Biomedical subjects
Publications and source records attributed to S Nathanson.
The prevalence of urolithiasis is higher in patients with cystic fibrosis than in the normal population. We report on a case of a 12-year-old child with cystic fibrosis who presented flank pain which revealed a calcium oxalate urolithiasis. Evolution was satisfactory with pharmacological and dietary treatment. The principal mechanisms of lithogenesis in patients with cystic fibrosis are described and preventive treatment is discussed.
Antibiotics are usually used to prevent childhood recurrent urinary tract infections: cystitis or pyelonephritis. The mechanism of action of these antibiotics, although imperfectly known, seems to be double: the antibiotic acts by its bactericidal effect, but also probably for minimal concentrations by reducing adhesion capability of bacteria to the urothelium. The most commonly used molecules are cotrimoxazole, trimethoprime, pivmecillinam, cefaclor and nalidixic acid. However all have not been studied rigorously as for their prophylactic capacity, and in particular very little is known for patients presenting with vesico-ureteral reflux.
Aerosol delivery of antibiotics offers the potential to achieve high antibiotic concentrations at the site of infection while reducing the risk of systemic untoward effects because of minimal resorption into the bloodstream. We reviewed knowledge acquired in this field over the two latter decades. While the earliest data were obtained with gentamycin, the most conclusive evidence presently regards aminoglycosides and colistin. Aerosol delivery of tobramycin was recently improved with the development of a new formulation for inhalation. Coupled with an adequate nebulization system, intermittent treatment with tobramycin for inhalation has been evaluated in randomized placebo-controlled studies. These studies have demonstrated a significant improvement of respiratory function.
Streptococcus pneumoniae-induced hemolytic uremic syndrome (HUS) is known to be a severe acute disease leading to death in one-third of cases, but data regarding the long-term follow-up are lacking. A new series of 11 patients with Streptococcus pneumoniae-induced HUS associated with meningitis and pneumonia constituted a multi-center review. Among 9 patients with a severe acute infectious disease, 3 died from meningitis and 1 from neurological sequelae after a partial recovery of renal function. The mean duration of dialysis was 32 days in patients with acute renal failure who survived the acute infectious period. Cortical necrosis was documented in five of six kidney specimens. Among the 7 surviving patients, 5 developed end-stage renal failure 4-17 years later.
A patient with homozygous factor H deficiency presented with hemolytic uremic syndrome (HUS) at the age of 7 months. After a 2-year period of stability, renal failure and erythrocyte fragmentation recurred between the age of 3 and 4 years. Fresh frozen plasma infusions allowed renal function to be improved and erythrocyte fragmentation to be stopped. Withdrawal of plasma therapy led to a relapse of the biological signs of HUS.
UNLABELLED: Alcaptonuria is a rare hereditary disease, characterized by an abnormal blackish coloration of the urine and dark pigmentation of the conjunctive tissue which is due to a deficiency in homogentisate 1,2-dioxygenase (HGO), a phenylalanine catabolizing enzyme. An accumulation of homogentisate (HGA) is then formed, and is responsible for the dark coloration which only occurs after the urine has been exposed to air over a period of time. Signs of this disorder therefore frequently remain unnoticed during childhood, because the urine requires a relatively long exposure to air before it changes color. Diagnosis is generally made at a later date, during adulthood, following complications such as ochronosis, inflammatory arthritis, or urinary calculi. CASE REPORT: In this study, the case has been described of alcaptonuria diagnosed in a five-month old infant. No efficient cure has yet been found, although certain treatments, including high doses of vitamin C, do seem to have a beneficial effect on limiting the complications associated with this disorder. Early diagnosis whenever possible is therefore important. CONCLUSION: This case report is interesting because of the early diagnosis involved. In the event of any abnormal coloration of the urine, diagnosis may be established via the addition of an alkylating agent, and the levels of HGA determined by chromatography.
Despite the progress in the knowledge of iron metabolism, its precise assessment remains uneasy. Serum ferritin assesses the extent of storage iron. Serum iron and the percentage of transferrin saturation evaluate the tissues' iron supply. But these parameters are indirect measurements and they do not reflect marrow iron supply. Serum transferrin receptors, red cell ferritin and red cell zinc protoporphyrin are good indicators of this iron supply to the erythroid marrow for erythropoiesis. Since the introduction of recombinant human erythropoietin, it has become apparent that an adequate iron supply to the bone marrow is essential for a satisfactory hematopoietic response. In some cases, despite a high baseline ferritin, iron may not be sufficiently released from reserves in the bone marrow, resulting in a functional iron deficiency. The percentage of hypochromic red cells and reticulocyte haemoglobin content tends to reflect direct marrow iron status.
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UNLABELLED: Few data are available about pediatric imported malaria, whereas the number of cases seems in constant increase. PATIENTS AND METHODS: all pediatric malaria cases diagnosed by a positive thin or thick blood film at the Versailles Hospital, from January 1997 to December 2001, were studied retrospectively. RESULTS: sixty cases of pediatric imported malaria were studied. They were 58 cases of uncomplicated malaria and 2 cases of severe malaria; 85% of the children had travelled to sub-Saharan Africa and 15% to Oceania; 90% of the children were of African origin. Plasmodium falciparum was found alone in 84% of the cases. The anti-malarial chemoprophylaxis was inappropriate in 92% of the cases. No child had profited from preventive measures against mosquitos. Fever > 37,5 degrees C was observed in 100% of the cases. The other clinical signs were present in less than 50% of the cases. The median of haemoglobin and platelet was 10.5 g/dL and 141,000/mm(3), respectively. After treatment, the evolution was good in all the cases, without relapse or any consequences. DISCUSSION/CONCLUSION: our study, in agreement with the national data, confirms the increase in the number of case of pediatric imported malaria, and underlines the mediocrity of the prevention, in particular in term of anti-malarial chemo-prophylaxis. These data, in a context of regular increase of international travels to endemic areas, suggest the necessity to improve the information of the general public, and the urgency of a better staff training of health care workers concerning malaria, in order to improve the prevention and the treatment of this potentially fatal disease.