Annales de pediatrie最新文献

Two studies of uremia-induced chronic metabolic acidosis (CMA) were carried out to determine: 1) the level of acidosis beyond which growth failure occurs; 2) the protein metabolism anomalies which are associated with growth failure. Rats rendered uremic by subtotal nephrectomy were fed a diet containing sufficient protein amounts (30% casein) to induce CMA. CMA was left uncorrected in half the rats (group A) and was corrected by administration of bicarbonate in the other half (group B). 1) Fifty-two group A rats were compared with 52 group B rats matched for renal function. Results showed that a) CMA failed to reduce food intake; b) weight gain decreased only when CMA was profound (pH < 7.20) whereas reductions in length gain occurred at less severe levels of acidosis (pH < 7.25) suggesting that bone may be more susceptible to CMA than muscle mass. 2) Protein fractional synthesis rate was evaluated in skeletal muscle after a flooding dose of 3H-phenylalanine in group A rats (pH 7.22 +/- 0.01, HCO3-: 15.2 +/- 0.8 mmol/l) and group B rats matched for renal function. Values were identical in both groups (10.4 +/- 0.5 vs 10.8 +/- 0.5%/day). However, fractional muscle protein accretion rate was decreased in group A rats. These data demonstrate that CMA-associated growth failure in uremia is due to increased breakdown of protein with no change in protein production.

The authors report on a infant who presented with an auto-immune enteropathy characterized by the association of a protracted diarrhea, a neonatal insulin-dependent diabetes, and a dermatitis and who developed a nephrotic syndrome at 4 months of age. A renal biopsy showed a membranous glomerulonephritis (MGN) with IgG linear deposits along the tubular basement membranes (TMB). By indirect immunofluorescence anti-enterocyte antibodies together with anti-TMB antibodies and anti-renal brush border (BB) antibodies were found in the serum of the patient. The patient received various immunosuppressive drugs that failed to improve the disease. In the course of the disease the anti-TBM antibodies disappeared progressively but the BB antibodies persisted. A review of the literature indicates that renal involvement is not uncommon in auto-immune enteropathy and in 5 cases it has been reported as being characterized by a nephrotic syndrome related to the presence of a MGN. In 4 of these cases MGN was associated with the presence of anti-TBM antibodies and in the remaining one with anti-BB antibodies. This case report shows that in human pathology, auto-antibodies to BB proteins may, as well as in experimental models, be responsible for the development of a MGN. It suggests a close relationship (probably a common epitope) between the renal BB proteins and the proteins of the gut epithelium.

Ultrasound is the first imaging modality used to evaluate acute renal disease in neonates. Normal findings and abnormalities seen in venous or arterial thrombosis, hemodynamic shock, or sepsis are reviewed. Transient changes due to intoxications, tubular disorders, or congenital nephrotic syndrome are considerably less common.

From 1976 through 1991, 50 renal transplants (with 7 kidneys from living related donors and 43 cadaver kidneys) were performed in 47 children under five years of age (range 11 to 59 months) at the Enfants-Malades Hospital, Paris, France. Donor age ranged from 3 months to 53 years. Six of the seven kidneys from living related donors are currently functioning after a follow-up of 6 months to 8 years, whereas actuarial survival of cadaver kidneys was 70% at one year and 66% at five years. The main cause of graft loss was vascular thrombosis (40% of lost kidneys). In the most recent years of the study period, graft survival was substantially improved by routine prophylactic anticoagulant therapy with low-molecular weight heparin: one-year graft survival rate was 83% in the 23 children grafted between 1989 and 1991. Posttransplantation growth was closely related to quality of graft function: among the 29 children with sufficiently long follow-up the 19 patients with normal renal function exhibited normal or catch-up growth, whereas the ten patients with chronic renal failure or recent rejection had poor growth. Complications were uncommon with the exception of hypertension. Mortality rate was 12%, i.e., only slightly higher than in older pediatric kidney recipients. Achievement at school was normal in most cases (with a lag in only five patients). Provided effective therapy is given to prevent the main adverse outcome (i.e., vascular thrombosis), renal transplantation does not involve excessive risks even in infants as young as one year of age.

