A Case of Castleman's Disease in Childhood

Castleman's disease(CD) is rare in childhood. It is defined as a localized nodal hyperplasia in mediastinum or cervical area. It is also called angiofollicular lymph node hyperplasia, lymph nodal hamartoma, giant lymph node hyperplasia. It was first described in 1956 by Castleman et al. as a lesion of mediastinal mass. The etiology of CD is not clear. The histologic classification of CD is hyaline vascular and plasma cell type. The hyaline-vascular type is more frequent, and characterized by small hyaline-folliclees and interfollicular capillary proliferation. The plasme cell type is characterized by the large follicles with intervening sheets of plasma cells. The clinical classification of CD is solitary and multicentric type. The solitary type is usually asymptomatic but, the multicentric type is usually combined systemic manifestations, such as fever, anemia, hyperglobulinemia. Complete surgical resection of involved lymph nodes is both diagnostic and therapeutic. The prognosis of solitary type is good, in a general way. We experienced CD cases in five-year-old girl, who had a 4x3 cm solid mass in postrior triangle of neck, right. The mass was removed completely and confirmed Castleman's disease microscopically. The histopathologic finding was a proliferation of germinal centers with hyaline thickening of the wall and the interfollicular stroma showed hyperplastic vessels admixed with lymphocytes, plasma cells and eosinophils. She discharged after six days of operation and her prognosis was good.

Immunologic Considerations in the Pathogenesis of Chronic and Recurrent Sinusitis in Children

PURPOSE: Although the chronic sinusitis is one of the most common and troublesome respiratory diseases in children, the pathogenesis still remains unclear. It is suggested that many of the immunologic factors including allergic conditions may contribute the nasal inflammatory changes. This study was designed to evaluate and demonstrate the possible role of various immunologic factors on the pathogenesis of chronic sinusitis in children. METHODS: During the 6 months study period from March to August, 1995, 33 children with chronic and recurrent clinical symptoms of sinusitis were evaluated. History taking, physical examinatin, paranasal radiologic examination, total IgE, allergen-specific IgE antibodies, serum IgA, IgG subclasses and T cell subsets were analysed in most of the children. RESULTS: The results were as follows; 1) The value of total serum IgE was increased in 19 cases(63.3% of the total). 2) All children in this study shows normal serum IgA levels. 3) In ten of 31 children(74.1%) with chronic sinusitis, serum concentrations of IgG2 were lower than geometric mean value. There was a decreased concentration of IgG1 in 3 cases(21.4%), IgG3 in 4 cases(28.6%), and IgG4 in 2 cases(14.3%). 4) The ratio of T4 to T8 was less than 2 in 23 cases(82.1%) out of 28 cases and reversed in 1 case. 5) Decreased serum concentration of IgG2 was more prevalent in cases with normal serum IgE levels rather than the cases with high serum IgE levels. 6) There was no correlation between the toral serum IgE levels and the ratio of T4 to T8 7) In the case with reversed T4 to T8 ratio, all the IgG subclasses are decreased except IgG3. 8) Chronic cough was the most common clinical manifestations in this study and postnasal drip, purulent nasal discharge, nasal stuffiness, fever and allergic shiner were noted in order. 9) Many of the children in this study have an allergic diseases, such as allergic rhinitis(48.5%), asthma(45.5%), and atopic dermatitis(33.3%). 10) PNS radiographs show diffuse opacification or mucosal thickening in all of the patients. CONCLUSIONS: In this study, we suggested that complex immunologic reactions including IgE-mediated allergic reaction, IgG subclasses deficiencies and cellular immunity are involved in the pathogenesis of chronic and recurrent sinusitis.

