Total Anomalous Pulmonary Venous Return in Siblings

Total anomalous pulmonary venous return (TAPVR) is a rare and critical congenital vascular anomaly that requires an early operation. However, initial symptoms of TAPVR may be non-specific, and cardiovascular findings may be minimal. The heart may not be enlarged and there is often no cardiac murmur. Without cardiac murmur, these symptoms are similar to those of respiratory distress syndrome in newborns. Therefore, a high degree of suspicion and an early diagnosis of TAPVR are important. This condition generally occurs without a family history and has a low recurrence rate, but several familial cases, including siblings, have been reported worldwide. Additionally, several chromosomal or gene abnormalities associated with TAPVR have been reported. In the case presented here, two brothers with a 6-year age gap were diagnosed with TAPVR. Surgery was performed without cardiac or neurological complications. This is the first report on TAPVR in siblings in Korea.

Late adverse reactions to iopromide (Ultravist(R)) diagnosed by the patch test: a case report / 소아과

Iodinated contrast media (CM) can cause immediate and late reactions. We treated a patient with a recurrent generalized maculopapular rash and a fever that occurred within two days of exposure to iodinated CM, iopromide (Ultravist(R)), for chest computed tomography. We performed skin testing including prick tests, intradermal tests, and patch tests. Our findings indicated a late skin reaction to Ultravist(R) in addition to cross-reactions to other iodinated CM such as ioversol (Optiray(R)), iohexol (Iobrix(R)), and iobitridol (Xenetix(R)). In this study, we report the case of a patient diagnosed with a late adverse reaction to Ultravist(R) in addition to cross-reactions to other iodinated CM.

Herpes zoster complicated by deep vein thrombosis : a case report / 소아과

Varicella zoster virus (VZV) causes two diseases: Varicella, a generalized, primary infection, and herpes zoster (zoster), a secondary infection caused by latent VZV reactivation. Zoster can also be caused by latent VZV reactivation after a varicella vaccination. The complications associated with varicella include cutaneous infections, which are the most common, as well as pulmonary and neurological involvement. However, a deep venous thrombosis (DVT) has been rarely described as a varicella-associated complication. Here, we describe the case of a child with varicella zoster who developed a DVT that completely resolved after intravenous acyclovir and subcutaneous low-molecular-weight heparin treatment.

Herpes zoster complicated by deep vein thrombosis : a case report / 소아과

Varicella zoster virus (VZV) causes two diseases: Varicella, a generalized, primary infection, and herpes zoster (zoster), a secondary infection caused by latent VZV reactivation. Zoster can also be caused by latent VZV reactivation after a varicella vaccination. The complications associated with varicella include cutaneous infections, which are the most common, as well as pulmonary and neurological involvement. However, a deep venous thrombosis (DVT) has been rarely described as a varicella-associated complication. Here, we describe the case of a child with varicella zoster who developed a DVT that completely resolved after intravenous acyclovir and subcutaneous low-molecular-weight heparin treatment.

Fatal plastic bronchitis with eosinophilic casts in a previously healthy child / 소아과

Plastic bronchitis is a rare disease characterized by the recurrent formation of branching mucoid bronchial casts that are large and more cohesive than those that occur in ordinary mucus plugging. Casts may vary in size and can be spontaneously expectorated, but some require bronchoscopy for removal. Plastic bronchitis can therefore present as an acute life-threatening emergency if obstruction of the major airways occurs. Three of 22 reported patients with eosinophilic casts were fatal, with death due to central airway obstruction. Here, we report a child with no history of atopy, allergy, or congenital heart disease who was diagnosed with plastic bronchitis with eosinophilic casts. Although he was administered intravenous (iv) antibiotics; iv corticosteroids; and a vigorous pulmonary toilet regimen, including chest physiotherapy and routine bronchoscopic removal of casts, he had brain death secondary to hypoxic brain damage. Plastic bronchitis can be fatal when casts obstruct the major airways, as in the present case. Clinicians should intervene early if a patient exhibits signs and symptoms consistent with plastic bronchitis.

