• Journal of the Korea Institute of Information and Communication Engineering
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• v.15 no.12
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• pp.2549-2554
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• 2011

This paper describes on development of integrated interface and applications to support stable digital data communication service for small and midium sized crafts exploiting SSB modems and HF wireless sets. Firstly, integrated interface module between GPS (Global Positioning System) and communication devices was developed. Secondly, maritime map was implemented into a display terminal to provide location tracking service and voyage planning function. In addition, condition monitoring service of crafts and fishing report functions as well as alarm functions were developed to support secure fishery. Results of performance measure on developed application services shows that error rate was less than 3% and communication delay was shorter than 1 second when a message length was less than 1 Kbyte along with one-to-many communication type.

• Clinical and Experimental Pediatrics
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• v.48 no.1
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• pp.108-111
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• 2005

Ring chromosome 20 mosaicism [r(20)] is a rare chromosomal anomaly associated with minor dysmorphism, mental retardation, autistic behavior, and intractable epilepsy. The proposed mechanism of ring formation is breakage of both short and long arms of a chromosome with subsequent end-to-end fusion. We encountered an 18-month-old boy who presented with developmental delay and mental retardation with seizure episodes, but showed normal brain magnetic resonance imaging. Chromosome study from peripheral blood showed 46,XY, r(20)(p13q13.3) karyotype. The authors report a case of ring chromosome 20 with mental retardation and epilepsy, with a review of the literature.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.11 no.1
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• pp.60-64
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• 2008

Kabuki syndrome is characterized by peculiar facial features, developmental delay, and mental retardation. Congenital hepatic abnormalities in Kabuki syndrome patients have been sporadically reported in the literature and consist of extrahepatic biliary atresia, neonatal sclerosing cholangitis, and transient neonatal cholestasis. We report here a case of congenital hepatic fibrosis in a patient with Kabuki syndrome. To our knowledge, only one case of congenital hepatic fibrosis has been reported in the setting of Kabuki syndrome.

• Clinical and Experimental Pediatrics
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• v.46 no.4
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• pp.389-392
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• 2003

Netherton's syndrome is an unusual disorder which consists of triad of ichtyosiform dermatosis, multiple defects of hair shaft and an atopic diathesis. The finding of bamboo hair is pathognomic in Netherton's syndrome and the ichthyosiform dermatosis may consist of either ichtyosis linearis circumflexa or congenital ichthyosiform erythroderma. Often, variability in the clinical features leads to a delay in diagnosis in many cases. We report a case of Netherton's syndrome diagnosed in the neonatal period. The patient presented with severe ichthyosis and confirmed microscopically distinctive bamboo hair.

• Journal of the Korean Association of Oral and Maxillofacial Surgeons
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• v.30 no.4
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• pp.345-348
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• 2004

Cephalic tetanus is a rare subtype of tetanus in which trismus is a charateristic symptom. The paralysis of one or more cranial nerves can occur. The 7th cranial nerve is most frequently involved. It account for 1 to 3% of the tetanus and has a mortality of 15 to 30%. The incubation period is 1 to 14 days, and approximately two thirds of tetanus cases progress to generalized tetanus. Generally, the symptoms of cephalic tetanus can include : facial pain, trismus, dysphagia, muscle twitching spasms of the face and jaw (risus sardonicus), neck stiffness and malaise. We present a case of cephalic tetanus who 54-year male patient had trismus and dysphagia. There was no history of trauma. As there was a delay in diagnosis of cephalic tetanus, respiratory disorder and intermittent general spasm occurred. The patient was treated by injection of antibiotics, muscle relaxant, and human anti-tetanus immunoglobulin. His symptoms were disappeared, and he was discharged ambulatory.

