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Precocious Puberty in a 5-Year-Old Girl with a Giant Hypothalamic Hamartoma Discovered Perinatally: Case Report

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Open Journal of Endocrine and Metabolic Diseases 2024年第4期14卷 101-106页

作者： Ghita Bourkadi Sanae Habibi Sana Abourazzak moustapha hida Department of Endocrinology Diabetology Metabolic Diseases and Nutrition Hassan II University Hospital Fez Morocco Department of Pediatrics Hassan II University Hospital Fez Morocco Faculty of Medicine and Pharmacy Sidi Mohamed Ben Abdellah University Fez Morocoo

Background: Hypothalamic hamartoma is a rare non-neoplastic lesion, typically identified in early childhood during investigations for precocious puberty and/or gelastic seizures. However, cases of diagnosis even earlier or during fetal development have been documented. Case Presentation: A newborn girl was diagnosed with hydrocephalus during pregnancy. An MRI revealed a large oval hypothalamic process, which suggested a hypothalamic hamartoma. At the age of 2, she experienced alternating crying and laughing seizures, followed by a decrease in visual acuity. Due to involvement of the optic pathways, surgery was not performed and she underwent antiepileptic medicines and gamma knife radiotherapy. At the age of 5 years and 3 months, she presented with breast development and laboratory tests confirmed central precocious puberty. Quarterly injections of GnRH agonists have since been administered with favorable results. Conclusion: Early-diagnosed hypothalamic hamartomas require close monitoring, by an experienced multidisciplinary, to promptly detect and treat potential complications, especially precocious puberty, and prevent any undesirable impact on final height.

关键词： Perinatally-Hypothalamic Hamartoma-Giant-Precocious Puberty

Kostmann’s Neutropenia: A Case Report

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Open Journal of Pediatrics 2024年第5期14卷 869-872页

作者： Khaoula Achir Ilham Tadmori Mounia Lakhdar Idrissi Fatima Zahra Souilmi Mohamed Hbibi moustapha hida Pediatric Department of CHU Hassan II in Fez Fez Morocco

Kostmann syndrome, or severe congenital neutropenia, is a rare condition in children marked by a neutrophil count of less than 500/mm 3 . This congenital agranulocytosis, an autosomal recessive genetic disorder, is often first identified by a neonatal infectious syndrome. The deficiency in neutrophils increases susceptibility to bacterial and fungal infections. Prior to the availability of hematopoietic growth factors, the disease was associated with significant morbidity and early mortality. We present the case of a 17-month-old boy who was admitted to the pediatric emergency department at Hassan II University Hospital in Fes with skin abscesses.

关键词： Kostmann Syndrome Severe Congenital Neutropenia Skin Abscesses

Lemierre Syndrome Revealed by Orbital Cellulitis

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Open Journal of Pediatrics 2023年第6期13卷 900-906页

作者： Issam Taha Ilham Tadmori moustapha hida Pediatric Emergency Department Faculty of Medicine and Pharmacy of Fez Sidi Mohamed-Ben-Abdellah University Fez Morocco Mother-Child Hospital Hassan II University Hospital Fez Morocco

Lemierre’s syndrome is a rare and severe sepsis, capable of rapidly compromising the vital prognosis in the absence of early intervention, characterized by an oropharyngeal infection complicated by septic thrombosis of the internal jugular vein and the formation of septic emboli primarily localized at the pulmonary level. We report the case of a 14-year-old boy, with no notable pathological history, who had presented for 15 days with unilateral orbital swelling which then became bilateral, all evolving in a context of fever.

关键词： Lemierre’s Syndrome Oropharyngeal Infection Jugular Septic Thrombosis Septic Embolism

Niemann-Pick A/B Disease in a 13-Year-Old Child and Review of the Literature

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Open Journal of Pediatrics 2023年第6期13卷 907-913页

Niemann-Pick disease (NPD) refers to a group of patients who have varying degrees of lipid storage and foam cell infiltration in tissues, as well as overlapping clinical features, including hepatosplenomegaly, insufficiency pulmonary and/or central nervous system (CNS). Thanks to the pioneering work of Roscoe Brady and colleagues, we now know that there are two distinct metabolic abnormalities that explain NPD. The first is due to the deficient activity of the acid sphingomyelinase enzyme (ASM;NPD “types A and B”), and the second is due to defective functioning in the transport of cholesterol (NPD “type C”). We report the case of a 13-year-old adolescent diagnosed with Niemann-Pick A/B disease.

