Anatomy and radiology of a bone island.

Anatomoradiological study, on an amputation specimen for osteosarcoma from a 13 year old girl, of a bone island in the distal femoral epiphysis. It consists of a mature bone core caused by localized remodeling of the spongeous trabeculae, old but still slightly active, clearly different from a bone infarct, a tumoral or infectious process. Discussed the interest of a precise radiological study for making the diagnosis (particularly xerography), the risks of error involved in scintigraphy and the utility of a block excision if a biopsy is performed.

Splenic cysts in pediatric patients--a report on 8 cases and review of the literature.

8 cases of splenic cysts are reported: treatment was surgical in 4 large cysts (greater than 5 cm, 3 epidermoids and 1 pseudocyst), and non-operative in 4 small cysts (less than 5 cm, 3 post-traumatic pseudocysts and 1 congenital cyst, probably epidermoid). Follow-up was over a period of 3 months to 7 years. 3 among the 4 post-traumatic pseudocysts were followed up by ultrasound until resolution (3 months to 3 years), and 1 large pseudocyst was operated on due to rupture following benign trauma. 3 large cysts were epidermoid in origin, 1 was electively operated, and 2 were discovered and operated on because of complications (rupture, infection). Only one total splenectomy was performed; surgery preserved the spleen in all other cases; but 1 epidermoid cyst presents now with a recurrence. Based on this experience, we suggest frozen section of the cyst wall during surgery, to decide the type of partial splenectomy to be performed: total cystectomy or marsupialization.

[Rotavirus enteritis in children]. / Entérites à rotavirus chez l'enfant.

Recent clinical experience with twelve cases of rotavirus gastroenteritis has enabled us to review this subject. After a historical introduction and a review of the literature, two patients are described in detail. The clinical and laboratory data of all the twelve children are reviewed and compared with cases reported in the literature. These observations suggest several conclusions: The disease is very contagious. The patients present with diarrhoea lasting a few days, vomiting and fever. They recover without complications usually within 6 days. All our cases occurred in winter. The blood count revealed a neutrophilia at the onset of the illness followed after 4-5 days by a lymphocytosis with 1-3% plasmocytes. The finding of increased serum transaminase levels indicates a hepatic involvement. These levels returned to normal values only several weeks after the clinical improvement. Treatment consisting only of rehydration and a usual diet for acute gastroenteritis was successful in all the patients.

[Cow's milk protein intolerance in childhood. Review of 30 cases]. / L'intolérance aux protéines du lait de vache chez l'enfant

Retrospective clinical study of 30 cases of cow's milk protein intolerance with a long term follow-up (3-141/2 years) in 22 cases. The onset of the disease is usually in the first 6 months, following a short exposure to cow's milk proteins. A later onset is also possible, particularly following an intestinal infection which seems to favor the sensibilisation. Usually the digestive disturbances predominate and are often associated with respiratory, cutaneous and other general problems (especially unexplained prolonged fever). In about half the cases, laboratory studies reveal signs of exsudative enteropathy or generalized malabsorption with lesions of varying severity of the jejunal mucosa. Clinical observation and investigation allows us to divide the patients into three groups according to different manifestations: 1. acute anaphylactic, 2. chronic and benign (colitis), 3. chronic and severe, with accompanying malnutrition and intestinal malabsorption. This last category leads occasionally to diagnostic difficulties in distinguishing it from coeliac disease. Whatever manifestations encountered, the disease is usually transitory, and disappears between the age of 2 and 21/2 years, but can also persist for a much longer time. The long-term follow-up study showed complete catch-up growth, absence of further gastro-intestinal problems, and a low incidence of other allergic diseases (18%).

Value of a chicken-based formula for refeeding of children with protracted diarrhea and malnutrition in a developing country.

Two hospital-made, lactose-free, hypoosmolar formulas were compared for therapy for 61 children aged 10-28 months who had severe malnutrition and protracted diarrhea. The formulas were isocaloric and contained dextrin-maltose, carrots, sunflower oil, minerals, and vitamins. One had chicken meat as the protein source and the other had hydrolyzed lactalbumin (LAD). Initially the formulas were given as a continuous enteral feeding by nasogastric tube and pump. Six patients died from metabolic and infectious complications: four failed to equilibrate and changed diet. The remaining 51 were divided into two groups; group A, chicken formula (n = 26), and group B, LAD formula (n = 25). The mean duration of diarrhea was similar in both groups; number of days with liquid stools = 6.0 +/- 4.1 in group A, 5.5 +/- 2.8 in group B; number of days with greater than 4 stools/day = 3.5 +/- 3.4 in group A, 4.1 +/- 2.1 in group B. The time until beginning nutritional recovery was 13.2 +/- 12.0 days in group A and 13.3 +/- 7.8 days in group B. Intercurrent infections were significantly more frequent in patients receiving chicken formula (12 of 30 patients) than in those receiving LAD formula (4 of 27 patients) (p less than 0.05). We conclude that chicken formula is an efficient substitute to expensive semielemental formulas for continuous enteral feeding of protracted diarrhea in severely malnourished children.

