Prevalence of Glucose-6-Phosphate Dehydrogenase Deficiency in Male Newborn Infants and Its Relationship with Neonatal Jaundice in Thailand.

OBJECTIVE: The study aimed to explore the prevalence of glucose-6-phosphate dehydrogenase (G-6-PD) deficiency among male newborn infants in northeastern Thailand and its relationship with neonatal jaundice (NJ). STUDY DESIGN: This prospective cohort study included male newborn infants with gestational age (GA) ≥35 weeks born between July 1, 2019, and March 1, 2021. Cord blood was sent for G-6-PD fluorescent spot test (FST) and results were reported as normal, partial, or complete deficiency. Infants with NJ would have blood tested for total serum bilirubin (TSB) level and other possible causes of NJ. Duration of phototherapy, length of hospital stays, and complications were documented. RESULTS: There were 922 male infants included in this study with 854 (93.1%) term and 63 (6.9%) preterm infants. FST showed 132 infants (14.4%) had G-6-PD deficiency. Incidence of NJ was significantly higher among infants with G-6-PD deficiency compared with infants with normal G-6-PD level (47.7 vs. 25.8%; relative risk [RR]: 2.62, 95% confidence interval [CI]: 1.79-3.82; p < 0.001). Regardless of G-6-PD level, preterm infants had significantly higher incidence of NJ than term infants (52.4 vs. 27.3%; RR: 2.93, 95% CI: 1.75-4.92; p < 0.001). Duration of phototherapy was significantly longer in infants with G-6-PD deficiency with NJ but hospital stays were similar. Infants with combined G-6-PD deficiency and other causes of hemolysis did not have higher TSB level than infants with isolated G-6-PD deficiency. Risk factors associated with NJ were G-6-PD deficiency and preterm infants, whereas more advance GA was associated with reduced risk for NJ. CONCLUSION: G-6-PD deficiency and preterm infants were important risk factors for NJ. Routine G-6-PD screening, close monitoring for signs of NJ in infant with risks, and appropriate parental counseling should be implemented. KEY POINTS: · G-6-PD deficiency increases risk of neonatal jaundice.. · Preterm infants have higher risk for neonatal jaundice.. · G-6-PD deficiency does not link with severe jaundice..

Mortality and Comorbidities in Extremely Low Birth Weight Thai Infants: A Nationwide Data Analysis.

This is the first nationwide study aimed to evaluate in-hospital mortality and comorbidities of extremely low birth weight (ELBW) infants in Thailand between 2015-2020. Data of ELBW infants were collected from the National Health Coverage Scheme. The incidence of ELBW Thai infants was 1.75 per 1000 live births. Sixty-five percent of ELBW infants were delivered in tertiary-care facilities, with 63% surviving until discharge. In-hospital mortality was 36.9%. Non-invasive respiratory supports were documented in just 17.6% of the study population, whereas total parenteral nutrition was used in 52.3% of neonates. There were several comorbidities, with the three most frequent including respiratory distress syndrome (70.7%), neonatal jaundice (66.7%), and sepsis (60.4%). The median hospitalization cost for one ELBW infant who survived was 296,438.40 baht ($8719). Conclusion: Thailand had an acceptable ELBW infant survival rate (63%), but comorbidities remained particularly severe and cost one hundred times the median hospital cost for one ELBW infant that survived in comparison to a normal newborn infant. Better health outcomes require strategies to raise awareness of the issues and the appropriate implementation of evidence-based solutions, particularly improving neonatal care facilities, as well as early referral of high-risk pregnant women and neonates, which will aid in the future reduction of neonatal morbidities and mortalities.

Early-Onset Neonatal Sepsis and Antibiotic Use in Northeast Thailand.

