Management of pediatric type III supracondylar humerus fractures in the United States: results of a national survey of pediatric orthopaedic surgeons.

BACKGROUND: Supracondylar humerus fractures are common injuries in the pediatric population. The most severe, type III injuries, have seen the most debate on treatment regimens. Traditionally, these fractures were treated as surgical emergencies, most often fixed with percutaneous pinning in a cross-pin configuration. The recent literature shows that delayed fixation is comparable to emergent fixation as long as there is no vascular compromise with the injury. METHODS: A short survey was sent to Pediatric Orthopaedic Society of North America (POSNA) members using an online survey and questionnaire service. The purpose of the survey was to establish an overview of current practices in the United States concerning treatment of type III supracondylar humerus fractures and the influence of the recent literature on the management of these injuries. RESULTS: A total of 309 members, representing a wide range of locations and years in practice, responded to our survey. About 81% preferred to splint type III supracondylar humerus fractures and plan for fixation the following morning, assuming there was no issue necessitating emergent fixation. The preferred method of percutaneous fixation was fairly evenly distributed between cross-pin configuration (30%), 2 lateral pins (33%), and 3 lateral pins (37%). About 56% of those surveyed stated that the recent literature showing comparable outcomes with 2 lateral pins versus a cross-pin configuration had not changed their approaches to management of these fractures concerning the method of fixation. CONCLUSIONS: The trend in management of type III supracondylar humerus fractures in children is progressing toward delayed treatment and lateral pin configuration. The results provide an overview of the current practice of POSNA members concerning management of these fractures. We believe this information is beneficial to both pediatric-trained and nonpediatric-trained orthopaedic surgeons to help guide their decisions when dealing with these injuries. LEVEL OF EVIDENCE: This study is a Level V Therapeutic Study reviewing trends in the management of type III supracondylar humerus fractures in children. The previously described experts represent various levels of expertise in their preferred method of fixation.

A novel multilocus genotyping assay to identify genetic predictors of stroke in sickle cell anaemia.

We describe a novel multilocus genotyping assay permitting simultaneous identification of 60 candidate markers for stroke in sickle cell anaemia (SCA). Based on cerebral magnetic resonance imaging (MRI), 69 patients were divided into stroke and control groups. The variant allele, CBS 278thr, showed protection from stroke, whereas the apoE3 allele showed a trend towards association with increased stroke risk. Several other variant alleles [TNFalpha (-308)A, CETP (-628)A, apoCIII (-641)A] showed a trend towards significant associations with stroke risk. These preliminary results on a small group of patients suggest that a multilocus genotyping assay may be valuable in identifying genes that increase the risk of stroke in SCA.

Patterns of arginine and nitric oxide in patients with sickle cell disease with vaso-occlusive crisis and acute chest syndrome.

PURPOSE: Our objective was to evaluate L-arginine and nitric oxide metabolite (NOx) levels in children with sickle cell disease (SCD) at steady-state and during vaso-occlusive crisis (VOC). Because alterations in nitric oxide production may have an important role in the pathophysiology of SCD, our second aim was to determine if a relationship exists between these levels and vaso-occlusive crisis (VOC). PATIENTS AND METHODS: Plasma L-arginine and serum NOx levels were examined in 36 patients with SCD with 39 episodes of VOC and 10 children with SCD at steady-state. Daily levels were obtained in children requiring hospitalization. RESULTS: Steady-state L-arginine levels were normal in children with SCD. L-arginine levels were low, however, in children with VOC (37.4 +/- 2.7 vs. 53.6 +/- 4.6 micromol/L; P = 0.008) but returned to baseline during hospitalization. In contrast, NOx levels were normal at presentation but decreased during hospitalization for both patients with VOC and patients with acute chest syndrome (ACS) (21.1 +/- 2.0, 17.4 +/- 2.4, and 12.3 +/- 1.6 micromol/L, respectively; P < 0.05). In the patients with VOC who had ACS develop, L-arginine decreased to the lowest levels at the time of the ACS diagnosis, correlating with decreasing NOx levels. CONCLUSION: These data suggest that there may be a relationship between the L-arginine-nitric oxide pathway and vaso-occlusion in SCD. Low arginine levels during VOC could reflect a state of acute substrate depletion that results in a decrease in nitric oxide production.

Changing outcome of homozygous alpha-thalassemia: cautious optimism.