Intramural diverticulosis of the esophagus in an eight-year-old boy.

A third pediatric case of intramural diverticulosis of the esophagus is reported in an 8-year-old boy. A hiatal hernia, chronic nonspecific esophagitis and defective esophageal motility was also documented in his 10-year-old brother.

[Marchesani's syndrome (spherophakia-brachymorphism)]. / Le syndrome de Marchesani (sphérophakie-brachymorphie).

The authors report the case of a patient with spherophakia and brachymorphism (Marchesani syndrome). This congenital and familial disorder is characterized by short stature, short limbs and thick extremities, limitation of joint mobility, a peculiar facies and spherophakia (rounded lens). Mentality is normal. The patient presented here, an eleven-year-old girl, had in addition a subvalvular fibromuscular aortic stenosis. A detailed radiological survey of the skeleton demonstrated a disorder of enchondral growth, particularly evident in the extremities, with short and wide diaphyses, thin cortical bone, and relatively discrete epiphyseal deformities. The vertebral bodies were slightly rounded anteriorly, th discs were thinned, and the ribs widened. The spinal canal and the foramen magnum were narrowed, and the lateral processes short and wide. There was thickening of the skull vault. A mucopolysaccharidosis and a mucolipidosis were excluded by biochemical study of skin fibroblasts in culture.

[Pseudomembranous entercolitis after antibiotic treatment: vancomycin therapy]. / Entérocolite pseudo-membraneuse après traitement antibiotique: traitement par la vancomycine.

After reporting the case of a child suffering from pseudomembranous colitis (PMC) rapidly cured by vancomycin, the authors present current knowledge which had led to this new mode of therapy. A patient of 2 years 7 months was treated with amoxycillin per os for 8 days for otitis media. A week after this treatment, diffuse abdominal pains appeared, with 8-12 diarrhoeic stools per day containing blood and mucus and recurrent episodes of rectal prolapsus. PMC was diagnosed after rectoscopy and rectal biopsy. The symptomatology persisted for 4 weeks at which time a treatment was begun with vancomycin 4 x 125 mg/day per os. Improvement of the gastrointestinal symptoms occurred 36 hours later, and a new rectal biopsy demonstrated recovery from PMC. Clinical and experimental studies have demonstrated that PMC is due to the proliferation of a toxin producing anaerobic bacterium, clostridium difficile. This organism is highly sensitive to vancomycin. Treatment with vancomycin eliminates clostridium difficile and its toxin from the stools of patients suffering from PMC and permits clinical and histological recovery.

Childhood tuberculosis at a Swiss university hospital: a 2-year study.

The incidence of tuberculosis (TB) presenting to the University Hospital, Geneva, Switzerland, was studied over 2 years. Clinical data on all children receiving antituberculous drug therapy was collected. Of the 43 identified cases, 42% had active disease (i.e. clinical evidence of organ involvement), the remaining 58% being asymptomatic. All 43 children originated from countries other than Switzerland. Symptomatic, drug induced hepatitis necessitated temporary interruption of therapy in 2 children (5%). Contact screening yielded three new cases of pulmonary TB, three previously diagnosed cases and seven instances of a positive medical history in relatives living in other countries. Of the 34 families in this study, contact screening could not be completed in 8. The results of this study indicate that children with TB are primarily associated with families originating from countries other than Switzerland, particularly those in which TB remains endemic. Tuberculin skin testing should therefore be targeted at this group. Contact tracing has also been shown to be beneficial.

Robinow syndrome in two siblings from consanguineous parents.

A Kurdish family had two children affected with Robinow syndrome. The daughter had short stature, macrocephaly, hypertelorism, hepatosplenomegaly, short forearms and marked vertebral anomalies. Her brother had hypertelorism, hypertrophied alveolar ridges, hepatosplenomegaly, short forearms, rib anomaly and ambiguous genitalia. The karyotype of the affected male sibling showed mosaicism for 45X, 46,X,dicY(q11.22), 47,X,dicY(q11.22),dicY(q11.22).

IgG, IgA and IgE gliadin antibody determinations as screening test for untreated coeliac disease in children, a multicentre study.

