bluuebunny/crossref_metadata_2025_split huggingface dataset:
Clinical significance of serum CD155 levels in acute lymphoblastic leukemia patients
Authors: Ibrahim Ibrahim Nahed Moawad , Osman Mostafa Mariam Ahmed , Azzazi Mohamed Osman , Abd-Allah Hegab Hany Mohamed , Mohamed Said Rasha Magdy , Abdelfatah Rana G 
Published: February 2024

Abstract

Background
Acute lymphoblastic leukemia (ALL) is a hematologic malignancy characterized by poor outcomes in adults. Traditional diagnostic and prognostic markers take into account clinical data but also rely heavily on expensive and invasive tests such as bone marrow studies with cytogenetics. CD155 is an adhesion molecule that also modulates immune response in malignancy. Previous studies have shown that serum CD155 (sCD155) levels are higher in patients with malignancies, and have demonstrated a correlation between higher levels of sCD155 and poor outcomes. This study investigated the diagnostic and prognostic value of sCD155 in adult patients with acute lymphoblastic leukemia.

Methodology
32 adult patients with acute lymphoblastic leukemia and 15 control patients were enrolled in this study. Clinical and outcome data were collected from patients and a blood sample from patients and controls was collected with ELISA testing for sCD155 levels.

Results
sCD155 was found to be significantly higher in patients than controls (P<0.05) and was significantly associated with bone marrow aspirate blast count. It was not associated with other clinical parameters including Sex, age, initial CBC parameter counts, presence of lymphadenopathy, presence of CNS infiltration, presence of Philadelphia chromosome, type of ALL, or risk stratification of ALL besides being associated with hepatosplenomegaly. ROC curve showed high sensitivity and specificity of sCD155 and Kaplan Meir curve showed a trend towards worse outcomes in patients with high sCD155 but it was not statistically significant.

Conclusion
sCD155 remains a promising marker for diagnosis of ALL but further studies are needed to confirm prognostic value.
BAFF as a predictive marker for treatment response in immune thrombocytopenia patients
Authors: Hamza Mona Ashraf Al-dosoky , Elbedewy Tamer Abd Elhamid , Adel El-Kholy Rasha , Sheira Gehan Mohamed 
Published: February 2024

Abstract

Background
Immune thrombocytopenic purpura (ITP) is a hematological autoimmune bleeding disorder that characterized by isolated thrombocytopenia (platelet count <100 × 109/L) with unclear pathophysiology due to contribution of many cytokines, one of them is B-cell activating factor (BAFF) which affect both B-cell and T-cell activation and survival.

Objectives
This work aimed to study serum BAFF levels in the Egyptian ITP patients, and the relation between its level and response to steroid therapy, as well its value as a predictive marker for steroid response in ITP patients.

Patients and methods
90 subjects were recruited, and divided into 45 patients with primary ITP, and 45 healthy subjects. Serum BAFF was estimated using the ELISA technique.

Results
BAFF level was significantly higher in the ITP patients than in the control group. Serum BAFF level was significantly higher in newly diagnosed ITP cases and steroids-resistant ITP cases. BAFF was negatively correlated with platelet count, while MPV, PDW, and LDH were positively correlated, BAFF level at a cut-off 201.01 pg/ml is a good predictive for steroid resistance in ITP cases.

Conclusion
BAFF could be a good inexpensive helper to determine the response to steroid treatment in ITP cases.
Clinical relevance of cyclooxygenase 2 and vascular endothelial growth factor-A expression in classical Hodgkin lymphoma
Authors: Elrefaey Fatma A , Khorshed Amira , Aboulenin Khaled M , Eissa Lobna A , Ghareeb Mohamed 
Published: February 2024

Abstract

Background
Classical Hodgkin lymphoma (cHL) is a clonal lymphoid neoplasm derived from B cells. Cyclooxygenase 2 (COX2) and vascular endothelial growth factor-A (VEGF-A) play major roles in angiogenesis and impact cHL prognosis.

Aim
To measure COX2 and VEGF-A expression in cHL patients and assess their potential association with other laboratory and clinical parameters.

Patients and methods
Seventy-six cHL bone marrow (BM) biopsy specimens were histopathologically examined and immunohistochemically stained for COX2 and VEGF-A expression. Correlations between COX2 and VEGF-A expression and clinicopathologic factors were evaluated.

