Pain management trend of vaso-occulsive crisis (VOC) at a community hospital emergency department (ED) for patients with sickle cell disease.

Pain management at the emergency department (ED) for vaso-occulsive crisis (VOC) for patients with sickle cell disease has not been optimum, with a long delay in giving the initial analgesic. We conducted a retrospective survey over a 7-year period to determine our ED's timing in giving pain medication to patients with VOC as a quality improvement project. We compared different periods, children vs adults, and the influence of gender in the analgesic administration timing. This is a retrospective chart review of three different periods: (1) years 2007-2008, (2) years 2011-2012, and (3) year 2013. We extracted relevant information from ED records. Data were analyzed using Student t test, chi-square analysis, and the Kruskal-Wallis test. There was a progressive improvement in the time interval to the 1st analgesic over these three periods. Children received analgesics more quickly than adults in all periods. Male adult patients received pain medication faster than female adult patients, although initial pain scores were higher in female than in male patients. Progressively fewer pediatric patients utilized ED over these three periods, but no difference for adult patients was observed. The proportion of pediatric patients admitted to the hospital increased with each period. The progressive decrease in both the number of patients and the number of visits to the ED by children suggested that the collective number of VOC in children has decreased, possibly secondary to the dissemination of hydroxyurea use. We failed to observe the same trend in adult patients. The need for IV access, and ordering laboratory tests or imaging studies tends to delay analgesic administration. Delay in administration of the first analgesic was more pronounced for female adult patients than male adult patients in spite of their higher pain score. Health care providers working in ED should make conscious efforts to respect pain in women as well as pain in men. Though not proven from this study, we believe that a significantly wider use of hydroxyurea by adult patients most likely would reduce their utilization of ED for the purpose of relief of pain, and further pediatric hematologists may be better positioned to increase hydroxyurea adherence by young adult patients, since they have had established rapport with them before transitioning to adult care.

Clear cell sarcoma of the jaw: a case report and review of the literature.

Clear cell sarcoma (CSS) is a unique malignant soft tissue tumor that mainly occurs from the aponeurotic tissue and tendons of extremities. It is rare in the pediatric population. The tumor does not respond well to chemotherapy or irradiation. Complete surgical resection offers the best chance for a cure. Most studies have demonstrated poor prognosis of this tumor, if it is >5 cm. The literature suggests that local recurrence and distant metastasis are not uncommon even with wide resection and that late recurrence and metastasis commonly occur. This case report discusses CSS in the jaw of a pediatric patient. To our knowledge, this is the only case of CSS of the jaw.

Symptomatic erythrocytosis associated with a compound heterozygosity for Hb Lepore-Boston-Washington (δ87-ß116) and Hb Johnstown [ß109(G11)Val→Leu, GTG>TTG].

Hb Johnstown [ß109(G11)Val→Leu, GTG>TTG] has previously been described as a high oxygen affinity variant in a heterozygous state and in combination with ß(0)-thalassemia (ß(0)-thal). Because the variant does not separate from Hb A by routine methods it may be easily missed unless clinical suspicion is high. Hb Lepore-Boston-Washington (Hb LBW; δ87-ß116) is a 뫧 hybrid variant that clinically manifests similarly to a ß(+)-thal. Hb LBW is not detected by routine polymerase chain reaction (PCR) sequencing but is easily detected by electrophoretic methods. We describe a 19-year-old African American male with a compound heterozygosity for Hb Johnstown and Hb LBW. The patient presented with progressively worsening chest pains, headaches and erythrocytosis. He was repeatedly phlebotomized with symptomatic improvement and subsequently was confirmed to have the high oxygen affinity hemoglobin (Hb) variant. The lowest Hb and hematocrit (packed cell volume, PCV) achieved by phlebotomy was 16.1 g/dL and 0.51 L/L, respectively. Currently, he is no longer being phlebotomized, and is feeling relatively well except for minor chest pain. It is unclear to what degree the phlebotomies contributed to his subjective improvement. The combination of Hbs Johnstown and LBW has not been heretofore described, and in this case, was associated with marked symptomatic erythrocytosis. This unique combination results in a more pronounced phenotype, similar to or slightly more severe than, compound Hb Johnstown/ß(0)-thal. This compound hemoglobinopathy will likely not be correctly classified using a single method of Hb detection and underscores the need for multiple characterization methods when indicated by the clinical picture.

