-
BMC Health Services Research May 2024Disease registries are comprehensive databases that record detailed information on patients diagnosed with specific conditions, providing valuable insights into their...
BACKGROUND
Disease registries are comprehensive databases that record detailed information on patients diagnosed with specific conditions, providing valuable insights into their diagnosis, treatment, and outcomes. This study aims to describe the pilot phase of the national pediatric Immune Thrombocytopenia(ITP) registry (NPITP) in Iran, serving as the inaugural interpretive report.
METHODS
This patient-centered software system was implemented as a national program across multiple pediatric centers in Iran. Several focus groups were conducted to establish a minimum data set (MDS) comprising six main classes, 14 sub-classes, and 187 data elements. Following expert consensus on the final data set, a web-based software tool was developed by the dedicated IT team, accessible online and offline via https://disreg.sbmu.ac.ir/q/ITP.html . The registry included children aged between two months and 18 years with a platelet count below 100 × 10/L, based on predefined inclusion criteria.
RESULTS
Within a four-month period, a total of 60 ITP patients were registered, including 41 (68.3%) newly diagnosed cases, 68 (13.6%) persistent cases, and 14 (23.3%) with chronic ITP. The mean age of the registered patients was 55.93 ± 9.72 months. The most frequently observed bleeding symptoms were petechiae (68.3%), purpura (51.6%), and ecchymosis (13.3%). Among the newly diagnosed patients, 20 (33.3%) received intravenous immunoglobulin (IVIG), 17 (28.3%) were treated with prednisolone, and 17 (28.3%) received combined IVIG and steroid therapy. Of all patients, 40 (66.7%) demonstrated a complete response to treatment, while 16 (26.7%) exhibited a partial response. Four patients (6.7%) remained unresponsive to therapy. Treatment-related complications, such as Cushing's syndrome, edema, weight gain, hirsutism, and mood disorders, were reported in 10 patients (16.6%). However, the majority of patients (81.7%) did not experience therapy-related complications.
CONCLUSION
The pilot phase of the NPITP registry successfully implemented a web-based software tool for data collection, aiming to enhance the quality of care, facilitate clinical research, and support health service planning in the future.
Topics: Humans; Registries; Child; Iran; Purpura, Thrombocytopenic, Idiopathic; Child, Preschool; Adolescent; Male; Female; Infant; Pilot Projects
PubMed: 38807135
DOI: 10.1186/s12913-024-11102-z -
Aesthetic Plastic Surgery May 2024The main causes of ecchymosis and edema are osteotomy (bone manipulation), dissection of subcutaneous tissue, and skin manipulation in the rhinoplasty procedure. Eyelid...
The main causes of ecchymosis and edema are osteotomy (bone manipulation), dissection of subcutaneous tissue, and skin manipulation in the rhinoplasty procedure. Eyelid edema following surgery can potentially affect visual acuity, particularly during the initial twenty-four hours after the procedure. These may also delay the patient's return to their normal social life therefore hampering their quality of life. Various surgical and medical methods have been reported to address these issues. This study aimed to compare the effects of using cold saline (0-4 °C) versus room temperature saline (20-25 °C) irrigation throughout the surgery on postoperative edema, ecchymosis, and pain. Fifty patients who underwent open-approach primary rhinoplasty between August 2022 and August 2023 at a tertiary academic center were included. Fifty patients were randomly divided into two groups depending on using cold saline (0-4 °C) (group 1) or room temperature saline (20-25 °C) (group 2) during surgical site irrigation. Patients were assessed for pain, edema, and bruising using a VAS (Visual Analog Scale) on the second and seventh postoperative days. Visual analog score (VAS) was used for subjective outcome analyses. Each patient scored the severity of their periorbital ecchymosis on day two and seven. Periorbital ecchymosis was also evaluated on the second and seventh postoperative days using the SPREE (Surgeon Periorbital Rating of Edema and Ecchymosis) scale. On the second postoperative day, the VAS pain score in group 1, where cold water was used, was found to be statistically and significantly different from the control group (group 2) (p < 0.05). However, there was no statistically significant difference between both groups when comparing the VAS pain scores on the seventh postoperative day. Regarding the VAS ecchymosis score on the seventh postoperative day, there was a statistically significant difference favoring group 1 (p < 0.05). The SPREE scale data also indicated that group 1 had significantly lower scores on the seventh day (p < 0.05). While the SPREE scores on the second day were lower in group 1 than in group 2, this difference did not reach statistical significance (p = 0.061). The findings from our study show that cold saline irrigation may contribute to intraoperative hemostasis by inducing local vasoconstriction. We observed that intraoperative bleeding decreased with the use of cold saline. This approach has the potential to improve patient satisfaction and overall quality of life by reducing postoperative ecchymosis without significantly increasing the cost of the surgical procedure.Level of Evidence III This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .
