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Biomedical subjects

D Rapoport

Publications and source records attributed to D Rapoport.

At least 19 recordsLinked to original sources
PubMed1994

Clinical outcome of long-term management of patients with vitamin B12-unresponsive methylmalonic acidemia.

We performed a retrospective study of all patients with methylmalonic acidemia diagnosed during the past 20 years. Only those patients who were nonresponsive to vitamin B12 in vivo and in vitro were included. The final study group consisted of 26 patients, of whom 16 had a neonatal (early) onset; in 10 patients the diagnosis was made after 2 months to 2.2 years (late onset). Of the early-onset patients, 14 (87%) died, with a mean survival time of 1.5 years (range, 10 days to 2.5 years), whereas four of the late-onset patients (40%) died (range, 1.2 to 15 years). At present, eight patients are alive; their mean age is 4.6 years (range, 1 to 10 years). In the early 1970s, treatment was based on the principles of treating patients with phenylketonuria: restricting natural protein intake and supplementing essential amino acids, vitamins, and trace elements. After about 1980, nasogastric tube feeding became a mainstay of the therapy, natural protein restriction became stricter, and the use of essential amino acid mixtures diminished. Carnitine was added to the therapy and, in later years, metronidazole. Since these changes were implemented, the number of episodes of metabolic decompensation and hospitalizations has decreased. Mean survival time of the patients, in particular those with early onset, has only slightly improved, partly because of psychosocial problems in many of these families. Almost all the patients, especially those with early onset, had some degree of neurologic impairment and mental retardation, and many patients were at less than 2 SD for weight or height or both. In contrast, the neurologic and mental status of the late-onset patients was frequently normal, and their weight and height were more often within normal limits. Our results show that the treatment of methylmalonic acidemia still poses considerable problems; despite intense medical efforts and familial stress, the prognosis for the early-onset patients is disappointing. The patients with late-onset disease, however, appear to have a fairly good prognosis with the present therapeutic approach. Liver transplantation or possibly genetic therapy might improve our results in the future.

Age Factors↗
PubMed1992

Shut out flu.

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Humans↗
PubMed1991

[Management of abused children: at what cost? what for?].

The prospective follow up of abused children after their hospitalization is illustrated by the report of a sibship. Dysfunction in management and therapy is often observed and is detrimental to the children. Many levels of our society are implicated. This means that there is still a lot to do to obtain adequate therapy for these children.

Child↗
PubMed1981

Regulation of ventilation in the obstructive sleep apnea syndrome.

The recent recognition of the sleep apnea syndrome has forced a re-evaluation of the mechanism of hypercapnia and disordered respiratory control in obese patients. Thirteen obese patients with sleep apnea were studied in an attempt to relate the pattern of sleep abnormality and awake ventilatory control to the presence of chronic hypercapnia. Patients with hypercapnia and/or hypoxemia had reduced ventilatory responses to hypercapnic and hypoxic stimulation, respectively. The presence of hypercapnia, however, did not separate the patients with respect to type, duration, or frequency of apneas. The degree of awake chemical drives could not be related to the severity of the sleep apnea phenomenon. However, patients with intact ventilatory control demonstrated augmented ventilation after apneas, which may explain their eucapnic state.

Adult↗
PubMed1981

[Mothers rooming-in in a department of pediatrics (author's transl)].

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.

Adult↗
PubMed1981

[Mothers rooming-in in a department of pediatrics (author's transl)].

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.

Adult↗
PubMed1981

[Bubble babies].

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Child Development↗
PubMed1980

[The development of young children reared in an insulated sterile box: psychological problems and parental relationship].

Five children with severe immunodeficiency who received a transplantation of lymphoid cells were placed in a Trexler's isolator for several months during the time necessary to obtain an immunological reconstitution. We have observed these patients longitudinally (from 2 to 7 years), and have considered the various parameters necessary to preserve a satisfactory psychological development and a good adaptative capacity. Among these, the careful and affective attention of nurses and parental behaviour appeared to be the most important. Excellent development was obtained in three patients who had no severe organic difficulties and for whom the above parameters were positive. The cause of two unsatisfactory results seemed to be due to intricate multifactorial events, including parental deprivation.

Child↗