Cs Mott Childrens Hospital

Ann Arbor, MI, United States

Cs Mott Childrens Hospital

Ann Arbor, MI, United States

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Lombel R.M.,University of Michigan | Lombel R.M.,Cs Mott Childrens Hospital | Hodson E.M.,Childrens Hospital at Westmead | Hodson E.M.,University of Sydney | Gipson D.S.,University of Michigan
Pediatric Nephrology | Year: 2013

Kidney Disease: Improving Global Outcomes (KDIGO) recently published the clinical practice guideline on glomerulonephritis (GN) to assist the practitioner caring for patients with GN. Chapter 4 of the guideline focuses on managing children aged 1-18 years with steroid-resistant nephrotic syndrome (SRNS), defined by an inability to achieve complete remission with corticosteroid therapy. Guideline development followed a thorough evidence review, and management recommendations and suggestions were based on the best available evidence. Limitations of the evidence, including the paucity of large-scale randomized controlled trials, are discussed. This article provides both the guideline recommendations and a brief review of relevant treatment trials related to each recommendation. This précis serves as a summary of the complete guidelines recently published. © 2012 IPNA.


Lombel R.M.,University of Michigan | Lombel R.M.,Cs Mott Childrens Hospital | Gipson D.S.,University of Michigan | Hodson E.M.,Childrens Hospital at Westmead Westmead | Hodson E.M.,University of Sydney
Pediatric Nephrology | Year: 2013

The 2012 Kidney Disease: Improving Global Outcomes (KDIGO) clinical practice guideline on glomerulonephritis (GN) is intended to assist the practitioner caring for patients with GN. Two chapters of this guideline focus specifically on nephrotic syndrome in children. Guideline development followed a thorough evidence review, and management recommendations and suggestions were based on the best available evidence. Critical appraisal of the quality of evidence and strength of recommendations followed the Grades of Recommendation Assessment, Development and Evaluation (GRADE) approach. Chapters 3 and 4 of the guideline focus on the management of nephrotic syndrome in children aged 1-18 years. Guideline recommendations for children who have steroid-sensitive nephrotic syndrome (SNSS), defined by their response to corticosteroid therapy with complete remission, are addressed here. Recommendations for those with steroid-resistant nephrotic syndrome (SRNS) (i.e., do not achieve complete remission) are discussed in the companion article. Limitations of the evidence, including the paucity of large-scale randomized controlled trials, are discussed. This article provides a short description of the KDIGO process, the guideline recommendations for treatment of SSNS in children and a brief review of relevant treatment trials related to each recommendation. © 2012 IPNA.


Selewski D.T.,Cs Mott Childrens Hospital | Symons J.M.,University of Washington
Pediatrics in Review | Year: 2014

On the basis of research evidence and consensus, the term acute kidney injury (AKI) has replaced acute renal failure, suggesting the spectrum of kidney damage that can occur (Table 1). (1)(2)(3)(4) • On the basis of research evidence, in developing countries the most common causes of AKI continue to be volume depletion, infections, and primary renal diseases. • On the basis of research evidence and expert opinion, in developed countries volume depletion and primary renal disease remain common causes of AKI in previously healthy children. • On the basis of research evidence, in hospitalized children, particularly in tertiary care centers, there has been a shift in the etiology of AKI from primary renal disease to secondary causes of AKI that are often multifactorial in nature and often complicate another diagnosis or its treatment (heart disease, sepsis, and nephrotoxic drug exposure). (1)(5) • On the basis of expert opinion, an ordered approach to the diagnosis of AKI divides the potential origins into prerenal, intrinsic renal, and postrenal causes. • On the basis of research evidence, expert opinion, and consensus, patients with AKI are subject to a number of fluid and electrolyte disturbances, including hypervolemia, dysnatremias, hyperkalemia, acidosis, and hyperphosphatemia. • On the basis of research evidence, expert opinion, and consensus, indications for renal replacement therapy include volume overload (10%-20% fluid excess), acidosis, hyperkalemia, uremia (typically blood urea nitrogen >100 mg/dL [>35.7 mmol/L] or symptomatic), or an inability to provide adequate nutrition in patients with renal dysfunction. (8)(9) • On the basis of research evidence and expert opinion, recent literature has reported that critically ill children who are discharged after an episode of AKI are at increased risk for chronic kidney disease later in life. Long-term follow-up of these patients is important, but the optimal follow-up plan remains unclear. (10)(11).


