Open Access Letter to the Editor

Open Access Case Study

Bartter Syndrome in Children; A Cause of Severe Hypokalemic Metabolic Alkalosis: Clinical Case Report and Literature Review

A. Radi, M. Akhrif, M. Kmari, A. Ourrai, A. Hassani, R. Abilkassem, A. Agadr

Asian Journal of Pediatric Research, Page 1-7
DOI: 10.9734/ajpr/2020/v4i430153

Bartter syndrome is an inherited renal tubular disorder caused by a defective salt reabsorption in the thick ascending limb of loop of Henle. It characterized by urinary loss of sodium, potassium, and chloride; hypokalemic metabolic alkalosis; normal blood pressure, high plasma levels of renin and aldosterone. There is phenotypical and genetic variability of Bartter syndrome since were identified five genes responsible for five different forms of Bartter syndrome. The objective of this work is to report a clinical case to study the pathophysiological, clinical, biological and therapeutic features of this syndrome.

Materials and Methods: We reported a case of 04-month-old male infant admitted for acute dehydration secondary to polyuro-polydipsia syndrome and vomiting. In clinical presentation the patient had a dysmorphic syndrome with triangular face, protruding ears and flattened nasal root. Laboratory tests revealed hypokalemia, hyponatremia, metabolic alkalosis and hypercalciuria. Treatment with indomethacin was started at 1 mg/kg per day with favorable outcome.

Open Access Case Study

An Extremely Rare Syndromic Form of Intellectual Disability: Temtamy Syndrome; About a Clinical Case

M. Akhrif, R. Abilkassem, A. Elyajouri, H. Ennouali, N. Amsiguine, A. Agadr

Asian Journal of Pediatric Research, Page 8-13
DOI: 10.9734/ajpr/2020/v4i430154

Temtamy syndrome is a congenital syndrome. It was first described by Temtamy et al. in 1991. Characterized by mental retardation, ocular coloboma, seizures, variable craniofacial dysmorphism, and brain abnormalities, including abnormalities of the corpus callosum and thalamus. The extreme clinical and genetic heterogeneity of these phenotypes posed a major diagnostic challenge until the advent of genomic tests that scan a large number of genes with little bias by the clinical phenotype.

Materials and Methods:  We reporting the case of a male child aged followed since the age of 3 months for epileptic seizure with corpus callosum agenesis, and clinical examination found craniofacial dysmorphia, mental retardation, strong myopia, an irian coloboma, chorioretinal atrophy. The aim of this study is to highlighting the specific features of temtamy syndrome and show the points of divergence with other similar syndromes.

Open Access Case Study

Status Dystonicus in Children with Secondary Dystonia: Reporting 3 Cases of Cerebral Palsy, Leigh Disease and Molybodynum Co Factor Deficiency

Kanij Fatema, Md Mizanur Rahman, Shaheen Akhter

Asian Journal of Pediatric Research, Page 17-23
DOI: 10.9734/ajpr/2020/v4i430156

Objective: Patients with primary and secondary dystonic syndromes occasionally develop severe episodes of generalized dystonia and rigidity which is known as status dystonicus (SD) or dystonic storm. This is a frightening hyperkinetic movement disorder and it is an emergency. Marked,                rapid exacerbation of dystonia requires prompt intervention and admission in the hospital. It is critical to recognize early and differentiate dystonic storm from other hyperkinetic movement disorder as it may lead to metabolic complications such as rhabdomyolysis, leading to acute            renal failure. This paper has been written to describe three cases of SD, all having secondary dystonia with different etiologies to highlight the mode of presentation, diagnosis, treatment and outcome.

Methodology: We report 3 cases of severe secondary dystonia culminating in SD necessitating management in hospital setting. All the three cases were admitted in a tertiary care hospital and evaluated.

Results: One patient was treated in intensive care unit. In brief 1st case was a 5 year boy with dyskinetic CP who was treated with trihexiphenidyl (THP), baclofen and midazolam infusion. Second case was a 15 month old boy, diagnosed case of mitochondrial encephalopathy (Leigh disease) who was treated with THP, baclofen, haloperidol, clonazepam and infusion midazolam. The third case was a 13 month old boy, diagnosed case of Molybdenum Cofactor deficiency who was treated with THP, tizanidine but they refused to take midazolam.