PubMed: 38806826
DOI: 10.1007/s00266-024-04105-y -
BMJ Case Reports May 2024
Topics: Female; Humans; Anticoagulants; Ecchymosis; Heparin; Thrombocytopenia; Middle Aged
PubMed: 38802255
DOI: 10.1136/bcr-2024-260309 -
Acta Chirurgiae Orthopaedicae Et... 2024Transphyseal fractures of the distal humerus are usually seen in children younger than 3 years of age and are considered as Salter-Harris Type I epiphysiolysis. Neonatal...
Transphyseal fractures of the distal humerus are usually seen in children younger than 3 years of age and are considered as Salter-Harris Type I epiphysiolysis. Neonatal transphyseal distal humerus injuries are extremely rare. It usually occurs due to trauma during difficult labour but can also be seen after child abuse. Since the distal humerus is composed of cartilaginous tissue in newborns, it is difficult to make a diagnosis with direct radiography. Patients are often diagnosed with elbow dislocation. However, elbow dislocation is almost never seen under the age of 3 years. Transphyseal fractures can be seen as a result of manoeuvres performed to deliver the baby during difficult normal delivery. Transphyseal humeral injuries can also be seen after caesarean section, child abuse and falling on the hyperextended arm. Clinical symptoms include pain, swelling, ecchymosis and crepitation at the elbow. Pseudoparalysis is present due to pain. In children with a history of difficult birth or trauma, evaluation with direct radiography should be performed initially. Radiocapitellar line is distorted on radiographs and the elbow joint appears subluxated. The treatment algorithm for transfusional humeral fractures in neonates is varied. It should be remembered that patients in this age group have a tremendous healing capacity. In conservative treatment, 2-4 weeks of follow-up with a long-arm splint after reduction is sufficient. In addition, closed reduction-internal fixation or open reduction-internal fixation can be applied according to the amount of displacement of the fracture. Cubitus varus, osteonecrosis, growth disturbance, decreased range of motion, compartment syndrome, neurovascular injury and infection are the main complications seen after transfusional humeral fractures.
Topics: Humans; Humeral Fractures; Infant, Newborn; Radiography; Elbow Injuries; Elbow Joint; Female; Male; Birth Injuries; Humeral Fractures, Distal
PubMed: 38801668
DOI: 10.55095/ACHOT2024/015 -
Aesthetic Plastic Surgery May 2024Eyelid surgery is one of the top five aesthetic procedures. It is performed to improve both appearance and function, but intraoperative bleeding leads to adverse events...
BACKGROUND
Eyelid surgery is one of the top five aesthetic procedures. It is performed to improve both appearance and function, but intraoperative bleeding leads to adverse events which perturb patients. The objective of this study was to demonstrate the efficacy of TXA combined with epinephrine in decreasing intraoperative blood loss and postoperative inflammation.
METHODS
This prospective randomized control trial was performed on the 30 eyelids of 15 patients who underwent upper blepharoplasty. One of each patient's eyes was randomly assigned to the TXA group, and the other eye was in the control group. Eyes in the TXA group were given 2% lidocaine with epinephrine (1:100000) mixed with TXA (50 mg/ml) in 1:1 mixture subcutaneously as a local anesthetic. The eyes in the control group received 2% lidocaine with epinephrine (1:100000) diluted with normal saline in 1:1 mixture. Intraoperative blood loss and postoperative swelling were compared between the two groups.
RESULTS
Intraoperative blood loss was significantly higher in the TXA group [4.86 (1.83) ml] than it was in the control group [2.53 (1.49) ml] (p < 0.001). There was no statistically significant difference between the two groups in operative time (p = 0.645), pain score (p = 0.498), lid crease (p = 0.548), or MRD1 (p = 0.626). On postoperative day 7, there was no difference in lid crease (p = 0.879), MRD1 (p = 0.463), pain score (p = 0.934), or ecchymosis (p = 0.976) between two groups.
CONCLUSIONS
TXA in lidocaine with epinephrine was found to increase intraoperative bleeding compared to lidocaine with epinephrine alone, but there was no difference in postoperative swelling or ecchymosis. TXA combined with lidocaine and epinephrine injected subcutaneously should be avoided until additional relevant data are obtained. Further drug interaction study is needed.
LEVEL OF EVIDENCE II
This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .
PubMed: 38789809
DOI: 10.1007/s00266-024-04112-z -
Medicine May 2024Acquired hemophilia-A (AHA) is a rare but potentially life-threatening impaired coagulation disorder characterized by the development of autoantibodies against clotting...