OBJECTIVE: Antibiotics are commonly prescribed in neonatal intensive care units (NICUs) for suspected sepsis because of the nonspecific clinical symptoms of sepsis. The overuse of antibiotic is associated with adverse outcomes. This study aimed to determine the rate of early-onset sepsis (EOS) and antibiotic use in neonates admitted to three NICUs in Northeast Thailand STUDY DESIGN: This is a descriptive study using the data collected in the South East Asia-Using Research for Change in Hospital-acquired Infection in Neonates project. Neonates admitted within 3 days of life were included. EOS was defined as neonates who presented with three or more clinical signs or laboratory results suggested sepsis and received antibiotics for at least 5 days. Those with positive blood culture were culture-proven EOS. Antibiotic use within 3 days of life and up to 28 days was described. RESULTS: Among 1,897 neonates, 160 cases were classified as EOS (8.4%) with culture-proven EOS in 4 cases (0.2%). The median durations of antibiotic use in culture-proven and culture-negative EOSs were 15 and 8 days, respectively. CONCLUSION: The rate of culture-proven EOS was low, but there was a high rate of antibiotic use. Antibiotic stewardship should be emphasized.

Presentation of Compound Heterozygous Hemoglobin Constant Spring and Hemoglobin Pakse in Neonates.

BACKGROUND: Mutations causing α thalassemia are divided into deletion and nondeletion groups. In the nondeletion group, hemoglobin constant spring (Hb CS) and hemoglobin Pakse (Hb Pakse) are both caused by a termination codon mutation leading to elongation of the α2 globin gene. In the case of Hb CS, the mutation is TAA→CAA, whereas the mutation causing Hb Pakse is TAA→TAT. Clinical hematologic phenotypes are not significantly different. It is important to identify compound heterozygotes for purposes of genetic counseling. METHODS: We report 5 neonates with compound heterozygous Hb CS/Hb Pakse mutations with respect to clinical courses, hematologic profiles, and management. RESULTS: Among 5 cases (3 male babies and 2 female babies) with mean birth weight 2982 g (range, 2660 to 3440 g), 3 were diagnosed as compound heterozygous Hb CS/Hb Pakse, 1 as homozygous Hb E with compound heterozygous Hb CS/Hb Pakse, and 1 as heterozygous Hb E with compound heterozygous Hb CS/Hb Pakse. Clinical manifestations included fetal anemia (1 case), neonatal hyperbilirubinemia (5), neonatal anemia (2), hepatosplenomegaly (1), and cholestatic jaundice (1). Three cases required a single phototherapy; 2 cases needed double phototherapy for treatment of severe hyperbilirubinemia. During the first few months of life, all cases had mild anemia, slightly low mean corpuscular volume, wide red cell distribution width, and low red cell counts. At 1 to 3 years of age, all patients still had mild microcytic hypochromic anemia with Hb levels around 10 g/dL, increased reticulocyte count, and wide red cell distribution width. CONCLUSIONS: Misdiagnosis of Hb Pakse could occur via Hb typing using Hb electrophoresis, because the band comigrates with that of Hb CS. DNA study is the definitive method for diagnosis.

Clinical Course of Homozygous Hemoglobin Constant Spring in Pediatric Patients.

