The prevalence of iron deficiency in later infancy and the toddler years(25% to 40% at 1 year of age) has not decreased remarkably , except in Western countries. The purpose of this study was to 1) determine the relationship between current feeding practices and iron status, and 2) assess compliance to infant feeding instructions. Two groupsof infants were examined. The first group of 302 infants aged 6 to 24months was seen at a well baby clinic while the second group of 135 infants of the same age group was assessed by venipuncture. Cutoff values for laboratory tests were as follows ; hemoglobin<11g/dL, mean corpuscular volume (MCV) <72fl ; red cell distribution width(RDW)>15% ; serum ferritin level<10ng/ml ; and transferrin saturation (serum iron(TIBC)<10%. The diagnosis of iron deficiency anemia (IDA) was made when a low hemoglobin level was associated with either low ferritin orlow transferrin saturation . Of the 302 children brought to the well baby clinic , 12.3%(n=37) were found to have anemia (hemoglobin<11.0/dL). In terms of children grouped according to feeding practices, it was found that children with anemial comprised 32.0% (24/75) of the prolonged breast-fed group (Group A), significantly more than the 4.0%(7/176) of the artificial milk feeding group(Group B). and 3.9%(2/51) of the switched from breast milk to iron -fortified weaning foods group(Group C).Among the 107 children with IDA , iron deficiency in 105 children(98.1%) was suggested by their dietary histories ; exclusive or prolonged breast-feeding for more than 6 months without iron fortification in 98 infants ; cow's milk consumption> 500ml/day without iron fortification during infancy(n=12), or >800ml without iron-fortified foods after infancy(n=15) ; and the use of unfortified forumula or unbalanced diets, mainly limited to rice gruel. Despite the relatively high (79.6%) motivation on the part of the infants mothers and supervison by professional personnel, the poor results in the infants receiving iron fortified foods were due to poor compliance(85.75). Among the mothers of 98 IDA patients who were contacted by telephone , it was revealed that 29% did not give the oral iron preparation for more than 2 months. Furthermore, negligence or disregard by the parents occurred in 14% of the case , discontinuance of the oral iron preparation by the parents due to side effects occurred in 6%, and the children's refusal or poor oral intake and no further trial occurred in 6%. The dietary history of a large group of infants was highly predictive of their risk for anemia . Continued consumption of breast milk until the age of 1 year is not warranted unless iron-fortified foods are given concomitantly. Because there is a problem with compliance, more successful and safe strategies for preventing iron deficiency woold included dual coverage in the from of therapeutic iron supplementation as well use of iron-fortified foods for teddlers who are at risk of iron deficiency.
Alport syndrome (ATS) is an inherited glomerular disease caused by mutations in one of the type IV collagen novel chains (${\alpha}3$, ${\alpha}4$, and ${\alpha}5$). ATS is characterized by persistent microscopic hematuria that starts during infancy, eventually leading to either progressive nephritis or end-stage renal disease. There are 3 known genetic forms of ATS, namely X-linked ATS, autosomal recessive ATS, and autosomal dominant ATS. About 80% of patients with ATS have X-linked ATS, which is caused by mutations in the type IV collagen ${\alpha}5$ chain gene, COL4A5. Although an 80% mutation detection rate is observed in men with X-linked ATS, some difficulties do exist in the genetic diagnosis of ATS. Most mutations are point mutations without hotspots in the COL4A3, COL4A4, and COL4A5 genes. Further, there are insufficient data on the detection of COL4A3 and COL4A4 mutations for their comparison between patients with autosomal recessive or dominant ATS. Therefore, diagnosis of ATS in female patients with no apparent family history can be challenging. Therefore, in this study, we used whole-exome sequencing (WES) to identify mutations in type IV collagen in 2 girls with glomerular basement membrane structural changes suspected to be associated with ATS; these patients had no relevant family history. Our results revealed de novo c.4688G>A (p.Arg1563Gln) and c.2714G>A (p.Gly905Asp) mutations in COL4A5. Therefore, we suggest that WES is an effective approach to obtain genetic information in ATS, particularly in female patients without a relevant family history, to detect unexpected DNA variations.
