Preliminary evaluation of vitiligo using in vivo reflectance confocal microscopy

BACKGROUND: Vitiligo is the most common pigmentary disorder with a global incidence from 0.1% to 2% in different geographical areas. Histopathology and histochemistry have shown the reduction of melanocytes in achromic patches, but microscopic changes of lesional and non-lesional skin are still not completely understood. Reflectance confocal microscopy (RCM), based on the different light reflectance index of cutaneous structures, allowed in vivo, en face microscopic evaluation of superficial skin layers with a resolution similar to skin histology. AIM: The purpose of this study was to evaluate RCM features of lesional and non-lesional skin of vitiligo patients. Moreover, re-pigmented areas were taken into consideration in order to evaluate melanocyte response to ultraviolet B (UVB) radiation. SUBJECTS AND METHODS: Sixteen patients of different phototypes affected by active non-segmental vitiligo and 10 controls were enrolled in the study. In vivo skin imaging was done using a commercially available RCM (Lucid, Vivascope 1500. Re-pigmented areas from 6 to 16 patients (after UVB narrow-band therapy) were also examined. RESULTS: Vitiligo lesions showed the disappearance of the bright rings normally seen at the dermo-epidermal junction. Moreover, non-lesional skin of vitiligo patients showed unexpected changes as the presence of half-rings or scalloped border-like features of the bright papillary rings. In re-pigmented areas after UVB narrow band therapy, the presence of activated, dendritic melanocytes was seen. CONCLUSIONS: Considering our results, and following further studies, RCM clinical applications could be used in the therapeutic monitoring and evaluation of the evolution of vitiligo.     

Familial hemiplegic migraine and autosomal dominant arteriopathy with leukoencephalopathy (CADASIL)

Cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy (CADASIL) is a recently described familial cerebrovascular disorder shown to map to chromosome 19q12. Familial hemiplegic migraine has also been shown in some families to map close to the CADASIL locus. The fully developed CADASIL phenotype consists of recurrent strokes developing in the fourth decade, progressing to a pseudobulbar palsy, spastic quadriparesis, and subcortical dementia. In an Irish family 15 members were fully investigated by magnetic resonance scanning; 10 had typical magnetic resonance features of CADASIL. Five members of this family had familial hemiplegic migraine and 4 of these had magnetic resonance evidence of CADASIL. Two other members had migraine with and without aura as a presenting clinical symptom of CADASIL. This disorder has been shown by linkage analysis to map to the CADASIL locus at chromosome 19. The phenotype at presentation of CADASIL in this family was variable and age related and included familial hemiplegic migraine, migraine with and without aura, transient ischemic attacks, strokes, and spinal cord infarction. This family study increases our understanding of the spectrum of clinical manifestations of this underrecognized familial cerebrovascular disorder.     

Palliative treatment of advanced colorectal carcinoma with the YAG laser.

The Nd-YAG laser was used to provide palliative treatment for eight patients (two women and six men), ranging in age from 53 to 76 years, who had locally advanced colorectal cancer. The main indications for treatment were obstruction, bleeding and copious rectal mucus discharge. Only one complication, a Staphylococcus aureus infection, followed the treatment; the infection was easily controlled. The laser treatment was considered to ease the symptoms in five patients, but survival was not influenced by the treatment. This form of palliation must be evaluated in larger numbers of patients to assess its real value in advanced colorectal cancer.     

Decreased expression of DMPK: correlation with CTG repeat expansion and fibre type composition in myotonic dystrophy type 1

Abstract Myotonic dystrophy type 1 (DM1) is an autosomal dominant disease caused by a trinucleotide repeatexpansion, cytosine-thymine-guanine (CTG)_n, in the 3′ untranslated region of a gene encoding the myotonic dystrophy protein kinase (DMPK). To correlate CTG expansion and protein expression, we studied muscle specimens from 16 adult DM1 patients using three anti-DMPK antibodies for immunoblotting. We estimated the amount of the full-length DMPK (85 kDa) in muscle biopsies from normal controls and from DM1 patients carrying different (CTG)_n expansions. We found that DMPK concentration was decreased to about 50% in DM patients’ muscles; the protein decrease did not seem correlated with the CTG repeat length. However, the fibre type composition in skeletal muscle seemed somehow to affect DMPK decrease, as the lowest level of the enzyme was found in patients with the lowest content of type 1 fibre.     

The use of endoprostheses (stents) in airway stenosis]

Six patients with obstruction of the trachea were treated with silicone rubber endothracheal stents implanted with flexible bronchoscope. In every case the stent caused significant clinical improvement of the ventilation. At the postintubation stenosis the stent can result a final recovery, at the malignant processes the implantation seems to be a new palliative method.     

Surgical treatment of cavernous haemangiomas of the liver

Cavernous haemangiomas of the liver were surgically treated in 36 women and 14 men over a 10-year period. The tumours were solitary in 42 cases and multiple in 8. Locations were the right lobe in 39 cases, the left one in 5 and both lobes in 6. The size of the tumours ranged from 0.4 to 14 cm in diameter. Enucleation of tumours was carried out in 29 cases, an atypical liver resection in 19 cases, anatomical lobectomy in 2 cases and a right trisegmentectomy in 1 case. There was no mortality. Morbidity rate seems to be lower in patients who underwent enucleation. The authors indicate the importance of enucleation for removal of liver haemangiomas of various sizes.

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Chirurgische Behandlung kavernöser Hämangiome der Leber

Zusammenfassung Über einen Zeitraum von 10 Jahren wurden kavernöse Hämangiome der Leber bei 36 Frauen und 14 Männern chirurgisch behandelt. Bei 42 Patienten waren die Tumoren solitär, bei 8 multipel. Sie waren in 39 Fällen im rechten, bei 5 Patienten im linken und bei 6 in beiden Leberlappen lokalisiert, ihr Durchmesser betrug 0,4 bis 14 cm. In 29 Fällen wurde eine Enukleation, in 19 eine atypische Leberresektion, in 2 eine anatomische Lobektomie und in einem Falle eine rechtsseitige Trisegmentektomie durchgeführt. Die Mortalität war gleich Null; die Morbiditätsrate scheint bei den Patienten geringer, die mit Enukleation behandelt wurden. Der Beitrag hebt die Bedeutung der Enukleation zur Beseitigung von Leberhämangiomen unterschiedlicher Größe hervor.

     

A comparison of the stressors experienced by parents of intubated and non-intubated children

When children are ill enough to require admission to paediatric intensive care, parents may become distressed about their child's medical condition and this distress may be compounded by the unfamiliar nature of the highly technological environment Parents of children who are sick enough to warrant intubation are particularly likely to be exposed to a frightening array of technological equipment Seventy-one parents of intubated and non-intubated children completed the Parental Stressor Scale Paediatnc Intensive Care Unit (PSS PICU) Overall the findings suggest that parents were most distressed (a) by the painful procedures to which their children were subjected, (b) by the sights and sounds of the intensive care unit and (c) by their children's reactions to intensive care The behaviour of staff towards parents and the way that staff communicated with them caused the least distress When the levels of stress reported by parents of intubated children were compared with those reported by parents of non-intubated children, different patterns of stress were found Painful procedures were a source of greater stress to parents of intubated children whereas the behaviour of staff and the children's reactions to the intensive care experience caused greater stress to the parents of the non-intubated children In general the findings suggest that the needs of parents of non-intubated children are being overlooked, with staff focusing more of their attention on the parents of intubated children