface antigen (HBsAg) with elevated serum transaminase levels for at least six months. A possible source of infection was found in 15 children. When they entered the study, all patients were anicteric and all but one asymptomatic. Hepatomegaly was detected in 15 patients and was associated with splenomegaly in two. Hypergammaglobulinemia was present in 4 children. Serological evaluation of hepatitis B virus markers showed evidence of complete viral replication (HBeAg positivity) in 24 cases and incomplete replication (anti-HBeAg positivity) in 5. Liver histology showed chronic persistent hepatitis (CPH) in 18 children, and chronic aggressive hepatitis (CAH) in 10 (3 moderately active and 7 with major signs of aggressivity ) associated with cirrhosis in 5. One patient had only minimal histological changes. Evaluation of clinical, biochemical and virological parameters did not strictly parallel the histological diagnosis in terms of "activity" of the disease. Follow-up for a mean period of 13 months showed good clinical tolerance to the disease in both CPH and CAH patients. Only 2 children with CAH were given corticosteroids and/or azathioprine for a short period. During follow-up no children with active disease developed liver insufficiency or evidence of portal hypertension. No significant difference in the percentage of children who had seroconversion to antiHBe was found between CPH and CAH groups. Only one child with CAH became HBsAg negative.(ABSTRACT TRUNCATED AT 250 WORDS)

A child with an immune-mediated disease is described, who presented two very rare clinical manifestations, a membranous glomerulopathy with circulating anti-renal tubular basement membrane antibody and a small-intestinal enteropathy with circulating antibody directed against the cytoplasm of intestinal epithelial cells. Steroid treatment was followed by complete resolution of the renal but not the intestinal manifestations.

REFERENCES 1. Schwartz RH, Rodriquez W J, Gundfast KM: Pharmacologic compliance with antibiotic therapy for acute otltis media: Influence on subsequent middle ear effusion. Pediatrics 68:619, 1981. 2. Buchanan GR, Siegel JD, Smith S J, et al: Oral penicillin prophylaxis in children with impaired splenic function: A study of compliance. Pediatrics 70:926, 1982. 3. Chretien JH, McGarvey M, Destwolinski A, Esswein JG: Abuse of antibiotics: A study of patients attending a university clinic. Arch Intern Med 135:1063, 1975. 4. Haggerty R J, Roghman K J: Noncompliance and self medication: Two neglected aspects of pediatric pharmacology. Pediatr Clin North Am 19:101, 1972.

Using a new colorimetric method we measured the faecal chymotrypsin in 407 subjects, divided as follows: 252 adult subjects with a normal exocrine pancreatic function as shown by duodenal intubation, 24 adult patients with a mild to moderate pancreatic insufficiency, and 26 adult patients with severe pancreatic insufficiency. In addition, 40 healthy children, 50 children with chronic diarrhoea, and 15 with cystic fibrosis were studied before and after substituting enzyme therapy. Faecal chymotrypsin was found to be useful in evaluating the degree of exocrine functional insufficiency in subjects with diseases of the pancreas that had, already been clinically ascertained. The same cannot be said for its ability to provide an early diagnosis of subjects with a slight-moderate insufficiency in exocrine pancreatic function.

No organism from the cradle lives germ-free or allergen-free. The modalities by which infections facilitate conditions of abnormal reactivity, in particular respiratory asthma and allergy to cow milk proteins, are examined. 216 asthmatic children and 50 infants with rotavirus enteritis have been considered. Infections, besides representing stimuli directly projected on the immune system, also constitute factors which more generally influence the way of reacting.

In order to evaluate the intrafamilial spread of hepatitis B virus (HBV) infection we studied the serological markers of HBV in 101 relatives of 35 children with chronic hepatitis B. Sixty per cent of relatives had markers of infection and 25% showed persistent HBs antigenemia. The high prevalence of HBeAg versus anti-HBe in chronically infected relatives suggests a close temporal relationship of the infection between adults and children. These results support the hypothesis that the child is the main carrier of HBV infection in his family.

In 8 children with selective IgA deficiency (serum IgA less than 5 mg/dl, secretory IgA less than 0.5 mg/dl in unstimulated saliva) immunofluorescent staining of intestinal biopsy specimens revealed the presence of IgA-containing plasma cells. This finding supports the hypothesis that in the intestinal mucosa of patients with IgA deficiency B lymphocytes undergo "sterile" differentiation into IgA-containing plasma cells probably incapable of secreting the IgA synthesized.

An 8-year-old girl with microhematuria of recent onset developed a picture of pulmonary hemosiderosis in the space of 2 months. Some months later while pulmonary involvement was improving severe extracapillary glomerulonephritis developed. Circulating anti-glomerular basement membrane antibody was detected and linear deposition of IgG along glomerular basement membrane was observed. A diagnosis of Goodpasture's syndrome was made and treatment was started with prednisone, cyclophosphamide and periodic plasmapheresis with complete progressive disappearance of circulating anti-glomerular basement membrane antibody. After suspension of plasmapheresis despite immunosuppressive therapy and lack of evidence for circulating anti-glomerular basement membrane antibody, the child went into terminal renal failure. The natural history of the disease in this case and the results of treatment are discussed. To our knowledge this is the first case of Goodpasture's syndrome reported in childhood with demonstration of the presence of anti-glomerular basement membrane antibody.

