[Primary hyperparathyroidism in 2 adolescents]. / Hyperparathyroïdie primitive chez deux adolescents.

The authors report on two cases of primary hyperparathyroidism (PHP) in adolescents. In a 15-year-old boy, the clinical picture included skeletal, dental, renal and psychological manifestations. In the second case, the first symptom was urolithiasis at age 12; following a period without any important clinical event, the diagnosis was established when the patient was 18. In both cases, increased calcium and low phosphorus plasma levels associated to moderately elevated plasma parathyroid hormone levels were noted. CT scan and ultrasounds were normal but MRI showed an enlarged gland in the second case. The surgical exploration of the neck revealed an adenoma in the first case and a hyperplastic gland in the second one. Surgery was followed by an immediate improvement of both clinical and laboratory findings. These observations bring us to examine the spectrum of PHP in childhood, the difficulties in biological diagnosis and localizing techniques, and the recent results of surgical treatment.

[Prognosis of canal treatment. Success and failure]. / Pronostic du traitement canalaire. Reussites et échecs.

This clinical review confirms the very positive prognosis for endodontic therapy, conditional to strict compliance with imperatives. Endodontics failures are transient and can be remedied. The authors define the attitude to be adopted and the measures to be taken in accordance with the type of difficulty encountered.

[Plant prickle arthritis in children. Apropos of 6 personal cases]. / Les arthrites à piquants végétaux de l'enfant. A propose de 6 observations personnelles.

Six children with arthritis after penetration of a vegetable prickle into the joint are reported. The affected joints were the knee (5 fold) and the elbow (1 fold). The prickles were of palm-tree (2 cases), sloe-tree (2 cases) or unknown (2 cases) origin. The culture of the synovial fluid was negative and the diagnosis was based on microscopic examination of the centrifugated fluid. Four patients needed synovectomy.

[Cysteamine in the treatment of cystinosis in children. In vitro and in vivo studies]. / La cystéamine dans le traitement de la cystinose infantile. Etudes in vitro et in vivo.

The effect of cysteamine was studied in 6 children with nephropathic cystinosis. In 3 of them an in vitro study on fibroblasts was performed. The cystine content of fibroblasts was immediately diminished (about 90% of total cystine content) as soon as the concentration of cysteamine in the medium was greater than or equal to 0,1 mmole/l. In vivo, 50 to 89 mg/kg/day of cysteamine was administered for 9 to 37 months (mean 21,3). There was no adverse reaction. In all cases a dramatic decline in leukocyte cystine level was observed (in 5 cases the level was within the range seen in clinically unaffected heterozygotes). Growth was not improved. The renal function was stabilised in 3 cases. Photophobia which was present in 4 children decreased in 2 cases or disappeared in 2 cases.

[Idiopathic syndrome of inappropriate secretion of antidiuretic hormone]. / Syndrome de sécretion inappropriée d'hormone antidiurétique idiopathique.

The authors report a case of idiopathic, chronic inappropriate secretion of antidiuretic hormone (S I A D H) in a 31 months old boy. This inappropriate secretion of ADH was demonstrated by three water tolerance tests and by the bioassays of ADH in urine. These findings were detected five months later suggesting chronic S I A D H. After a review of the literature the mechanism and the treatment of this disease are discussed.

[Dyssegmental dwarfism. A new case]. / Nanisme dyssegmentaire. Une nouvelle observation.

In a 3 1/2 month old child with micromelic dwarfism, a case of dyssegmental dwarfism is reported. This affection is characterized, on one hand, by a shortening and a squat aspect of long bones with diaphyseal curve, and on the other hand, by a trouble of vertebral segmentation associated to a variability in the size of vertebral bodies. The differential diagnosis appears adequate to differentiate the dyssegmental dwarfism from other types of neonatal chondrodysplasia, in particular from Kniest syndrome and micrognathia dwarfism.

[Diffuse cortical nephroblastomatosis. Apropos of a case]. / La néphroblastomatose corticale diffuse. A propos d'une observation.

The authors report a case of massive bilateral, and asymmetric nephroblastomatosis which was treated successfully by nephrectomy and chemotherapy. The authors discuss the clinico-pathologic significance of the present case and insist upon the requirement of a treatment (i-e-chemotherapy) in every case.

Severe ornithine transcarbamylase deficiency. Two and a half years' survival with normal development.

The clinical course and management of a boy with severe ornithine transcarbamylase deficiency are described. In addition to treatment with sodium benzoate and amino acid keto analogues, mannitol may be useful in hyperammonaemia and nocturnal gavage feeding aids maintenance treatment.

[Intramural hematoma of the duodenum and chronic pancreatitis after jejunal biopsy]. / Hématome intramural du duodénum et pancréatite chronique après biopsie jéjunale.

A 12 year-old boy presented with duodenal hematoma in the hours following small bowel biopsy. Two years later, he presented with abdominal pain and diarrhea. Investigations allowed to find a chronic calcified pancreatitis, which is suggested to be the consequence of the previous duodenal hematoma.

[Chronic inflammatory rheumatism in children and the HLA B27 antigen]. / Rhumatisme chronique inflammatoire de l'enfant et antigène HLA B 27.

The characteristics and evolution of 15 HLA B 27 positive children presenting a chronic arthritis have been studied. Most typical was an asymmetric oligoarthritis usually of the lower limbs, particularly the knees, in a boy over 10 years old. This symptomatology resembles juvenile ankylosing spondylitis of peripheral onset. Follow up is not yet long énough to allow definite assimilation.

[Corynebacterium acnes osteitis. A case in a child]. / Ostéite à Corynebacterium acnes. Une observation chez l'enfant.

The authors report a case of Corynebacterium acnes osteitis in an 11 year old girl, involving the upper of the right tibia. The lesion resembled a malignant tumour radiologically and tuberculosis histologically. Scintigraphy revealed involvement of the seventh right rib. 3 months treatment with Pristinamycin resulted in cure.

[Case of mucolipidosis type I with a primary alpha-D-neuraminidase deficiency]. / Une observation de mucolipidose de type I par déficit primaire en alpha-D-neuraminidase.

The authors describe a case of mucolipidosis I, the 9th reported in the world literature. The diagnosis was suspected in a 5 year old boy, from the appearance of hurler-like facial features. Lumbar kyphosis, dysostosis multiplex, cherry-red macular spot and foam cells in the bone marrow and it was confirmed by the characteristic oligosacchariduria (on thin layer chromatography) and excess sialic acid in cultured fibroblasts which also showed profoundly diminished activity of alpha D Neuraminidase (on two substrates - N-acetyl-Neuramin Lactose and 4 Methyl umbelliferyl N-acetyl-Neuraminide). The parents had intermediate enzyme activities, suggesting heterozygotism. After a review of the literature, the authors attempt to define the place of mucolipidosis I in the sialidoses.