Use of graft flow measurement and computerized tomography angiography to evaluate patency of endoscopically harvested radial artery as sequential graft in coronary artery bypass surgery.

AIM: Endoscopic radial artery (RA) graft harvesting in coronary artery bypass surgery (CABG) is attractive but concern remains regarding early graft failure. We evaluated RA graft patency via intraoperative graft flow measurements and mid-term computerized tomography angiography (CTA). METHODS: The patients who had RA harvested by endoscopic technique which was used as sequential grafts were retrospectively reviewed. Graft quality was confirmed by intraoperative transit time flow measurements. Graft stenosis was defined as stenosis >70% on CTA, 6-12 months postoperatively. RESULTS: From 2007 to 2011, 58 patients underwent endoscopic RA harvesting for sequential bypassed grafts. All received total arterialized grafts, including 22 (38%) bilateral internal thoracic arteries (ITAs), with 208 total bypassed grafts (mean: 3.59±0.52) and 128 RA bypassed grafts (mean: 2.21±0.35). Off-pump technique was performed in 43 (84%) of 51 isolated CABG patients. The pulsatility index of graft flow of the left, right ITA and sequential RA grafts with 2 or 3 targets were 1.8±0.7, 2±0.8, 1.9±0.4, and 1.7±0.7, respectively. There was no hospital mortality, and median intensive care unit and hospital stay was 2 and 8 days. Follow-up was completed in 57 patients, but 3 patients refused CTA due to lack of exertional angina. Stenosis of the left, right ITA, and RA grafts occurred in 1/54 (1.9%), 1/21 (4.8%), and 11/120 (9.2%). After a mean of 35.8±10.9 (median: 30.7) months follow-up, there was no late mortality and one documented myocardial infarction was reported. Age, diabetes, previous percutaneous coronary intervention, off-pump technique, RA target number, and graft flow or pulsatility index did not predict RA stenosis. Only RA grafts targeting the circumflex territory had an adverse impact. CONCLUSION: The RA of appropriately selected patients can be harvested safely by endoscopic technique and can be used as sequential grafts for CABG with satisfactory outcomes. Intraoperative flow measurement can assure the quality of the grafts. CTA is a valuable tool for patency follow-up.

Meconium aspiration syndrome: experiences in Taiwan.

Meconium aspiration syndrome (MAS) is still one of the most challenged diseases for the neonatologists. We reviewed our earlier studies of MAS in an attempt to provide some idea for more understanding of MAS. This study is a retrospective review and summarization of our earlier studies in MAS at two tertiary neonatal centers in Taiwan. Incidence of MAS was decreased sharply in Taiwan. MAS infants who required resuscitation in the birth room being out-born, birth asphyxia and infants who developed persistent pulmonary hypertension (PPHN) and pneumothrax were associated with increasing mortality. In MAS infants who neither required mechanical ventilation nor had a history suggestive of perinatal infection, antibiotic treatments would not affect the outcome of MAS. Dexamethasone did reduce inflammation response and improve cardiopulmonary perfusion. However, steroids did not prevent the development of PPHN. Our review provided the risk factors of mortality for MAS. Antibiotic treatments should not be a routine for every infant with MAS. Although steroids reduce pulmonary inflammation, their role in the prevention of PPHN remains to be further studied.

The effects of early postnatal dexamethasone therapy on pulmonary outcome in premature infants with respiratory distress syndrome: a two-year follow-up study.

AIM: To evaluate the pulmonary outcome at corrected age of 2 y on preterm infants who participated in a double-blind trial of early postnatal dexamethasone therapy (< 12 h after birth) for the prevention of chronic lung disease. METHODS: Clinical respiratory status, blood gases, acid-base balance and pulmonary function were evaluated at corrected age of 2 y in 116 preterm infants (59 infants in the control group; 57 in the dexamethasone-treated group). In the dexamethasone-treated group, dexamethasone was administered intravenously every 12 h in tapering doses: 0.25 mg/kg on days 1 through 7, 0.12 mg/kg on days 8 through 14, 0.05 mg/kg on days 15 through 21, and 0.02 mg/kg on days 21 through 28. RESULTS: The clinical and laboratory characteristics in the perinatal period were comparable between the groups. At the time of follow-up (mean +/- SD corrected age was 25.1 +/- 4.8 mo for the control group and 24.6 +/- 5.1 mo for the dexamethasone-treated group), there was a slightly lower mean body weight and body length, and a lower psychomotor developmental index in the dexamethasone-treated group than in the control group (10.9 +/- 2.1 vs 11.5 +/- 1.9 kg, 84.4 +/- 6.1 vs 85.9 +/- 5.8 cm, and 82 +/- 24 vs 89 +/- 26, respectively); however, these differences were not statistically significant. There were no significant differences between the control and dexamethasone-treated groups in clinical respiratory status, blood gases, acid-base balance or in lung mechanics (V(T): 9.5 +/- 2.0 vs 9.4 +/- 1.9 ml/kg; V(min): 0.23 +/- 0.04 vs 0.23 +/- 0.03 l/min/kg; C(RS): 13.1 +/- 3.9 vs 12.6 +/- 3.6 ml/kPa/kg; R(RS): 1.56 +/- 0.64 vs 1.62 +/- 0.58 kPa/l/s, respectively). CONCLUSION: There was no apparent adverse respiratory outcome associated with early postnatal dexamethasone therapy.

