Immune reconstitution after haematopoietic transplantation with two different doses of pre-graft antithymocyte globulin.

Antithymocyte globulin is widely used before haematopoietic transplantation with HLA-matched unrelated donors or mismatched relatives to prevent rejection and graft-versus-host disease (GVHD). However, optimal dosage is still under debate. Thirty-one consecutive children, mainly with haematological malignancies, were transplanted in a single institution with such donors, selected by HLA-A -B compatibility by serology and DRB1* by DNA typing. Antithymocyte globulin (Thymoglobuline; Sangstat) was infused at days -3, -2, -1. Total dosage varied: 16 patients received a median of 7.5 mg/kg (2.5 to 10.5: low-dose group), and 15 a median of 15.5 mg/kg (14.4 to 19.4: high-dose group). Post-transplant GVHD prophylaxis consisted of cyclosporine, short-course methotrexate and steroids. CD3(+), CD4(+) and CD19(+) cell reconstitution was slower in the high-dose group. Median time to reach 100 CD4(+) cells was 8 months vs 4 months (P = 0.03). Median time to normal CD19(+) cells was 16 months vs 8 months (P = 0.01). CD16(+)CD56(+) and CD8(+) cell reconstitution was similar. Nine patients in the high-dose group and two in the low-dose group experienced life-threatening opportunistic infections (P = 0.009). Although obtained from a limited number of patients, our data suggest that a higher pre-graft dose of antithymocyte globulin may negatively influence immune reconstitution.

Ethanol sclerotherapy of peripheral venous malformations.

BACKGROUND: Venous malformations are congenital lesions that can cause pain, decreased range of movement, compression on adjacent structures, bleeding, consumptive coagulopathy and cosmetic deformity. Sclerotherapy alone or combined with surgical excision is the accepted treatment in symptomatic malformations after failed treatment attempts with tailored compression garments. OBJECTIVES: To report our experience with percutaneous sclerotherapy of peripheral venous malformations with ethanol 96%. PATIENTS AND METHODS: 41 sclerotherapy sessions were performed on 21 patients, aged 4-46 years, 15 females and 6 males. Fourteen patients were treated for painful extremity lesions, while five others with face and neck lesions and two with giant chest malformations had treatment for esthetic reasons. All patients had a pre-procedure magnetic resonance imaging (MRI) study. In all patients, 96% ethanol was used as the sclerosant by direct injection using general anesthesia. A minimum of 1-year clinical follow-up was performed. Follow-up imaging studies were performed if clinically indicated. RESULTS: 17 patients showed complete or partial symptomatic improvement after one to nine therapeutic sessions. Four patients with lower extremity lesions continue to suffer from pain and they are considered as a treatment failure. Complications were encountered in five patients, including acute pulmonary hypertension with cardiovascular collapse, pulmonary embolus, skin ulcers (two) and skin blisters. All patients fully recovered. CONCLUSION: Sclerotherapy with 96% ethanol for venous malformations was found to be effective for symptomatic improvement, but serious complications can occur.

[Langerhans-cell histiocytosis in an adult with initial vulvar involvement: 2 cases]. / Histiocytose langerhansienne de l'adulte avec atteinte vulvaire initiale: 2 cas.

Langerhans' histiocytosis is a proliferation of atypical Langerhans cells which may affect several different organs. Two women had lesions of the vulva which led to the diagnosis. Vulvular involvement is the most frequent in gynaecological localization of Langerhans' histiocytosis. Pathological and immunohistochemical tests confirm the diagnosis.

[Persistent photosensitivity: treatment with puvatherapy and prednisolone (corticopuvatherapy)]. / Photosensibilité rémanente: traitement par puvathérapie et prednisolone (corticopuvathérapie).