• Clinical and Experimental Pediatrics
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• v.46 no.11
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• pp.1135-1138
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• 2003

KID syndrome was named after the initials of the major three symptoms of the disease; keratitis, ichthyosis, and deafness. The syndrome was first introduced by Dr. Burns in 1915 who described one of his patients with those symptoms. In 1981, Dr. Skinner and his colleagues reported 17 patients who had keratitis, ichthyosis, and deafness. They also called the disease KID syndrome for the first time. After that, there have been only 60 cases of KID syndrome reported. KID syndrome may be presented with neurosensory deafness, vascularizing keratitis and such skin disorders as ichthyosis, ichthyosiform skin eruptions, and alopecia. Also, those with KID syndrome may suffer from repetitive infections, dental affections, hypohidrosis, growth delay and hepatic and renal abnormalities. The authors report the first case of KID syndrome in Korea with some references. The patient presented with keratitis, neurosensory deafness, and such characteristic skin disorders as ichthyosis and hyperkeratosis.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.21 no.4
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• pp.351-354
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• 2018

Congenital antral webs are a rare but relevant cause of gastric outlet obstruction in infants and children. The condition may lead to feeding refusal, vomiting, and poor growth. Due to the relative rarity of the disease, cases of congenital antral web are frequently misdiagnosed or diagnosed with significant delay as physicians favorably pursue diagnoses of pyloric stenosis and gastric ulcer disease, which are more prevalent. We report a case of an eight-month-old female who presented with persistent non-bilious emesis, feeding difficulties, and failure to thrive and was discovered to have an antral web. The web was successfully treated with endoscopic balloon dilation, which resolved her symptoms. Two years later, the patient remains asymptomatic and is thriving with weight at the 75th percentile for her age.

• Journal of Genetic Medicine
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• v.14 no.2
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• pp.86-89
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• 2017

Waardenburg syndrome (WS) is a rare genetic disorder, including clinical features of pigmentary abnormalities of irides, skin, hair and sensorineural hearing loss and facial dysmorphism. Among the four types, WS type IV (Waardenburg-Shah syndrome) additionally represents Hirschsprung's disease. Mutations in the SOX10, END3, or EDNRB genes are known to cause WS type IV. Here, we report a 6 year-old girl who was diagnosed as WS type IV by typical clinical manifestations, including skin hypopigmentation, heterochromia of both irides, unilateral sensorineural hearing loss, mild developmental delay and Hirschsprung's disease. The diagnosis was confirmed by molecular genetic analysis of EDNRB. Two novel EDNRB mutations were identified, and each mutation was segregated from each of her parents. During the follow-up period, the patient underwent a surgery for spleen torsion and was medically managed due to recurrent enterocolitis. Also, she suffered from impaired immunity including Hirschsprung's associated enterocolitis.

• Archives of Plastic Surgery
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• v.32 no.3
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• pp.385-388
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• 2005

In cleft lip and/or palate patients with the complex congenital heart diseases, surgical repair of the cleft lip and/or palate has been postponed after the open heart surgery because the heart problem of the patient might cause more complications associated with anesthesia and surgery. There has been little report about experiences in the surgical management of these patients and optimal time of surgical intervention. Authors are introducing the experiences of performing corrective surgery of cleft lip and/or palate in the patients with congenital heart diseases before and after the open heart surgery. We managed five patients from May 1992 to March 2004. Two patients were male and the rest were female. One of them had cleft lip alone and others had cleft lip and palate. Two of them underwent delayed cleft lip and/or palate surgery after open heart surgery, and the rest had immediate intervention for cleft lip and/or palate. There was no complication during the operation and postoperative period. There would be no need to delay the corrective surgery of the cleft lip and/or palate after the open heart surgery, if solid medical team approach was available with the pediatric cardiologist and the anesthesiologist.

• Pediatric Gastroenterology, Hepatology & Nutrition
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• v.8 no.2
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• pp.275-278
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• 2005

We report a boy with acute pancreatitis without abdominal pain accompanied by hepatic hematoma and massive ascites due to unperceived trauma. The case was difficult to diagnose with image study and the level of amylase. Strong suspicion of pancreatic injury based on mechanism of injury and clinical evaluation would be required to avoid a delay in diagnosis.