bluuebunny/crossref_metadata_2025_split huggingface dataset:
Skin Prick Tests and Subcutaneous Immunotherapy with Standardized Allergens in Children with Moderate-to-Severe Allergic Rhinitis
Authors: Mishra Nihar Ranjan , Nagaraju Karthik , Voorakaranam Kasyapi 
Published: November 2023

Background:
Allergic rhinitis is common in Indian children, one of the common triggers being house dust mites. Skin prick tests (SPTs) and subcutaneous immunotherapy (SCIT) using standardized extracts of these aeroallergens are increasingly being used in the diagnosis and the management of allergic rhinitis in India.

Clinical Description:
We describe three children with moderate-to-severe allergic rhinitis who were considered ideal candidates for SCIT based on persistent typical clinical symptoms causing significant functional impairment, despite multiple medications for years, and positive family history. Each displayed characteristic local signs (crease over nasal bridge, pale nasal mucosa, and hypertrophy of the inferior nasal turbinates) and normal systemic examination.

Management:
Each of the children displayed significant sensitization with allergens containing standardized preparations of Dermatophagoides pteronyssinus (Dp) and Dermatophagoides farinae (Df) on the basis of which it was decided to start them on SCIT using the standard protocol. Large local reactions developed and were managed conservatively. All three responded well to bi-weekly shots of progressively increasing concentrations used in the induction phase of SCIT, and are currently asymptomatic on monthly maintenance doses that will continue for 2–3 years.

Conclusion:
Pediatricians should consider referring children with moderate-to-severe allergic rhinitis to Pediatric Allergists for SCIT if significant wheals are observed on SPT. SCIT not only stops the progression of disease, and improve the quality of life, but is also known to prevent the development of bronchial asthma.
Childhood Vasculitis Syndrome Mimicking Guillain Barre Syndrome
Authors: Jena Pradeep Kumar , Satpathy Swasthi Kabi , Naik Sarthak , Nayak Alok Satyaprakash 
Published: November 2023

Background:
Childhood vasculitis is a heterogeneous disorder, characterized by inflammation of the blood vessel walls. Multiple organs and/or tissues are affected, either simultaneously or successively. Vascular neuropathy occurs when the vasa nervorum is affected. Presentation includes mononeuritis multiplex, acute motor/motor-sensory axonal neuropathy, pure sensory neuropathy, and chronic inflammatory demyelinating polyneuropathy. Making a diagnosis is a challenge when neurological manifestations appear for the first time or are isolated.

Clinical Description:
A 12-year-old girl presented with acute pain and progressive weakness of both lower limbs for 12 days, followed by diffuse abdominal pain and low-grade fever. Salient neurological findings were diminished power and hyporeflexia in all limbs. Guillain–Barre syndrome was suspected in view of symmetric ascending paralysis and a suggestive nerve conduction study. The child had neutrophilic leukocytosis but sterile cultures. The successive development of inflammatory demyelinating polyneuropathy, persistent fever, vasculitic phenomena (hypertension, severe myalgia, rashes, multiple infarcts, acute renal cortical necrosis, and gangrene of the digit), and elevated acute-phase reactants was suggestive of a multisystemic small-vessel and medium-sized vasculitis syndrome, such as polyarteritis nodosa (PAN). A diagnosis of PAN was established based on the satisfaction of clinical criteria.

Management:
The patient was administered pulse methylprednisolone and oral steroids, with which there was a dramatic recovery. Monthly cyclophosphamide was continued in view of major systemic involvement.

Conclusion:
Early recognition and management of childhood vasculitis syndrome is associated with good outcomes.
Recurrent Tension Pneumopericardium in a Term Neonate on High-Frequency Ventilation
Authors: Natarajan Rupesh Kumar , Becker Robert , Sheurer Johannah , Berry James M. , Aggarwal Varun 
Published: November 2023

Background:
Air leak syndrome (ALS) can be seen in sick neonates on positive pressure ventilation. Tension pneumopericardium is uncommon, but life-threatening if not promptly identified and treated. Needle decompression can be life-saving. Recurrent tension pneumopericardium is difficult to treat and ideally indicates the placement of a pericardial drain under fluoroscopic guidance. We describe an alternative technique of percutaneous drain placement using intrapericardial saline infusion when the transport of the neonate to a catherization laboratory was not feasible.

Clinical Description:
A neonate with hyaline membrane disease and persistent pulmonary hypertension of new-born on high-frequency mechanical ventilation developed recurrent tension pneumopericardium that caused hemodynamic compromise.