The diagnostic value of gliadin IgG, IgA and IgE antibody (AB) determinations using the fluorescent immunosorbent test was examined in 586 children with malabsorptive disorders and/or failure to thrive. All patients underwent jejunal biopsy and were on a gluten-containing diet. IgG AB were found in all patients (331/331) with untreated coeliac disease (CD) in our study, but IgA AB in only 295/331 (89%). Therefore a screening test based only on IgA AB determinations is not recommended. By contrast, 203 (80%) of 255 children with other malabsorptive disorders had no gliadin AB, 43 (16.5%) had only IgG AB and only 9 (3.5%) had IgG and IgA AB. IgE AB proved to be of no additional value as a diagnostic tool because they were found in a quarter of the children without CD. Statistical evaluation of combined IgG and IgA AB determination showed at least 96% sensitivity and a specificity of 97%. The subjective ("Bayesian") probability that an actual patient with a given AB test result has CD, is considered: a patient very probably has CD in the case of positive IgG and IgA AB, and no CD in the case of a negative AB result. In the case of negative IgA AB but positive IgG AB the physician's judgement ("prior probability") influences the ("posterior") probability of CD for an actual patient. In contrast to IgG AB, IgA AB decline rapidly after the introduction of a gluten-free diet and may be used for diet control after diagnosis.(ABSTRACT TRUNCATED AT 250 WORDS)

Prolactin and the gut: a controversy.

Previous reports suggest that prolactin could be one of the factors controlling intestinal mucosal growth. Therefore plasma levels of prolactin have been measured at the time of jejunal biopsy performed for suspicion of celiac disease. One hundred eighty-seven biopsies from 166 children have been reviewed according to histology, age, diagnosis, and plasma prolactin. No difference in the plasma prolactin could be detected among a group of 117 normal biopsies (9.4 +/- 0.4 ng/ml, mean +/- SEM), 31 biopsies with partial atrophy of various degree (9.0 +/- 0.9 ng/ml), and 39 biopsies with flat mucosa (9.1 +/- 0.7 ng/ml), nor could we demonstrate an increase in prolactin according to age and diagnosis (celiac disease before and after treatment, cow's milk protein intolerance, isolated postenteritic syndrome, selective sugar intolerance, and functional gut problems). Prolactinlike material has been detected by immunoperoxidase in the jejunal mucosa. The intracellular granules are located in the infranuclear portion of isolated epithelial cells mainly in the crypts. This material could not be correlated with the corresponding prolactinemias, whatever the histological appearance of the mucosa. These results would suggest that plasma prolactin is not a marker of jejunal regeneration in children. The nature and function(s) of this prolactinlike material remain to be established.

[Phosphorus-calcium metabolism and plasma 25-hydroxycholecalciferol in intestinal malabsorption]. / Métabolisme phospho-calcique et 25-hydroxycholécalciférol plasmatique dans la malabsorption intestinale.

One hundred and four children aged 0-15 years, with suspicion of malabsorption were studied. They had a proximal jejunal biopsy and, at the same time, the following measurements: 25-hydroxycholecalciferol (25-OH-CC), calcium, phosphorus and serum alkaline phosphatase, bone age on X-ray of the left hand and wrist, cortical thickness of the 2nd, 3rd, 4th metacarpal bones. For the analysis of the results, the patients were divided into two groups according to the season (winter vs. summer). None of the patients in either group had clinical or radiological signs of rickets. The following results were obtained: 1. The 25-OH-CC serum levels were significantly lower during the winter than during the summer months. This was observed more frequently in the cases with atrophy of the jejunal mucosa. 2. During the summer, the 25-OH-CC serum levels were not different in the cases with normal or pathological mucosa. This demonstrates the importance of the skin synthesis of vitamin D during the summer months. 3. The mean of the serum calcium levels was significantly lower in the group of children with atrophy of the jejunal mucosa than in children with normal intestinal biopsy. The serum calcium levels were not correlated with the serum 25-OH-CC levels. 4. The serum phosphorus levels were significantly lowered during the winter months in the children aged 0-3 years with pathological jejunal biopsy. 5. The serum alkaline phosphatase levels were lowered in cases of total atrophy of the jejunal epithelium cells. 6. Cortical thickness of the metacarpal bones becomes thinner with the progression of the alteration of the jejunal epithelium cells, independently of season or age. However, only the group of children aged 0-3 years studied during the winter months and with total atrophy of the jejunal mucosa have a significantly diminished cortical thickness of the metacarpal bones. The lowering of the calcium levels and the decrease of the cortical thickness are probably secondary to an impaired intestinal absorption of calcium. In the syndrome of malabsorption, the integrity of the jejunal epithelial cells seems to play a more important role than a vitamin D deficiency in the genesis of this calcium malabsorption.

Cortical scintigraphy in the evaluation of renal parenchymal changes in children with pyelonephritis.

