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Neuromyelitis optica(NMO) or Devic's syndrome is an uncommon clinical syndrome associating with unilateral or bilateral optic neuritis and transverse myelitis. It is rarely found in children and usually reported in adults with serious neurologic manifestations. We report a case of an 8-year-old girl with neuromyelitis optica whose first clinical manifestation was acute visual loss of both eyes. Initially the patient had been diagnosed with central retinal artery occlusion and optic neuritis by ophthalmologic examination, a brain magnetic resonance imaging, and cerebrospinal fluid findings. She was treated with intravenous methylprednisolone pulse therapy and heparinization. Then the treatments were replaced with oral prednisolone and warfarin. At the fifteenth day after the start of oral prednisolone tapering, she visited our emergency room for voiding difficulty and paresthesia on both legs. A spinal magnetic resonance imaging revealed increased signal intensity in T2-weighted images from cervical to lumbar level, and neuromyelitis optica- IgG(NMO-IgG) was detected in the patient's serum. After we diagnosed her as having neuromyelitis optica, intravenous methylprednisolone and nine courses of daily plasmapheresis were tried. However, the patient still had visual loss, pain, and sensory loss below the sixth thoracic dermatome, and we tried maintenance therapy with intravenous rituximab. We report our case with reviews of the related literatures.
Adult ; Antibodies, Monoclonal, Murine-Derived ; Brain ; Child ; Emergencies ; Eye ; Heparin ; Humans ; Leg ; Magnetic Resonance Imaging ; Methylprednisolone ; Myelitis, Transverse ; Neurologic Manifestations ; Neuromyelitis Optica ; Optic Neuritis ; Paresthesia ; Plasmapheresis ; Prednisolone ; Retinal Artery ; Retinal Artery Occlusion ; Warfarin ; Rituximab

The use of caffeine citrate for treatment of apnea in very low birth weight infants showed short-term and long-term benefits. A systematic review and meta-analysis of the literature was undertaken to document the effect providing caffeine early (0-2 days of life) compared to providing caffeine late (> or =3 days of life) in very low birth weight infants on several neonatal outcomes, including bronchopulmonary dysplasia (BPD). We searched MEDLINE, the EMBASE database, the Cochrane Library, and KoreaMed for this meta-analysis. The quality of the included studies was assessed using the Newcastle-Ottawa Scale and Jadad's scale. Studies were included if they examined the effect of the early use of caffeine compared with the late use of caffeine. Two reviewers screened the candidate articles and extracted the data from the full-text of all of the included studies. We included a total of 59,136 participants (range 58,997-59,136; variable in one study) from a total of 5 studies. The risk of death (odds ratio [OR], 0.902; 95% confidence interval [CI], 0.828 to 0.983; P=0.019), bronchopulmonary dysplasia (BPD) (OR, 0.507; 95% CI, 0.396 to 0.648; P<0.001), and BPD or death (OR, 0.526; 95% CI, 0.384 to 0.719; P<0.001) were lower in the early caffeine group. Early caffeine use was not associated with a risk of necrotizing enterocolitis (NEC) and NEC requiring surgery. This meta-analysis suggests that early caffeine use has beneficial effects on neonatal outcomes, including mortality and BPD, without increasing the risk of NEC.
Apnea/*drug therapy ; Bronchopulmonary Dysplasia/drug therapy ; Caffeine/*administration & dosage/adverse effects ; Citrates/*administration & dosage/adverse effects ; Enterocolitis, Necrotizing/etiology ; Humans ; Infant ; Infant Mortality ; Infant, Newborn ; Infant, Very Low Birth Weight ; Risk Factors ; Treatment Outcome