Management:
Emergency needle decompression was performed twice. Further recurrence necessitated pericardial drain placement under echocardiographic guidance. Rapid exit of air from the pericardial cavity made advancement of the guidewire challenging. A small amount of sterile saline was injected in the pericardial space to assist advancement following which placement of the pericardial drain was successful. Subsequently, the neonate had an uneventful course and was discharged after 3 weeks.

Conclusion:
Prompt recognition and management of ALS can be life-saving. Urgent needle decompression and pericardial drain placement can be performed under fluoroscopic (ideally) or echocardiographic guidance by trained personnel. Instillation of saline in the pericardial space may assist with pericardial drain placement.
Treatment of Highly Fatal Extensive Childhood Mucormycosis with Complications
Authors: Chidambaram Aakash Chandran , Ramamoorthy Jaikumar Govindaswamy , Gulati Reena , Badhe Bhawana A 
Published: November 2023

Background:
Mucormycosis is a highly fatal infection that affects immunocompromised individuals. Treatment is difficult and mortality is high when associated with complications. It is rare as a presenting feature of diabetes mellitus (DM) in children.

Clinical Description:
We describe a child who presented with extensive rhino-orbital invasive mucormycosis and subsequently diagnosed as Type 1 DM. She further developed cavernous sinus thrombosis and internal carotid artery thrombosis known to be associated with very high risk of mortality.

Management:
Aggressive medical management with posaconazole and liposomal amphotericin B for 12 weeks and early debridement of orbito-cerebral lesions led to complete resolution.

Conclusion:
Saving children with complicated extensive invasive mucormycosis is possible with aggressive prolonged antifungal therapy and early debridement of lesions.
Guillain–Barre Syndrome Following SARS-CoV-2 Infection in Children
Authors: Sinha Amrita , Varshi Sumitra , Dwivedi Deepak 
Published: November 2023

Background:
Guillain–Barre Syndrome (GBS) is an autoimmune polyneuropathy involving mainly motor but also sensory and autonomic nerves. It is considered postinfectious and is been linked with many infectious organisms such as human immunodeficiency virus, Zika virus, and others. Since the onset of the pandemic, a few cases of GBS with a temporal association with SARS-CoV-2 infection has been reported in adults and children. The manifestations are similar to the classic clinical phenotype of progressive, ascending, symmetrical flaccid paralysis of the limbs with areflexia/hyporeflexia, and with or without cranial nerve involvement. This series describe three children with post-COVID GBS.

Case Description:
Three unrelated boys, 14 years, 11 years, and 7 years in age presented with varying combinations of pain, paraesthesias, and ascending weakness involving their limbs for a few days. We suspected GBS based on the clinical phenotype of rapidly progressive symmetrical weakness. The Modified National Institute of Neurological Disorders and Stroke Criteria for establishing the clinical diagnosis of GBS were satisfied. The typical albuminocytological dissociation in cerebrospinal fluid was observed in all.

Management:
The GBS was presumed to be postinfectious secondary to the COVID-19 virus in each one, based on elevated COVID-19 immunoglobulin G (IgG) and IgM antibody titers, exclusion of other common infectious organisms, and normal magnetic resonance imaging. All these patients were started on intravenous immunoglobulin and methylprednisolone. Two children recovered without any neurological deficits. The third child expired due to respiratory involvement.

Conclusion:
COVID-19 infection may result in GBS, the clinical profile of which is similar to that seen after other infectious organisms.
High Axial Myopia in Neurofibromatosis Type 1
Authors: Garg Rajiv , Madan Siddharth , Yadav Nisha , Yadav Rekha 
Published: November 2023

Background:
Clinicians must be aware of phenotypic variability in neurofibromatosis type 1 (NF 1) presentations. There is perhaps a limited understanding on progression of NF 1 in prepubertal years and the subsequent threat to vision. Progressively increasing myopia may go unnoticed under a severely ptotic eyelid that gathers attention, due to a disfiguring mass in patients with NF 1. High myopia may result in recalcitrant amblyopia if not tackled early with multidisciplinary management.

