In loving memory

Professor Mohammad Ismaeili

Hypokalemia is one of the most common electrolyte disorders in hospitalized patient. Causes of hypokalemia include impaired renal potassium (K+) excretion, gastrointestinal losses or transcelluar shifts. Assessments of urinary K+excretion, acid-base status, and blood pressure are three major components to the causes ofhypokalemia.A random urine K+-to-creatinine (K+/Cr) less than 13 mEq/g Cr (<1.5 mEq/mmol) in a patient with hypokalemic metabolic alkalosis suggests poor intake, surreptitious vomiting, congenital pyloric stenosis, a shift of K+from extracellular fluid into the cells, laxative abuse, familial or sporadic periodic paralysis. In the setting of hypertension, urine K/Cr >1.5 mEq/mmol indicates primary and secondary hyperaldosteronism, Liddle syndrome, or apparent mineralocorticoid excess. By contrast, in the absence of hypertension, a urine K+ /Cr>1.5, is usually suggestive of surreptitious use of diuretic, Bartter syndrome or Gitelman syndrome. Measurements of the plasma renin activity and plasma aldosterone concentration are necessary to differentiate these conditionsfrom one another.Severe or symptomatic hypokalemia, if not recognized early or treated appropriately can lead to significant mortality and morbidity. In this article the basic principles of normal K+homeostasis and the pathophysiology that can disturb this balance are discussed. A selected case report focusing on the essential aspect of patient’s presentation, signs and laboratory data followed by series of questions with particular attention to the diagnosis and management of hypokalemia needed to assist in the differential diagnosis and treatmentare also discussed.Each question is followed by detailed discussion and reviews the recent publications that are useful at thebedside.

Keywords:Hypokalemia; Metaboloc alkalosis; Causes; Diagnoses; Treatment.

Children with nephrotic syndrome (NS) develop complications due to either the disease state or its treatment. Infections, thromboembolism and acute kidney injury are the most common complications in children with NS. Several studies in children with NS have reported that urinary tract infections, upper respiratory tract infections, peritonitis and sepsis are the most commonly reported infections. Infection is one of the common triggering factors for relapse, and prophylaxis against infections is required in patients unresponsive to steroids or with frequently relapsing disease. In this review article, we summarize the strategies for prevention of infections in NS. The most commonly studied drug for the prevention of infection in NS is intravenous immunoglobulin G (IVIg), while other drugs include thymosin, oral transfer factor, Bacillus Calmette-Guérin (BCG) vaccine, mannan peptide tablet, polyvalent bacterial vaccine and Chinese herbal medications (Tiaojining and Huangqui granules). Several vaccination programs including pneumococcal, influenza A, varicella and measles have been effective in the prevention of infections in nephrotic children. However, established measures for preventing infections in nephrotic children are lacking, and to draw any conclusion, randomized controlled trials are required.

Background and aim: Little is known about what happens in primary health structures without nephrology care, especially in the paediatric population. We sought describe the epidemiology of AKI in children at risk in district hospitals in Cameroon.

Methods: We prospectively screened consenting children aged 2-18 years of age in paediatric wards of 3 large urban district hospitals over a period of 4 months. Participants with AKI were then followed up till discharge. Outcomes of interest were need and access to dialysis, and renal recovery on hospital discharge. Written assent was obtained from parents or caregivers.

Results: Among the 211 children admitted during the study period, 82% (n=173) were at risk of AKI, of whom 19 (11%) did not consent. Of the 154 children included 54.5% were males and the median age was 6 years [IQ 3-10]. Sepsis and volume depletion were the most common risk factors of AKI. The incidence of AKI was 12.3% (n=19). AKI was mostly community acquired and 47.4% (n=9) patients were in KDIGO stage 3. Pre-renal AKI and acute tubular necrosis accounted for 63.2% and 36.8% respectively. Gastro-intestinal losses, malaria, bacterial sepsis and nephrotoxins were the common aetiologies of AKI. On discharge, 71.7% of AKI had complete recovery renal function.

Conclusion: Risk factors of AKI are very common in children on admission in general district hospitals in Cameroon. Efforts should be made to raise awareness of primary health caregivers about risk assessment, prevention, early recognition and management of AKI in children.

Background and Aim: The aim of this study was to evaluate urinary metabolic features as a risk factor in stone formation.

Methods: In this case-control study, 222 children ranging from 6 months to 16 years old suffering from urolithiasis in our university hospitals in Iran in 2019-2020 were selected through random sampling and were subsequently evaluated. The research group  were cchildren with urinary stones and urinary tract infection and the control group encompassed children with urinary stones and without urinary tract infection. Data was analyzed using statistical package for social sciences (SPSS) version 16 (SPSS Inc. Chicago, IL) for windows.

