Congenital rhabdomyosarcoma of shoulder

A 16-day-old female was referred with congenital swelling on her right shoulder. On examination, there was a hard, round, ecchymotic, nontender, slightly movable, warm and shiny 10x15 cm mass on the right axillary pits which was extended to the right side of neck and chest wall. The mass separated the shoulder from the chest wall causing paralysis of right hand. Chest X-ray, ultrasound and MRI with contrast demonstrated a soft tissue mass suspected to be a hemangioma. The mass rapidly increased in size despite aggressive steroid therapy with rupture and bleeding. On the 45th post natal day the baby was taken to operating room to control the bleeding and if possible total excision of the mass. The mass was separated easily from the surrounding tissue and was excised along with right upper extremity. At the end of surgery the baby had cardiac arrest, and apparently died of Disseminated Intravascular Coagulation [DIC]. The final pathology report was Rhabdomyosarcoma [RMS]

Congenital arhinia: case report of a rare congenital anomaly

Congenital absence of the nose or arhinia is a rare defect of embryogenesis often associated with other anomalies. Arhinia is a life-threatening condition that requires a highly skilled neonatal resuscitation team in the delivery room. The associated anomalies often have a significant effect on the immediate as well as long-term outcome of the neonate. This report presents a case of congenital arhinia and reviews the management of such cases

comparison of CRIB, CRIB II, SNAP, SNAPII and SNAP-PE scores for prediction of mortality in critically ill neonates

Clinical Risk Index of Babies [CRIB], Score for Neonatal Acute Physiology [SNAP], an update of the Clinical Risk Index for Babies score [CRIB II] and Score for Neonatal Acute Physiology - Perinatal Extension [SNAP-PE] are scoring devices developed in neonatal intensive care units. This study reviewed these scoring systems in critically ill neonates to determine how well they could predict mortality. This prospective cohort study was conducted at the neonatal intensive care units of Mofid and Mahdieh hospitals between March 2006 and May 2009. We evaluated CRIB, CRIB II, SNAP, SNAPII and SNAP-PE score for each neonate and the final scores were then obtained. The predictive accuracy of these parameters were expressed as area under the receiver operative characteristic curve, sensitivity, specificity, positive predictive value and negative predictive value. Of 404 neonate evaluated 53% were male. Primary diagnoses were respiratory distress syndrome, gastrointestinal obstruction, sepsis, prematurity, and neuromuscular diseases. The authors detected mortality in 20.5% and found a significant difference in scoring systems between survived and death groups. The mean CRIB score in survived neonates was 2.57 +/- 3.66 and in death neonates 8.43 +/- 4.66 [p value<0.001]. We also found that the SNAP score had the highest area under the curve and the highest sensitivity, specificity, positive predictive value, negative predictive value and we had the lowest score for CRIB II. We concluded that the neonatal scoring systems could be a useful tool for prediction of mortality in NICUs and SNAP can predict the mortality better than the others

[ study of the hyperuricemia in obese children and correlation between hyperuricemia and factors related to the obesity]

Recently obesity in pediatric population is rising fast and its early onset complications [like those seen in adults] are keeping pace with it. It seems that a high level of urinary uric acid [UUA] could be the result of reproduction of uric acid in the body. On the other hand, it was shown to be the result of decreased urinary clearance of uric acid in some obese adults. Target of study is the mechanism of hyperuricemia in obese children and also the correlation between hyperuricemia and factors related to the obesity. We studied 75 severely obese children [42 boys and 33 girls] and compared them with 30 non-obese children [20 boys and 10 girls]. Serum leptin, lipid profile, liver tranaminases, uric acid and creatinine and also 24 hours urinary uric acid and creatinine were measured, and uric acid clearance was calculated in each case. Serum uric acid level [U.A] in obese children were significantly higher than those in control children, but urinary uric acid [UUA], uric acid clearance [CUA] and fractional excretion of uric acid [FEUA = CUA/CCR * 100] were lower in the obese children than those in controls. The low UUA in the obese children suggests that their hyperuricemia was not the result of over intake of food containing uric acid. There was a close correlation between uric acid and age/body weight and it becomes closer as they aged and gained weight. These results suggest that hyperuricemia in obese children is mainly attributable to impaired renal clearance of uric acid and not to overproduction in the body and it will higher age and body weight

Perinatal testicular torsion: a case report

Torsion of the testicles is typically seen in children of prepubertal age. This condition is extremely rare in perinatal period. The perinatal diagnosis of spermatic cord torsion is often difficult. Here, we report a case of a male newborn baby who was born with swollen and erythematous scrotum. Ultrasonography showed an enlarged left testis, with a nonhomogeneous texture and a regular border, bilateral hydrocele, and septate fluid collection on the left side. Following the diagnosis of acute scrotal condition, surgical exploration was performed. The left testis was gangrened with a blue coloration because of complete extravaginal torsion. Left orchidectomy, along with a fixation of right testis were carried out. Perinatal extravaginal testicular torsion, although a rare condition, is a true immediate emergency that needs early and quick medical attention, and surgical intervention to save testicular tissue