Twenty out of 108 infants with vomiting, who underwent an upper gastrointestinal X-ray study during a period of 2 years, showed a peculiar shape and position of the stomach already described as chronic gastric torsion. We examined clinical, radiologic and laboratory findings of these 20 infants. Our results showed that chronic gastric torsion is frequently associated with gastroesophageal reflux and has a wide spectrum of symptoms, complications and nutritional abnormalities.

Hypophosphatasia is a rare familial disease characterized by abnormalities of the skeleton, low serum alkaline phosphatase level and presence of abnormal quantities of phosphorylethanolamine in plasma and urine. The biochemical bases of inadequate calcification of the bone matrix are unknown. We report the case of a 4 month-old infant who presented symptoms of rickets. Laboratory analysis showed normal serum Ca and P levels, low serum alkaline phosphatase activity, PEA level increased in plasma and urine. X-ray examination of the bones disclosed poor mineralization and the presence of focal defects of the skull. The therapeutic problems are discussed.

• Schonlein-Henoch purpura developed in two children in association with hepatitis B virus (HBV) infection. The first child, an 8-year-old boy, first had a clinical picture of Schonlein-Henoch purpura and then was found to have HBV-related chronic persistent hepatitis. In the second child, a 6-year-old girl, characteristic skin lesions, arthralgia, and proteinuria developed during acute hepatitis B. Immunofluorescence demonstrated IgA deposition in the renal glomeruli of the first patient. We suggest that evidence of HBV infection should be sought in patients with Schonlein-Henoch purpura. ( AJDC 1984;138:681-682)

In order to evaluate the diagnostic value of a lymphocyte stimulation test with casein and beta lactoglobulin in cow milk intolerance (CMI), we studied 26 patients with a diagnosis of CMI and 25 age-matched controls with intestinal diseases other than CMI. Lymphocyte stimulation test to casein (P less than .001) and beta lactoglobulin (P less than .001) was significantly higher among CMI patients. Seventeen patients were RAST positive and nine negative for whole milk proteins. No differences were found in the lymphocyte stimulation test response between RAST-positive and RAST-negative subjects. These results suggest that lymphocyte stimulation test with highly purified antigens in optimal concentrations is specific and could be useful in the diagnosis of CMI.

• Neutrophil motility was assessed in 31 children with chronic liver disease to estimate the eventual increased susceptibility of these patients to bacterial infections. Twelve children had chronic hepatitis (seven with chronic persistent hepatitis and five with chronic active hepatitis), which was mostly related to hepatitis B virus (HBV) infection. Nineteen children had chronic intrahepatic or extrahepatic cholestasis. A total of six serious bacterial infections occurred in four of the 31 patients during the study. Twenty of the 31 children had a persistent defect of neutrophil chemotaxis. This defect was found in four types of childhood chronic liver disease: HBV-related chronic hepatitis and idiopathic intrahepatic cholestasis of infancy, in which the defect did not seem to predispose significantly to bacterial infection, and in Byler's disease and biliary atresia, in which this neutrophil defect was associated with an increased frequency of severe infections. ( Am J Dis Child 1983;137:768-770)

A case of celiac disease presenting as an asymptomatic chronic persistent hepatitis in an 11-year-old girl is reported. Liver biopsy performed because of long-standing elevation of serum transaminase levels showed a mild portal fibrosis with mononuclear infiltrate. Immunofluorescence staining did not reveal deposits of immunoglobulins or complement in the liver specimen. Although the girl was totally asymptomatic, she had steatorrhea, a delayed bone age, and an abnormal D-xylose test. A jejunal biopsy showed villous atrophy and increased intraepithelial lymphocytes. On a gluten-free diet the level of transaminases fell to normal within 1 month and remained normal. According to biological remission, a second intestinal biopsy performed after 1 year of gluten-free diet revealed a normal intestinal mucosa. Our report suggests that an underlying chronic intestinal disorder, and particularly celiac disease, must be ruled out when evaluating a child with elevated levels of serum transaminase.

A case of malignant small cell tumor of the thoracopulmonary region in a 3-year-old boy is presented. The rarity of this tumor in children justifies the presentation of a new case. Differential diagnosis with other childhood neoplasms is discussed.

The purpose of this report is to evaluate whether a new, simple, non-invasive method for chymotrypsin measurement in stools is useful for the diagnosis of exocrine pancreatic insufficiency in cystic fibrosis (CF). A hundred children aged from 2 months to 12 years were tested: 50 children had been admitted for chronic diarrhoea, 15 for cystic fibrosis and 40 acted as controls. Chymotrypsin in stools was assayed using a kinetic measurement with Succ-Ala-Ala-Pro-Phe-pNa as substrate in a simple photometric assay. In 13 of 15 children with cystic fibrosis, stool enzyme levels were always remarkably low, while all control subjects and all children not presenting cystic fibrosis had normal stool levels of chymotrypsin. Our data suggest that stool chymotrypsin measurement is a simple and reliable "tubeless" test for the evaluation of exocrine pancreatic insufficiency in children with cystic fibrosis.

A FOLLOW-UP STUDY OF CHILDREN WITH COW'S MILK INTOLERANCE. DEVELOPMENT OF MULTIPLE FOOD INTOLENCE