Major brain lesions detected on sonographic screening of apparently normal term neonates.

Major congenital or acquired structural abnormalities of the brain, with significant prognostic implications have rarely been emphasised in apparently normal infants. Our purpose was to investigate the prevalence and types of major brain lesions in clinically normal term neonates using sonography. From January 1999 to December 2001, we examined 2309 clinically normal term neonates within 3 days of birth. Of these, six (0.26%) had major brain lesions, including three cases of intracerebral haemorrhage (two grade II intraventricular and one temporal lobe haemorrhage), two of corpus callosum agenesis and one lacunar infarct in the territory of the left middle cerebral artery. One child was lost to follow-up, because of adoption, and four had borderline to significant developmental delay at a mean age of 24 months (range 12-36 months). Although the prevalence of major brain lesions was low, these infants have a higher risk of later neurodevelopmental disability, despite an early asymptomatic period. Universal neonatal brain ultrasound screening may help early diagnosis of rare major lesions, some of which have prognostic implications.

Fuzzy oxygen control system for the indirect calorimeter of premature infants.

The indirect calorimetric system of measuring O2 consumption and CO2 production has been developed for energy expenditure estimation of premature infants. This apparatus requires an input room air mixing with pure oxygen to obtain a stable gas with definite oxygen concentration flowing into the hood for neonatal breath or supplemental oxygen treatments. In this paper, we propose an oxygen control system based on fuzzy control logic to automatically adjust the mixing ratio of room air to pure oxygen gas from the hospital's supply system, designed for premature infants. It is designed to reduce the risks of oxygenic toxicity and retinopathy of prematurity by lowering the overshoot of oxygen concentration. Its performance was evaluated and optimal membership functions were obtained. As a result, the system is quite robust with little effect caused by disturbance and has little or no overshoot when step changing the level of oxygen concentration in the mixed gas.

Effect of antenatal dexamethasone on the expression of endothelial nitric oxide synthase in the lungs of postnatal pups.

Activities of endothelial nitric oxide synthase (eNOS) are developmentally regulated and its presence at birth may play a role in the transition of cardiopulmonary circulation. Antenatal dexamethasone (Dex) therapy accelerates fetal lung maturation. We speculate that Dex therapy may enhance pulmonary eNOS protein expression in the newborn. This article examines whether antenatal Dex therapy affected the expression of eNOS in the lungs of rat pups in the postnatal period. Time-dated pregnant Wistar rats were subjected to 2 doses of Dex (0.8 mg/kg, intramuscularly, daily) or equivalent volume of normal saline at the 18th and 19th gestational day and delivered naturally. The newborn pups were randomly assigned to 4 groups by age: days 1, 3, 5, and 7. After homogenization, abundance of eNOS protein in lungs was determined by Western blot analysis. There were 7 dams in each group. Mean body weights of the pups in the Dex group were lighter than those in the control at birth and remained stunted up to day 7 (5.68+/-0.47 g v 6.34+/-0.47 g, P <.01). However, there were no differences in wet lung weights and lung/body weight ratios between both groups in the study period. Abundance of eNOS protein expression decreased in both the control and Dex groups (P < .01). Pups that received antenatal Dex had 39% more in abundance of eNOS protein expression in lungs when compared to the control on day 1 (P < .05) but there were no differences between both groups from day 3 to 7. We conclude that antenatal Dex therapy enhances the abundance of eNOS protein expression in the lung at birth and could be a factor in improving respiratory functions in infants who received antenatal steroid therapy.

Vallecular cyst: an uncommon cause of stridor in newborn infants.

