An Enigmatic Color Change of Urine: Alkaptonuria: Alkaptonuria

Alkaptonuria is an exceedingly rare tyrosine metabolism disorder of autosomal recessiveinheritance. Only a few instances of it have been observed in Bangladeshi children. Here,we talk about a 2-year-old boy who had dark urine and was later found to have alkaptonuria

Immunoglobulin A Vasculitis Nephritis in Children: Experience from a Tertiary Care Hospital, Bangladesh

Background and Aim: Immunoglobulin A vasculitis (IgAV), formerly known as Henoch-Schönlein purpura (HSP),is the most common vasculitis in children with multiorgan involvement. Renal involvement is one of the important causes of morbidity and mortality. The objective of this study was to evaluate the frequency, clinical profile, and outcome of IgA vasculitis nephritis (IgAVN) in children. Methods:This prospective cross-sectional study was conducted in Dr. MRKhan Children Hospital & Institute of Child Health, Dhaka, over a period of 5years from January 2015 to December 2019. Data were collected using a structured questionnaire form and analyzed by the SPSS software version 20.0. Results:A total of 57cases of IgA vasculitis were admitted of whom 16 (28%) had renal involvement. The mean age was 7.7years. Regarding renal involvement, the majority of the patients (56.25%) had isolated hematuria. All nephritis patients (100%) had purpura and 75% of the patients had severe abdominal pain. The mean hematocrit and the mean platelet count were significantly higher in the nephritis group compared to patients without nephritis (41.49±4.47vs.39.98±5.16, p-value<0.005 and485.51±58.29 vs. 293.89±65.15, p-value<0.001, respectively). The level of complement C3 was significantly lower in the nephritis group compared to patients without nephritis (0.85±0.4 vs. 1.5±0.3, p-value <0.01). The majority (68.75%) of the patients recovered and 18.75% were in remission with immunosuppressant. None of the cases progressed to ESRD. Conclusion:Severe abdominal pain, high platelet counts, high hematocrit levels, and low C3 concentrations are common findings in nephritis. Nephritis resolvespontaneously in most cases but severe nephritis requires treatment with immunosuppressive drugs for remission.   &nbsp

PCRRT Expert Committee ICONIC Position Paper on Prescribing Kidney Replacement Therapy in Critically Sick Children With Acute Liver Failure

Management of acute liver failure (ALF) and acute on chronic liver failure (ACLF) in the pediatric population can be challenging. Kidney manifestations of liver failure, such as hepatorenal syndrome (HRS) and acute kidney injury (AKI), are increasingly prevalent and may portend a poor prognosis. The overall incidence of AKI in children with ALF has not been well-established, partially due to the difficulty of precisely estimating kidney function in these patients. The true incidence of AKI in pediatric patients may still be underestimated due to decreased creatinine production in patients with advanced liver dysfunction and those with critical conditions including shock and cardiovascular compromise with poor kidney perfusion. Current treatment for kidney dysfunction secondary to liver failure include conservative management, intravenous fluids, and kidney replacement therapy (KRT). Despite the paucity of evidence-based recommendations concerning the application of KRT in children with kidney dysfunction in the setting of ALF, expert clinical opinions have been evaluated regarding the optimal modalities and timing of KRT, dialysis/replacement solutions, blood and dialysate flow rates and dialysis dose, and anticoagulation methods

Frasier Syndrome: A Rare Disorder in a Patient With Nephrotic Syndrome

Frasier syndrome is a  rare genetic disorder characterized by the association of progressive renal glomerulopathy, 46,XY complete gonadal dysgenesis with a high risk of developing   gonadoblastoma. Mutations in the Wilms' tumor suppressor gene (WT1) located in 11p23 responsible for this syndrome. Patients with this syndrome commonly present with normal female genitalia, streak gonads, and  46, XY karyotyping. Nephropathy in Frasier syndrome conferred as a nephrotic syndrome (NS) with proteinuria that begins early in childhood and progressively increases with age, mainly due to nonspecific focal and segmental glomerular sclerosis (FSGS). We herein discuss a 4 year-old-girl who presented as a steroid-resistant nephrotic syndrome and later on diagnosed as a Frasier syndrome. &nbsp

