A Serendipitous Finding of Coagulation Factor VII Deficiency in Two Asymptomatic Patients.

Congenital Factor VII (FVII) deficiency is a rare autosomal recessive disorder with a prevalence of approximately 1:500,000. It plays a crucial role in initiating coagulation by activating Factors IX and X. Diagnosis typically involves prolonged prothrombin time (PT) and varies widely in clinical presentation. Management includes fresh frozen plasma (FFP), prothrombin complex concentrates (PCC), and recombinant activated FVII (rFVIIa), with rFVIIa often preferred due to its safety and efficacy. We present two pediatric cases: a five-year-old boy with a prolonged PT at 55% and FVII levels at 25.1%, and a two-year-old boy with a PT at 24% and FVII levels at 4.6%. Both cases highlight the importance of thorough hemostatic evaluation and tailored management strategies in FVII deficiency.

Portal Hypertension in Children: Investigating Umbilical Catheterization in the Neonatal Period.

Portal cavernoma is a major cause of extrahepatic portal hypertension (EHPH) in children. It is a serious condition, due to the frequency and severity of digestive hemorrhages secondary to the rupture of esophageal varices (EV). Neonatal umbilical catheterization is a significant risk factor for the development of portal vein thrombosis (PVT) and portal hypertension. We report a case of a five-year-old male who presented with upper gastrointestinal (GI) bleeding on ruptured esophageal varices resulting from a portal cavernoma, complicating neonatal umbilical vein catheterization. This case illustrates the risk of severe vascular complications, particularly portal hypertension that can result from neonatal umbilical vein catheterization.

Sanjad-Sakati Syndrome Revealed by Hypocalcemic Convulsions.

Sanjad-Sakati syndrome is an autosomal recessive disorder characterized by facial dysmorphia, growth retardation, and congenital hypoparathyroidism. Epidemiologically, this syndrome is primarily observed in children of Arabian descent. However, cases have also been reported in non-Arab countries. Although its exact prevalence is uncertain, the estimated incidence in Saudi Arabia ranges from one in 40,000 to one in 600,000 live births. We report a case of Sanjad-Sakati syndrome in a female infant, born to first-degree consanguineous parents, who presented with convulsive seizures since the age of four months. Laboratory findings indicated severe hypocalcemia and elevated phosphate levels, consistent with congenital hypoparathyroidism. The treatment involved calcium and vitamin D supplementation, which led to a marked improvement in the patient's condition. The objective of this clinical case is to highlight an uncommon cause of hypocalcemia and to describe certain clinical and endocrinological manifestations of Sanjad-Sakati syndrome, which is prevalent in the Arab population.

Vitamin B12 Deficiency in Infants With Axial Hypotonia and Psychomotor Regression: Insights From a Moroccan Case Study.

Vitamin B12 deficiency is a rare entity in the pediatric population. It is often of maternal origin in exclusively breast-fed infants. Its clinical manifestations are multiple and unspecific, encompassing hematological problems and neurodevelopmental consequences. Positive diagnosis and early treatment with vitamin B12 supplementation have a rapidly reversible effect on symptoms. Delayed diagnosis, however, may result in irreversible neurological sequelae. We report the case of a six-month-old infant, admitted with hypotonia and psychomotor regression since the age of four months. The laboratory work-up revealed macrocytic anemia with the presence of megakaryocytes and megaloblasts on the myelogram. Vitamin B12 levels were low, and homocysteine levels were high. A maternal workup showed vitamin B12 deficiency in the mother. A brain MRI showed bilateral frontoparietal cortical atrophy. The patient was put on vitamin B12 supplementation with good evolution. The aim of our work is to shed light on the misleading and varied clinical profile of vitamin B12 deficiency in an exclusively breastfed infant, the serious consequences of maternal vitamin B12 deficiency, and the importance of early diagnosis of this condition.

Primary Immunodeficiency-Type Ataxia-Telangiectasia Revealed by Splenic Abscesses.

Ataxia-telangiectasia (A-T) is a rare inherited autosomal recessive disease. It is associated with an alteration in the ATM gene, located on chromosome 11q22-23, which codes for a protein involved in a complex way in cell cycle regulation and cell protection. It is characterized by cerebellar ataxia, cutaneous and ocular telangiectasia, and an immune deficiency responsible for recurrent infections. Diagnosis is generally delayed due to the late onset of neurological symptoms and telangiectasia. People suffering from this condition are particularly sensitive to ionizing radiation, which considerably increases their risk of developing neoplasia. We report an observation of a primary immunodeficiency-type A-T revealed by recurrent fever and multiple splenic abscesses.