关键词： Nieman Pick Splenomegaly Hepatomegaly Enzymatic Test

Erysipelas in Children: Risk Factors and Management in the Pediatric Emergency Department

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Open Journal of Pediatrics 2023年第6期13卷 914-920页

作者： Issam Taha Ilham Tadmori Widade Kojmane moustapha hida Mother-Child Hospital Hassan II University Hospital Fez Morocco

The aim of this study is to describe the epidemiological, clinical, biological profile, etiology and management of erysipelas in pediatric emergency departments. In this retrospective study carried out over a period of 12 months, we identified 20 cases of erysipelas, with an incidence of 2%, and a female predominance of 65% of cases. Age average patient age was 6 years, and the average consultation time was 5 days. The Diagnosis of erysipelas is mainly based on clinical features, including the presence of erythema associated with inflammatory signs, which were present in all cases. Satellite lymphadenopathy was observed in five cases, and fever was present in all patients. The therapeutic approach is mainly based on the administration of injectable antibiotics in children, using amoxicillin-clavulanate dose of 80 mg/kg/day in three doses.

关键词： Erysipelas Fever Antibiotic Therapy Child

Multi-Systemic Inflammatory Syndrome in Children

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Open Journal of Pediatrics 2023年第4期13卷 529-542页

作者： Ilham Tadmori Soukaina Seddiki Samir Atmani moustapha hida Department of Pediatrics and Pediatric Emergencies Mother and Child Hospital CHU Hassan II Fez Morocco

The objective of our work is to study the multi-systemic inflammatory syndrome (PIMS) in children, to determine its frequency, by analyzing the epidemiological, clinical, paraclinical, therapeutic, and evolutionary profile of these patients. A retrospective study spanning a period of 2 years from April 2020 to March 2022. It concerns all children under the age of 16 admitted and cared for in the pediatric emergency department of the university hospital Hassan II of Fez for multi-system inflammatory syndrome (PIMS). Twenty cases of PIMS were collected over this period. Multi-system inflammatory syndrome in children has been described in temporal association with COVID-19, usually within 2 to 6 weeks of illness or exposure. The age of the patients varies between 8 months and 15 years. All patients presented with fever and cutaneous signs, followed by digestive signs and neurological signs. The inflammatory syndrome is frankly positive in all patients who had a COVID-19 PCR and/or positive serology. The treatment is based on the administration of immunoglobulins in association with corticosteroid therapy and non-specific antibiotic therapy in the majority of cases (80%). The evolution was favorable. PIMS should be considered in all children presenting with a clinical and/or biological inflammatory syndrome associated with COVID-19.

关键词： Multisystemic Inflammatory Syndrome Child COVID-19 PIMS

Jacob’s Syndrome and Deficiency of 11-Beta-Hydroxylase Enzyme Association Revealed by a Statural Advance: A Case Report

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Case Reports in Clinical Medicine 2023年第6期12卷 207-211页

作者： Wafa Aitifali Fatima Zahra Lahmamssi Sana Abourazzak Laila Bouguenouch moustapha hida Endocrinology Unit of the Pediatrics Department Hassan II University Hospital Fez Morocco

47XYY syndrome is a rare sex chromosome variant with an extra Y chromosome. Most patients with a 47XYY karyotype have a normal phenotype. This disorder seems to be associated with a higher risk of developing behavior and cognitive problems, tall stature and infertility in adulthood. We report here a rare case of 47, XYY syndrome associated with an 11-beta-hydroxylase deficiency revealed by a stature advance along with precocious puberty after obtaining informed consent from parents. To our knowledge, this is the first case reported in the literature.

关键词： Jacob’s Syndrome 47XYY Syndrome 11-Beta-Hydroxylase Deficiency

Multi-Systemic Inflammatory Syndrome in Children

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Jacob’s Syndrome and Deficiency of 11-Beta-Hydroxylase Enzyme Association Revealed by a Statural Advance: A Case Report

Open Journal of Pediatrics（儿科学期刊） 2023年第4期

作者： Ilham Tadmori Soukaina Seddiki Samir Atmani moustapha hida

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