BACKGROUND: Hemoglobin (Hb) Constant Spring is an alpha-globin gene variant due to a mutation of the stop codon resulting in the elongation of the encoded polypeptide from 141 to 172 amino acid residues. Patients with homozygous Hb Constant Spring are usually mildly anemic. METHODS: We retrospectively describe clinical manifestations, diagnosis, laboratory investigations, treatment, and associated findings in pediatric patients with homozygous Hb Constant Spring followed-up at Srinagarind Hospital. RESULTS: Sixteen pediatric cases (5 males and 11 females) were diagnosed in utero (N=6) or postnatal (n=10). Eleven cases were diagnosed with homozygous Hb Constant Spring, 4 with homozygous Hb Constant Spring with heterozygous Hb E, and 1 with homozygous Hb Constant Spring with homozygous Hb E. Three cases were delivered preterm. Six patients had low birth weights. Clinical manifestations included fetal anemia in 6 cases, hepatomegaly in 1 case, hepatosplenomegaly in 2 cases, splenomegaly in 1 case. Twelve cases exhibited early neonatal jaundice, 9 of which required phototherapy. Six cases received red cell transfusions; 1 (3), >1 (3). After the first few months of life, almost all patients had mild microcytic hypochromic anemia and an increased reticulocyte count with a wide red cell distribution (RDW), but no longer required red cell transfusion. At 1 to 2 years of age, some patients still had mild microcytic hypochromic anemia and some had normocytic hypochromic anemia with Hb around 10 g/dL, increased reticulocyte count and wide RDW. Associated findings included hypothyroidism (2), congenital heart diseases (4), genitourinary abnormalities (3), gastrointestinal abnormalities (2), and developmental delay (1). SUMMARY: Pediatric patients with homozygous Hb Constant Spring developed severe anemia in utero and up to the age of 2 to 3 months postnatal, requiring blood transfusions. Subsequently, their anemia was mild with no evidence of hepatosplenomegaly. Their Hb level was above 9 g/dL with hypochromic microcytic blood pictures as well as wide RDW. Blood transfusions have not been necessary since then.

Fetal Anemia Causing Hydrops Fetalis From an Alpha-Globin Variant: Homozygous Hemoglobin Constant Spring.

BACKGROUND: Fetal anemia is often assumed to be due to red cell alloimmunization and Parvovirus infection, and can lead to hydrops fetalis and death in utero. Other causes, such as mutations of hemoglobin alpha, are less commonly considered. METHODS: We report 7 cases with fetal anemia causing hydrops fetalis. Serial Doppler ultrasound for measurement peak systolic velocity (PSV) of middle cerebral artery (MCA) was used for evaluation of fetal anemia. Fetal anemia is suggested if the MCA/PSV ratio is >1.5 multiple of median. Cordocentesis was performed subsequently to find the cause of fetal anemia and check fetal hemoglobin for consideration of intrauterine infusion. Investigations for fetal anemia include complete blood count, blood morphology, and blood group of mother and fetus, reticulocyte counts, red cell indices, screening for thalassemia, hemoglobin typing, acid elution test, parvovirus B 19 serology, and TORCH titer (toxoplasmosis, rubella, cytomegalovirus, herpes simplex virus, human immunodeficiency virus, and syphilis). Intrauterine infusion, using irradiated prestorage filtered red cell with hematocrit level of 80%, is indicated if fetal hemoglobin is <10 g/dL. RESULT: Seven cases with fetal anemia were prenatally diagnosed from gestational ages 20 to 34 weeks. Initial hematocrit in these cases varied from 9% to 17.2%. In each case, causes of anemia were determined using the investigations listed above. All cases underwent uneventfully up to 3 intrauterine transfusions. DNA study for thalassemia demonstrated homozygous Constant Spring (CS) in 5 cases, homozygous CS with heterozygous E in 1 case, and compound heterozygous CS and Pakse in 1 case. The perinatal outcomes were normal term in 5 cases, preterm in 2 cases. Low birth weight was determined in 2 cases. The screening for thalassemia major, including the osmotic fragility and dichlorophenol indophenol precipitation test (DCIP), is not helpful for detecting hemoglobin variants such as Constant Spring or Pakse. SUMMARY: This study emphasizes homozygous Constant Spring and compound heterozygous CS and Pakse as a cause of hydrops fetalis. Proper management for the fetus after diagnosis can lead to a good fetal outcome. Prevention control programs should include screening of parents for the heterozygous state.

Vitamin A supplementation for prevention of bronchopulmonary dysplasia in very-low-birth-weight premature Thai infants: a randomized trial.