Clinical Profile of Hospitalized Varicella Cases From Pediatric Emergency Consultations: A Retrospective Cohort Study.

BACKGROUND: Varicella is a very common childhood infectious disease. It is generally benign, but it can lead to fatal complications. Our study aimed to describe the clinical and therapeutic profile of varicella based on consultations in the pediatric emergency department, to determine the incidence of hospitalized varicella cases in the pediatric department for complementary management, and to specify the incidence of varicella complications in hospitalized patients. MATERIALS AND METHODS: We conducted a retrospective descriptive cohort study over 12 months. It took place in the pediatrics and pediatric emergency departments of the Mother-Child Hospital of the Mohammed VI University Hospital, Mohammed I University, in Oujda, Morocco. RESULTS: We collected 120 cases of varicella. The mean age of patients was 4.5 years. The most common age range was 4-6 years (69%). Males predominated. The reason for consulting the pediatric emergency department was a febrile rash in 65% of cases. Treatment in pediatric emergencies was mostly symptomatic. Antibiotic treatment for superinfection of lesions was used in 11% of cases. The number of hospitalizations due to complicated and/or severe varicella was 17 cases. The median age was 6.3 years. Most of the children (82%) were immunodeficient and 18% were immunocompetent. Sixteen patients had underlying risk factors. Infectious skin and soft tissue complications were noted in most hospitalized patients (47%). They were mainly presented by cutaneous reinfections with alteration of general health (41%). Neurological complications ranked second (23%). The majority were febrile convulsions (17%). One case of bronchopulmonary complication was noted. No hematological, digestive, renal, or cardiac complications were noted. Intravenous antiviral treatment was used in 88% of hospitalized cases. The drug of choice was acyclovir. Antibiotic therapy was used in 53% of cases. No patient received corticosteroid therapy. The median length of hospitalization for our patients was 14 days. The evolution was favorable in 100% of cases. CONCLUSION: Varicella remains a benign disease in children, rarely leading to hospitalization. However, complications may develop in cases of comorbidity or children with risk factors. The introduction of the varicella vaccine into the national immunization program could considerably reduce the number of children hospitalized in the near future.

Celiac Disease and Autoimmune Diseases in a Pediatric Population in Morocco: A Cross-Sectional Study.

INTRODUCTION: Celiac disease (CD) is defined as an autoimmune disease (AD) caused by gluten ingestion in genetically sensitive individuals. Several publications have demonstrated the increased risk of AD in patients with CD, both adults and children, which requires systematic research. Our study aimed to determine the prevalence of AD in 60 patients diagnosed with CD and to highlight risk factors that may contribute to the emergence of AD. MATERIALS AND METHODS: We collected medical data from all CD patients under 16 years of age who also had AD. Our study was conducted in the Gastroenterology-Hepatology and Pediatric Nutrition Unit of the Pediatrics Department of the Mohamed VI Hospital and University Center in Oujda, Morocco, during a seven-year period between January 2017 and January 2024. RESULTS: We studied 60 patients with CD in our study. Eight patients (13%) had an associated AD. Their average age was eight years, with extremes varying between two and 15 years. AD was diagnosed before CD in six cases (75%), in parallel with CD in one patient (12.5%), while in only one case, it was diagnosed after CD (12.5%). All our patients had a single AD associated with CD. These ADs were mainly type 1 diabetes in seven cases and autoimmune thyroiditis in only one case. All our patients followed a gluten-free diet in addition to specific treatment for associated AD. Nevertheless, despite regular medical follow-up and targeted dietary advice for the management of CD and associated AD, three patients encountered difficulties in following the recommended diet. CONCLUSION: Younger patients with CD have an increased risk of hypothyroidism and insulin-dependent diabetes. These data necessitate improved surveillance to discover these illnesses as early as possible in order to optimize management and reduce related consequences.

Exploring the Link Between Profuse Warts and Hyper-Immunoglobulin E (IgE) Syndrome: A Pediatric Case Report.