Rothmund-Thomson syndrome (RTS) with osteosarcoma due to RECQL4 mutation.

Rothmund-Thomson syndrome (RTS) is a rare autosomal recessive disorder with clinical features consisting of poikiloderma, skeletal abnormalities, sparse hair, absent or scanty eyelashes and eyebrows and short stature. Patients with RTS due to genetic mutations of RECQL4 genes carry a high risk of developing osteosarcoma during childhood. Because of this, early genetic diagnosis is important. Here, we describe a 14-year-old white boy who developed an erythematous rash on both cheeks before the age of 3 months and was noted to have absent eyelashes and scanty eyebrows. He was found to have compound heterozygous mutations of the RECQL4 gene alleles at the age of 6 months and was diagnosed to have RTS type II. He subsequently developed osteosarcoma at age 10 which was successfully treated, and currently he has been tumour free for over 3 years.

Optimal conditions for visualizing water-conducting pathways in a living tree by the dye injection method.

To elucidate the water-conducting pathways in living trees by the dye injection method, suitable sample preparation procedures are needed. We evaluated quantitatively the properties and concentrations of three dyes (acid fuchsin, basic fuchsin and safranin) widely used for this purpose, and determined the optimal conditions required to avoid artifacts after dye injection into the sap stream of Pieris japonica D. Don. Among the dyes tested, an aqueous solution of acid fuchsin at a concentration of 0.1% or more was the most useful for delineating water movement. In non-transpiring stem segments, the vertical movement of acid fuchsin by capillarity and diffusion from the dye injection site was limited. However, acid fuchsin moved rapidly in the horizontal direction by capillarity and diffusion, and most xylem cells were stained within 2 h. A delay of more than 2 h between dye injection and examination of the tissues greatly reduces the precision of the method. Use of the dye injection method without appropriate, well-defined experimental procedures may give rise to misleading information about the functional water-conducting pathway in living trees.

[Epidemiological survey of sexually transmitted male urethritis in Kyoto Prefecture].

The data of sexually transmitted urethritis in males have been collected at 24 institutes in Kyoto Prefecture since October, 2002. The data collected from January to December in 2004 are summarized herein. A total of 1,275 patients were diagnosed with urethritis during this period. Microbiological examinations isolated Neisseria gonorrhoeae alone in 368 (29%), Chlamydia tracomatis alone in 336 (26%), both in 85 (7%), and others in 453 (36%). Male patients under 20 years old tended to have Chlamydial urethritis, alone or combined with gonococcal infection, and had a predominant infectious source, a non-commercial-sexual-worker female partner, suggesting a profound problem in sexual life of adolescents. The urologist preferred to use quinolones as the first therapeutic modality against male urethritis. However, drug resistance of N. gonorrhoeae, especially against quinolones, has rapidly progressed, which was also observed by a sensitivity examination test. Antibiotics should be used adequately against male urethrits according to the recent guidelines.

Adherence to hydroxyurea medication by children with sickle cell disease (SCD) using an electronic device: a feasibility study.

Adherence to hydroxyurea (HU) is a significant modifying factor in sickle cell vaso-occlusive pain. We conducted a study using an electronic medication container-monitor-reminder device (GlowCap™) to track adherence and determine whether use of this device affected rates of HU adherence. Subjects were regular attendees to our clinic. They were given a 37-item questionnaire and were asked to use a GlowCap containing HU. When the device cap is opened, it makes a remote "medication taken" record. The device also provides usage reminder in the form of lights and alarm sounds if the cap opening is delayed. Nineteen subjects participated in the survey, and 17 in the intervention phase. Of the 17, 12 had reliable adherence data. Seventeen caregivers of patients and two patients completed the survey. Two most common barriers to adherence identified were lack of reminders and absence of medicine home delivery. The intervention component of this study, which used both the electronic (GlowCap) method and medication possession ratio showed that the median adherence rate for the 12 patients evaluated was 85 %. The GlowCap device accurately kept a record of adherence rates. This device may be an effective tool for increasing HU medication adherence.

