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1.
Archives of Plastic Surgery ; : 82-84, 2013.
Article in English | WPRIM | ID: wpr-162723

ABSTRACT

No abstract available.


Subject(s)
Football , Korea
2.
Journal of the Korean Society of Plastic and Reconstructive Surgeons ; : 809-816, 2003.
Article in Korean | WPRIM | ID: wpr-80442

ABSTRACT

Apoptosis is a physiologic or programmed cell death process which is controlled by genes and it is essential for the function and the appropriate development of multicellular organism. Apoptosis is also thought to be one of the main mechanisms of cell death in ischemic tissues. The effect of prostaglandin E1(PGE1) is proven to be useful in the recovery of ischemic changes by inducing vasodilation of peripheral vessels and platelet disaggregation. Prostaglandin is also known to suppress apoptosis in a serum deprived cell. The purpose of this study is to evaluate the effects of PGE1 on the apoptosis in the ischemic skin island flap. Thirty Sprague-Dawley rats were used. In control group(n=15), 3x5cm sized skin island flap based on the superficial epigastric vessel was elevated and its pedicle was occluded for 14 hours. After removing the vessel clamp, skin flap was reperfused for 5 hours and harvested. In experimental group(n=15), a ischemic skin island flap was also made as in the control group except the interarterial administration of the PGE1 right after elevation of the flap and after removing the clamp. H&E, TUNEL and immunohistochemical stains for p53 and bax proteins were performed. There were ischemic changes in gross and microscopic findings in both groups. Immunohistochemical staining for p53 protein shows many positive cells with nuclear staining in squamous epithelium of the control group, but sparse positive cells in the experimental group. Immunohistochemical stainings for bax protein shows many positive cells with cytoplasmic staining in squamous epithelium of the control group, but sparse positive cells in the experimental group. The apoptotic index was significantly lower in the experimental group(2.39+/-1.76(p=0.0001)) than in the control group(7.53+/-2.05). These data indicate that PGE1 suppresses the apoptosis in the ischemic skin island flap.


Subject(s)
Animals , Rats , Alprostadil , Apoptosis , bcl-2-Associated X Protein , Blood Platelets , Cell Death , Coloring Agents , Cytoplasm , Epithelium , In Situ Nick-End Labeling , Prostaglandins I , Rats, Sprague-Dawley , Skin , Vasodilation
3.
Journal of the Korean Child Neurology Society ; : 122-128, 2001.
Article in Korean | WPRIM | ID: wpr-112639

ABSTRACT

PURPOSE: Children with developmental language disorder occupy considerable health care resources, particularly in the preschool ages. The exact size of problem for health planning remains somewhat problematic, as differences in reported estimates of prevalencies reflect the range of definitions used. In this study, we evaluated the clinical features of children with developmental language disorder and examined effctiveness of the Gesell Screening Inventory and REEL scale to assess language development. METHODS: The 171 children were involved in the study, they were referred to Child Development Clinic in Kangnam St. Mary's Hospital for evaluation of suspected language problem. Among the children, 75 cases between 15 and 54 months of age were included as developmental language disorder in this study. RESULTS: 1) The mean age of subjects was 31.4 months and the ratio of male to female was 6.5:1. 2) The mean gestational age of the subjects was 38.8 weeks. The proportion of full-term infants was 82.7% and the one of premature infants was 17.3%. 3) According to the birth ordering history, the proportion of the first baby was 82.7%, the one of second baby was 13.3% and the one of third baby was 4%. 4) Ten children had been checked up the MRI, four of them had abnormal findings, 23 children had been checked up the EEG and two of them showed abnormal findings. 5) The mean age of first spontaneous word with used meaning was 13.8 months. 6) According to the Gesell Screening Inventory, the proportion of children who had less than DQ 70 had showed 4% in motor sector, 17% in adaptive sector, 66.7% in laguage sector and 33.3% in personal social sector. 7) According to the REEL Scale, the proportion of children who had less DQ 70 had showed 70.7% of in receptive language sector and 90.7% in expressive language sector. 8) The proportion of the children who had been maintaining language disorder was 17.3% and that of those who showed normal linguistic development was 82.7% on the follow-up test 6 months later. CONCLUSION: The large proportion of the children who had language delay was developmental language disorder without organic problem. Most of them showed symptom improvement on the follow up test 6 months later.