The relationship between warts and hyper-immunoglobulin E (IgE) syndrome lies in the fact that patients with this syndrome may have recurrent or persistent skin warts because of their immune dysfunction. Therefore, it is important to consider this possibility when evaluating a patient with skin warts, especially if they are associated with other symptoms such as recurrent infections or pulmonary issues. Warts can thus be an important clinical sign indicating the presence of this syndrome. We report the case of a young girl presenting with numerous warts accompanied by pulmonary involvement and weight delay, in whom the diagnosis of hyper IgE syndrome was established.

Hematemesis in Children: A Potential Sign of Parental Psychiatric Disorder.

Munchausen's syndrome by proxy (MSBP) is a rare form of abuse characterized by the fabrication or induction of symptoms of illness in a child by a close relative, typically a parent, leading to multiple consultations and varying degrees of invasive medical interventions. Various clinical presentations are described in the literature, ranging from organic manifestations to psychiatric expressions. This syndrome remains a challenging diagnosis to make and requires increased awareness among healthcare professionals. Prompt recognition is key to preventing potential long-term comorbidities and even fatalities. Here, we are reporting two cases of MSBP manifested by bleeding, with the perpetrator being the mother.

When Salmonella Manifests Through the Skin: Erythema Nodosum in a Pediatric Case.

Erythema nodosum (EN) is a non-specific nodular dermo-hypodermic rash characterized by the sudden occurrence of painful lumps located especially in the legs following a non-specific reaction to different internal and external antigens. Clinical and histological manifestations are stereotyped, regardless of the etiology. Erythema nodosum is most frequently associated with infections, particularly bacterial and less commonly viral, fungal, and parasitic. Other conditions can be discussed, including systemic diseases, malignant tumors, medicines, and vaccines. In almost half of cases, erythema nodosum is idiopathic if no cause is found. We report a case of erythema nodosum secondary to a Salmonella infection in a seven-year-old male. The peculiarity of our observation is the initial presentation of systemic signs that preceded the gastrointestinal symptoms.

Albinism and Primary Immunodeficiency in Infants: A Case Study of Griscelli Syndrome.

Griscelli syndrome (GS) type II is a rare hereditary disorder characterized by partial albinism, immunodeficiency, and the subsequent development of hemophagocytic syndrome (HPS). Herein, we present a case involving a four-month-old infant admitted to our facility due to a prolonged fever complicated by HPS. The diagnosis of GS type 2 was established based on a constellation of clinical and laboratory findings: consanguinity, familial history of early infectious fatalities, ocular-cutaneous hypopigmentation, characteristic silvery hair sheen, onset of HPS, and notably, the pathognomonic appearance upon microscopic examination of a hair sample. The absence of giant granules within nucleated cells helped exclude Chediak-Higashi syndrome.

Ophthalmic Shingles in Children: A Rare Form and a Danger to Be Aware of.

Shingles in children is rare, especially the ophthalmic form. The occurrence of shingles in children is exceptional but often benign, requiring only symptomatic treatment. Antivirals are exceptionally used for complicated forms, which are mainly seen in immunocompromised individuals or in ophthalmic locations that can lead to severe ocular complications. Various studies agree on the benign nature of this condition in immunocompetent children and an excellent prognosis. We report a case of ophthalmic shingles in an immunocompetent child aged two years and seven months. The purpose of this clinical case was to emphasize the importance of early antiviral treatment to limit corneal involvement and preserve visual function.

Compartment Syndrome: An Uncommon Twist in Childhood Henoch-Schönlein Purpura.

Henoch-Schönlein purpura (HSP) also known as rheumatoid purpura is the most common vasculitis in children. This condition affects small blood vessels, predominantly targeting the skin, digestive system, joints, and kidneys. Short-term prognosis mainly depends on abdominal complications, while long-term prognosis is mainly determined by the severity of kidney involvement, which occurs in about 35% of cases. Although uncommon, other organs such as the lungs, heart, or nervous system may also be affected. Compartment syndrome of the hand and forearm is a very rare complication of HSP. To our knowledge, only two cases have been reported in the literature. We describe the case of a four-year-old child who presented with rheumatoid purpura complicated by compartment syndrome of the hand and forearm successfully managed through emergency fasciotomy.

Pediatric Limb Asymmetry: A Unique Presentation of Angioosteohypertrophic Syndrome.

