Evaluation of Proteinuria and Hematuria in Ambulatory Setting.

Proteinuria and/or hematuria are common findings in ambulatory settings. Proteinuria can be glomerular and/or tubular in origin and it may be transient, orthostatic, or persistent. Persistent proteinuria may be indicative of a serious kidney pathology. Hematuria, which denotes the presence of an increased number of red blood cells in the urine, can be gross or microscopic. Hematuria can originate from the glomeruli or other sites of the urinary tract. Asymptomatic microscopic hematuria or mild proteinuria in an otherwise healthy child is less likely to be of clinical significance. However, the presence of both requires further workup and careful monitoring.

Managing urinary tract infections.

Urinary tract infections (UTI) are common in childhood. Presence of pyuria and bacteriuria in an appropriately collected urine sample are diagnostic of UTI. The risk of UTI is increased with an underlying urological abnormality such as vesicoureteral reflux, constipation, and voiding dysfunction. Patients with acute pyelonephritis are at risk of renal scarring and subsequent complications such as hypertension, proteinuria with and without FSGS, pregnancy-related complications and even end-stage renal failure. The relevance and the sequence of the renal imaging following initial UTI, and the role of antimicrobial prophylaxis and surgical intervention are currently undergoing an intense debate. Prompt treatment of UTI and appropriate follow-up of those at increased risk of recurrence and/or renal scarring are important.

Iron deficiency in children with early chronic kidney disease.

Iron deficiency (ID) contributes to the development of anemia in patients with chronic kidney disease (CKD). The frequency of ID in children with early CKD has not previously been reported. This was a retrospective chart review of children with CKD stages 2 and 3 followed at the CKD clinic of Children's Hospital of Michigan. ID was defined as low ferritin and transferrin saturation <20%. Patients on iron supplements were considered as iron-deficient cases. There were 50 patients included in the study (72% male) with a mean age of 10.31 (±5.21). The mean glomerular filtration rate (GFR) was 55.4 ml/min/1.73 m(2) (±14.61). ID was present in 42% of patients, out of whom almost half (42.9%) presented with anemia. Females had a higher frequency of ID (64.3%). The frequency of ID with anemia increased from 4.3% to 29.6%, (p = 0.03) in stage 2 to stage 3 CKD, respectively. The frequency of ID without anemia also increased with progression of CKD from stage 2 to stage 3, however, the difference was not statistically significant. ID is frequent in patients with early CKD. Monitoring of iron tests and treatment of ID is important in this population of patients.

Weight or body surface area dosing of steroids in nephrotic syndrome: is there an outcome difference?

Although prednisone is the treatment of choice for nephrotic syndrome (NS) in childhood, the dosing regimen varies between 60 mg/m(2)/day, as recommended in early studies, to the often prescribed 2 mg/kg/day dose, which is used in common practice. Mathematical models have demonstrated that weight-based dosing can be less than body surface area (BSA)-based dosing in smaller children. To test our hypothesis that weight-based dosing would result in altered treatment outcomes in children with NS, we analyzed a cohort of 56 children (mean age 5.4 ± 3.8 years) treated with a weight-based dosing regimen. Theoretical underdosing of corticosteroids was tested by calculating a relative underdosing percentage (RUP), which was defined as the dose difference between the theoretical BSA-based dose and the actual weight-based doses divided by the BSA-based dose × 100. We found that the mean "actual" prednisone dose in our patients was 43.6 ± 19.3 mg/day; in contrast, the mean theoretical BSA-based dose was calculated to be 48.8 ± 16.7 mg/day. Among the 56 patients, 43 (76.7%) were initial responders, of whom 58% followed a frequently relapsing (FR) course. RUP was significantly higher in FR (16.6 ± 7.9%) than in infrequent relapsers (8.7 ± 9.8%) (P = 0.03). RUP was not significantly different among initial responders and nonresponders. Based on these results, we conclude that prednisone underdosing, when dosing is prescribed according to weight, does not affect the initial response to treatment, but it does increase the likelihood of a FR course in responders.

Pediatric Nephrolithiasis: Risk Factors, Evaluation, and Prevention.

Pediatric nephrolithiasis is a disease of increasing prevalence and economic burden, with data showing a rising trend among girls and adolescents. Kidney stones in children tend to have a high recurrence rate related to underlying risk factors, and the aim of evaluation should be to understand such causes so that targeted therapy can be provided to decrease recurrence and complications. Metabolic, anatomic, and genetic abnormalities as well as environmental risk factors have all been implicated in the pathogenesis of nephrolithiasis. Recent studies have focused on identifying monogenic causes of nephrolithiasis and/or nephrocalcinosis in young patients that would result in personalization of treatment as well as future diagnostic implications for family members. Treatment aims to decrease stone burden and recurrence as well as minimize complications and is guided by stone size and location as well as surgical expertise. Multidisciplinary care for these patients has been on the rise to provide a more comprehensive medical and surgical support. [Pediatr Ann. 2020;49(6):e262-e267.].

Clinical significance of IgM deposition in pediatric minimal change disease.