Subject(s)
Child , Female , Humans , Infant , Infant, Newborn , Male , Child Development , Delivery of Health Care , Electroencephalography , Follow-Up Studies , Gestational Age , Health Planning , Infant, Premature , Language Development , Language Development Disorders , Language Disorders , Linguistics , Magnetic Resonance Imaging , Mass Screening , Parturition
4.
Korean Journal of Nephrology ; : 593-605, 2001.
Article in Korean | WPRIM | ID: wpr-116374

ABSTRACT

Recent studies in the developing rat kidney have demonstrated that renin in juxtaglomerular cells and aquaporin-1(AQP1), a kind of water channel, participate in the development of renal arterial system. The purpose of this study was to identify the association among renin and AQP1 in the developing renal arterial system. Sprague-Dawley rat kidneys from 14-, 16-, 18- and 20-day-old fetuses, and 1-, 4-, 7-, 14-, 21- and 28-day-old pups were preserved with periodate-lysine-2% paraformaldehyde solution for single or multiple immunohistochemistry. Renin- positive, smooth muscle, and endothelial cells were detected using renin polyclonal(1 : 5,000), alpha-smooth muscle actin(ASMA) monoclonal(1 : 1,000), and AQP1 polyclonal(1 : 500) antibodies, respectively. Immunoreactivity for renin and AQP1 was not detected in the developing vessels in fetal kidneys on the 14 th day of gestation. At the 16th day of gestation, AQP1 appeared in the developing kidney. Immunoreactivity for AQP1 was observed in endothelial cells of the arterial capillary plexus and of the arcuate artery, but not in the venous capillary plexus or the arcuate vein. At the 18th day of gestation, renin-positive cells appeared throughout the arterial system including the arcuate artery. After birth, immunoreactivity for renin and AQP1 was gradually decreased in the developing artery. No renin immunoreactivity was detected in the arcuate artery from 7 days after birth, and in the interlobular artery from 14 days after birth. Renin immunoreactivity was confined to juxtaglomerular cells in the afferent arteriole from 21 days after birth. AQP1 immunoreactivity in endothelial cells was not observed in the arcuate artery from right after birth, or in the interlobular artery, or afferent arteriole from 14 days after birth. This study indicates that the expression and loss of AQP1 in endothelial cells spatiotemporally coexists with renin in smooth muscle cells during the development of arterial system in the rat kidney. It is suggested that AQP1 and renin might play an important role in the development and growth of the rat kidney arterial system by functioning through a paracrine or autocrine mechanism.


Subject(s)
Animals , Pregnancy , Rats , Antibodies , Arteries , Arterioles , Capillaries , Endothelial Cells , Fetus , Growth and Development , Immunohistochemistry , Kidney , Muscle, Smooth , Myocytes, Smooth Muscle , Parturition , Rats, Sprague-Dawley , Renin , Veins , Water
5.
Korean Journal of Nephrology ; : 263-269, 2001.
Article in Korean | WPRIM | ID: wpr-17002

ABSTRACT

During a 4 year period, 118 children(62 boys) below 14 years of age(median 2.9+/-2.7 years) with symptomatic urinary tract infection were studied by 99mTc-dimercaptosuccinc acid scintigraphy(DMSA scan), ultrasonography and voiding cystoureterography(VCUG). The male to female ratio was 1.1 : 1, and 2.4 : 1 under 1 year of age, and 0.54 : 1 above 1 year age, respectively. Forty-seven patients presented with positive urine cultures and 71 were negative. DMSA scan was performed in 108 patients, and showed abnormalities in 50(46.3%) of them. Renal scars were observed in 24(51.0%) of 47 patients with positive urine culture and 26(42.6%) of 61 patients with negative urine culture, however there was no statistical difference between the two groups. Of the 118 patients VCUG was performed in 92 patients. Vesicoureteral reflux was found in 29(31.5%) of them. VURs were found in 25(50.0%) of 50 patients with positive and 4(9.6%) of 42 patients with negative findings in DMSA scan. Also, DMSA scan demonstrated scarring in 11(64.7%) of 17 renal units with grade I-II, 12(92.3%) of 13 renal units with grade III, and 9(90.0%) of 10 renal units with grade IV-V. The incidence of positive findings in DMSA scan was higher above grade III. VURs were found in 21 (52.5%) of 40 patients with abnormal findings in ultrasonography and 8(15.3%) of 52 patients with normal findings. Ultrasonographic findings were abnormal in 29(58.0%) of 50 patients with positive findings in DMSA scan and were normal in 47(81.0%) of 58 patients with negative findings. Our study shows that DMSA scan is useful in the diagnosis of acute pyelonephritis and detection of VUR. It is advisable that DMSA scan should be included as an initial evaluation tool for symptomatic urinary tract infection in children and used as a follow-up tool.