UNLABELLED: Vallecular cyst, a rare but generally benign lesion in the larynx, may cause stridor and even life-threatening airway obstruction in early infancy. We retrospectively studied 14 cases of newborn infants with vallecular cyst. There was no gender predilection and most cases were full-term and appropriate for gestational age. The clinical presentations included stridor, chest wall retraction, feeding difficulties and failure to thrive. Laryngomalacia was the most common associated anomaly. Flexible laryngoscopy was sufficient for diagnosing the vallecular cyst and larygmalacia. Maintenance of airway patency, nutritional support, and de-roofing of the cyst were the mainstays of management. CONCLUSION: Vallecular cyst should be included in the differential diagnosis of stridor in newborn infants. Respiratory and feeding difficulties in these patients can be dramatically improved after appropriate surgical removal of the cyst.

Mortality, morbidity, length and cost of hospitalization in very-low-birth-weight infants in the era of National Health Insurance in Taiwan: a medical center's experience.

The impact of implementation of the National Health Insurance on the outcome, cost, and length of hospitalization of very-low-birth-weight (VLBW) infants is not clear in Taiwan. These data are important for the planning of medical care and regionalization in this area. This study was an attempt to examine these questions. We retrospectively collected mortality, morbidity, and length and cost of hospitalization data of VLBW (BW < 1500 g) infants between March 1995 and February 1998. There were totally 162 patients enrolled. The overall mortality rate was 21.6%; the birth weight (BW)-specific mortality rate was 72%, 31%, 19%, and 3% for infants with BWs of < 750 g, 750-999 g, 1000-1249 g, and 1250-1499 g, respectively. The incidence of morbidities were: respiratory distress syndrome (74%), patent ductus arteriosus (36%), necrotizing enterocolitis (9%), sepsis (42%), intraventricular hemorrhage (15%), retinopathy of prematurity (31%), failure to pass auditory brainstem response (ABR) (34%), and chronic lung disease (17%). The average length of hospitalization was 67.2 days, and the cost per infant was 62 x 10(4) NT dollars; 108 +/- 38 days, 73 +/- 32 x 10(4) NT dollars if BW < 750 g; 94 +/- 15 days, 99 +/- 35 x 10(4) NT dollars if BW 750-999 g; 66 +/- 23 days, 64 +/- 36 x 10(4) NT dollars if BW 1000-1249 g; and 43 +/- 14 days, 39 +/- 37 x 10(4) NT dollars if BW 1250-1499 g. In conclusion, VLBW infants are associated with high mortality and morbidity rates. They have long lengths and high costs of hospitalization, and therefore deserve attention in the implementation of the National Health Insurance and regionalization.

Early dexamethasone therapy and blood cell count in preterm infants.

OBJECTIVE: To assess the effects of early postnatal dexamethasone therapy on hematologic values in preterm infants. MATERIALS AND METHODS: We reviewed the hematologic data of 179 preterm infants who participated in a double-blind clinical trial of early postnatal dexamethasone therapy (<12 hours after birth) for the prevention of chronic lung disease. One group (86 infants) received saline and the other group (93 infants) received dexamethasone. Dexamethasone was given intravenously every 12 hours in tapering doses: 0.25 mg/kg on days 1 to 7, 0.12 mg/kg on days 8 to 14, 0.05 mg/kg on days 15 to 21, and 0.02 mg/kg on days 21 to 28. Blood samples were obtained on days 0, 3, 7, 10, 14, 21, and 28. None of the infants received prenatal steroid therapy. RESULTS: Multiple regression analysis revealed significant differences in the values versus time curves of the white blood cell, neutrophil, lymphocyte, basophil, and eosinophil counts between the two groups. The white blood cell count was significantly higher in the dexamethasone group on days 7 through 14, and this was associated with significantly higher numbers of segmented neutrophils and band forms and significantly lower numbers of lymphocytes and eosinophils. The hematocrit and platelet counts were similar in the two groups throughout most of the trial. Except for platelet count, steroid therapy did not alter the hematologic values for infants with bacteremia. CONCLUSION: Dexamethasone affects white blood cell, segmented neutrophil, lymphocyte, basophil, and eosinophil counts in neonates. This should be taken into consideration when evaluating preterm infants who are receiving dexamethasone.early dexamethasone therapy; neonatal blood count; preterm infant; respiratory distress syndrome.

Neurologic complications in children with enterovirus 71 infection.