Results: The ratio of average amounts of calcium, magnesium, oxalate, cystine, uric acid, and citrate   to creatinine showed no significant differences between the two groups.

Conclusion: Urinary tract infection cannot be considered as a factor for stone formation in the urinary tract due to changes in urinary biochemical characteristics.

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.

Background and Aim: Despite several studies about COVID-19, many factors remain unknown. Apart from pulmonary involvement, the other systemic association needs to be explored. Since information is lacking this study was conducted to see the impact of COVID-19 infection in children with kidney diseases.

Methods: This retrospective study was carried out at Dhaka Shishu Hospital and Square Hospitals Ltd. All children below 18 years who had renal diseases and tested RT PCR positive for the SARS-COV-2 virus were included in the study.  Data regarding patient’s demography, clinical presentation, hospital course, and outcome were collected from the hospital database and were analyzed.

Results: Among the COVID-19 positive pediatric patients, the proportion of patient with a history of kidney disease were 12%. The commonest age group belongs to 6 -10 years with a female predominance. Nephrotic syndrome (50%) was the commonest primary renal etiology followed by acute kidney injury (26%). Along with COVID-19 related symptoms like fever (38%) and respiratory tract infection (31%), a good number of them remain asymptomatic (27%) during diagnosis. Hypertension (50%) and hematuria (35%) were the two prevailing clinical findings. Moreover, anemia (65%), and elevated creatinine (50%) were found surprisingly higher irrespective of the primary etiology. A better outcome was observed in children under 5 years.

Conclusion: Covid -19 is frequent in patients with a history of kidney diseases and it may present with an atypical presentation like hypertension and or hematuria. Hence, clinicians should increase their awareness and concern to deal with COVID-19 infection among renal patients.

Background and Aim: Steroids are the mainstay of initial treatment in children with Idiopathic Nephrotic Syndrome (INS). The role of diuretics in children with NS is less clear in comparison to adults. In cases with severe or refractory edema, furosemide is often combined with albumin infusion (0.5 to 1 g/kg) to provide symptomatic relief.

Methods: This study was a retrospective chart review of 17 patients with a diagnosis of Steroid Dependent Nephrotic Syndrome (SDNS) admitted for relapse of NS with severe edema who were resistant to diuretic therapy alone. The patients were treated as per unit protocol with an infusion of 100 ml intravenous albumin 20% over 4 hours and 2 doses (one in the morning at 10 AM and the other in the evening at 6 PM) of furosemide 1mg/kg for 3 days. Response to therapy and adverse events were evaluated.

Results: The mean age of the study population was 10.58±1.5 years. All of the patients had severe edema and none of them had responded to intravenous diuretics alone. After co-administration of intravenous albumin infusion and furosemide, the mean weight loss per day per patient was 0.87±0.16 kg.

Conclusion: A significant improvement was noted in all of the patients following co-administration of albumin and furosemide without any adverse events.

Background and Aim: Steroids are the mainstay of initial treatment in children with Idiopathic Nephrotic Syndrome (INS). The role of diuretics in children with NS is less clear in comparison to adults. In cases with severe or refractory edema, furosemide is often combined with albumin infusion (0.5 to 1 g/kg) to provide symptomatic relief.

Methods: This study was a retrospective chart review of 17 patients with a diagnosis of Steroid Dependent Nephrotic Syndrome (SDNS) admitted for relapse of NS with severe edema who were resistant to diuretic therapy alone. The patients were treated as per unit protocol with an infusion of 100 ml intravenous albumin 20% over 4 hours and 2 doses (one in the morning at 10 AM and the other in the evening at 6 PM) of furosemide 1mg/kg for 3 days. Response to therapy and adverse events were evaluated.

Results: The mean age of the study population was 10.58±1.5 years. All of the patients had severe edema and none of them had responded to intravenous diuretics alone. After co-administration of intravenous albumin infusion and furosemide, the mean weight loss per day per patient was 0.87±0.16 kg.

Conclusion: A significant improvement was noted in all of the patients following co-administration of albumin and furosemide without any adverse events.

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.

Pediatric hypertension is not uncommon inchildren; yet, it is very commonly missed by primary physicians and end organ damage has already started by the time it is diagnosed. Hypertension is sometimes considered a consequence of an illness when it actually is the etiology of that illness, for example, intracerebral hemorrhage with raised intracranial pressure.