We were recently encountered a cases of newborn infant with a congenital lumbar mass with associated anomalies which proved to be quite bizarre. This case if presented with review of the literatures because we were seldom able to find a cases of myelomeningocele as usual, interesting as this. A 15 day-old infant, the product of full term, was admitted to Busan National University Hospital on May 26th, 1972, because of a large pedunculated lumbar mass without any neurologic deficits. The tumor mass was translucent, flabby, infected and increased in tension when the infant cried. Chest film showed the underdeveloped with agenesis and fork rib in right upper 5 ribs and narrowed intercostal spaces between right 5,6,7 and 8th ribs. Right scapula was higher in position of axis. Total spine films showed non-fusion pattern of 3rd and 4rh thoracic vertebral bodies with cleft vertebrae, scoliosis in upper thoracic region, widening of lumbar canal, a large soft tissue mass overlying the abdominal region and hypoplasia of the spinous process at 4th lumbar vertebra. The sac of myelomeningocele was repaired successfully. He has regularly been followed up in the out-patient after discharge and has remained well. Repeated neurologic examination performed after discharge showed no neurologic deficits. We feel our present case will be of added interest.
Axis, Cervical Vertebra ; Busan ; Humans ; Infant ; Infant, Newborn ; Meningomyelocele* ; Neurologic Examination ; Neurologic Manifestations ; Outpatients ; Ribs ; Scapula ; Scoliosis ; Spine ; Thorax

BACKGROUND AND OBJECTIVES: High dose systemic steroid therapy is currently the mainstay of the treatment for sudden sensorineural hearing loss (SSNHL). However, it makes a glycemic control worse in patients with diabetes. Intratympanic steroid injection (ITSI) can result in reduced systemic steroid toxicity and higher perilymph steroid level selectively. The purpose of this study is to compare the efficacy of ITSI (IT group) with that of systemic steroid (IV group) on SSNHL with diabetes. SUBJECTS AND METHOD: Thirty eight SSNHL patients who were diagnosed with diabetes were divided into the IV group (19 patients) and the IT group (19 patients). In the IV group, prednisolone was administrated intravenously for 7 days followed by tapered doses orally for 7 days. In the IT group, dexamethasone was administrated 4 times within a 2 week-period. Hearing outcome was assessed before and after treatment. RESULTS: In the IV group, 10 patients (58.8%) showed an improvement in the pure tone audiogram (PTA), with a mean improvement of 17.6 dB (p=0.023). In the IT group, 16 patients (84.2%) showed improvement in the PTA, with the mean improvement of 25.1 dB (p=0.000). But there was no significant difference in hearing gain and the recovery rate between the two groups. And it is more difficult to control blood sugar in the IV group rather than in the IT group. CONCLUSION: ITSI treatment is as effective as the systemic steroid treatment for SSNHL patients with diabetes and it can avoid a significant side effect of systemic steroids. So it could be considered as an initial treatment for the SSNHL patient with diabetes.
Blood Glucose ; Dexamethasone ; Hearing ; Hearing Loss, Sensorineural ; Hearing Loss, Sudden ; Humans ; Perilymph ; Prednisolone ; Steroids

Regulation of osteoblast and osteocyte viability is essential for bone homeostasis. Smad4, a major transducer of bone morphogenetic protein and transforming growth factor-β signaling pathways, regulates apoptosis in various cell types through a mitochondrial pathway. However, it remains poorly understood whether Smad4 is necessary for the regulation of osteoblast and osteocyte viability. In this study, we analyzed Smad4Δ(Os) mice, in which Smad4 was subjected to tissue-specific disruption under the control of the 2.3-kb Col1a1 promoter, to understand the functional significance of Smad4 in regulating osteoblast/osteocyte viability during bone formation and remodeling. Smad4Δ(Os) mice showed a significant increase in osteoblast number and osteocyte density in the trabecular and cortical regions of the femur, whereas osteoclast activity was significantly decreased. The proliferation of osteoblasts/osteocytes did not alter, as shown by measuring 5′-bromo-2′deoxyuridine incorporation. By contrast, the percentage of TUNEL-positive cells decreased, together with a decrease in the Bax/Bcl-2 ratio and in the proteolytic cleavage of caspase 3, in Smad4Δ(Os) mice. Apoptosis in isolated calvaria cells from Smad4Δ(Os) mice decreased after differentiation, which was consistent with the results of the TUNEL assay and western blotting in Smad4Δ(Os) mice. Conversely, osteoblast cells overexpressing Smad4 showed increased apoptosis. In an apoptosis induction model of Smad4Δ(Os) mice, osteoblasts/osteocytes were more resistant to apoptosis than were control cells, and, consequently, bone remodeling was attenuated. These findings indicate that Smad4 has a significant role in regulating osteoblast/osteocyte viability and therefore controls bone homeostasis.
Animals ; Apoptosis ; Blotting, Western ; Bone Morphogenetic Proteins ; Bone Remodeling ; Caspase 3 ; Femur ; Homeostasis* ; In Situ Nick-End Labeling ; Mice ; Osteoblasts ; Osteoclasts ; Osteocytes ; Osteogenesis ; Skull ; Transducers

