Retraction: Aortic Dissection and Rupture in a Child

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Aortic Dissection and Rupture in a Child

After developing sudden severe chest pain, an 11-year-old boy presented to the emergency room with chest pain and palpitations and was unable to stand up. The sudden onset of chest pain was first reported while swimming at school about 30 minutes prior to presentation. Arterial blood pressure (BP) was 150/90 mmHg, heart rate was 120/minute, and the chest pain was combined with shortness of breath and diaphoresis. During the evaluation in the emergency room, the chest pain worsened and abdominal pain developed. An aortic dissection was suspected and a chest and abdomen CT was obtained. The diagnosis of aortic dissection type B was established by CT imaging. The patient went to surgery immediately with BP control. He died prior to surgery due to aortic rupture. Here we present this rare case of aortic dissection type B with rupture, reported in an 11-year-old Korean child.

Early Onset Renal Failure in Congenital Nephrotic Syndrome associated with Congenital Diaphragmatic Hernia by WT1 Gene Mutation

We experienced a female neonate with congenital nephrotic syndrome (CNS) associated with congenital diaphragmatic hernia (CDH). Because of the rare combination of two conditions, we report this case with literature review. CDH was found immediately after birth and emergency operation was done for hernia repair. But on the next day, generalized edema and oliguria(0.59 mL/kg/hour) was found and her blood chemistry showed hypoalbuminemia (1.6 g/dL), increased BUN (27.7 mg/dL) and serum creatinine( 1.8 mg/dL) along with heavy proteinuria (4+). We started albumin infusion with a bolus of intravenous furosemide. We suspected the neonate had congenital nephrotic syndrome and her 24hr urine protein was 1,816 mg/day. In spite of immunosuppressive therapy, the nephrotic syndrome and renal failure progressed. We started peritoneal dialysis on the day of life 22 but it was not satisfactory. She was complicated by intracranial hemorrhage and multi-organ failure and expired at 34 days of age. Kidney necropsy was performed which showed diffuse mesangial sclerosis (DMS). Her chromosome study revealed 46, XX and her gene study revealed a heterozygous missense mutation, Arg366His, in Wilms tumor suppressor gene (WT1). This case deserves attention on account of the 4th case of CNS with CDH revealing the Arg366His mutation in the WT1 gene andG the 1st case of early onset renal failure without male pseudohermaphroditism and Wilms tumor with CNS, CDH and the Arg366His mutation in the WT1 gene. So, this report gives support to the hypothesis that Arg366His mutation in the WT1 gene can result in CNS and CDH.

Seven-Year Follow Up of Microscopic Polyangiitis Presenting with Rapidly Progressive Glomerulonephritis

Microscopic polyangiitis(MPA) is a systemic necrotizing vasculitis that involves many organ systems including the skin, joint, kidneys, and lungs. In spite of early diagnosis and intensive care, the five-year actuarial patient and kidney survival rates are 65% and 55%. We experienced a case in 7-year-old girl of microscopic polyangiitis presenting with rapidly progressive glomerulonephritis which was confirmed by renal biopsy and positive serum perinuclear antineutrophil cytoplasmic autoantibodies(p-ANCA). The diagnosis of patients first renal biopsy was MPA, p-ANCA-associated crescentic glomerulonephritis. The patients second renal biopsy was done 5 years 6 months later since first renal biopsy, and pathologic diagnosis was chronic sclerosing glomerulonephritis, advanced, due to MPA. We began methylprednisolone pulse therapy, combined with a low dose of cyclophosphamide and plasmapheresis therapy. ACE inhibitor, angiotensin II receptor blocker, and cyclophosphamide were used until now and the patients current age is 14 years old. On admission, the patients laboratory findings showed BUN 117 mg/dL and Cr 2.3 mg/dL, while on the hospital day BUN and Cr values fell to 20.8 mg/dL and 1.6 mg/dL. But renal function was progressed to chronic failure with latest laboratory data BUN 51.7 mg/dL and Cr 3.2 mg/dL. ACE inhibitor, angiotensin II receptor blocker and small dose of immunosuppressant with close observation is the key to maintain the patient survival.

