Infantile myofibromatosis (IM) is a rare mesenchymal disorder, typically observed during infancy and characterized by the development of myofibroblastic tumors within skin, muscle, bone or viscera. In most cases, spontaneous regression of the lesions occurs before the age of four, however therapeutic tools such as surgery and chemotherapy sometimes need to be implemented. Metastatic recurrence of this condition is very rare. We report the case of a newborn infant with multicentric IM involving the skin, intestinal tract and bone, who required long-term symptomatic treatment. Spontaneous regression was noticed at the age of four but twenty years later she presented with a complete spontaneous right pneumothorax revealing cystic pulmonary metastases of IM. There have been very few reports of metastatic recurrence of IM in adulthood and this unique presentation underlines the need for long-term follow-up of these patients to detect and prevent possible complications.
BACKGROUND: HIT-SKK protocol is used for the treatment of low risk medulloblastomas in young children with the aim of eliminating cranial irradiation and its long-term side effects, in particular neuropsychological (NP) sequelae. This therapy includes IV and intraventricular (ITV) methotrexate (MTX) potentially responsible for leukoencephalopathy (LE) and neurocognitive disorders.The objectives are to describe the risk factors and the course of LE, and to investigate its impact on long-term neurocognitive and behavioural outcome. METHODS: A French retrospective, multicenter study including 35 children under 5 years of age, treated between 2009 and 2017, with a median follow up of 72 months. All follow-up MRIs including assessment of the severity of the LE (Fazekas and CTCAE grading) and all NP evaluations were centrally rewieved. RESULTS: 25/34 evaluable patients presented a LE during follow up, in a median delay of 2 months (1 - 17 months) after the start of chemotherapy. Grade 2 and 3 abnormalities were correlated with higher cumulative dose of ITV -MTX (p=0,01). Full Scale IQ (FSIQ) and Wechsler indexes were in the average or low average of the reference population. FSIQ was deficient in 7/20 evaluable patients. Processing speed (PSI) was the most frequently impaired neurocognitive domain: 9/20 patients with borderline or very low score, all having received a significantly higher cumulative dose of ITV-MTX (p=0,04). A decrease in overall NP scores was observed in patients for whom grade 2 or 3 LE persisted at the end of follow-up with an average FSIQ estimated at 82.1 (SD 16.9) versus 94.2 (SD 20.6). This decrease was significant for PSI (p=0,049). LE and neurocognitive impairments were not correlated with a younger age at diagnosis. CONCLUSION: This study confirmed the responsibility of MTX, and in particular ITV-MTX therapy in the onset and, most often, persistence of LE and its association with neurocognitive disorders.
In our experience many doctors and medical students believe that dextrose is a carbohydrate that is different from glucose and do not realise that dextrose is the name given in the British Pharmacopoeia (1973) and the British Pharmaceutical Codex (1973) for glucose monohydrate. This causes confusion in understanding disease and in treatment. Glucose is the scientifically acceptable trivial name for D(+)-glucose. We make a plea for the universal use in medicine of "glucose" as the only name for this compound and of "glucose monohydrate" when it is necessary to specify that the glucose molecule is not anhydrous (as it carries one molecule of water)-and for the abandonment by pharmacists of the name "dextrose." D N BARON
The Aboriginal people in Robinvale do not want separate schools, classes and housing. Some of the more dependent ones need extra help, but the capable ones are proud of succeeding on their own merits as people.However, the children do have problems at school. For this reason the Save the Children Fund has set up a kindergarten for three-year-old children. All the Aboriginal children attend four days a week; even itinerant families and visitors bring their three-year-olds along. They may stay with the children, leave them, come back early, join in the program, or just watch, any day and every day. Two Aboriginal aides take a major part in running the program, with a kindergartner.Non-Aboriginal children are welcome. They attend one or two days a week, and their families are also welcome. Visiting parents are introduced to one another, and encouraged to act as guests to whom all the children play host.As the Aboriginal children attend more often, they get to know the program routines, the songs, stories, puzzles, games and equipment better. They are the confident ones, the helpers, the messengers.
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