Clinical Description:
A 12 year old girl had a history of progressively increasing left upper eyelid ptosis due to an upper eyelid mass, first noticed at the age of 1 year and eventually resulted in severe ptosis by the age of 5 years. However, this went unnoticed until she was diagnosed with NF 1 at the age of 12 years. Best corrected visual acuity was 6/6 (Plano) in the right eye (OD) and counting finger 2 m with − 15.0 diopter spheres in the left eye (OS). Peripheral fundus examination was normal in both eyes. Levo elevation and abduction were limited OS. Hypotropia, pulsatile proptosis, and depression of the globe were clinically attributable to enlargement of orbital tissues and lid problems. Contrast enhanced computed tomography scan revealed plexiform NF with extraconal extensions. Axial length was 21.94 mm OD and 28.92 mm OS. B scan ultrasound revealed a posterior staphyloma OS.

Management:
The patient underwent a debulking surgery of the eyelid mass which on histopathological examination confirmed plexiform NF. Surgery resulted in a cosmetic reduction in ptosis; however, any intervention was relatively too late to rehabilitate the left eye.

Conclusions:
Eye care certainly has its regional differences. High axial myopia may result in low VA and recalcitrant amblyopia that may go unrecognized and comes with management challenges to the attending ophthalmologist and allied specialties dealing with these cases of NF 1.
Kikuchi-Fujimoto Disease
Authors: Sawant Sangeeta Priyadarshi , Amin Alpa S. , Gaddam Prachi R. , Chaturvedi Uma P. 
Published: November 2023

Background:
Kikuchi Fujimoto disease (KFD) is a rare, benign self-limited disease characterized by prolonged regional lymphadenopathy associated with or without systemic signs or symptoms. It is a rare diagnosis in children. Due to the lack of pathognomonic clinical symptoms/signs, KFD poses a significant challenge to the clinician. Its diagnosis is confirmed by lymph node biopsy.

Clinical Description:
A 12.5-year boy presented with chronic cervical lymphadenopathy of 6-week duration, associated with mild-moderate fever, pain, and weight loss. He had raised erythrocyte sedimentation rate, leukopenia, lymphopenia, and thrombocytopenia. Mantoux test was positive. He was managed as a case of tuberculous lymphadenopathy till the lymph node biopsy confirmed the diagnosis of KFD.

Management:
The child recovered without medications. There has been no recurrence or relapse in 1.5 years of follow-up.

Conclusion:
This case report highlights the importance of considering the diagnosis of KFD in children presenting with persistent or chronic lymphadenopathy.
Necrotizing Enterocolitis as a Possible Manifestaion of Multisystem Inflammatory Syndrome in Neonates Related to SARS-CoV-2
Authors: Kumar Chanchal , Pothala Rajeev , Bathina Sushma Poornima , Dodda Deepika 
Published: November 2023

Background:
Multisystem inflammatory syndrome in neonates (MIS-N) is hypothesized to be caused either following transplacental transfer of severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) antibodies or antibodies developed in the neonate after infection with SARS-CoV-2. The common clinical manifestations include fever, respiratory distress, shock, and encephalopathy. Gastrointestinal manifestations include vomiting, diarrhea, bloody stools, and gastric residues. Necrotizing enterocolitis (NEC) has not been described before in MIS-N.

Clinical Description:
We report a term neonate presenting on day 10 of life with 2 days history of fever, abdominal distension, vomiting, reduced feeding, and not passing stool. He had tachycardia, tachypnea, a distended tense and tender abdomen with absent bowel sounds, and excessive irritability. Salient investigation results were anemia, neutrophilic leukocytosis, hyponatremia, metabolic acidosis, and elevated inflammatory markers. Dilated bowel loops on abdominal radiograph and thickening of bowel wall on abdominal ultrasonography were consistent with NEC. In view of the unusual presentation of NEC, the elevated biomarkers and setting of the pandemic, MIS-N was suspected, investigated, and the diagnostic criteria were found to be satisfied.

Management:
The baby received supportive care. Methylprednisolone, intravenous immunoglobulin, aspirin, and enoxaparin were started resulting in defervescence and decline in inflammatory markers. However, he developed fixed bowel loops and underwent an exploratory laparotomy. This revealed stricture and extensive necrosis of the terminal ileum with adhesive obstruction. The affected segment was excised, and loop ileo-colostomy was done. He gradually improved and was discharged on the 11th postoperative day on enteral feeds. The infant was thriving on follow-up.

Conclusion:
NEC may be a possible manifestation of MIS-N.
Unclassified Sudden Infant Death Due to Congenital Long QT Syndrome with TRPM4 Mutation
Authors: Kaur Jasleen , Puri Ratna Dua , Mukherjee Sharmila B. , Vyas Bijal 
Published: November 2023

Background:
Congenital long QT syndrome (cLQTS) are heritable disorders due to genetic mutations causing prolonged corrected QT (QTc) interval that may result in fatal arrhythmias.

