Sequential Bilateral Hip Fractures in Elderly Patients / 대한고관절학회지

Purpose@#To evaluate the incidence and presentation of osteoporotic sequential bilateral hip fractures (SBHF) in Center for Joint Disease, Chonnam National University Hwasun Hospital as there are limited studies with variable results reported in Korea. @*Materials and Methods@#Records of 507 patients aged >60 years old presenting with osteoporotic hip fractures between 2009 and 2015 were retrospectively reviewed to document the occurrence and presentation of sequential hip fractures; mean post-treatment follow-up was 48 months. Additionally, any correlations between sequential fractures and initial fracture and risk factors were assessed. Bone mineral density (BMD) was measured before and after sequential hip fracture for comparison. @*Results@#There were 246 femoral neck (Group A) and 261 intertrochanteric (Group B) fractures. The cumulative incidence of SBHF was 8.2% (42 patients total; 29 in Group A and 13 in Group B). Average interval of SBHF for Group A and Group B were 37.4 months and 29.9 months, respectively. There was significant correlation between the initial fracture type and sequential fractures, particularly the trochanteric and subgroup of those with neck fractures. Hypertension as a co-morbidity and female sex have been identified as risk factors for SBHF. No significant findings were noted regarding BMD and risk factors in both groups. @*Conclusion@#The clinical presentations of SBHF noted here concur with other worldwide studies and may guide efforts to develop relevant programs to prevent SBHF.

Breakage of the Tail Portion of the Lag Screw during Removal of Proximal Femoral Zimmer Natural Nail: Report of Two Cases with Technical Notes / 대한고관절학회지

Proximal femoral cephalomedullary nailing is one of the treatments of choice for intertrochanteric fracture. Zimmer Natural Nail® (ZNN; Zimmer) is one of the alternatives. We report two cases of broken tail portion of the ZNN lag screw during the removal procedure. This may be the first reported cases in scientific literature with this pattern of failure. We report these cases to highlight one of the possible problems that could occur during removal of this implant which can possibly prolong the operation time significantly if the required device is not prepared.

Breakage of the Tail Portion of the Lag Screw during Removal of Proximal Femoral Zimmer Natural Nail: Report of Two Cases with Technical Notes / 대한고관절학회지

Proximal femoral cephalomedullary nailing is one of the treatments of choice for intertrochanteric fracture. Zimmer Natural Nail® (ZNN; Zimmer) is one of the alternatives. We report two cases of broken tail portion of the ZNN lag screw during the removal procedure. This may be the first reported cases in scientific literature with this pattern of failure. We report these cases to highlight one of the possible problems that could occur during removal of this implant which can possibly prolong the operation time significantly if the required device is not prepared.

Parkinson's Syndrome Caused by Organophosphate Intoxication: A case report

Organophosphate intoxication causing the extrapyramidal symptom is not frequent. A case of Parkinson's syndrome caused by organophosphate intoxication was observed, of which is reported with the quantitative measurement of tremor using Tri-axial accelerometry. A fifty nine year-old male was admitted to Wonju Christian Hospital after the intake of organophosphate for the purpose of suicide and three days after the accident, involuntary movements were detected. The encephalography and MRI showed no abnormality. With Tri-axial accelerometry, we detected less than 4 Hz resting tremor. The tremor did not response to L-dopa, and in the follow up examination performed 149 days after the accident, an increase in amplitude was detected. Gait disturbance and dysarticulation became more severe. In a case of the organophosphate intoxication patient, very rare Parkinson's syndrome findings were detected, and the tremor during the resting period was measured quantitatively by electromyography and Tri-axial accelerometry.

Defibrotide Treatment for Hepatic Veno-occlusive Disease after Umbilical Cord Blood Transplantation / 대한소아혈액종양학회지

Veno-occlusive disease (VOD) of the liver is a life-threatening complication occurring early after blood or bone marrow transplantation (BMT). Effective treatment has not been established in case of severe forms of VOD. Defibrotide, a single-stranded polydeoxyribonucleotide, has been used on a compassionate basis in recent clinical trials with promising results. We report here with the first Korean experience of using defibrotide for the treatment of hepatic VOD occurring after unrelated umbilical cord blood transplant in a 2-year-old child with acute lymphoblastic leukemia. Defibrotide was administered for 23 days without any significant side effects with resolution of signs and symptoms of VOD.

