Kostmann's disease is a rare hematologic disease entity which is characterized by persistent neutropenia and recurrent bacterial infections since early in infancy. The authors experienced a 2-month-old female infant with disseminated bacterial skin infection since 3 weeks of age and persistent neutropenia. Diagnosis was made by neutropenia in the peripheral blood, maturation arrest of myeloid hemopoiesis at myelocyte stage in the finding of bone marrow aspiration and improving response of neutropenia with G-CSF administration. She had experienced 4 episodes of sepsis and one episode of acute otitis externa during follow-up until 14 months of age.
Bacterial Infections ; Bone Marrow ; Bone Marrow Transplantation ; Diagnosis ; Female ; Follow-Up Studies ; Granulocyte Colony-Stimulating Factor ; Granulocyte Precursor Cells ; Hematologic Diseases ; Humans ; Infant ; Neutropenia ; Otitis Externa ; Sepsis ; Skin

No abstract available.
Blood Transfusion* ; Humans ; Infant, Newborn ; Infant, Premature*

Purpose: We report on the 10-year clinical hip function and radiologic outcomes of patients who underwent hip arthroplasty using a COREN stem. Materials and Methods: A consecutive series of 224 primary cementless hip arthroplasty implantations were performed using a COREN stem between 2009 and 2011; among these, evaluation of 128 hips was performed during a minimum follow-up period of 10 years. The mean age of patients was 65.4 years (range, 40-82 years) and the mean duration of follow-up was 10.8 years (range, 10-12 years). Evaluation of clinical hip function and radiologic implant outcomes was performed according to clinical score, thigh pain, and radiologic analysis. Results: Dramatic improvement of the mean Harris hip score (HHS) from 59.4 preoperatively to 93.5 was observed at the final follow-up (P≤0.01). Stable fixation was demonstrated for all implants with no change in position except for one case of Vancouver type B2 periprosthetic femur fracture. A radiolucent line (RLL) was observed in 16 hips (12.5%). Thigh pain was observed in only two hips (1.6%) at the final follow-up. There were no cases of osteolysis around the stem. The survival rate for the COREN stem was 97.7%. Conclusion Good long-term survival with excellent clinical and radiological outcomes can be achieved using the COREN femoral stem regardless of Dorr type.

BACKGROUND/AIMS: Therapies involving bone-marrow-derived mesenchymal stem cells (BM-MSCs) have considerable potential in the management of hepatic disease. BM-MSCs have been investigated in regenerative medicine due to their ability to secrete various growth factors and cytokines that regress hepatic fibrosis and enhance hepatocyte functionality. The aim of this study was to determine the antifibrosis effect of BM-MSCs on activated hepatic stellate cells (HSCs) and the mechanism underlying how BM-MSCs modulate the function of activated HSCs. METHODS: We used HSCs in both direct and indirect co-culture systems with BM-MSCs to evaluate the antifibrosis effect of BM-MSCs. The cell viability and apoptosis were evaluated by a direct co-culture system of activated HSCs with BM-MSCs. The activations of both HSCs alone and HSCs with BM-MSCs in the direct co-culture system were observed by immunocytochemistry for alpha-smooth muscle actin (alpha-SMA). The levels of growth factors and cytokines were evaluated by an indirect co-culture system of activated HSCs with BM-MSCs. RESULTS: The BM-MSCs in the direct co-culture system significantly decreased the production of alpha-SMA and the viability of activated HSCs, whereas they induced the apoptosis of activated HSCs. The BM-MSCs in the indirect co-culture system decreased the production of transforming growth factor-beta1 and interleukin (IL)-6, whereas they increased the production of hepatocyte growth factor and IL-10. These results confirmed that the juxtacrine and paracrine effects of BM-MSCs can inhibit the proliferative, fibrogenic function of activated HSCs and have the potential to reverse the fibrotic process by inhibiting the production of alpha-SMA and inducing the apoptosis of HSCs. CONCLUSIONS: These results have demonstrated that BM-MSCs may exert an antifibrosis effect by modulating the function of activated HSCs.
Apoptosis ; Bone Marrow Cells/*cytology ; Cell Differentiation ; Coculture Techniques ; Hepatic Stellate Cells/*cytology/metabolism ; Hepatocyte Growth Factor/metabolism ; Humans ; Immunophenotyping ; Interleukin-10/metabolism ; Interleukin-6/metabolism ; Liver Cirrhosis ; Mesenchymal Stromal Cells/*cytology/metabolism ; Transforming Growth Factor beta1/metabolism

No abstract available.
Humans*

No abstract available.
Arthritis* ; Immunoblastic Lymphadenopathy*

Typhlitis is one of the most ominous complications in immunocompromised patients. Neutropenic enterocolitis or typhlitis is a clinical syndrome characterized by fever, diarrhea and abdominal pain that occurs in neutropenic patients. It has been reported as a complication of childhood leukemia, but is now known to occur in adults with solid malignancies, acquired immunodeficiency syndrome (AIDS) or bone marrow transplantation (BMT). The association of typhlitis and propylthiouracil has not been previously reported. We report a case of a 42-year-old female patient with typhlitis due to propylthiouracil patient with hyperthyroidism.
Abdominal Pain ; Acquired Immunodeficiency Syndrome ; Adult ; Bone Marrow Transplantation ; Diarrhea ; Enterocolitis, Neutropenic ; Female ; Fever ; Humans ; Hyperthyroidism* ; Immunocompromised Host ; Leukemia ; Propylthiouracil* ; Typhlitis*

No abstract available.

No abstract available.

The literature concerning cerebral vasospasm associated with subarachnoid hemorrhage(SAH) due to ruptured intracranial aneurysm contains no definitive study of patients to determine whether there is (1) any clinical picture consistently present coincident with known cerebral vasospoasm, (2) any relationship between mortality and known vasospasm, and (3) any relationship between serious brain damage(morbidity) and known vasospasm. To answer these important questions, experience with 138 consecutive acute SAH patients due to ruptured intracranial aneurysm was studied. The results were (1) The vasospasm was seen in 61 patients(44.2%) and the clinical vasospasm was seen in 41 patients(29.8%);(2) The average interval between last SAH and vasospasm was 8.3 days;(3) The most common neurological deficit was a hemiparesis or a hemiplegia;(4) There is no relationship between mortality and vasospasm.
Brain ; Humans ; Intracranial Aneurysm* ; Mortality ; Paresis ; Vasospasm, Intracranial