The Influence of the Use of Iodine during Perinatal Period on the Screening Test for Congenital Hypothyroidism

PURPOSE: This study was performed to detect the diseases of congenital hypothyroidism by the neonatal mass screening test early and to demonstrate the possible role of topical iodinated antiseptics(povidone-iodine, PVP-I) on transient hyperthyrotropinemia in newborn infants. METHODS: We performed neonatal screening tests for inborn errors of metabolism since 1985 by Guthrie test for PKU, maple syrup urine disease, histidinemia, and homocystinuria and enzyme-linked immunosorbent test for TSH. And for our prospective study, 366 newborns with a appropriate gestational age (between 37 and 42 weeks) were submitted to TSH screening test. We divided the study subjects into 4 groups; the group used PVP-I for mother and alcohol for cord care was categorized as Group A, the group used PVP-I for both mother and cord care as Group B, the group used triple dye for mother and PVP-I for cord care as Group C, and the group used triple dye for mother and alcohol for cord as Group D. RESULTS: 1) By neonatal screening test for inborn errors of metabolism during the last 10 years, we detected one case of phenylketonuria (PKU), one case of histidinemia and 28 cases of congenital hypothyroidism. The incidence of congenital hypothyroidism was 1 in 3,918 live births. 2) The mean TSH levels were 6.25+/-5.09 microU/ml(male 5.85+/-4.77 and female 6.6+/-5.41 microU/ml) in Group A, 8.30+/-7.68 microU/ml(male 6.30+/-7.15 and female 9.98+/-7.77U/ml) in Group B, 7.42+/-5.35 microU/ml(male 7.40+/-6.11 and female 7.44+/-4.63 microU/ml) in Group C, and 6.33+/-1.95 microU/ml(male 6.19+/-1.90 and female 6.42+/-2.03 microU/ml) in Group D, There were statistically significance between Group A and Group B, and Group D, but there was no statistically significance between gender. 3) The number of the newborns showed high levels of TSH over 15 muU/ml were 4.2%, 26.7% and 7.7% in Group A, B, and C, respectively. But nobody showed high TSH level over 15 microU/ml in Group D without use of PVP-I. CONCLUSIONS: In conclusion, we might be concerned for neonatal mass screening test on a national scale to prevent mental retardation and it is necessary to switch the povidone-iodine to other preparations as a perinatal antiseptics because of the possibility of the effect on neonatal screening test for congenital hypothyroidism.

A Case of Congenital Lipoid Adrenal Hyperplasia

Congenital lipoid adrenal hyperplasia is the rarest type among salt losing types of congenital adrenal hyperplasia. The defect of this disorder is in the cholesterol side chain cleavage enzyme(P450SCC)which converts cholesterol to pregnenolone. W experienced a case of 20,22 desmolse deficiency in a 21-day old phenotypically female who was admitted to our hospital due to lethargy and dark skin pigmentation. The characteristic findings were decreased serum cortisol, aldosterone, testosterone, increased ACTH. The ACTH and hCG stimulation test were performed and there were no response. The sex chromosomal analysis showed made XY. One year later after hormone therapy, growth and development are normal.

Intracraial Hemorrhage in Premature and Low Birth Weight Infants by Craniosonography

No abstract available.

A Case of Sjogren-Larsson Syndrome

Sjogren-Larsson Syndrome is a rare disorder chracterized by congenital icthyosis, spastic diplegia or tetraplegia and mental retardation. The inheritance is autosomal recessive, due to deficiency of alcohol dehydrogenase activity of fatty acid matabolism. We have experienced a case of Sjogren-Larsson Syndrome in a 16 month-old male children who have dried thick skin, developmental delay, and spastic diplegia. He also has glistening spot on macula area with irregular high voltage slow wave (2-3Hz) on electroencephalography. From Biopsy finding, Lamella ictyosis with acanthosis and hyperkeratosis in epithelium is shown. He was much improved clinically for skin lesion and developmental status with etretinate supplement. We report the case with brief review of related literatures.

The change of anti-HBs titer after hepatitis B vaccination in newborn according to dosage and time

We studied anti-HBs titer, positive and effective rate in relation to dosages(5microgram, 10microgram) and time interval after third vaccination in 23 infants born to HBsAg negative mother. The babies were divided into two groups. In one group(n=12), 5microgram of Hepavax was administered intramusculary at 1 month, 2 months and 6 months of age, in other group (n=11), 10microgram of Hepavax at same time interval. And the anti-HBs was studied at 2 months and 3 year after third vaccination by radioimmunoassay. The results were as follows: 1) The anti-HBs positive rate was 100% in two groups at 2 months and 3 years after vaccination. 2) The geometric mean anti-HBs titer at 2 months after third vacciantion was 9418.3+/-13041.5 IU/L in 5microgram group and 12750.0+/-12750.5 IU/L in 10microgram group, and 3 year after vaccination, 949.4+/-1404.0 IU/L in 5microgram group, 1067.4+/-1067.7 IU/L in 10microgram group. There were no significant difference between two groups, although mean anti-HBs titer decreased significantly after 3 years. 3) The effective rate at 3 years after vaccination was 66.7% in 5 microgram group and 72.8% in 10microgram group. There was no statistical significant difference between two groups.