Results
COX2 and VEGF-A were expressed in 67/76 (88.2%) and 48/76 (63.2%) of BM specimens, respectively. VEGF-A was associated with advanced cHL stage (P=0.044) and BM infiltration confirmed by CD30 positivity (P=0.023). A significant association was found between VEGF-A positivity and mediastinal lymphadenopathy (P=0.049), inguinal lymphadenopathy (P=0.046), and pulmonary nodules (P=0.048). COX2 positivity was significantly associated with cervical lymphadenopathy (P=0.011). A positive association was found between expression of both markers (COX2 and VEGF-A) (P=0.001). Coexpression of COX2 and VEGF-A was associated with disease staging (P=0.016), mediastinal lymphadenopathy (P=0.019), and inguinal lymphadenopathy (P=0.044).

Conclusion
COX2 and VEGF-A, as major players in angiogenesis, are associated with tumor progression in cHL. These findings support targeting both markers as the potential therapeutic approach in cHL.
Vitamin B12 deficiency without anemia in Saudi Arabia: a single-center experience
Authors: AlShomar Ahmad 
Published: February 2024

Abstract

Objectives
The study aimed to estimate the prevalence of vitamin B12 deficiency in adult Saudi patients and anemia in those patients. Furthermore, the mean level of vitamin B12 in anemic and nonanemic participants was estimated.

Methodology
This retrospective study involved the evaluation of medical records from adult Saudi Arabian participants who underwent vitamin B12 level testing for nonspecific symptoms during 1 years (2022–2023) at a single center in the Al Qassim region of Saudi Arabia. The data for vitamin B12 level and anemia status among B12-deficient patients were estimated to calculate the prevalence of vitamin B12 deficiency and the prevalence of anemia in those patients.

Results
The prevalence of vitamin B12 deficiency was 11.46% among 8365 participants. The mean age among all vitamin B12-deficient patients was significantly lower compared with those with normal vitamin B12 levels. Female patients had a lower mean vitamin B12 level than male patients. The prevalence of anemia in the vitamin B12-deficient group was 23.46% among the total participants, with female patients having a significantly higher prevalence of anemia than males. The mean value for mean corpuscular volume (MCV) in anemic vitamin B12-deficient patients was normal, and the prevalence of macrocytosis was low (1.3%). The effect of gender on vitamin B12 deficiency and anemia status was significant.

Conclusion
The present study has provided additional evidence regarding the lack of a causal correlation between vitamin B12 deficiency and anemia. Moreover, the prevalence of vitamin B12 deficiency and anemia was higher in female patients than in males.
Double Aneuploidy (Trisomy 21 and XXX) in a Child with Down Syndrome Phenotype: A Case Report with Review of Literature
Authors: Mehta Parth , Chowdhary Samridhi , Rajeshwari K. , Kumar Deepak 
Published: February 2024

Abstract

Background:
Down syndrome is the most common and extensively studied disorder arising out of human chromosomal aberration. Several aneuploidies are associated with Down syndrome, but double trisomy (48 XXX + 21) is rarely described.

Clinical Description:
A 3-year-old girl presented with respiratory distress and a chronic history of feeding fatigue and failure to thrive. She had phenotypic features suggestive of Down syndrome and clinical signs of acyanotic heart disease in congestive cardiac failure.

Management and Outcome:
Echocardiography revealed ventricular septal defect and atrial septal defect. Karyotyping showed double trisomy (trisomy 21 and XXX). Trisomy X was an incidental finding on karyotyping with no clinical features suggestive of XXX syndrome specifically at this age. The patient was managed conservatively with decongestive therapy for congestive heart failure and oral sildenafil for pulmonary hypertension. Additional evaluations were done to identify associated anomalies, and the parents were counseled.

Conclusion:
Down and XXX syndromes share many clinical signs and symptoms, making karyotyping the only way to make an early diagnosis. A child having a phenotype characteristic of Down syndrome may have an unsuspected double trisomy of X chromosome along with 21 trisomy, which can only be identified by karyotyping.
Alopecia, Dermatitis and Seizures since Infancy: A Case of Biotinidase Deficiency
Authors: Ramesh Reshma , Rajendran Sakthivel 
Published: February 2024

Abstract

Background:
Biotinidase deficiency is a rare autosomal recessive metabolic disorder, having a wide spectrum of neurological, cutaneous, and immune-dysfunction-related clinical manifestations, which may be overlooked as common pediatric ailments.

Clinical Description:
A 2-year–7-month-old girl child, second born to third-degree consanguineous parents, presented with fast breathing and altered sensorium for a day, with a history of seizures and skin lesions since early infancy, developmental delay, and ataxia.

