Presse Med
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MAIN CLINICAL FEATURES: Xeroderma pigmentosum is a rare, recessive disorder clinically characterized by extreme photosensitivity, pigmented abnormalities of the skin-exposed areas, and frequent ocular and neurological abnormalities. Xeroderma pigmentosum syndrome is associated with an estimated 2000-fold increase in the risk to develop skin cancer (basal cell carcinoma, squamous cell carcinoma and melanoma).
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A HISTOPATHOLOGICAL DIAGNOSIS: Histiocytic cytophagic panniculitis is the specific skin finding in the reactive hemophagocytic syndrome. It is a feverish nodular eruption which accompanies the other clinical and biological features of the reactive hemophagocytic syndrome. Histopathologic changes are diagnostic and consist in a lobular panniculitis characterized by the presence of a histiocytic infiltration of the fat. Histiocytes are benign in appearance and show variable degree of phagocytosis.
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ANTIREFLUX SURGERY: Peri and postoperative morbidity is higher with Nissen fundoplication than with a 180 degrees posterior fundoplasty (Toupet procedure) which carries the risk of a compromised result with time. Although a 270 degrees posterior gastric valve may be the best technique, the surgeon's skill looks more important than the procedure used, especially for laparoscopic surgery. Its advantages include reduced postoperative pain, shorter hospital stay and convalescent time.
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FUNDAMENTAL: Analgesia is a fundamental part of management as it helps avoid the morbid effects of pain itself and improves confidence so the child and his parents can accept more easily the diagnosis and proposed treatment. The World Health Organization has established a classification of analgesics. USE OF PLACEBOS: The placebo effect depends on several factors including anxiety, confidence, and the patient's- and prescriber's-expectations and convictions). ⋯ Local anesthetics improve comfort without compromising safety. Neurogenic pain does not respond to usual analgesics and can be relieved with tricyclic antidepressors for burning sensations or antiepileptic drugs for fulgurant pain. TREATMENT-RELATED PAIN: Iatrogenic pain, by definition, must be systematically anticipated and prevented.
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A NEURODEGENERATIVE DISEASE: Idiopathic Parkinson's disease is initially due to a selective and specific destruction of the pars compacta dopaminergic neurons in substantia nigra. Since the inaugural description in 1817 by J. Parkinson, therapeutic progress rapidly accelerated mainly due to a better knowledge of the neurobiology of the central nervous system dopaminergic pathways.