Publications by authors named "Hitesh Shah"

141 Publications

The spectrum of tibial pseudarthrosis with constriction band syndrome in children.

J Pediatr Orthop B 2021 Feb 17. Epub 2021 Feb 17.

aRainbow Superspeciality Hospital and Children's Orthopaedic Centre, Honorary Paediatric Orthopaedic Surgeon, Department of Orthopaedics, NHL Municipal Medical College, Ahmedabad, Gujarat, bDepartment of Orthopaedics, Paediatric Orthopaedics services, Kasturba Medical College, Manipal Academy of Higher Education, Manipal cGovernment Medical College Hospital, Chandigarh, India.

Objective: Constriction band syndrome (CBS) may be rarely associated with pseudarthrosis of tibia. Published literature includes only case reports. We evaluated the outcome of children with frank pseudarthrosis of the tibia with CBS and compared our results with other reported cases. We aimed to formulate a clinical classification of tibial involvement in CBS on the basis of the treatment guidelines.

Material And Methods: Seven patients with the presence of one or more constriction bands and radiological signs of tibial pseudarthrosis were included in the study. Eight reported cases of CBS with tibial involvement with management and follow-up details were reviewed.

Results: Only two tibial pseudarthrosis united spontaneously after the release of the constriction band. Two patients underwent simultaneous correction of tibial deformity and nailing with band release, one of which needed bone grafting for the pseudarthrosis union. Three patients subsequently needed an excision of tibial pseudarthrosis with nailing to achieve tibial union, two of them had autologous bone grafting along with nailing. Additional procedures were required for the correction of foot deformity and limb length discrepancy. Our findings were similar to the reported cases.

Conclusion: The pseudarthrosis of the tibia with CBS does not always heal following band release. Additional surgeries may be required for persistent pseudarthrosis, limb length discrepancy and residual foot deformities. A functional and radiological classification is proposed for the treatment of tibial pseudarthrosis with CBS.
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http://dx.doi.org/10.1097/BPB.0000000000000856DOI Listing
February 2021

Prevalence and outcomes of hyponatremia and hypernatremia in patients hospitalized with COVID-19.

Nephrol Dial Transplant 2021 Mar 16. Epub 2021 Mar 16.

Division of Kidney Diseases and Hypertension, Department of Medicine, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Great Neck, NY, USA.

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http://dx.doi.org/10.1093/ndt/gfab067DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7989196PMC
March 2021

Valgus-impacted fracture of neck of femur in a 12-year-old child.

BMJ Case Rep 2021 Mar 8;14(3). Epub 2021 Mar 8.

Orthopaedics, Kasturba Medical College, Manipal Academy of Higher Education, Manipal, Manipal, Karnataka, India

Though uncommon, fracture of neck of femur in children is a devastating injury due to the complications it may cause. Treatment depends on the age of the child, the displacement of the fracture and the type of fracture based on Delbet classification. Surgical treatment is indicated in displaced fractures. We report a case of an impacted fracture of neck of femur in a 12-year-old girl. The girl was managed non-operatively. The fracture united uneventfully. An impacted fracture of neck of femur is common in the adult population. To the best of our knowledge, this fracture pattern has not been reported in the paediatric age group.
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http://dx.doi.org/10.1136/bcr-2020-240707DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7942265PMC
March 2021

Impact of the COVID-19 Pandemic on Nephrology Fellow Training and Well-Being in the United States: A National Survey.

J Am Soc Nephrol 2021 Mar 3. Epub 2021 Mar 3.

Division of Nephrology, Department of Medicine, Johns Hopkins University School of Medicine; and Department of Epidemiology, Johns Hopkins Bloomberg School of Public Health, Baltimore, Maryland.

Background: The coronavirus disease 2019 (COVID-19) pandemic's effects on nephrology fellows' educational experiences, preparedness for practice, and emotional wellbeing are unknown.

Methods: We recruited current adult and pediatric fellows and 2020 graduates of nephrology training programs in the United States to participate in a survey measuring COVID-19's effects on their training experiences and wellbeing.