Fatal plastic bronchitis with eosinophilic casts in a previously healthy child / 소아과

Plastic bronchitis is a rare disease characterized by the recurrent formation of branching mucoid bronchial casts that are large and more cohesive than those that occur in ordinary mucus plugging. Casts may vary in size and can be spontaneously expectorated, but some require bronchoscopy for removal. Plastic bronchitis can therefore present as an acute life-threatening emergency if obstruction of the major airways occurs. Three of 22 reported patients with eosinophilic casts were fatal, with death due to central airway obstruction. Here, we report a child with no history of atopy, allergy, or congenital heart disease who was diagnosed with plastic bronchitis with eosinophilic casts. Although he was administered intravenous (iv) antibiotics; iv corticosteroids; and a vigorous pulmonary toilet regimen, including chest physiotherapy and routine bronchoscopic removal of casts, he had brain death secondary to hypoxic brain damage. Plastic bronchitis can be fatal when casts obstruct the major airways, as in the present case. Clinicians should intervene early if a patient exhibits signs and symptoms consistent with plastic bronchitis.

Congenital hernia of the lung through the azygoesophageal recess / 소아과

A lung hernia, defined as the protrusion of pulmonary tissue and pleural membranes through a defect in the thoracic wall, is a rare event. It can be congenital or acquired, and cervical, thoracic, or diaphragmatic in location. We report the rare occurrence of a congenital atraumatic lung herniation through the azygoesophageal recess. An 8 -month-old male infant, who was born at 3 5 weeks gestation, had a chronic cough. Chest radiography showed haziness at the right lower lobe of the lung (RLL). Chest computed tomography (CT) revealed herniation of the RLL through the azygoesophageal recess. If persistent unilateral haziness is observed on chest radiography, the possibility of lung herniation should be considered.

Bilateral Congenital Pulmonary Vein Stenosis with a Normal Connection

Congenital pulmonary vein stenosis (CPVS) with an anatomically normal connection is a rare cardiac malformation. This cardiac anomaly usually is accompanied by other cardiac abnormalities. Bilateral CPVS is a more severe form of a CPVS and it usually leads to progressive pulmonary hypertension and death if it is not treated. Here, we report a patient with a history of cough, tachypnea and hemoptysis and suspected CPVS due to an abnormal thoracic roentgenogram with dilated right pulmonary arteries and pulmonary cornus. The two-dimensional and color Doppler echocardiography demonstrated three stenosed pulmonary veins connected to the left atrium. However, the fourth vessel could not be visualized. There were no other cardiac malformations associated with the CPVS. The Technetium-99m macro-aggregate lung perfusion scan showed absent or diminished perfusion to the affected lobes of the lungs. In addition, the chest computed tomography with angiogram and cardiac catheterization confirmed the findings of the echocardiogram.

A case of vancomycin-induced drug hypersensitivity syndrome / 소아과

Drug hypersensitivity syndrome (DHS) has rarely been reported in association with vancomycin treatment. Here, we describe an 11-year-old girl who developed fever and a maculopapular rash on day 18 of intravenous vancomycin for treatment of infective endocarditis. The patient presented with fever, a maculopapular skin rash, hepatitis, and acute renal failure caused by vancomycin-induced DHS. The symptoms resolved in less than 24 h after withdrawal of vancomycin and treatment with corticosteroids. We present this case of DHS associated with vancomycin.

A case of imipramine induced toxicity with Brugada electrocardiographic pattern in a toddler / 소아과

Imipramine, a tricyclic antidepressant (TCA), is used for the treatment of non-polar depression and nocturnal enuresis in children in whom an organic pathology has been excluded, anxiety disorders, and neuropathic pain. Clinical toxicity following the treatment of TCAs, including imipramine, is well known. The anticholinergic effects initially present include a dry mouth, ileus, dilated pupils, urinary retention, and mild sinus tachycardia. The central nervous system toxicity includes delirium, agitation, restlessness, hallucinations, convulsions, and CNS depression or coma. However, the most life-threatening toxicity remains the development of cardiac dysrhythmias. Conduction delays such as QRS and corrected QT prolongation, wide QRS complex tachycardia, and the Brugada electrocardiographic pattern have been reported. Sodium bicarbonate decreases QRS widening and suppresses dysrhythmias by providing excess sodium to reverse the TCA-induced sodium-channel blockade and possibly by binding directly to the myocardium. There are no pediatric case reports on imipramine or other TCA associated toxicity in Korea. Here, we describe a patient who presented with convulsions, tachycardia with a wide QRS complex, a Brugada electrocardiographic pattern, and anuresis associated with an accidental overdose of imipramine and the outcome of treatment with sodium bicarbonate.

Epidural emphysema and pneumoscrotum caused by bronchial foreign body aspiration / 소아과

Epidural emphysema and pneumoscrotum with subcutaneous emphysema are rare in a child past the neonatal period. Their most common causes are bronchial asthma and respiratory infection. Here, we report an 18-month-old boy who was presented with severe air leak, consisting of epidural emphysema, pneumoscrotum, subcutaneous emphysema, and pneumomediastinum, complicated by a bronchial foreign body. The air leak was resolved dramatically after removing the foreign body.