PURPOSE: This study assessed the incidence and outcome of congenital anomalies of the kidney and urinary tract (CAKUT) detected by prenatal ultrasonography METHODS: There were 906 cases of CAKUT detected by prenatal ultrasonography and postnatally confirmed at the Asan Medical Center from October 1989 to October 2007. We investigated the incidence and outcome of these cases by reviewing medical records retrospectively. RESULTS: The order of incidence was hydronephrosis, multicystic dysplastic kidney (MCDK), duplex kidney, vesico-ureteral reflux (VUR), single kidney, hydroureteronephrosis, ectopic kidney, polycystic kidney, ureterocele, and posterior urethral valve (PUV). There were 520 cases (57.4%) of hydronephrosis, and 20% of these needed an operation due to significant obstruction. MCDK was associated with other CAKUT in 25.4% of all cases. Approximately 57.9% of duplex kidney cases needed surgical treatment due to ureterocele and VUR. VUR had a male: female ratio of 10:1. Two out of seven cases of autosomal recessive polycystic kidney had progressed to chronic renal failure. Patients with PUV were relatively uncommon, and one out of nine cases progressed to end-stage renal disease. CONCLUSION: CAKUTs detected by prenatal ultrasonography were composed of various anomalies, and almost all of them had a good outcome without any intervention. However, in some cases, recurrent urinary tract infection or renal failure occurred, especially in bilateral cases. For further management, a long-term multicenter study is needed to investigate the precise incidence and outcome of each anomaly in the general population.
Female ; Humans ; Hydronephrosis ; Incidence ; Kidney ; Kidney Failure, Chronic ; Medical Records ; Multicystic Dysplastic Kidney ; Polycystic Kidney Diseases ; Polycystic Kidney, Autosomal Recessive ; Renal Insufficiency ; Retrospective Studies ; Ultrasonography, Prenatal ; Ureterocele ; Urinary Tract ; Urinary Tract Infections ; Vesico-Ureteral Reflux

PURPOSE: To assess the usefulness of amplitude integrated electroencephalography (aEEG), we compared aEEG with electroencephalography (EEG), which is one of the most useful tools for assessment of neonatal seizures in the neonatal intensive care unit. METHODS: We retrospectively studied 24 neonates who had been suspected of seizure activity in the neonatal intensive care unit from January 2009 to February 2010. The 24 neonates had seizure or apnea that did not respond to aminophylline or continuous positive airway pressure. We compared the correlation and efficacy of diagnosis among aEEG, EEG, and neurosonography. RESULTS: We analyzed 24 aEEGs in the neonatal intensive care unit. The aEEGs showed a seizure pattern in 15 (62.6%), burst suppression pattern in 2 (8.3%), flat pattern in 4 (8.3%), and normal in 5 (20.8%). The results of EEGs showed 15 neonatal seizures (62.5%), 2 cerebral dysfunctions (8.3%), and 7 normal findings (29.2%). A correlation between aEEG and EEG was noted (P<0.05). However, there was no correlation between the results of aEEG and echoencephalography. CONCLUSION: There is correlation between aEEG and EEG for seizure detection in the neonatal intensive care unit. We think that aEEG is an useful screening tool for seizure detection in neonates.
Aminophylline ; Apnea ; Continuous Positive Airway Pressure ; Electroencephalography ; Humans ; Infant, Newborn ; Intensive Care, Neonatal ; Mass Screening ; Retrospective Studies ; Seizures

Calcification in patients with endstage renal disease on renal replacement therapy can occur in extraskeletal area such as conjunctiva and cornea. Conjunctival and corneal calcification (CCC) has mostly has been reported in adults with endstage renal disease on hemodialysis. CCC seems to be associated with the duration of renal replacement therapy, and high CaxP value. We report a 10-year-old girl who was on peritoneal dialysis for 31 months and presented with CCC on both eyes. Her corneal calcification was resolved after the epithelial debridement and ethylenediaminetetraacetic acid(EDTA) soaking therapy.
Adult ; Child ; Chlormequat ; Conjunctiva ; Cornea ; Debridement ; Eye ; Humans ; Peritoneal Dialysis ; Renal Dialysis ; Renal Replacement Therapy

PURPOSE: The unique nutrient requirements of premature infants necessitate knowledge of the composition of human milk produced by mothers of such infants. We investigated longitudinal changes in iron concentration of preterm human milk and compared to those observed in human milk of mothers of 1-week old term infants to determine optimal iron supplementation guidelines when preterm infants are nourished exclusively by breast feeding. METHODS: Human milk samples were collected at 1, 2, 4, 6, 8 and 12 weeks postpartum from 103 mothers who delivered infants of gestational age <34 weeks or weighing <1,800 g. Term human milk samples were collected at 1 week postpartum from 24 mothers. RESULTS: There were no significant differences in the iron concentrations of preterm human milk obtained at 2 to 8 weeks postpartum (36.3+/-23.1 to 45.8+/-26.0 microg/dL), but these concentrations were higher than those noted at 1 week in preterm (23.1+/-14.6 microg/dL) and term (25.2+/-7.55 microg/dL) infants. The iron concentration in preterm human milk obtained at corrected term age (42.2+/-19.4 microg/dL) was significantly higher than that of mature term human milk (25.2+/-7.55 microg/dL). CONCLUSION: The concentration of iron in preterm human milk was consistently low during the first 3 months of lactation. Supplementation with iron of at least 2 mg/kg/day should be considered for preterm infants who are exclusively breastfed and who have low body iron stores, to meet the minimum enteral iron requirements recommended by AAP-CON (2004).
Breast ; Female ; Gestational Age ; Humans ; Infant ; Infant, Newborn ; Infant, Premature ; Iron ; Lactation ; Longitudinal Studies ; Milk, Human ; Mothers ; Postpartum Period ; Premature Birth ; Term Birth