BACKGROUND: Bronchopulmonary dysplasia (BPD) is one ofthe most significant complications among very-low-birth-weight (VLBW) premature infants. Vitamin A deficiency increases the risk of BPD in VLBWinfants. OBJECTIVE: To assess the effect of vitamin A supplementation for prevention of bronchopulmonary dysplasia in VLBW premature Thai infants. STUDY DESIGN: Randomized control trial. MATERIAL AND METHOD: Eighty premature infants weighing <1,500 g who received mechanical ventilation or oxygen supplementation at 24 hours ofage-admitted to Neonatal units ofSrinagarind Hospital, Khon Kaen University, Khon Kaen, Thailand-were assigned to receive either intramuscular vitaminA 5, 000 IU3 times/week (treatment group) or sham procedure (control group) for four weeks. Serum vitamin A levels were measured before and after administration of the vitamin A. RESULTS: The baseline of mean serum vitamin A levels were similar in both groups. The mean serum level of vitamin A was significantly higher in the vitamin A supplemented infants than in the control infants on day 7 (1.41 +/- 0.48 vs. 0.92+0.38 pmol/ L, p<0.001), day 14 (1.48 +/- 0.90 vs. 0.96 +/- 0.36 micromol/L, p = 0.001) and day 28 (1.42 +/- 0.63 vs. 0.76 +/- 0.30 micromol/L, p<0.001) after vitamin A supplementation. None of the infants in the vitamin A supplemented group, compared to 5% of the infants in the control group, had vitamin A level <0.35 micromol/L, (indicating severe vitamin A deficiency) at 28 days. Fewer of the premature infants in the vitamin A supplemented group required oxygen supplementation at 36 weeks postmenstrual age than in the control group albeit not statistically significant (22.5 vs. 35% relative risk 0.71; 95% CI 0.40 +/- 1.26; p = 0.21). Supplementation with vitamin A was also associated with a significant reduction in the duration ofintubation (10.8 +/- 3.1 days vitamin A supplemented group vs. 26.1 +/- 6.4 days control group, p = 0.03), days on oxygen therapy (29.8 +/- 5.1 days vitamin A supplemented group vs. 58.2 +/- 9.1 days control group, p = 0.01) and length of hospital stay (61.9 +/- 4.2 days vitamin A supplemented group vs. 88.3 +/- 7.2 days control group, p = 0.002). CONCLUSION: The dose of vitamin A used in this study reduced biochemical evidence of vitamin A deficiency and, without complications, resulted in reducing duration of intubation, days of oxygen therapy, and length of hospital stay in premature infants suffering VLBW

Neonatal morbidity and mortality for repeated cesarean section vs. normal vaginal delivery to uncomplicated term pregnancies at Srinagarind Hospital.

OBJECTIVE: To evaluate the morbidities and mortality of neonates delivered by elective repeated cesarean section vs. normal vaginal delivery among women with uncomplicated term pregnancies. MATERIAL AND METHOD: A retrospective descriptive study was done between January 2009 and December 2011 to determine the morbidities and mortality among uncomplicated term pregnancies at Srinagarind Hospital. Three hundred seventy two neonates delivered by elective repeated cesarean section vs. 1,581 by normal vaginal delivery. RESULTS: A significantly greater number of neonates in the elective repeated cesarean section group required oxygen for neonatal resuscitation compared to neonates in the normal vaginal delivery group (37.6% vs. 20.9%, p < 0.001). Neonates delivered by elective repeated cesarean section were more frequently admitted to the neonatal intensive care unit (1.1% vs. 0%, p < 0.001) and had longer hospital stays (4.56 +/- 2.45 vs. 4.07 +/- 1.44 days, p < 0.001). The latter not only had a higher rate of respiratory distress syndrome (0.8% vs. 0%, p < 0.001) and transient tachypnea of the newborn (3.2% vs. 0.3%, p < 0.001), which required more respiratory support, they also had a higher rate of infection (2.4% vs. 0.8%, p < 0.05) than neonates delivered by normal vaginal delivery. Neonates born by normal vaginal delivery, however had more birth trauma and hyperbilirubinemia than neonates born by elective repeated cesarean section (8.8% vs. 2.4%, p < 0.001 and 31.8% vs. 22.6%, p < 0.05, respectively). There was no difference in the mortality rate between the groups. CONCLUSION: Even among uncomplicated term pregnancies, cesarean section is associated with more neonatal respiratory morbidity and sepsis while those delivered by normal vaginal delivery tend to have a higher rate of birth trauma and hyperbilirubinemia. Clinicians should therefore be concerned about the route of delivery and the probability of negative neonatal outcomes.

