Navigating Hemostasis of Bleeding Among Children With ß-Thalassemia.

Bleeding phenotype is reported in ß-thalassemia patients. However, the underlying etiology remains elusive. We aimed to assess coagulation profile and the platelet aggregation in ß-thalassemia children with bleeding diathesis. Fifty ß-thalassemia children with a positive bleeding history were recruited. Bleeding phenotype was explored through full history taking and thorough clinical examination. Complete blood count, prothrombin time, international normalized ratio, and platelets aggregometry were performed for children with negative workup. Mucosal bleeding was manifest among most of our patients (96%). Two-third of patients had decreased aggregation with ristocetin (68%), adenine di-phosphate (64%), and arachidonic acid (64%). While half of the patients (48%) had deficient response to epinephrine. Collagen, ristocetin, and arachidonic acid induced aggregation were negatively correlated to frequency of blood transfusion (P=0.021, r=-0.325; P<0.001, r=-0.465; P=0.018, r=-0.333, respectively). Aggregation to collagen and epinephrine demonstrated a negative correlation with age (P=0.04, r=-0.287; P=0.03, r=-0.315). Deferiprone was associated with a deficient response to ristocetin and collagen when compared with deferasirox or no chelation (P=0.021 and 0.006, respectively). Impaired ristocetin response was linked to hydroxyurea (P=0.035). Platelets function defect should be considered in ß-thalassemia patients with bleeding symptoms.

Bilateral hydroureters and hydronephrosis in a neonate.

: A newborn boy was diagnosed antenatally with bilateral hydronephrosis. Postnatal renal ultrasound scan (USS) measured a renal pelvic anteroposterior diameter (APD) of 12 mm on the left side and 7 mm on the right side. The baby had good urine stream. Parents missed the repeat USS at the age of 1 week. An ultrasound done at 4 weeks revealed progressive hydronephrosis, bilateral hydroureters, with increased renal echogenicity. Normal bladder wall thickness was noted but two intravesical lesions were seen (figures 1 and 2). The APD was 13.5 and 11 mm on the left and right side, respectively.edpract;103/1/20/EDPRACT2016311091F1F1EDPRACT2016311091F1Figure 1Renal ultrasound scan of (A) left kidney (LT) and (B) right kidney (RT) showing bilateral hydronephrosis (white arrows) and hydroureters (red arrow). Increased renal echogenicity is not shown in the figure.edpract;103/1/20/EDPRACT2016311091F2F2EDPRACT2016311091F2Figure 2Showing normal bladder wall thickness but two intravesical lesions were seen (white arrows). QUESTION: How would you describe the intravesical lesions in figure 2? Bilateral ureterocelesBilateral vesicoureteral reflux (VUR)Bilateral pelvi-ureteric junction obstructionPosterior urethral valves (PUVs)Which complication(s) may you expect in such cases? Urinary tract infection (UTI)Obstructive voiding symptomsFailure to thriveUreteral calculusAll of the aboveHow would you treat this problem? Endoscopic punctureDeflux surgeryPyeloplastyVesicostomy Answers are on page ▪▪▪.

Amlodipine: The double edged sword.

AIM: Amlodipine, a calcium channel blocker became one of the most popular antihypertensive medications used in the paediatric population. This is mainly due to its long half-life making it easy to use as a single daily dose. Amlodipine is associated with common side effects like flushing, headache and dizziness. The incidence of amlodipine-related oedema was noted to be lower in the paediatric population compared to adults. We emphasise the importance of monitoring the development of oedema in patients treated with amlodipine. METHODS: Hereby, we report four paediatric cases with amlodipine-related generalised oedema. CONCLUSION: Resolution of the oedema with stoppage of amlodipine confirms its culprit as a cause of the oedema. To our knowledge, this is the largest case series of amlodipine-related generalised oedema in the paediatric population.

A child with phenylketonuria and focal segmental glomerulosclerosis, the bright side of proteinuria.

Phenylketonuria (PKU) is the most common inborn error of amino acid metabolism. Phenylalanine hydroxylase is the underlying deficient enzyme. If left untreated, growth failure, microcephaly, global developmental delay, seizures and severe intellectual impairment would characterize the clinical picture of PKU. On the other side of protein homeostasis lies nephrotic syndrome. It is a well-known quantitative defect due to significant proteinuria. Focal segmental glomerulosclerosis (FSGS) is a special congenital variant affecting children and adults. Hereby, we describe a three- year old male child who presented with generalized edema and global developmental delay. Investigations revealed PKU along with FSGS. We assume that congenital nephrosis ameliorated the picture of PKU, and had a salutary effect on the growth and development. Such coexistence between PKU and FSGS hasn't been described before.

