STUDY OF SPLENOMEGALY IN CHILDREN.

Splenomegaly is common clinical finding in pediatric practice. Splenic enlargement occurs when the spleen is increased by cells or tissue components or by vascular engorgement. Various etiologies can cause splenomegaly. The spleen is rarely the primary site of a disease. Splenomegaly is classified according to the length palpable below the costal margin as mild: <3 cm, moderate: 4-7 cm & massive: >7 cm. Severe/massive splenomegaly doesn't commonly occur in first 5 years of age, occurs after 5 years of age. So, clinical examination of every child is important to diagnose splenomegaly at early stages. Only in the age group of 1 to 5 years females predominated as compared to males. In present study there is obvious male predominance as male: female ratio is 1.8:1. This difference could be due to more priority to male child to seeking medical care with such chronic illness.Present study also suggested that severe/massive splenomegaly doesn't commonly occur in first 5 years of age, occurs after 5 years of age. So, clinical examination of every child is important to diagnose splenomegaly at early stages.

Characteristics, Treatment and Outcome of 15 to 18 Years-Old Adolescents with Chronic Myeloid Leukemia (CML): The Experience of the International Registry of Childhood CML (I-CML-Ped Study)

Introduction: CML is a rare disease in children and adolescents, accounting for 2-3% of leukemia cases in this population. Pediatric CML is more frequent in the 2 nd decade of life but data on adolescents are limited. Only response to treatment has been analyzed in few studies and seems to be poorer in this age cohort. The International Registry of Childhood Chronic Myeloid Leukemia gave us the opportunity to analyze the characteristics and outcome in a large cohort of adolescents. Aims and objectives: To report on clinical features and response to treatment in adolescents (15-18 years of age at diagnosis) with CML in chronic phase (CML-CP). Material and methods: The International Registry of Childhood CML (I-CML-Ped Study, registered at www.clinicaltrials.gov NCT01281735) enrolled patients less than 18 years of age at diagnosis of CML in all phases according to the criteria of the European Leukemia Net (ELN). Data from this registry collected from Jan 2011 - Mar 2021 into the I-CML-Ped Study (Poitiers, France) were retrospectively analyzed. Results: Out of 614 patients (pts) registered in the I-CML-Ped Study, we identified 144 (23.4%) adolescents (15-18 years of age at diagnosis) with sufficient available data. Among them, according to the ELN criteria, 132 (92%), 7 (5%), and 5 (3%) pts presented with CML-CP, accelerated phase (AP), and blastic phase (BP), respectively. The median age of the cohort comprising 132 adolescents with CML-CP was 16.2 years [range, 15-18]. Ratio male/female was 1.75. Splenomegaly was reported in 66% of the pts with a median spleen size of 11.5 cm below the