Intracranial hemorrhage in full-term neonates by ultrasonography

Intracranial hemorrhage is the most common neuropathologic finding in premature infants. But in full-term infants, it is less common and rarely causes death. We found out intracranial hemorrhages in 21 full-term neonates by real-time neurosonography and concluded as followings. 1) Among 21 neonates, 17 infants were male and 4 infants were female. 2) In 11 (52.3%) infants the hemorrhage was detected within 7 days after birth. 3) The intracranial hemorrhage was not related with delivery type nor Apgar score. 4) In 13 cases (61.9%) the hemorrhage was in the subependymal germinal matrix and the degree was Grade I. 5) Precipitating or associated factors were asphyxia, pneumonia, ventilator care, RDS and congenital heart disease. 6) Symptoms and signs were seizure, apnea, lethargy, cyanosis, jaundice, anemia or bulging fontanel.

A study of screening for anemia in 9 month old infants in well baby clinic

Iron deficiency remains the most common cause of anemia in infants and chidren despite increasing availability of iron-fortified foods. We screened out anemia in 9-month old infants in well baby clinic to know the prevalence of anemia and the weaning status. The results were as follows: 1) Among 345 infants screened, 24 infants (7%) were found to have anemia. 2) The kind of anemia was microcytosis and 7 infants (2.2%) revealed hypochromia. 3) Among the remaining 311 non-anemic infants, 6 infants (1.9%) revealed microcytosis and 7 infants (2.2%) revealed hypochromia. 4) In anemic group the mean hemoglobin concentration was 8.75+/-1.03gm/dl, hematocrit was 27. 73+/-3.11%, MCV was 65.84+/-8.21 fL, MCHwas 20.95+/-2.93 pg and MCHC was 31.34+/-1.89 gm/dl. 5) In 9 anemic infants, weaning was delayed until 9 months of age.

Pterin & DHPR measurement and DNA analysis in Korean PKU patients

Phenylketonuria is metabolic disorder that results from a deficiency of the hepatic phenylalanine hydroxylase. But among patients with hyperphenylalaninemia, the defect resides in one of the enzymes necessary for production or recycling of tetrahydrobiopterin (BH4). The reduction of BH4 affects not only phenylalanine metabolism but also formation of the tyrosine related neurotransmitter, dopamin and tryptophan related neurotransmitter, serotonin. Administration of L-dopa and 5-hydroxytriptophan seems to be the most effective treatment and may prevent irreversible neurologic damage if started early in life in hyperphenylalaninemia due to deficiency of cofactor BH4. Therefore, all patients with PKU and hyperphenylalaninemia should be tested for BH4 deficiency as early as passible. So we measured reduced forms of biopterin in urines of 19 phenylketonuria patients by Funkushima and Nixon method and 13 of PKU patients measured dihydropterin reductase (DHPR) in white blood cells by modified Narisawa method. We could not find abnormal pterin patterns of cofactor BH4 and normal value of DHPR. All Korean 19 PKU children were classic PKU. A missense mutation has been identified in the phenylalanine hydroxylase (PAH) gene of 16 Koran PKU patients. 5 mutations (IVS4, Y204 C, R243Q, Y356 X, R413 P) have been identified. The frequency of these mutations was found to be 50% of PKU alleles. The IVS4 mutation had a high frequency in Korea and southern China, due to the result of the founder effect and genetic drift. the R413 P mutation, which may have originated in the regions surrounding the Baikal, expanded to northen China and Japan. We were not able to find Caucasian mutations in Korean ptiets. PKU mutations occured after racial divergence between Caucasian and Mongoloids. We observed that PKU patients with Y 204 C and R413 P mutations showed mild mild clinical phenotype but IVS4 mutation had severe mental retardation. the establishment of genotype will therefore aid in the prediction of clinical phenotypes in patients with this disease. So, pterin and DHPR measurement and DNA analsis will be useful for prognosis and proper treatment of PKU patients.

