One Stage Metatarsal Lengthening of Brachymetatarsia Involving Both 1st and 4th Metatarsal Bone (A case report) / 대한족부족관절학회지

19 years old girl presented with bilateral 1, 4th brachymetatarsia. We have used an autograft interposition technique to lengthen the brachymetatarsia involving first and fourth metatarsal. The technique was to graft the bone fragment from the relatively long second and third metatarsal bone to be used as an autograft to the short first and fourth metatarsal bone. The method is superior in not having the necessity of a long term external fixator which is needed using callotasis method and in also avoiding the inconvenience of performing an allograft. Absence of donor site complication during autograft from iliac bone is also an advantage to be mentioned. Our technique can therefore be ascertained as a successful method in both cosmetic results and improvement of symptoms including reduction in length of recovery.

Volar Open Reduction of Traumatic Dislocation of the 5th Metacarpophalangeal Joint Without Releasing Volar Plate: A Case Report

Traumatic dislocation of the metacarphophalangeal joint is a rare injury. Complex dislocation often require surgical procedure due to its anatomical characteristics. We report one case of surgically treated traumatic dislocation of the 5th metacarpophalangeal joint that was successfully reduced by volar open reduction without splitting the volar plate.

A case of acquired acrodermatitis enteropathica with a normal serum zinc level but a low level in the hair / 소아과

Acrodermatitis enteropathica (AE) is a rare autosomal recessive disorder of early infancy, and is characterized by periorificial dermatitis, alopecia, and intractable diarrhea. Serum zinc levels are usually low in untreated patients and the oral administration of zinc sulfate can clear skin lesions and other symptoms. Although premature and cow`s milk-fed infants are at particular risk of developing AE, there have been a few reports about AE in term and breast-fed infants. We report a case of transient AE in a 4-month-old breast-fed infant. This patient suffered from diarrhea and dermatitis for more than a month. Her skin lesions were erythematous, scaly, crusted, psoriasiform, eczematous, with an eruption at the chin, and a periorificial disposition with involvement of the flexural areas of lower extremities. Her serum zinc level was almost normal at 129 microgram/dL (reference range: 60-121 microgram/dL), but the zinc level in her hair was low: 8 mg percent (reference range: 10-21 mg percent). Skin biopsy findings were consistent with AE. Seven days after zinc supplementation, the skin lesions and diarrhea improved. The authors recommend that a clinical trial of zinc supplementation be considered in cases where there are suspicious of AE, even when the serum zinc level is normal.

An Unusual Case of Acute Pyelonephritis Caused by Shigella dysenteri in a Child

Shigella infection usually produces gastrointestinal symptoms but rarely causes urinary tract infection. A 7-year-old girl was admitted for fever, chills, right flank pain, and dysuria. She had no vomiting or diarrhea. There was mild tenderness in her right lower abdomen, and right CVA tenderness was also noted. Acute pyelonephritis was diagnosed by abdominal CT. She showed improvement with intravenous administration of antibiotics. The first urine culture grew 1 x 10(5) CFU/mL Shigella dysenteri. Although urinary tract infections due to Shigella species are extremely rare, Shigella species should be considered as a possible cause of pediatric urinary tract infection. We report the first case of urinary tract infection caused by S. dysenteri, which presented as acute pyelonephritis without gastrointestinal symptoms in a child.