costal margin [range, 1-32]. Hepatomegaly was reported in 31% of the pts with a median liver size of 2.5 cm below the costal margin [range, 1-19]. Pain, asthenia and weight loss were the most frequent symptoms at diagnosis in 35%, 33%, and 20% of pts, respectively. The performance status according to the OMS and Karnofsky scores were 0 and 100% in 81% and 57% of the pts, respectively. At diagnosis, median leukocyte count was 181 G/L [range, 7-820]; median platelet count was 516 G/L [range, 102-2619], and median hemoglobin level was 10.45 g/dL [range, 4-17]. BCR-ABL1 transcript type b3a2 was present in 53% of the assessable pts. Additional chromosomal abnormalities and variants were found in 5% of the pts. 9% and 40% of pts were considered at high-risk according to the ELTS score and to the Sokal score (for pts less than 45 years), respectively. The majority of pts (97%) were treated with imatinib as first line treatment. After 12 months of treatment, in pts with data available, rate of complete cytogenetic response (CCyR) was 74/109 (68%) pts with imatinib first line. Overall, rate of CCyR was 101/109 (93%) pts with imatinib first line [88/109 (81%) pts receiving imatinib only, 13/109 (12%) pts after switching to another TKI, 8/109 (7%) pts did not achieve CCyR]. Median time to achieve CCyR was 8.2 months [range, 2.7-106.7]. The cumulative rate of MMR at month 12 was 40/111 (36%) pts. Overall rate of MMR was 91/111 (82%) pts [72/111 (65%) pts with first line imatinib only; 19/111 (17%) pts with another TKI]. Median time to achieve first MMR was 14.5 months [range, 0.9-63.1]. Out of all patients on first line imatinib, 11/123 (9%) pts progressed: 3/11 pts progressed to AP and 8/11 pts to BP. Further progress to BP was observed in 2 of the 3 pts with AP. BP phenotype was myeloid in 5/10 pts, lymphoid in 3/10 pts and bilineage in 1/10 pts (no data for 1/10 pts). With imatinib first line, deaths occurred in 5/123 (4%) pts, among them 4/5 due to complications associated with hematopoietic stem cell transplantation. With a median follow up of 37 months [range, 0.9-231], the overall survival rate was 89.2% [83.6%; 94.7%] for pts treated with imatinib first line (N = 123). Conclusion: Our results are in line with publications describing adolescents and young adults (AYAs) as a risk population for a poorer treatment outcome: when comparing AYAs (15-29 yrs) to adults (&gt;30 yrs), CCyR and MMR were inferior in AYAs (cumulative CCyR 84% vs 93%, cumulative MMR 75% vs 86%, respectively) [Pemmaraju N et al., Haematologica 2012] and rates of progression to AP and BP were 8.7% in AYAs (16-29 yrs) but only 5.3% and 6.1% in adults (45-59 yrs and &gt;60 yrs, respectively) [Kalmanti L et al. , Ann Hematol. 2013]. Additional research is required in adolescents with CML to shed light on the cause(s) for the observed differences and to further improve the outcome. Disclosures No relevant conflicts of interest to declare.