Subject(s)
Child , Female , Humans , Male , Cicatrix , Diagnosis , Follow-Up Studies , Incidence , Pyelonephritis , Succimer , Technetium Tc 99m Dimercaptosuccinic Acid , Ultrasonography , Urinary Tract Infections , Vesico-Ureteral Reflux
6.
Journal of the Korean Society of Plastic and Reconstructive Surgeons ; : 78-82, 2000.
Article in Korean | WPRIM | ID: wpr-8992

ABSTRACT

Tretinoin(all-trans retinoic acid) is a metabolite of vitamin A and it is useful in the treatment of photoaging skin. Photoaging skin is characterized by wrinkles, mottled pigmentation, dry and rough skin, and loss of skin tone. Current use of topical tretinoin mainly acts on the epidermis, requires a long period to obtain the desired results and may cause skin hyperpigmentation. A combination of topical and injectable tretinoin has been used to reduce the treatment period as a result of its potentialized effect on the dermis when compared to graditional topical cream use. in this study, we observed histologic alterations in 5 white rabbits after using 0.05% topical tretinoin cream and 0.1% injectable tretinoin. Tretinoin was treated on the rabbits ears-group 1 and 2 on the right ear for study 1, and group A and B on the left ear for study 2. Study 1 was done to differentiate whether the dermal thickening is due to the simple physical stretching of dermis by intradermal injection, or whether it is duer to the histologic change by tretinoin. In group 1, saline was injected intradermally and in group 2, tretinoin was injected intradermally. Study 2 was done to compare the dermal thickening between the topical tretinoin cream treatment group (group A) and the combined topical and injectable tretinoin group (group B). Injection was done once a week immediately followed by 340nm blue light skin exposure. These treatment were done for 12 weeks. We harvested skin stripe from all group, group 1 and 2, and group A and B respectively, after 2, 6, and 12 weeks after treatment. Histologic differences were observed and measured. Dermal thickening was observed in group 2 and in group B(p<0.05). The results showed that intradermal injection of tretinoin mainly acts on the dermis and potentialtes the effect on photo-aging skin and fine wrinkles.


Subject(s)
Rabbits , Dermis , Ear , Epidermis , Hyperpigmentation , Injections, Intradermal , Pigmentation , Skin , Tretinoin , Vitamin A
7.
Korean Journal of Pediatric Gastroenterology and Nutrition ; : 222-226, 2000.
Article in Korean | WPRIM | ID: wpr-741337

ABSTRACT

Common bile duct stones are an unusual occurrence in children. Endoscopic retrograde cholangiopancreaticography and endoscopic papillary sphincterotomy are excellent tool for diagnosis and therapy. Bleeding after endoscopic papillary sphincterotomy occurs in approximately 0.5∼12% of procedures. We experienced a case of common bile duct stone in 5-year-old boy. After endoscopic papillary sphincterotomy, the stone was passed. 5 days after the procedure bleeding occurred, but it was controlled spontaneously.


Subject(s)
Child , Child, Preschool , Humans , Male , Common Bile Duct , Diagnosis , Hemorrhage
8.
Journal of the Korean Pediatric Society ; : 1471-1474, 1999.
Article in Korean | WPRIM | ID: wpr-113216

ABSTRACT

Marden-Walder syndrome is characterized by a mask-like face, blepharophimosis, joint contractures, and psychometer retardation. We report a newborn infant with the clinical features of the syndrome. He was diagnosed with clinical findings of mask-like face, blepharophimosis, micrognathia, camptodactyly, arachnodactyly, multiple contractures, and hypotonia. The infant died of aspiration pneumonia at 5 months. A brief review of related literature is also presented.


