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Macrophage Activation Syndrome as the Primary Presentation of Pediatric Systemic Lupus Erythematosus: A Case Report and Review of the Literature

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Open Journal of Pediatrics 2024年第1期14卷 132-138页

作者： Hanane Hajaj Hanae Bahari Aziza El Ouali Ayyad Ghanam Maria Rkain abdeladim babakhouya Department of Pediatrics Mohammed VI University Hospital Oujda Morocco Mohammed VI University Hospital Medical School University Mohammed First Oujda Morocco

Macrophage activation syndrome (MAS), in its secondary form, often complicates rheumatic diseases but rarely constitutes a mode of revelation. Systemic lupus erythematosus (SLE) is a systemic autoimmune disease of unknown etiology that primarily affects women in adulthood. MAS is a serious condition that may be the first presentation of SLE. Here, we report the case of a 4-year-old female with MAS as the primary manifestation of Systemic Lupus Erythematosus (SLE). In this case, we outline the characteristics of a complex case of SLE that was initially accompanied with MAS, and also review the literature to discuss the clinical, biological, and therapeutic aspects of this condition.

关键词： Macrophage Activation Syndrome Systemic Lupus Erythematosus Child

Attitudes and Practices of Lumbar Puncture among Students, Interns, and Residents of the Pediatric Department of the Mohammed VI University Hospital Center of Oujda, Morocco

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Open Journal of Pediatrics 2024年第3期14卷 598-608页

作者： Hasnae Elhaddadi Ayyad Ghanam Hind Zahiri Amal Hamami Aziza Elouali abdeladim babakhouya Maria Rkain Department of Pediatrics University Hospital Center Mohammed VI Oujda Morocco Faculty of Medicine and Pharmacy Mohammed First University Oujda Morocco

Introduction: Even though lumbar puncture (LP) represents an important tool in the diagnosis of certain neurological diseases, this procedure is little practiced by our students. We aimed to evaluate the attitudes and practices of students, interns, and residents about LP, and to assess their feelings about how this procedure is taught. Materials and Methods: We conducted a cross-sectional study of 160 participants, using an anonymous questionnaire, to evaluate the attitudes and practices of students, interns and residents in the pediatrics department concerning lumbar puncture. Results: Half of the participants had never performed LP, usually because of the risk involved or lack of confidence, while 20% had performed it more than 4 times. None of the participants had learned to perform the procedure through simulation sessions, while (42%) had learned it under the supervision of a senior physician and had not been able to perform it successfully the first time. Most participants inform the patient about the procedure before performing it. Only 44% of participants acknowledged that LP can be performed under local anesthetic. The sitting position (60%) was by far the most commonly used. Most LPs were performed for diagnostic purposes. Most participants stated that the pediatric ward and the pediatric emergency department are among the departments that perform LP most frequently, and that they would be interested in taking part in simulated lumbar puncture sessions in children in the future. Conclusion: The results of our study show that LP is perceived by students as a risky procedure that is difficult to perform. Teachers should reconsider how this technical procedure is taught, by integrating simulation on mannequins into student training.

关键词： Lumbar Puncture Learning Healthcare Simulation Stress Clinical Competency

Extra Dural Hematoma of the Dorso-Lumbar Region in a Hemophiliac: A Rare Entity

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Open Journal of Pediatrics 2024年第1期14卷 84-88页

作者： Hanane Hajaj Hanae Bahari Ayyad Ghanam Aziza El Ouali abdeladim babakhouya Maria Rkain Department of Pediatrics Mohammed VI University Hospital Oujda Morocco Faculty of Medicine and Pharmacy Mohammed Ist University Oujda Morocco

Introduction and objective: Hemophilia is a genetic bleeding disorder inherited as a recessive train linked to the male gender. Bleeding into the central nervous system in patients with hemophilia is an extremely dangerous condition that can be directly life-threatening, if left untreated. Extradural hematoma of the dorso-lumbar region is rare but potentially deadly disease in children. This condition can result in severe neurological deficits. We aim to discuss the clinical, radiological and progressive clinical aspects of this illness. Case report: We report the case of a 5-year-old child with severe hemophilia A treated for extradural hematoma of the dorso-lumbar region resulting from trauma. A spinal magnetic resonance imaging revealed an extradural hematoma. The patient was successfully treated with intensive replacement therapy and did not require surgical intervention. Conclusion: Extradural hematoma is a rare complication of hemophilia, that needs to be looked for in children who have bleeding disorders. For the best neurological outcome, early diagnosis is crucial.