A Case of The Successful Treatment of Pentobarbital for Posthypoxic Action Myoclonus(Lance-Adams Syndrome) with Refractory Status Epilepticus

Lance-Adams syndrome is a rare complications of cardiorespiratory arrest. We report a 15-month-old child with Lance-Adams syndrome who fell into a refractory status epilepticus after a successful resuscitation after the cardiopulmonary arrest from upper airway obstruction due to the croup. On the day of the admission, he went through a generalized tonic-clonic seizure with poor mental status. His condition became aggravated on 18th day of admission when he developed focal myoclonus in his left arm and leg. The EEG findings before the pentobarbital treatment show partial electrical seizure. The seizures were intractable despite the administration of midazolam, phenobarbital, phenytoin, and valproic acid. Therefore, a pentobarbital(PTB) therapy was required. PTB administered by a continuous infusion pump at a loading dose of 5 mg/kg was sufficient to produce a burst suppression pattern and a seizure control, followed by a maintenance infusion of 0.5-1.5 mg/kg/hour. At that time, the brain MRI showed a diffuse distribution of high signal intensity and swelling in both basal ganglia, thalamus and the temporo-occipital area. The therapy continued for 10 days. Thereafter, PTB was gradually tapered after a minimum 48-hours of a seizure-free status. So we report a case with a brief review of related literature.

A Two-Month-Old Infant with Subglottic Hemangioma / 소아알레르기및호흡기학회지

Subglottic hemangioma is a rare condition which has the potential to cause life-threatening complications during the pediatric period. The most common presenting symptom is inspiratory and expiratory stridor, which is often exacerbated by crying and upper respiratory tract infection. The natural history of subglottic hemangioma is characterized by progressive airway obstruction during the proliferative phase for the first year, but after 1 year of age, the symptoms resolve spontaneously as the lesion regresses. We report a 50-day-old infant with subglottic hemangioma who suffered from episodes of cyanosis and persistent stridor with viral pneumonia. In addition, she had cutaneous hemangioma on her right cheek. The hemangioma was successfully controlled with just systemic steroids, because the lesion occupied 40% of total subglottic space. We concluded that if an infant with cutaneous hemangioma displays biphasic then stridor subglottic hemangioma should be included in differential diagnosis.

Revaluation of Neonatal Screening Test for Congenital Adrenal Hyperplasia / 대한소아내분비학회지

PURPOSE: Newborn screening based on measurement of 17alpha-hydroxyprogesterone (17-OHP) in a dried blood spot on filter paper is an effective tool for early diagnosis of congenital adrenal hyperplasia (CAH) due to 21-hydroxylase deficiency. High levels of 17-OHP are frequently observed in premature infants without CAH. The purpose of this study is to evaluate cut-off limits of 17-OHP on the basis of gestational age and birth weight. METHODS: A total of 1,218 newborn were screened for CAH by measuring blood spot 17-OHP concentration. An enzyme linked immunosorbent assay (ELISA) method (Bayer(TM), Neonate 17-OHP Kit, Tokyo, Japan) was used. The values were analyzed on the basis of birth weight and gestational age at birth to decide the appropriate cut-off limits in neonatal mass screening for 21-hydroxylase deficiencies. RESULTS: In the neonatal mass screening for CAH, the median value of 17-OHP in the subject population was 3.13 ng/mL (0-62.3 ng/mL). Median values of 17-OHP of full term infants and premature infants were 2.44 ng/mL (0.03-27.1 ng/mL) and 4.85 ng/mL (0.06-62.3 ng/mL). 17-OHP levels are higher in preterm infants, and decrease in relation to gestational age (P or =2,500 g] infants and low birth weight (BW<2,500 g) infants were 2.57 ng/mL (0-32.40 ng/mL) and 5.02 ng/mL (0.06-62.30 ng/mL). 17-OHP levels increased with decreasing birth weight (P<0.01). When we applied 99.5 percentile, the cutoff levels were 18.33 ng/mL in normal birth weight infants and 56.46 ng/mL in low birth weight infants. Recall rates were markedly decreased when these cutoff levels were applied; 30.2-fold decrease in gestational age <30 wks and 8.2-fold decrease in birth weight <1,000 g. CONCLUSION: The screening for CAH using 17-OHP should be considered on the basis of gestational age and birth weight. Recall rate and false positive rate markedly decrease in preterm infants and low birth weight infants when new cutoff levels of 17-OHP are applied.