Current challenges in reducing neonatal morbidity and mortality in Thailand.

BACKGROUND: Information on neonatal mortality and burden of illness during the neonatal period is an essential guide for prioritizing interventions for solving health problems and allocating resources. OBJECTIVE: To evaluate the burden of diseases and the current health situation among Thai neonates under the Universal Health Insurance Coverage Scheme. MATERIALS AND METHOD: The number of admissions according to mortality, length of hospital stay and cost of hospital charges during the neonatal period was analyzed. RESULTS: There were 638,795 live births according to the data extracted from the three healthcare schemes supporting universal healthcare' in Thailand, which is lower than the data from the Health Information Unit of the Bureau of Health Policy and Strategy at the Ministry of Public Health. The neonatal death rate was 3.98 per 1,000 live births comprising 58.9% of all infant deaths. Major proportion of neonatal deaths (700%) occurred in early neonatal period and 43% of which occurred within the first two days of life. The leading causes of neonatal deaths were prematurity, respiratory problems, congenital malformation, birth asphyxia and infection. The most prevalent diagnosis for admissions was neonatal jaundice, disorders related to short gestation, respiratory disorders and neonatal infection. CONCLUSION: More investment is required to improve education and implement health interventions that can be integrated into existing health systems for better neonatal outcomes.

Outcome of neonates exposed to active pulmonary tuberculosis.

OBJECTIVE: To determine the incidence of tuberculosis infection and disease in neonates exposed to an active pulmonary tuberculosis patient in a nursery and maternity ward. MATERIAL AND METHOD: Descriptive cohort study was carried out in Srinagarind Hospital, Khon Kaen University, North-East Thailand. A smear positive pulmonary tuberculosis mother with productive cough was diagnosed on the fifth day of admission. The authors urged parents of all exposed neonates to accept isoniazid (INH) prophylaxis for their infants for six months. All neonates underwent chest x ray (AP, lateral view) and tuberculin skin test on the 24 months follow-up. RESULTS: The 48 neonates were identified as exposed. The age of follow-up ranged from 30 to 32 months. Only three were lost to follow-up. Of the remaining 45 neonates, six refused to take INH prophylaxis. Complete six months of LNH prophylaxis were observed in 27 (60%) of 39 contacts. Tuberculin skin tests (TST) were performed in all of 45 contacts. No cases were positive for TST. Abnormal chest radiographies were found in nine of INH group, three patients had hilar lymphadenopathy and six had pneumonia. The repeat chest x ray, two weeks later was normal in all cases. After 30 to 32 months follow-up, none of the 39 neonates who received INH prophylaxis or the six neonates progressed to have active tuberculosis. CONCLUSION: In exposed neonate identified as the high-risk group, appropriate INH prophylaxis, and long-term follow-up, especially in the first-2 years, seemed to be effective in preventing the development of active tuberculosis.

Incidence and risk factors associated with hearing loss in high-risk neonates in Srinagarind Hospital.