关键词： Extradural Hematoma Children Hemophilia

Ecthyma Gangrenosum in Patient with Bone Marrow Aplasia: A Case Report and Review of the Literature

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Open Journal of Pediatrics 2024年第2期14卷 272-278页

作者： Hanane Hajaj Hanae Bahari Hind Zahiri Ayyad Ghanam Aziza El Ouali abdeladim babakhouya Maria Rkain Department of Pediatrics Mohammed VI University Hospital Oujda Morocco Mohammed VI University Hospital Medical School Mohammed First University Oujda Morocco

Background: Ecthyma gangrenosum (EG) is an infrequent and discernible cutaneous disease caused by Pseudomonas aeruginosa. In situations where it is associated with septicemia in debilitated patients, the prognosis is usually unfavorable. Objective: In this case, we aim to verify risk factors, clinical, bacteriological and therapeutic characteristics of ecthyma gangrenosum and we review the literature to highlight the features of this rare condition and discuss the role of early diagnosis and treatment. Case Report: We describe the clinical case of a 4-year-old male with bone marrow aplasia who was presented with characteristic skin lesions of EG and developed sepsis later. Conclusion: EG is a cutaneous disease characterized by its aggressive nature. The presence of delayed diagnosis and therapy, along with sepsis, is closely linked to a high mortality rate. Treatment is empirically founded on an aggressive initial approach.

关键词： Ecthyma Gangrenosum Child Bone Marrow Aplasia

Juvenile Xanthogranuloma: A Case Report with Literature Review

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Open Journal of Pediatrics 2024年第3期14卷 531-535页

作者： Ayad Ghanam Manal Azizi Hind Zahiri Aziza El Ouali abdeladim babakhouya Maria Rkain Department of Pediatrics Mohammed VI University Hospital Faculty of Medicine and Pharmacy Mohammed First University Oujda Morocco

Juvenile xanthogranuloma (XGJ) is the most common form of non-Langerhansian histiocytosis. We report a pediatric case of multiple XGJ without visceral involvement in a 6-month-old female infant who was hospitalized with disseminated congenital skin nodules, of firm consistency, of variable diameter ranging from a few millimetres to 1 cm, involving the face, scalp, trunk and limbs. There was no mucosal localization. The remainder of the somatic examination was unremarkable. The diagnosis of disseminated XGJ was confirmed by skin biopsy without visceral or systemic involvement. Therapeutic abstention was recommended. This observation underlines the fact that XGJ is a rare clinical entity that should not be overlooked when faced with congenital skin nodules.

关键词： Cutaneous Nodules Juvenile Xanthogranuloma Child

Sacrococcygeal Teratoma a Rare Tumor in Children: Case Report

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Open Journal of Pediatrics 2024年第1期14卷 78-83页

作者： Hanae Bahari Hanane Hajaj Hind Zahiri Ayyad Ghanam Aziza El Ouali abdeladim babakhouya Maria Rkain Department of Pediatrics Mohammed VI University Hospital Oujda Morocco Medical School University Mohammed First Oujda Morocco

Sacrococcygeal teratomas (SCTs) are uncommon congenital tumors that typically develop in newborns, they are rarely associated with chromosomal abnormalities or other congenital anomalies. The majority of pediatric teratomas are benign in the neonatal age group, but the risk of malignancy increases with age. Diagnosis is based on a combination of clinical, radiological, and hormonal findings, but confirmed by anatomopathological study. Treatment is primarily surgical, with the aim of achieving complete resection to prevent recurrence. We present the case of a 22-month-old child who was admitted for management of a sacrococcygeal mass and was diagnosed with an immature teratoma.

关键词： Sacrococcygeal Mass Alpha-Fetoprotein Surgery

Rhabdomyosarcoma in Children: About 10 Cases

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Open Journal of Pediatrics 2024年第3期14卷 522-530页

作者： Ayad Ghanam Manal Azizi Hind Zahiri Houssain Benhaddou Imane Kamaoui Amal Bennani Aziza El Ouali abdeladim babakhouya Maria Rkain Noufissa Benajiba Department of Pediatrics Mohammed VI University Hospital Faculty of Medicine and Pharmacy Mohammed First University Oujda Morocco Department of Pediatric Surgery Mohammed VI University Hospital Faculty of Medicine and Pharmacy Mohammed First University Oujda Morocco Department of Radiology Mohammed VI University Hospital Faculty of Medicine and Pharmacy Mohammed First University Oujda Morocco Department of Anatomopathology Mohammed VI University Hospital Faculty of Medicine and Pharmacy Mohammed First University Oujda Morocco