The Frequency of Glycophosphatidyl Inositol-Anchored Protein-Negative Paroxysmal Nocturnal Hemoglobinuria Cells in Pediatric Aplastic Anemia Analyzed by Flow Cytometry / 대한소아혈액종양학회지

PURPOSE: Paroxysmal nocturnal hemoglobinuria (PNH) has been known to be a late clonal complication of aplastic anemia (AA). Flow cytometric analysis using CD55 and CD59 antibodies became the gold standard of diagnosing PNH, replacing a traditional, less sensitive Ham's test, as the pathophysiologic mechanism was identified as the deficiency of glycophosphatidyl-inositol anchored protein. Although the incidence of AA seems to be higher in Korea than that of other Western countries, the study of PNH in Korean pediatric AA has never been accomplished. We studied the frequency of PNH in AA, and tried to compare the characteristics of them with those from other countries. METHODS: Twenty-two pediatric AA patients were enrolled for the study. As a control, 5 patients with inherited bone marrow failure syndromes (Fanconi anemia, 1; Diamond-Blackfan anemia, 3; dyskeratosis congenita, 1) and 11 normal children were pooled. For the flow cytometry, 10muL each of CD55-PE and CD59-FITC was mixed with 50muL of whole blood and incubated for 15 min. Red cells were lysed with Q-prep apparatus (Coulter, Fullerton, USA). Beckman Coulter XL flow cytometer was used for the analyses. RESULTS: The median age for the patients was 14 years (range, 2~21). CD55- and CD59-negative cells from controls were 0.13+/-0.18%. Cut-off value for the diagnosis for PNH was designated as > 0.49%, which was mean +2 S.D. of controls. All the patients showed CD55- and CD59-negative PNH cell proportions within the normal ranges, except for a 19-year-old boy who was still cyclosporine-dependent after initial response to immunosuppressive therapy 4 years before. He had 4.79% of CD55- and CD59-negative PNH population. CONCLUSION: The frequency of PNH clones in Korean children with AA was low (1/22=4.5%). This might reflect the relatively low association of PNH in childhood AA, the limitation caused by small numbers of the study population, or true ethnic differences. A further study incorporating more patients seems to be warranted.

2-Chlorodeoxyadenosine for Children with Recurrent or Refractory Langerhans Cell Histiocytosis / 대한소아혈액종양학회지

PURPOSE: Langerhans cell histiocytosis (LCH) is a disorder characterized by the proliferation of activated Langerhans cells. Although current therapies are very effective at inducing remission, multiple recurrences and long-term sequelae are common for young patients. For this reason, more effective therapies based on the pathogenesis of LCH are needed. We investigated the use of 2-chlorodeoxyadenosine (2-CdA), a purine analogue with an antiproliferative effect on histiocytes and lymphocytes, in patients with recurrent or refractory LCH. METHODS: Four children with recurrent or refractory LCH received 2-CdA (5~7 mg/m2/day for 5 days, given as a 24-hr continuous infusion and repeated every 21~28 days for 5~7 courses). RESULTS: All four patients had multiorgan involvement, and were heavily pretreated. Of the two children with recurrent diseases, one had complete response and the other showed no active disease except for the remaining diabetes insipidus. Two infants who showed poor early response to previous combination chemotherapy also responded poorly: partial response in one, and progressive disease resulting in death in the other. Toxicity consisted mainly of myelosuppression, but significant infections did not occur. The peripheral neuropathy was not seen. CONCLUSION: 2-CdA, tolerable in children without significant side effects, might be effective for the treatment of recurrent LCH in children. However, the efficacy in infants with multi-system, refractory diseases needs further study. The feasibility of 2-CdA treatment as the first-line therapy for high-risk diseases, and the possibility of combination with other agents needs to be addressed in the future.