BACKGROUND: Idiopathic nephrotic syndrome (INS) is a common pediatric disease. Minimal change disease (MCD) is the most common histopathological subtype and usually has good prognosis. However, in less common presentations, INS may have an unusual course that makes renal biopsy a necessity to identify its etiology. Immunoglobulin M (IgM) occasionally deposits in the mesangium and can be seen under immunofluorescence (IF). The role of IgM is controversial in MCD. It is likely associated with less favorable outcomes for MCD. This study aims to investigate the clinical significance of mesangial IgM deposits on the outcome of MCD in a pediatric population. METHODS: In this retrospective cohort study, we obtained native kidney biopsy samples from 192 children who were diagnosed with MCD from 2003 to 2014. The samples were divided into groups according to the histopathological deposition of IgM in biopsies under IF. The group for which biopsies showed IgM was labeled as IgM + IF (n = 77), and the group for which biopsies were without IgM was labeled as IgM-IF (n = 115). We reviewed hypertension, hematuria, and estimated glomerular filtration rate (eGFR) at the time of presentation to our institute; response to steroid therapy (remission, dependence, frequent relapses, and resistance) and response after adjuvant immunosuppressive therapy (complete remission, partial remission, frequent relapses, and no response) when indicated; development of chronic kidney disease (CKD) and end-stage renal disease during the course of the disease (ESRD). RESULTS: Our results showed that mesangial IgM deposition in MCD showed significant statistical association with hypertension at the time of presentation (P = .05). There was statistically significant association between the presence of IgM deposition and the development of steroid dependence (P = .05) and CKD during the course of the disease (P = .05). CONCLUSIONS: Our study showed that IgM deposition in MCD showed statistical association with hypertension by the time the patient presented to our institute, development of steroid dependence, and CKD. IgM may play a role in MCD. However, we recommend a prospective study to verify the role of IgM in MCD outcomes.

Departmental collaborative approach for improving in-patient clinical documentation (five years experience).

INTRODUCTION: Health care institutes are cooperative areas where multiple health care services come together and work closely; physician, nurses and paramedics etc,. These multidisciplinary teams usually communicate with each other by documentation. Therefore, accurate documentation in health care organization is considered one of the vital processes. To make the documentation useful, it needs to be accurate, relevant, complete and confidential. OBJECTIVES: The aim of this paper is to demonstrate the effect of the collaborative work in the Department of Pediatrics on improving the quality of inpatient clinical documentation over 5 years. METHODS: Improving clinical documentations went through several collaborative approaches, these include: Departmental Administration involvement, establishment of quality management team, regular departmental collaborative meeting as a monitoring and motivating tool, establishment of the residents quality team, Integration of quality projects into the new residents annual orientation, considering it as a part of the trainee personal evaluation, sending reminders to the consultants and residents on the adherence for admission note initiating and 24 h's verification, utilization of standardized template of admission note and progress note and emphasizing on the adherence to the approved medical abbreviation list only for any abbreviation to be used. RESULTS: During the period between the first quarter of 2012 to the fourth quarter of 2017; a significant improvement was noticed in the overall in-patient clinical documentation compliance rate, as it was ranging from lower 50% in 2012 and 2013, and increased gradually to reach upper 80% in the last quarters of 2016 and 2017. These figures are based on an independent audit that being done by the hospital quality management department and received by the department in a quarterly basis. CONCLUSION: Despite multiple challenges for improving the compliance for clinical documentations, major improvement can be achieved when the collaboration and efforts among all stakeholders being shared and set as a common goal.

A three-year-old boy with X-linked adrenoleukodystrophy and congenital pulmonary adenomatoid malformation: a case report.

INTRODUCTION: X-linked adrenoleukodystrophy leads to demyelination of the nervous system, adrenal insufficiency, and accumulation of long-chain fatty acids. Most young patients with X-linked adrenoleukodystrophy develop seizures and progressive neurologic deficits, and die within the first two decades of life. Congenital or acquired disorders of the respiratory system have not been previously described in patients with X-linked adrenoleukodystrophy. CASE PRESENTATION: A 3-year-old Arabic boy from Yemen presented with discoloration of the mucous membranes and nail beds, which were considered cyanoses due to methemoglobinemia. He also had shortness of breath, fatigue, emesis and dehydration episodes for which he was admitted to our hospital. Chest radiograph and chest computed tomography scans showed congenital pulmonary adenomatoid malformation. A few weeks before the removal of the malformation, he had a significant episode of hypotension and hypoglycemia. This development required further in-hospital evaluation that led to the diagnosis of adrenal insufficiency and the initiation of treatment with corticosteroids. One year later, he developed seizures and loss of consciousness. Magnetic resonance imaging of his head showed diffuse demyelination secondary to X-linked adrenoleukodystrophy. He was treated with anti-seizure and anti-oxidants, and was referred for bone marrow transplant evaluation. CONCLUSION: The presence of adrenal insufficiency, neurologic deficits and seizures are common manifestations of X-linked adrenoleukodystrophy. The association of congenital lung disease with X-linked adrenoleukodystrophy or Addison's disease has not been described previously.