[Mothers rooming-in in a department of pediatrics (author's transl)]. / L'hospitalisation du jeune enfant avec sa mère dans un service de pédiatrie générale. I. Caractéristiques de la population du secteur "mère-enfant" dans un service de pédiatrie générale.

The socio-economic and medical characteristics of a population of 100 children hospitalized in a "mother and child" section of a general pediatric department were studied in order to point out some objective data. This population appears socio-economically privileged. The majority of them are single children, with ages ranging from 0 to 10 years and with many babies under 6 months. There are as many working mothers in this group as in the average population of the department. Types of diseases are varied as the children were not chosen on medical criteria. Lastly, the length of the stay in hospital becomes noticeably shorter.

[Mothers rooming-in in a department of pediatrics (author's transl)]. / L'hospitalisation du jeune enfant avec sa mère dans un service de pédiatrie générale. IL Les parents, l'équipe soignante et les médecins face à l'hospitalisation de l'enfant avec sa mère.

In order to better understand the reactions arising from the admission of a child with his mother to the hospital, the reactions of the families, of the hospital staff and of doctors to the permanent presence of the mothers have been analysed. It appears that, in spite of the constraints linked with this type of hospital treatment, it does have positive aspects as compared to the usual type of hospitalization : the organization of the admission, the existence of a single medical contact, a closer observation, a team which works closer together, better possibilities of obtaining information, a shorter stay in hospital; all these improvements could be generalized. However, this concept of hospital treatment is quite demanding for the hospital staff, who must have the support of all the doctors concerned and of the administration.

[Kearns and Sayre syndrome with hypoparathyroidism and diabetes (author's transl)]. / Syndrome de Kearns et Sayre avec hypoparathyroïdie et diabète.

The authors give a very complete description of the Kearns and Sayre syndrome : external ophthalmoplegia, pigmentary retinitis, cardiac conduction disorders, cerebellar syndrome, hypacusis, reduced height, raised CSF protein levels, mitochondrial anomalies in muscle, skin, and conjunctivae, and low density zones on scanning. They emphasize the not-infrequent association of endocrine anomalies : hypoparathyroidism, which may be the first manifestation of the disease, and diabetes. The etiology of this syndrome, which occurs sporadically and starts in infancy, is still unknown.

[Surgical excision of a hepatoma complicating chronic tyrosinemia (author's transl)]. / Hépatome compliquant une forme chronique de tyrosinose. Evolution favorable quinze mois après l'exérèse chirurgicale.

A girl with chronic hereditary tyrosinemia is described in whom the diet caused an immediate resolution of tubular defect and rapid healing of the rickets. However cirrhosis was not prevented and at the age of 8 she developed a hepatoma. Complete surgical excision was possible and she remained well 15 months later. The value of blood alpha-fetoprotein levels and liver echotomography to monitor the course of the disease is emphasized. The apparent ineffectiveness of the low phenylalanine and tyrosine diet in preventing hepatic complications is discussed.

[Propionicacidemia. A report on two cases (author's transl)]. / Acidémie propionique. A propos de deux observations.

Two cases of propionicacidemia are reported. The first patient was a child aged 16 months with psychomotor retardation, severe disturbances of wakefulness, and vomiting; the second case concerned a new-born baby with metabolic acidosis and neurological disorders. In both cases the diagnosis of propionicacidemia was made after the discovery of ketonuria, and raised blood ammonia and glucose levels. Appropriate dietary measures led to great improvement in the first case, and nearly normal psychomotor development in the second case at 7 months of age. Problems related to biotin sensitivity, leucine intolerance, and raised blood ammonia levels are discussed.

[Bourneville's tuberose sclerosis in children (author's transl)]. / La sclérose tubéreuse de Bourneville chez l'enfant. Etude de trente cas.

In 20 out of a series of 30 children with Bourneville's disease, the diagnosis was made from the association of epilepsy and multiple achromic spots, before the appearance of adenomas. A total of 27 of these 30 children had these spots, and their characteristics are reviewed, as well as the average age of onset and frequency of the other typical cutaneous signs noted in this group: sebaceous adenomas, Koenen's tumors, and leather-grained skin plaques. Severe epilepsy was a constant finding and was often resistant to treatment: West's syndrome (18 cases), and Lennox Gastaut's syndrome (16 cases). Severe mental deficiency was also an almost constant finding and was increased by behavioural disorders: 11 out of the 13 children aged more than 5 years. Other locations of the disease included more particularly the ocular (8 cases), skeletal (11 cases), and renal (1 case) regions. In 5 cases, the disease had been transmitted by a parent, but in 3 of these it had not been recognized.

[Leri's melorheostosis. Apropos of 3 cases in children]. / La mélorhéostose de Léri. A propos de trois observations chez l'enfant.

Three pediatric cases of Leri's melorheostosis are reported. Growth disorders and deformities of the limbs are often the first signs in children. X ray shows areas of very opaque condensation of the long bones parallel to the main axis of the bones parallel to the main axis of the bone, and on the epiphyses, small dense and irregular islets. The course is benign but there may be orthopedic complications.

[Bronchopulmonary aspergilloma in children and bronchogenic cyst. A new case]. / Aspergillome bronchopulmonaire de l'enfant et kyste bronchogénique. Une nouvelle observation

The authors report a new case of aspergilloma developing in an intra-pulmonary bronchogenic cyst in a girl aged 10 years. A cure was obtained after surgical excision. The possibility of serious complications, such as hemoptysis, abscess formation and spread of the aspergillus, has two consequences; the first is the utility of regular supervision of any intra-pulmonary air cavity and, eventually, surgical removal. The second is the necessity of operation as soon as the diagnosis of aspergilloma has been made.

[Nanism caused by water deprivation, with transitory growth hormone deficiency]. / Le nanisme par privation d'eau, avec deficit transitoire en hormone de croissance

Report of a case of a girl admitted for alcoholic poisoning then for a toxic syndrome with considerable hypernatremia. Clinical examination showed a dwarfism (-4 S.D.) with break of the growth curve and bone-age retardation. History revealed cruelty and thirsting since the age of 2 years. Isolated somatotropin insufficiency was found. Separation from the family and free drinking induced a spectacular recovery of growth and normalization of growth hormone level. The relative role and relationships between growth hormone deficiency and chronic dehydration due to water deprivation in the physiopathology of dwarfism due to frustration are discussed.