Clinical Description:
A well 20-day-old, exclusively breastfed boy had an episode of unresponsiveness with no other symptoms. There was a history of four previous unexplained infantile deaths after similar complaints. General and systemic examination was normal. The differential diagnoses were neonatal apnea, seizures, and inborn errors of metabolism.

Management:
Sepsis, hypoglycemia, and electrolyte imbalance were ruled out. Electrocardiography (ECG), chest radiograph, and echocardiograph were normal. Ultrasonogram of the cranium ruled out structural abnormality and bleed. Electroencephalogram was normal. First-line metabolic investigations were normal. On day 28 of life, he had a recurrent episode of apnea. ECG evaluation during this episode revealed a prolonged QTc interval. Suspecting cLQTS, Trio Whole Exome Sequencing for mutations in the cLQTS susceptibility genes was performed in the proband and parents. A heterozygous variation, c.290C>T; p. Thr97Met in TRPM4 was identified in the symptomatic neonate and asymptomatic mother, suggesting autosomal dominant inheritance. The baby was started on oral propranolol, but succumbed at 8 weeks. The mother was referred for cardiac management and parents counseled about possible prenatal diagnosis in subsequent pregnancies.

Conclusion:
cLQTS should be suspected in neonatal apnea, when no other cause is appreciable. A normal ECG does not exclude cLQTS. If there is a strong suspicion, repeat ECGs and appropriate genetic testing should be done. Patients should be managed according to standard guidelines.
Giant Juvenile Fibroadenoma in a Young Female-A Diagnostic Dilemma
Authors: Gautam Vasu , Khanna Chetna , Meena Atul Kumar , Debnath Pinaki Ranjan 
Published: November 2023

Background:
Fibroadenoma is the most common breast lesion among pediatric female population. A giant fibroadenoma is rare, characterized by a rapidly growing tumor, with a mass >5 cm diameter in greatest dimension and/or weighing more than 500 gm. Phyllodes tumor range from benign to malignant. Both have similar presentation.

Clinical Description:
An 11-year-old girl presented with rapidly progressive, painless increase in the size of the left breast over 2 months. Local examination revealed a firm nontender mass involving the upper inner and outer quadrants of the left breast along with the retro-areolar region. The mass was mobile and measured approximately 11 cm × 10 cm. The overlying skin was normal, although with dilated veins and free from underlying mass. The clinical phenotype was suggestive a phyllodes tumor in view of rapid progression and large size. However, the ultrasonogram and fine-needle aspiration cytology favored a fibroadenoma.

Management:
It was decided to perform a “Nipple-areolar complex sparing lumpectomy” with deferment of reconstructive surgery. Adequate lump excision was achieved while maintaining proper cosmesis. The histopathological findings of the excision biopsy confirmed the final diagnosis of fibroadenoma.

Conclusion:
A large breast mass in a pediatric/adolescent girl poses a diagnostic dilemma to the treating surgeon. Proper evaluation is needed to differentiate between several benign breast masses from malignant ones. The definitive diagnosis is made histologically. Total excision of the lump with conservation of nipple and areola is indicated to make a definitive diagnosis and to relieve the compression of the normal breast tissue.
Matched Unrelated Hematopoietic Stem Cell Transplant in Juvenile-Onset Krabbe's Disease
Authors: Misra Ruchira , Bhat Sunil , Shah Suhani , Hegde Anaita , Panwala Hiren , Hingwala Divyata 
Published: November 2023

Background:
Krabbe's disease is a neurodegenerative condition caused by a deficiency of a lysosomal enzyme, galactocerebrosidase (GALC), found mainly in the brain. There are four variants based on the age of onset: Infantile, late infantile, juvenile, and adult. Hematopoietic stem cell transplants can be curative as the GALC-positive donor cells migrate to the brain and provide GALC, thus correcting the deficiency. However, this is most effective when performed in the asymptomatic or mildly symptomatic phase. Maximum experience is in infantile-onset disease. The types of transplants usually used are matched sibling donor transplant or umbilical cord blood transplant.