Splenomegaly (SPML) in Polycythemia Vera (PV): Its Clinical Significance and Relation to Myelofibrosis and Survival

Introduction: SPML is considered a hallmark of PV, but its frequency, as reported in the literature, ranges from 20% to 75%. It has been assumed, without documentation, that SPML may be accompanied by symptoms and signs and may affect prognosis. Moreover, expensive radiographic tests have become mandatory in some recent phase 2 drug trials in PV (and ET) to carefully document spleen size on trial entry and its change, if any, during therapy. Because systematic studies have not been performed, we studied SPML in patients (pts) with PV at their initial diagnosis (DX) or at first presentation (PRES) at our institution, Weill Cornell Medicine (WCM), and determined its clinical significance. Methods: This single-center retrospective study was approved by the WCM institutional review board. A systematic literature search including PubMed, Embase, and Cochrane School relevant to the specific search questions was unrevealing. We used a research data repository based on an automated query system which had aggregated longitudinal clinical information pertaining to our PV patients for our analysis of spleen size (Abu-Zeinah et al. Leukemia 2021). Standardized PV diagnostic criteria were used for all pts (Silver et al. Blood 2013). As a tertiary referral center within a major metropolitan area, our PV population is composed of those diagnosed at WCM (DX) and those presenting to WCM some time after diagnosis (PRES). Degree of SPML was categorized into 3 subgroups: (1) &lt;1 cm if the spleen was not enlarged, (2) palpable 1-5 cm, or (3) more than 5.0 cm below the left costal margin of the abdomen in the medial clavicular line in the supine or left lateral decubitus position; it was also considered enlarged after splenic ultrasound scan (US) based upon the method and verified formula of Chow KU, et al. Radiology 2016. Spleen size was correlated with age, sex, race, and ELN risk score, and symptoms including pruritus, night sweats, anorexia, abdominal discomfort and pain. Progression to myelofibrosis (MF) with myeloid metaplasia was defined per ELN/IWG-MRT criteria. Spleen measurements after progression to MF were excluded. Peripheral blood smears were routinely examined to view RBC morphology and to exclude leukoerythroblastosis. MF-free survival (MFS) and overall survival (OS) were calculated using the Kaplan-Meier log rank test among the various spleen subgroups. Multivariable survival analysis (MVA) was performed using a Cox proportional hazards model. Results: From our 470 PV dataset, 351 pts had documented spleen size at DX (165) or PRES (186). The median age for all patients was 60 years (yr), for DX 54 yr, for PRES 62 yr. The median DX ages of SPML &lt;1 cm, 1-5 cm, and &gt;5 cm were 56, 50, and 54 yr respectively (p=0.011). The median time between first evaluation for PV and first visit at WCM (PRES) was 2 years (range 0-30). 49% were female and 13% were non-white (Figure 1a). There was no correlation between spleen size and ELN risk scores. The linkage between SPML and symptoms will be reported. Overall survival of the three groups was similar at 12 years (Figure 1b). SPML at presentation, however, was associated with increased risk of MF (1-5cm versus 0: HR 2.56, p=0.026; &gt;=5cm versus 0: HR 5.64, p&lt;0.001), independent of age or disease duration in MVA. Discussion & Conclusion: Patients who had SPML &gt; 5cm at presentation had a worse MFS than those with a lesser degree of SPML or no SPML (1-5 cm or &lt;1 cm). For determining SPML, clinical examination and calculated US length were equally satisfactory. In the absence of clinical SPML, radiographic tests appear unnecessary. However, for SPML &gt; 5cm, and unusual body types, more detailed radiographic studies may still be required. SPML was more common in younger patients, suggesting more aggressive disease and earlier progression to MF. ELN risk category did not correlate with SPML, suggesting an additional reason for its revision. Our PV patients with SPML &gt; 5cm at DX or PRES did not have a decreased OS at 12 years, but did have a reduced MF-free survival. These data support the WHO mandated requirement for marrow biopsy for diagnosis of PV, especially for patients with SPML but also as a baseline requirement to establish the presence of MF less than grade 2. Patients with SPML&gt; 5cm appear to be at high risk of MF progression and must be monitored closely for this event and treated appropriately. Figure 1 Figure 1. Disclosures Silver: Abbvie: Consultancy; PharamEssentia: Consultancy, Speakers Bureau. Scandura: CR&T (Foudation): Research Funding; European Leukemia net: Honoraria, Other: travel fees ; MPN-RF (Foundation): Research Funding; Constellation: Research Funding; Abbvie: Consultancy, Honoraria, Membership on an entity's Board of Directors or advisory committees, Research Funding. Abu-Zeinah: PharmaEssentia: Consultancy.

A new species of Epeorus (Caucasiron) (Ephemeroptera, Heptageniidae) from Azerbaijan and Iran

A new species, Epeorus (Caucasiron) hyrcanicussp. nov., is described based on larval morphology and molecular data (COI) containing sequences from all Caucasian Caucasiron species described to date. The species is distributed in the Hyrcanian forest of southeastern Azerbaijan and northwestern Iran. Based on our wide-range sampling, the new species is likely endemic to this area. The most pronounced larval morphological diagnostic characters are the coloration pattern of abdominal sterna (a pair of oblique stripes and stripe-like medio-lateral maculae) and terga (triangular medial maculae), poorly developed projection of the costal margin of gill plates III, presence of hair-like setae on the surface of abdominal terga, and relatively wide shape of gill plates VII (in natural position from ventral view). The diagnostic characters are compared to related species, and primary information to habitat is provided.

