• Clinical and Experimental Pediatrics
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• v.51 no.10
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• pp.1123-1126
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• 2008

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 35 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.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.11 no.1
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• pp.56-59
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• 2008

Extrahepatic portosystemic shunts, known as Abernethy malformations, were first reported by John Abernethy in 1793. They are classified into two types: Type I refers to a congenital absence of the portal vein and Type II refers to a shunt involving a side-to-side anastomosis with reduced portal blood flow into the liver parenchyma. This malformation is so rare that less than 100 cases have been reported in the medical literature. We report the case of a 13-month-old boy who had a congenital extrahepatic portocaval shunt with a hypoplastic portal vein. This case was complicated with an atrial septal defect and a large hyperplastic nodule in the liver. The patient was diagnosed with a Type II Abernethy malformation. We planned on surgical occlusion of the extrahepatic portocaval shunt. However, six months later, the patient had a sudden onset of a fever of unknown origin and developed hepatic encephalopathy. Although he underwent a liver transplantation, he died of acute hepatic failure.

• Childhood Kidney Diseases
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• v.13 no.2
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• pp.248-251
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• 2009

The urachus is a normal embryonic remnant of the primitive dome. It generally exists as a fibrous cord extending from the dome of the bladder to the umbilicus. Disorders of the urachus are developed as a result of its incomplete regression. The urachal cyst is the most common urachal anomaly, and is usually asymptomatic in infancy and childhood. However, when the cysts are large or accompanied with secondary infection, they may be detected in its early stage. A sonography or CT scan may be helpful to confirm the diagnosis of urachal cyst. The managements of infected urachal cyst are varied from simple drainage to radical excision. Here, we report an unusual case of urachal cyst infection that occurred during corticosteroids therapy in a girl with IgA nephropathy.

• Clinical and Experimental Pediatrics
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• v.53 no.6
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• pp.722-726
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• 2010

Chronic granulomatous disease (CGD) is an uncommon inherited disorder caused by mutations in any of the genes encoding subunits of the superoxide-generating phagocyte NADPH oxidase system, which is essential for killing catalase producing bacteria and fungi, such as $Aspergillus$ species, $Staphylococcus$ $aureus$, $Serratia$ $marcescens$, $Nocardia$ species and $Burkholderia$ $cepacia$. In case of a history of recurrent or persistent infections, immune deficiency should be investigated. Particularly, in the case of uncommon infections such as aspergillosis in early life, CGD should be considered. We describe here a case of CGD that presented with invasive pulmonary aspergillosis in a 2-month-old girl. We confirmed pulmonary aspergillosis noninvasively through a positive result from the culture of bronchial alveolar lavage fluid, positive serological test for $Aspergillus$ antigen and radiology results. She was successfully treated with Amphotericin B and recombinant IFN-${\gamma}$ initially. Six weeks later after discharge, she was readmitted for pneumonia. Since there were infiltrates on the right lower lung, which were considered as residual lesions, voriconazole therapy was initiated. She showed a favorable response to the treatment and follow-up CT showed regression of the pulmonary infiltrates.

• Clinical and Experimental Pediatrics
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• v.45 no.2
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• pp.261-266
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• 2002

Vascular ring, originating from abnormal regression of the aortic arch during fetal life, can cause prolonged and recurrent respiratory symptoms and dysphagia when the diagnosis is delayed. We report a 4 month old girl with vascular ring, who had been treated for persistent respiratory symptoms including stridor, wheezing, and dyspnea soon after birth. Initially her respiratory symptoms were thought to be due to bronchiolitis, for which respiratory syncytial virus was confirmed by immunofluorescent staining. Her clinical course was again complicated with tracheitis and pneumonia due to Haemophilus influenzae type b. The possibility of anatomical anomaly was investigated when it was felt to be difficult to insert a suction catheter deep down through a endotracheal tube which was placed for adequate ventilatory management. A three-dimensional chest CT revealed a vascular ring consisting of a double aortic arch. For 5 months following surgery, her respiratory symptoms have slowly been improving. She developed another episode of pneumonia which was milder than the one which occurred before the surgery.

• Clinical and Experimental Pediatrics
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• v.46 no.4
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• pp.400-403
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• 2003

Congenital adrenal hyperplasia(CAH) is a recognized cause of precocious pseudopuberty. Some children with CAH also develop true precocious puberty with early maturation of the hypothalamic-pituitary-gonadal axis. We review a case of CAH who eventually developed central precocious puberty nine months after initial treatment with corticosteroid. A 3-year-old boy visited complaining of rapid growth, a large penis and frequent penile erections. This patient was diagnosed with CAH with elevated 17-OH progesterone and cortical hypertrophy of adrenal gland on CT scan. His gonadotropin levels were within the normal prepubertal range. Even on treatment with corticosteroid he grew rapidly and had testicular enlargement, pubic hair development and rapid bone maturation. At second admission, his gonadotropin levels were elevated both basally and in response to LHRH stimulation, suggesting that the CAH led to early activation of pubertal gonadotropin secretion(true precocious puberty). He was treated with monthly depot injections of a LHRH analog in addition to the hydrocortisone. His second sexual characteristics regressed gradually and rate of linear growth and bone maturation decreased.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.7 no.1
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• pp.92-97
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• 2004