A case of multicystic encephalomalacia in a twin is reported. The other twin died in utero at 32 weeks gestational age. Because there was no evidence of fetal distress the pregnancy was allowed to continue until 36 weeks gestational age. Injuries to the surviving twin due to disseminated intravascular coagulation (DIVC) and vascular thrombosis or to anoxia and ischemia may occur when there are anastomoses between the circulatory systems of the two twins, i.e., in monochorionic pregnancies. The classically recommended strategy is to wait for adequate maturity of the surviving fetus (36 weeks). It is suggested that this attitude may be overly expectant and may deserve reappraisal.

Eleven cases of simultaneous HbC hemoglobinopathy and beta-thalassemia were detected during a study of 11,200 subjects at high risk for inherited hemoglobin anomalies. In seven cases, main clinical manifestations were anemia and enlargement of the spleen, whereas the four other patients were apparently free of symptoms and were diagnosed during routine tests in family members of affected patients. Microcytosis and hypochromia were found in every case. Most of the patients were from the North-Western part of Tunisia. Blood transfusions were required in only one patient, who was an infant with HbC/beta + thalassemia.

A case of acquired giant lobar emphysema in a premature triplet born at 31 weeks gestational age and treated by artificial ventilation is reported. Corticosteroid therapy was remarkably successful.

The treatment of insulin-dependent diabetes mellitus in a child generates new constraints in the family and requires adjustments of the daily routine. Refusal of these changes may lead to poor compliance with the treatment regimen. Poor or mistaken daily results and repeated episodes of ketoacidosis may occur as a result. Clandestine injection of insulin responsible for apparently inexplicable episodes of hypoglycemia is less common. Three new cases are reported herein. Clinical diagnosis is fairly easy and biological findings can provide confirmation. Acknowledgement of the injections by the patient is important in order to gain insight into his or her motives. Depression is known to be common among diabetics and the injections may be a symptom of depression. Another possibility is that the child expects to achieve an "irrational recovery" from the disease by taking control over the treatment. Furthermore, a child with access to a highly active drug like insulin can use this situation to acquire and maintain exceptional status within the family. The diagnosis of factitious hypoglycemia requires in every case an in-depth evaluation which may lead to psychotherapy for the child or for the entire family.

Ureaplasma urealyticum and Mycoplasma hominis were recovered from nasopharyngeal aspirates from 25% of 63 infants admitted to a neonatal unit; this proportion is significantly higher than that seen in a control population of maternity ward babies (0%). Birth by cesarean section was associated with a reduced risk of recovery of mycoplasmas. No specific diseases were significantly associated with recovery of mycoplasmas; furthermore, no obstetrical factors were associated with recovery of mycoplasmas from the neonates and no association was found between mycoplasma infection and respiratory distress. However, fetal distress, probably of multifactorial origin, was found in 44% of neonates with positive cultures for Ureaplasma urealyticum; this proportion was significantly elevated as compared with the subgroup of infants negative for U. urealyticum, suggesting that fetal distress may increase the infectivity of this opportunistic organism.

Development of the digestive tract intestinal flora is the result of a specific selection process to which the multiple maternal or environmental bacteria that penetrate into the neonatal gut are subjected. In breast-fed infants, Escherichia coli and streptococci are the first bacteria to appear in the gut. They are usually, but not always, followed by a population of Bifidobacterium which quickly becomes predominant. In bottle-fed infants, the intestinal flora is more variable and often includes, in addition to the organisms mentioned above, other enterobacteria and a wider range of obligate anaerobes. Studies of experimental models have shown that the nature of milk fed to the offspring and even the lactating mother's diet have substantial effects on the sequence of development of the neonatal intestinal flora. A large number of factors capable of inhibiting or permitting in vitro growth of various bacterial species have been identified in milk. However, no in vitro activity of these factors added to milk has ever been demonstrated. These factors include "bifidus factors", which promotes the growth of Bifidobacterium, and lactoferrin and immunoglobulins, which prevent colonisation of the gut by pathogenic enterobacteria. Immune factors in milk play a key role in interactions between the microbial flora and gut mucosa. However, they seem to have no effect on the growth of bacterial populations in the gut lumen. A number of pioneer bacteria, which are the first to arrive in the gut, are capable of effectively blocking growth of other bacteria introduced later in the ecosystem. In some instances, these pioneer bacteria also inhibit production of toxins by pathogenic species. Consequently, it is important to adhere to the recommended gradual changes in diet which allow these species to sequentially colonize the gut.