Randall W. Powell

Causes of death in sickle cell disease: an autopsy study

Summary. More precise analysis of causes of death is needed to focus research efforts and improve morbidity and mortality in sickle cell disease. In this study, the morphological evidence of the cause of death was studied in 306 autopsies of sickle cell disease, which were accrued between 1929 and 1996. The most common cause of death for all sickle variants and for all age groups was infection (33–48%). The terminal infection was heralded by upper respiratory tract syndromes in 72·6% and by gastroenteritis in 13·7%. The most frequent portal of entry in children was the respiratory tract but, in adults, a site of severe chronic organ injury. Other causes of death included stroke 9·8%, therapy complications 7·0%, splenic sequestration 6·6%, pulmonary emboli/thrombi 4·9%, renal failure 4·1%, pulmonary hypertension 2·9%, hepatic failure 0·8%, massive haemolysis/red cell aplasia 0·4% and left ventricular failure 0·4%. Death was frequently sudden and unexpected (40·8%) or occurred within 24 h after presentation (28·4%), and was usually associated with acute events (63·3%). This study shows that the first 24 h after presentation for medical care is an especially perilous time for patients with sickle cell disease and an acute event. Close monitoring and prompt aggressive treatment are warranted.

Pediatric maxillofacial trauma: Unique features in diagnosis and treatment†‡

The purpose of this study was to review mechanisms, etiologies, associated injuries, and treatment of maxillofacial trauma in children and to compare them to similar adult injuries. Thirty blunt injuries (1984‐1986) comprised the childrens group, and 176 injuries, the adult group. Multiple associated injuries were more common in children, the most frequent being CNS and orthopedic injuries. Detailed anatomy of mandibular fractures required pleuridirectional tomography in 63% of the pediatric cases compared to 12% in adults. In the childrens group, the mandibular fractures were favorable in 56% of cases compared to 15% in adults. Children required shorter periods of intermaxillary fixation with no child requiring reapplication of fixation. Based on these comparisons, a protocol for the management of pediatric maxillofacial injuries has been developed.

Intraoperative diagnostic pneumoperitoneum in pediatric patients with unilateral inguinal hernias: The goldstein test

Over a four-year period 256 infants and children presenting with unilateral inguinal hernias underwent intraoperative diagnostic pneumoperitoneum to evaluate the contralateral groin. Sixty one patients had positive results and underwent contralateral hernia repair. Of the 195 negative tests 3 presented later with either a hernia, communicating hydrocele or cord hydrocele (false negative rate of 1.5%). This test represents a safe, economical, rapid, and accurate means to evaluate the contralateral groin in the pediatric patient presenting with a unilateral inguinal hernia.

Congenital colonic atresia

Nineteen cases of congenital colon atresia are presented with two deaths resulting in a mortality rate of 10.5%. The authors conclude that operative management of atresias proximal to the splenic flexure should include resection of the dilated proximal colon with primary ileotransverse colostomy or ileosigmoidostomy. Atresias distal to the splenic flexure should be managed with a colostomy with later establishment of gastrointestinal continuity.

The predictive validity of the Pediatric Trauma Score

The Pediatric Trauma Score was evaluated in 450 injured children by a paramedic in the field and a physician in an E.D. There was agreement between the scores of these two individuals 93.6% of the time, correlation coefficient 0.991, r2 = 0.982. Further testing at the 0.01 level of significance indicated that a positive association existed between these two variables in the population from which our sample was drawn. Mortality for the group was 2.9%. No deaths occurred in patients whose PTS was greater than 8, which was defined as the Critical Triage Point. The sensitivity of the PTS when used for triage at the critical triage point was 95.8%. The specificity of the PTS was 98.6%. The Pediatric Trauma Score appears to be highly accurate, reliable, predictable, and easy to use for assessing the severity of injury and hence is a straightforward modality for triage of injured children.

Pneumonectomy in infants and children: The use of a prosthesis to prevent mediastinal shift and its complications*

Twenty cases of pneumonectomy in infants and children performed over a 35-yr period have been reviewed. Pneumonectomy may be necessary for inflammatory, congenital, neoplastic, and traumatic conditions. The postoperative complications of progressive mediastinal shift can be minimized by the use of a pleural prosthesis.