Dyskeratosis Congenita in a Girl / 대한소아혈액종양학회지

Dyskeratosis congenita (DC) is a rare genetic disorder encompassing abnormal skin pigmentation, dystrophic nails, leukoplakia of mucous membranes and others. Bone marrow failure is the cause of early mortality. Moreover, DC is known for its predisposition to malignancy. X-linked recessive, autosomal dominant and autosomal recessive forms of the disease are recognized. We describe here a rare case of DC in a 4-year-old girl showing dark skin, dystrophic toe nails, and mild bone marrow failure. Autosomal recessive disease was suggested as the patient is female, and tests for DKC1 and hTR mutations were negative. Intermittent treatment with oxymetholone and prednisolone for about 26 months resulted in stable hemoglobin and platelet response.

Minimally Differentiated Acute Myeloid Leukemia (AML-M0) in Children: Clinico-biological Characteristics of 4 Cases / 대한소아혈액종양학회지

PURPOSE: AML M0, a newly defined entity of AML, is a rare subtype with dismal outcome. We investigated the clinico-biologic characteristics of 4 AML M0 cases in childhood. METHODS: We reviewed the medical records of 4 AML M0 patients diagnosed at the Pediatric Department of Chonnam National University Hospital from Jan. 1995 to Dec. 2001. We analyzed the clinical, cytologic, cytochemical, immunologic and cytogenetic findings. Details on the treatments and their results were also described. RESULTS: AML M0 accounted for 5.4% (4/75) of newly diagnosed AML. Two of them were less than 2 years of age. One case was secondary leukemia following autologous transplantation for ALL L3. Morphologically, 3 cases displayed myeloblasts. All cases were having less than 3% of the blasts positive for myeloperoxidase (MPO) or Sudan black B (SBB) by light microscopy. Early precursor markers, such as CD34, HLA-DR, and CD7 were expressed in 100%, 100%, and 50% of cases, respectively. CD13 and/or CD33 were positive in all cases. Karyotypic abnormalities were demonstrated in 3: 2 involving chromosome 7 and one with complex abnormality. Three patients died from relapse and treatment related complications with the median survival duration of 4 mo (range 1-7 mo). CONCLUSION: AML M0 should be considered in leukemia cases negative for MPO/SBB, negative for lymphoid specific markers, and positive for myeloid markers. As those patients may have low possibility of getting remission as well as high risk of early relapse, more effective treatment strategies, such as stem cell transplantations, should be developed.

Invasive Aspergillosis in Immunocompromised Children / 대한소아혈액종양학회지

PURPOSE: An increasing number of immunocompromised patients are contracting opportunistic infections caused by Aspergillus, resulting in a significant morbidity and mortality. We reviewed the clinical presentation, radiologic characteristics, histopathologic findings, treatment strategies, and outcome of invasive aspergillosis (IA) in immunocompromised children. METHODS: Thirteen children having IA were retrospectively analyzed. RESULTS: Acute myelogenous leukemia (n=9, 69.2%) was the most common underlying disease, followed by acute lymphocytic leukemia (n=2), Fanconi anemia (n=1), and chronic granulomatous disease (CGD, n=1). Pulmonary involvement was present in 12 patients (92.3%). The sinuses or nose were involved in 4 (30.8%). The patient with CGD had lung, soft tissue, and bone involvement. Central nervous system, gastrointestinal, heart involvement were not documented. Histology or culture proven IA were found in 6 patients (46.2%). In pulmonary IA, typical findings of thoracic computed tomography, such as halo sign or air-crescent sign, was observed in 6 patients. Amphotericin B was given to all patients along with itraconazole (69.2%), G- or GM-CSF (84.6%). AmBisome was subsequently substituted for Amphotericin in 4. One patient with pulmonary mycetoma underwent lobectomy. Seven patients (53.8%) were improved by antifungal measures, but no patients achieved a long term survival. IA was implicated as a cause of death in 7 (4 with massive pulmonary hemorrhage). Most of the rest succumbed to the relapse of underlying leukemia. CONCLUSION: IA remains a formidable infection in immunocompromised children despite current treatment. Lung was the most common site of infection and massive pulmonary hemorrhage might ensue. Early diagnosis and development of effective measures, including surgery, are warranted.