Results: Of 1005 nephrology fellows-in-training and recent graduates, 425 participated (response rate 42%). Telehealth was widely adopted (90% for some or all outpatient nephrology consults), as was remote learning (76% of conferences were exclusively online). Most respondents (64%) did not have in-person consults on COVID-19 inpatients; these patients were managed by telehealth visits (27%), by in-person visits with the attending faculty without fellows (29%), or by another approach (9%). A majority of fellows (84%) and graduates (82%) said their training programs successfully sustained their education during the pandemic, and most fellows (86%) and graduates (90%) perceived themselves as prepared for unsupervised practice. Although 42% indicated the pandemic had negatively affected their overall quality of life and 33% reported a poorer work-life balance, only 15% of 412 respondents who completed the Resident Well-Being Index met its distress threshold. Risk for distress was increased among respondents who perceived the pandemic had impaired their knowledge base (odds ratio [OR], 3.04; 95% confidence interval [CI], 2.00 to 4.77) or negatively affected their quality of life (OR, 3.47; 95% CI, 2.29 to 5.46) or work-life balance (OR, 3.16; 95% CI, 2.18 to 4.71).

Conclusions: Despite major shifts in education modalities and patient care protocols precipitated by the COVID-19 pandemic, participants perceived their education and preparation for practice to be minimally affected.
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http://dx.doi.org/10.1681/ASN.2020111636DOI Listing
March 2021

Comparison of outcome between idiopathic and non-idiopathic congenital vertical talus treated with soft tissue release.

J Pediatr Orthop B 2020 Oct 29. Epub 2020 Oct 29.

Paediatric Orthopaedic Service, Kasturba Medical College, Manipal Academy of higher education, Manipal, Karnataka, India.

The aim of the study was to compare the clinical, radiological and functional outcomes between idiopathic and non-idiopathic congenital vertical talus (CVT) treated with soft tissue release. All the consecutive cases with CVT treated with soft tissue release were prospectively followed up. Indication for soft tissue release was either late presented or failed serial manipulation and cast application. Nineteen children (33 feet) with a minimum follow-up of 2 years following the soft tissue release (the mean 5.5 years follow-up) were included. The children without any other joint involvement with the normal spine without syndromic association were categorized as idiopathic (15 feet) and the rest were categorized as non-idiopathic (18 feet). Clinical and radiological scoring was done using Adelaar and Kodros scores. The morphological, functional and radiological outcomes were assessed using the International Clubfoot Study Group evaluation scale. The clinical, radiological and functional outcomes of the idiopathic and non-idiopathic groups were compared. The range of motion was significantly higher in the idiopathic group. The functional outcome was satisfactory in both groups. All the radiological parameters improved significantly in both groups. The functional outcome was considerably better in the idiopathic group. There was no significant difference in clinical and radiological outcomes among the two groups. Soft tissue release is an effective surgery for deformity correction in children with CVT. A good clinical outcome is observed in both groups. The range of motion was significantly higher in the idiopathic group. The overall outcome was similar in both groups.
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http://dx.doi.org/10.1097/BPB.0000000000000815DOI Listing
October 2020

Congenital pseudoarthrosis of the radius in Neurofibromatosis Type 1: An entity not to be missed!

J Orthop 2020 Nov-Dec;22:427-430. Epub 2020 Sep 23.

Department of Orthopedics, Kasturba Medical College, Manipal Academy of Higher Education, Manipal, Karnataka, 576104, India.

Congenital pseudoarthrosis of the radius is exceedingly rare. We report an isolated pseudoarthrosis of the radius in a five-year-old girl child with Neurofibromatosis type-I (NF-I). She underwent excision of the pseudoarthrosis tissue, ulna osteotomy, iliac crest cortico-cancellous bone grafting, and K wire fixation of both bones. A sound union of the radius and the ulna was achieved after 4 months. At two-year follow-up, mild shortening of the forearm persisted with maintenance of sound union of both bones. Accurate diagnosis of the condition is central in avoiding complications and the need for complex surgical procedures.
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http://dx.doi.org/10.1016/j.jor.2020.09.013DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7527621PMC
September 2020

Comparison of plaster-of-Paris casts and Woodcast splints for immobilization of the limb during serial manipulation and casting for idiopathic clubfoot in infants.

Bone Joint J 2020 Oct;102-B(10):1399-1404

Department of Orthopaedics, Aster Medcity, Kochi, India.

Aims: The study was undertaken to compare the efficacy of Woodcast splints and plaster-of-Paris casts in maintaining correction following sequential manipulation of idiopathic clubfeet.