Periocular anthropometric study among adult Bangladeshi Buddhist Chakma females

The dimensions of the human body are affected by geographical, racial, ethnic, gender and age factors. So, a normative data of periocular measurements based on above factors are indispensa-ble to identify any ocular pathology and to precise determination of the degree of deviations from the normal. The study was descriptive, observational and cross-sectional in nature with some analytical components. The study group consisted of a convenient sample of 100 adult Bangladeshi Buddhist Chakma females aged between 25 and 45 years. Eleven variables were analyzed using photographic procedures and nine indices were calculated from photographically measured variables. Regression analyses showed that interpupillary distance has significant positive correlation with each of the orbital measurements except for right and left eye fissure height. Regression equations were calculated from those orbital variables showing significant positive correlation with interpupillary distance

Role of Non-pharmacological Therapy in Children With Bed Wetting: Non-pharmacological Therapy and Bed Wetting

Background and Aim: Bedwetting or enuresis is not uncommon in children, althoughits prevalence varies by age. Spontaneous remission of enuresis may occur at a rate ofapproximately 15% per year, but treatment protocols should be carried out because of itssignificant impact on a child’s family, emotional state, self-esteem, and totally on the qualityof life (QoL). Today, non-pharmacological therapy is an initial treatment for enuresis,in which both the parents and children are motivated to take the behavioral managementapproach. This study was done to determine the effectiveness of non-pharmacologicaltherapy in pediatrics with enuresis.Methods: This prospective observational research was carried out in pediatrics aged 5to 15 years with bedwetting who visited the outpatient department of Asgar Ali Hospitalfrom January 2021 to December 2021. They were categorized into two groups, primaryand secondary (monosymptomatic and non-monosymptomatic) enuresis according to thedefinition of the international children’s continence society (ICCS). Also, our patients weredivided into different age groups: Group A: 5-7 years, group B: 8-10 years, and group C: >10years, and the response was classified as no response: <50%, partial response: 50-99%, andcomplete response: 100% reduction in baseline symptom frequency.Results: Among the 74 patients, 28 cases (38%) were male and 46 cases (62%) were female,with most of them having primary enuresis 72(97%), and only two patients had secondaryenuresis. Most patients were monosymptomatic 62(83.7%), and only 12 cases (16%) werenon-monosymptomatic. Among 32 patients (43%) of group A, 87.5% had complete responsewithin three months of follow-up, in group B, of a total of 20 patients (27%), 40% hadcomplete and 40% had partial response, and in group C, among 22 patients (30%), only 9%had complete and 46% had partial response.Conclusion: Non-pharmacological therapy in enuretic patients showed encouragingrecovery

A Boy with Nephrotic Syndrome and Methemoglobinemia: A Diagnostic Challenge

Methemoglobinemia is a rare disease characterized by the elevated levels of methemoglobin in the blood. It may be congenital or acquired. Co-trimoxazole is an antibiotic that belongs to the sulfone group. Sulfone group drugs may produce drug-induced acquired methemoglobinemia. Methemoglobin is an oxidized form of hemoglobin that has an increased affinity to oxygen and a reduced ability to release oxygen to tissues. High levels of methemoglobin inred blood cells cause tissue hypoxia. This disorder may present with several symptoms such as cyanosis, fatigue, dyspnea, and headache. Because it is a rare cause of cyanosis and hypoxemia, the diagnosis of methemoglobinemia is often delayed. We herein discuss a five-year-old boy with steroid-resistant nephrotic syndrome who presented with exertional dyspnea and cyanosis and was later diagnosed as a case of co-trimoxazole-induced methemoglobinemia

Renal Involvement in Children with Dengue Fever: A Study in Tertiary Care Hospital of Bangladesh