Subject(s)
Humans , Infant , Infant, Newborn , Arachnodactyly , Blepharophimosis , Contracture , Joints , Muscle Hypotonia , Pneumonia, Aspiration
9.
Korean Journal of Nephrology ; : 989-993, 1999.
Article in Korean | WPRIM | ID: wpr-87849

ABSTRACT

Although spontaneous bacterial peritonitis is a frequent complication in the childhood nephrotic syndrome, it is very rare in adults with nephrotic syndrome. It frequently develops when the patients are either in relapse or receiving steroid therapy at the time peritonitis is diagnosed. We report an unusual case of a spontaneous bacterial peritonitis as the presenting feature in a 15-year-old male patient with nephrotic syndrome. He presented with diffuse abdominal pain and distension for 15 days. Abdominal paracentesis revealed the diagnostic laboratory findings of peritonitis, and the bacterial culture of the ascites showed a mixed growth of Escherichia coli and Pseudomonas aeruzinosa. His serum albu- min level was 1.6gldL and the amount of 24 hours proteinuria was 21.0g/day. Although he was treated with adequate antibiotics for 3 weeks, the peritonitis was more aggravated. We decided to insert a catheter into the peritoneal cavity for continuous drainage of the intractable ascites. Two weeks after drainage, the peritonitis improved as the peritonitis subsided, the proteinuria disappeared completely without a steroid therapy. Six months after spontaneous remission, the proteinuria have recurred, and the kidney biopsy then showed focal segmental glomerulorsclerosis.


Subject(s)
Adolescent , Adult , Humans , Male , Abdominal Pain , Anti-Bacterial Agents , Ascites , Biopsy , Catheters , Drainage , Escherichia coli , Kidney , Nephrotic Syndrome , Paracentesis , Peritoneal Cavity , Peritonitis , Proteinuria , Pseudomonas , Recurrence , Remission, Spontaneous
10.
Journal of the Korean Pediatric Society ; : 733-737, 1999.
Article in Korean | WPRIM | ID: wpr-7733

ABSTRACT

Chylothorax is an accumulation of lymphatic fluid or chyle in the pleurual cavity resulting from a leak of the thoracic duct or one of its major divisions. If the loss of chyle persist, life threatening nutritional and immunologic deficiencies ensue. Initial conservative managements consist of tube thoracostomy drainage and dietary modification(low fat diet and total parenteral nutrition). In some refarctory cases, surgical intervention is required. Pleuroperitoneal shunt is an alternative surgical method recommended prior to thoracic duct ligation. The shunted fluid in the peritoneal cavity is reabsorbed by peritoenal lymphatic vessels running to join the right lymphatic duct. We experienced a case of chylothorax in a two-month-old female infant. Because there was no underlying disease to cause chylothorax, the diagnosis of idiopathic chylothorax was made. She failed to respond to conservative management and showed severe hypoalbuminemia and lymphopenia. On day 22, a pleuroperitoneal shunt was inserted between the right pleural cavity and the peritoneal cavity. Three weeks after insertion of the shunt, there was no fluid in the right pleural space. After one more week of observation, the shunt was removed.


Subject(s)
Female , Humans , Infant , Chyle , Chylothorax , Diagnosis , Diet , Drainage , Hypoalbuminemia , Ligation , Lymphatic Vessels , Lymphopenia , Peritoneal Cavity , Pleural Cavity , Running , Thoracic Duct , Thoracostomy
11.
Journal of the Korean Child Neurology Society ; : 351-355, 1998.
Article in Korean | WPRIM | ID: wpr-225954

