The Clinical Characteristics and the Neurodevelopmental Outcomes of the Neonates with Cerebellar Hemorrhage

PURPOSE: We present our experience of 14 neonates with cerebellar hemorrhage and describe the clinical characteristics and the neurodevelopmental outcomes. METHODS: Fourteen neonates were identified as having cerebellar hemorrhage, based on the brain MRI findings at the two university hospitals from January 2007 to July 2011. Twelve preterm infants with the brain MRI taken before the discharge, and 2 term infants having taken the brain MRI with birth asphyxia were enrolled. The electronic medical records of the infants were reviewed. RESULTS: Ten (71.4%) infants were found to have multifocal or lobar cerebellar hemorrhage involving both hemispheres. Three infants had unilateral lesions in the right hemisphere, and one infant had a left hemisphere lesion. Six infants (42%) had co-existing high grade IVH (> or =grade III); 6 infants (42%) had periventricular leukomalacia (PVL); and 6 infants (42%) had posthemorrhagic hydrocephalus (PHH). After discharge, 12 infants (85.7%) were followed at the outpatient clinics for at least 6 months. Two out of 9 infants (22.2%) with other neurological co-morbidities (IVH> or =grade III, PVL, or PHH) had cerebral palsy, and 5 infants (55.6%) had developmental delay assessed by the Bayley scales. None of the infants without other neurological co-morbidities had cerebral palsy or developmental delay. CONCLUSION: Neonatal cerebellar hemorrhage is often diagnosed incidentally on the brain MRI in the preterm infants. If there is no concomitant high-grade IVH, PVL or PHH, cerebellar hemorrhage seems to hardly accompany its own specific symptoms, and the neurodevelopmental outcomes seem to be favorable as well. However, further long-term, large scale studies are necessary.

The Time When the Metabolic Compensation for Hypercapnia Begin to Occur in Very Low Birth Weight Infants

PURPOSE: Lung injury from mechanical ventilation is one of the major pathogenetic factors of bronchopulmonary dysplasia. Permissive hypercapnia (PH) is one of the strategies for reducing lung injury. However, PH is frequently infeasible in very low birth weight infants (VLBWI) due to their immature renal compensation for respiratory acidosis. The purpose of this study was to identify time when metabolic compensation for hypercapnia begin to occur in VLBWIs. METHODS: Data were retrospectively collected from 82 VLBWI who were admitted to Seoul National University Bundang Hospital from January 2011 to December 2012. The postnatal day when the difference between actual bicarbonate and expected bicarbonate levels became less than 2.0 mmol/L consistently for the first time under hypercapnea (>40 mmHg) was defined as the time when metabolic compensation for hypercapnea occurred. RESULTS: Metabolic compensation for hypercapnea occurred on 9.1+/-3.9 postnatal day. The younger the gestational age (GA) was and the smaller the birth weight was, the later metabolic compensation for hypercapnea occurred. Late metabolic compensators (> or =9 days) were significantly younger in GA (P=0.001), lighter at birth (P=0.041), intubated longer (P=0.002), and less frequently afflicted with respiratory distress syndrome (P=0.036) compared to early metabolic compensators (<9 days). However, logistic regression analysis revealed only young GA was associated with late metabolic compensation with marginal significance (P=0.068). CONCLUSION: Metabolic compensation for hypercapnea occurred 9 days after birth on average. PH strategy for reducing lung injury should be considered after renal metabolic compensation for hypercapnea occurs in VLBWI.

