Parents spend an average of nine hours a day providing palliative care for children at home and need to maintain an average of five life-saving devices.

AIM: This Italian study investigated home-based palliative care for young children and how long it took parents to meet their needs. METHODS: The study population consisted of 33 families with a child under the responsibility of the Veneto Regional Center for Pediatric Palliative Care, northern Italy, who needed medical support in at least two of the following areas: respiratory, feeding, pain and seizures. RESULTS: The children had a mean age of 6.8 ± 4.7 years. We found that 72% of the patients needed medical devices for feeding, 36% had a tracheostomy and 55% were on mechanical ventilatory support. The children needed an average of five different life-supporting medical appliances, and the time taken to provide for their care increased significantly with each additional appliance (p = 0.016). Their most time-consuming daily needs were feeding (174 minutes) and support when they woke up at night (67 minutes). The average daily time that parents spent taking care of their child amounted to eight hours and 54 minutes per day. CONCLUSION: Parents providing palliative care for children with life-limiting diseases spent an average of nine hours a day caring for them each day and had to maintain an average of five medical appliances.

[Churg-Strauss syndrome presenting as polymyositis: report of a case]. / La sindrome di Churg-Strauss ad esordio polimiositico: descrizione di un caso clinico.

We reported the case of a male patient with Churg-Strauss syndrome (CSS) heralding as symptoms typical of polymiositis. During high-dose cortisone therapy (1.5 mg/kg/day), he developed a severe multiplex mononeuritis, poorly responsive to immunoglobulins and methotrexate administration. After 6 months he developed a partial deficiency of the right sciatic popliteus and the radial nerves. Sural nerve biopsy showed a characteristic necrotizing vasculitis of the epineural vessels with granulocyte and eosinophil infiltrates. In the course of CSS, peripheral nervous system involvement is frequent and can lead to disability. For this reason, it must be promptly recognized and properly treated.

[Takayasu's arteritis. A concise review and some observations on a putative case reported by Giovanni Battista Morgagni (1761)]. / L'arterite di Takayasu. Una breve sintesi della letteratura e qualche riflessione sopra un possibile caso descritto da Giovanni Battista Morgagni (1761).

The discovery of Takayasu's arteritis is likely to date back as far as 1830, owing to the first description of the Japanese Rokushu Yamamoto. Thereafter, several authors from certain geographical areas and in various historical periods described such a vascular disorder, by introducing a quantity of definitions. At present, it is defined as an eponymic disease, namely Takayasu's arteritis, since Makito Takayasu, a Japanese ophtalmologist, reported in 1908 the clinical history of a woman showing some particular retinal anastomotic shunts of arterioles and venules. In the present study the description of an about 40 year-old woman suffering from a pulseless disease, as reported by Giovanni Battista Morgagni in 1761, is summarized. Such a description could be the first case report of Takayasu's arteritis, according to some previous literature data and our critical analysis.

Hyper-IGD syndrome: a new case treated with colchicine.

We report a new case of hyper-IgD syndrome, a recently described disease characterized by recurrent episodes of fever with headache, bilateral cervical lymphadenopathy and, more rarely, abdominal pain and diarrhoea. Polyclonal increase of serum IgD is the most important laboratory finding. Etiopathogenesis and differences with familial Mediterranean fever are discussed. Moreover, good results obtained with colchicine treatment are also reported.

[Proglumetacin for the treatment of inflammatory and degenerative arthropathies]. / La proglumetacina per il trattamento delle artropatie infiammatorie e degenerative.

Twenty patients with inflammatory (15) or degenerative (5) joint disorders had been treated with 450 mg/day of proglumetacin during 35 +/- 18 days. Articular symptoms showed a definite and continued improvement, particularly evident during the initial 15 days of treatment on both painful and inflammatory components. The final physician's evaluation rated 75% of results as excellent or good, versus 15% of poor (3 patients, one of whom already refractory to diclofenac). The tolerance was defined as excellent to good in 90% of patients: one (5%) was dropped out upon the onset of sweating and palpitation, already observed with other drugs. Overall, only one case each of heartburn, anorexia and diarrhoea were considered as possibly related to the treatment. Laboratory tests did not show any variation that could be attributed to the drug. Proglumetacin therefore, by force of its efficacy and safety, appears to be particularly suited as a first-choice drug for the management of both inflammatory and degenerative joint disorders.