BACKGROUND: Enterovirus 71 infection causes hand-foot-and-mouth disease in young children, which is characterized by several days of fever and vomiting, ulcerative lesions in the oral mucosa, and vesicles on the backs of the hands and feet. The initial illness resolves but is sometimes followed by aseptic meningitis, encephalomyelitis, or even acute flaccid paralysis similar to paralytic poliomyelitis. METHODS: We describe the neurologic complications associated with the enterovirus 71 epidemic that occurred in Taiwan in 1998. At three major hospitals we identified 41 children with culture-confirmed enterovirus 71 infection and acute neurologic manifestations. Magnetic resonance imaging (MRI) was performed in 4 patients with acute flaccid paralysis and 24 with rhombencephalitis. RESULTS: The mean age of the patients was 2.5 years (range, 3 months to 8.2 years). Twenty-eight patients had hand-foot-and-mouth disease (68 percent), and 6 had herpangina (15 percent). The other seven patients had no skin or mucosal lesions. Three neurologic syndromes were identified: aseptic meningitis (in 3 patients); brain-stem encephalitis, or rhombencephalitis (in 37); and acute flaccid paralysis (in 4), which followed rhombencephalitis in 3 patients. In 20 patients with rhombencephalitis, the syndrome was characterized by myoclonic jerks and tremor, ataxia, or both (grade I disease). Ten patients had myoclonus and cranial-nerve involvement (grade II disease). In seven patients the brain-stem infection produced transient myoclonus followed by the rapid onset of respiratory distress, cyanosis, poor peripheral perfusion, shock, coma, loss of the doll's eye reflex, and apnea (grade III disease); five of these patients died within 12 hours after admission. In 17 of the 24 patients with rhombencephalitis who underwent MRI, T2-weighted scans showed high-intensity lesions in the brain stem, most commonly in the pontine tegmentum. At follow-up, two of the patients with acute flaccid paralysis had residual limb weakness, and five of the patients with rhombencephalitis had persistent neurologic deficits, including myoclonus (in one child), cranial-nerve deficits (in two), and ventilator-dependent apnea (in two). CONCLUSIONS: In the 1998 enterovirus 71 epidemic in Taiwan, the chief neurologic complication was rhombencephalitis, which had a fatality rate of 14 percent. The most common initial symptoms were myoclonic jerks, and MRI usually showed evidence of brainstem involvement.

Clinical spectrum of enterovirus 71 infection in children in southern Taiwan, with an emphasis on neurological complications.

An outbreak of enterovirus 71 (EV71) infection occurred in Taiwan in 1998. The clinical spectrums and laboratory findings for 97 patients with virus culture-proven EV71 infections were analyzed. Eighty-seven percent of the patients were younger than age 5 years. Hand-foot-and-mouth syndrome occurred in 79% of the children and central nervous system (CNS) involvement in 35%, including nine fatal cases. The predominant neurological presentations were myoclonus (68%), vomiting (53%), and ataxia (35%). Brain stem encephalitis was the cardinal feature of EV71 CNS involvement during this outbreak. Magnetic resonance imaging and pathological findings illustrated that the midbrain, pons, and medulla were the target areas. EV71 brain stem encephalitis can present either with cerebellar signs and an initially mild, reversible course or with overwhelming neurogenic shock and neurogenic pulmonary edema (NPE) resulting in a fatal outcome. Brain stem encephalitis that progressed abruptly to neurogenic shock and NPE was indicative of poor prognosis in this epidemic. Early aggressive treatment and close monitoring of the neurological signs are mandatory to improve the chance of survival.

The role of pulmonary inflammation in the development of pulmonary hypertension in newborn with meconium aspiration syndrome (MAS).

1. There was no clear correlation between the tracheal aspirate cytokines and the elevation of pulmonary arterial pressure in newborn piglets with MAS. The use of dexamethasone significantly suppressed tracheal aspirate cytokines but did not significantly alter pulmonary arterial pressure. Dexamethasone significantly increased the cardiac stroke volume and blood pressure. 2. Early dexamethasone therapy (< 12 hrs) for one week in infants with MAS significantly improved pulmonary ventilation and facilitated weaning from mechanical ventilation. 3. The mechanisms for the improvement in cardiopulmonary status following early dexamethasone therapy in MAS remain unclear. An overall improvement in cardiac hemodynamics, along with a significant decrease in lung inflammation may be responsible for the improvement.

Prevention of chronic lung disease in preterm infants by early postnatal dexamethasone therapy.