Golbus J.R.,University of Michigan | Wojcik B.M.,University of Michigan | Charpie J.R.,Cs Mott Childrens Hospital
Pediatric Cardiology | Year: 2011

Gastrointestinal and feeding complications after the Norwood procedure in infants with hypoplastic left heart syndrome increases morbidity and mortality. These problems are the result of intraoperative challenges, shunt-dependent physiology, and the absence of best-practice guidelines. In response, a systematic review of feeding-related complications and management strategies was performed. A literature search from 1950 to March 2010 identified 21 primary research articles and 4 reviews. Dysphagia, necrotizing enterocolitis (NEC), and poor nutritional status are significant feeding-related complications. Three studies directly compared the modified Blalock-Taussig shunt with the right ventricle-to-pulmonary artery conduit (RV-PA). Patients palliated with either shunt had impaired mesenteric blood flow. Mortality did not differ between shunt types. Three studies demonstrated improved outcomes, e.g., increased survival, decreased incidence of NEC, and decreased median time to recommended daily allowance of calories, with a postoperative feeding algorithm. Two studies showed increased survival between stage I and II surgical palliation after implementation of a home-monitoring system consisting of daily weight and systemic oxygen saturation measurements. The RV-PA shunt does not significantly alter mortality or increase mesenteric blood flow. A postoperative feeding algorithm and a home-monitoring system may improve outcomes and decrease average hospital length of stay (LOS). Additional studies are needed to determine which interventions, as part of a standardized protocol, improve survival and decrease complications. © 2011 Springer Science+Business Media, LLC.


Butcher J.L.,University of Michigan | Butcher J.L.,CS Mott Childrens Hospital | Nasr S.Z.,University of Michigan | Nasr S.Z.,CS Mott Childrens Hospital
Journal of Pediatric Psychology | Year: 2015

Objective Use a standardized system to code parent-child interactions during respiratory treatments for cystic fibrosis (CF) and analyze relations between behaviors during treatments and medical regimen adherence. Methods A total of 15 families (53% girls; M age = 8.9 years; SD = 1.8) had three respiratory treatments recorded in the home environment and coded. Families provided six 24-hr recalls of child medical regimen activities, and electronic airway clearance time was recorded over 3 months to measure medical regimen adherence. Results Parent positive attention, instructions, and avoidance of negative statements were significantly related to child cooperation during respiratory treatments. Parental presence, positive attention, instructions, and child cooperation during treatments were related to higher respiratory adherence rates. Conclusions Direct observation methodology has led to effective nutritional adherence intervention for children with CF. These preliminary data demonstrate that an observational method could also be used to develop interventions to promote respiratory medication adherence. © The Author 2014. Published by Oxford University Press on behalf of the Society of Pediatric Psychology.


Twin-twin transfusion syndrome (TTTS) is a complication of multiple gestation in which arteriovenous vascular communications occur in a shared placenta. Due to these communications, there is an imbalance of blood flow between the developing fetuses. This results in 1 twin becoming the donor and the other becoming the recipient. In severe cases, the recipient may experience polycythemia, polyhydramnios, and hydrops fetalis, while in the donor oligohydramnios and severe anemia may develop. It has been reported that fetal mortality can reach as high as 60% to 80% if TTTS develops before 26 weeks' gestation and goes untreated. Therapeutic options available include decompression amniocentesis, amniotic septostomy, interruption of the placental vessel communications, and selective fetal reduction. Selective fetoscopic laser photocoagulation of abnormal vascular communications has shown to result in increasing survival rates and has become a definitive treatment option for severe TTTS. The case report presented involves a 32-year-old, gravida 5, para 2 patient at 19 weeks' estimated gestational age with a monochorionic diamniotic twin gestation with TTTS diagnosed using ultrasonography presenting for an elective laparoscopic fetal laser photocoagulation.


Li Y.,Cs Mott Childrens Hospital | Glotzbecker M.,Childrens Hospital Boston | Hedequist D.,Childrens Hospital Boston
Current Reviews in Musculoskeletal Medicine | Year: 2012

The incidence of surgical site infection (SSI) after spinal deformity surgery for adolescent idiopathic scoliosis ranges from 0.5-6.7%. The risk of infection following spinal fusion in patients with neuromuscular scoliosis is greater, with reported rates of 6.1-15.2% for cerebral palsy and 8-41.7% for myelodysplasia. SSIs result in increased patient morbidity, multiple operations, prolonged hospital stays, and significant financial costs. Recent literature has focused on elucidating the most common organisms involved in SSIs, as well as identifying modifiable risk factors and prevention strategies that may decrease the rates of infection. These include malnutrition, positive urine cultures, antibiotic prophylaxis, surgical site antisepsis, antibiotic-loaded allograft, local application of antibiotics, and irrigation solutions. Acute and delayed SSIs are managed differently. Removal of instrumentation is required for effective treatment of delayed SSIs. This review article examines the current literature on the prevention and management of SSIs after pediatric spinal deformity surgery. © Springer Science+Business Media, LLC 2012.