Management and Outcome:
Investigations revealed a normal septic screen, with metabolic acidosis which was unresponsive to therapy. After stabilizing the child with respiratory and vasopressor support, investigations for immunodeficiency were sent. Tandem mass spectrometry revealed increased C5OH levels, suggestive of holocarboxylase deficiency. Specific enzyme assay showed biotinidase activity deficiency. The child was initially empirically treated with metabolic cocktail comprising carnitine, biotin, thiamine, C0-enzyme Q10, and riboflavin, followed by specific therapy with biotin. The child showed dramatic clinical improvement and normalization of blood gas. Treatment has been advised lifelong with biotin supplementation.

Conclusion:
Careful observation by a pediatrician can identify the typical combination of clinical features in a child, suggestive of biotinidase deficiency. Evaluation and management of the condition are simple and rewarding.
Isolated Renal Manifestations as Presenting Features of Acute Leukemia: A Case Report with Review of Literature
Authors: Rajeswari Raja , Srinivasan Arathi , Vasanthi T. , Sairam V. K. 
Published: February 2024

Abstract

Background:
Although renal complications are well-known in hematological malignancies, they occur mostly after initiation of steroids and chemotherapy. We describe an unusual scenario of isolated renal complications as a presenting feature in children with acute lymphoblastic leukemia (ALL).

Clinical Description:
A 2-year-old girl presented with abdominal distension due to bilateral palpable renal masses associated with stage 2 hypertension. Investigations revealed ALL and impaired renal functions with enlarged kidneys on ultrasound, with no involvement of any other organ or lymph node.

Management and Outcome:
She required three antihypertensives for normalizing her blood pressure (BP). After initiation of chemotherapy, BP improved, kidney sizes reduced, and acute kidney injury resolved.

Conclusion:
Renal functions, BP and imaging for kidney sizes should not be missed in the initial work-up in children with ALL.
Acute Compartment Syndrome in a Neonate: May Be the First Manifestation of Hemophilia A
Authors: Tonge Rucha , Lalwani Shivkumar , Vageriya Natasha , Shrivastava Bijal 
Published: February 2024

Abstract

Background:
Acute compartment syndrome (ACS) most commonly results from traumatic or ischemic injuries, though rarely it may result from an underlying bleeding disorder. Reports of ACS in hemophilia are present; however, neonatal presentation secondary to hemophilia A is extremely rare.

Clinical Description:
A 5-day-old neonate presented with increasing swelling and hematoma over the dorsum of both hands following venipuncture. There was no other bleeding manifestations. Antenatal history and natal history were uneventful, and the baby had received a birth dose of Vitamin K. On examination, the hematoma was larger on the right hand than the left, extending from the proximal interphalangeal joints of all the digits to the wrist proximally, with tension on palpation and increased capillary refilling time, associated with bluish discoloration of hands and fingers with claw hand appearance. Oxygen saturation on pulse oximeter was 80%–85%.

Management and Outcome:
The baby had a hemoglobin of 16.3 g/dL, white blood cell count of 5300/mm3, platelet count of 2.33 lakhs/mm3, and elevated activated partial thromboplastin time >120 s with a normal prothrombin time of 12 s. Factor assay confirmed severe factor VIII deficiency (<1%). Ultrasonography Doppler of the hand confirmed compartment syndrome. Cryoprecipitate was given perioperatively, and fasciotomy was done. Perfusion of the hand improved, and oxygen saturation normalized. Healing was accomplished without any closure, with good cosmesis and complete functional recovery by 14 days.

Conclusion:
ACS may be an unusual primary presentation of hemophilia as early as in the neonatal period. Timely identification of ACS and early suspicion of an underlying bleeding disorder can help in undertaking appropriate evaluation and management strategies and preventing catastrophic consequences.
Hydranencephaly Associated with Bilateral Megalocornea and Bilateral Cleft Lip in an Infant of Mother with Gestational Diabetes Mellitus
Authors: Dehneh Younes , Khoulali Mohammed , Oulali Noureddine , Moufid Faycel 
Published: February 2024

Abstract

Background:
Hydranencephaly is a rare congenital disorder of the central nervous system, in which the cerebrum is replaced by a membranous sac of cerebrospinal fluid.

Clinical Description:
A 2-month-old baby girl presented with abnormal body movements. She was born to a mother with gestational diabetes and had been diagnosed with hydrocephalus antenatally. The baby had features of facial dysmorphism such as bilateral megalocornea, bilateral cleft lip, and cleft palate. A magnetic resonance imaging (MRI) brain revealed the absence of cerebral hemispheres with remnants of parenchyma in the orbitofrontal area that had been replaced by cerebrospinal fluid, diagnostic of hydranencephaly. Karyotyping and echocardiography findings were normal.