Comparison of the Incidences of Neonatal Morbidities by Different Criteria of Histologic Chorioamnionitis in Extremely Low Gestational Age Newborns

PURPOSE: The objective of the study was to compare the incidences of the neonatal morbidity, using the various criteria of histologic chorioamnionitis (HC) in the extremely low gestational age newborns (ELGANs), who were born before the 28 weeks of gestational age. METHODS: Sixty two ELGANs who were born and admitted to the Seoul National University Bundang Hospital from 2007 to 2011 were included. A total of seven criteria of HC were made according to the site within the placenta and the extent of the neutrophil infiltration. The incidence of the major neonatal morbiditiy was compared between the HC-exposed and the HC-unexposed ELGANs according to each criterion of HC. RESULTS: There were no significant differences in the birth weight, gestational age, and sex between the HC-exposed and the HC-unexposed ELGANs by any criteria. The incidence of respiratory distress syndrome (RDS) was significantly lower in the HC-exposed ELGANs only in the criterion of any extent of the umbilical cord inflammation (funisitis). The incidence of retinopathy of prematurity (ROP) was significantly higher in the HC-exposed ELGANs in the criterion of any extent of amnionitis or funisitis. The incidences of other major morbidities did not differ between the HC-exposed and the HC-unexposed ELGANs by any criteria. CONCLUSION: The presence of funisitis reflected the lung maturation effect of chorioamnionitis, as indicated by the decreased incidence of RDS. Among other major neonatal morbidities, only ROP was significantly associated with HC. The ROP occurred more frequently in the funisitis or amnionitis-exposed ELGANs.

Neonatal Mortality and Morbidity of Twin-Twin Transfusion Syndrome

PURPOSE: This study was aimed to evaluate the neonatal mortality and morbidity of infants affected by twin-twin transfusion syndrome (TTTS) compared to the control twins matched for gestational age. Also the perinatal outcomes of donor parts of TTTS twins with their counterpart recipients were compared. METHODS: A retrospective case-control study was conducted from infants born at Seoul National University Children's Hospital and Seoul National University Bundang Hospital between April 2005 and July 2011. Eighteen pairs of TTTS infants were allocated to the TTTS group. The control group consisted of 36 pairs of twin infants unaffected by TTTS who were matched for gestational age. Neonatal deaths and morbidities were recorded. RESULTS: The mortality in TTTS group was significantly higher than control group (27.8% vs. 4.2%, P=0.001). The incidence of acute renal failure (41.2% vs. 9.7%, P or =2 intraventricular hemorrhage (33.3% vs. 11.1%, P=0.012) and grade > or =2 periventricular leukomalacia (24.2% vs. 2.8%, P=0.001) were significantly higher in TTTS group than control group. There was no significant difference in mortality and morbidities between donors and recipients except significantly higher incidence of acute renal failure in donors (70.6% vs. 11.8%, P=0.001). CONCLUSION: Twin infants affected by TTTS have higher risk of neonatal death and several severe morbidities. These results indicate that alert monitoring and checking about possible morbidities are very important in newborns with TTTS and early intervention is critical for improving the overall outcome of the affected infants.

A case of mucolipidosis II presenting with prenatal skeletal dysplasia and severe secondary hyperparathyroidism at birth / 소아과

Mucolipidosis II (ML II) or inclusion cell disease (I-cell disease) is a rarely occurring autosomal recessive lysosomal enzyme-targeting disease. This disease is usually found to occur in individuals aged between 6 and 12 months, with a clinical phenotype resembling that of Hurler syndrome and radiological findings resembling those of dysostosis multiplex. However, we encountered a rare case of an infant with ML II who presented with prenatal skeletal dysplasia and typical clinical features of severe secondary hyperparathyroidism at birth. A female infant was born at 37(+1) weeks of gestation with a birth weight of 1,690 g (T (p.Arg1031X) and c.3456_3459dupCAAC (p.Ile1154GlnfsX3), the latter being a novel mutation. The infant was treated with vitamin D supplements but expired because of asphyxia at the age of 2 months.

The Significance of Maturation Score of Brain Magnetic Resonance Imaging in Extremely Low Birth Weight Infant

