[Rickets. Physiopathology, diagnosis, preventive and curative treatment].
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Diagnosis, Differential↗
Biomedical subjects
J Gaudelus
Publications and source records attributed to J Gaudelus.
[Pneumococcal meningitis revealing dysplasia of the bony labyrinth in an infant].
BACKGROUND: Dysplasias of the bony labyrinth are frequently associated with cerebrospinal fluid fistula and are usually discovered because of recurrent meningitis. CASE REPORT: A 1 year-old infant was admitted for a pneumococcal meningitis which appeared 2 days after the occurrence of a clear otorrhea from the right ear. The same organism was isolated from the otorrhea fluid, which also contained cerebrospinal fluid as confirmed cytochemically. The meningitis rapidly resolved with antibiotic treatment. Auditory brain stem responses were abolished from the right ear. CT of the temporal bones showed a pseudo-Mondini type labyrinth dysplasia at the right ear and Mondini type dysplasia at the left one. A translabyrinthine cerebrospinal fluid fistula was discovered by surgical exploration of the right ear, occurring through a perforation in the stapedial foot plate. The leak was cured by packing the vestibule and obturating both oval and round windows. Three years after the operation, the child did not experience any further episode of otorrhea or meningitis. CONCLUSIONS: Features suggesting a translabyrinthine fistula, especially otorrhea and deafness, should be systematically searched in any child with bacterial meningitis. Closure of these fistulas can prevent severe infectious recurrences.
Cerebrospinal Fluid Otorrhea↗
[Fulminant encephalopathy due to Shigella flexneri].
BACKGROUND: In some rare cases, Shigella flexneri infection may be associated with fulminating encephatopathy. CASE REPORT: A 6-year-old girl was admitted with fever, diarrhea and consciousness abnormalities. Brain CT scan was normal. Shigella flexneri type 2 was isolated from the stools. Despite antibiotic therapy, the encephalopathy was lethal. Two siblings were also infected, the first with only diarrhea, the second with headache and mild consciousness abnormalities: both children had a favorable course. CONCLUSIONS: Shigella flexneri encephalopathy is associated with high mortality rate in developed countries. Presence of headache could be a poor prognostic factor.
Child↗
[Treatment of migraine in children].
Simple migraine attacks are usually controlled by rest and an analgesic (acetylsalicylic acid or paracetamol), eventually associated with metoclopramide. More severe cases with failure of these measures may benefit from antimigraine medications such as ergotamine derivatives. Preventive treatment is only indicated in case of frequent (> or = 3 per month) and complicated attacks.
Adolescent↗
Corynebacterium diphtheriae osteomyelitis in an immunocompetent child: a case report.
UNLABELLED: Septic osteomyelitis of the hip in a previously healthy child is described. A weakly toxigenic Corynebacterium diphtheriae strain was isolated from the bone aspirate. The results of the treatment were rapidly satisfactory, after surgical drainage and antibiotic therapy with pristinamycin. CONCLUSION: This case report shows that C. diphtheriae has not disappeared in the developed world and can be responsible of systemic infections.
Child, Preschool↗
[Massive chronic fetomaternal hemorrhage].
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Chronic Disease↗
The Wiedemann-Rautenstrauch neonatal progeroid syndrome: a case report and review of the literature.
We present the case of a post-term newborn with intrauterine growth retardation, pseudohydrocephalus, a tiny face and mouth, thin wrinkled skin, an aged appearance, lipoatrophy and a normal cranial CT scan, suggestive of the Wiedemann-Rautenstrauch neonatal progeroid syndrome. He developed hypothyroidism on day 18 due to a partial organification disorder as did a later born sib. His mental development remains normal at age 2 with delayed growth at -2.5 SD. The case is presented and discussed and the literature is reviewed.
Fetal Growth Retardation↗
[Homozygote CB deficiency revealed by recurrent Neisseria meningitidis infections in an adolescent].