Background: This meta-analysis was performed to examine the association between maternal hypertension during pregnancy (HDP) and neonatal bronchopulmonary dysplasia (BPD). Methods: We systematically searched PubMed, EMBASE, the Cochrane Library, and the KoreaMed database for relevant studies. We used the Newcastle-Ottawa Scale for quality assessment of all included studies. The meta-analysis was performed using Comprehensive Meta-Analysis software (version 3.3). Results: We included 35 studies that fulfilled the inclusion criteria; the total number of infants evaluated came to 97,399 through review process. Maternal HDP was not significantly associated with any definition of BPD, i.e., oxygen dependency at 36 weeks of gestation (odds ratio [OR], 1.162; 95% confidence interval [CI], 0.991–1.362; P = 0.064) in pooled analysis of 29 studies or oxygen dependency at 28 days of age (OR, 1.084; 95% CI, 0.660–1.780; P = 0.751) in pooled analysis of 8 studies. Maternal HDP was significantly associated only with severe BPD (OR, 2.341; 95% CI, 1.726–3.174; P < 0.001). BPD was not associated with HDP in the overall analysis (OR, 1.131; 95% CI, 0.977–1.309; P = 0.100) or subgroup analysis according to the definition of HDP. Conclusion Maternal HDP was not associated with neonatal BPD defined by the duration of oxygen dependency (at either 36 weeks of gestation or 28 days of life) but was associated with severe BPD.

Purpose: Intussusception is the most common cause of bowel obstruction in children; however, it is rarely diagnosed in newborn infants. This study aimed to describe the clinical features of intussusception in newborn infants. Methods: Medical records of eight patients diagnosed with intussusception during the newborn period at Ulsan University Hospital between March 2007 and March 2020 were retrospectively reviewed. Results: Among the eight cases, two occurred in the intrauterine period and six occurred in the postnatal period. Intrauterine intussusception presented with symptoms of bowel obstruction within 1 to 2 days after birth, and ileal atresia was diagnosed simultaneously through exploratory laparotomy. All the postnatal patients were extremely low birth weight infants (median gestational age and birth weight: 25+6 weeks and 745 g, respectively). Four cases were diagnosed preoperatively using abdominal ultrasonography. One patient was diagnosed by exploratory laparotomy because the clinical symptoms were nonspecific and difficult to differentiate from those of necrotizing enterocolitis, a more prevalent complication in preterm infants. The site of intussusception in all six patients was the small bowel. Meckel’s diverticulum (one case) and meconium obstruction (two cases) were found to be the lead point. Conclusion Neonatal intussusception tends to show different clinical features according to its period of occurrence. Intussusception, especially in preterm infants, has nonspecific clinical features; therefore, clinicians should always be cautious of this disease for its early diagnosis.

Purpose: Atrial flutter is an uncommon arrhythmia in the neonatal period. This study aimed to describe the cause and clinical course of atrial flutter in neonates. Methods: The medical records of 14 patients diagnosed with atrial flutter at Ulsan University Hospital Neonatal Intensive Care Unit (NICU) between March 2008 and August 2020 were reviewed retrospectively. Results: All 14 cases occurred on the first day of birth. Of these, two were term infants, and 12 were preterm infants. Causes of atrial flutter included three cases of the umbilical venous catheter misplacement, one with a diabetic mother, and one ivolving atrial flutter after an intravenous aminophylline injection. Thirteen patients had structurally normal hearts with no congenital heart diseases. The patient, born to a diabetic mother, had an atrial septal defect and ventricular hypertrophy. Adenosine was administered first to differentiate it from paroxysmal supraventricular tachycardia. Synchronized cardioversion was attempted in 11 patients, while one received it after an esmolol injection that failed to convert to sinus rhythm. One patient had a recurrence after the intrusion of a peripherally inserted central catheter; however, atrial flutter disappeared after repositioning it. No patient had a recurrence after discharge. Conclusion Neonatal atrial flutter is a rare tachyarrhythmia with the risk factors often unknown; however, it could occur in structural heart disease, mispositioning of the umbilical venous catheter, and if the mother has diabetes. During umbilical venous catheterization, clinicians should be cautious and ensure appropriate monitoring of infants in the NICU as it may cause complications.