Introduction: Rhabdomyosarcoma (RMS) is a malignant soft-tissue tumor arising from striated muscle cells. It accounts for 60% - 70% of malignant mesenchymal tumors and 5% of pediatric cancers. Two-thirds of these cancers are diagnosed in children under 6 years of age, with a slight male predominance. Materials and Methods: This is a retrospective descriptive study of 10 cases of RMS collected in the pediatric hematology and oncology department of the Oujda university hospital, over a 5-year period, running from January 2018 to December 2022. Results: The median age at diagnosis was 3 years, with a sex ratio of 1. The mean time to diagnosis was 2 months. The most common site was the head and neck (50%), followed by the genitourinary tract (20%), the extremities (20%) and finally the abdomen (10%). The most frequent mode of discovery was a mass or swelling found in 90% of patients (all sites included), followed by exophthalmos in 30% of cases. At the diagnostic stage, CT scans were performed in 70% of cases and MRI in 5 patients (50%). Histological diagnosis was determined by immunohistochemical pathology in all our patients, with a predominance of embryonal (70%) versus alveolar (20%) and spindle cell types (10%). All patients underwent an extension workup, and a cervico-thoraco-abdominopelvic CT was performed in all patients (100%);MRI was performed in 2 patients (20%);lymph node involvement was present in 5 patients (50%). Metastases at the time of diagnosis were noted in only 1 patient (10%), who simultaneously presented with two metastatic sites;testicular and abdominal wall. Sixty percent of patients presented with advanced disease (high risk) and 40% with standard risk. Chemotherapy was used in all patients (100%), with upfront tumor resection performed in 40%. Fifty percent of patients received radiotherapy at a mean dose of 43 Gy, with the orbit the most frequently irradiated area (30%). All patients underwent CTscan and/or MRI and/or ultrasound surveillance.

关键词： Rhabdomyosarcoma Child Chemotherapy Surgery Radiotherapy

Case Report: The Paratesticular Rhabdomyosarcoma in Children

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Journal of Cancer Therapy 2021年第6期12卷 358-364页

作者： Manal Azizi abdeladim babakhouya Maria Rkain Noufissa Benajiba Hematology and Oncology Unit Department of Pediatric UHC Mohammed VI University Mohammed I Oujda Morocco

Rhabdomyosarcoma is a rare and aggressive mesenchymal tumor, whose paratesticular location is the most common genitourinary site. It is the prerogative of the child and the young adult. On physical examination, a painless scrotal tumefaction is observed. His diagnosis is essentially provided by testicular ultrasound. His therapeutic strategy is codified and based on the combination of chemotherapy and surgical excision or radiotherapy. The precocity of the management and the accuracy of the extension and radiology tests are important for the vital and functional prognosis. We report a case of paratesticular rhabdomysarcoma of alveolar type, diagnosed in front of a large left painless scrotal mass, which has been evolving for less than one month in a 7-year-old child. An inguinal orchiectomy was performed. A histological examination of the excised tissue revealed an epididymal RMS of the alveolar type. Our patient had 9 chemotherapy sessions with vincristine, actinomycin C and cyclophosphamide. Each chemotherapy session took place over four days, with a 21-day cycle. He was evaluated for 4 years and demonstrated good clinical improvement. The experience gained from the index case is crucial for the management of patients with intratesticular rhabdo

关键词： Rhabdomyosarcoma Tumor Paratesticular Treatment

Intracardiac Thrombosis Secondary to the Silent Killer: A Case Report about Monoxide Carbon Poisoning in a Child

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Open Journal of Pediatrics 2023年第4期13卷 502-506页

作者： Madiha Benhachem Mariem Labrahimi Aziza Elouali Ayyad Ghannam Maria Rkain abdeladim babakhouya Department of Pediatrics Faculty of medicine and Pharmacy of Oujda Mohamed 1 University Oujda Morocco

Carbon monoxide (CO) poisoning is a frequent cause of emergency room admissions, especially during winter days, the symptoms are varied ranging from a simple headache to a serious cardiac and neurological impairment that can be deadly. Diagnosis is based on the circumstances of occurrence as well as the dosage of carboxyhemoglobin in the blood. Exposure to CO has serious consequences, neurological and cardiac manifestations are not negligible and vary from repolarization disorders to heart attack. Treatment is urgent with normobaric or hyperbaric oxygen therapy. We report a case of a 2-year male child admitted to the emergency room for CO intoxication with an intracardiac thrombus subsequently complicated by an ischemic stroke with a fatal outcome in order to highlight this complication rarely described in literature.

关键词： Carbon Monoxide (CO) Poisoning Intracardiac Thrombosis Child