Kimura Disease Associated with Allergy and Remote Parasite Infection / 소아알레르기및호흡기학회지

Kimura disease is rare chronic inflammatory disorder of an unknown etiology and it has a predilection for males of Asian descent. It typically presents with non-tender subcutaneous swellings in the head and neck region, peripheral eosinophilia and an elevated immunoglobulin E level. We present here the case of 8-year-old boy with a past history of food allergy, and he developed swelling of the left neck and incidental eosinophilia. Kimura disease was diagnosed on the basis of the clinical and histological findings. The serum IgG titer of cysticercosis was twice the normal level. After surgical excision of the subcutaneous lesion, the patients eosinophilia was dramatically decreased. We suggest that allergy and remote parasite infection may be the causality or a triggering factor for Kimura disease.

Values of Lung CT in Children with a Vague History of Aspiration / 소아과

PURPOSE: The purpose of this study was to investigate the diagnostic value of spiral computed tomography (CT) using contiguous slices with partially thin sections around the hilar level in the bronchial foreign bodies of children with a vague history of aspiration. METHODS: Fourteen children were identified to be examined with spiral CT due to obscure histories of aspiration episodes. A retrospective analysis of the medical records provided information concerning the clinical and radiological findings. RESULTS: Of the 14 patients examined with spiral CT, there were no significant differences among the clinical and initial radiological findings with the exception of gender distribution. However, the spiral CT scans allowed accurate discrimination among patients with a vague history of aspiration episodes, in which seven were identified with bronchial foreign body aspiration and seven with bronchiolitis, asthma, tracheobronchitis and/or pneumonia. CONCLUSION: We found that spiral CT using contiguous slices with partially thin sections around the hilar level are a useful non-invasive method in the early diagnosis of bronchial foreign bodies in children with a vague history of aspiration.

Monosomy 21 Mosaicism in a Child with Dyserythropoiesis

All complete monosomy 21 appear to be lethal early in their development in humans and only survive in mosaic forms. Complete monosomy 21 is a very rare and usually debilitating genetic disorder. Partial monosomy 21 is also rare and is thought to constitute a clinical syndrome consisting of peculiar faces, hypertonia, psychomotor retardation, and slow growth. We experienced a case of monosomy 21 mosaicism. Chromosome analysis demonstrated mosaicism for cell lines in the lymphocytes examined; 45, XX, -21/46, XX. The main clinical features were craniofacial dysmorphism including high arched palate, submucosal cleft, micrognathia and arthrogryposis-like symptoms including flexion deformity of fingers. And hematological findings were revealed dyserythropoiesis, thrombocytopenia and eosinophilia. Currently, the patient has nearly compatible growth, but a mild degree of mental retardation. We report here an 8 years old female child with apparent monosomy 21 mosaicism associated with dyserythropoiesis, thrombocytopenia and eosinophilia, with a review of the associated literatures.

Detection of Helicobacter pylori by Pre-embedding Immunoelectron Microscopy: Comparison with Immunoblotting Method

PURPOSE: We tried to evaluate whether the detection rate of Helicobacter pylori in gastric biopsy specimens could be improved by using pre-embedding immunoelectron microscopy. METHODS: A total of 119 children who complained of upper gastrointestinal symptoms were endoscoped at the Gyeongsang National University Hospital from July, 1996 to July, 1999. Five biopsy specimens(three for urease test, one for hematoxylin-eosin(H and E) staining, and one for pre- embedding immunoelectron microscopy) were obtained from each antrum and body. Immunoblotting analysis were also performed. RESULTS: Among the 119 patients, H. pylori were found in 116 patients(97.5%) by the immunoelectron microscopy. Among three patients who were found H. pylori negative in immunoelectron microscopy, two patients showed H. pylori in H and E stained slides and one patient was urease test positive(color change within six hours). Urease tests were positive in 107 patients(89.9 %). The positive rate of immunoblotting tests was 81.5%. However, only 13 patients(10.9%) showed H. pylori on the H and E stained antrum or body tissue. CONCLUSION: In this study, we found H. pylori histopathologically in most of the pediatric patients who complained of upper gastrointestinal symptoms. This study showed that pre-embedding immunoelectron microscopic examinations can be used as a gold standard in the diagnosis of childhood H. pylori infection. However, this method also has limited capacity to detect widely scattered H. pylori compared to the other histopathologic diagnostic methods.

