Transcription factor genetics and biology in predisposition to bone marrow failure and hematological malignancy.

Transcription factors (TFs) play a critical role as key mediators of a multitude of developmental pathways, with highly regulated and tightly organized networks crucial for determining both the timing and pattern of tissue development. TFs can act as master regulators of both primitive and definitive hematopoiesis, tightly controlling the behavior of hematopoietic stem and progenitor cells (HSPCs). These networks control the functional regulation of HSPCs including self-renewal, proliferation, and differentiation dynamics, which are essential to normal hematopoiesis.

Effectiveness of a calcium hydroxide and chlorhexidine digluconate mixture as disinfectant during retreatment of failed endodontic cases.

Objective: The purpose of this in vivo investigation is to compare the effect of a slurry of Ca(OH)2 mixed in aqueous 2% chlorhexidine (CHX) versus aqueous Ca(OH)2 slurry alone on the disinfection of the pulp space of failed root-filled teeth during endodontic retreatment.

Study Design: Forty single-rooted previously root-filled teeth with associated periradicular lesions were included. The teeth were nonsurgically retreated and medicated over 3 treatment visits with 7-10-day intervals with either Ca(OH)2 in water or Ca(OH)2 in 2% aqueous CHX.

Molecular detection of Enterococcus species in root canals of therapy-resistant endodontic infections.

Objectives: The objectives were to identify Enterococcus spp in nonhealing endodontic cases using PCR amplification and molecular sequencing, and to determine if the prevalence of enterococci is increased in diabetic patients.

Study Design: Specimens from 40 cases undergoing retreatment were incubated in prereduced thioglycollate broth at 37 degrees C. Extracted DNA had PCR amplification using primers that target the tuf gene of 14 Enterococcus spp.

Surgical management of coccidioidomycosis in children.

Background/purpose: Operations for Coccidioides immitis infection in children have received little study. The authors perceived an increase in the number of patients requiring operations for coccidioidomycosis at Phoenix Children's Hospital. The authors therefore reviewed the surgical management of children with C immitis infection in Phoenix, Arizona.

Foreign body aspiration in children: value of radiography and complications of bronchoscopy.

Purpose: The authors undertook a 10-year review of bronchoscopies for airway foreign bodies in children to test assertions in previous reports that (1) characteristic abnormalities in chest radiographs are important indicators of the need for bronchoscopy and (2) experienced operators incur negligible complications.

Methods: The medical records of 293 children who underwent bronchoscopies by experienced pediatric surgeons for suspected airway foreign bodies were reviewed for patient age, symptoms, duration of symptoms before bronchoscopy, prebronchoscopy radiographs, type of foreign body, anatomic location of foreign body, success of bronchoscopic removal, length of hospital stay, and complications.

Results: Of the 293 bronchoscopies, 265 showed airway foreign bodies.

Undescended testicle associated with spigelian hernia.

The authors encountered a patient with a spigelian hernia (SH) and undescended testicle (UDT), making the fourth reported case with this combination. This patient provides further evidence that congenital SH predisposes neonates to UDT.

A 3-year survivor of near-total intestinal aganglionosis.

A newborn girl had no ganglion cells beyond 2 cm distal to the ligament of Treitz. The proximal jejunum was dilated. A jejunostomy constructed 46 cm distal to the ligament of Treitz functioned.

Survival of nonpalpable undescended testicles after orchiopexy.

Among 496 boys who underwent operations for the preoperative diagnosis of undescended testicle, 41 patients had high undescended testicles that were not palpable preoperatively. Four of the 41 were bilateral, for a total of 45 high undescended testicles. Eight of the 45 were lost to long-term follow-up.

Obstruction of the neonatal airway from teratomas.

Most neonates with cervical or oral-pharyngeal teratomas have airway obstruction and an obvious mass. In previous reports, obstruction of the airway had caused 49 deaths in 164 newborns with cervical teratomas and five deaths in 24 newborns with oral-pharyngeal teratomas. Most were reported before the era of prenatal ultrasound.

Surgical management of neonatal interstitial emphysema.

In the past 6 years, 50 patients with interstitial emphysema in our newborn nursery were treated with open operative procedures to remove interstitial air. Thirty-one were critically ill newborns with severe progressive pulmonary interstitial emphysema treated with pleurotomies. Five underwent pleurotomies on both sides, for a total of 36 operations in the 31 patients.

Indomethacin versus immediate ligation in the treatment of 82 newborns with patent ductus arteriosus.

In the past 10 years, echocardiography and prostaglandin inhibition have changed the management of the patent ductus arteriosus (PDA) in newborns with respiratory distress syndrome (RDS). To judge the results of our present management for morbidity and survival, we analyzed data retrospectively on 82 newborns who were treated from 1977 to 1983 for PDA. We compared their hospital courses and outcomes with a similar group of 45 infants reported by us 10 years ago who were treated by early ductal ligation.

Pneumoperitoneum in infants without gastrointestinal perforation.

Pneumoperitoneum signals gastrointestinal perforation and, as a rule, requires prompt laparotomy. However, we have recently encountered infants with severe respiratory distress who developed pneumoperitoneum without any gastrointestinal leak. These 10 patients were managed without operations.

Intrapericardial teratoma--neonatal cardiorespiratory distress amenable to surgery.

Intrapericardial teratoma in early infancy produces critically severe cardiorespiratory distress and often results in the death of the infant. A recent patient in our newborn nursery displayed the distinctive combination of cardiorespiratory distress and enlargement of the "cardiothymic" silhouette. The echocardiographic demonstration of pericardial effusion and cardiac displacement suggested the diagnosis.

Extremity swelling in an infant with copper and zinc deficiency.

A female infant born with jejunal atresia and a severely shortened bowel acquired progressive thickening of her thighs at 7 mo of age. While receiving parenteral nutrition, all four extremities rapidly became massively thickened and tender, and the child made no effort to move them. X-rays revealed large subperiosteal hematomas encompassing each femur, tibia, and humerus as the cause of the swelling.

Emergency splenectomy for idiopathic thrombocytopenic purpura in children.

Splenectomies were performed in 30 children out of a total of 183 with idiopathic thrombocytopenic purpura (ITP). Of these 30, ten splenectomies were performed on an emergency basis. Significant spontaneous gross hematuria, gastrointestinal bleeding or epistaxis appear to represent harbingers of intracranial hemorrhage and constitute indications for emergency splenectomy.

Jejunal atresia with absent mesentery and a helical ileum.

Of 59 infants with jejunoileal atresia, seven presented with absence of mesentery, the main superior mesenteric artery ceasing to exist beyond the origin of the right colic or ileocolic artery, and a helical ileum. This distinctive form of jejunal atresia has been recognized with increasing frequency in recent years. Distal to the atresia, the small bowel receives its blood supply retrograde from an artery derived from the ileocolic or right colic arcades, and the ileum coils around its nutrient artery in an "apple peel" or "Christmas tree" deformity.

Jejunoileal atresia: a proposed classification.

An experience with 59 infants with jejunal and ileal atresia is presented along with a proposed classification based on a combination of morphology and clinical characteristics. It would appear that the characteristics of each type are sufficiently distinct as to render this worthwhile classification.