Forme Fruste of HLH (haemophagocytic lymphohistiocytosis): diagnostic and therapeutic challenges.

Infants and young children often present with a persistent febrile episode, sick appearance and negative infectious disease work-up. These patients present serious diagnostic and therapeutic problems to those who provide medical care, particularly since these children are clinically sick. We present a 13 month old child who presented with this clinical challenge. She was ultimately thought to have an incomplete form of HLH with underlying pathophysiology of hypercytokinemia, but also could have been a case of incomplete form of Kawasaki disease. She responded to IVIG, but this does not differentiate one diagnosis from another. Unfortunately we failed to obtain tests to exclude genetic etiologies of HLH, which would be important for predicting severity and risks of future recurrence. We wish to present this case so that one should do a thorough work up to establish a firm diagnosis of HLH and to search for genetic causes of this disorder.

Evaluation of the compression characteristics and physical properties of the newly invented one-step dry-coated tablets.

This study was conducted in order to clarify the compression characteristics, and to confirm the superiority of the physical properties, of the newly invented One-Step DRy-Coated tablets (OSDRC). We compared both the compression characteristics and the physical properties of OSDRC with those of physical-mixture tablets (PM) that were prepared with the same ingredients, quantity, and compression pressures. We selected potassium chloride (KCl) and acetaminophen (AAP) as the model drugs, since the former is known for its appropriate compression characteristics and the latter for its brittleness. The advantage of OSDRC is that they are capable of maintaining any kind of drug in their core, because the core is tightly surrounded by the outer layer, even when the drugs in the core have poor compression characteristics, which causes difficulties in forming a solid core tablet using conventional dry-coated tablet methods. The radial tensile strength of OSDRC was the same as, or superior to, that of PM containing of AAP. The results were in accordance with the compression process analysis performed according to Kawakita's equation. The friability of OSDRC was also superior to that of PM. These preferable characteristics were attributable to the high intensity of the OSDRC outer layer surface in comparison to that of PM. It was difficult to clarify the difference between OSDRC and PM in their physical properties when KCl was applied, since the tabletability of the whole tablet was high due to KCl's physical properties. The OSDRC containing AAP in their cores showed a controlled release pattern, though no other materials that have been known to influence drug release was present. It was considered that this controlled release pattern was caused by a reduced AAP particle surface area due to compression. It was confirmed with these experiments that the compression characteristics and the physical properties of AAP-OSDRC were superior to those of PM. These results indicated that it is possible to produce tablets that have materials with poor compression characteristics in the core portion, and high tabletability materials for the outer layers. In other words, it is possible to produce capsule-like tablets using the OSDRC compression method.

Comparison of the compression characteristics between new one-step dry-coated tablets (OSDRC) and dry-coated tablets (DC).

One-step dry-coated tablets (OSDRC) were prepared using materials which are generally used in pharmaceutical tablets. The radial tensile strength of OSDRC was measured for various compression pressures and core porosities before the final compression to compare with that of conventional dry-coated tablets (DC). Furthermore, stress relaxation in the compression process was investigated. Radial tensile strength and stress relaxation profiles of OSDRC were the same as those of conventional DC. X-ray computerized tomography (CT) of the tablets showed that the density distribution of both tablets was also the same. Thus, we concluded that OSDRC and conventional DC have the same compression characteristics and physical properties. The OSDRC-system was executed by the use of upper and lower punches, which had a double structure, a center punch, and an outer punch surrounding the center punch. The OSDRC process consists of three compressions to make the lower-outer layer (1st-outer layer), the core, and the whole tablet including the upper-outer and side-outer layers (2nd-outer layer). At first, the powder for the 1st-outer layer fills a space, which is made by the lower-center punch and lower-outer punch, and is pre-compressed by the upper-center punch. Then, while the upper-center punch pushes the pre-compressed 1st-outer layer, the lower-center punch is slid down. The upper-center punch is then pulled away to make a space, which is filled with the powder for the core. This is then pre-compressed by the upper-center punch. Finally, the lower-outer punch is slid downward and the powder for the 2nd-outer layer fills and surrounds the pre-compressed core/1st-outer layer completely. The core/1st-outer layer and the 2nd-outer layer complex is then compressed by the upper and lower punches in which the center punches are unified with the outer punches, respectively. This system can be assembled onto the turn table of a rotary tableting machine, and can make a dry-coated tablet in a single turn.