Only a few long-term survivors of homozygous alpha-thalassemia, a usually fatal condition, have been reported. The authors present a surviving infant with this disorder and discuss the complications, treatments, and implications of this genetic hemoglobinopathy. The child had no antenatal intervention and has been treated with regular transfusions. She has had normal growth and development and is currently 2.5-years-old. A literature review of survivors with Bart hemoglobinopathy reveals an intense perinatal course and a great prevalence of congenital urogenital and limb defects. Advances in antenatal diagnosis, intrauterine intervention, and postnatal treatments have resulted in extended survival of children with congenital defects that until recently were considered invariably fatal. Transfusion and chelation therapy and bone marrow transplantation provide long-term treatment and potential curative options.

Use of hydroxyurea in children ages 2 to 5 years with sickle cell disease.

The efficacy and side effects of hydroxyurea in young children with sickle cell disease are unknown. The authors followed-up eight young children (mean age 3.7 years) during therapy with hydroxyurea for an average of 137 weeks. Total and fetal hemoglobin levels rose with hydroxyurea therapy. Hospital admission rates and total hospital days decreased during hydroxyurea therapy. No unexpected toxicity occurred, and growth and development were unaffected. This pilot study suggests that hydroxyurea is safe and effective in young children with sickle cell disease.

Effects of red blood cell transfusion on resting energy expenditure in adolescents with sickle cell anemia.

BACKGROUND: Previous studies indicate that resting energy expenditure is elevated in children with sickle cell anemia, possibly caused in part by hemolysis and increased erythropoietic activity. The purpose of the present investigation was to determine whether erythrocyte transfusion normalizes resting energy expenditure in sickle cell anemia. METHODS: Five adolescents with sickle cell anemia (12-16 years old; 4 boys, 1 girl) were studied before and 1 week after erythrocyte transfusion before elective surgery or at the initial transfusion for growth failure. Resting energy expenditure was measured by indirect calorimetry, and laboratory measures were determined by routine, validated methods. Data comparisons were by nonparametric analysis. RESULTS: After erythrocyte transfusion, total hemoglobin levels increased (difference (D) = 15 g/l; p < 0.05), whereas hemoglobin S (D = -0.36; p < 0.05) and reticulocyte count (D = -0.12; p < 0.05) decreased. Mean pretransfusion resting energy expenditure was elevated to 124% above predicted levels (p < 0.05) and increased further to 134% above prediction (p < 0.05 vs. pretransfusion levels). Plasma triiodothyronine (T3) levels increased (D = 0.17 nmol/l; p < 0.05), reverse T3 (rT3) levels tended to decline (D = -0.04 nmol/l; p = 0.14), and rT3/T3 decreased (D = -0.03; p < 0.05). Plasma insulin-like growth factor-I (IGF-I) levels were low-normal before transfusion and did not change, despite the change in resting energy expenditure. CONCLUSIONS: The results confirm that resting energy expenditure is elevated in patients with sickle cell anemia. However, resting energy expenditure further increased after transfusion, despite decreased erythropoietic activity. A posttransfusion decrease in rT3/T3 may contribute to the increased resting energy expenditure. That there was no change in IGF-I implies that the growth hormone-IGF system is not involved in posttransfusion regulation of resting energy expenditure. Therefore, our data are not consistent with the hypothesis that increased resting energy expenditure in sickle cell anemia is directly related to erythropoietic activity. The mechanisms by which resting energy expenditure increases after transfusion in sickle cell anemia require additional investigation.

The mortality of Royal Naval submariners 1960-89.

OBJECTIVES: To examine the mortality pattern of submariners in the Royal Navy to assess the long term effects on health of serving in submarines. Any specific cause of death which was increased was considered in advance to be of interest, but attention focused particularly on cancer mortality. METHOD: A mortality follow up study: 15 138 submariners who had conducted their first submarine training between 1960 and 1979 were followed up through their time in the Navy and into civilian life, up to the end of 1989. The main outcome measures were the numbers of deaths and standardised mortality ratios (SMRs) which indicate whether the mortality from all causes and specific causes, particularly cancers, exceeds that in men in England and Wales. RESULTS: Mortality in submariners was lower than that for men in England and Wales with an all cause SMR of 86; this was comparable with that found in other studies of armed forces personnel. Cancer mortality was particularly low with an SMR of 69 and there was no particular cancer site which showed an excess. Increased mortality from digestive diseases was found, the excess being attributable to cirrhosis of the liver, which had an SMR of 221 based on 12 deaths, alcohol being a contributory factor in eight. Deaths from accidents and violence were also higher than expected with an SMR of 115, but this was due to high levels of accidents occurring after discharge from the Navy. There was no apparent trend in mortality with time since starting submarine work. Likewise there was no pattern by calendar period, although the excess of cirrhosis of the liver was confined to the period 1970-9. CONCLUSION: The submariners seemed to be a healthy group with low mortality overall. Working in submarines was not associated with any increased cancer mortality. Excess deaths from cirrhosis of the liver, and from accidents and violence after leaving the Navy, were of some concern but they cannot be attributed directly to the submarine environment.

