Unusual Presentation of a Hemiplegic Migraine in a Seven-Year-Old Child: A Case Report.

In the pediatric population, headache is a common presenting symptom, and migraine is often the diagnosis. A hemiplegic migraine is characterized by an aura and sudden-onset weakness on one side of the body that usually resolves without causing any permanent neurological damage. In this case, we present a seven-year-old male child with a known case of proximal tubular dysfunction (homozygous mutation in the SLC4A4 gene) who presented to the emergency department with a one-day history of weakness on the right side of his body.

Intravesical gentamicin instillation in the prevention of recurrent urinary tract infections in children with neurogenic bladder- a single-center retrospective observational study.

Background: Recurrent urinary tract infections (UTI) in children with neurogenic bladder (NGB) put them at high risk of morbidity and mortality from urosepsis and end-stage renal disease (ESRD). Since the efficacy of low-dose prophylactic antibiotics to prevent these recurrences has been declining since the emergence of extended-spectrum beta-lactamase (ESBL) organisms, intravesical gentamicin instillation has also been used, but only scarce data in children is available in the literature.

Objective: We evaluate the efficacy of intravesical gentamicin instillation to reduce UTIs in children with NGB, compare it with oral antibiotic prophylaxis and determine its effect on pathogens resistance to antibiotics.

Case Report: BCG-Triggered Hemophagocytic Lymphohistiocytosis in an Infant With X-Linked Recessive Mendelian Susceptibility to Mycobacterial Disease Due to a Variant of Chronic Granulomatous Disease.

In the United Arab Emirates, BCG (Bacillus Calmette-Guérin) is administered to all newborns. We present here a young infant with an inborn error of immunity (IEI) who developed fatal adverse events to this live-attenuated vaccine. This male infant received BCG (Serum Institute of India Pvt.

Epidemiology of Bronchiolitis in Hospitalized Infants at Tawam Hospital, Al Ain, United Arab Emirates.

Background: Bronchiolitis is the commonest lower respiratory tract infection, found worldwide in children < 2 years of age. Over sixty percent of cases are caused by Respiratory Syncytial Virus (RSV). The disease is known to have significant morbidity, mortality and health care costs.

Corrigendum to "Chronic Immune Thrombocytopenia and Hashimoto's Hypothyroidism in an Adolescent: Presentation and Implications".

[This corrects the article DOI: 10.1155/2021/6649155.].

Chronic Immune Thrombocytopenia and Hashimoto's Hypothyroidism in an Adolescent: Presentation and Implications.

Immune thrombocytopenia (ITP) is a disorder characterized by immune-mediated destruction of thrombocytes leading to peripheral blood platelet count of <100 × 10^9/L. Primary ITP is a terminology used in the absence of other causes or disorders that may be associated with thrombocytopenia, i.e.

Unique Mutation in SP110 Resulting in Hepatic Veno-Occlusive Disease with Immunodeficiency.

Familial hepatic veno-occlusive disease with immunodeficiency (VODI, OMIM: 235550) is a rare form of combined immune deficiency (CID) that presents in the first few months of life with failure to thrive, recurrent infections, opportunistic infections along with liver impairment. Herein, we are describing a Pakistani patient with a homozygous novel variant in the gene, presenting with classical phenotypic manifestations of VODI. He presented at the age of 3 months with opportunistic infections and later developed liver failure.

Significance of platelet count in children admitted with bronchiolitis.

Aim: To determine the true prevalence of thrombocytosis in children less than 2 years of age with bronchiolitis, its association with risk factors, disease severity and thromboembolic complications.

Methods: A retrospective observational medical chart review of 305 infants aged two years or less hospitalized for bronchiolitis. Clinical outcomes included disease severity, duration of hospital stay, admission to pediatric intensive care unit, or death.

Hyponatraemia and neurological complications in children admitted with bronchiolitis.

Background: Hyponatraemia occurs during bronchiolitis, sometimes with neurological manifestations. The prevalence of the latter differs widely and little is known about the time of occurrence and associated factors. This study was undertaken to investigate these complications.

Bartter and Gitelman syndromes: Spectrum of clinical manifestations caused by different mutations.

Bartter and Gitelman syndromes (BS and GS) are inherited disorders resulting in defects in renal tubular handling of sodium, potassium and chloride. Previously considered as genotypic and phenotypic heterogeneous diseases, recent evidence suggests that they constitute a spectrum of disease caused by different genetic mutations with the molecular defects of chloride reabsorption originating at different sites of the nephron in each condition. Although they share some characteristic metabolic abnormalities such as hypokalemia, metabolic alkalosis, hyperplasia of the juxtaglomerular apparatus with hyperreninemia, hyperaldosteronism, the clinical and laboratory manifestations may not always allow distinction between them.