Conclusion: In this case series, three cases with SD with different etiology have been described with clinical features, modalities of treatment and outcome.

Open Access Short Communication

Acute Renal Failure Related to Malaria in Admitted Patients in Pediatric Hospitals from Dakar, Senegal: A Series of Eleven Cases

Khadim Diongue, Aliou Abdoulaye Ndongo, Lamine Thiam, Ndiogou Seck, Amy Gaye, Assane Sylla, Daouda Ndiaye

Asian Journal of Pediatric Research, Page 24-30
DOI: 10.9734/ajpr/2020/v4i430157

Aims: To firstly determine the incidence of acute renal failure (ARF) related to malaria in a series of 11 cases among admitted patients at the pediatric hospitals in Dakar, Senegal and to lastly measure the performance of rapid diagnostic test (RDT) and microscopy in malaria diagnosis using polymerase chain reaction (PCR) as a gold standard.

Study Design: A bi-centric and descriptive study was carried out.

Place and Duration of Study: From June 2018 to January 2019 in two university hospitals of Dakar, Senegal: Aristide Le Dantec university hospital and Albert Royer university hospital.

Methodology: Pediatric patients aged under 18 years with confirmed malaria by RDT or microscopy and ARF defined by anuria or oligo-anuria and a decrease in glomerular filtration rate were included. Nested PCR was performed to confirm malaria diagnosis and Plasmodium species typing.

Results: In total, 11 ARF cases (8.5%) related to malaria among 130 children infected with malaria parasites were included out of 4,474 hospitalized. Affected children were aged between 2 and 16 years with a mean age of 11 years and a sex ratio of 0.57. For malaria diagnosis, RDTs were positive for all patients while microscopy only revealed 6 cases (54.5%) as well as PCR. However, microscopy and PCR presented two discrepancies. Considering PCR as the gold standard tool, RDT showed a relative high sensitivity (100%) and zero specificity with a positive predictive value (PPV) of 54.6% while microscopy respectively showed a sensitivity and a specificity of 66.7 and 60%.

Conclusion: This study showed the relatedness between ARF and P. falciparum malaria. Even though microscopy remains the gold standard for the diagnosis of malaria but microscopists must be regularly trained. In addition, RDT should always be confirmed by microscopy and preferably by PCR.

Open Access Original Research Article

Evaluation of Procalcitonin Serum Levels in Children Younger than Five Years Old with Urinary Tract Infection

Mohsen Akhavan Sepahi, Mohammad Kazem Moslemi, Mohammad Reza Haeri

Asian Journal of Pediatric Research, Page 31-37
DOI: 10.9734/ajpr/2020/v4i430158

Background: Children with vesicoureteral reflux (VUR) are posed to the danger of recurrent pyelonephritis, kidney scar and renal failure. Nowadays, the evaluation of VUR is carried out using different imaging methods that are accompanied with different limitations. Pediatricians usually look for other evaluation methods that are feasible, easy to implement and carries the least amount of danger to the patient. 

Objectives: The aim of this study was to investigate the level of serum Procalcitonin (PCT) as a predictor of VUR instead of the voiding cystourethrogram (VCUG).

Methods: This case-control study was conducted from 2013 to 2014. One hundred and ten children younger than five years old were divided into two groups: (i) the case group with 76 children diagnosed with urinary tract infection (UTI) using urine culture test, and (ii) the control group with 34 healthy children. Serum levels of PCT were measured by a commercial kit. Demographic data were collected using a questionnaire and analyzed by software SPSS.

Results: Of the samples, 69.1% of them had no VUR, 20% and 10.9% of the samples suffered from severe unilateral VUR and severe bilateral VUR, respectively. With regard to the level of serum PCT, 61.8% of the samples were positive. One-half of those samples (38 people) who was diagnosed to be healthy had a normal level of serum PCT. However, 30 people (88.2%) of the samples diagnosed to be healthy using VCUG had increased level of serum PCT. The positive and negative predictive values of the level of serum PCT were 44% and 90%, respectively. In this respect, 50% of the samples diagnosed by using level of serum PCT were false positive and 11.8% were false negative. The kappa score for the level of serum PCT was 0.3 (P < 0.0001). The positive predictive value of the serum level of PCT for the female and male samples were 43% and 50% respectively.