ABSTRACT

Miller-Dieter syndrome consists of severe type I lissencephaly, abnormal facial appearance, and sometimes other birth defects. Lissencephaly is a brain malformation manifested by a smooth cerebral surface, thickened cortical mantle, and microscopic evidence of incomplete neuronal migration. It comprises the agyria-pachygyria spectrum of malformation, thus excluding polymicrogyria and other cortical dysplasia. Type I lissencephaly results from abnormal migration between about 10 and 14 weeks gestaion. The brain is often small, and the ventricle is enlarged posteriorly The corpus callosum may be small or absent. The structural pattern of the cerebral hemispheres and ventricles is distintly immature, reminiscent of fetal brain. The superficial cellular layer resembles an immature cortex, with some separation into zones similar to layers III, V, and VI of normal cortex, although the cell population is decreased. In 1963 Miller described a malformation syndrome in a brother and sister with postnatal growth deficiency, craniofacial defects, and serious abnormalities of neurologic function. Autopsy at 3 and 4month of age, respectively, revealed lissencephaly. Subsequently, Dieker reported four additional patients with this disorder and referred to it as the 'lissencephaly syndrome'. We have experienced a case with this syndrome. Then we report this rare case with brief review of literature.


Subject(s)
Humans , Autopsy , Brain , Cerebrum , Congenital Abnormalities , Corpus Callosum , Lissencephaly , Malformations of Cortical Development , Neurons , Siblings
12.
Journal of Korean Society of Pediatric Endocrinology ; : 219-227, 1998.
Article in Korean | WPRIM | ID: wpr-42960

ABSTRACT

Pseudohypoparathyroidism(PHP) is a genetic disorder characterized by target cell resistance to the effect of parathyroid hormone(PTH). The disorder is classified into type I a, I b, I c and II depending on the phenotype and biochemical findings. In type I a, urinary cyclic AMP and urinary phosphate excretion are not increased after PTH stimulation because of deficient G unit activity in target cells. Deficiency of the G unit is a generalized cellular defect and accounts for the association of other endocrine disorders with type I a PHP. Type I b PHP shows resistance to PTH but not to other hormones and normal phenotypic appearance. In type I c PHP affected children have defect in catalytic unit of adenylate cyclase and in addition to resistance to PTH, resistance to the metabolic effects of TSH, gonadotropins, and glucagon may be detected. Typical appearance of Albright's hereditary osteodystrophy is common in PHP type Ia and Ic. In type II PHP, urinary cyclic AMP response is generated but this does not lead to phosphaturia. We experienced two patients with PHP. One is a 11-year-old girl diagnosed type I a PHP and the other is a 11-year-old boy suspected type I b PHP. They visited emergency room because of tetanic movement. Both patients had no previous history of tetany and showed low serum calcium level, high phosphorus level and high PTH level. The girl had typical features of Albright's hereditary osteodystrophy such as round face, short neck, obese feature, brachydactyly and mental retardation but didn't have basal ganglia calcification on brain CT. The boy showed normal appearance and no mental retardation.


Subject(s)
Child , Female , Humans , Male , Adenylyl Cyclases , Basal Ganglia , Brachydactyly , Brain , Calcium , Cyclic AMP , Emergency Service, Hospital , Glucagon , Gonadotropins , Hypophosphatemia, Familial , Intellectual Disability , Neck , Phenotype , Phosphorus , Pseudohypoparathyroidism , Tetany
13.
Journal of the Korean Society of Pediatric Nephrology ; : 196-199, 1998.
Article in Korean | WPRIM | ID: wpr-109703

ABSTRACT

Fine needle aspiration cytology of the salivary lesions was performed on 221 patients at Soonchunhyang University Hospital for 10 years. Of 221 aspirates, 6 aspirates(2.7%) were inadequate, 116 cases(52.5%) were non-neoplastic lesions, 76(34.4%) cases were benign neoplasms and 23 cases(10.4%) were malignant neoplasms. The cytologic diagnoses could be correlated with histologic findings in 58 cases. FNAC correctly discriminated between neoplastic and nonneoplastic lesions in fifty-seven lesions and failed in a case, and overall accuracy, sensitivity, and specificity were 98.3%, 98.0%, and 100.0%. FNAC correctly discriminated malignant neoplasms from benign neoplastic/non- neoplastic lesions in fifty-three cases and failed in five cases, and overall accuracy, sensitivity, and specificity were 91.3%, 72.7%, and 95.7%. Among three false negative cases, two mucoepidermoid carcinomas were misdiagnosed as mucocele and benign neoplasm, and an acinic cell carcinoma were misdiagnosed as Warthin's tumor. Two false positive cases were a Warthin's tumor misdiagnosed as squamous cell carcinoma and a pleomorphic adenoma misinterpretated as suggestive of malignancy. In conclusion, diagnostic accuracy of FNAC of salivary lesions is high, and the possibilities of low grade mucoepidermoid carcinoma and acinic cell carcinoma should be considered on hypocellular smears with mucoid or fluidy background.


