Open Access Case Study

Congenital Deficiency in Factor VII Revealed by Menorrhagia: Case Report

Nadia Mebrouk, Abdelilah Radi, Mohamed Selouti, Amal Hassani, Abdelhakim Ourrai, Mohamed Kmari, Rachid Abilkassem, Aomar Agadr

Asian Journal of Pediatric Research, Page 1-5
DOI: 10.9734/ajpr/2020/v4i130138

Factor VII (FVII) deficiency is the most common among rare inherited autosomal recessive bleeding disorders. It is a multifaceted disease because of the lack of a direct correlation between plasma levels of coagulation FVII and bleeding manifestations. Clinical phenotypes range from asymptomatic condition—even in homozygous subjects—to severe, life-threatening bleedings (e.g., central nervous system and gastrointestinal bleeding). Menorrhagia is a frequent type of bleeding in FVII deficiency, with a prevalence rate of two in three women aged 10 to 50 years and with a peak prevalence in teenagers. When menorrhagia is observed and once the gynecological causes are excluded, it is important to carry out a hemostasis assessment because, if an anomaly is found, specific treatment can be administered and preventive measures taken. Basic diagnostic work-up includes routine assays, prothrombin level, activated partial thromboplastin time and platelet count, followed by FVII coagulant activity measurement for isolated decreased prothrombin level. To confirm the diagnosis, FVII assay should be repeated at least once. Several treatment options are currently available for FVII deficiency: Recombinant activated Factor VII (rFVIIa), plasma-derived Factor VII, fresh frozen plasma and prothrombin complex concentrates. rFVIIa is the most used replacement therapy. Other medical therapies of menorrhagia includes hemostatic agents and hormonal treatments (combined oral contraceptives, levonorgestrel intrauterine devices), in combination or not with rFVIIa.

We report the case of a fourteen-and-a-half-year-old girl who presented menorrhagia of great abundance at the age of thirteen, the exploration of which revealed a congenital deficit in FVII.

Open Access Case Study

Congenital Isolated Folic Acid Malabsorption: Case Report

Nadia Mebrouk, Rachid Abilkassem, Mohamed Kmari, Amal Hassani, Abdelhakim Ourrai, Aomar Agadr

Asian Journal of Pediatric Research, Page 6-10
DOI: 10.9734/ajpr/2020/v4i130139

We report the case of a female child with congenital isolated malabsorption of folic acid. The patient was referred to our hospital for pancytopenia and a tendency to various infections, but with no neurological disturbances. A bone marrow aspiration demonstrated megalobastic anemia and serum folic acid was low. An immunodeficiency test was therefore performed, which revealed impairment of both cellular and humoral immunity. The defect persisted after considerable doses of folate were administered orally to the patient. However, parenteral administration of folic acid removed pancytopenia, and restored normal levels of T CD8+, NK, B and immunoglobulins. Our patient, responded only to parenteral folate and her immunologic recovery after parenteral folate repletion was dramatic and provides strong in vivo evidence of the importance of folates for lymphocyte function in humans. Our patient’s history shows that while it is easy to reverse the systemic consequences of folate deficiency, patients must not stop taking their folate supplementation because they believe that their disorder disappeared.

Open Access Minireview Article

Physical and Emotional Repercussions of Pregnancy during Adolescence

Luiz Antonio Del Ciampo, Ieda Regina Lopes Del Ciampo

Asian Journal of Pediatric Research, Page 17-22
DOI: 10.9734/ajpr/2020/v4i130141

Adolescence is a phase marked by physical, emotional and, social changes culminating with sexual development and final adult height, which occur at different stages during the second decade of life. Due to their characteristics of immaturity, impulsiveness, and tendencies to take risky behaviors with sexual activity starting earlier, adolescents are subject to becoming pregnant during the first years after menarche. This article presents some characteristics of the adolescent's growth and development and the repercussions of pregnancy on her physical growth and emotional maturity.

Open Access Original Research Article

Interest of Hyperbaric Oxygen Therapy in Childhood Carbon Monoxide Poisoning: A Retrospective Study

Zakaria Iloughmane, Mohamed Chemsi, Ahmed Kaddouri, Abdelilah Radi, Fatime Zahra Argubi, Mohamed Sellouti, Rachid Abilkassem, Amale Hassani, Aomar Agadr

Asian Journal of Pediatric Research, Page 11-16
DOI: 10.9734/ajpr/2020/v4i130140

Carbon monoxide poisoning is one of the common causes of poisoning in patients. However, few studies have focused on the pediatric group. We performed this study in order to clarify the clinical characteristics of pediatric patients with Carbon monoxidepoisoning.

Methods: We retrospectively reviewed the medical records of pediatric patients (age < 14 years)  diagnosed with CO intoxication and  admited in the Hyperbaric Oxygen chamber of the military hospital MOHAMED V of RABAT between January 2018 and March 2020, epidemiologic and clinical data were collected, analyzed  and compared with  literature.

Results: A total of 112 children with CO poisoning were identified. Their ages ranged from one to 14 years, symptoms were nausea and vomiting (31%), headache (23%), altred consciousness (19%), dizziness (15%) and seizure (8%).  Most events (67.8%) occurred during winter, (100%) of cases were recorded as accidental poisoning. Only one child developed delayed neurologic sequelae, with no death enregistred in our series.

The most common cause of intoxication was improperly vented exhaust produced by gas, hot water heaters (98%).(And) (20%) of patients needed two or more sessions of Hyperbaric Oxygen therapy.

Conclusion: The treatment of Carbon monoxide poisoning is a race against time. Patients requiringHyperbaric Oxygen therapy must be carefully selected within the first few hours, and kept under close observation. Children with Carbon monoxide poisoning had good outcomes in this series.

Open Access Original Research Article

Children with Myocarditis: Clinical Profile and Electrocardiographic Changes and Their Prognostic Significance: A Prospective Observational Study

Jayendra R. Gohil, Tushar S. Agarwal

Asian Journal of Pediatric Research, Page 23-30
DOI: 10.9734/ajpr/2020/v4i130142

Aims and Objective: To study the clinical profile and electrocardiographic changes in children with myocarditis and their prognostic significance.

Methods: 223 children presented with signs and symptoms of myocarditis from June 2016 to May 2017. Amongst them, 21 children with congenital heart disease or rheumatic heart disease and 166 children with negative cardiac markers were excluded. The remaining 36 patients with myocarditis and elevated levels of both SGOT and CKMB were studied. The patient outcome was recorded as expired or discharged. Data were analyzed using the chi-square test.

Results: Majority (13; 36%) were infants. Post infancy, there was a uniform age distribution of cases. Myocarditis was commonly seen in association with culture-negative (probably viral) pneumonia, and diphtheria. Bradycardia and A-V block, although seen less frequently, were having a significant association with mortality. Congestive cardiac failure (28.6%) and cardiomegaly (25.0%) were not significantly associated with mortality. ECG changes like Sinus tachycardia and T wave inversion (most common) and ST elevation, Q waves and low amplitude (less common) were insignificantly associated with mortality.  

Conclusion: In children, myocarditis should be suspected especially in infants with unexplained breathlessness or fatigue, arrhythmia, or signs of acute cardiac decompensation. It was seen more with bacterial-culture-negative (viral) pneumonia and diphtheria. Continuous ECG monitoring and chest X-ray should be done. Congestive cardiac failure and cardiomegaly, though observed in a quarter of patients were not significantly associated with mortality. Bradycardia and A-V block have a poor prognosis and cardiac pacing should be considered. Echocardiography should be available in-house.