Results of rhabdomyosarcoma treatment in a developing country

Fifty-one children (median age: 4.5 years; 4 months-16 years) diagnosed with rhabdomyosarcoma were treated in our center between 1980-1999. The primary sites were head and neck in 31.4%, the genito-urinary system in 21.6%, and extremities in 9.8% of the patients. The histopathologic subtypes were embryonal in 80.4%, alveolar in 9.8%, and undifferentiated in 9.8%. The majority of the patients were considered group III (47%) and group IV (25.5%) according the criteria of the Intergroup Rhabdomyosarcoma Study (IRS). Primary total tumour resection was performed in only 27.5% of the patients. The patients were treated with assigned regimens of IRS II and IRS III protocols. Radiotherapy was applied to 92.1% of the patients. Thirty-four patients (66.7%) were lost to follow up, and of the remaining 17 patients, 7 patients (41.2%) died, relapse occurred in 9 patients (52.9%) and 10 patients (58.8%) are alive. The percentage of cases lost to follow up during the first 10 years and the following 9 years of the study were 77.4% and 50%, respectively. In compliance with cancer treatment remains a major problem in developing countries.     

Pneumatosis Cystoides Intestinalis Associated with Perforated Chronic Duodenal Ulcer and Meckel’s Diverticulum

A case of pneumatosis cystoides intestinalis (PCI) associated with perforated duodenal ulcer and Meckel’s diverticulum is presented. The patient was managed with direct suturing of the perforation and reinforcement with an omental patch. Meckel’s diverticulum was excised. PCI was followed up and disappeared postoperatively in the 4^th week.     

Long‐Term Exposure to Biomass Fuel and Its Relation to Systolic and Diastolic Biventricular Performance in Addition to Obstructive and Restrictive Lung Diseases

Background: Previous studies have demonstrated an increased risk for cardiovascular events and pulmonary disease in patients with biomass fuel exposure (BFE). However, biventricular heart function has yet to be investigated in these patients. Left ventricular (LV) myocardial performance index (LVMPI), which is an index of global ventricular function, incorporates ejection, isovolumic relaxation, and contraction times. In this study, pulmonary function and biventricular heart function were investigated in nonsmoking female patients with BFE. Methods: Our study population consisted of 46 female patients with BFE (group 1) and 31 control subjects (group 2). Pulmonary function tests and transthoracic echocardiographic examination were performed. Right ventricular myocardial performance index (RVMPI) and LVMPI were obtained by tissue Doppler imaging echocardiography (TDI). Results: BFE caused obstructive and restrictive spirometric impairments. RVMPI was higher in group 1 (0.55 ± 0.07) than group 2 (0.46 ± 0.06) (P = 0.042) and LVMPI was higher in group 1 (0.54 ± 0.08) than group 2 (0.47 ± 0.05) (P = 0.032). Also, pulmonary artery systolic pressure was higher in group 1 than group 2 (P = 0.02). Conclusions: BFE causes both obstructive and/or restrictive lung disease and systolic and diastolic biventricular dysfunction. Nonetheless, long‐term studies are needed to understand on BFE‐related ventricular dysfunctions and to document subsequent cardiovascular events. (Echocardiography 2011;28:52‐61)     

Effects of Low Sodium Dialysate in Chronic Hemodialysis Patients: An Echocardiographic Study

Background. Chronic kidney disease (CKD) and hemodialysis (HD) patients who cannot restrict sodium consumption in their diets sometimes develop significant saline excess and hypertension between dialyses. This study assessed the effect of relatively low sodium dialysate dialysis on changes of echocardiography in hemodialysis patients. Methods and Results. Eighteen patients with end stage renal failure on chronic HD were studied (8 females, 10 males) with a mean age 48.3 ± 14.6 (24–70) years. The mean time on HD was 30.8 ± 14.0 (12–60) months. Patients with hematocrit levels under 24% were excluded from the study. In all patients, echocardiography was performed thrice weekly before and after eight-week HD treatment with low sodium dialysate hemodialysis by the same operator (135 mEq/L for patients with sodium levels less than 137, 137 for patients with sodium levels over 137). Left atrium (LA) and left ventricle (LV) volumes and ejection fractions were measured, specifically: LV systolic diameter (LVSD), LV diastolic diameter (LVDD), interventricular septum (IVS), tricuspid regurgitation (TR), mitral regurgitation (MR), pulmonary artery pressure (PAP), and inferior vein cava diameter (IVCD). Results. In terms of echocardiographic parameters, LVSD, TR, PAP, and IVCD were statistically decreased after low-sodium dialysate treatments (p?=?0.002, 0.04, 0.013, and 0.00, respectively). Predialysis systolic and diastolic blood pressure (BP), post-dialysis systolic blood pressure, and interdialytic weight gain was statistically decreased when compared to basal levels (p?=?0.00, p?=?0.011, p?=?0.022, p?=?0.001, respectively). Conclusion A reduction of the dialysate sodium concentration based on the predialysis sodium levels of the patients could reduce the systolic BP and decrease the volume load on the heart as assessed by echocardiography. Within this short period, postdialysis diastolic BP could not be lowered. The effect of this approach should be studied in broad and lengthy series.     