Subject(s)
Adenoma
14.
Journal of the Korean Pediatric Society ; : 719-723, 1998.
Article in Korean | WPRIM | ID: wpr-119981

ABSTRACT

Movement disorders (chorea, athetosis, ballism) are a rare complication that develops during the course of bacterial meningitis although associations with tuberculous meningitis are established to some extent. Movement disorders are generally believed to reflect injury to the basal ganglia, thalamus, cerebellum and cerebral cortex. Ischemic infarctions of these areas have been proposed as the cause of dyskinesias in bacterial meningitis. We experienced a case of chorea which developed in the recovery phase of pnemococcal meningitis in a 37-month-old boy. The choreic movement was initially misinterpreted as a seizure, and anticonvulsants were administered. The movement continued during the alert state in spite of anticonvulsant therapy but subsided during sleep. EEG showed severely depressed background activitiy with no epileptiform discharge. When the movement was recognized as a choreic movement, anticonvulsants were withheld and haloperidol was administered orally. The abnormal movement gradually disappeared in 3 months. But like the two other previously reported cases of postmenigitic movement disorders, this patient shows severe impairment in cognitive and motor function in the 10 months of follow up. He can only recognize some close persons and can not control the trunk and extremities well. The movement disorder which develops in the recovery phase and lasts for a long period may be associated with poor neurologic prognosis.


Subject(s)
Child, Preschool , Humans , Male , Anticonvulsants , Athetosis , Basal Ganglia , Cerebellum , Cerebral Cortex , Chorea , Dyskinesias , Electroencephalography , Extremities , Follow-Up Studies , Haloperidol , Infarction , Meningitis , Meningitis, Bacterial , Meningitis, Pneumococcal , Movement Disorders , Prognosis , Seizures , Thalamus , Tuberculosis, Meningeal
15.
Journal of the Korean Pediatric Society ; : 1603-1607, 1997.
Article in Korean | WPRIM | ID: wpr-226242

ABSTRACT

Type IV renal tubular acidosis (RTA) is due to renal tubular bicarbonate wasting associated with mineralocorticoid deficiency. In its five subtypes, IV-4 is due to pseudohypoaldosteronism (PHA) evidenced by increased plasma renin and aldosterone. PHA is believed to result from distal tubular unresponsiveness to circulating aldosterone and has normal renal and adrenal fuction. Hypoaldosteronism can easily be suspected when the patient shows typical electrolyte imbalance (hyponatremia coupled with hyperkalemia) and the diagnosis of PHA is confirmed by elevated serum aldosterone level. But some patients of PHA show negligible electrolyte imbalance, thus metabolic acidosis is a sole abnormal finding in routine laboratory examination. We experienced a case of IV-4 RTA in a 2-month-old male infant who presented with normal anion gap-metabolic acidosis as a sole abnormal finding in routine laboratory examination. RTA was suspected and the test of urine pH during systemic acidosis and fractional excretion of bicarbonate (FEHCO3-) during the condition of normal plasma bicarbonate concentration revealed the disease to be type IV RTA. With elevated plasma renin activity and aldosterone level, the diagnosis of type IV-4 RTA (pseudohypoaldosteronism) was made. Type IV RTA is the most common form of RTA, therefore it is recommended that young infants with suspected RTA should be checked for serum aldosterone level first.


Subject(s)
Humans , Infant , Male , Acidosis , Acidosis, Renal Tubular , Aldosterone , Diagnosis , Hydrogen-Ion Concentration , Hypoaldosteronism , Plasma , Pseudohypoaldosteronism , Renin
16.
Journal of the Korean Pediatric Society ; : 584-587, 1997.
Article in Korean | WPRIM | ID: wpr-142148

ABSTRACT

Citrullinemia is one of the five urea cycle defects and is caused by argininosuccinic acid synthetase deficiency ; conversion of citrulline to argininosuccinic acid is blocked. Severe hyperammonemia typically develops in the neonate within a few days and symptoms such as vomiting, lethargy, convulsion, coma follows rapidly. The diagnosis is supported by high citrulline level in serum, urine, CSF and decreased activity of argininosuccinic acid synthetase in liver biopsy. We experienced a 3-day-old male neonate with poor activity, lethargy, convulsion, and coma who was diagnosed as citrullinemia by markedly elevated plasma and urine citrulline level with hyperammonemia. We report this case with brief review of the related literature.