Posttranplantations lymphoproliferative disease (PTLD) is a common and life-threatening complication for soid organ transplantation associated with the use of chronic immunosuppression and Epstein-Barr virus. There is no standardized treatment algorithm, but numerous management strategies are vaiable. Partial splenic embolization (PSE) had been demonstrated to be an effetive alternatie to splenectomy for patients hypersplenism and portal hypertension. PSE has the advantages of non-invasive intervention and resolution of the complications of hypersplenism. We report the effect of the PSE in a 6-year old male liver transplantation recepient with PTLD who has undergone persistent hypersplenism post-transplant. We reduced immunosuppression agent, started antiviral agent. We started with interferon and IV globulin one month after admission. Hepatosplenomegaly and cervical lymphadenopathy were improved. But fever was not subside. We selectively embolized the lower pole of the spleen to achieve a 50~60% reduction in flow as determined by angiography. After embolization, fever subside and peripheral blood findings were improved. Follow up abdominal CT revealed reduced volume of spleen due to ischemic change and there was no multiple enlarged mesenteric lymphnode compared to preembolization state. We thick that PSE is a safe an effetive treatment modality of PTLD with persistent hypersplenism in patients twho failed to medical treatment.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.3 no.2
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• pp.217-221
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• 2000

Solid and papillary epithelial neoplasm of the pancreas is an uncommon low-grade malignant tumor found predominantly in young females. It is rare in childhood. The origin of the tumor is probably from a multipotential stem cell of the pancreas. Neoplasm usually behave like a very low grade malignancy, so that complete removal is the treatment of choice for the tumor arising anywhere in the pancreas. We report a case of solid and papillary epithelial neoplasm of the pancreas in a 14 year -old girl who presented with intermittent abdominal pain and abdominal mass. CT scan shows a large, about $7{\times}6\;cm$ sized, well-marginated hypodense round mass in the head of the pancreas with some ill defined enhancing solid internal portion. She had taken complete excision of the pancereatic mass and Roux-en-Y pancreaticojejunostomy and histologically comfirmed solid and papillary epithelial neoplasm of the pancreas. A brief review of literature was made.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.23 no.1
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• pp.79-88
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• 2020

Purpose: Management of eosinophilic esophagitis (EoE) varies from center to center. In this study, we evaluated the effectiveness of a dairy-free diet (DFD) and the 6-Food Elimination Diet (SFED) as initial therapies for the treatment of EoE in our practice. Methods: This was a retrospective study of children who had been treated for EoE at Connecticut Children's Medical Center, Hartford, CT, USA. Pre- and post-treatment endoscopy findings and histology results of patients treated with DFD or SFED were examined. Results: One hundred fifty-two patients (age 9.2±5.2 years, 76.3% male, 69.7% caucasian) met the inclusion criteria for initial treatment with DFD (n=102) or SFED (n=50). Response for DFD was 56.9% and for SFED was 52.0%. Response based on treatment duration (<10, 10-12, and >12 weeks) were 81.8%, 50.0%, and 55.1% for DFD, and 68.8%, 50.0%, and 40.0% for SFED. Response based on age (<6, 6-12, and >12 years) were 59.3%, 42.9%, and 67.5% for DFD, and 36.4%, 58.8%, and 72.7% for SFED. In patients treated with DFD, concomitant proton pump inhibitor (PPI) administration resulted in improved outcomes (p=0.0177). Bivariate regression analysis showed that PPI with diet is the only predictor of response (p=0.0491), however, there were no significant predictors on multiple regression analysis. Conclusion: DFD and SFED are effective first line therapies for EoE. DFD should be tried first before extensive elimination diets. Concomitant therapy with PPI's may be helpful.

• Archives of Plastic Surgery
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• v.38 no.1
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• pp.96-101
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• 2011

Purpose: In treatment of facial bone fracture, occurred in children, we generally use wires, miniplate, absorbable plate or their combination. These foreign bodies can be palpable, and sometimes may cause infections, and need reoperation for removing. When bone fragments are multiple, small or thin, they are hard to handle and make accurate reduction of all fragments. In these cases, a biodegradable tissue adhesive, Histoacryl$^{(R)}$ (n-butyl-2-cyanoacrylate), can be used as fixation technique for small, multiple, and thin fracture fragments. Methods: 3 years old and 6 years old children, who has comminuted fracture on frontal sinus, we used Histoacryl$^{(R)}$ for fixation of multiple bone fragments. After approaching by coronal incision, we pulled out all bone fragments and reconstructed bone fragments by Histoacryl$^{(R)}$ and fixed those on frontal sinus by absorbable plates. Results: Photographs and 3-dimensional CT obtained preoperatively and postoperatively. And we compared them each other in terms of accuracy of reconstructed bony contour. We could reconstruct almost all bone fragments easily along 3-dimensional structure and get excellent aesthetic results. There was no complication such as infection. Conclusion: In treatment of comminuted facial bone fracture, occurred in children, Histoacryl$^{(R)}$ is an excellent method for accurate reconstruction in small and thin bone fragments which cannot be fixed by wire, miniplate or absorbable plate without complication.