Case report: Recurrent hyperammonaemic encephalopathy due to citrullinaemia in a 52 year old man

The present report describes a Chinese male who presented for the first time with recurrent encephalopathy and hyperammonaemia at the age of 52 years. He was found to have citrullinaemia. To our knowledge, this is the first Chinese with citrullinaemia and the first non-Japanese who has the variant form of presentation. The patient also has the longest asymptomatic period for citruliinaemia so far described. The patient's biochemical derangement, clinical features and the postulation of his late presentation are discussed. It is noteworthy that simple therapeutic measures, such as lactulose and dietary protein restriction, controlled his symptoms well.     

5-Hydroxy-3-indole acetic acid levels in infantile colic: Is serotoninergic tonus responsible for this problem?

Abstract Levels of 5-hydroxy-3-indole acetic acid (5-OHIAA), a metabolite of serotonin, were studied in infants with infantile colic in order to investigate the aetiology of infantile colic pathogenesis. The study included 16 patients with infantile colic and 10 control subjects. Random urinary 5-OH IAA levels of colicky infants were found to be higher than those in the control group. This finding suggested that high serotonin levels may be responsible for infantile colic.     

Factors which affect the use of lumpectomy and mastectomy in an underinsured,safety net hospital population

Background

This study was performed to evaluate variables that affect the use of mastectomy and lumpectomy in an underinsured population.

Methods

A retrospective review of all patients who underwent breast cancer operations from July 2001 to February 2011 at a safety net hospital was performed. Univariate and multivariate analyses were performed to identify variables, which were associated with the type of operation.

Results

Of the 412 patients, 81% of the patients were underinsured or uninsured. Most patients (58%) presented with clinical stage 2A/B disease. Mastectomy was performed in 37% of patients and lumpectomy in 63%. In multivariate analysis, clinical tumor size (P = .035) and pathologic stage (P = .003) remained associated with mastectomy, while use of preoperative chemotherapy (P = .004) and type of surgeon (P = .001) was associated with lumpectomy.

Conclusions

Most patients underwent lumpectomy despite later stage at presentation. Preoperative chemotherapy was associated with increased likelihood of lumpectomy.     

Megakaryocyte growth and development factor accelerates platelet recovery in peripheral blood progenitor cell transplant recipients

We have investigated the potential of PEGylated recombinant human megakaryocyte growth and development factor (PEG-rHuMGDF), a molecule related to thrombopoietin (mpl ligand or TPO) in minimizing the thrombocytopenia associated with hematopoietic ablation and peripheral blood progenitor cell (PBPC) transplant. Irradiated mice that received PBPC mobilized by PEG-rHuMGDF or granulocyte colony-stimulating factor (G-CSF) had a reduced number of thrombocytopenic days with platelets below 100 x 10(6) per mL of blood. Recipients of unmobilized PBPC had a 9 day thrombocytopenic phase which was shortened to 7 days if they were given granulocyte-macrophage colony-stimulating factor (GM-CSF)- mobilized PBPC. This was further reduced to 2 or 3 days of thrombocytopenia in recipients of G-CSF- or PEG-MGDF-mobilized PBPC. Despite our observation that PEG-rHuMGDF is a relatively modest stimulator of the mobilization of myeloid progenitors to the blood, MGDF-mobilized PBPC do effect accelerated recovery of platelets after transplantation. However, the most effective use of PEG-rHuMGDF is when it is given during the recovery phase after PBPC transplantation to hematopoietically ablated mice. Posttransplant treatment with PEG- rHuMGDF reduces thrombocytopenia to a single day or less, in recipients of most types of PBPC. Mice that were treated during the first 2 weeks after PBPC transplant with PEG-rHuMGDF had 1 thrombocytopenic day compared to 9 days in carrier-treated recipients of unmobilized PBPC and 2 to 3 days in carrier-treated recipients of the optimally mobilized PBPC from G-CSF or G-CSF/PEG-rHuMGDF treated donors. In groups where PEG-rHuMGDF was included in the mobilization protocol and used to treat recipients as well thrombocytopenia was effectively eliminated. These data show that PEG-rHuMGDF is a highly effective agent in eliminating the thrombocytopenia associated with PBPC transplantation.     

Studies on the myeloid synergistic factor from 5637: comparison with interleukin-1 alpha

A synergistic factor that is produced by the human bladder carcinoma cell line 5637 (SF-1) stimulates primitive bone marrow progenitor cells, termed high proliferative-potential colony-forming cells (HPP- CFC), in the presence of an optimal dose of macrophage colony stimulating factor (CSF-1). Recent reports have demonstrated that interleukin-1 alpha (IL-1) is identical to hemopoietin 1 and have suggested that IL-1 is the synergistic factor present in 5637 conditioned medium (cm). We have compared the ability of recombinant human IL-1 alpha and partially purified preparations of SF-1 to synergize with optimal doses of CSF-1 to stimulate HPP-CFC. In all experiments performed the numbers of HPP-CFC colonies formed with IL-1 were significantly less than with SF-1. Replating experiments demonstrated that SF-1 plus CSF-1 generated HPP-CFC (responsive to IL-3 plus CSF-1); however, IL-1 plus CSF-1 resulted in no generation of HPP- CFC. Multiple factor combinations of IL-1 and SF-1 with G-CSF, GM-CSF, and CSF-1 also resulted in less HPP-CFC colony formation in cultures containing IL-1 compared with SF-1. Incubation of SF-1 with an antibody to IL-6 had no effect on HPP-CFC colony formation and IL-6 did not synergize with IL-1 plus CSF-1 or SF-1 plus CSF-1. These data suggest the presence of a factor in 5637 cm, which is distinct from G-CSF, GM- CSF, and IL-6, which synergizes with IL-1 to produce the SF-1 effect.     