Echocardiography in PICU: when the heart sees what is invisible to the eye / Ecocardiografia na UTIP: quando o coração vê o que é invisível aos olhos

ABSTRACT OBJECTIVE: Echocardiography has become an indispensable bedside diagnostic tool in the realm of pediatric intensive care units (PICU). It has proven to be an influential factor in the formula of clinical decision-making. This study aimed to delineate the impact of echocardiography on the management of critically ill pediatric patients in the PICU at Sultan Qaboos University Hospital, Oman. METHOD: This was a retrospective cohort study conducted in a five-bed PICU. Patients admitted to the PICU from January of 2011 to December of 2012 were reviewed. Those who have undergone bedside echocardiography during their ICU stay were recruited. Electronic patient record was used as data source. RESULTS: Over a-24-month period, 424 patients were admitted in this PICU. One hundred and one clinically indicated transthoracic echocardiograms were performed. 81.8% of these presented new findings (n = 82) that significantly impacted the clinical decision of patient management, namely, alteration in drug therapy and procedure, whereas no difference in the management was yielded in the remaining 17.8% of the studied cases. CONCLUSIONS: Echocardiography had a significant impact on the management of PICU patients. Such salutary effect was consequently reflected on the outcome. Pediatric intensivists are encouraged to acquire such bedside skill.

RESUMO OBJETIVO: A ecocardiografia se tornou uma ferramenta de diagnóstico relevante, indispensável no âmbito das unidades de terapia intensiva pediátrica (UTIP). Ela se tornou um fator influente na tomada de decisões clínicas. O objetivo deste estudo foi delinear o impacto da ecocardiografia sobre o manejo de pacientes pediátricos gravemente doentes na UTIP do Hospital Universitário Sultan Qaboos, em Omã. MÉTODO: Este é um estudo de coorte retrospectivo feito em uma UTIP de cinco leitos. Foram analisados pacientes internados na UTIP entre janeiro de 2011 e dezembro de 2012. Foram recrutados os pacientes que passaram por ecocardiografia durante sua internação na UTI. O registro eletrônico dos pacientes foi usado como fonte de dados. RESULTADOS: Em 24 meses, 424 pacientes foram internados em nossa UTIP, 101 pacientes foram encaminhados para ecocardiografias transtorácicas, 81,8% deles tiveram novos achados (n = 82) que afetaram significativamente a decisão clínica, como a terapia medicamentosa e os procedimentos, ao passo que não houve diferença no manejo nos outros 17,8% dos casos estudados. CONCLUSÕES: A ecocardiografia teve um impacto significativo sobre o manejo de pacientes da UTIP. Esse efeito refletiu-se nos resultados. Os intensivistas pediátricos devem ser incentivados a adquirir essa habilidade relevante.

Echocardiography in PICU: when the heart sees what is invisible to the eye.

OBJECTIVE: Echocardiography has become an indispensable bedside diagnostic tool in the realm of pediatric intensive care units (PICU). It has proven to be an influential factor in the formula of clinical decision-making. This study aimed to delineate the impact of echocardiography on the management of critically ill pediatric patients in the PICU at Sultan Qaboos University Hospital, Oman. METHOD: This was a retrospective cohort study conducted in a five-bed PICU. Patients admitted to the PICU from January of 2011 to December of 2012 were reviewed. Those who have undergone bedside echocardiography during their ICU stay were recruited. Electronic patient record was used as data source. RESULTS: Over a-24-month period, 424 patients were admitted in this PICU. One hundred and one clinically indicated transthoracic echocardiograms were performed. 81.8% of these presented new findings (n=82) that significantly impacted the clinical decision of patient management, namely, alteration in drug therapy and procedure, whereas no difference in the management was yielded in the remaining 17.8% of the studied cases. CONCLUSIONS: Echocardiography had a significant impact on the management of PICU patients. Such salutary effect was consequently reflected on the outcome. Pediatric intensivists are encouraged to acquire such bedside skill.

Hepatoblastomas in Oman: Unveiling success.