BACKGROUND: Meningococcal infections associated with late complement component deficiency are rarely severe and usually occur during adolescence and adulthood. We report severe manifestations in a boy in whom the first episode appeared early. CASE REPORT: A 14 year-old gypsy boy was admitted because of a febrile meningococcal meningitis that was complicated by a rapidly extensive and necrotic purpura, obnubilation and clotting abnormalities without hemodynamic anomalies. The patient was given symptomatic therapy and a 12-day course of antibiotics that resulted in rapid and complete recovery. Medical history of this patient showed that he had been admitted at the age of 3 years for a severe febrile purpura with septic shock and clotting abnormalities followed by rapid and complete recovery after symptomatic and antibiotic therapy. No germ had been then isolated. The complement system was studied 3 weeks after the second hospitalization: total hemolytic complement activity could not be detected and C2, C3 and C4 were normal. Examination of the terminal pathway-revealed total C8 deficiency. The patient received meningococcal vaccine and was discharged on oral penicillin prophylaxis. He remained healthy during the ensuing 4 years. CONCLUSIONS: Meningococcal infections associated with late complement component deficiency are generally uncomplicated but they remain potentially severe. Early screening for this late complement component deficiency should be considered after severe clinical manifestations.
Adolescent↗
[Hyperglycemia revealing neonatal infection].
BACKGROUND: Hyperglycemia as the first manifestation of neonatal sepsis is rare. CASE REPORT: A breast-fed neonate was admitted at the age of 6 days because of vomiting. Group B streptococci had been isolated in secretions of the ear at birth but the neonate had not been treated. At admission, physical examination was normal but glucosuria, without ketonuria, and hyperglycemia (9.7 mmol/l) were noted. Because hyperglycemia was not explained by usual causes, the baby was systemically given antibiotics. The next day, blood, spinal fluid and urine cultures taken on admission were positive for group B streptococci while blood fibrinogen and C-reactive protein were increased. Hyperglycemia and glycosuria were normal after 24 hours of antibiotic therapy and follow-up was uneventful. Subsequently, the same bacteria was isolated from the mother's milk. CONCLUSION: Isolated hyperglycemia may reveal an infection; therefore its discovery might contribute to early diagnosis and treatment.
Breast Feeding↗
[Vitamin D. Let us practice supplementation].
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Animals↗
Optic atrophy, microcephaly, mental retardation and mosaic variegated aneuploidy: a human mitotic mutation.
A 12-year-old girl presents with optic atrophy, pale papilla, amblyopia and microcephaly (-3 s.d.) with mild mental retardation and facial dysmorphism. She had mitral insufficiency with mitral prolapse and moderate short stature (-2.5 d.s.). She had normal flash visual evoked potentials, normal electroretinograms and electrooculograms and normal cranial CT scan as well as other lab tests to rule out malformations, tumors or multiple sclerosis. Her lymphocyte karyotype showed a variegated mosaicism with: 2 cells with 49, XX, +mar,+mar,+mar; 21 cells with 48, XX, +mar,+mar; 57 cells, with 47, XX,+mar; 20 cells with 46,XX; while parental karyotypes were normal. This syndrome therefore associates optic atrophy, mental retardation and microcephaly and short stature with chromosomal instability in the form of variegated mosaicism.
Abnormalities, Multiple↗
Heterogeneity of Clostridium difficile isolates from infants.
In order to improve our understanding of the role of Clostridium difficile in infants we characterised the strains isolated from this population. The production of toxin A and toxin B was studied. The toxin A, playing a major role in the disease, was searched for in faecal samples. The serogroup of the isolates was determined because some serogroups have been shown to be more pathogenic than others. Over a 9-month period, 102 faecal samples from 102 hospitalised infants (0-12 months) were analysed and 26% of the children were colonised with C. difficile. Fifteen isolates secreted neither toxin A nor B (62.5%). Nine isolates were toxigenic and secreted both toxins (37.5%). Of the eight toxigenic strains tested, six were from serogroup H and two serogroup K. Of the 13 nontoxigenic strains tested, 8 belonged to serogroup D, 2 to serogroup X, and 1 each to serogroup A, serogroup B and serogroup C. Three infants out of 102 studied had toxin A in their faeces. In summary, the infants can be colonised by (1) nontoxigenic strains, most of them from nonpathogenic serogroup D, without toxin A in the faeces; (2) toxigenic strains of virulent serogroups H and K, with or without toxin A in the faeces. Although some infants had diarrhoea, none needed a specific treatment for C. difficile. No specific C. difficile pathology could be retained and different mechanisms are advanced to explain this absence of pathogenicity.
Bacterial Toxins↗
Enterobacter cloacae cross-colonization in neonates demonstrated by ribotyping.