Parks-Weber syndrome (PWS), also known as Klippel-Trenaunay-Weber syndrome, is a rare congenital bone vascular syndrome first described in 1900. It is characterized by arteriovenous malformations in a limb, leading to disproportionate limb growth and potential heart failure. Unlike Klippel-Trenaunay syndrome, PWS manifests arteriovenous malformations with abnormal connections between the arteries and veins of the affected limb. The management of this syndrome, similar to that of Klippel-Trenaunay syndrome, relies mainly on symptomatic treatment. We report the first case of angioosteohypertrophic syndrome diagnosed at CHU Med VI Oujda, in a patient aged seven years and eight months. This syndrome manifested primarily in the right upper limb, characterized by asymmetry in both upper limbs, thermal disparity, a cutaneous nevus, and venous ectasia in the right arm. The diagnosis was further substantiated through arteriography, confirming the presence of an arteriovenous fistula.

Acute Myocardial Infarction in a Seven-Year-Old Child With Type 1 Diabetes: A Rare Case Report.

Myocardial infarction (MI) is extremely rare in children and can have different etiologies, including congenital heart defects and Kawasaki disease. Cardiovascular disease (CVD) is the primary cause of death in patients with type 1 diabetes (T1D). Effective management of risk factors like blood pressure, cholesterol, and blood sugar levels is essential for individuals with T1D to mitigate the risk of cardiovascular complications, including MI. We present the case of a seven-year-old child diagnosed with type 1 diabetes one month before this admission, without any other notable medical history, who was admitted to the pediatric emergency department due to chest pain. The symptoms had begun two hours prior to admission. Upon arrival, the patient reported severe and persistent retrosternal constrictive chest pain radiating to the left arm without other associated signs, with a strictly normal clinical examination. An electrocardiogram (ECG) revealed typical ST segment elevation in inferior leads (II, III, and aVF) with reciprocal changes in V1 to V4. Troponin level was elevated at 7254 ng/l. Echocardiography revealed mild dilation of the left coronary artery (4 mm) and the right coronary artery (3 mm), while other radiological and laboratory investigations showed no abnormalities. The patient responded well to treatment with acetylsalicylic acid, clopidogrel, and heparin, resulting in a favorable outcome.

Wilson Disease in Children in the Eastern Region of Morocco: Analysis of 24 Cases.

Wilson's disease (WD), or "hepato-lenticular degeneration," is a rare genetic disorder of autosomal recessive inheritance causing toxic tissue accumulation of copper, mainly in the liver, brain, and cornea. Its phenotypic and genotypic heterogeneity characterizes it. This study aimed to clarify the clinical features and spectrum of Wilson's disease in children from the eastern region of Morocco and to study the evolutionary profile and survival in this population while discussing and highlighting the various diagnostic and therapeutic difficulties encountered in the management of WD in our context. This retrospective study encompassed 24 children diagnosed with Wilson's disease, selected from the gastroenterology-hepatology and pediatric nutrition units at Mohamed VI University Hospital in Oujda, Morocco, over a span of nine years, from January 2015 to November 2023. Our series results show 14 boys and 10 girls; the median age of discovery was 11 years, with extremes ranging from 18 months to 15 years. The consanguinity was found in 13 patients. Clinically, the edemato-ascitic syndrome was noted in 14 patients with an alteration of the general state; icterus was found in 13 patients; signs of portal hypertension were present in six patients; and neurological signs in seven cases. Skin manifestations occurred in three cases, and arthralgia in three cases. Six children were diagnosed on the occasion of a family screening. Biologically, hepatic cytolysis was found in 20 patients, with signs of hepatocellular failure in 15 cases. Hemolytic anemia was present in nine patients. Ceruloplasminemia was decreased in 21 patients and cupremia in 19 patients. Cupruria was increased in 22 cases. The Kayser-Fleicher ring was found in 10 cases. Abdominal ultrasound showed ascites in 16 patients, hepatomegaly in 1, splenomegaly in two cases, hepatosplenomegaly in five cases, and cirrhosis in two. MRI showed signal abnormalities in 11 patients. Therapeutically, D-penicillamine was initially introduced in 18 patients and zinc acetate in 6 patients. The evolution was favorable for 15 patients still followed up in the department. Three patients died of hepatocellular failure, and two died of hepatic encephalopathy. Four patients were lost to follow-up.

Prevalence and Determinants of the Use of Antibiotics by Self-Medication in the Pediatric Population in Oujda, Morocco.