PURPOSE: The aim of this study was to investigate the effect of perinatal risk factors on brain maturation and the relationship of brain maturation and neurodevelopmental outcomes with brain maturation scoring system in brain MRI. METHODS: ELBWI infants born at the Seoul National University Children's Hospital from January 2006 to December 2010 were included. A retrospective analysis was performed with their medical record and brain MR images acquired at near full term. We read brain MRI and measured maturity with total maturation score (TMS). TMS is a previously developed anatomic scoring system to assess brain maturity. The total maturation score was used to evaluate the four parameters of maturity: (1) myelination, (2) cortical infolding, (3) involution of glial cell migration bands, and (4) presence of germinal matrix tissue. RESULTS: Images from 124 infants were evaluated. Their mean gestational age at birth was 27.1+/-2.1 weeks, and mean birth weight was 781.5+/-143.9 g. The mean TMS was 10.8+/-2.0. TMS was significantly related to the postmenstrual age (PMA) of the infant, increasing with advancing postmenstrual age (P<0.001). TMS showed no significance with neurodevelopmental delay, and with brain injury, respectively. CONCLUSION: TMS was developed for evaluating brain maturation in conventional brain MRI. The results of this study suggest that TMS was not useful for predicting neurodevelopmental delay, but further studies are needed to make standard score for each PMA and to re-evaluate the relationship between brain maturation and neurodevelopmental delay.

Diagnosis of Functional Gastrointestinal Disorders with Rome III Criteria in Korean Pediatric and Adolescent Patients: Clinical Usefulness of QPGS / 대한소아소화기영양학회지

PURPOSE: We diagnosed pediatric functional gastrointestinal disorders in Korean children and adolescents using Rome III criteria and investigated the clinical validity of QPGS-Rome III. METHODS: Diagnosis based on QPGS was compared with the physician's diagnosis based on Rome III criteria. One hundred and thirty eight children and their parents completed the QPGS. Agreement rates were measured using Kappa method. RESULTS: In physician's diagnoses, the most prevalent disorders were functional dyspepsia (39.1%), irritable bowel syndrome (38.4%), and functional abdominal pain (18.8%). Among QPGS based diagnoses, the most prevalent disorders were irritable bowel syndrome (39.1%), functional dyspepsia (29.7%), and functional abdominal pain (21.7%). The agreement rate was substantial (kappa=0.72, p=0.00). Diagnostic disagreements probably resulted from different patient responses to bowel movement form and bowel frequency. CONCLUSION: Functional dyspepsia, irritable bowel syndrome, and functional abdominal pain were the most common disorders by Rome III criteria in the Korean pediatric and adolescent patients. The agreement rate between physician's diagnoses and QPGS based diagnoses supported the validity of the QPGS-Rome III in Korean pediatric and adolescent patients. QPGS seems to be useful in diagnosis of patients with functional gastrointestinal disorders by Rome III criteria.

Eight cases of incidentally diagnosed as subclinical rickets / 소아과

PURPOSE: Vitamin D plays a key role in bone mineralization of the skeleton and vitamin D deficiency can lead to rickets. It is well known that vitamin D deficiency is common in breast fed infants. Of these patients, clinically, some have no signs of rickets, but laboratory and radiographic findings are diagnostic for vitamin D deficiency rickets (subclinical vitamin D deficiency rickets). The purpose of this study is to clarify current causes and ways to prevent this disease. METHODS: We reviewed the clinical and laboratory characteristics of children who were incidentally diagnosed as subclinical rickets during treatment of other disease such as pneumonia, gastroenteritis, urinary tract infection at Eulji Hospital, Seoul, Korea from March, 2003 to July 2007. RESULTS: Eight patients (six boys and two girls) were diagnosed with subclinical vitamin D deficiency rickets. The mean age of the patients was 12.6+/-5.8 months, and they were diagnosed from January to July. The associated diseases were pneumonia, urinary tract infection, acute gastroenteritis, and iron deficiency anemia. All patients were breast-fed. Two showed growth failure. The mean serum alkaline phosphatase was 1995.8+/-739.5 IU/L, the mean calcium count was 9.5+/-0.6 mg/dL, and the mean phosphorus content was 3.6+/-1.5 mg/dL mg/dL. The mean intact parathyroid hormone was 214.8+/-155.9 pg/mL (reference range, 9-65), the mean 1,25-dihydroxyvitamin D was 82.4+/-49.3 pg/mL (reference range, 2070), and the mean 25-hydroxyvitamin D was 29.6+/-10.6 ng/mL (reference range, 1030). A radiographic examination showed cupping, fraying, and flaring of metaphyses in all patients. Six patients were administered calcitriol (400 IU/day) for three months. A consequent radiographic and laboratory examination showed improvement. The first two patients were initially diagnosed with metaphyseal dysplasia, without the detection of vitamin D deficiency and they spontaneously improved without vitamin D supplements. However, two years later, they showed mild scoliosis and metaphyseal dysplasia, respectively. CONCLUSION: Breast-feeding without supplementation involves high risk of vitamin D deficiency. Some infants may also develop rickets; therefore, such groups should be considered for vitamin D supplementation.