Recent studies suggest that early dexamethasone therapy may lessen the pulmonary inflammation in preterm infants with respiratory distress syndrome (RDS). To investigate whether early (<12 hr) postnatal dexamethasone therapy would reduce the incidence of chronic lung disease (CLD), a randomized, double-blind, controlled trial was conducted in 40 infants (birth weights from 500 to 1,999 gm) who had severe RDS and required mechanical ventilation within 6 hr of birth. All infants received one dose of Survanta before they were randomly assigned to control (saline placebo) or dexamethasone-treated groups (0.5 mg/kg/d for 1 week, then tapered over 3 weeks). Sequential analysis was performed with the end point of assessment being the presence or absence of CLD on postnatal Day 28. Statistical significance favoring dexamethasone was reached when 12 consecutive pairs in which one infant had CLD and the other did not have CLD showed that ten pairs favored dexamethasone and two pairs favored control treatment. Among the survivors, 12/15 were extubated in the dexamethasone group and 9/16 in the control group at the end of study. Infants in the treated group had transient hyperglycemia and hypertension. There was no difference between the groups in mortality and in incidence of sepsis or intraventricular hemorrhage. We conclude that early postnatal dexamethasone therapy is potentially effective in the lessening of CLD in preterm infants. To substantiate our result, large randomized controlled trials are needed and warranted.

Basic fibroblast growth factor in experimental and clinical bacterial meningitis.

Basic fibroblast growth factor (bFGF), a neurotrophic factor in the CNS, is expressed at high levels in response to seizures or strokes. We examined the expression of bFGF during experimental bacterial meningitis and the levels of bFGF in the cerebrospinal fluid (CSF) of children with bacterial meningitis. For the experimental study, a mouse model of meningitis was established by intracranial injection of Streptococcus pneumoniae. Twenty-four hours after induced meningitis, the brains were sectioned and stained immunohistochemically for bFGF. Neutrophils and macrophages infiltrating the leptomeninges and the ventricles exhibited strong bFGF immunoreactivity. The neurons in the areas adjacent to the inflamed ventricles also showed enhanced bFGF expression. For the clinical study, we used an enzyme immunoassay to measure bFGF in CSF in 18 children with bacterial meningitis, 12 with aseptic meningitis, and 18 controls. The CSF levels of bFGF were twice as high in children with bacterial meningitis (medians 6.75-7.21 pg/mL) compared with those who had aseptic meningitis (2.9 pg/mL) or in control subjects (2.65 pg/mL, p < 0.0001, respectively). In patients with bacterial meningitis who survived, CSF bFGF decreased significantly after 24-50 h of antibiotic therapy (p < 0.0005). Patients who developed major sequelae or died had much higher levels of CSF bFGF than those without (134.9 pg/mL versus 7.38 pg/mL, p < 0.05). These findings of enhanced immunoreactivity of bFGF in experimental bacterial meningitis and an association of CSF levels of bFGF with disease severity in childhood bacterial meningitis suggest a biologic role for this neurotrophic factor in the pathophysiology of bacterial meningitis.

Efficacy of nasal intermittent positive pressure ventilation in treating apnea of prematurity.

The efficacy of nasal intermittent positive pressure ventilation (NIPPV) in treating apnea of prematurity was evaluated. Apneic preterm infants were randomly assigned to receive either NIPPV or continuous positive airway pressure (NCPAP) for 4 hr when they failed to respond to conservative therapy. The amount of reduction in apneic spells and bradycardia in the two groups after treatment was compared. Thirty-four infants (18 with NIPPV, 16 with NCPAP) were enrolled. Their birth weights ranged from 590-1,880 g (mean, 1,021 g) and gestational ages from 25-32 weeks (mean, 27.6 weeks). The baseline characteristics were comparable in the two groups. Frequency of apnea and bradycardia was reduced during both forms of treatments. However, the infants receiving NIPPV had a greater reduction of apneic spells (P = 0.02) and a tendency to greater decrease in bradycardia (P = 0.09) than those receiving NCPAP. We conclude that NIPPV is more effective than NCPAP in reducing apnea in preterm infants. NIPPV may reduce bradycardia; however, this needs to be validated by a larger number of observations.

Effects of early postnatal dexamethasone therapy on calcium homeostasis and bone growth in preterm infants with respiratory distress syndrome.

