Van Wyk-Grumbach syndrome: The importance of thyroid function tests in a child presenting with multicystic ovaries.

INTRODUCTION: Van Wyk-Grumbach syndrome (VWGS) is a rare presentation of long-standing hypothyroidism, characterized by delayed bone age, enlarged bilateral multicystic ovaries, and isosexual precocious puberty. CASE PRESENTATION: We report here the clinical details of the case of Van Wyk Grumbach Syndrome in an 11-year-old girl who was first presented to the pediatric surgeon for hemoperitoneum. The patient underwent an emergency exploratory laparotomy, which showed enlarged ovaries with multiple cysts that were bleeding. She also had severe hypothyroidism, hyperprolactinemia, hyperestrogenism, pituitary adenoma, and delayed bone age. Significant improvement in symptoms was noted after thyroid hormone replacement therapy. IMPORTANCE AND CONCLUSION: This case highlights that it is crucial to consider thyroid assessment in a patient with multicystic ovaries to avoid misdiagnosis, unnecessary investigations for malignancy and/or surgical intervention, and possible complications.

[A rare cause of cervical swelling in a child: Solitary plexiform neurofibroma]. / Une cause rare de masse cervicale chez l'enfant : le neurofibrome plexiforme isolé.

Plexiform neurofibroma is a rare benign tumor of the peripheral tissue cells developed in the perineurium. Often considered pathognomonic of neurofibromatosis type 1 (NF1 or von Recklinghausen disease), it can be solitary without NF1, especially in children. The diagnosis is essentially pathological and treatment is primarily surgical to avoid malignant degeneration. We report on a case of cervical solitary plexiform neurofibroma discovered in a 9-year-old child.

[Partial facial duplication (a rare diprosopus): Case report and review of the literature]. / Duplication faciale partielle (un diprosope rare): à propos d'un cas et revue de littérature.

INTRODUCTION: Diprosopus, or partial facial duplication, is a very rare congenital abnormality. It is a rare form of conjoined twins. Partial facial duplication may be symmetric or not and may involve the nose, the maxilla, the mandible, the palate, the tongue and the mouth. OBSERVATION: A male newborn springing from inbred parents was admitted at his first day of life for facial deformity. He presented with hypertelorism, 2 eyes, a tendency to nose duplication (flatted large nose, 2 columellae, 2 lateral nostrils separated in the midline by a third deformed hole), two mouths and a duplicated maxilla. Laboratory tests were normal. The cranio-facial CT confirmed the maxillary duplication. DISCUSSION: This type of cranio-facial duplication is a rare entity with about 35 reported cases in the literature. Our patient was similar to a rare case of living diprosopus reported by Stiehm in 1972. Diprosopus is often associated with abnormalities of the gastrointestinal tract, the central nervous system, the cardiovascular and respiratory systems and with a high incidence of cleft lip and palate. Surgical treatment consists in the resection of the duplicated components.

[Congenital afibrinogenemia: a case report]. / Afibrinogénémie congénitale: à propos d'une nouvelle observation.

Afibrinogenemia is a rare autosomal recessive disease. Its clinical manifestations vary in severity, ranging from minimal bleeding to cataclysmic hemorrhage, and can begin at birth or, sometimes, later. We report a case of a female infant, 10 months of age, hospitalized in the pediatrics department because of a postvaccination hematoma. Biologic exploration found congenital afibrinogenemia. Through this case, we review the clinical features of this disease and its management.

Knowledge and management of fever among Moroccan parents.

Parents often have misperceptions about childhood fever, and little information is available about the home management of feverish children in Morocco. In this study of the perceptions, knowledge and practices of families regarding children's fever, the parents of 264 febrile children aged 0-16 years were interviewed in a paediatric emergency department in Rabat in 2011. Only 3.5% of parents knew the correct temperature definition for fever, 54.4% determined their children's fever using a thermometer, and the preferred site was rectal. Most of them (96.8%) considered that fever was a very serious condition, which could lead to side-effects such as brain damage (28.9%), seizures (18.8%) paralysis (19.5%), dyspnoea (14.8%) and coma (14.8%). Paracetamol was used by 85.9% and traditional treatments by 45.1%. Knowledge about the correct definition of fever was significantly associated with parents' profession, educational level and receipt of previous information and advice from health professionals.

