Dysplasia Epiphysealis Hemimelica is a rare developmental disorder of epiphyseal growth characterized by asymmetrical cartilagenous overgrowth. This disorder was first described with the “la tarsomegalie” by Mouchet and Belot in 1926 but this name, Dysolasia Epiphysealis Hemimelica, was first proposed by Fairbank in 1956. This disorder is usually limited to either the medial or lateral half of a single extremity in childhood. It is asymptomatic until the protruding epiphyseal mass interferes joint function. The character istic abnormalities are deformities, restricted motion and pain. The most frequent finding at the initial examination is an irregular, often multicentric radiopacity, and when the lesion is matured it looks irregular, and frequently there is a lobulated osseous mass protruding from the epiphysis of tarsal and carpal bones. Diagnosis by the roentgenogram requires primarily knowledge of this condition. The lesion is often microscopically indistinguishable from an osteochondroma. A case of Dysplasia Epiphysealis Hemimelica in a 17 year old male, showing typical roentgenographic appearances, is reported together with a brief review of literature in this paper.
Carpal Bones ; Congenital Abnormalities ; Diagnosis ; Epiphyses ; Extremities ; Humans ; Joints ; Male ; Osteochondroma

Many unsolved probIems still remain in these in management of trochanteric fracture of hip, especially unstable type of trochanteric fracture. Among many cases of the trochanteric fracture treated during period from Jan. 1963 to July, 1973 at Busan National University Hospital, six patient has severe sequelae such as coxa vara deformity, shortening of the affected limb and ankylosis of the hip joint, which were analyzed clinically and results obtained were as follow. 1. It is the most important factor that the medial and the posterior cortex is good alignment in reduction with internal fixation and maintainance of unstable trochanteric fracture. 2. It is dangerous for the unstable type of trochanteric fracture to be reducted and maintained with only screws or plates. Jewett nail is more effective than the S-P nail and Thornton plate in internal fixation of unstable type of trochanteric franture. 3. The posterior fragments can hardly be found in A-P view but easily in lateral view 4. In cases that had not good alignments between posterior fragments the distaI fragment displaced medially and migration of the naiI, distraction of the pIate, malunion have developed in spite of prolonged immobilization in cast. 5. In one case that the severe coxa vara deformity have been developed. we performed the transverse osteotomy & fixed with Blount-V-blade plate like device. The result was good but the shortening of the limb could not prevent.
Ankylosis ; Busan ; Congenital Abnormalities ; Coxa Vara ; Extremities ; Femur ; Hip Joint ; Hip ; Humans ; Immobilization ; Osteotomy

BACKGROUND: Mannitol is widely used in neurosurgical patients and may induce an increase in serum potassium concentration according to doses and administration rates with unknown mechanism. The treatment of hyperkalemia is aimed at eliminating the causes and includes calcium, sodium bicarbonate, glucose with insulin, loop diuretics and hyperventilation. This study was undertaken to observe the effects of hyperventilation on the serum potassium concentration following infusion of mannitol (2.0 gm/kg). METHODS: We studied 30 patients who were operated brain aneurysm clipping surgery and were divided into 3 groups (n=10). In control group, mild hypocapnia was maintained (PaCO2, 32 2 mmHg) before and after mannitol infusion. In group I, moderate hypocapnia was maintained (PaCO2, 27 2 mmHg) before and after mannitol infusion. In group II, mild hypocapnia (PaCO2, 32 2 mmHg) was maintained before 30 minutes of mannitol infusion and moderate hypocapnia (PaCO2, 27 2 mHg) after mannitol infusion. We started infusion of 20% mannitol with a dosage of 2.0 gm/kg, 15~20 min after cranium was opened. RESULTS: The changes of serum potassium were as follows (Mean SD mEq/l) (just before and 15min, 30min, 60min after mannitol infusion): 3.79 0.48, 4.66 0.60, 4.44 0.48, 4.13 0.40 (Control group), 3.62 0.18, 3.63 0.42, 4.14 0.51, 3.95 0.33 (Group I), 3.76 0.20, 3.91 0.15, 4.11 0.30, 4.04 0.23 (Group II). After 15 minutes of mannitol infusion, the serum potassium levels of group I and II were lower than that of control group (p<0.05) and there was no significant difference between group I and II. CONCLUSIONS: These results suggest that hyperventilation may blunt the increase in serum potassium concentration following rapid infusion of high dose mannitol.
Calcium ; Glucose ; Humans ; Hyperkalemia ; Hyperventilation* ; Hypocapnia ; Insulin ; Intracranial Aneurysm ; Mannitol* ; Potassium* ; Skull ; Sodium Bicarbonate ; Sodium Potassium Chloride Symporter Inhibitors