To Observe the Treatment Outcomes of Duodenal Injury in Penetrating and Blunt Trauma Patients

Aim: To study about the management of duodenal injury in two clinical aspects, blunt and penetrating injury, along with its complications. Study design: Observational case series. Place and duration of study: Accident & Emergency and General Surgery Departments at M. Islam Teaching Hospital, Gujranwala from March 2019 to March 2020. Methodology: One hundred patients presenting in Accident & Emergency and General Surgical Department of with penetrating chest trauma as diagnosed clinically were included. Routine investigations like complete blood tests, X-rays and special investigations i.e. ultrasound, CT scan were done only in cases where patients were stable. Each hemithorax was divided into medial and lateral hemithorax by an imaginary line drawn longitudinally from clavicle down to the costal margin passing through the nipple. All patients were observed for the type of treatment they were getting i.e. thoracotomy or tube thoracostomy. Patients who were initially treated with tube thoracostomy were cautiously observed for any developing indications for thoracotomy. If such indications arose thoracotomy would be arranged at the earliest possible. Results: A total of 100 patients, 85 (85%) were males and 15 (15%) were females. Male to female ratio was 5.66:1. The mean age of patient was 35.65±9.75 years. There were 38 (38%) had road traffic accidents, 10 (10%) were fall, 7 (7%) injured with fight, 41 (41%) victims of firearm injury and only 4 (4%) victims of stab. The mean blood pressure was 82.15±7.97mmHg. Eighty five (85%) patients were stay in the hospital for 2 weeks and while 15 (15%) were hospital stay >2 weeks. The mean values of hospital stay was 12.45±4.16 days. Conclusion: It is concluded that penetrating thoracic trauma is a major cause of morbidity and mortality. The overall complications rate for blunt trauma injuries after adequate treatment is 18% and mortality rate is 8%. Keywords: Blunt trauma, Thorocotomy, Tube thoracostomy, Pneumothorax

Effectivity of Acupressure in Low Back Pain

Low back pain is defined as located pain and discomfort below the costal margin and above the superior gluteal line, with related pain in the lower limb, and repeated incorrect posture of work activities that demand effort with excess of flexion, rotation, vibration on the chest and carrying weight, being chronic if its persists for three months or more. This research aimed to find out the efficacy of accupressure on Dachangshu, Shenshu, and Yaoyangquan points using pretest-posttest group design by comparing the mean decrease in pain threshold, RMDQ and Test Straight Leg between control using Dingchuan and Waihuaijian points and therapy group using Shenshu, Dachangshu, and Yaoyangquan acupoints twice a day with paired T test formula. The mean decreased in pain threshold VAS, RMDQ and Test Straight Leg obtained form therapy group toward 16 elderly above 45 years for 4 weeks showed that there is a significant result (p=0,000). In conclusion, accupressure therapy using Dachangshu, Shenshu and Yaoyangquan can be used to treat low back pain.

Non-sebaceous Lymphadenoma of the Thymus: a Case Report

Background: non-sebaceous lymphadenoma (NSL) is a rare benign tumor with a predominant lymphoid background within which is embedded solid or duct-like structures squamous epithelial nests, lacking of sebaceous differentiation. Non-sebaceous lymphadenoma most commonly arises within the salivary glands. However, Non-sebaceous lymphadenoma arises in the thymus have not been reported.Case presentation: A 53-year-old female patient, computed tomography (CT) scan of the chest for patient showed a nodular (19 ×13 × 16-mm) in the anterior mediastinum, and with mild homogeneous enhancement on contrast-enhanced CT. The patients underwent total thymectomy with removal of the anterior mediastinal nodule by thoracoscope via below the costal margin of the Xiphoid process. Microscopically, the tumor was composed of epithelial nests and prominent lymphoid stroma, with a capsule and clearly demarcated from the surrounding thymus tissue. The epithelial nests are arranged in solid nests or duct-like structures, lacking of sebaceous differentiation and cytological atypia. The pathological diagnosis was lymphadenoma, non-sebaceous type. There were no signs of recurrence 6 months after the surgery.Conclusions: There are very few reported cases of non-sebaceous lymphadenoma occurred in thymus in the medical literature. So accurate understanding of the histopathologic diagnosis of this rare tumor is important to avoid unnecessary overtreatment.

