Intrauterine Ureaplasma is associated with small airway obstruction in extremely preterm infants.

BACKGROUND: The long-term follow-up of lung function (LF) in extremely preterm (EP) infants with bronchopulmonary dysplasia (BPD) has shown a worldwide increase in small airway obstructions (SAO). OBJECTIVES: We investigated the relationships between intrauterine Ureplasma infection in EP infants and bubbly/cystic lung, BPD, and SAO at school age. METHODS: Placental pathology, placental Ureaplasma DNA (pU-DNA), and cord blood immunoglobulin M (IgM) (C-IgM) were investigated in 360 EP infants born from 1981 to 2004. Maternal amniotic inflammatory response (M-AIR) scores and hemosiderin deposition (HD) were estimated in the chorioamnion. The study subjects were divided into groups based on their M-AIR scores. Their LF at school age was compared with those of 33 healthy siblings. FINDINGS: pU-DNA and C-IgM were significantly related to SAO at school age (p < 0.012). M-AIR score 3 and pU-DNA >1000 units had an odds ratio (OR) of 35 (95% confidence interval: 10-172) and 18 (5.6-67) for bubbly/cystic lung, and 11 (3.1 - 43) and 31 (4.5-349) for severe BPD, and 5.3 (2.1-11) and 12 (2.4-74) for SAO, respectively. The ORs of surfactant treatment, BPD grade III, O2 at 40 weeks, HD, and C-IgM >30 mg/dl for SAO were 0.21 (0.075-0.58), 5.3 (2.1-15), 2.5 (1.4-4.6), 3.6 (1.5-9.1) and 2.5 (1.0-5.2). 84% (90/107) SAO infants showed no or mild BPD in infancy, and 61% of infants had no severe CAM. CONCLUSION: Our long-term cohort study of LF in EP infants revealed that intrauterine Ureaplasma was associated with bubbly/cystic lung, severe BPD, and SAO at school age.

Impact of prematurity and the CTG repeat length on outcomes in congenital myotonic dystrophy.

OBJECTIVE: Patients with congenital myotonic dystrophy (CDM) tend to be born preterm. Although the CDM severity generally depends on the CTG repeat length, prematurity may also affect the prognosis in patients with CDM. Given that preterm birth is expected to increase the risk of CDM in newborns, we investigated the outcomes of newborns with CDM according to gestational age to assess prematurity and the CTG repeat length for predicting prognosis. RESULTS: We assessed the outcomes of 54 infants with CDM using data collected from our hospitals and previously published studies. The patients were divided into mild and severe groups based on clinical outcomes. Logistic regression analysis was performed to estimate odds ratios (ORs) for CDM prognosis according to gestational age and the CTG repeat length and to construct a predictive model. Logistic regression analysis showed both the CTG repeat and gestational age were significantly associated with severe outcomes in patients with CDM (OR: 32.27, 95% CI 3.45-300.7; p = 0.002 and OR: 0.73, 95% CI 0.58-0.93; p = 0.0094, respectively). This predictive model for CDM prognosis exhibited good sensitivity (63%) and specificity (86%). Both prematurity and the CTG repeat length were significantly associated with the CDM severity.

[Suspected case of pseudohypoparathyroidism type II].

We experienced a suspected case of pseudohypoparathyroidism type II. The patient came to our emergency room with no thermal convulsion. The Ellsworth-Howard test was applied to the patient to determine the type of PHP.

Perinatal factors associated with long-term respiratory sequelae in extremely low birthweight infants.

OBJECTIVE: To assess lung function at 8 years old in extremely low birthweight (ELBW) survivors and to identify perinatal determinants associated with impaired lung function. DESIGN: Retrospective cohort study. SETTING: Level III neonatal intensive care unit. PATIENTS: ELBW survivors born in 1990-2004 with available spirometry at 8 years old were studied. Children were excluded if they had a Wechsler Intelligence Scale for Children Third Edition full IQ <70. MAIN OUTCOME MEASURES: Multivariate logistic regression analysis was used to identify perinatal determinants associated with airway obstruction (forced expiratory volume in 1 s (FEV1)/forced vital capacity (FVC) ratio <80%) at school age and the predictive power of potential determinants. Potential risk factors and predictors assessed in this study were gestational age, birth weight, small for gestational age, sex, chorioamnionitis, premature rupture of membranes, antenatal steroids, surfactant administration, respiratory distress syndrome, postnatal steroids, severe bronchopulmonary dysplasia and bubbly/cystic appearances of the lungs by X-ray during the neonatal period. RESULTS: Of 656 ELBW survivors, 301 (45.9%) had attended a school-age follow-up at 8 years old. A total of 201 eligible children completed the lung function test. Bubbly/cystic appearance of the lungs (OR 4.84, 95% CI 1.26 to 18.70) was associated with a low FEV1/FVC ratio. Children with bubbly/cystic appearance had characteristics of immaturity and intrauterine inflammation. CONCLUSIONS: Within a cohort of ELBW infants, a bubbly/cystic appearance of the lungs in the neonatal period was the strongest determinant of a low FEV1/FVC ratio at school age.

Inhaled nitric oxide therapy decreases the risk of cerebral palsy in preterm infants with persistent pulmonary hypertension of the newborn.

OBJECTIVE: The aim was to determine whether inhaled nitric oxide therapy improves neurodevelopmental outcomes for infants with preterm persistent pulmonary hypertension of the newborn. METHODS: We conducted a historical cohort study to compare the 3-year incidence of cerebral palsy in preterm singleton infants (< 34 gestational weeks) with hypoxemic respiratory failure caused by persistent pulmonary hypertension of the newborn who received inhaled nitric oxide (16 patients) or 100% oxygen (15 patients) therapy. All neonates had clinical and echocardiographic evidence of pulmonary hypertension without structural heart disease. RESULTS: The incidence of cerebral palsy among patients treated with inhaled nitric oxide was 12.5%, whereas that among patients treated with 100% oxygen was 46.7%. After adjustment for maternal fever (> or = 38 degrees C) during delivery, birth weight, Apgar score at 5 minutes, high-frequency oscillatory ventilation, and surfactant therapy, inhaled nitric oxide therapy, compared with 100% oxygen therapy, was associated with a decreased risk of cerebral palsy in preterm infants with persistent pulmonary hypertension of the newborn. CONCLUSION: Inhaled nitric oxide therapy decreases the risk of cerebral palsy in preterm infants with persistent pulmonary hypertension of the newborn.

A case of primary hyperparathyroidism in childhood found by a chance hematuria.

A 10-yr-old boy visited Minoh City Hospital complaining of gross hematuria. Laboratory investigations revealed hypercalcemia, hypophosphatemia, and elevated serum levels of parathyroid hormone. A stone was found in the right ureter with drip infusion pyelography. A parathyroid adenoma was successfully diagnosed with computed tomography, ultrasonography, and methoxy-2-isobutyl isonitrile (MIBI) scintigraphy. Multiple endocrine neoplasia was ruled out by normal results of endocrine laboratory examinations. Extracorporeal shock wave lithotripsy was performed to treat the urolithiasis, and the parathyroid adenoma was surgically removed. Primary hyperparathyroidism is rare in childhood; however, this case suggests that gross hematuria is an important sign of hyperparathyroidism.