Methods: In this randomized prospective trial, 23 idiopathic clubfeet were immobilized with plaster-of-Paris casts and 23 clubfeet were immobilized with a splint made of Woodcast that encircled only two-thirds the circumference of the limb. The number of casts or splints needed to obtain full correction, the frequency of cast or splint-related complications, and the time taken for application and removal of the casts and splints were compared.

Results: The mean number of casts required to obtain full correction of the deformity (Pirani Score 0) was 4.35 (95% confidence interval (CI) 3.74 to 4.95) when plaster-of-Paris was used and 4.87 (95% CI 4.33 to 5.41) when the Woodcast splint was used (p = 0.190). The time required for application and removal of the Woodcast splint were significantly less than that required for application and removal of plaster-of-Paris casts (p < 0.001). Woodcast splint-related complications were not more frequent than plaster-of-Paris cast related complications.

Conclusion: Though Woodcast splints covering two-thirds of the circumference of the lower limbs of infants were effective in maintaining the correction of clubfoot deformity during serial manipulation and casting treatment, the superiority of Woodcasts over plaster-of-Paris could not be established. Cite this article: 2020;102-B(10):1399-1404.
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http://dx.doi.org/10.1302/0301-620X.102B10.BJJ-2020-0181.R4DOI Listing
October 2020

Outcomes Among Patients Hospitalized With COVID-19 and Acute Kidney Injury.

Am J Kidney Dis 2021 02 19;77(2):204-215.e1. Epub 2020 Sep 19.

Division of Kidney Diseases and Hypertension, Department of Medicine, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Great Neck, NY. Electronic address:

Rationale & Objective: Outcomes of patients hospitalized with coronavirus disease 2019 (COVID-19) and acute kidney injury (AKI) are not well understood. The goal of this study was to investigate the survival and kidney outcomes of these patients.

Study Design: Retrospective cohort study.

Setting & Participants: Patients (aged≥18 years) hospitalized with COVID-19 at 13 hospitals in metropolitan New York between March 1, 2020, and April 27, 2020, followed up until hospital discharge.

Exposure: AKI.

Outcomes: Primary outcome: in-hospital death.

Secondary Outcomes: requiring dialysis at discharge, recovery of kidney function.

Analytical Approach: Univariable and multivariable time-to-event analysis and logistic regression.

Results: Among 9,657 patients admitted with COVID-19, the AKI incidence rate was 38.4/1,000 patient-days. Incidence rates of in-hospital death among patients without AKI, with AKI not requiring dialysis (AKI stages 1-3), and with AKI receiving dialysis (AKI 3D) were 10.8, 31.1, and 37.5/1,000 patient-days, respectively. Taking those without AKI as the reference group, we observed greater risks for in-hospital death for patients with AKI 1-3 and AKI 3D (HRs of 5.6 [95% CI, 5.0-6.3] and 11.3 [95% CI, 9.6-13.1], respectively). After adjusting for demographics, comorbid conditions, and illness severity, the risk for death remained higher among those with AKI 1-3 (adjusted HR, 3.4 [95% CI, 3.0-3.9]) and AKI 3D (adjusted HR, 6.4 [95% CI, 5.5-7.6]) compared with those without AKI. Among patients with AKI 1-3 who survived, 74.1% achieved kidney recovery by the time of discharge. Among those with AKI 3D who survived, 30.6% remained on dialysis at discharge, and prehospitalization chronic kidney disease was the only independent risk factor associated with needing dialysis at discharge (adjusted OR, 9.3 [95% CI, 2.3-37.8]).

Limitations: Observational retrospective study, limited to the NY metropolitan area during the peak of the COVID-19 pandemic.

Conclusions: AKI in hospitalized patients with COVID-19 was associated with significant risk for death.
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http://dx.doi.org/10.1053/j.ajkd.2020.09.002DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7833189PMC
February 2021

Demographics and Clinical Presentation of Early-Stage Legg-Calvé-Perthes Disease: A Prospective, Multicenter, International Study.