A new species of Erythrogonia Melichar, 1926 (Insecta: Hemiptera: Cicadellidae: Cicadellini) from the Mantiqueira mountain range, southeastern Brazil, associated with olive orchards

A new species of the diverse Neotropical sharpshooter genus Erythrogonia Melichar, 1926 is described and illustrated from the Mantiqueira mountain range, municipality of Maria da Fé, state of Minas Gerais, southeastern Brazil. The new taxon is associated with olive orchards and has been implicated as a vector of the bacterium Xylella fastidiosa Wells et al., 1987, which causes a disease known in Brazil as olive leaf scorch syndrome. Erythrogonia sinvali sp. nov. can be distinguished from the other 90 known species of the genus by the following combination of features: (1) crown black without conspicuous contrasting spots; (2) frons with large yellow median spot; (3) pronotum black with large yellow area covering most of disk; (4) forewing dark red, without contrasting spots or stripes, costal margin narrowly black, apical portion dark brown; (5) aedeagus elongate, curved dorsally, with pair of spiniform apical processes; (6) paraphyses greatly reduced; (7) basal portion of male anal tube with pair of spiniform curved processes; (8) posterior margin of female abdominal sternite VII broadly emarginate and with broad central lobe; (9) female sternite VIII with sclerotized areas, including a transverse bar located at bases of ovipositor valvulae I, followed by a pair of elongate sclerites and a posterior bilobed sclerite. Both males and females of the new species are described in detail. A putative group of five species within Erythrogonia is preliminarily proposed, including the new taxon, E. separata Melichar, 1926, E. dorsalis (Signoret, 1853), E. calva (Taschenberg, 1884), and E. melichari Schmidt, 1928. Among these species, the male terminalia of E. sinvali sp. nov. are more similar to those of E. separata.

Ultrasound-Guided Lateral Transversus Abdominis Plane (TAP) Block in Rabbits: A Cadaveric Study

The aims of the study were to describe the ultrasonographic-guided lateral TAP block in rabbit cadavers and evaluate the spread of a lidocaine/methylene blue solution through a single fascial infiltration. The US-guided block and anatomical dissections were performed in 17 New Zealand rabbit cadavers. The probe was placed perpendicular to the column, one centimetre ventrally to the transverse processes, halfway between the iliac crest and the costal margin. External oblique (EO), internal oblique (IO), and transversus abdominis (TA) muscles were visualised, and 1 mL/kg of lidocaine 2% plus methylene blue 1% was injected. After dissection, the branches of spinal nerves stained were measured. Moreover, the percentage of length and height of the area marked were calculated. A good visualisation of the TAP was obtained in all 34 hemiabdomens. T11 nerve eminence was successfully stained in 52% of cases. T12, L1, and L2 were stained in 75%, 95%, and 100% of cases, respectively. L3 and L4 were stained in 60% and 40% of cases, respectively. The lateral TAP block with a single point of injection can be easily performed in rabbits, but it is not sufficient to cover the nerve eminences of the cranial abdomen. The two-point TAP block (lateral and subcostal) could represent a better option, particularly when large surgical incisions are required.

LUMBOCOSTOVERTEBRAL SYNDROME (LCVS) - A RARE ENTITY

Full term female neonate, weighing 2.7 kg, was born to 37- year-old mother with an uneventful antenatal course. On examination, child was active and anthropometric parameters were normal. Head to toe examination revealed a globular swelling below left costal margin lateral to dorsolumbar spine measuring approximately 6 cm X 6 cm. It was soft, nontender, reducible and used to become prominent on crying. Rest of general and systemic examination was th th normal. X-ray of chest and spine showed dysplastic 9 , 10 , th th th 11 , 12 thoracic vertebrae (Fig 1 red arrow) with absent left 9 , th, th th 10 11 , 12 ribs (Fig 1 blue arrow) and left lumbar hernia (Fig 1 yellow arrow). Ultrasonography abdomen showed a hernial defect in left lumbar region measuring 5 cm X 5 cm containing small bowel loops. Ultrasonography spine showed spina bida occulta. Echocardiography was normal. Lumbar hernia was repaired by pediatric surgeons, operative ndings showed 5 cm X 4 cm hernial sac containing small bowel loops. Child had uneventful post-operative course and discharged successfully. Baby was advised regular follow up.