Patients with cleft lip and palate (CLP) must undergo corrective surgeries during infancy and early childhood. Many patients with CLP undergo orthognathic surgery during their childhood for correction of skeletal asymmetries or pharyngoplasty with a pharyngeal flap to improve the quality of speech and velopharyngeal function. During orthognathic surgeries, nasotracheal intubation is performed under general anesthesia. In our case report, the patient had undergone palatoplasty and pharygoplasty previously. During the orthognathic surgery, a flexible fiberoptic bronchoscope-guided nasotracheal tube was inserted through the pharyngeal flap ostium; however, active bleeding occurred in the nasopharynx. Bleeding occurred because the flap was torn. After achieving hemostasis, the surgery was completed successfully. Thus, if a patient may show the potential for velopharyngeal port obstruction, nasotracheal intubation should be performed with utmost care.
There appears to be significant problems remained in the treatment of thoracic empyema inspire of the more potent broad spectrum antimicrobial agents available and improved surgical managements. Clinical analysis of 60 patients of thoracic empyema was done who received major and/or minor surgical intervention at the Dept. of Thoracic and Cardiovascular Surgery, Korea University Hospital in the period of 3 years from May 1973 to July 1976. Following was the results: 1. Male was predominent to female with the ratio of 2.16 to 1, and adult to infant was 2.75: 1. 2. Most frequent predisposing factors of thoracic empyema in infancy and childhood was pneumonia [38.3%], and in adult was pulmonary tuberculosis [36.7%]. 3. Cardinal symptoms were dyspnea, fever, chest pain and productive cough etc. 4. Positive result of bacteriological culture study was reported in 26. 7%, and among them, Staph. aureus [10%], Diplo. pneumonia [3.3%], Streptococcal group [1.7%], Pseu. aeruginosa [1.7%], Paracolon bacilli [1.7%] and others [8.3%] in respectively. No growth of pathologic organism was reported in 60.0%, and not requested in 13.3%. Among the negative group, tuberculosis was confirmed on pathological tissue slide in half and other half was not determined bacteriologically and pathologically. 5. Surgical treatment was performed to the almost all of the patients with closed thoracostomy [48.3%], empyemectomy [28.3%], empyemectomy with pulmonary resection [11.7%] and others [I1.6%] in respectively with favorable results. 6. Mortality rate was 8.3% [5 cases], each one with lung cancer, diabetes mellitus, respiratory insufficiency, sepsis and another, two months old infant with asphyxia.
Thyroglossal duct cyst is ectodermal remnant, which may develop along the line of descent of the thyroid gland from the foramen cecum of the tongue to the pyramidal lobe of the thyroid gland. Meticulous dissection of the cyst and duct, along with the body of the hyoid bone is necessary to avoid recurrence. Eighty-one patients with thyroglossal duct cyst treated at Hanyang University Hospital between January 1980 and December 2000 were reviewed to determine the incidence and to analyze the result of management. The male-to-female ratio was 1.4:1(47:34) with a male preponderance. They are most commonly present at 3-8years(54.2 %) of age, but rarely present at infancy. The most common symptom was a painless midline neck mass(76.5 %, 62cases). Eighty-one patients underwent modified Sistrunk operation without evidence of recurrence. Eight-nine percent(72 cases) of these lesions were located between thyroid substance and hyoid bone, and 11 %(9 cases) were above the hyoid bone. There were 22 infected cysts(27.2 %). The Sistrunk operation is a gold standard for treating the thyroglossal duct cysts. For best results in thyroglossal duct cyst surgery, one should make every effort to remove the cyst intact in continuity with the body of the hyoid bone. In our institute, Sistrunk operation modified by the authors showed a good result.
Coarctation of aorta is rather common congenital cardiovascular defect in the western countries, but it is known to be rare in Korea. And no coarctation of Aorta has been reported and operated upon during early infancy in Korea. During 4 months period from May 1984 to September 1984, four small infants of coarctation of aorta were seen and treated surgically by subclavian flap aortoplasty in Guro Hospital, Korea University. All infants were male, ranging from 7 days to 54 days old [mean 29 days], weighing between 3.2 Kg and 5.0 Kg [mean 4.1 Kg], and all were in congestive heart failure. Examination of the femoral artery pulse gave in all cases clues to the diagnosis. By Two dimensional echocardiogram, detail anatomical features as well as the diagnosis were revealed and associated anomalies, for example, ventricular septal defect could be seen. Besides one case of isolated coarctation of aorta, other three infants had associated VSD. The 3 patients with VSD were treated by pulmonary artery banding in addition to aortoplasty. The postoperative course were smooth in all patients. In conclusion, coarctation of aorta may not be so rare in Korea as we thought previously. The importance of femoral pulse examination can not be overemphasized for the diagnosis. As we experienced, two-dimensional echocardiography in most cases can substitute those invasive examinations such as angiography and catheterization which carry difficulty and risk in infants.