The clinical usefulness of organ-preserving pancreatectomy is not well established due to technical difficulty and ambiguity of functional merit. The purpose of this study is to evaluate the clinical efficacy of organ-preserving pancreatectomy such as duodenum-preserving resection of the head of the pancreas (DPRHP), pancreatic head resection with segmental duodenectomy (PHRSD), central pancreatectomy (CP) and spleen-preserving distal pancreatectomy (SPDP). Between 1995 and 2007, the DPRHP were performed in 14 patients, the PHRSD in 16 patients, the CP in 13 patients, and the SPDP in 45 patients for preoperatively diagnosed benign lesions or tumors with low-grade malignant potential. The clinical outcomes including surgical details, postoperative complications and long-term functional outcomes were compared between organ-preserving pancreatectomy and conventional pancreatectomy group. Major postoperative complications constituted the following: bile duct stricture (7.1% [1/14]) in DPRHP, delayed gastric emptying (31.2% [5/16]) in PHRSD, pancreatic fistula (21.4% [3/14]) in CP. There were no significant differences in postoperative complications and long-term functional outcomes between two groups. Organ-preserving pancreatectomy is associated with tolerable postoperative complications, and good long-term outcome comparing to conventional pancreatectomy. Organ-preserving pancreatectomy could be alternative treatment for benign or low-grade malignant potential lesion of the pancreas or ampullary/parapapillary duodenum.
Adult ; Aged ; Female ; Follow-Up Studies ; Humans ; Male ; Middle Aged ; *Pancreatectomy ; Pancreatic Neoplasms/diagnosis/*surgery ; Postoperative Complications ; Suture Techniques ; Treatment Outcome

OBJECTIVE: Human embryonic stem cell derived from blastocyst randomly differentiates into multiple cell types during embryoid body development. Bone morphogenetic proteins (BMPs) are members of the transforming growth factor type beta superfamily. We have a question whether BMP2 and/or BMP4 can induce trophoblast specific genes in human ES cells using SNU hES3 cell line. METHODS: Human embryonic stem cell line (SNU hES3) was supplied by Miz Medi Hospital Seoul National University. Cultured hES cells were divided into small clumps and then allow for EB formation in differentiation medium. After EB formation, EBs were transferred onto gelatin coated dishes and given hES conditioned medium alone (control) or supplemented as following treatment for 6 days; rhBMP4 100 ng/mL; rhBMP2 100 ng/mL; BMP4 100 ng/mL +BMP2 100 ng/mL. RT PCR was performed for trophoblast specific genes. During culture, supernatant was collected and measured for estradiol (E2), progesterone, and hCG beta by enzymeimmuno assay (EIA) kit. RESULTS: BMP4 and BMP2 increase chorionic gonadotropin beta (hCG beta), glical cell missing 1 (GMC1), and CD9 as trophoblast specific gene markers confirmed by RT PCR. However, the non classical HLA class I molecule HLAG1, was not expressed in our studies. And we cannot find significant differences of the level of estradiol, hCG nd progesterone in this study. CONCLUSION: The results suggest that BMP 4 and 2 have an additive effect on induction of trophoblast related genes in SNU hES3 cell line. Although we failed to induce the differentiation of human ES cells to trophoblast, this study could provide the possibility for the differentiation of early human trophoblast cells and thus need further studies.
Blastocyst ; Bone Morphogenetic Proteins ; Cell Line* ; Chorionic Gonadotropin ; Chorionic Gonadotropin, beta Subunit, Human ; Culture Media, Conditioned ; Embryoid Bodies ; Embryonic Stem Cells* ; Estradiol ; Gelatin ; Humans* ; Polymerase Chain Reaction ; Progesterone ; Seoul ; Transforming Growth Factors ; Trophoblasts

Acute hemorrhagic edema of infancy (AHEI) is an uncommon form of cutaneous leukocytoclastic vasculitis that occurs in infants and children younger than 2 years. AHEI is characterized clinically by marked peripheral edema and fever as well as large palpable purpuric and ecchymotic skin lesions in a target-like pattern, mainly on the face, ears and extremities, similar to the skin findings of Henoch-Schonlein purpura (HSP). The skin lesions heal spontaneously within one to three weeks and internal organs are rarely affected. We report a case of AHEI occurring in a 23-month-old boy who was initially misdiagnosed as HSP, and was later diagnosed according to his clinical symptoms and histochemical characteristics.
Child ; Ear ; Edema* ; Extremities ; Fever ; Humans ; Infant* ; Male ; Purpura ; Purpura, Schoenlein-Henoch* ; Skin ; Vasculitis, Leukocytoclastic, Cutaneous