Clinical Description:
A 5-year-old girl, the only child of a nonconsanguineous marriage, presented with complaints of frequent falls and progressive visual deterioration for 2 months. After evaluation, the clinical phenotype was ascertained to be that of a juvenile-onset (>5 years) neurodegenerative disorder with neuro-axis involvement of the brain (predominantly, white motor involvement-because of gait abnormalities and visual involvement) and absence of organomegaly or any other systemic involvement. Krabbe's disease was suspected. Supportive evidence was found on neuroimaging and confirmed by a homozygous mutation in the GALC gene that was consistent with Krabbe's disease.

Management:
The patient was treated with a matched unrelated bone marrow transplant, a modality that is generally not used in the management of Krabbe's disease. The clinical, neurophysiological, and neuroradiological profiles are compared during a 2-year follow-up. Currently, 2 years' posttransplant, she has not deteriorated further neurologically, and though legally blind is attending a special school for the visually impaired and performing most activities of daily living independently.

Conclusion:
Early matched unrelated donor transplant can be beneficial in juvenile-onset Krabbe's disease.
Intraoperative Diagnosis of Neoplastic Lesions by Imprint Cytology and Frozen Section with Histopathological Correlation
Authors: Deoghare Saroj , Ali Syed S. , Dhawan Shrutika , Sawaimul Komal D. 
Published: November 2023

ABSTRACT

Introduction:
Intraoperative consultation examinations are required by surgeons from pathologists for immediate important decisions regarding the optimal extent of surgery. Surgeons particularly want to know whether a lesion is malignant or not. Both frozen section (FS) and touch impression cytology serve this purpose well. Both provide accurate results in minutes while the patient is under anesthesia. The surgeon then modifies his surgical plan based on the intraoperative consultation from pathologist.

Objectives:
The objective of this study is to access the role of imprint cytology (IC) and FS in diagnosis of tumors of different organs, to correlate IC and FS findings with histopathological diagnosis and to determine their diagnostic accuracy.

Materials and Method:
This present prospective cross-sectional study was done in the pathology department of BKL Walawalkar Rural Medical College, Dervan, Chiplun, for a period of one and a half year. A total of 161 surgical specimens from various organs were studied using IC and FS. The results of both the techniques were compared with the gold standard histopathological diagnosis.

Results:
Statistical correlation of IC and FS with histopathology shows that true positive cases on IC are 88.7% and on FS are 85.1%. Sensitivity, specificity, and diagnostic accuracy of IC and FS are 95.7%, 81.8%, 94.7% and 93.2%, 78.9%, 92%, respectively. Combined diagnostic accuracy is 93.5%.

Conclusion:
IC and FSs are interdependable intraoperative diagnostic modalities. IC is a simple, accurate, rapid, and cost-effective diagnostic tool, intraoperatively. FS preserves crisp cellular and nuclear detail and also closely approximates permanent histopathology sections. Thus, a combination of IC and FS helps in achieving high diagnostic accuracy rates and in distinguishing benign and malignant lesions.
Effect of Light Intensity on Photosynthesis in Aquatic Plants in Australia
Authors: Frankie Darcy 
Published: November 2023

Purpose: The aim of the study was to investigate the Effect of Light Intensity on Photosynthesis in Aquatic Plants in Australia.
Methodology: The study adopted a desktop methodology. Desk research refers to secondary data or that which can be collected without fieldwork. Desk research is basically involved in collecting data from existing resources hence it is often considered a low cost technique as compared to field research, as the main cost is involved in executive’s time, telephone charges and directories. Thus, the study relied on already published studies, reports and statistics. This secondary data was easily accessed through the online journals and library
Findings: In the study examining the impact of light intensity on photosynthesis in Australian aquatic plants, key findings emerged. It was evident that light intensity played a crucial role, with higher light levels in shallower waters leading to increased photosynthesis rates. This underscored the significance of light as a primary determinant of photosynthesis in these ecosystems. Additionally, the study highlighted the impressive adaptability of Australian aquatic plants, some of which employed unique photo protective mechanisms to prevent photo inhibition under varying light conditions.
Unique Contribution to Theory, Practice and Policy: Theory of Liebig's Law of the Minimum, Theory of The Beer-Lambert Law and Optimal Foraging Theory may be used to anchor future studies on the Effect of Light Intensity on Photosynthesis in Aquatic Plants in Australia. Develop practical guidelines for growers to optimize light conditions for maximum photosynthetic efficiency. Advocate for policies that recognize the significance of aquatic plants in maintaining healthy aquatic ecosystems. Promote the conservation and restoration of natural habitats for aquatic plants, especially in areas threatened by pollution or habitat destruction.