Gastrointestinal Foreign Bodies: Review of 96 Cases / 대한소아소화기영양학회지

PURPOSE: Foreign body swallowing is common in children. Most of foreign bodies passed upper esophagus are removed spontaneously. But recently, therapeutic endoscopy in children is widely used. In this study, we reviewed gastric foreign bodies regarding types, location, interval from swallowing, complications, and treatment or method of removal. METHODS: We reviewed medical records of 96 cases with foreign bodies in gastrointestinal tract at Department of Pediatrics in Gyeongsang National University Hospital (GNUH) from Feb 1987 to Feb 2002. RESULTS: The peak age of patients (male=60, female=36) with foreign bodies in gastrointestinal tract was 2 to 5 years of age. Sixty two patients (64.5%) were asymptomatic. The location of foreign bodies in gastrointestinal tract were detected by simple X-ray in eighty one patients (86.0%), barium study (1 case), and gastroduodenoscopy in 37 cases. The most common location was stomach (63.5%). The most common foreign body was coin (41.7%). In thirty seven cases (38.5%), foreign bodies were removed with flexible gastroduodenoscopy. Fifty four patients (56.3%) visited GNUH in 24 hours after swallowing foreign bodies. The long interval (over 24 hours) of swallowing of foreign bodies is related with high frequency of endoscopic removal. CONCLUSION: The flexible gastroduodenoscopy was effective in diagnosis and removal of foreign bodies. Further studies for indication and validity of endoscopic removal of foreign bodies in upper gastrointestinal tract are needed.

Prolonged Remission Following Autologous Peripheral Blood Stem Cell Transplantation in an Infant with Familial Hemophagocytic Lymphohistiocytosis / 대한혈액학회지

Familial hemophagocytic lymphohistiocytosis (FHL) is a fatal illness with a median life expectancy of 2 months if not treated. However, with the recent employment of allogeneic bone marrow transplantation in the treatment of FHL, even complete cure might be anticipated. We report here a case of prolonged remission following autologous peripheral blood stem cell transplantation (Auto-PBSCT) which was first attempted for FHL in Korea. A 4-month-old girl presented with high fever and a huge hepatosplenomegaly. Bone marrow examination revealed the proliferation of both non-malignant histiocytes engulfing red blood cells and lymphocytes. Her brother died at the age of 18 months showing quite similar manifestations. A diagnosis of FHL was made. Following 8 courses of chemotherapy with multiple drugs, Auto-PBSCT was performed. She has been well without any evidence of disease over 8 months of post- transplant period. This case illustrates that it might be worth trying Auto- PBSCT in the treatment of FHL.

A Clinical Study of Shigellosis Epidemics over 10 Years in the Kyung Nam Area

PURPOSE: The occurrence of shigellosis patients has increased radically in 2 recent years. For this, the authors investigated the epidemic aspect of shigellosis by regional groups to know whether the epidemic aspect of shigellosis is a general situation which occurs identically in various areas of Kyung Nam. METHODS: We reviewed pediatric patients who were hospitalized in Ulsan(eastern), in Masan(cen tral), in Chinju(western) of Kyung Nam from January, 1988 to April, 1999. RESULTS: A total of 54 cases of shigellosis were noted from Ulsan(12), Masan(21) and Chinju(21) over ten years. The number of patients was 1 each in 1991 and 1993, 5 each in 1998 and 1999 at Ulasan, 9 in 1988, 8 between 1990 and 1995, 3 in 1998, 1 in 1999 at Masan, 1 each in 1990, 1991, 1994, 4 in 1998, 14 in 1999 at Chinju. The shigellosis patients increased from 1998, especially in Chinju. S. flexneri was reported in Masan(AMP)(R)5, AMP+TMP/SMX(R)4) before 1990, in Masan (2) between 1990 and 1995 and in Chinju(AMP(R)7, AMP+MP/SMX(R)1) in 1999. S. sonnei was reported in Ulsan(AMP(R)1, TMP/SMX(R)1), in Masan(6) and in Chinju(AMP(R)2, TMP/SMX(R) 1) between 1990 and 1995 and in Ulsan(TMP/SMX(R)9, AMP+TMP/SMX(S)1), in Masan(TMP/ SMX(R)2, AMP+TMP/SMX(R)1+(S)1) and in Chinju(TMP/SMX(R)6) between 1998 and 1999. CONCLUSION: Independent of the past, shigellosis patients occurred by regional groups in three parts of Kyung Nam. The present epidemic aspect of the eastern area is similar to that of the central area, but is partially similar to that of western Kyung Nam.