A case of pulmonary thromboembolism in a healthy infant / 소아과

A pulmonary thromboembolism (PTE), which is a sudden blockage in a pulmonary artery, usually due to a blood clot, is rare in children. The clinical presentation is often subtle or masked by the underlying clinical condition and the condition must be suspected during clinical testing. Although the choice of treatment depends on the clinical presentation, anticoagulation is the mainstay of therapy for children with PTE. We report the case of a healthy 1-month-old boy who presented with hemoptysis without hemodynamic instability. He was diagnosed based on chest computed tomography with angiography and 99mTc macroaggregated albumin lung perfusion scintigraphy and treated with low-molecular-weight heparin.

A Case of Norwegian Scabies in Patient with Acute Myeloid Leukemia Associated Graft-versus-host Disease / 대한피부과학회지

Norwegian scabies is a crusted non-itchy rash caused by massive infestation with the mite Sarcoptes scabiei. It is usually associated with immunosuppression or neurologic impairment, although it has rarely been reported in normal persons. We report a case of Norwegian scabies in 6-year old girl with acute myeloid leukemia associated graft-versus-host disease. The lesion showed pruritic, hyperkeratotic scaly plaques and fissures on the entire body. Application of a mineral oil preparation from hyperkeratotic areas showed numerous scabies mites. Treatment with 10% crotamiton cream for two weeks resulted in marked regression of the skin lesions, but finally she died of sepsis due to deterioration of her systemic conditions.

Clinical characteristics and outcomes of status epilepticus as an initial seizure in children / 소아과

PURPOSE: To evaluate the morbidity and mortality of children with status epilepticus(SE) as an initial seizure and to compare these according to age groups. METHODS: The 78 cases(38 cases or = 2 years) with SE as an initial seizure admitted to the Chonnam national university hospital from Jan. 2000 to Jan. 2004 were reviewed. Developmental profiles, laboratory findings, etiologies and seizure types of SE and outcomes were compared in between two age groups, under and over 2 years. RESULTS: SE occurred predominantly in less than 5 years old. Febrile causes were the most common, which is significantly more in those under 2 years than over 2 years(P or = 2 years). The estimated occurrence of epilepsy after SE was 24.4 percent(19 cases:8 cases or = 2 years). The neurologic sequelae after SE in cases that had developed normally before SE(62 cases:32 cases or = 2 years) were observed in 20 cases(32.3 percent), and were more frequent over 2 years(21.9 percent vs. 43.3 percent, P<0.05). CONCLUSION: In this study death was less common and the neurologic sequelaes of SE as an initial seizure were less severe in children under 2 years of age. The reason seemed to be the difference in the etiology of SE with age.

A Clinical Study of Benign Convulsions Associated with Acute Gastroenteritis / 대한소아신경학회지

PURPOSE: The aim of this study was to reveal detailed clinical features of benign childhood convulsions associated with acute gastroenteritis(BCAGE). METHODS: The medical records of 28 patients with BCAGE, who were admitted to the department of pediatrics, Chonnam National University Hospital between March 2000 and October 2004, were reviewed on the aspects of clinical symptoms, diagnostic tests and treatment. RESULTS: The ages of the subjects cases ranged from one to 96 months old and most of them had BCAGE in late autumn and winter seasons. Seizures were developed mostly 2 to 4 days after the symptoms of gastroenteritis appeared. Most of them showed generalized seizures which occurred repetitively during the episode of acute gastroenteritis (mean 2.7+/-0.9 times of the seizures attack), mostly 2-4 times. The duration of seizure was largely below 5 minutes, but in 2 cases it was prolonged over 15 minutes. The Rotazyme tests of stool were positive only in 3 cases(16.7%), and the cerebrospinal fluid and blood examinations revealed normal results. Either brain CT or MRI was performed in 17 patients and variable results were demonstrated. Most of them showed normal or mild brain swelling, but one showed cortical dysplasia of the frontal and parietal lobes, while another one showed cerebrospinal fluid collection in the posterior fossa. 17 patients were treated with diazepam, 12 of whom were prescribed additional phenbarbital or phenytoin. 2 patients who did not take diazepam were administered antiepileptics. Antiepileptic drugs were continuously given to 4 patients for up to one year after discharged from hospital. During follow-up periods, there occurred no further seizures in all the patients. CONCLUSION: BCAGE can be considered as a situation related seizure which does not need any long-term antiepileptic medications.