Aprile 2008 - Volume XXVII - numero 4

Medico e Bambino


Pagine elettroniche ; Caso Contributivo

È giustificato un diverso approccio terapeutico nel trattamento dell’atrofia muscolare spinale di tipo 1? Insegnamenti da un caso clinico

C. Geraci1, G. Paloni1, A. Saccari1, L. Badina1, E. Barbi1, E. Catarruzzi2, R. Sartori3, G. Ottonello4

1Clinica Pediatrica, IRCCS “Burlo Garofolo”, Trieste
2UO di Radiologia,IRCCS “Burlo Garofolo”, Trieste
3UO di Fisioterapia, IRCCS “Burlo Garofolo”, Trieste;
4UO di Anestesia e Rianimazione, Istituto “G. Gaslini”, Genova

Indirizzo per corrispondenza: lau.bad@gmail.com

COULD A DIFFERENT APPROACH TO RESPIRATORY COMPLICATIONS IN TYPE 1 SPINAL MUSCULAR ATROPHY BE JUSTIFIED?

Key words: Spinal muscular atrophy, Atelectasis, Boussignac CPAP valve, Mechanical in-exsufflator, Non-invasive ventilation

Children affected by spinal muscular atrophy (SMA) can develop serious respiratory complications including atelectasis, pneumonia, small airway obstruction and acidosis, principally because of impaired cough secondary to weakness of chest wall. Ineffective airway clearance can be exacerbated by acute respiratory illnesses, when secretion production increases and respiratory muscle function acutely deteriorates. In the past the only chance to prolong survival for children with SMA intubated for acute respiratory failure was often tracheotomy and long-term mechanical ventilation. Most physicians discourage endotracheal intubation and tracheotomy, assuming that the prognosis for survival would not be greatly improved and the quality of life is too poor to justify such an invasive intervention. Recent reports highlight that tracheotomy for most SMA 1 children could be delayed by the praecox use of non-invasive ventilation (NIV) and mechanical in-exsufflator (MI-E), thus increasing their quality of life. We report a case of a SMA 1 infant presenting migrating atelectasis who was successfully treated by using Boussignac CPAP valve, MI-E and NIV.

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C. Geraci, G. Paloni, A. Saccari, L. Badina, E. Barbi, E. Catarruzzi, R. Sartori, G. Ottonello È giustificato un diverso approccio terapeutico nel trattamento dell’atrofia muscolare spinale di tipo 1? Insegnamenti da un caso clinico. Medico e Bambino 2008;27(4):260 https://www.medicoebambino.com/?id=0804_260.pdf

Pagine elettroniche ; Caso Contributivo

Malattia di Ménétrier in un bambino di 3 anni

M. Lorusso, S. Bevilacqua, E. Pozzi, F. Mangiantini, A. Gissi, P. Lionetti

Dipartimento di Pediatria, Ospedale “Meyer”, Firenze

Indirizzo per corrispondenza: paolo.lionetti@unifi.it

A 3-YEAR-OLD CHILD WITH MÉNÉTRIER’S DISEASE

Key words: Ménétrier’s disease, Enteropathy, Hypoprotidemia, Child

We report a case of a 3-year-old boy, who presented vomiting and edema for one week. The latter was first localized in the periorbital region and then generalized. Laboratory studies revealed severe hypoproteinemia (total protein: 2.9 g/dl) and CMV infection. Endoscopy showed erythematosus gastric mucosa of body and fundus and biopsies revealed foveolar hyperplasia and morphological evidence of CMV infection. The child was treated with intravenous albumin transfusions that led to complete clinical resolution. Protein- losing enteropathy, hypertrophic gastropathy and CMV infection are typical of Ménétrier’s disease. These childhood forms, in contrast to classic adult Ménétrier’s disease, have a typical benign and transient course, and require only supportive therapy

Vuoi citare questo contributo?

M. Lorusso, S. Bevilacqua, E. Pozzi, F. Mangiantini, A. Gissi, P. Lionetti Malattia di Ménétrier in un bambino di 3 anni. Medico e Bambino 2008;27(4):260 https://www.medicoebambino.com/?id=0804_260.pdf


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