OBJECTIVE: This study is performed to evaluate the risk factors, clinical manifestations, treatments, and prognostic factors among patients with postoperative wound infection after spinal instrumentation. METHODS: The records and radiologic data of 28 patients diagnosed as postoperative wound infection from Jan. 1991 to Oct. 2002 who had underwent spinal instrumentation initially were retrospectively evaluated. RESULTS: All patients(mean age, 50.4 yrs) diagnosed as postoperative wound infection and subsequently received IV antibiotics and continuous irrigation system. The 13 among 28 cases had significant preoperative risk factors(for example, diabetes mellitus, obesity, alcoholism, long-term corticosteroid usage, and chronic renal failure). Infection was diagnosed at an average of 15.9 days after operation. The most common presenting features were local heating, fever, and wound discharge. All patients could be discharged after infection control. At follow up evaluation, 21 case(75%) have not shown recurrence but, the recurrence was developed among 7 cases. The C-reactive protein(CRP) was more correlated with clinical outcomes than other laboratory findings and the recurred group exhibited significant(p<0.05) higher CRP level, compared with the non-recurred group, from 7 days to 21 days after operation. CONCLUSION: Without removal of instruments, surgical removal of infective tissues and continuous irrigation with susceptible antibiotics can be effective in the treatment of postoperative wound infection after spinal instrumentation. CRP level can be an effective parameter of infection treatment and prognosis.
Alcoholism ; Anti-Bacterial Agents ; Diabetes Mellitus ; Fever ; Follow-Up Studies ; Heating ; Hot Temperature ; Humans ; Infection Control ; Obesity ; Prognosis ; Recurrence ; Retrospective Studies ; Risk Factors ; Spine* ; Surgical Instruments ; Surgical Wound Infection ; Wound Infection* ; Wounds and Injuries*

Incontinentia pigmenti (IP) is an uncommon X-linked, dominantly inherited disorder due to a mutation in the NEMO (NF-kappaB essential modulator) gene on the X chromosome. IP mostly occurs in female infants, it is usually embryonic lethal in males. The mechanisms for survival of affected males are explained by the presence of an extra X chromosome (Klinefelter's syndrome), hypomorphic mutations, and somatic mosaicism. We report here a rare case of incontinentia pigmenti in a 13-year-old boy with Klinefelter's syndrome, NEMO gene mutation, and whorled, hyperkeratotic, hyperpigmented, linear lesions along the lines of Blaschko on the trunk and leg.
Adolescent ; Female ; Humans ; Incontinentia Pigmenti* ; Infant ; Klinefelter Syndrome* ; Leg ; Male ; Mosaicism ; X Chromosome

Osteogenesis imperfect (OI) is a generalized connective tissue disorder. We report the female neonate case with OI type III who showed severe bone deformities and fractures in utero. At birth, she showed multiple fractures in the clavicle, rib, femur, and wormian bone in the skull X-ray. We initiated pamidronate disodium infusion 30 mg/m2 in cycles of 3 consecutive days from when she was 4 days old, monthly for the first 3 months and every 2 months thereafter without adverse effects.
Bone Density ; Clavicle ; Congenital Abnormalities ; Connective Tissue ; Diphosphonates ; Female ; Femur ; Fractures, Bone ; Humans ; Infant ; Infant, Newborn ; Osteogenesis ; Osteogenesis Imperfecta ; Parturition ; Ribs ; Skull

Osteogenesis imperfect (OI) is a generalized connective tissue disorder. We report the female neonate case with OI type III who showed severe bone deformities and fractures in utero. At birth, she showed multiple fractures in the clavicle, rib, femur, and wormian bone in the skull X-ray. We initiated pamidronate disodium infusion 30 mg/m2 in cycles of 3 consecutive days from when she was 4 days old, monthly for the first 3 months and every 2 months thereafter without adverse effects.
Bone Density ; Clavicle ; Congenital Abnormalities ; Connective Tissue ; Diphosphonates ; Female ; Femur ; Fractures, Bone ; Humans ; Infant ; Infant, Newborn ; Osteogenesis ; Osteogenesis Imperfecta ; Parturition ; Ribs ; Skull