OBJECTIVES: Primary malignant liver tumours account for more than 1% of all paediatric malignancies, with the most common form being hepatoblastomas (HB). Such malignancies among Arab populations have rarely been addressed in the literature. Using data from Oman's sole national referral centre for childhood solid malignancies, this study aimed to present the nationwide Omani experience with HB over the past 21 years. METHODS: This retrospective study reviewed the medical records of all children with HB who were managed in the Royal Hospital, Muscat, Oman, between January 1991 and April 2012. Clinical, radiological and laboratory characteristics were examined as well as treatments and outcomes. RESULTS: During the study period, 15 patients with HB were treated. Of these, 10 have survived to date. Nine of the survivors were no longer receiving treatment and one patient still had the disease but was in a stable condition. Of the remaining five patients, three did not survive and two were lost to follow-up. The survival rate among patients who completed therapy was 91%. CONCLUSION: HB has an excellent prognosis in Oman. The main obstacle to improving outcomes among Omani patients is non-compliance with therapy.

Hypertrophic Obstructive Cardiomyopathy in An Infant With Hemophagocytic Lymphohistiocytosis; Answer to a Riddle.

Familial hemophagocytic lymphohistiocytosis (FHLH) is a hereditary hyperinflammatory condition with T-cell and macrophage activation. Treatment consists of immunosuppressive therapy plus bone marrow transplantation. Cardiac manifestations of FHLH were scarcely mentioned in the literature with conflicting pathophysiological explanations. We report a case of hypertrophic obstructive cardiomyopathy associated with FHLH. Guided by such a case, a clear vision regarding the real cause is thought to be obtained in the cloudy landscape of pathophysiology.

Renal mass: is it always worrisome?

SUMMARY: 10-month male with large abdominal mass.

Limitations of Living Donor Liver Transplantation in Egyptian Children.

BACKGROUND/AIMS: In Egypt, the liver transplantation (LTx) program that became available since 2001 is a living donor program. We aimed to assess the obstacles to pediatric LTx. METHODS: Over a six-month-period, 41 pediatric patients were indicated for LTx; their ages ranged between 1.5 months to 17 years. Patients and potential donors were evaluated according to the program protocol. RESULTS: The obstacles for performing LTx were classified into recipient, donor and program obstacles or limitations. Each patient may have more than one limitation. Late presentation and co-morbid conditions were on the top of the recipient list of obstacles. Refusal of potential donors to donate was the commonest limitation on the donor side (33%). The commonest program limitations were young age and small size of the recipient. CONCLUSIONS: Limitations in recipient characteristics as well as donor shortage are still the main obstacles for living donor liver transplantation (LDLT) in our pediatric liver disease patients. Small weight and young age of potential LDLT candidates are the principle causes for delaying this life saving procedure. Increasing community awareness about living organ donation and nutritional support for end stage liver disease (ESLD) babies is pivotal, given our limitation to a living donor program.

Brain is not always the last fortress; osteosarcoma with large brain metastasis.

Osteosarcomas are the most common malignant primary bone tumors in children and adolescents. Brain metastases of osteosarcoma are very rare and carry a dismal prognosis. We report a case of chondroblastic osteosarcoma of right humerus presented with right frontal lobe metastasis in a 10-year-old girl with small pulmonary lesions.

Postvaricella thrombosis-report of two cases and literature review.

Varicella (chickenpox) is a common disease of childhood, caused by varicella-zoster virus. Postviral thromboembolism is a rare complication of varicella-zoster virus in childhood. We describe 2 children who developed lower limb deep venous thrombosis shortly after varicella infection, along with a review of 130 previously reported cases.

Knowledge, misconceptions, and future intentions towards breastfeeding among female university students in Kuwait.

A cross-sectional study using a self-administered questionnaire was conducted on female university students (N = 1106) to explore their knowledge and misconceptions on breastfeeding. Most participants recognized the benefits of breastfeeding, but only a few were aware of the recommendation for exclusive breastfeeding in the first 6 months of life. Misconceptions were common; 66%, 60%, and 55% of participants thought mothers should temporarily stop breastfeeding if they had a fever, skin rash, or sore throat, respectively. Approximately 20% thought mothers should stop breastfeeding if the child had diarrhea, vomiting, or skin rash. Support of breastfeeding in public places was low, but 38% supported breastfeeding in female prayer rooms in public places. Efforts should be made to correct common misconceptions on breastfeeding and increase the support of breastfeeding in public places among university students. Female prayer rooms that exist in all public places in Kuwait can be used to promote breastfeeding in public places in Kuwait.