Mesenteric lymphangioma, which is rare and often diagnosed incidentally, is a benign tumor in infants and children. Most cases of lymphangioma are in the neck and axilla. About 5% of these lesions are in the mediastinum, mesentery or retroperitoneal region. Although most lymphangioma produce no symptoms, it may present an acute surgical condition suggesting acute intestinal obstruction. We report one case of histologically confirmed mesenteric lymphangioma causing billous vomiting and bloody stool in a 3-day-old infant.
Axilla ; Child ; Humans ; Infant* ; Intestinal Obstruction ; Lymphangioma* ; Mediastinum ; Mesentery ; Neck ; Vomiting*

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.
Bone Marrow Examination ; Bone Marrow Transplantation ; Diagnosis ; Drug Therapy ; Employment ; Erythrocytes ; Female ; Fever ; Histiocytes ; Humans ; Infant* ; Korea ; Life Expectancy ; Lymphocytes ; Lymphohistiocytosis, Hemophagocytic* ; Peripheral Blood Stem Cell Transplantation* ; Siblings

Juvenile granulosa cell tumor (JGCT) is one of the rare sex cord stromal tumors of the ovary. The majority of JGCT are found in prepubertal girls and young women. Unlike adult granulosa cell tumor (AGCT), JGCT is characterized by high frequency of mitosis and early relapse after treatment. Patients with JGCT in FIGO stage Ia have an excellent outcome with an event-free survival of about 90% following surgical resection alone, while those in advanced stages have an unfavorable outcome. We report two cases of JGCT in stage Ic, who showed a quite different outcome. One was managed initially with surgical resection alone and died of subsequent relapse. Another was managed with multimodality treatments including surgical resection, adjuvant chemotherapy and radiotherapy, and didn't show any evidence of relapse during 16 months of follow-up period. Further studies to evaluate the beneficial effects of chemotherapy and/or radiotherapy are required in patients with JGCT in stage Ic.
Adult ; Chemotherapy, Adjuvant ; Disease-Free Survival ; Drug Therapy ; Female ; Follow-Up Studies ; Granulosa Cell Tumor* ; Granulosa Cells* ; Humans ; Mitosis ; Ovary ; Radiotherapy ; Recurrence ; Sex Cord-Gonadal Stromal Tumors

Chondrodysplasia punctata is a heterogenous skeletal dysplasia characterized by small focal calcifications in articular and other cartilages in infancy, with subsequent epiphysial dysplasia and associated anomalies of the face, eyes, and skin. Chondrodysplasia punctata is classified with autosomal recessive rhizomelic type, autosomal dominant nonrhizomelic type(Conradi-Hunermann type), and X-linked dominant type. These types have different clinical manifestations respectively. We report cases of autosomal dominant type of chondrodysplasia punctata which occurred in brothers, who were presented with typical face(short collumella leading to depressed tip of the nose), punctate calcification on the lumbar spine, dislocation of right hip, no obvious skin lesions, and atropic optic nerve without cataracts.
Cartilage ; Cataract ; Chondrodysplasia Punctata* ; Dislocations ; Hip ; Humans ; Optic Nerve ; Siblings* ; Skin ; Spine

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.
Adenoviridae ; Adenoviridae Infections ; Ascorbic Acid* ; Child ; Fever ; Gastrointestinal Hemorrhage ; Human Body ; Humans ; Ileum ; Intussusception* ; Leukocytosis ; Lymph Nodes ; Peyer's Patches ; Plasma* ; Vitamins*

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.
Abdominal Pain ; Biopsy ; Desmin ; Diagnosis ; Drug Therapy ; Ganglion Cysts ; Ganglioneuroma ; Humans ; Hydronephrosis ; Immunohistochemistry ; Lung ; Male* ; Neoplasm Metastasis ; Neural Plate ; Pubic Bone ; Radiotherapy ; Recurrence ; Rhabdomyosarcoma ; Urinary Bladder ; Vimentin

PURPOSE: Respiratory failure arises from derangements in pulmonary gas exchange. The causes may be classified as due to either lung disease or respiratory pump dysfunction. Problems with lung mechanics is an important cause of acute respiratory failure in children clinically. The aims of this study were to survey the cause and prognosis of children diagnosed with acute respiratory failure by intrapulmonary lesion. METHODS: We performed a retrospective study of 52 children(M:F=31:21) diagnosed with acute respiratory failure by intrapulmonary lesion in Gyeongsang National University Hospital from January, 1989 to July, 2003. The age distribution was from 0.1 to 12 years old(2.6+/-3.3 yr). RESULTS: The causes of acute respiratory failure were bronchiolitis and infectious pneumonia(24 cases, 46.1%), acute respiratory distress syndrome(14 cases, 26.9%), near drowning(seven cases, 13.5%), aspiration pneumonia(three cases, 5.8%), sepsis with intrapulmonary lesion(three cases, 5.8%) and croup(one case, 1.9%). The underlying diseases of acute respiratory distress syndrome were aspiration pneumonia(four cases, 28.6%), near drowning(three cases, 21.4%), pneumonia(three cases, 21.4%), sepsis with pneumonia(two cases, 14.3%), and shock(two cases, 14.3%). The survival rate of all subjects was 67.3%. The survival rate of underlying diseases were:83.3% in cases of bronchiolitis and infectious pneumonia, 57.1% in near drowning, 42.8% in acute respiratory distress syndrome, 33.3% in sepsis, and 100% in aspiration pneumonia and croup. CONCLUSION: The major cause of acute respiratory failure by intrapulmonary lesion in children was respiratory infection. And acute respiratory distress syndrome and sepsis with intrapulmonary lesion had relatively poor prognoses, thus active and prompt intervention is important in these conditions.
Age Distribution ; Bronchiolitis ; Child ; Croup ; Humans ; Lung ; Lung Diseases ; Mechanics ; Near Drowning ; Pneumonia ; Pneumonia, Aspiration ; Prognosis* ; Pulmonary Gas Exchange ; Respiratory Distress Syndrome, Adult ; Respiratory Insufficiency* ; Retrospective Studies ; Sepsis ; Survival Rate