INTRODUCTION
Acquired hemophilia-A (AHA) is a rare but potentially life-threatening impaired coagulation disorder characterized by the development of autoantibodies against clotting factor VIII. Only a few case reports have been experienced with influenza vaccine-triggered AHA. Here, we report a case of severe hemorrhagic disorder due to AHA following influenza vaccine, which was successfully treated.
PATIENT CONCERNS
The patient presented to the emergency department because of several severe, progressively worsening bruises after receiving the influenza vaccination. Consequently, the patient required intubation due to nasal-oral bleeding, which compromised the airway, and retroperitoneal hemorrhage with shock also developed.
DIAGNOSIS
AHA was confirmed through a coagulation factor assay, including coagulation activity and antibody testing, which is possibly triggered by influenza vaccination.
INTERVENTION
Low-dose cyclophosphamide and hydrocortisone were prescribed until activated partial thromboplastin time showed normal levels. Coagulation factor VIIa was administered, and aggressive blood transfusion was carried out concurrently to address the blood loss.
OUTCOMES
The upper airway bleeding subsided and bleeding tendencies had been corrected to normal. The patient was smoothly weaned from the ventilator and recovered from critical illness. She was then discharged on the 19th day.
LESSONS
The activated partial thromboplastin time mixing test can be performed immediately to establish the initial differential diagnosis and treatment plan for severe coagulopathy. AHA may be triggered by vaccination, with the hypothesis of activation of autoantibodies and molecular mimicry; this mechanism should be further studied.
Topics: Humans; Hemophilia A; Female; Ecchymosis; Hemorrhage; Retroperitoneal Space; Influenza Vaccines; Middle Aged
PubMed: 38787989
DOI: 10.1097/MD.0000000000038300 -
BMJ Case Reports May 2024A female patient in her middle childhood presented to the paediatric emergency room (ER) after a bicycle accident with an abdominal impact on the bicycle handlebar. On...
A female patient in her middle childhood presented to the paediatric emergency room (ER) after a bicycle accident with an abdominal impact on the bicycle handlebar. On physical examination, a painful ecchymosis on the upper left quadrant was the only abnormal finding. Abdominal ultrasound showed no intra-abdominal lesions, and the patient was discharged home after 24 hours under monitoring. Nine days after the accident, she returned to the ER due to the emergence of an abdominal mass around the area of impact. Abdominal examination detected a tender non-fluctuating mass on the epigastric and left hypochondrium, and abdominal ultrasound revealed a muscle and aponeurosis disruption of the rectus muscle, with fat herniation and cytosteatonecrosis. A conservative approach was chosen, with ambulatory follow-up. One month after the accident, the patient was asymptomatic, no abdominal mass was palpable, and an abdominal CT showed a reduction of the muscle disruption and hernial content.
Topics: Humans; Bicycling; Female; Hernia, Abdominal; Child; Abdominal Injuries; Tomography, X-Ray Computed; Ultrasonography; Abdominal Wall
PubMed: 38782431
DOI: 10.1136/bcr-2024-260806 -
Cureus Apr 2024Background During the COVID-19 pandemic, Americans transitioned away from their normal routines, drove in motor vehicles less, and reduced their physical activity,...
Background During the COVID-19 pandemic, Americans transitioned away from their normal routines, drove in motor vehicles less, and reduced their physical activity, ultimately influencing the incidence and nature of orthopedic injuries that were operatively managed. The purpose of this study was to evaluate the effect of the COVID-19 pandemic lockdown and subsequent deconditioning on the mechanism of injury and severity of Lisfranc injury. Methods This retrospective study included patients with a traumatic Lisfranc injury who were surgically treated by a foot and ankle fellowship-trained orthopedic surgeon between 2015 and 2021. Electronic health records were queried for patient demographics, mechanism of injury, physical exam findings, and pain scores. Preoperative radiographs were reviewed to grade Lisfranc injuries using the previously described Nunley-Vertullo classification system. Descriptive and univariate statistics were performed to compare 15 patients in the pre-COVID-19 cohort and 15 patients in the post-COVID-19 cohort. Results In the pre-COVID-19 cohort, 80% (n=12/15) of the patients were female, the mean age was 46±15 years, the mean BMI was 29.7±7 kg/m, and the mean follow-up period was 18.1±12 months. In the post-COVID-19 cohort, 53% (n=8/15) of the patients were female, the mean age was 48.5±17 years, the mean BMI was 31.4±7 kg/m, and the mean follow-up period was 9.5±4 months. Significantly higher proportions of plantar ecchymosis (n=8/15, 53%), neuropathic pain (n=7/15, 47%), and swelling (n=12/15, 80%) were present in the post-COVID-19 cohort. A low-energy mechanism of injury was sustained by 73% (n=11/15) of the pre-COVID-19 cohort and 80% (n=12/15) of the post-COVID-19 cohort. Lisfranc injuries for the pre-COVID-19 cohort and the post-COVID-19 cohort demonstrated the following classifications: Grade 1 (33%, n=5/15 vs. 40%, n=6/15), Grade 2 (60%, n=9/15 vs. 53%, n=8/15), and Grade 3 (7%, n=1/15 vs. 7%, n=1/15). Conclusion Although a higher proportion of plantar ecchymosis, neuropathic pain, and swelling was observed, there was no association between a low mechanism of injury and a higher grade of Lisfranc injury following the COVID-19 pandemic.
