Allogeneic bone marrow transplantation versus chemotherapy in high-risk childhood acute lymphoblastic leukaemia in first remission

We compared the outcome of children with high-risk acute lymphoblastic leukaemia (HR-ALL) in first complete remission (first CR) treated with chemotherapy (CHEMO) or with allogeneic bone marrow transplantation (BMT) in a multicentre study.   All children treated by the Italian Paediatric Haematology Oncology Association for HR-ALL in first CR between 1986 and 1994 were eligible for the study. 30 children were given BMT at a median of 4 months from first CR, with preparative regimens including total-body irradiation ( n  =25/30). 130 matched controls for BMT patients were identified among 397 HR-ALL CHEMO patients. Matching on main prognostic factors and duration of first CR was adopted to control the selection and time-to-transplant biases. The comparative analysis was based on the results of a stratified Cox model. The estimated hazard ratios of BMT versus CHEMO at 6 months, 1 year and 2 years after CR were 1.38 (CI 0.59–3.24), 0.69 (CI 0.27–1.77) and 0.35 (CI 0.06–1{\raise 5mu ..91), with an overall non-significant difference between the two groups ( P  = 0.34). With a median follow-up of 4 years, the disease-free survival was 58.5% (SE 9.3) in the BMT group and 47.7% (SE 4.8) in the CHEMO group, at 4 years from CR. Non-leukaemic death occurred in 4% of CHEMO and 10% of BMT patients. In the BMT group the estimated cumulative incidence of relapse at 1.5 years from CR was 31.5% (SE 8.8) and did not change thereafter, whereas in the CHEMO group the corresponding figure was 29.2% (SE 4.1) and the incidence continued to increase thereafter (48.2% (SE 4.8) at 4 years from CR).   The results of this study suggest that, with respect to the CHEMO group, the higher risk of early failure in the BMT group is outweighed by the lower risk of relapse after 1 year. Results prompt the need for a prospective study, in order to demonstrate the likely advantage of BMT in HR childhood ALL in first CR.     

Comparative Sensitivity of Eight- and 24-hour Bile Acid Breath Tests and Schilling Test in Ileopathies

The conventional eight-hour bile acid breath test ("acute measurements"), was compared with a modified, extended, 24-hour breath test ("ratio measurements") and the Schilling test to assess relative sensitivity in detecting ileal dysfunction. Sixty-four patients with ileopathies were studied. The presence or absence of bile acid malabsorption was documented by fecal excretion studies of bile acid labels. The sensitivity of the "acute measurements" in the breath test was not significantly different from that of the "ratio measurements" in the ileopathies, regardless of whether or not bile acid malabsorption was present. The "acute measurements" were positive in 49 (77%), the "ratio measurements" in 54 (84%) and the Schilling test in 49 (77%) of the patients. In about 30%, bile acid breath test and Schilling test were not positive in the same patient. A combination of "acute measurements" (breath test) and Schilling test increased the percentage of cases with at least one positive test to 91%. The results of the study show: 1. The sensitivity of the bile acid breath test does not increase if ¹⁴CO₂ measurements are carried out beyond eight hours. 2. Although the breath test and Schilling test are of similar sensitivity in ileopathies, they are frequently not positive in the same patient. Therefore, it would be clinically useful to combine both tests in order to increase the likelihood of diagnosing ileal dysfunction.     

A Randomized, Double-Blind Comparison of Two Topical Anesthesic Formulations Prior to Electrodesiccation of Dermatosis Papulosa Nigra

BACKGROUND: Liposomal lidocaine 4% (L.M.X.4 cream, Ferndale Laboratories Inc., Ferndale, MI, USA) has been proposed as a more rapidly acting topical anesthetic than the eutectic mixture of lidocaine 2.5% and prilocaine 2.5% (EMLA cream, AstraZeneca LP, Wilmington, DE, USA) for venipuncture and laser procedures. However, their anesthetic efficacy has not been previously compared for electrosurgical destruction of superficial skin lesions. OBJECTIVE: To test the hypothesis that L.M.X.4 and EMLA differ in anesthetic efficacy when applied under occlusion for 30 minutes prior to electrodesiccation of papules of dermatosis papulosa nigra. METHODS: Forty adults were randomly assigned to treatment with either agent for 30 minutes under Tegaderm. The study drug was administered for an additional 30 minutes if the electrodesiccation of the first few papules was too painful. RESULTS: One subject treated with EMLA versus none treated with L.M.X.4 experienced complete anesthesia after a single 30-minute application. Nineteen of 20 (95%) subjects treated with EMLA versus 18 of 20 (90%) subjects treated with L.M.X.4 required only a single application (p = .49). Pain scores after the initial 30-minute application (scale: 0 = none to 10 = very severe) were EMLA 3.3 +/- 2.2 (mean +/- SD) versus L.M.X. 4 2.9 +/- 2.0 (p = .46). CONCLUSION: EMLA and L.M.X.4 provide comparable levels of anesthesia after a single 30-minute application under occlusion prior to electrodesiccation of superficial skin lesions.     

Incidence of Atrial Fibrillation in Patients with Different Mode of Pacing. Long-term Follow-up

We evaluated the incidence of atrial fibrillation in 189 patients (92males, 97females, mean age 75 ± 12yrs, range 41–100yrs) with pacemaker, during a mean follow-up of 5.5yrs (range 1–24yrs). The indications for implant were: complete AV block (115pts), second degree Möbilz 2 AV block (51pts). bifascicular block (5pts). sick sinus syndrome (14pts), symptomatic bradycardia (4pts). The mode of stimulation considered were VVI (105pt), VVI rate responsive (21pts), single lead VDD (43pts), DDD (20pts). The occurrence of retrograde VA conduction in patients with VVI or VVI rate responsive pacing was also evaluated. Atrial fibrillation occurred in 40 pts (21%). The highest incidence was evidenced in patients with sick sinus syndrome (9pts, 64%), and in patients with VVI stimulation (28pts, 27%). On the contrary, the lowest incidence was found in single lead VDD stimulation (4pts, 9%). The patients with dual chamber pacing showed a relatively high incidence of the arrhythmia (5pts, 25%). Atrial fibrillation occurred in 9 out of 32 patients with retrograde VA conduction, and in 22 out of 94 patients without retrograde conduction (28% versus 23%, p=NS). In conclusion, it is confirmed that patients with sick sinus syndrome are at high risk for atrial fibrillation. Single lead VDD stimulation seems to be the better mode of pacing in preventing atrial fibrillation, while dual chamber pacing showed minor efficacy. The presence of retrograde VA conduction could not predict the occurrence of the arrhythmia.     

A case of optic atrophy possibly induced by quinoline in acrodermatitis enteropathica

A 2-year-old girl with acrodermatitis enteropathica, developed optic atrophy during quinoline treatment (600 mg/day, later gradually increased to 1700 mg/day; a total dose of 280 g was given). The optic atrophy developed rapidly 9 months after the start of the quinoline treatment. The right eye was blinded and developed a convergent strabismus. Eight months after the quinoline had been stopped, the left eye began a slow and partial recovery of vision with an improvement in the appearance of the optic disc.