New therapies and approaches to transfusion in sickle cell disease in children.

Until recently, transfusion was the only effective therapy for children with sickle cell disease (SCD). Although transfusion can be used to prevent or treat many of the complications of SCD, it has often been used indiscriminantly and with considerable risk to the patient. Recent studies attempted to define those clinical situations in which transfusion is effective as well as to optimize its delivery while minimizing complications. Other therapies are emerging, and in the future many will likely play an important role in the treatment of children with SCD. These therapies include pharmacologic interventions, such as hydroxyurea; bone marrow and cord blood transplantation; and improvements in supportive and preventive care. The future for new therapies looks promising, but the appropriate role for many of these therapeutic interventions remains to be determined.

Pulmonary complications.

The management of patients with acute chest syndrome is changing as its etiology and pathophysiology are being defined. Current management should include aggressive evaluation and monitoring, and treatment should be tailored to each patient's clinical course. New therapies show promise in reducing morbidity of acute chest syndrome in the future.

Core decompression in avascular necrosis of the hip in sickle-cell disease.

Sickle-cell disease (SCD) is the most common cause of avascular necrosis (AVN) of the hip in childhood. It results in significant physical impairment and chronic pain, and often progresses to require hip replacement. Conservative therapy is ineffective. We evaluated whether core decompression can arrest progression of AVN. We performed 13 coring procedures in 10 patients with SCD and AVN. Patients ranged from age 9-21 years at diagnosis (mean, median age, 15 years); five hips were stage I, six hips were stage II, and two hips were stage III. Mean follow-up on these patients was 3.7 years. Efficacy of the procedure was evaluated by clinical improvement in pain, radiographic progression, and need for further surgery. All 5 stage I patients had substantial improvement in pain, and only one showed X-ray progression. Five of the 6 (83%) stage II patients had improvement in pain, and 2 patients progressed on X-ray. Both stage III patients progressed on X-ray, but one was clinically improved. None of the 10 patients has required further surgery. Our results demonstrate that in early AVN, core decompression was beneficial for almost all patients, even with progression on X-ray. Core decompression should be considered in the management of SCD patients with early AVN.

Ototoxicity in hemoglobinopathy patients chelated with desferrioxamine.

PURPOSE: Ototoxicity often limits the dose of desferrioxamine (DFO) tolerated by patients who are transfusion dependent. Current recommendations advise doses of < 50 mg/kg/day after early reports noted higher rates of oxotoxicity with increasing doses. There have been no follow-up studies to determine the effect of this recommendation on oxotoxicity and iron overload. METHODS: We followed 28 patients who were chronically chelated with serial audiograms over a 5-year period. Patients with and without oxotoxicity were compared with respect to age, disease, DFO dose, peak DFO dose, length of DFO therapy, ferritin, and therapeutic index. RESULTS: Eight of the 28 patients (29%) had an abnormal audiogram during threshold testing. Two patients had two separate episodes with hearing deficit. Nine of the 10 episodes were high-frequency losses, with seven being moderate and three mild. All deficits were rapidly reversible with DFO dose reduction. No significant differences were found between the affected and unaffected groups with respect to age, DFO dose or duration, ferritin, or therapeutic index. Numbers of affected patients were small, but patients with SCD differed from patients with thalassemia in that they developed ototoxicity earlier and with lower doses of DFO and lower therapeutic indexes. CONCLUSIONS: Despite DFO doses usually felt to be low risk for ototoxicity, we found a high rate of ototoxicity in our patients who we've chronically chelated. No variables were identified that reliably predicted ototoxicity. We stress the need for regular audiological exams and feel no dose of DFO is "safe" from the development of ototoxicity.