OBJECTIVE: To determine incidence and risk factors associated with hearing loss in high-risk neonates in Srinagarind Hospital. STUDY DESIGN: Prospective cohort study. MATERIAL AND METHOD: High-risk neonates were screened with TEOAE/AABR. All infants were followed for hearing and developmental evaluation until one year of age. RESULTS: Four hundred twenty five neonates underwent hearing screening tests. Nine infants (2.1%) had abnormal TEOAE, AABR and ABR tests. During follow-up, two of the nine infants that had abnormal initial hearing tests were found to have normal hearing at eight and nine months of age. Therefore, seven high-risk neonates (1.6%) had permanent hearing loss. Significant risk factors for permanent hearing loss were craniofacial anomalies, very low birth weight, low Apgar scores at 5 minute and mechanical ventilation usage for more than five days. CONCLUSION: Continuing evaluation of hearing and development during follow-up is important in children with abnormal hearing tests. Invasive procedures as early intervention during the first six months of life should be considered with caution because some premature infants can have false positive tests or transient hearing loss and subsequently have normal hearing and development.

Perinatal lethal Gaucher disease.

Perinatal Lethal Gaucher Disease (PLGD) is a rare form of Gaucher disease and is often considered a distinct form of type 2 Gaucher disease. The authors report on an infant who presented with progressive hepatosplenomegaly, ichthyosis, generalized skin edema and neonatal encephalopathy and died at 6 h of age. Autopsy revealed massive hepatosplenomegaly, ichthyosis, a diffuse collodion picture and histological evidence of infiltration by Gaucher cells in the liver, spleen, lung, thymus, lymph node and bone marrow. Genetic testing of the parents revealed both to be carriers of Gaucher disease.

Neonatal ventricular tachyarrhythmias in medium chain acyl-CoA dehydrogenase deficiency.

We describe an unusually severe case of medium chain acyl-CoA dehydrogenase (MCAD) deficiency in a term female neonate, who presented at 12 h of age with lethargy, poor feeding, hypoglycemia and ventricular tachyarrhythmias. While arrhythmias are common in other disorders of fatty acid beta-oxidation, ventricular tachyarrhythmias have rarely been reported with MCAD deficiency in childhood. Since the results of newborn metabolic screening are usually not available within the first 3 days of life, our case highlights the need for health care professionals to be made aware of this early and uncommon but potentially fatal presentation of MCAD deficiency.

Hydrops fetalis caused by parvovirus B19 infection: case report and literature review.

An intrauterine parvovirus B19 infection can result in severe fetal anemia and hydrops fetalis, which can lead to death. A case of fetal hydrops, diagnosed at 31 weeks gestation, is reported Cordocentesis revealed fetal hemoglobin of 5 g/dL. Due to fetal distress 18 hours later, the baby was delivered by emergency cesarean section and died two days later. Characteristic intra-nuclear inclusions in nucleated red blood cells were found in histopathological examinations of the liver and placenta, which supported the diagnosis of parvovirus B19 infection. Literatures about parvovirus B19 infection, especially intrauterine infection, its effects on the fetus, methods of diagnosis and management, were reviewed.

Once-daily gentamicin dosing of 4 Mg/Kg/dose in neonates.

Since gentamicin is one of the most commonly prescribed antibiotics for culture-proven or suspected sepsis in neonates, interest has increased in refining dosing regimens for improved efficacy and decreased toxicity. Usually, 2.5 mg gentamicin/kg is infused twice daily, but its large volume of distribution, slow renal clearance and concentration-dependent character, suggests longer dosing intervals would be more appropriate. From a previous study, 22% of neonates who received a once-daily gentamicin dosage of 5 mg/kg/day had unacceptably high trough levels (i.e. > 2 microg/mL). The authors studied 105 neonates (of > or = 34 wk gestational age or > or = 2, 000 g body weight) admitted to the Neonatal Unit, Srinagarind Hospital, Khon Kaen University; at risk, or with clinical features of sepsis, receiving a once-daily gentamicin dosing of 4 mg/kg intravenously. Peak (i.e. efficacy) and trough (i.e. toxicity) serum gentamicin concentrations were collected on day 3 of therapy. On days 1 and 3, nephrotoxicity was evaluated from serum creatinine and ototoxicity by a hearing test. Neonates treated with 4 mg gentamicin/kg once-daily had a mean steady-state peak vs trough concentration of 7.33 (+/- 2.77) vs 0.99 (+/- 0.57) microg/mL, respectively. The peak serum concentration achieved a therapeutic level > 4 microg/mL in 102 neonates (97%), while 7 (6.67%) had an undesirable trough level (viz. > 2 microg/mL); notwithstanding, no nephrotoxic or ototoxic effects were identified. Gentamicin once-daily at 4 mg/kg/dose in neonates at > or = 34 wk gestation achieved appropriate trough levels. the regimen was convenient and did not increase renal or ototoxicity.