Acute haemolytic anaemia and myolysis due to G6PD deficiency.

A 2-year-old African-American male patient with sickle cell trait developed cough, red coloured urine, pallor and fatigue. The patient was hospitalised. Diagnostic workup showed that he was glucose 6 phosphate dehydrogenase (G6PD) deficient in erythrocytes. He also had chest X-ray findings of pneumonia. His urine examination showed the presence of haemoglobin and myoglobin. On repeated questioning it was found that he had a moth ball in his mouth a few days prior to this medical episode. This case illustrates a rarely described complication of myolysis in G6PD deficient persons on exposure to a strong oxidant. A review of the literature showed that most people with G6PD deficiency tolerate exercise well without untoward effect in muscles. However, assay of myoglobin in urine has not been routinely performed in these patients during acute haemolytic episode, and thus it is uncertain how frequent myoglobulinaemia occurs in a similar stress situation.

Postinduction Supportive Care of Pediatric Acute Myelocytic Leukemia: Should Patients be Kept in the Hospital?

Children with AML become profoundly neutropenic while they undergo remission induction chemotherapy. It is unknown whether these children should be kept in the hospital while they are severely neutropenic to prevent life-threatening complications associated with neutropenia and reduce fatality. We at our institution routinely discharge patients after completing remission induction chemotherapy in the presence of profound neutropenia, unless it is clinically contraindicated. We reviewed all AML patients who were consecutively treated at our hospital from 1989 to 2011. Thirteen patients were electively discharged after completion of induction I chemotherapy. Of the 13, 4 died due to relapse or complications of stem cell transplants (not due to neutropenia related complications). Another eight are long term survivors. In this very small series, discharge from the hospital even though patients were severely neutropenic did not adversely affect the survival.

A rapid increase in the normal white cell counts without blasts as the initial presentation of T-cell acute lymphoblastic leukaemia.

A 5-year-old girl with multiple enlarged cervical lymph nodes consistent with Epstein-Barr virus infection showed a rapid increase in the white blood cell counts (myeloid cells and lymphocytes) without blasts over a week period. Bone marrow evaluation performed after a week's observation unexpectedly showed replacement of the marrow with T lymphoblasts. A presentation of T-cell acute lymphoblastic leukaemia (T-ALL) in this fashion is very unusual. We postulate that the T-lymphoblasts may have been secreting granulocyte colony stimulating factor like substance. We are unable to find a similar case report in the literature, and thus we wish to report this case. The patient has been treated with Children's Oncology Group T-ALL protocol, and has been in continuous remission.

Multiple fractures due to osteogenesis imperfecta mistaken as child abuse.

UNLABELLED: A 15-month-old African-American boy receiving chemotherapy for Wilms tumour was diagnosed to have a fracture of left femur at the emergency department (ED) of our hospital. A month earlier, the patient had been seen at the same ED for a fracture of right femur. The skeletal survey this time also showed an old posterior rib fracture. Child abuse was suspected. The child's custody was transferred to the maternal grandparents. However, 2 months later while with the grandparents, he sustained a fracture of the left distal tibia. This led to an investigation for osteogenesis imperfecta (OI). The child was found to have a collagen mutation, COL1A1, strongly suggesting that the child's multiple fractures were most likely due to OI.The child had no physical stigmata of classical OI except for blue sclera. Multiple bone fractures alone without other physical signs of abuse should always raise a possibility of OI. TRIAL REGISTRATION NUMBER: POG9440.