PubMed: 38770506
DOI: 10.7759/cureus.58644 -
Journal of Clinical Immunology May 2024Chronic Granulomatous Disease (CGD) is a rare immunodeficiency disorder characterized by impaired phagocytic function, leading to recurrent infections and granuloma...
BACKGROUND
Chronic Granulomatous Disease (CGD) is a rare immunodeficiency disorder characterized by impaired phagocytic function, leading to recurrent infections and granuloma formation. Genetic mutations in NADPH oxidase complex components, such as CYBB, NCF1, NCF2, and CYBA genes, contribute to the pathogenesis. This case report explores the possible ocular and hematologic complications associated with CGD.
CASE PRESENTATION
A 6-year-old girl with a history of vitrectomy, membranotomy, and laser therapy due to congenital blindness (diagnosed with chorioretinopathy) was referred to the hospital with generalized ecchymosis and thrombocytopenia. Diagnostic workup initially suggested chronic immune thrombocytopenic purpura (ITP). Subsequent admissions revealed necrotic wounds, urinary tract infections, and recurrent thrombocytopenia. Suspecting immunodeficiency, tests for CGD, Nitroblue tetrazolium (NBT) and dihydrorhodamine (DHR) were performed. She had a low DHR (6.7), and her NBT test was negative (0.0%). Her whole exome sequencing results confirmed autosomal recessive CGD with a homozygous NCF1 mutation.
CONCLUSION
This case underscores the diverse clinical manifestations of CGD, including recurrent thrombocytopenia and possible early-onset ocular involvement. The diagnostic challenges highlight the importance of a multidisciplinary approach involving hematologists, immunologists, and ophthalmologists for accurate diagnosis and management. The rare coexistence of ITP in CGD emphasizes the intricate link between immunodeficiency and autoimmunity, requiring tailored therapeutic strategies.
Topics: Humans; Female; Granulomatous Disease, Chronic; Child; Purpura, Thrombocytopenic, Idiopathic; NADPH Oxidases; Mutation; Exome Sequencing
PubMed: 38760640
DOI: 10.1007/s10875-024-01731-8 -
Clinical Case Reports May 2024In a patient with de novo AML, co-existing isoform and rearrangement is accompanied by a very poor prognosis including limited response to treatment and no molecular...
KEY CLINICAL MESSAGE
In a patient with de novo AML, co-existing isoform and rearrangement is accompanied by a very poor prognosis including limited response to treatment and no molecular remission. It is essential to develop a consensus on the therapeutic modalities different from the current regimen.
ABSTRACT
Acquisition of fusion as a primary or secondary event and fusion independently is reported in de novo and therapy-related MDS/AML, albeit with low frequency (<0.5%). Coexistence of and translocation is known to cause leukemogenesis in animal models and progression towards blast crisis CML but not AML. Here we report a unique case of pediatric AML with concomitant BCR::ABL1 and RUNX1::MECOM fusion.Routine diagnostic work-up included WBC manual differential, immunophenotype, morphology, qPCR, FISH, and NGS-based CNV analyses. The patient presented with history of fever, dizziness, fatigue, gingival bleeding, and epistaxis associated with ecchymosis in right hand and heavy, prolonged menstrual period. At presentation, her hemoglobin was 5.3 g/dL, WBC 52.1(10*9/L), PLT 10(10*9/L), ESR 5 mm/h and LDH 2658 U/L. Bone marrow was hypercellular with 71% blasts, and flow cytometry showed myeloid markers including CD11c, CD33, CD34, and CD45 among others indicating AML with monocytic differentiation. FISH analyses showed variant t(9;22) (q34.1;q11.1), one additional copy each of chromosome 8 and gene, while NGS-based CNV analyses revealed a terminal and proximal pathogenic gain within 9q34.12q34.3 and 22q11.1q11.23, respectively, and gain of entire chromosome 8 and 12 in mosaic state. qPCR confirmed the presence of and also revealed fusion. Patient received ADE (cytarabine, daunorubicin, and etoposide) induction regimen but required multiple ICU admissions due to sepsis, cardiac shock, acute myocarditis, and thyroiditis. Coexisting and fusion is suggestive of poor prognosis, and a need for consensus on the treatment modalities other than the current regimen is warranted.
PubMed: 38751957
DOI: 10.1002/ccr3.8917