BACKGROUND: The lymphocytes including morphologically immature lymphoid cells are frequently increased in the marrow aspirates of children with neuroblastoma. We studied about the clonality of these lymphoid cells and its effects on the marrow involvement and prognosis of disease. METHODS: We evaluated 30 marrow aspirates of 23 children with neuroblastoma from 1990 to 1998. We tested the immunoglobulin heavy chain gene rearrangement PCR for B cell clonality and T cell receptor gamma gene rearrangement PCR for T cell clonality with bone marrow specimens. RESULTS: Younger children showed negative bone marrow involvement more than older children. In this group, the proportions of immature lymphoid cells and total lymphocytes were higher (3.4+/-3.2% vs. 0.8+/-1.9%, 31.3+/-17.0% vs. 14.7+/-12.0%). Immunoglobulin heavy chain gene rearrangements were present in 19/30 (64%) specimens and more frequently observed in negative marrow involvement cases. Seven cases with the proportions of total lymphocytes more than 30% showed significantly high long-term survival probability (P=0.05). Ten cases with B cell monoclonality showed the tendency of high long-term survival probability (P=0.13). CONCLUSION: The increase of lymphocytes including morphologically immature lymphoid cells in the marrow aspirates of children with neuroblastoma were frequently observed in the children without marrow involvement of malignancy and closely related to B cell clonality. The increase of total lymphocytes and related B cell monoclonality may be one of possible explanations of goodprognosis of children with neuroblastoma.
Bone Marrow* ; Child* ; Gene Rearrangement ; Humans ; Immunoglobulin Heavy Chains ; Lymphocytes* ; Neuroblastoma* ; Polymerase Chain Reaction ; Prognosis* ; Receptors, Antigen, T-Cell

BACKGROUND AND OBJECTIVES: There have been heated controversies over the choice of the canal wall down mastoidectomy (CWD) and canal wall up mastoidectomy (CWU), which are operational methods used to eliminate the lesion of cholesteatoma. Combining the advantages of both methods, we reconstructed the posterior canal wall with conchal cartilage plate and obliterated mastoid cavity with bone chips (group I), or hydroxyapatite mixed with bone chips (group II) since 2001. This study was designed to evaluate the surgical outcomes of posterior canal wall reconstruction with mastoid obliteration in the treatment of cholesteatoma. SUBJECTS AND METHOD: From January of 2001 to March of 2007, the posterior canal wall reconstruction with mastoid obliteration was conducted on 66 patients. There were 30 cases of cholesteatoma and 36 cases of old radical cavity. The postoperative observation period ranged from 5 to 74 months, with the average period of 34.7 months. We analyzed the postoperative complications, and hearing results of the 33 ossicular reconstruction cases. RESULTS: There was 1 case of residual cholesteatoma in the middle ear cavity, but no recurrent cholesteatoma. In most cases, reconstructed canal wall was maintained well, but partial canal wall resorption and postauricular dimpling occurred in 5 cases of group I. On the other hand, the epithelization of posterior canal wall was incomplete in 4 cases of group II. After surgery, no patients complained any cavity problems at all. CONCLUSION: The present study suggests that this procedure can prevent cavity problems and reduce the recurrence of cholesteatoma with destructed canal wall.
Cartilage ; Cholesteatoma ; Durapatite ; Ear, Middle ; Hand ; Hearing ; Hot Temperature ; Humans ; Mastoid ; Postoperative Complications ; Recurrence