The intestinal colonization by Enterobacter cloacae strains with a derepressed cephalosporinase was studied in a paediatric ward between February 1990 and January 1991. Environmental sampling was performed simultaneously. Fifty-two isolates were recovered from 200 neonates (stool, blood) and 14 strains were isolated from the neonatal environment. An epidemiological study based on the typing of 36 Enterobacter cloacae isolates was carried out using antibiotyping, biotyping and ribotyping methods. The isolates selected were from 21 neonates (24 isolates), the neonatal ward environment (8 isolates) and from other wards (4 isolates). Thirty-two isolates had the same antibiotic resistance pattern, corresponding to a derepressed cephalosporinase and resistance to the following aminoglycosides: kanamycin, gentamicin, tobramycin and netilmicin. No predominant biotyping pattern could be established. Ribotyping done with two endonucleases (EcoRI and BamHI) showed 28 Enterobacter cloacae isolates to have a single pattern. Ribotyping was the most discriminating method used in this study, permitting identification of cross-contamination with Enterobacter cloacae in the paediatric ward.
Bacteremia↗
Bacillus Calmette-Guérin infection after vaccination of human immunodeficiency virus-infected children.
The use of Mycobacterium bovis/Bacillus Calmette-Guérin (BCG) to vaccinate against tuberculosis remains controversial. The development of tuberculosis in human immunodeficiency virus (HIV)-infected children demands specific evaluation of the risk/benefit ratio of BCG vaccination in this situation. In our institution 9 of 68 HIV-infected children vaccinated with BCG before the diagnosis of HIV infection was suspected developed vaccine-related complications: 7 of these children had a large satellite adenopathy with or without skin fistulae, whereas the other 2 had disseminated BCG infection beyond the satellite ganglion (involvement of the spleen and mesenteric and mediastinal lymph nodes in one case and the liver and lungs in the other). The children were vaccinated soon after birth; no particular problems were observed at that time, but complications appeared 3 to 35 months later. All but one of these children had a rapidly progressive form of HIV disease. The possibility of delayed local or disseminated BCG infection must be considered in analysis of the risk/benefit ratio of vaccination of HIV-infected children. The prognosis of HIV infection must be taken into account, even if the child is asymptomatic when vaccination is being considered.
BCG Vaccine↗
[Rickets in older children in France. Failure to screen and treat a population at risk].
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Adolescent↗
[Chronic intestinal pseudo-obstruction and delayed myenteric ganglion cell maturation].
BACKGROUND: Enteric nerve cells begin to mature during the last trimester of pregnancy and become mature only after birth. The degree of maturation seems to be related to bowel motility. CASE REPORT: A girl was born from a pregnancy complicated by hydramnios, and did not pass meconium before the 64th hr of life. Barium enema on day 4 showed a left microcolon with no distension of the transverse colon. Tests for cystic fibrosis were negative. On day 12, the patient presented with septicemia due to Pseudomonas maltophylia. Parenteral alimentation by central catheter was instituted. Surgical rectal biopsy showed that the number of ganglion cells was normal but the cells were immature. Progressive feeding was possible for the 3rd month of life. A second rectal biopsy at 3 1/2 months showed some remaining immature ganglion cells. CONCLUSION: Immature ganglion cells can account for neonatal functional intestinal obstruction, as has been established for the small left colon syndrome. The progressive loss of symptoms seems to be correlated with histological maturation of the neurological apparatus of the large bowel. Severe complications, such as occlusion, sepsis, nutritional disorders can occur during this long period of functional intestinal obstruction.
Female↗
Unbalanced karyotype due to adjacent 1 segregation of t(11;22)(q23.3;q13.2).
The 11q;22q translocations, whatever the breakpoints may be, are of particular interest because of their propensity to 3:1 segregation of the chromosomes at meiosis I. Until now, no unbalanced karyotype resulting from 2:2 adjacent segregation was published among offspring of 11q;22q translocation carriers. The authors report the case of an unbalanced karyotype due to adjacent 1 segregation of a maternal translocation (11;22)(q23.3;q13.2). The proband's karyotype was 46,XX,-22,+der(22)(11;22)(q23.3;q13.2)mat. This finding demonstrates that adjacent 1 segregation is possible in t(11;22) with breakpoints at 11q23 and 22q13, and can lead to birth of viable infants.
Abnormalities, Multiple↗