Li Y.,Cs Mott Childrens Hospital | Gold M.,Childrens Hospital Boston | Karlin L.,Childrens Hospital Boston
Spine Deformity | Year: 2013

Study Design Retrospective review of patients who had undergone vertical expandable prosthetic titanium rib (VEPTR) treatment at a single institution. Objectives To evaluate whether clinically significant proximal junctional kyphosis (PJK) occurs after VEPTR insertion. Summary of Background Data PJK is a potential problem after posterior spinal instrumentation and fusion. PJK after VEPTR insertion has not been well-described. Methods A total of 68 patients underwent VEPTR treatment between 1999 and 2009. Diagnosis, age at time of VEPTR insertion, location of VEPTR anchors, preoperative and postoperative scoliosis, T2-T12 kyphosis and PJK, time from VEPTR insertion to development of PJK, revision procedure for significant PJK, change in PJK after the revision procedure, and PJK at final follow-up were recorded. Results Four patients developed PJK (6%). One patient had congenital scoliosis with rib fusions, 1 had scoliosis associated with a syndrome, and 2 had neuromuscular scoliosis. Mean follow-up was 5.7 years. Average T2-T12 kyphosis and PJK before VEPTR insertion were 77 and 14, respectively. Mean T2-T12 kyphosis and PJK after VEPTR insertion were 63 and 33, respectively. Average T2-T12 kyphosis and PJK before the recommended revision procedure for treatment of PJK were 89 and 53, respectively. All patients developed PJK within the first year after VEPTR insertion. Two patients underwent revision to growing rods. One of these patients had preoperative halo-gravity traction. Mean PJK in these 2 patients improved from 39 to 18 after revision and remained stable at 19 at an average follow-up of 2.9 years. Conclusions PJK after VEPTR insertion can occur. Patients with preoperative thoracic hyperkyphosis may be at higher risk. PJK can develop within the first year of VEPTR treatment, and can become progressive and severe enough to require complex interventions. In this small case series, patients were revised to growing rods. © 2013 Scoliosis Research Society.


Parrott L.M.,Cs Mott Childrens Hospital
AANA Journal | Year: 2013

Pain protects the body from damaging effects of harmful stimuli. Congenital insensitivity to pain is a rare inherited disorder characterized by diminished or absent sensitivity to pain, touch, and pressure that leads to frequent trauma and self-mutilation. The disorder is part of the hereditarysensory and autonomic neuropathy (HSAN) family, in which 5 types have been recognized. Research and case reports of anesthetic risks and analgesic needs of these patients is limited due to the infrequent nature of the disorder. Recommendations for anesthesia include modification of intraoperative opioid requirements, use of anesthetics to ensure cooperation and immobility,and intraoperative temperature monitoring. It is imperative for anesthesia providers to understand which type of HSAN their patient experiences and toconduct a thorough preoperative interview because a different interpretation of sensory loss may occur in each HSAN category. This article reports thecase of a patient with HSAN type 2 who presented for knee arthroscopy.


Bruch S.W.,CS Mott Childrens Hospital | Hirschl R.B.,CS Mott Childrens Hospital | Coran A.G.,CS Mott Childrens Hospital
Journal of Pediatric Surgery | Year: 2010

Purpose: This review provides a blueprint to deal with the diagnosis and management of recurrent tracheoesophageal fistulas. Methods: A retrospective review over 27 years found 26 recurrent tracheoesophageal fistulas. Descriptive statistical analyses were performed. Results: In this cohort of 26 patients, 18 had a leak after their primary operation; and 22 had respiratory symptoms leading to the discovery of the recurrent fistula. The diagnosis was made by contrast study in 24. The repairs entailed placing a catheter through the fistula; separating the trachea and esophagus using sharp dissection; and placing tissue, preferably pericardium, between the suture lines. Postoperative complications included 7 anastamotic leaks, 4 strictures, and 3 recurrent fistulas. Long-term follow-up (median of 84 months) showed that 21 took all of their nutrition by mouth, 3 were tube fed, and 2 required a combination. Of the 23 patients with growth chart data, 16 fell in the first quartile of the growth chart, whereas none fell between the 75th and 100th percentile. Conclusion: This series, the largest to date, describes characteristics of recurrent tracheoesophageal fistulas, including techniques to make the diagnosis and provide a secure closure of the fistula, and the long-term outcomes of these patients. © 2010.

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