A Case of Grayanotoxin Intoxication Presenting with Mental Changes and Vomiting / 대한소아소화기영양학회지

Rhododendron species is one of the largest and most diverse genera in the plant kingdom, comprising over 800 species and existing all over the globe. Grayanotoxin intoxication is caused by ingestion of honey and flowers. Grayanotoxin exists in honey, flowers, pollen, and nectar of the Rhododendron species. Grayanotoxin-intoxicated patients may present with nausea, vomiting, dizziness, weakness, hypotension, bradycardia, and syncope for several hours. We report a case of grayanotoxin intoxication associated with mental changes. A 9-year-old male presented with impaired consciousness and delirium 26 hours after eating about 10 rhododendron sclippenbachii flowers. A brain MRI and EEG were normal. Parenteral fluids were administered and these symptoms resolved completely in 17 hours.

A Case of Grayanotoxin Intoxication Presenting with Mental Changes and Vomiting / 대한소아소화기영양학회지

Rhododendron species is one of the largest and most diverse genera in the plant kingdom, comprising over 800 species and existing all over the globe. Grayanotoxin intoxication is caused by ingestion of honey and flowers. Grayanotoxin exists in honey, flowers, pollen, and nectar of the Rhododendron species. Grayanotoxin-intoxicated patients may present with nausea, vomiting, dizziness, weakness, hypotension, bradycardia, and syncope for several hours. We report a case of grayanotoxin intoxication associated with mental changes. A 9-year-old male presented with impaired consciousness and delirium 26 hours after eating about 10 rhododendron sclippenbachii flowers. A brain MRI and EEG were normal. Parenteral fluids were administered and these symptoms resolved completely in 17 hours.

A Case of Child with Obstructive Sleep Apnea Syndrome Recurred after Adenotonsillectomy / 수면정신생리

The most common cause of obstructive sleep apnea syndrome (OSAS) in childhood is adenotonsillar hypertrophy. Adenotonsillectomy improves the symptoms quite well in most cases. However, some patients could experience the OSAS again after adenotonsillectomy, who might have several risk factors such as incomplete operation, misdiagnosis, combined anatomical malformation, sinusitis or chronic allergic rhinitis, obesity, initial severe OSAS, and early onset OSAS. We report a case of 11-year-old obese boy who presented with snoring for several years. He was obese with body mass index (BMI) of 26.3 kg/m2 and also found to have fatty liver by ultrasonogram. Initial polysomnography (PSG) showed that he met the criteria of severe OSAS with the apnea-hypopnea index (AHI) of 70.5. He underwent adenotonsillectomy and symptoms improved immediately. Four months later symptoms were relieved with AHI of 0, but 1 year after the adenotonsillectomy he started to complain snoring again and the subsequent PSG results showed that OSAS has relapsed with AHI of 43. Paranasal sinus X-ray and physical examination showed sinusitis and re-growth of adenoid. Obesity was proved not to be a contributing factor because his BMI decreased to normal range (23.1 kg/m2) after diet control and regular exercise. Also, liver transaminase was normalized and fatty liver was disappeared on follow-up abdominal ultrasonogram. After treatment of sinusitis, symptoms were relieved with decreased AHI (8.5). This case suggests that simple adenotonsillectomy might not be the end of OSAS treatment in childhood. Patients who had adenotonsillectomy should be followed by subsequent PSG if symptoms recur. It is also important to be aware of risk factors in the recurrent OSAS for the proper intervention according to the cause.