Mycophenolate mofetil in children with steroid/cyclophosphamide-resistant nephrotic syndrome

The purpose of this study was to assess the results of therapy with mycophenolate mofetil (MMF) in children with idiopathic nephrotic syndrome (INS) who were both steroid- and cyclophosphamide-resistant. Treatment lasted a minimum of 6 months, and follow-up data were collected over a 2-year period. The children were divided into two groups: Group 1 (n?=?34) comprised patients who had received cyclosporine A (CsA) before the initiation of MMF therapy; Group 2 (n?=?18) comprised patients who received only MMF. Among the 34 patients of Group 1, complete and partial remission were achieved in seven (20.6%) and 13 patients (38.6%), respectively; there was no response in 14 patients (41.2%). Among the 18 patients in Group 2, complete and partial remission occurred in five (27.8%) and six (33.3%) patients, respectively; there was no response in seven patients (38.9%). Eight patients developed chronic kidney disease. The main side-effects were gastrointestinal complaints (n?=?11, 21%), recurring severe infections (n?=?1, 1.9%), and mild thrombocytopenia/leucopenia (n?=?1, 1.9%). MMF proved to be therapeutically effective in 59.5% of the cases. These beneficial effects need to be confirmed in studies with a long-term follow-up after discontinuation of the treatment. Our statistical analysis of the results of therapy with MMF did not reveal any significant difference between its use alone or following CsA administration.     

Spontaneously reduced isolated orbital roof fracture

We report a case of a spontaneously reduced isolated orbital roof blow-in fracture with resolution of associated diplopia and blepharoptosis highlighting the need for a low threshold for reimaging this cohort of facial fracture patients.     

Treatment of infantile haemangioma with captopril

Background Infantile haemangioma (IH) has recently been reported as an aberrant proliferation and differentiation of a primitive mesoderm‐derived haemogenic endothelium regulated by the renin‐angiotensin system (RAS), leading us to propose angiotensin converting enzyme (ACE) as a potential therapeutic target. Objectives To present initial results of our open‐labelled observational clinical trial using captopril, an ACE inhibitor (ACEi), in the treatment of problematic proliferating IH. Methods After initial screening investigations, infants with problematic IH were admitted for initiation of captopril with a 0·1 mg kg^?1 test dose orally, followed by 0·15 8‐hourly over 24 h. This was then followed by dose escalation to 0·3 mg kg^?1 8‐hourly for another 24 hours. The dosage was increased to 0·5 mg kg^?1 8‐hourly 1 week later, if a noticeable involution had not already occurred. The response of IH to captopril was documented clinically and photographically before and after treatment and any side‐effect was recorded. Results Two boys and six girls aged 5–22 weeks (mean 12·9) with problematic IH were recruited with the lesions located in nasal tip (n = 1), cervicofacial (n = 3), periorbital (n = 1) and perineal (n = 2) areas, and shoulder (n = 1). Transient mild renal impairment occurred in one subject but resolved spontaneously. No other complication was observed. The IHs in all patients responded to captopril at a dosage of 1·5 mg kg^?1 daily which led to a dramatic response in three, moderate response in two, and slow response in three patients. Continued involution of IHs was observed during the follow‐up period of 8–19 months (mean 15·8) in all subjects. Treatment was ceased at 14 months of age in seven patients with no rebound growth. In the remaining patient, rapid healing occurred with ongoing gradual reduction in the size and colour of a large ulcerated retroauricular lesion following 5·5 months of treatment. The lesion was excised to address its persistent distortion of the ear. Conclusions The response of IH to an ACEi supports a critical role for the RAS in IH and represents a paradigm shift in the understanding and treatment of this enigmatic condition.