Background and Objective: Dengue has emerged globally as the most relevant viral infection transmitted by a mosquito bite and represents a major threat to public health. Dengue-related renal manifestations such as proteinuria, hematuria, acute kidney injury (AKI), and rhabdomyolysis are not uncommon, and acute kidney injury (AKI) is a serious complication of dengue fever. There is relatively few data on the renal manifestations of dengue fever in children. Hence, this study was conducted to evaluate the incidence, characteristics, and clinical outcome of dengue fever with renal manifestations. Method. This prospective cross sectional study was conducted in Dr. M R Khan Children Hospital and Institute of Child Health, Dhaka, over a period of 1 year from January 2018 to December 2018. The study was approved by the ethical committee of the institute. A total number of 316 patients were admitted with the diagnosis of dengue fever either NS1 positive or antibody IgM positive or both IgM and IgG positive. Data were collected in a structured questionnaire form and were analyzed by SPSS version 20.0. The disease severity was classified according to the World Health Organization criteria. Renal manifestations were divided into AKI groups using pRIFLE criteria. Proteinuria was defined as urinary protein >1+ (30 mg/dL) by dipstick test. Hematuria was defined as red blood cell (RBC) >5/μL in a fresh uncentrifuged urine specimen. Result. Among 316 dengue patients, thirty-one patients (9.8%) had renal involvement. Most of the patients (54.83%) with renal manifestations were aged between 1 and 5 years. A total of 14 patients were found to have proteinuria (4.4%). Nephrotic-range proteinuria was seen in only one patient (0.3%). AKI was defined by pRIFLE criteria and was seen in 13 patients (4.1%); among AKI 6 (46.15%) had risk, three patients (23.07%) had injury and 4 (30.7%) had failure and needed peritoneal dialysis. Death occurred in 3 patients (9.6%) in dengue with AKI who had failure. The incidence of renal manifestations (proteinuria, hematuria, and AKI) is as high as 9.8% among patients with dengue, and those with AKI had significant morbidity and mortality. Conclusion. Renal involvement in children with dengue is not uncommon. Dengue associated with AKI had significant mortality and morbidity

Knowledge and Practice About Blood Pressure Measurement in Children: Healthcare Provider’s Perspective: Knowledge About Blood Pressure Measurement

Background and Aim: Hypertension (HTN) has become more prevalent among youngsters.It is frequently under-recognized due to a lack of routine blood pressure measurement in manyhealth centers, partly owing to a shortage of devices and possibly because of the notion that itis not the foremost disease in children. In Bangladesh, there is a scarcity of data on how doctorsview childhood HTN and their practice of measuring blood pressure in children. Methods: This cross-sectional mailed-based survey was done on pediatricians and approved bythe institutional review board of Dr. Khan Shishu Hospital & the Institute of Child Health fromJune to December 2021. We obtained the email addresses of all pediatricians from the BangladeshPediatric Association. Results: Of the 536 pediatricians in the mailing sample, 257 cases responded and the responserate was 47.9%. The majority of respondents (62.4%) were general pediatricians and only 12.2%were pediatric nephrologists. This survey revealed that 77.2% of pediatricians did not measureblood pressure routinely among children 3-18 years of age, whereas 66% reported measuringblood pressure if children had risk factors. Conclusion: The findings of our study point to a knowledge and practice gap among pediatricians,who are primary health care providers, when diagnosing hypertension in children. For childrenaged 3 to 18 years, most pediatricians reported no regular assessment of blood pressure. Mostpediatricians did not repeat blood pressure measurements for diagnosis, nor did they often useblood pressure cuffs or charts for children. These issues need to be addressed for better diagnosisand treatment of childhood HTN

Easy fabrication of l-glutamic acid/ZnS composites for efficient photo-catalytic and supercapacitor performance

l-Glutamic acid/ZnS (L-GA/ZnS) composites were prepared by varying the amount of ZnS addition ranging from 1-5 wt% by means of an easy solvent casting approach. The morphological investigation, antimicrobial activity, photocatalytic enactment, and electrochemical properties of the composites were evaluated. The formation of L-GA/ZnS composites was confirmed by FTIR, UV-Vis, and photoluminescence (PL) spectroscopy. Besides, FTIR, UV-Visible, and PL data revealed the possible incorporation of ZnS into L-GA. The L-GA/ZnS composites demonstrated similar plate-like structure of L-GA with agglomerated ZnS morphology on the plate surface with diameter in the range of 50-500 nm, confirmed by FESEM/EDS measurements. The prepared composites showed excellent photocatalytic depiction towards methylene blue (MB) degradation in comparison to L-GA and ZnS. A set of supercapacitor devices were fabricated using L-GA/ZnS composites. The performance of the supercapacitor was assessed by GCD and exhibited good energy storage capacity. The prepared composites showed promising prospects for hybrid supercapacitor application. These outcomes may offer new insight into the fabrication of L-GA/ZnS composites as photocatalysts for organic contaminants treatment