We designed a prospective study to evaluate the ability of dimercaptosuccinic acid cortical scintigraphy and ultrasonography to detect renal parenchymal lesions in children with pyelonephritis. One hundred eleven patients 1 week to 16 years of age (median 5.5 months) with a urine culture positive for pathogens were included in the study; cortical scintigraphy and ultrasonography were repeated in 25 children after a mean follow-up of 10.5 months. Cortical scintigraphy showed renal changes in 74 children (67%), and ultrasonography showed renal changes in 39 (35%) (p < 0.001); results of the two examinations were discordant in 49 patients (kappa = 0.19). Children more than 1 year of age had a higher incidence of renal lesions than did younger children (85% vs 66%; p = 0.04). The presence of inflammatory signs (erythrocyte sedimentation rate or C-reactive protein) had an 89% sensitivity and a 25% specificity in identifying renal lesions. Among children with renal changes, vesicoureteric reflux was present in 39%. At follow-up examination, 16 children (64%) had scars. Thus we found a high incidence of renal involvement in children with pyelonephritis. We found that cortical scintigraphy is more sensitive than ultrasonography in detecting renal changes, and we believe that it should be added to the initial examination of children with suspected pyelonephritis.

Antigliadin and antiendomysium antibody determination for coeliac disease.

The value of IgG and IgA gliadin antibodies (AGA) was compared with that of IgA endomysium antibodies (EMA) for the diagnosis of coeliac disease. Three hundred and six of 340 (90%) children with untreated coeliac disease (flat mucosa) had EMA and 338/340 (99.4%) had IgG AGA and/or IgA AGA. Only 1/340 (a 7 year old boy with selective IgA deficiency) had neither AGA nor EMA. Absence of EMA is more frequent in coeliac patients younger than 2 years than in older patients (32/277 compared with 1/62). EMA were present in 4/211 (2%) of comparison subjects (normal mucosa), IgA AGA in 12/211 (6%), and IgG AGA in 74/211 (35%). The specificity of AGA cannot be calculated from these figures as they are biased. The combined determination of AGA and EMA, taking advantage of the high sensitivity of AGA and the high specificity of EMA, gives an excellent prediction of the condition of the mucosa: 247/248 patients (99.6%) with positive EMA and positive IgG AGA and IgA AGA had a flat mucosa, whereas 136/137 patients (99.3%) with neither AGA nor EMA had a normal mucosa. During a gluten free diet EMA and AGA disappear. Their presence or absence is therefore an indicator of dietary compliance. After reintroduction of gluten into the diet 110/134 (82%) of the patients who had a flat mucosa at diagnosis relapsed, but 24/134 still had a normal mucosa after 2-15 years of challenge. All these patients without a morphological relapse were less than 2 years old at diagnosis so we conclude that patients who are young at diagnosis should be challenged. AGA often reappear earlier than EMA. After one month of challenge 93% of patients are AGA and 69% EMA positive. After more than three years of gluten intake the percentage of AGA positive patients decreased to about 50% whereas the percentage of EMA positive sera was then highest (93%). Therefore EMA are more sensitive for the detection of 'silent' relapse after prolonged periods of gluten intake.

Abnormal biliary lipid composition in cystic fibrosis. Effect of pancreatic enzymes.

Because of the increased incidence of gallstones in cystic fibrosis we compared biliary lipid composition in 26 patients with cystic fibrosis, seven children with cholelithiasis but no cystic-fibrosis and 13 controls. Eighteen of the cystic fibrosis group had cholecystograms, and only one had gallstones. In 14 patients with cystic fibrosis who had stopped taking pancreatic enzymes for one week molar percentage of lipid composition accounted for by cholesterol (mean +/- S.E., 16.3 +/- 2.9) and saturation index (2.0 +/- 0.3) were comparable to values of the cholelithiasis group and higher (P less than 0.01) than those of controls. In 12 patients with cystic fibrosis taking pancreatic enzymes, molar percentage of cholesterol (8.6 +/- 1.7) and saturation index (1.0 +/- 0.1) did not differ from those of controls; in cystic fibrosis there was a preponderance of cholic over chenodeoxycholic acid both off (1.7 +/- 0.2) and on (1.9 +/- 0.3) therapy as compared to the cholelithiasis (0.7 +/- 0.1) and control (0.8 +/- 0.0) groups. The glycine/taurine ratio of conjugated bile acids were lower in enzyme-treated patients with cystic fibrosis (3.7 +/- 0.6) than in patients off treatment (6.4 +/- 1.0), but was higher (P less than 0.01) than in controls (1.8 +/- 0.2). Bile is lithogenic in untreated cystic fibrosis and responds to pancreatic enzymes.