Subject(s)
Humans , Infant, Newborn , Male , Argininosuccinic Acid , Biopsy , Citrulline , Citrullinemia , Coma , Diagnosis , Hyperammonemia , Lethargy , Ligases , Liver , Plasma , Seizures , Urea , Vomiting
17.
Journal of the Korean Pediatric Society ; : 584-587, 1997.
Article in Korean | WPRIM | ID: wpr-142145

ABSTRACT

Citrullinemia is one of the five urea cycle defects and is caused by argininosuccinic acid synthetase deficiency ; conversion of citrulline to argininosuccinic acid is blocked. Severe hyperammonemia typically develops in the neonate within a few days and symptoms such as vomiting, lethargy, convulsion, coma follows rapidly. The diagnosis is supported by high citrulline level in serum, urine, CSF and decreased activity of argininosuccinic acid synthetase in liver biopsy. We experienced a 3-day-old male neonate with poor activity, lethargy, convulsion, and coma who was diagnosed as citrullinemia by markedly elevated plasma and urine citrulline level with hyperammonemia. We report this case with brief review of the related literature.


Subject(s)
Humans , Infant, Newborn , Male , Argininosuccinic Acid , Biopsy , Citrulline , Citrullinemia , Coma , Diagnosis , Hyperammonemia , Lethargy , Ligases , Liver , Plasma , Seizures , Urea , Vomiting
18.
Korean Journal of Hematology ; : 365-371, 1993.
Article in Korean | WPRIM | ID: wpr-720735

ABSTRACT

No abstract available.


Subject(s)
Leukemia
19.
Journal of the Korean Pediatric Society ; : 1740-1746, 1993.
Article in Korean | WPRIM | ID: wpr-22924

ABSTRACT

In this study I assesed the efficacy and tolerance of cyclosporin A (CyA) in the treatment of steroid-dependent MCNS (minimal change nephrotic syndrome) and steroid resistant FSGS (focal segmental glomerulosclerosis). The results showed, that 1) CyA was effective in sustaining a remission in steroid-dependent MCNS without prednisone treatment, and, therefore, could be an alternative therapeutic choice. 2) After discontinuation of CyA, relapses reoccurred as frequently as before, and the children remained steroid responsive. 3) In steroid resistant FSGS the CyA treatment was followed by a partial remission (normalization of serum albumin) in 2 out of 7 Patients, and proteinuria was diminished in most cases. However, no complete remission with loss of proteinuria was observed. 4) CyA treatment was well tolerated, especially signs of definitive nephrotoxicity were not observed. It was remarkable, the the well being of all patients improved markedly under CyA treatment and that a normal growth was achieved.


Subject(s)
Child , Humans , Cyclosporine , Equidae , Glomerulosclerosis, Focal Segmental , Nephrosis, Lipoid , Prednisone , Proteinuria , Recurrence
20.
Journal of the Korean Pediatric Society ; : 1761-1764, 1993.
Article in Korean | WPRIM | ID: wpr-22921

ABSTRACT

Fetus-in-fetu is a very rare condition in which a malformed monozygotic twin lies within the body of its fellow, usually in the retroperitoneal cavity. Today it is generally accepted that the distinction between fetus-in-fetu and teratoma is largely determined by whether an axial skeleton system is present. We experienced a 6 months female infant who was admitted to our unit because of incidental abdominal mass. a plain abdominal X-ray showed that the mass contained completely formed fetal skeletal system. It was removed by surgery. By pathologic and radiologic study of mass, We confirmed as a fetus-in-fetu. We report the case with brief review of related literatures.


Subject(s)
Female , Humans , Infant , Skeleton , Teratoma , Twins, Monozygotic
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