Renal tract abnormalities missed in a historical cohort of young children with UTI if the NICE and AAP imaging guidelines were applied.

Objective: In a historical cohort of children with a urinary tract infection (UTI) who had already undergone all the imaging procedures, the aim was to determine renal tract abnormalities which would have been missed had we implemented the new guidelines from the National Institute for Health and Care Excellence in the United Kingdom (NICE) or the American Academy of Pediatrics (AAP).

Material And Methods: After a UTI episode, forty-three children (28 females, 65%) aged between 2 months and 2 years presenting at two general hospitals with a febrile UTI before 2008 underwent all the recommended imaging studies predating the new guidelines. Hydronephrosis was defined and graded according to the Society for Fetal Urology (SFU) classification.

Mixed Bartter-Gitelman syndrome: an inbred family with a heterogeneous phenotype expression of a novel variant in the CLCNKB gene.

Patients with renal diseases associated with salt-losing tubulopathies categorized as Gitelman and classic form of Bartter syndrome have undergone genetic screening for possible mutation capture in two different genes: SLC12A3 and CLCNKB. Clinical symptoms of these two diseases may overlap. Bartter syndrome and Gitelman syndrome are autosomal recessive salt-losing tubulopathies with hypokalemia, metabolic alkalosis, hyperreninemia, hyperplasia of the juxtaglomerular apparatus, hyperaldosteronism, and, in some patients, hypomagnesemia.

Uric acid excretion in rotavirus gastro-enteritis.

Background: Urate urolithiasis in children has been reported in Japan in association with rotavirus (RV) gastro-enteritis (GE).

Aims: To test the hypothesis that children with RV GE have an increased risk of hyperuricosuria compared with GE presumably caused by other viruses.

Objectives: Prospective analytic cohort study of urinary uric acid excretion in children presenting with GE between 1 January 2011 and 31 May 2012.

Familial hypomagnesemia with hypercalciuria and nephrocalcinosis (FHHNC): report of three cases with a novel mutation in CLDN19 gene.

Familial hypomagnesemia with hypercalciuria and nephrocalcinosis (FHHNC) is a rare autosomal recessive disorder that is caused by mutation in the genes coding for tight junction proteins Claudin-16 and Claudin-19. Affected individuals usually develop nephrocalcinosis and progressive renal failure; some of them may have ophthalmologic involvement as well. Phenotypic description of three affected individuals from the same Middle Eastern kindred (two sisters and their cousin) is presented.

Bradycardia following oral corticosteroid use: case report and literature review.

Introduction: Corticosteroids are used in various clinical conditions that include many immune-mediated inflammatory diseases. Different side effects were described including cardiac arrhythmias. Most of those arrhythmias were in the form of bradycardia which usually occurs with high intravenous steroid doses.

Systemic lupus erythematosus with severe nephritis that mimicked Henoch-Schoenlein purpura.

Introduction: Systemic lupus erythematosus (SLE) belongs to a family of related autoimmune rheumatic disorders that are capable of affecting multiple organs, and they are all associated with a variety of autoantibodies. Henoch Schoenlein purpura (HSP) is a sort of systemic vasculitis that is not associated with auto-antibodies and can affect different organs including the kidneys.

Case Report: A 12 year-old girl presented with abdominal pain, low grade fever, swollen and tender feet and left hand, skin rash on the lower extremities, and high blood pressure.

Novel DNA mutation in the GATA3 gene in an Emirati boy with HDR syndrome and hypomagnesemia.

We report the case of a young Emirati boy with HDR (Hypoparathyroidism, sensorineural Deafness, and Renal hypoplasia) syndrome due to the novel heterozygous deletion of two nucleotides (c.35_36delGC ) in exon 2 of the GATA3 gene. The patient developed hypocalcemia and hypomagnesemia at 3 weeks of age with high fractional excretion of magnesium, indicating renal magnesium loss.

Lupus cystitis in an Omani girl.

Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by multiple organs involvement. Bladder involvement (Lupus cystitis) is a rare manifestation of SLE, and occurs in association with gastrointestinal manifestations. We report a case of lupus interstitial cystitis with bladder irritation and bilateral hydroureteronephrosis in an adolescent female who was treated with intravenous methylprednisolone pulse therapy followed by oral prednisolone and mycofenolate mofetil (MMF).

The significance of fetal renal pelvic dilatation as a predictor of postnatal outcome.

Objectives: To define the clinical outcome of fetal renal pelvic dilatation (FRPD) in cohort of infants in United Arab Emirates.

Study Design: Data were collected from all fetuses having FRPD from January 2005 to February 2008. FRPD was graded as normal (<5 mm), mild (5-9 mm), moderate (10-15 mm), and severe (>15 mm).