Relationship between Intussusception and Vitamin C Concentrations of Whole Blood and Plasma / 대한소아소화기영양학회지

PURPOSE: Adenovirus infection with swollen Peyer patches in the ileum, known as one of the causes of intussusception, may cause vitamin C depletion in human body because vitamin C is a first line antioxidant. Or low vitamin C status in human body makes the man more susceptible to infection of adenovirus in the ileum with a markedly swollen lymph node. In this study, we tried to find out the relationship between pediatric intussusceptions and vitamin C concentrations of whole blood and plasma. METHODS: Whole blood and plasma vitamin C concentrations of fifty-seven patients with intussusceptions from May 1995 to December 1998 at the Gyeongsang National University Hospital were compared with those of 256 normal healthy children. Vitamin C was measured by the 2,4-dinitrophenylhydrazine methods. RESULTS: The average age of patients (male=39, female=18) with intussusceptions was fifteen months of age. Whole blood vitamin C concentrations of patients and healthy children were 1.49+/-0.64 mg/dL, and 2.18+/-0.49 mg/dL, respectively. Plasma vitamin C concentrations of patients and healthy children were 0.59+/-0.36 mg/dL, 1.47+/-0.56 mg/dL, respectively. But no differences in the vitamin C concentrations of whole blood and plasma according to age, degree of leukocytosis, fever, interval from onset, hematochezia, and need for operation were found. CONCLUSION: Whole blood and plasma vitamin C concentrations of patients with intussusceptions were lower than those of healthy children (P=0.0001). Prospective studies are needed to elucidate whether these results were consequences or causes of intussusceptions.

A Case of Gastric Perforation Presenting with Secondary Localized Peritonitis Due to Bamboo in Stomach

Perforation after foreign body ingestion is rare. We report a 25-month-old girl who was diagnosed with a stomach perforation with secondary localized peritonitis caused by long, sharp bamboo. On admission, she had abdominal pain and fever. Simple abdominal erect & supine X-ray, abdominal ultrasonogram and computed tomography scan were normal. On the 6 th hospital day, gastrofiberoscopy was performed because a sharp mass was palpated by doctor on abdominal examination. Endoscopic findings revealed a 8 cm-long sharp bamboo tip was pierced to the posterior wall of antrum. This foreign body was removed with Olympus FG-8L alligator forcep. She was managed with NPO and prompt intravenous antibiotics and discharged with clinical improvement.

Malignant Ectomesenchymoma in a Two-Month-Old Boy

Malignant ectomesenchymoma is a rare tumor originating from remnants of migratory neural crest(ectomesenchyme) and composed of neuroectodermal as well as mesenchymal components. Neuroblasts and ganglion cells constitute the neuroectodermal components and rhabdomyosarcoma is the most frequently encountered mesenchymal components. We report a case of malignant ectomesenchymoma in a two-month-old boy who was presented with abdominal pain and urinary difficulty. The tumor appeared to arise from the prostatic region and enlarged to compress the bladder leading to bilateral hydronephrosis. Immunohistochemical studies for the resected tumor confirmed the presence of mixed ganglioneuroma and rhabdomyosarcoma establishing the diagnosis of malignant ectomesenchymoma. However, in the initial biopsy specimen of tumor, poorly differentiated round to oval cells positive for both desmin and vimentin staining were uniformly noted and the incorrect diagnosis of rhabdomyosarcoma was made. Intensive multi-agents chemotherapy, surgery and radiotherapy had failed in preventing the development of local recurrences. Subsequently, invasion of pubic bone and lung metastases ensued. This report enlightens the need for immunohistochemistry to seek possible neuroectodermal components in a tumor specimen suggestive of rhabdomyosarcoma.