Development of the principal nucleus trigeminal lemniscal projections in the mouse

The principal sensory (PrV) nucleus‐based trigeminal lemniscus conveys whisker‐specific neural patterns to the ventroposteromedial (VPM) nucleus of the thalamus and subsequently to the primary somatosensory cortex. Here we examined the perinatal development of this pathway with carbocyanine dye labeling in embryonic and early postnatal mouse brains. We developed a novel preparation in which the embryonic hindbrain and the diencephalon are flattened out, allowing a birds‐eye view of the PrV lemniscus in its entirety. For postnatal brains we used another novel approach by sectioning the brain along an empirically determined oblique horizontal angle, again preserving the trigeminothalamic pathway. PrV neurons are born along the hindbrain ventricular zone and migrate radially for a short distance to coalesce into a nucleus adjacent to the ascending trigeminal tract. During migration of the spindle‐shaped cell bodies, slender axonal processes grow along the opposite direction towards the floor plate. As early as embryonic day (E) 11, pioneering axons tipped with large growth cones cross the ventral midline and immediately make a right angle turn. By E13 many PrV axons form fascicles crossing the midline and follow a rostral course. PrV axons reach the midbrain by E15 and the thalamus by E17. While the target recognition and invasion occurs prenatally, organization of PrV axon terminals into whisker‐specific rows and patches takes place during the first 4 postnatal (P) days. Initially diffuse and exuberant projections in the VPM at P1 coalesce into row and whisker specific terminal zones by P4. J. Comp. Neurol. 521:299–311, 2013. © 2012 Wiley Periodicals, Inc.     

Corticosteroids in Duchenne muscular dystrophy: Major variations in practice

Introduction: In 2004, a Cochrane Review and AAN practice parameter concluded that prednisone 0.75 mg/kg/day is of short‐term efficacy in Duchenne muscular dystrophy (DMD). Subsequent efforts to standardize care for DMD indicated wide variation in corticosteroid use. Methods: We surveyed physicians who follow patients with DMD, including: (1) clinics in the TREAT‐NMD (Translational Research in Europe—Assessment and Treatment of Neuromuscular Diseases) network (predominantly Europe) and (2) U.S. MDA clinic directors. We also documented the co‐administered corticosteroids in a trial of a putative treatment (ataluren) for DMD. Results: Of 105 Treat‐NMD clinicians, corticosteroids were not used in 10 clinics, and 29 different regimens were used—the most frequent 0.75 mg/kg/day prednisone (61 centers); 10 days on/10 days off (36 centers); 0.9 mg/kg/day deflazacort (32 centers); and 5 mg/kg/day on weekends (10 centers). Similar diversity was identified in MDA clinics and in the ataluren trial. Conclusions: Variability in corticosteroid use suggests uncertainty about risks/benefits of corticosteroid regimens for DMD. Muscle Nerve, 2013     

The Abbé island flap for the reconstruction of severe secondary cleft lip deformities

Primary repair of the cleft lip is often associated with secondary deformities, which require revision and secondary reconstruction. Patients with one or all of the following, a tight lip restricting use of orthodontic appliances, absence of a Cupid's bow, or absence of vermilion tubercle, were treated with the Abbé island flap. A triangular muco-musculo-cutaneous island flap was designed in the central segment of the lower lip. A full-thickness incision of skin, muscle, and mucosa was made in the midline of the upper lip, dividing the lip into two segments, and the island Abbé flap was inserted. One week after surgery, the pedicle of the island flap was divided and the inset of the flap completed. Sixty patients with severe secondary cleft lip deformities (36 males and 24 females) were treated. Good aesthetic and functional results were achieved during 1 to 17 years of follow-up. Insertion of the Abbé flap resulted in release of the tight upper lip and a new, inconspicuous scar. Use of orthodontic appliances was facilitated, and tightness of the tissue, which restricted the expansion of the alveolar process, was eliminated. Adequate tissue was transferred to the upper lip, which improved the bulk of the lip and vermilion tubercle. The retrusion of the midface and the projection of the upper lip were also improved, and the upper and lower lips became better balanced. Donor site morbidities were insignificant. Use of the Abbé flap in selected patients resulted in successful reconstruction of secondary cleft lip deformities.