Conclusions: There was no statistically significant relationship between VUR and the serum level of PCT.

Open Access Original Research Article

Perception and Practices of Home Management of Acute Watery Diarrhoea and Its Impact on Dehydration

M. Z. Uddin, K. Fatema, R. S. Begum, T. Saad, M. M. Hossain, A. R. M. S. H. Khan, M. Mohsin

Asian Journal of Pediatric Research, Page 38-46
DOI: 10.9734/ajpr/2020/v4i430159

Background: Diarrhoea is one of the commonest causes of death in children in developing countries. Proper home management can reduce morbidity and mortality. High rates of dietary restriction, poor knowledge of preparation of ORS solutions and low attention given to clinical indicators are causes for seeking hospital management. This study was done to observe the knowledge of caregivers regarding home management of acute watery diarrhoea and to assess the impact of home management on severity of dehydration.

Methods: This study was a prospective study carried out on children with acute diarrhea who attended outpatient department (OPD) of Rangpur Medical College Hospital and duration of the study was 6 months. A total of 100 cases were included in this study who fulfilled the definition of acute diarrhea by WHO. The investigator questioned each mother with the help of a preformed questionnaire.

Results: In 85% of cases, caregivers perception about diarrhea was an increase in the frequency and fluidity of stool. About 95% of cases were initially treated at home; treatment was given at home with ORS (95%). There was change in feeding pattern and modified food was given (24%). Perception about ORS preparation was appropriate in 64% and inappropriate in 31% of cases. Inadequate amount of ORS was given in 74% cases. There was no interruption of breast feeding; dietary restriction during diarrhoea was observed only in 2% cases. Most of the parents did not know when to return immediately to hospital.  Dehydration status of diarrhoeal cases was no dehydration (81%), some dehydration (18%) and severe dehydration (1%). In majority of the cases different drugs were used.

Conclusion: Most of the caregivers’ decided to treat diarrhoea at home. Many of them had wrong perception about preparation of ORS and some of them did not know how to make ORS solution. Inadequate amount of ORS was given in most of the cases. Thus to lessen hospital burden and to prevent dehydration increased emphasis should be given to health education of home management of diarrhea.

Open Access Original Research Article

Accuracy of Current Non-invasive Methods in Estimating Aortic Coarctation Gradients

Wejdan Khaled Ba- Atiyah, Riad Abou Zahr, Zaheer Ahmad, Yahia Mohamed El Mahdi, Mohammed Omar Galal

Asian Journal of Pediatric Research, Page 47-57
DOI: 10.9734/ajpr/2020/v4i430160

Aims: To understand the accuracy of non-invasively obtained blood pressure gradients (cuff vs Doppler gradient) with an invasively measured pressure gradient.

Study Design:  Retrospective study.

Place and Duration of Study: Department of Pediatrics, Section of Pediatric Cardiology, King Faisal Specialist Hospital & Research Center, Jeddah, Saudi Arabia, between Jan, 2010 till Jan, 2020.

Methodology: A retrospective study of patients with CoA who underwent cardiac catheterization between Jan, 2010 till Jan, 2020 was performed. Cuff BP gradients and Doppler echocardiography were measured prior to cardiac catheterization and afterwards, when an intervention was performed. Student t test and Bland-Altman analysis were performed.

Results: 55 patients with aortic coarctation underwent 92 cardiac catheterizations. Among them 75 needed interventions. This resulted in a total of 162 cardiac catheterizations (cath), from them 67 pressure gradients included in the analysis. There was no statistically significant difference between mean Doppler measurements and invasively derived catheter gradients (p=0.12). In contrast peak Doppler measurement (p < 0.00001) and cuff blood pressure gradients (p=0.03) showed significant differences to the cath gradient. We found that cuff blood pressure gradients accurately reflected cath measurement in native COA (p=0.40) and in those who weigh less than 10 kg (p=0.67). Mean Doppler measurements had a small tendency for underestimation. Peak Doppler gradient and cuff pressure gradient usually overestimated cath measurement. 

Conclusion: The mean Doppler echocardiography seems to be the most accurate among the other noninvasive methods in use to estimate severity of aortic coarctation. It provided reasonable agreement with the invasively obtained aortic coarctation gradient.