Knowledge and management of fever among Moroccan parents

كثرا ما يكون لدى الآباء أفكار خاطئة عن الحمى التي تصيب الأطفال، كما أن المعلومات المتاحة عن التدبير العلاجي المنزلي للطفل المصاب بالحمى قليلة في المغرب. وقد استهدفت هذه الدراسة معرفة مدركات ومعارف وممارسات الأسر حول الحمى التي تصيب الأطفال، فأجرى الباحثون مقابلات مع 264 من آباء الأطفال الذين أصيبوا بالحمى في أعمار تراوحت بين 0 - 16 عاما، وذلك في قسم طوارئ الأطفال في الرباط في عام 2011 . واتضح للباحثين أن 3.5 % فقط من الآباء يعرفون التعريف الصحيح لدرجة الحرارة أثناء الحمى، وأن 54.4 % منهم يتعرفون على إصابة الطفل بالحمى باستخدام مقياس الحرارة، وأن الموضع المفضل لقياس درجة الحرارة هو الشرج، وأن معظم الآباء [96.8 % منهم]يعترون الحمى حالة خطرة قد تقود إلى تأثرات جانبية مثل تخرب الدماغ [28.9 %]، والاختلاجات [18.8 %]، والشلل [19.5 %]، وضيق النفس [14.8 %]، والسبات [14.8 %]. وقد تم استخدام الباراسيتامول في 85.9 % من الحالات، والمعالجات التقليدية في % 45.1 من الحالات. وقد ترابطت المعرفة المتعلقة بالتعريف الصحيح للحمى بمقدار يعتد به إحصائيا مع مهنة الآباء، ومستواهم التعليمي ومع تلقيهم معلومات مسبقة ومنشورات من المهنيين الصحيين

ABSTRACT Parents often have misperceptions about childhood fever, and little information is available about thehome management of feverish children in Morocco. In this study of the perceptions, knowledge and practicesof families regarding children’s fever, the parents of 264 febrile children aged 0–16 years were interviewed in apaediatric emergency department in Rabat in 2011. Only 3.5% of parents knew the correct temperature definitionfor fever, 54.4% determined their children’s fever using a thermometer, and the preferred site was rectal. Most ofthem (96.8%) considered that fever was a very serious condition, which could lead to side-effects such as braindamage (28.9%), seizures (18.8%) paralysis (19.5%), dyspnoea (14.8%) and coma (14.8%). Paracetamol was usedby 85.9% and traditional treatments by 45.1%. Knowledge about the correct definition of fever was significantlyassociated with parents’ profession, educational level and receipt of previous information and advice from healthprofessionals.

RÉSUMÉ Les parents ont souvent des perceptions erronées concernant la fièvre chez l'enfant, et les informationssur la prise en charge des enfants fébriles à domicile sont rares au Maroc. Dans la présente étude sur lesperceptions, les connaissances et les pratiques des familles au sujet de la fièvre chez l'enfant, les parents de 264enfants fébriles âgés de 0 à 16 ans ont été interrogés au sein d'un service d'urgence pédiatrique de la ville deRabat en 2011. Seuls 3,5 % des parents connaissaient la température exacte définissant un état fébrile et 54,4 %déterminaient la fièvre de leur enfant à l'aide d'un thermomètre, de préférence par voie rectale. La plupartd'entre eux (96,8 %) considéraient que la fièvre était une affection très grave qui pouvait conduire à des effetssecondaires tels que des lésions cérébrales (28,9 %), des convulsions (18,8 %), une paralysie (19,5 %), une dyspnée(14,8 %) et un coma (14,8 %). Le paracétamol a été utilisé par 85,9 % des parents et les traitements traditionnelspar 45,1 %. La connaissance de la définition exacte de la fièvre était significativement associée à la profession desparents, à leur niveau d'études et à la prise de conseils et d'informations préalable auprès des professionnels desanté.