A Study of Clinical Significance of Dizziness in Diagnostic Approach for Children with Chronic Headache

PURPOSE: Headache is a common neurologic symptom in children but chronic headache in them must be diagnosed in different ways. The aim of this study is to determine the algorithm of diagnostic approach for children with chronic headache and know the clinical significance of dizziness in evaluating those children. METHODS: We recruited children with headache at Myongji Hospital from January 2004 to July 2006. The study was based on a retrospective review of their medical records and interviews with the children and their parents. We divided them into two groups : Group A headache with dizziness, Group B headache without dizziness. RESULTS: The medical records of 86 children and adolescents with headache were reviewed. Age for group A ranged from 10 to 15 years(73.7%) and gender ratio was 1:1.7. Age for group B ranged mostly under 7 years(56.3%) and gender ratio was 1:0.85. Headache in group A frequently occurred predominantly in the morning(37.3%). The duration of the attack was over 90 min(87.5%) in group A and over 90 min(50.0%) in group B. Associated symptoms were mainly nausea(34.2%), vomiting(18.4%), blurred vision(12.7%) in group A. Over 90% of them had headache 4 times a week and so did dizziness. The final diagnoses in cases of group A were migraine(31.6%), orthostatic hypotension(15.8%), postural orthostatic tachycardia syndrome(13.2%), and benign paroxysmal positional vertigo(10.5%). While in group B diagnoses included sinusitis(50.0%), migraine(31.3%), and complications of migraine(8.3%). CONCLUSION: This study revealed high percentage of headache concurred with dizziness in children. The association of headache with dizziness is complex and maybe coincidental. If chronic headache with dizziness in girls occurs mainly in the morning, it maybe related to vestibular disorder and orthostatic instability. In conclusion, dizziness and other associated symptoms or clinical features should carefully be assessed in the diagnosis of headache.

A Case of Cerebral Sinus Expansion and Concurrent Diffuse Meningeal Enhancement in Postlumbar Puncture Headache

Post-lumbar puncture headache (PLPH) is a common complication developed after lumbar puncture, and it usually begins within 48 hours. The pain is postural; the it is relieved by reclining and worsened by sitting. Moreover, there are some related symptoms such as nausea, vomiting, tinnitus and blurred vision. PLPH is rare in children and it is effectively treated by bed rest and conservative treatment. However, when the pain is too severe or continuous or different types of headache are seen after LP, it is worthwhile to conduct brain MRI for exact diagnosis and proper treatment. Therefore, we report a patient with PLPH in whom brain MRI showed diffuse meningeal enhancement and concurrent cerebral venous expansion, with a review of literatures.

Myocardial Function in Infant Kawasaki Disease with Tissue Doppler Imaging

PURPOSE: The purpose of this study is to assess myocardial function in infant Kawasaki disease, with strain and tissue Doppler imaging, in addition to conventional echocardiographic measures. METHODS: Strain and tissue Doppler imaging were performed in 20 patients with infant Kawasaki disease. Peak velocities of systolic and early, late diastolic pulsed tissue Doppler velocities, strain, and time to peak strain values were measured at ventricular septal annulus from apical view. The data set were obtained at both acute period and convalescent period. RESULTS: All patients represents coronary arteriopathy. Measures of Systolic peak velocity (S') of pulsed wave tissue Doppler value and strain decreased in convalescent phase, and time to peak strain was delayed (P<0.01). Meanwhile, the diastolic measures (E' and A') demonstrated no significant differences between acute and convalescent phases using tissue Doppler velocity data. CONCLUSION: In convalescent phase, myocardial systolic function may not be completely improved yet. After convalescent period, continuous myocardial assessment should be followed.

A Case of Multiple Large Renal Abscesses Completely Resolved by Conservative Antibiotics Administration

Renal abscess in childhood is a rare disease, and generally treatment of renal abscesses in childhood follows the guidelines in adults. The guidelines of treatment of renal abscesses in adults include the following:renal abscesses smaller than 3 cm in size can be managed by antibiotics administration, while renal abscesses above 3 cm in size must be considered for percutaneous abscess drainage or open drainage. We experienced a case of a 2 year-old girl with multiple renal abscesses greater than 4 cm in size which resolved by oral antibiotics administration after 2 weeks of intravenous administration. We report this case with literature review.