Congenital Narrowing of a Pulmonary Vein: Slit‐like Pulmonary Vein Ostium

We report the case of a 60‐year‐old female with a history of refractory paroxysmal atrial fibrillation. Preablation contrast enhanced pulmonary vein computed tomography (CT) scan demonstrated a slit‐like narrowing of the left inferior pulmonary vein ostium. The narrowing measured approximately 3 mm, with poststenotic dilation. The patient had no prior history of ablation. The patient subsequently underwent segmental antral isolation of all four pulmonary veins and cavo‐tricuspid isthmus ablation with bidirectional block. The diagnosis of preexisting congenital pulmonary vein stenosis had an impact on the type of ablation procedure performed (antral rather than ostial) and will affect the interpretation of postablation CT scans. (PACE 2013; 36:e150–e152)     

EFFECTS OF SOFTFNFD AND UNSOFTFNED FABRICS ON INFANT SKIN

Background. The effects of softened and unsoftened fabrics on the skin of infants of 1–12 months of age were evaluated under real life conditions of skin contact with fabrics. Methods. During 4 weeks, 24 infants wore cotton fabrics washed with a granular detergent on one side of their lower back, and on the other side, cotton fabrics washed with the same detergent and softened with a liquid fabric softener. Skin effects were evaluated by visual grading for redness, dryness, and smoothness, by skin stripping and measuring of Chroma C* (squamometry), by measurements of elasticity and bioelastic ratio, and by instrumental measurements of skin parameters (pH, capacitance, transepidermal water loss (TEWL), and erythema by colorimetry). Results. No deleterious effects were observed in any infant. A decrease in squamometry (Chroma C*) and an increase in capacitance were detected in skin exposed to softened fabrics relative to unsoftened ones. Values of pH tended to be higher in the sites treated with softened versus unsoftened fabrics (pH 6.06 and 5.87, respectively, at end of study). All other parameters showed no significant differences in the two treatment groups. Conclusions. Neither softened nor unsoftened fabrics produced any adverse effects on the skin of infants after continuous wearing during 29 days. A slight beneficial effect on the infants' skin was observed with softened relative to unsoftened fabrics. Methods measuring the structure and function of the stratum corneum were more sensitive discriminators of the effects of fabrics on the skin than traditional methods of visual clinical evaluation.     

Langerhans cell histiocytosis manifesting as recurrent simultaneous bilateral spontaneous pneumothorax in early infancy

Langerhans cell histiocytosis (LCH) is a rare disorder characterized by infiltration of either single or multiple organs by a distinct cell type that is S-100 and CD1a positive and contains ultrastructural Birbeck granules on electron microscopy. Historically, LCH included four main clinical forms: Letter-Siwe disease, Hand-Schuller-Christian disease, eosinophilic granuloma (together grouped as histiocytosis) and Hashimoto-Pritzker disease. The writing group of the Histiocytotic Society in 1987 proposed the uniform term of 'Langerhans cell histiocytosis' to encompass all the aforementioned eponymous forms. Lung involvement occurs in up to half of all children with multisystem disease and usually parallels overall disease activity. Spontaneous pneumothorax (SP) occurs in approximately 10% of children with pulmonary disease and may be a fatal complication. Patients with pulmonary LCH are likely predisposed to the development of pneumothorax based on destructive changes in the lung parenchyma. Here, we report a case of multisystem LCH in which the patient presented at 2 months of age because of simultaneous bilateral pneumothorax.     

Angiotensin-converting enzyme gene polymorphism and the progression rate of focal segmental glomerulosclerosis in Iranian children

Aim: Focal segmental glomerulosclerosis (FSGS) is one of the most common forms of glomerulonephritis leading to end-stage renal disease (ESRD). A few clinical and paraclinical factors are considered as contributing factors in progression rate. However, there are controversial reports on the relationship between ACE gene polymorphism and rapidity of progression of FSGS to ESRD in different populations. To elucidate this issue, we investigated the relationship between the insertion (I) and deletion (D) ACE gene polymorphism and rapidity of progression of FSGS to ESRD in Iranian children. Methods: Forty-one children aged 1–18 years admitted to St AlZahra Hospital, Isfahan, and St Ali Asghar Hospital, Tehran, Iran, with idiopathic FSGS were enrolled. Renal death was defined as a glomerular filtration rate (GFR) of less than 50 mL/min per 1.73 m² or a decreased GFR to less than 50% compare to baseline. Reaching renal death in less or more than 2 years were labelled as rapid progressors (RP) or slow progressors (SP), respectively. Intron 16 of the ACE gene was amplified by the polymerase chain reaction technique. Results: Twenty-eight patients were male and 13 were female. In 15 RP patients, the genotype distribution was 26.6% DD, 6.7% II and 66.7% ID. In 26 SP patients, the genotype was similar (38.6% DD, 7.6% II and 53.8% ID, P > 0.05). There were no statistically significant differences for ACE I/D gene polymorphism between the two groups of patients (P > 0.05). Conclusion: Our study revealed no correlation between ACE I/D gene polymorphism and rapidity of progression of FSGS to ESRD in Iranian children.     

New Combination of Triamcinolone, 5-Fluorouracil, and Pulsed-Dye Laser for Treatment of Keloid and Hypertrophic Scars

BACKGROUND: Keloids and hypertrophic scars are benign growths of dermal collagen that usually cause major physical, psychological, and cosmetic problems. METHODS: In this 12-week single-blinded clinical trial, 69 patients were randomly assigned into three study groups. In Group 1, intralesional triamcinolone acetonide (TAC, 10 mg/mL) was injected at weekly intervals for a total of 8 weeks. In Group 2 [TAC+5-fluorouracil (5-FU)], 0.1 mL of 40 mg/mL TAC was added to 0.9 mL of 5-FU (50 mg/mL). This combination was injected weekly for 8 weeks. In Group 3, in addition to weekly TAC+5-FU injection for 8 weeks, lesions were irradiated by 585-nm flashlamp-pumped pulsed-dye laser (PDL, 5-7.5 J/cm2) at the 1st, 4th, and 8th weeks. Lesions were assessed for erythema, pruritus, pliability, height, length, and width. RESULTS: Sixty patients completed the study. At the 8- and 12-week follow-up visits, all groups showed an acceptable improvement in nearly all measures, but in comparison between groups, these were statistically more significant in the TAC+5-FU and TAC+5-FU+PDL groups (p<.05 for all). At the end of the study, the erythema score was significantly lower, and itch reduction was statistically higher in the TAC+5-FU+PDL group (p<.05 for both). Good to excellent improvements (>50% improvement) were reported by the patients as follows: 20% in Group 1, 55% in Group 2, and 75% in Group 3, all of which were significantly different (p<.05). Good to excellent responses were reported by the blinded observer as follows: 15% in Group 1, 40% in Group 2, and 70% in Group 3. Their differences were statistically significant (p<.05). Atrophy and telangiectasia were seen in 37% of patients in TAC group. CONCLUSION: Overall efficacy of TAC+5-FU was comparable with TAC+5-FU+PDL, but the TAC+5-FU+PDL combination was more acceptable by the patients and produced better results. Its effect on lightening of the lesion was promising. The TAC+5-FU+PDL combination seems to be the best approach for treatment of keloid and hypertrophic scars.