Megarectum: A rare complication of imperforate anus repair and its surgical correction by endorectal pullthrough

Nine patients ranging in age from 3 mo to 15 yr presented with severe constipation and/or increasing incontinence. All had a huge, dilated atonic rectum and rectosigmoid demonstrated by barium enema. Six patients underwent resection of the abnormal bowel by an endorectal pull-through procedure with good to excellent results while 1 patient was corrected by a Swenson procedure.

Acute splenic sequestration crisis in sickle cell disease: Early detection and treatment

Acute splenic sequestration crisis (ASSC) in children with various forms of sickle cell disease can result in life-threatening circulatory collapse due to the loss of circulating blood volume. Over a 6-year period we have treated 12 patients ranging in age from 5 1/2 months to 7 years presenting with acute sequestration crisis. Eleven had homozygous sickle cell disease and the other had sickle-thalassemia. One patient died of acute circulatory collapse. Eight patients underwent splenectomy after a major episode of sequestration with no serious infectious complications up to 5 years following splenectomy. Three patients with minor episodes have been followed with no recurrences. To foster early detection of this potentially lethal complication of sickle cell disease, an educational program in our Comprehensive Sickle Cell Center instructs the parents to examine the spleen and bring their child in for evaluation if the spleen enlarges. A newly developed videotape describes the common symptoms of ASSC and illustrates the technique of palpating the spleen. With early detection of sickle cell disease by neonatal screening and the educational program, the morbidity and mortality from this complication of sickle cell disease can be reduced.

Castleman's disease in children

A three and one-half-year-old girl and a 12-year-old boy presented with features of the two clinical presentations of Castlemans disease or giant lymph node hyperplasia. The girl presented with anemia, fever, night sweats, hypergammaglobulinemia, and a palpable abdominal mass. Her symptoms were consistent with those seen in the plasma-cell type of this disease. The boy presented with acute appendicitis and a left hilar mass was noted on his chest radiograph. His asymptomatic presentation was typical of the hyaline-vascular form. Both patients are well without evidence of recurrence four years following resection. Castlemans disease is a benign disorder of lymph nodes that occurs rarely in children. Since the original report in 1954 we could find only 18 cases in the 16-year and younger age group and our patient with the symptomatic form represents the youngest patient reported in the English literature. The management requires surgical resection of the enlarged nodes both for diagnosis and therapy since the enlarged nodes can mimic malignant tumors of the lymphoid system. No recurrences have been reported in pediatric patients.

Regional blood flow response to hypothermia in premature, newborn, and neonatal piglets

BACKGROUND/PURPOSE Hypothermia (HT) remains a significant stress to the newborn and has been implicated in the pathogenesis of necrotizing enterocolitis (NEC). The authors assessed the effect of transient HT (32 degrees C) on regional organ blood flow in anesthetized piglets at age 7 to 10 days preterm (PREM), 1 to 2 days (NB), and 1 to 2 weeks (NEO). METHODS Radiolabeled microspheres were used to determine organ blood flows (mL/min/g) at baseline, 15, and 60 minutes after HT and 60 minutes after rewarming to baseline core temperature. RESULTS Heart rate and cardiac output decreased significantly in all groups. Cardiac flow decreased significantly in the NEO group, and central nervous system (CNS) flow decreased significantly in the NB and NEO groups. Both returned to baseline levels after rewarming. The PREM group experienced decreased cardiac, CNS, and intestinal blood flows but not to significant levels. NB and NEO intestinal blood flow showed significant decreases, which remained so after rewarming (a response not seen in hypoxia or hypovolemia). Cardiac output did not return to baseline levels in any group. CONCLUSIONS HT causes derangements in organ blood flows that differ from other deleterious stimuli such as hypoxia and hypovolemia. The prolonged intestinal ischemia supports HT as a factor in the development of NEC. This delay may offer opportunity to intervene in an attempt to lessen ischemia-reperfusion injury.