Once versus twice daily dose of gentamicin therapy in Thai neonates.

OBJECTIVE: To compare the peak and trough gentamicin concentrations in neonates after a once (ODD) vs. twice daily dosing (TDD) to establish the appropriate dosage for Thai neonates. MATERIAL AND METHOD: Neonates of gestational age > or = 34 weeks, or body weight > or = 2000 g, suspected of having bacterial infection were randomized to receive gentamicin intravenously, either 5 mg/kg every 24 hours or 2.5 mg/kg every 12 hours. The peak and trough serum gentamicin levels (SGLs) were measured. Serum creatinine cued nephrotoxicity. RESULTS: Neonates were evaluated and baseline characteristics between the groups were compared. The ODD and TDD group had a mean gentamicin peak and trough concentration of 10.1 +/- 3.0 vs. 7.8 +/- 2.0 microg/ml (p < 0.05) and 1.6 +/- 1.1 vs. 2.6 +/- 1.2 microg/ml (p < 0.05), respectively. The peak SGL of > or = 4 microg/ml was achieved in 100% vs. 96% of the ODD vs. TDD group, respectively. SGL troughs > or = 2 microg/ml were detected less often in the ODD group (22% vs. 68%, p < 0.05). Abnormal change in serum creatinine was not observed in either group. CONCLUSION: A once daily dose of gentamicin 5 mg/kg achieved a significantly higher peak SGL and safer trough than a twice-daily dose of 2.5 mg/kg albeit about a quarter of the ODD group had high troughs. A single daily dose of gentamicin 3.5-4 mg/kg/d in Thai neonates should be tested.

Tracheal agenesis: a case report.

Tracheal agenesis is a rare congenital anomaly and typically has fatal consequences. Associated congenital malformations are present in 90 per cent of cases, most frequently affecting the cardiovascular or gastrointestinal systems and the genitourinary tract. Affected infants lack prenatal symptoms and usually present with severe respiratory distress, absence of audible crying and difficult or impossible endotracheal intubation, leading to failed airway management and irreversible cerebral hypoxia. The authors report an infant with tracheal agenesis who presented with respiratory failure after birth. The clinical features, embryology and classification schemes are presented in the hope of increasing awareness, thus making earlier diagnosis possible and thereby improving survival.

Newborn screening for congenital hypothyroidism in Khon Kaen University Hospital, the first three years, a preliminary report.

Between January 2000 and December 2002, 9,558 of 10,868 live births at Srinagarind Hospital, Khon Kaen University, Khon Kaen, Thailand, were screened for congenital hypothyroidism (CH). Dried blood spot thyroid stimulating hormone (TSH) was collected at age 48 hours or older. The cut-off TSH level for recall test was > 25 mu/L. Serum thyroxine (T4), Free T4 and TSH were performed during the confirmatory test. Six of 24 infants recalled for confirmatory thyroid function tests had abnormal tests. Primary CH was confirmed in 3 infants and thyroxine treatment was given. Two of the three infants had thyroid dysgenesis, one had normal thyroid gland. Three infants showed borderline CH from the confirmatory test, only one had borderline CH from the second confirmatory test and also received thyroxine treatment. Twenty infants with false positives during the screening and confirmatory tests were regularly followed-up for growth, development and thyroid function tests. The incidence of primary CH in this sole tertiary care government hospital in Northeast Thailand was 1:3,186. Routine newborn CH screening would ensure early detection and treatment.