In vivo synergy between recombinant human stem cell factor and recombinant human granulocyte colony-stimulating factor in baboons enhanced circulation of progenitor cells

Recombinant human stem cell factor (rhSCF) and recombinant human granulocyte colony-stimulating factor (rhG-CSF) are synergistic in vitro in stimulating the proliferation of hematopoietic progenitor cells and their precursors. We examined the in vivo synergy of rhSCF with rhG-CSF for stimulating hematopoiesis in vivo in baboons. Administration of low-dose (LD) rhSCF (25 micrograms/kg) alone did not stimulate changes in circulating WBCs. In comparison, administration of LD rhSCF in combination with rhG-CSF at 10 micrograms/kg or 100 micrograms/kg stimulated increases in circulating WBCs of multiple types up to twofold higher than was stimulated by administration of the same dose of rhG-CSF alone. When the dose of rhG-CSF is increased to 250 micrograms/kg, the administration of LD rhSCF does not further increase the circulating WBC counts. Administration of LD rhSCF in combination with rhG-CSF also stimulated increased circulation of hematopoietic progenitors. LD rhSCF alone stimulated less of an increase in circulating progenitors, per milliliter of blood, than did administration of rhG-CSF alone at 100 micrograms/kg. Baboons administered LD rhSCF together with rhG-CSF at 10, 100, or 250 micrograms/kg had 3.5- to 16-fold higher numbers per milliliter of blood of progenitors cells of multiple types, including colony-forming units granulocyte/macrophage (CFU-GM), burst-forming unit-erythroid (BFU-E), and colony-forming and burst-forming units-megakaryocyte (CFU- MK and BFU-MK) compared with animals given the same dose of rhG-CSF without rhSCF, regardless of the rhG-CSF dose. The increased circulation of progenitor cells stimulated by the combination of rhSCF plus rhG-CSF was not necessarily directly related to the increase in WBCs, as this effect on peripheral blood progenitors was observed even at an rhG-CSF dose of 250 micrograms/kg, where coadministration of LD rhSCF did not further increase WBC counts. Administration of very-low- dose rhSCF (2.5 micrograms/kg) with rhG-CSF, 10 micrograms/kg, did not stimulate increases in circulating WBCs, but did increase the number of megakaryocyte progenitor cells in blood compared with rhG-CSF alone. LD rhSCF administered alone for 7 days before rhG-CSF did not result in increased levels of circulating WBCs or progenitors compared with rhG- CSF alone. Thus, the synergistic effects of rhSCF with rhG-CSF were both dose- and time-dependent. The doses of rhSCF used in these studies have been tolerated in vivo in humans.(ABSTRACT TRUNCATED AT 400 WORDS)     

Endovenous Laser Ablation of Varicose Veins after Direct Percutaneous Puncture: Early Results

BACKGROUND: Although endovenous laser ablation has been demonstrated to effectively occlude incompetent saphenous veins, it does not treat branch varicosities directly. OBJECTIVE: The objective was to evaluate the technical feasibility and early results of the direct ablation of branch varicosities using a 980-nm diode laser. MATERIALS AND METHODS: From July to August 2005, 17 patients (21 limbs; male:female ratio, 7:10; mean age, 50.1 years; range, 24-62 years) with varicose veins were enrolled in this study. Endovenous laser ablation was performed in incompetent saphenous veins. Thereafter, branch varicosities were percutaneously punctured and a direct laser ablation was performed using a 400-microm laser fiber. Patients were evaluated at 1 week and at 1-, 3-, and 6-month intervals. RESULTS: Technical success in branch varicosities was achieved in 11 of 15 limbs (71.4%). Continued closure of treated saphenous veins was evident in 11 of 11 limbs (100%) at 1-month follow-up. Successful ablation in varicose tributaries was seen in 6 of 11 limbs (54.5%) at 1-month follow-up and ablation failed in 5 limbs. One of the 11 limbs (9%) had a skin burn that required treatment. CONCLUSION: Despite partly successful occlusion, we suggest that direct laser ablation cannot replace classic methods of treating branch varicosities, because of its high failure rate and the risk of skin burns.     

Radiology Image Management in a Teaching Hospital Network Scenario: Initial Experience

INHS Asvini has the distinction of emerging as the first Armed Forces Hospital with networked radiology images, sourced principally from a recently installed Digital Substraction Angiography (DSA) machine (Siemens Polystar Top 1000 MA). In recent times, newer imaging machines offer digital images that are in DICOM format. DICOM denotes ‘Digital Imaging and Communications in Medicine”. Image management at INHS Asvini, involves transfer of DSA images in DICOM format, their conversion into Joint Photographic Experts Group (JPG) format and subsequent transmission of JPG images through a robust networking system that connects all departments/OPD/Wards, facilitating direct access of images to a variety of specialists and specialities. Clearly, this encouraging outcome is a small but useful step heralding the concept of ‘anytime anywhere’ images, and has the scalable expansibility of including CT and MRI images in future.Key Words: DICOM, Hospital network, Image management