[Femoral and humeral head osteonecrosis in a patient with hypofibrinolysis and hyperhomocysteinemia. A case report and a review of the literature]. / Osteonecrosi delle teste femorali ed omerali in un paziente con ipofibrinolisi e iperomocisteinemia. Descrizione del caso e revisione della letteratura.

Osteonecrosis is a disease characterized by the death of marrow and bone tissues. All bones may be affected, most commonly those of the hip, knee, shoulder, ankle as well as the small bones of the hands and feet. When the disease involves a weight-bearing joint there is a significant risk that subarticular fracture may develop leading to disabling arthrosis and requiring, therefore, arthroplasty surgery. Osteonecrosis typically affects patients in their third, fourth and fifth decades of life and is associated with many factors including other diseases and co-morbidities. Multifocal osteonecrosis is defined according to the involvement of at least three separated anatomic sites. We describe the case of a young man with osteonecrosis of the shoulder and hip joints which required total arthroplasty. Among biochemical investigations, an increase in the plasminogen activator inhibitor type 1 (PAI-1) levels associated with mild hyperhomocysteinemia was present. Another finding was the HLA B27, without signs of spondyloarthropathies. In patients with osteonecrosis, especially if multifocal, a careful medical history, a complete physical examination and some biochemical investigations, particularly those related to thrombophilia and hypofibrinolysis, should be performed.

Renal artery thrombosis and hypertension in a 13 year old girl with antiphospholipid syndrome.

The case of a 13 year old girl with renal artery thrombosis and hypertension is described. A cerebrovascular accident and a probable occlusion of the superior mesenteric artery also occurred. Very high levels of 'lupus anticoagulant', anticardiolipin antibodies as well as false positive Venereal Disease Research Laboratory tests were repeatedly shown. Moreover, the patient fulfilled at least four classification criteria for systemic lupus erythematosus, but only a slight positivity for antinucleolar antibodies was present. The striking relation between antiphospholipid antibody levels and clinical events and the treatment of this complex syndrome are discussed.

[Assessment and measurement of pain in infants and children]. / Valutazione e msiurazione del dolore in età pediatrica.

The assessment of global pain in infants and children involves the analysis of subjective, behavioural and physiological components, beyond the psychological, familiar and environmental factors that influence and modulate the manifestation of painful symptom. Therefore, researchers and clinicians need valid and reliable instruments to obtain a correct measure of pain. Although subjective indices carry out a fundamental role in the assessment of pain (instruments adjusted to the age of the child), behavioural and physiological indices should be used when intelligence deficit or serious physical handicaps exclude such possibility. Behavioural aspects, associated and correlated to physiological changes, allow to obtain a reliable enough measure of pain. In this paper, the main instruments for measuring pain in infants and children will be described, and their advantages and disadvantages underlined.

[Stress hormones liberated by fangotherapy. ACTH and beta-endorphin levels under heat stress]. / Stresshormone, freigesetzt durch Fangotherapie. ACTH- und Beta-Endorphin-Konzentrationen unter Wärmestress.

In 6 healthy subjects submitted to fango therapy in the Euganean thermal baths (Italy), the plasma concentrations of beta-endorphin and ACTH increased transitorily but significantly. These results correlate with the release of these peptides by the pituitary in response to thermal stressing. The analgesic and hypothermic action responsible for good toleration of thermal stress induced by fango therapy, can be explained by this increase in plasma beta-endorphin. The repeated brief increases in plasma beta-endorphin during thermal treatment result in progressive improvement in articular and muscular symptomatology. The results of our study on plasma levels of ACTH confirm that the thermal stress associated with fango therapy activates the pituitary gland. Immunomodulatory effects are discussed.

Thrombosis of the subclavian vein in SAPHO syndrome. A case-report.

The hallmark of SAPHO Syndrome (Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis) is osteitis of the anterior chest wall, which can be so pronounced as to cause thoracic outlet syndrome, thrombosis of the subclavian vein, and compression of the superior vena cava. Suggestive skin manifestations, namely palmoplantar pustulosis and severe acne, generally antedate the bone and joint lesions. We report a case of SAPHO syndrome that went unrecognized for many years and eventually caused compression and thrombosis of the right subclavian vein requiring cleidectomy. SAPHO syndrome should be considered in every patients with anterior chest wall inflammation, particularly when skin lesions are also present.