Management and Outcome:
The prognosis was explained to parents, who opted for no therapeutic intervention. The thus patient kept on supportive care and she succumbed at the age of 6 months.

Conclusion:
Our case creates awareness regarding a possible association between gestational diabetes and hydranencephaly. Furthermore, the hydranencephaly in this case was unique due to its association with bilateral megalocornea and cleft lip. The case reiterates the fact that a hydrocephalus diagnosed antenatally may actually be a hydranencephaly, which is confirmed in MRI brain imaging postnatally.
Spontaneously Regressing Retropharyngeal Neuroblastoma – A Rare Cause of Upper Airway Obstruction in a Neonate: A Case Report with Review of Literature
Authors: Piridi Raga Deepthi , Manugu Navya Sree , Kamuni Nikhitha , Reddy Sai Rama 
Published: February 2024

Abstract

Background:
Retropharyngeal masses may rarely cause persistent respiratory distress, stridor, and feeding difficulties in a neonate. We report a rare case of a primary cervical neuroblastoma presenting as a retropharyngeal mass, which underwent spontaneous regression.

Clinical Description:
A 29-day-old male baby was referred for recurrent vomiting since day 10 of life followed by severe difficulty in breathing since day 14 of life. At admission, the baby had stridor, cyanosis with respiratory failure, without any facial dysmorphism or cardiovascular involvement.

Management and Outcome:
After initial stabilization, investigations showed pneumonia in chest X-ray, with no evidence of sepsis. On intubation, a retropharyngeal bulge was noticed which was later confirmed by direct laryngoscopy. Later, computed tomography delineated the soft-tissue mass extending from C2–C5. Endoscopy with marsupialization of the tumor was done, and on histopathological examination, it was diagnosed as a small round blue cell tumor, suggestive of neuroblastoma, along with positive immunohistochemistry. According to the International Neuroblastoma Staging System, the tumor belonged to Stage I. As the baby had started improving clinically, further management by a multidisciplinary team was collectively decided to continue conservative observation. On follow-up, there was spontaneous regression in size of tumor with no recurrence of symptoms till the last follow-up at 11 months.

Conclusion:
Neuroblastoma, the most common solid tumor malignancy in children, may rarely have a primary cervical presentation, causing breathing and feeding difficulties. Depending on the course of symptoms, a strategy of observation and close monitoring by a multidisciplinary team can be taken, as such tumors may regress spontaneously.
Transfacetal and transpedicular anterior decompression with posterior stablilization: Effective way for managing all cases of andersson lesion
Authors: Anand Manikant , Nair Vishnu Vikraman , Shetty Abhijith , Kundnani Vishal , Raut Saijyot , Dewnany Nikhil 
Published: February 2024

Abstract

Background:
Andersson lesion (AL), also known as aseptic spondylodiscitis, is a pathological condition characterized by a destructive lesion in the vertebral or discovertebral region. The aim of this study was to assess functional, radiological, and neurological outcome of midline posterior surgery with transfacetal/transpedicular decompression and long-segment instrumentation and fusion in all patients with AL.

Materials and Methods:
A total of 36 consecutive patients with AL with or without neurodeficit were managed with midline posterior approach with long-segment fixation, decompression and anterior vertebral reconstruction added by same midline approach. The demographic data, visual analogue scale (VAS), modified Oswestry Disability Index (mODI), ankylosing spondylitis quality of life (ASQoL), local and global kyphosis angle, Frankel neurological grade, blood loss, duration of surgery, perioperative complications, and fusion rate were compiled and assessed.

Results:
The mean age of the patients was 52 years (26 males and 10 females). Among the patients, 9 patients had thoracic, 25 patients had thoracolumbar, and 2 patients had lumbar vertebral involvement. All patients in the study achieved fusion at final follow-up period of 24 months. The mean duration of hospital stay, intraoperative blood loss, and surgery duration was 6.17 days, 252 mL, and 130 min, respectively. VAS improved from mean 7.69 ± 0.98 to 1.67 ± 0.72, mODI from mean 70.28 ± 3.55 to 21.14 ± 2.13, ASQoL from mean 14.22 ± 1.59 to 7.17 ± 1.25, local kyphosis from 23.61 ± 1.67 to 18.52 ± 1.05, and global kyphosis from 57.02 ± 0.72 to 36.16 ± 1.23, leading to better sagittal balance. Except two patients all had full neurological recovery.