OBJECTIVE: Antibiotic resistance driven by antibiotic self-medication and inappropriate use of antibiotics is a growing global health threat. Our study aimed to describe parents' self-medication practices with antibiotics, determine the factors favoring their use in the pediatric population, and assess parents' knowledge of the role of antibiotics and antimicrobial resistance. MATERIALS AND METHODS: We conducted a cross-sectional study over two months (September and October 2023). Data collection was performed using a questionnaire-guided interview. We included 460 parents of children consulting or hospitalized in the Pediatric Department of the University Hospital Mohammed VI in Oujda, Morocco. RESULTS: A total of 62% of parents questioned were mothers. Self-medication with antibiotics was noted in 313 families (68%). Parents used antibiotics mainly to treat cough (43%) and fever (24%). Betalactams were the antibiotic class most used by parents (72%). Information on dosage and methods of antibiotic administration was provided by pharmacy staff but was not respected by most parents (78%). Efficacy of treatment (58%), saving consultation costs and time (47%), and the ease of obtaining treatment without a prescription (42%) were reported in the majority of cases as reasons for using antibiotics by self-medication. In our context, the probability of self-medicating with antibiotics in the pediatric population is increased by female gender (OR=1.04), low level of education (OR=1.02), low socio-economic status (OR=1.09), and buying antibiotics without a prescription (OR=1.22). CONCLUSION:  Although antibiotic self-medication in children is a worldwide phenomenon, influenced by several geographical, cultural, and economic factors, there is an urgent need to promote a global health strategy.

Exceptional case of a hemangioma-like rabdomyosarcoma in the hand's palm.

Rhabdomyosarcoma is a malignant tumor in children that might mimic a benign tumor, such as infantile hemangioma, particularly when detected early. Although rhabdomyosarcoma rarely occurs in the hand, its prognosis is generally poor, and successful treatment relies on a complete and radical surgical excision. We present a case of rhabdomyosarcoma located in the palm of an infant's hand, initially presenting clinical and radiological features suggestive of a vascular tumor. The resection of this mass was radical, and histological analysis and immunohistochemistry returned in favor of embryonic rhabdomyosarcoma. In similar cases recorded in the literature, the diagnosis may be first mistaken for that of a hemangioma, then confirmed by histology. This underlines the importance of a systematic anatomopathological examination of all tissues removed surgically.

A Case Report of an Uncommon Association of Total Intracardiac Anomalous Pulmonary Venous Return and Tetralogy of Fallot.

Total anomalous pulmonary venous return (TAPVR) represents a group of anomalies consisting of a lack of connection between the pulmonary veins and the left atrium. All oxygenated pulmonary venous return flows directly or indirectly into the right atrium. Survival is only possible with a right-to-left atrial shunt. It remains rare, accounting for less than 1% of all congenital heart diseases. Its association with tetralogy of Fallot is much rarer and has been documented in medical literature as isolated cases. Early prenatal diagnosis, rapid surgical repair, and optimal postoperative resuscitation appear to be the best guarantee of a favorable outcome following total repair of a pulmonary venous connection anomaly. Non-obstructed forms present as high-flow shunts with moderate cyanosis. The symptomatology of blocked forms is dominated by the obstruction to venous return; a clinical picture of respiratory distress with intense cyanosis and severe pulmonary arterial hypertension develops from the first days of life. Echocardiography is fundamental in diagnosing TAPVR. If the results are inconclusive, magnetic resonance imaging and computed tomography are appropriate alternatives for establishing a complete and accurate diagnosis. We report a case of a two-month and 22-day-old infant who is a product of a twin pregnancy, presenting with a non-obstructed TAPVR associated with tetralogy of Fallot, and his twin who died on day 20 of life, likely due to a complex cyanotic congenital heart disease.

A Fatal Case Report of Chenopodium ambrosioides L. (M'khinza) Intoxication.

Chenopodium ambrosioides L. is a plant belonging to the Chenopodiaceae family, known for its use in traditional medicine as a remedy for its antiseptic, analgesic, antipyretic, antispasmodic, and anti-inflammatory properties. It is used for its therapeutic properties internally as an infusion or externally as a vermifuge. Its use can be responsible for serious, even fatal, side effects and intoxications, particularly in infants and children. These may include neurological, digestive, hepatic, or renal complications. We present a case of Chenopodium ambrosioides L. intoxication in a four-year-old girl, resulting from repeated high-dose infusions of this plant for antipyretic purposes. She was admitted to the pediatric emergency department for management of a disorder of consciousness four hours after ingesting Chenopodium ambrosioides L. to treat acute fever.