Eight patients, six men and two women, had chronic photosensitivity of 2.0 +/- 1.1 years' duration. The clinical and photobiological signs were consistent with a diagnosis of chronic actinic dermatitis syndrome. Photosensitivity was extremely severe, and the minimal erythematous dose (MED) in polychromatic light was dramatically decreased in all eight patients (82 +/- 20 mJ/cm2; range: 25-200 for a normal MED range of 1.000 to 2.000 mJ/cm2). Photopatchtests were positive to phenothiazine in four patients, to fragrance mix and oxybenzone in two patients, to balsam of Peru and musk ambrette in one patient each. Seven patients were treated with corticopuvatherapy. They all were markedly improved after one month of treatment, recovering normal MED and outdoor activities. Corticosteroid therapy was gradually reduced and stopped after 3 months, while puvatherapy was continued for several months. Treatment was withdrawn in three patients. The minimal erythematous dose was monitored after discontinuation of therapy: a progressive decrease of MED was observed, accompanied by a relapse in two of the three patients. This relapse was well controlled by another course of corticopuvatherapy. Four other patients had, after discontinuation of treatment, another course of corticopuvatherapy reinstituted in the early spring. Puvatherapy was stopped at the end of october. One patient did not receive corticopuvatherapy because she was living far from a puvatherapy centre. She was treated with azathioprine for 6 months without improvement, then with azathioprine+prednisolone 20 mg daily during 2 years with slight improvement. Corticopuvatherapy is a very efficient means of treating severe chronic actinic dermatitis.(ABSTRACT TRUNCATED AT 250 WORDS)

[Photobiological study of lupus erythematosus]. / Exploration photobiologique des lupus érythémateux.

BACKGROUND: Although cutaneous photosensitivity is one of the major criteria used for the diagnosis of systemic lupus erythematosus, this criterion is not precise. OBJECTIVE: To evaluate the interest of photobiological exploration in patients with cutaneous lupus erythematosus, and to compare the results to clinical photosensitivity. METHODS: Nineteen patients consecutively seen in one year, have been tested using a standardized method. RESULTS: Clinical photosensitivity was present in 16 cases. The clinical presentation of cutaneous disease was classified in 3 groups: acute, subacute, and chronic. One patient with no history of photosensitivity had positive photobiological tests, and five patients had negative tests though they experienced clinical photosensitivity. Positive phototests with UVA and UVB were present in 6/8 subacute lupus cases, 3/6 acute lupus cases, 3/5 chronic lupus cases. Histological aspect of cutaneous biopsies from phototest was not characteristic of lupus. Minimal erythematous doses was comparable in lupus group and in controls. No correlation between positivity of phototests and the presence of systemic involvement or anti-Ro/SSA antibodies was established. CONCLUSION: Phototesting is useful to assess photosensitivity in lupus patients.

[Ophthalmologists without borders in Northern Cameroon]. / Ophtalmo sans Frontières au nord Cameroun.

Ophthalmologist without Borders (OWB) is a non-governmental organization founded in 1987 that operates mainly through medical and surgical care centers located in northern Cameroon. The goal of OWB is advancement of the fight against blindness and low vision in the third world. To achieve this goal OWB has four activities. In less than 10 years OWB has dispensed more than 90,000 consultations not only to patients with cataracts, the first cause of curable blindness, but also to numerous patients with conjunctivitis. Surgical therapy is another major activity of the OWB which has performed over 15,000 procedures with cataracts accounting for 85% of indications. Results have been encouraging but have demonstrated the need for dauntless surgeons and the importance of postoperative follow-up. Prevention and screening are priorities that are being progressively implemented. Training is an essential part of the mission of OWB with the ultimate aim being to leave local physicians and nurses with adequate facilities.

[Rational approach for the treatment of postoperative endophthalmitis in impoverished populations]. / Approche rationnelle pour le traitement des endophtalmies post-chirurgicales des populations vivant dans des conditions de pauvreté

Due to the need for treatment guidelines for endophthalmitis in impoverished areas, we have formulated an approach which takes into account pharmacokinetic data, keeping in mind that, whether oral or intramuscular, antibiotics must achieve therapeutic intraocular levels, antibiotic susceptibility of the most common pathogens in endophthalmitis, and routine availability of bioequivalent generics in the areas in question. In this work, we present the basic guidelines for the management of postoperative endophthalmitis by ophthalmology services in impoverished areas.

Mass treatment of trachoma with azithromycin 1.5% eye drops in the Republic of Cameroon: feasibility, tolerance and effectiveness.