A case of laryngeal cleft

Laryngotracheoesophageal cleft is a rare congenital anomaly characterized by a midline defect of variable length between the posterior larynx and trachea and the anterior wall of the esophagus which was first reported by Richter in 1792. The male, birth weight 2780 gm, was born our hospital, After birth the infant breathed spontaneously, cried immediately but weak and did well initially but after minutes appeared moderate amount of mucus in the mouth, and sterile water was given but immediately vomited with chocking, cough and cyanosis. A nasogastric tube was inserted through the esophagus without resistance and kinking. Esophagogram was showed spillage of contrast media simultaneously into trachea and esophagus. In direct laryngoscopy, there appeared to be small laryngeal cleft posteriorly, to the level of vocal cord. But bronchoscopy could not be performed due to narrow tracheal orifice. So we confirmed the laryngeal cleft by means of CT and MRI of neck. CT and MRI scan of neck demonstrated a cleft in interarytenoid lesion and connection between esophagus and laryngeal lumen.

A clinical study of histiocytosis syndrome

We made a clinical study on 10 cases of histiocytosis syndrome who had been admitted to the pediatric department of Soon Chun Hyang University Hospital from Jan. 1982 to Dec. 1991. The results were obtained as follows 1) The sex incidence revealed male predominance with the ratio 4:1. 2) Among 10 cases, 4 cases were classified as eosinophilic granuloma, 1 case as Letterer-Siwe disease, 1 case as linfection associated hemophagocytic syndrome and 4 cases as malignant histiocytosis. 3) The mean age of symptom onset was 4 and 7/12 years in all disease group. 4) The common clinical symptoms and signs at dignosis were dyspnea, mass, pain on lower extremities and fever. 5) The most common organ involved among 9 organ systems was liver-spleen and the number of organ systems involved were 1 in 3 2 ases(30%), 3 in 2 cases(20%), 8 in 2 cases (20%) and 6, 7 and 10 organs in each 1 case. 6) The abnormal hematologic findings (Hb; 10g/dl and/or WBC; 4,000/mm3 and/or PLT; 100,000/mm3) were found in 6 cases. 7) Low serum albumin was found in 2 cases. Results of blood culture were Pseudomonas aeruginosae and Salmonella typhi in each 1 case. 8) The common findings on tissue biopsy were histiocytic proliferation and infiltration. 9) 4 patients of MH who recieved chemotherapy, a combination of adriamycin, vincristine, cyclophosphamide and prednisone were given in a total of four courses every 2 weeks ad induction therapy. When complete response was attained, a combination of adriamycin, vincristine, prednisone (AOP) and cyclophosphamide, vincristine, prednisone(COP) was administered alternately every 4 weeks as maintenance therapy for 6-64 months. 10) Among 4 patiens of MH who recieved chemotherapy, 1 patient was lost during induction chemotherapy, for 1 day.2 patients expired during induction chemotherapy, for 1 month, 1 patient expired during maintenance chemotherapty, for 8months, Eosinophilic granuloma cases (3) were recieved currettage and no recurrence. IAHS case due to typhoid fever was improved spontaneously.

Ultrasonographic measurement of the neonatal adrenal glands

TO determine the normal neonatal adrenal gland size, ultrasonographic examinations were performed in 145 newborn infants. They were divided into 3 groups according to the days of age. The group I is 1~3 days, group II is 6~9 days and group III is 21~50 days of age. 1) The adrenal gland size was as follows. In group I, the length was 29.05mm and the width was 4.02mm. In group II, the length was 24.04mm and the width was 2.79mm. In group III, the length was 19.54mm and the width was 2.21mm. 2) With increasing age, the size of adrenal gland became smaller. 3) The size of adrenal gland correlated well with the birth weight and height. We conclude that the ultrasonographic examination is useful in infant adrenal gland disease.

A case of neonatal hydrometrocolpos causing urinary obstruction

No abstract available.

A case of Prune Belly syndrome associated with Turner syndrome

No abstract available.

A study of serologically confirmed measles

No abstract available.

A case of omental cyst

No abstract available.

A clinical study in phenylketonuria

No abstract available.

A nonfamilial case of multiple juvenile polyposis

No abstract available.

A case of neonatal hypothyroidism due to maternal TSH-binding inhibitor immunoglobulin

No abstract available.