Empyema is a severe infection encountered in the pediatrics. With advance of the antibiotics and chemotherapeutics, there was a marked decrease in number of empyema. Empyema complicated by staphylococcal pneumonia in infant and children has been distressing problem, and the management of this complication has been discussed repeatedly in the past. In Korea, tuberculous empyema is also troublesome. If empyema is localized within thick capsule, tube thoracostomy and closed drainage alone is unacceptable, and early open thoracotomy to eliminate the empyema has proved good result. A clinical analysis of 39 patients with thoracic empyema was done. They were managed surgical intervention at Dept. of Thoracic & Cardiovascular Surgery at Kyung-Hee University Hospital from Jan. 1974 to December, 1984. 1. Age and sex distribution, infancy 9, early childhood 11. late childhood 9, puberty 10. The male to female ratio was 21:18. 2. The highest seasonal incidence was winter [21 cases]. 3. Cardinal symptoms were cough [76%], fever and chill [66%], and dyspnea [40%]. 4. The location of the empyema was right in 27 cases [69%] and 12 cases in left side. 5. The most frequent lesion to predisposing factor was pneumonia [67%]. 6. The commonest organism was Staphylococcus aureus in 15 [38%] cases, and Mycobacterium tuberculosis in 10 cases [26%]. 7. The surgical treatment was performed in all patients. The surgical procedure was closed tube thoracostomy in 25 cases [64%], decortication in 7 cases [18%], pulmonary resection in 4 cases [10%], and decortication with curettage in 2 cases. 8. One patient died from sepsis complicated by lymphoma and in one patient bronchopleural fistula was developed postoperatively.
Lipoblastoma is a rare benign soft tissue tumor occurring in infancy and early childhood. It is characterized by fat lobules with varying degrees of maturity, multivaculoated lipoblasts, fibrocapillary networks and myxoid stroma. Lipoblastoma has a good prognosis with no metastases despite its potential for local invasion. From Jan, 1990 through April, 2007, 12 children underwent the operation for lipoblastoma, 7 boys and 5 girls, diagnosed at median 22 months (5 ~ 43 months). Median follow up was 6 year 7 months. Primary sites included back (n = 5), intraabdominal (n = 2) and one in each of buttock, chest wall, neck, nose and scalp. Tumors presented with a growing mass in 9 patients, abdominal distension in 2, and an incidental finding on chest radiography in one. Complete excisions were done in all patients. There was one recurrence in a patient with a scalp mass. After reoperation, he has been doing well without evidence of recurrence. Lipoblastoma has a favorable prognosis, but recurrence can occur even with complete excision. Regular follow up is necessary to detect recurrences.
The congenital cystic adenomatoid malformation of the lung consists of an enlarged, meaty, multicystic lobe with smooth-walled cysts of varying sizes, which can communicate with major bronchi through malformed air passages that usually lacks cartilage. This abnormality is usually symptomatic in infancy with signs of respiratory distress such as tachypnea, substernal retraction and cyanosis. Prompt surgical resection is choice of treatment in life-threatening respiratory distress patients. We recently experienced a case of congenital cystic adenomatoid malformation of the lung in a patient with situs inversus totalis. The patient was 40 days old female who showed severe respiratory difficulty. Emergency left middle lobectomy was undergone successfully. Her postoperative course was uneventful. She was discharged from hospital on the postoperative eighth day in good condition.
Congenital cystic adenomatoid malformation [CCAM] of the lung is a rare but often fatal congenital lung bud anomaly that causes acute respiratory distress in the newborn infants. In those who survive infancy and early childhood, its clinical manifestations are not unusual to detect. Recently we experienced two children with CCAM; one with coughing and high fever, another without any symptom On case I, the patient was a 9 year-old female, and was suffered from coughing and high fever since 15 days before. Medical treatment was failed and then, exploratory thoracotomy was performed under the impression of loculated empyema. Finally, a right lower lobectomy was done with a satisfactory postoperative course. On case II, the patient was a 12 year-old female, and admitted for evaluation of known large pulmonary cyst which was checked at the outpatient clinic. Preoperative evaluations gave no more informations, so obliteration of cysts was performed with open biopsy. Two patients with CCAM are described in Korean Univ. Medical Center.
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