AIMS: An epidemiological study carried out in 2006 indicated a high prevalence of blinding trachoma in the Kolofata Health District, Far North Region, Republic of Cameroon. As a result, the national blindness control programme of Cameroon instituted a trachoma elimination programme using the SAFE strategy. METHODS: A campaign to treat the entire district population with azithromycin 1.5% eye drops was undertaken in February 2008. To measure the effectiveness of treatment on the prevalence of active trachoma, two epidemiological studies were conducted on a representative sample of children aged between 1 and 10 years. The first study was performed just prior to the treatment campaign and the second study was performed 1 year later. RESULTS: The prevalence of active forms of trachoma (trachomatous inflammation--follicular (TF) + TF/trachomatous inflammation--intense (TI)) dropped from 31.5 (95% CI 26.4 to 37.5)% before treatment to 6.3 (95% CI 4.1 to 9.6)% 1 year after treatment-a reduction of nearly 80%. There were no reports of serious or systemic side effects. Tolerance was excellent and no treatment was interrupted. CONCLUSION: Mass treatment with azithromycin 1.5% eye drops is feasible, well tolerated and effective.

Large renal angiomyolipomas: digital subtraction angiographic grading and presentation with bleeding.

AIM: To investigate whether a grading system of renal AMLs based on digital subtraction angiography (DSA) and computerized tomography (CT), could help to select patients for embolization. MATERIALS AND METHODS: Thirty patients with 35 renal angiomyolipomas (size range 4-20 cm, mean 9.9 cm) underwent both digital subtraction angiography (DSA) and computed tomography (CT). Based on the DSA appearance the tumours were graded into three grades: grade I, minimal vascularity; grade II, moderate vascularity; grade III, marked vascularity. RESULTS: There were seven grade I (mean 9.3 cm, range 4.5-20 cm), 18 grade II (mean 8.9 cm, range 5-18 cm) and 10 grade III tumours (mean 12.1 cm, range 4-20 cm). Five grade I tumours did not cause symptoms (71%) and two caused flank pain and haematuria, respectively (14.3% each). Nine of the grade II tumours were asymptomatic (50%), seven caused bleeding (39%) and two caused flank pain (11%). Four grade III tumours were asymptomatic (40%), five caused bleeding (50%) and one pain (10%). CONCLUSIONS: According to our criteria, large angiomyolipomas with minimal vascularity are less likely to bleed, and do not need prophylactic treatment. This needs to be confirmed in larger studies.

Contact and photocontact allergy to oxybenzone.

The purpose of this study was to determine the frequency of contact allergy and photocontact allergy to sunscreens. A consecutive series of 54 patients with suspected clinical photosensitivity were assessed. All had the same standardized photobiological investigation from January 1989 to December 1990, including patch tests and photopatch tests with 6 sunscreen agents. Oxybenzone was found to cause 4 cases of allergic contact dermatitis (with photoaggravation in 2), and 3 cases of photocontact dermatitis (13% of patients). This is probably due to the wide distribution of oxybenzone in sunscreens and other cosmetics, 2 patients with polymorphic light eruption and persistent light reactivity, respectively, were regular sunscreen users. Photobiological investigation is necessary to ensure accurate diagnosis, since sunscreen contact or photocontact allergy may simulate other photosensitivity eruptions.

[Trial of initial non surgical treatment of subdural empyema]. / Tentative de traitement non chirurgical d'emblée d'un empyème sous-dural.

The authors report on an 8-year-old girl who experienced bilateral subdural frontoparietal and interhemispheric empyema following sinusitis. The child improved after initial treatment with a 3 weeks course of parenteral antibiotics. Surgical drainage was further required because of clinical aggravation; however, this evolution was related to bilateral frontoparietal brain edema and abscesses fluid was sterile.

[A severe form of vitamin D deficiency with hypocalcemic cardiomyopathy]. / Une forme sévère de carence en vitamine D avec cardiomyopathie hypocalcémique.

The authors report on a case of cardiomyopathy with congestive heart failure in an infant with severe hypocalcemia related to vitamin D deficient rickets. The heart failure was successfully treated with calcium gluconate and vitamin D, associated with dobutamide.