[Algodystrophy, a little known disease in children. General review apropos of 4 cases].
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Biomedical subjects
Publications and source records attributed to A M Bertrand.
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Of 99 patients with acute lymphoblastic leukemia in first bone marrow or isolated CNS relapse seen between 1968 and 1980, 48 were treated without standardized protocol and 51 according to a relapse protocol. Of 16 patients with bone marrow relapse after cessation of the initial treatment 6 survived 8 1/2 years or more, of 66 with bone marrow relapse while on therapy only 4 survived. All of the latter were low risk patients with an initial WBC of less than 20 x 10(9)/l and no enlargement of the mediastinum. All of the 17 patients with isolated CNS relapse died. The relapse protocols used probably improved the chances of children with first bone marrow but not of those with isolated CNS relapse.
Of 45 children with ALL who had a first hematological recurrence between 1981 and 1984, 33 relapsed while still on treatment and 12 after cessation of therapy. Of the former 1 of 16 high risk (initial WBC greater than or equal to 20 x 10(9)/l and/or enlargement of the mediastinum) and 5 of 17 low risk patients (initial WBC less than 20 x 10(9)/l and no enlargement of the mediastinum), of the latter 6 patients survived after a minimum follow-up of 20 months. During the same time period, a first isolated CNS relapse was observed in 24 children of whom 16 survived. These results suggest that at the time of evaluation 1. the prognosis of children with ALL in first hematological relapse during the years 1981-1984 was not significantly different from that of similar children treated earlier; and 2. the prognosis of children with isolated CNS relapse had improved.
Four patients with aspergillosis of the central nervous system collected in less than two years are reported. Three patients had hematologic malignancies (acute myelogenous leukemia, Hodgkin's disease) and were treated with corticosteroids and chemotherapy. One patient received antimicrobial agents fort a post operative meningitis (after acoustic neuroma surgery). Analysis of these cases and review of literature available us to point out the increased frequency of invasive and cerebral aspergillosis particularly in immunocompromised hosts treated by cytotoxic drugs or broad spectrum antibiotic therapy. Diagnosis is very difficult because: --there are non specific radiologic features for aspergillus granuloma, abscess, aneurysm or meningitis, --blood and cerebrospinal fluid cultures are invariably negative, --serologic tests have limited value in immunosuppressed patients (poor capacity to elaborate antibodies). Diagnosis can be made only by surgical biopsy who isolate fungal elements. However diagnosis in greatest cases is only made at autopsy. Treatment consist by antifungal drugs administered intravenously and surgery when it is possible. Prognosis of cerebral aspergillosis is very poor and mortality rate very high about 70%.
Eight children with steroid-response nephrotic syndrome were investigated to study the relation-ship between steroid responsive syndrome, allergy and HLA-antigens. Six of them had clinical and/or biological allergic symptoms. The allergen was identified in four out of six cases (grass-pollen 2 cases, house dust 1 case, frullania 1 case). Six children had DR7 HLA-antigen, five had B12 antigen. Three of the four children with cortico-dependent nephrotic syndrome have associated B12-DR7 HLA antigens. Search of an allergen seems to be of interest in children with steroid-responsive nephrotic syndrome, since eviction of the allergen may be proposed as a complement to cortico-therapy.
Droperidol 1:50 given with Fentanyl increases the analgetic action of the latter by one third. This effect is not enhanced by increasing the dosage of droperidol.
A 15 years old boy was deferred to our department with growth hormone deficiency, deafness with middle ear bones involvement, and a facial dysmorphy called Binder syndrome. He presented ocular abnormalities with juvenile glaucoma and iris anterior insertion and goniodysgenesis. All these pathologic features are linked with the development of the neural crests. In our knowledge, it is the first so complete clinical association reported, which emphasizes the important role of the neural crest cells in early organogenesis.
Simultaneous monitoring of esophageal and gastric pH in infants makes clear some peculiarities of digestive physiology and improves the sensibility of the simple method, usual mean of diagnosis of GER. In that way, a gastro-esophageal pH gradient is showed; gradient absolutely necessary to a good interpretation of this test. In twenty-two infants, younger than one year, continuous and extended monitoring of esophageal and gastric pH was realised with a specially-designed-combination microelectrode. Results pointed out that, after a meal, especially a milked meal, the post prandial period isn't suitable for the diagnosis of GER. Indeed, the gastric acidity is buffered, sometimes during many hours: that decreases or cancels the gastro-esophageal pH gradient. Besides, transient neutralisation periods of gastric acidity may occur, perhaps related with duodenogastric-biliary reflux. Before two months, the gastric pH is less acid that involves, one more time, on insufficient gastro-esophageal pH gradient for the diagnosis of GER.