Conclusions:
Posterior midline approach with long-segment instrumentation and anterior decompression by transpedicular/transfacetal approach and bone grafting with selective use of cages can give satisfactory results with excellent fusion rates and good functional outcomes.
Hindi translation and modification of modified JOA score for cervical myelopathy and its validation for the Indian population
Authors: Nishant Nishant , Agarwal Varun Kumar , Garg Praveen , Somashekarappa Thimmappa 
Published: February 2024

Abstract

Objective:
To do a cross-cultural adaptation and validation of the translated Hindi version with Indian modification of the Japanese Orthopedic Association (In-mJOA-H) score for cervical myelopathy.

Background:
Indian modification of the Japanese Orthopedic Association (In-mJOA-H) score has never been translated and studied in any vernacular language. This is one of the biggest limitations in assessing disease severity where most of the population is speaking Hindi or other regional languages.

Materials and Methods:
A cross-sectional study of 193 patients, from November 2020 to May 2021, with cervical myelopathy or neck pain with radiculopathy was done. The acceptability, validity, responsiveness, reproducibility, and cross-cultural adaptation were evaluated for the Hindi version (In-mJOA-H).

Results:
A strong correlation was found between In-mJOA-H with the English version and its individual component of the English version Indian modification of the JOA scale (In-mJOA-E).

Conclusion:
Good acceptability, validity, responsiveness, and reproducibility were established for the Hindi version (In-mJOA-H) with Indian modifications and its correlation to the English version Indian modification of the JOA scale (In-mJOA-E).
A rare case of phrenic nerve palsy after cervical spine surgery
Authors: Lodha Shubham Sanjay , Mane Akash Vishnu , Borde Mandar , Menon K V 
Published: February 2024

Abstract
Phrenic nerve palsy is a rare complication of cervical spine surgery. There are very few previously reported cases of unilateral diaphragmatic paralysis following posterior cervical spine surgery. We present a case of a 61-year-old man with cervical canal stenosis and myelopathy for which we performed C3–C6 laminectomy and lateral mass screw fixation.
Postoperatively the patient developed right C5 palsy affecting his deltoid function which gradually improved. The patient developed respiratory distress postoperatively and was found to have an elevated hemidiaphragm secondary to phrenic nerve palsy. He was treated with respiratory support, with significant improvement in dyspnea.
It is a very rare case of unilateral diaphragmatic paralysis causing dyspnea due to phrenic nerve palsy following cervical spine surgery. This rare complication should be kept in mind when assessing any patient with respiratory distress following cervical spine surgery.
Effect of transforaminal lumbar interbody fusion on spinopelvic parameters in degenerative spondylolisthesis and its correlation to patient-reported functional outcome
Authors: Patil Deepak Dinkar , Kothari Abhishek , Hadgaonkar Shailesh , Kothari Ajay , Sancheti Parag K , Aiyer Siddharth Narasimhan 
Published: February 2024

Abstract

Background:
Degenerative spondylolisthesis (DS) is associated with altered radiological spinopelvic parameters (SPP) which leads to poor patient-reported clinical outcomes. Transforaminal lumbar interbody fusion (TLIF) is performed for DS however, its ability to adequately restore SPP is not well reported. The study was performed to determine the effect of TLIF on the SPP and its correlation to patient-reported functional outcomes.

Materials and Methods:
Prospective, consecutive recruitment of patients was performed, with single and double-level DS treated with TLIF between March 2019 to March 2020 with a 2-year follow-up. Functional outcomes were measured using the visual analog score (VAS) score, Oswestry Disability Index (ODI) score, and SF-36 questionnaire. SPPs (pelvic tilt [PT], pelvic inclination, sacral slope [SS], lumbar lordosis [LL], and PI-LL mismatch) were recorded. The correlation between SPP and functional outcomes was analyzed using Spearman’s correlation coefficient.

Results:
A total of 40 patients were included in the final analysis. Significant improvement in VAS score, ODI score, and SF-36 score was observed (P < 0.05 for all) at 2 years follow-up. No significant difference was noted in the PI, LL, and SS following surgery (P > 0.05). A significant change in mean PT from 15.76° to 19.53° was noted (P = 0.023). The mean PI-LL mismatch improved from 14.7° to 11.2° which was statistically significant (P < 0.05). However, no significant correlation between ODI score with PT (r = 0.156) and post-op PI-LL mismatch (r = 0.105) could be demonstrated.

Conclusion:
TLIF led to satisfactory improvement in the patient-reported outcomes, PT, and PI-LL mismatch. However, the improvement of the SPP did not correlate with improvements in the ODI score.