The effects of dexamethasone therapy on calcium homeostasis and bone growth were evaluated in 49 infants (24 placebo and 25 dexamethasone) who participated in a double-blind trial of early dexamethasone therapy for the prevention of chronic lung disease. Dexamethasone (0.25 mg kg(-1) b.i.d. on d 1-7; 0.12 mg kg(-1) b.i.d. on d 8-14; 0.05 mg kg(-1) b.i.d. on d 15-21; 0.02 mg kg(-1) b.i.d. on d 22-28) or saline placebo was given i.v. Serum calcium (Ca), phosphorus (P) and parathyroid hormone (PTH), and the corresponding urinary excretion of calcium (FECa) and phosphorus (FEP) were measured on d 2, 3, 7, 10, 14, 21 and 28 after starting the study. Radiographic evaluations of bone growth were also evaluated. Infants in the dexamethasone group had significantly higher PTH on d 2 (p < 0.01), 7 and 14 (p < 0.05) than infants in the placebo group. The dexamethasone-treated infants also had significantly higher FEP on d 2, 7 and 14 (p < 0.05) and lower FECa on d 7 and 14 (p < 0.05) than control infants. There was no significant difference between the groups in bone growth during the study. It was concluded that early dexamethasone therapy causes a transient elevation in PTH without apparent change in bone growth. The long-term effect remains to be evaluated further.

Early dexamethasone therapy in preterm infants: a follow-up study.

OBJECTIVES: To study the outcome at 2-year corrected age of infants who participated in a double-blind controlled trial of early (<12 hours) dexamethasone therapy for the prevention of chronic lung disease (CLD). METHODS AND MATERIALS: A total of 133 children (70 in the control group, 63 in the dexamethasone-treated group) who survived the initial study period and lived to 2 years of age were studied. All infants had birth weights of 500 to 1999 g and had severe respiratory distress syndrome requiring mechanical ventilation within 6 hours after birth. For infants in the treatment group, dexamethasone was started at a mean age of 8.1 hours and given 0.25 mg/kg every 12 hours for 1 week and then tapered off gradually over a 3-week period. The following variables were evaluated: interim medical history, socioeconomic background, physical growth, neurologic examinations, mental and psychomotor development index score (MDI and PDI), pulmonary function, electroencephalogram, and auditory and visual evoked potential. RESULTS: Infants in the control group tended to have a higher incidence of upper respiratory infection and rehospitalization than did the dexamethasone-treated group because of respiratory problems. Although there was no difference between the groups in somatic growth in girls, the dexamethasone-treated boys had significantly lower body weight and shorter height than the control boys (10.7 +/- 3.0 vs 11.9 +/- 2.0 kg; 84.9 +/- 5.7 vs 87.5 +/- 4.8 cm). The dexamethasone-treated group had a significantly higher incidence of neuromotor dysfunction (25/63 vs 12/70) than did the control group. The dexamethasone-treated infants also had a lower PDI score (79 +/- 26) than did the control group (87 +/- 23), but the difference was not statistically significant. Both groups were comparable in MDI, incidence of vision impairment, and auditory and visual evoked potential. Significant handicap, defined as severe neurologic defect and/or intellectual defect (MDI and/or PDI

Blunted serum erythropoietin response to anemia in patients polytransfused for beta-thalassemia major.

PURPOSE: To investigate the response of erythropoietin (EPO) to anemia in patients polytransfused for beta-thalassemia major. PATIENTS AND METHODS: We measured the serum EPO levels and the concurrent hemoglobin (Hb) concentrations in 40 patients polytransfused for beta-thalassemia major, in 18 patients with iron deficiency anemia (IDA), and 32 healthy subjects. Serum EPO levels were assayed by an enzyme immunoassay. RESULTS: In both groups with beta-thalassemia major and IDA, serum EPO levels were significantly elevated (114 +/- 71 and 239 +/- 217 mU/mL, respectively). There was a significant inverse correlation between log EPO values and Hb concentrations in patients with beta-thalassemia major (r = 0.61; p < 0.01) and IDA (r = 0.81; p < 0.01). In a semilogarithmic plot, the slope of the regression line obtained in patients with beta-thalassemia major was significantly lower than that of IDA (p < 0.01), suggesting a blunted EPO response to anemia in patients polytransfused for beta-thalassemia major. The elevation of serum EPO in patients with beta-thalassemia major was poorly related to clinical variables except serum ferritin. CONCLUSIONS: We conclude that a significant inverse relationship between serum EPO levels and Hb concentration exists in patients with beta-thalassemia major. However, this EPO response in patients with anemia caused by beta-thalassemia major may be blunted when compared to patients with IDA.