J Am Acad Orthop Surg 2021 Jan;29(2):e85-e91

From Gillette Children's Specialty Healthcare, Saint Paul, MN (Dr. Laine and Dr. Novotny), the Department of Orthopaedic Surgery, University of Minnesota, Minneapolis, MN (Dr. Laine), the Rehabilitation Science Graduate Program, University of Minnesota, Minneapolis, MN (Dr. Novotny), the Department of Orthopaedic Surgery, John Hopkins School of Medicine, Baltimore, MD (Dr. Tis), the Division of Orthopaedic Surgery, Children's Hospital of Philadelphia, Philadelphia, PA (Dr. Sankar), the Division of Orthopaedic Surgery and Sports Medicine, Children's National Hospital, Washington, DC (Dr. Martin), Campbell Clinic Orthopedics (Dr. Kelly), the Department of Orthopedics and Biomechanical Engineering, University of Tennessee (Dr. Kelly), and Le Bonheur Children's Hospital (Dr. Kelly), Memphis, TN, Childrens of Alabama (Dr. Gilbert), and the Department of Orthopaedic Surgery, University of Alabama Birmingham (Dr. Gilbert), Birmingham, AL, the Department of Orthopaedics, Paediatric Orthopaedic Service, Kasturba Medical College, MAHE, Manipal, India (Dr. Shah), Aster Medcity, Kuttisahib Road, Cheranalloor, South Chittoor, Kochi, Kerala, India (Dr. Joseph), Center for Excellence in Hip Disorders, Scottish Rite for Children (Dr. Kim), and the Department of Orthopaedic Surgery, University of Texas Southwestern Medical Center (Dr. Kim), Dallas, TX.

Background: Children with Legg-Calvé-Perthes disease (LCPD) are classically described as small, thin, high-energy children presenting with a painless limp. Epidemiologic studies have historically been retrospective and regional in nature. The purpose of this study was to determine the demographic and clinical features of children presenting in the early stages of LCPD in an international, multicenter cohort.

Methods: Children (6 to 10 years) in the early stages of LCPD (modified Waldenström stage I to IIa) were enrolled in a prospective, multicenter study. Demographic and clinical data at presentation were analyzed. Body mass index percentiles were determined using country-specific growth charts for children in the United States and India, two countries with largest enrollment. Statistical analyses included t-tests and chi-square.

Results: A total of 209 children (86% males; mean age 7.9 ± 1.2 years) from 25 centers (six countries) were included. Eight-four percent of children presented with pain with or without a limp. Average pain score at presentation was 3 ± 2 (range 0 to 9), and 63% of children (n = 105) used pain medications. Of these children 65% required medication more than once per week. Thirty percent of children missed school due to pain in the past month, and of those, 74% missed at least 1 day per week. Twenty-nine percent of children from the United States and 20% of children from India were overweight or obese. Nineteen percent reported household smoking.

Discussion: This prospective study provides a new international multicenter representation of early LCPD. The frequency of pain and missed school highlights the substantial morbidity and potential social cost and burden for children and families. The prevalence of being overweight/obese in our LCPD cohort was comparable to rates within the pediatric cohort as a whole, and fewer children have a history of smoke exposure than in previous reports.

Level Of Evidence: Level II, prospective comparative study.
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http://dx.doi.org/10.5435/JAAOS-D-19-00379DOI Listing
January 2021

ANCA-Associated Vasculitis With Glomerulonephritis in COVID-19.

Kidney Int Rep 2020 Nov 20;5(11):2079-2083. Epub 2020 Aug 20.

Division of Kidney Diseases and Hypertension, Glomerular Center at Northwell Health, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Northwell Health, Great Neck, New York, USA.

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http://dx.doi.org/10.1016/j.ekir.2020.08.012DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7439090PMC
November 2020

Surgical management of the congenital dislocation of the knee and hip in children presented after six months of age.

Int Orthop 2020 12 8;44(12):2635-2644. Epub 2020 Aug 8.

Department of Orthopaedics, Kasturba Hospital, Kasturba Medical College, Manipal Academy of Higher Education, Manipal, 576104, India.

Purpose: Congenital dislocation of the knee and hip is a rare congenital disorder. The specific aim of the study was to evaluate the clinical and radiological outcomes of the children with congenital dislocation of the knee and hip who presented after six months of age.

Methods: All the consecutive children with congenital dislocation of the knee and hip joints were retrospectively reviewed. We included cases that were treated after six months of age and followed up for a minimum of two years. Twenty-four children with congenital dislocation of the knee and hip (thirteen with ligamentous laxity, eleven children with stiff joints) were included. The knee was dislocated in 45 limbs; the hip was dislocated in 40 instances. The knee joint dislocation was treated with quadricepsplasty in all twenty-four children (45 knees). The hip dislocation (n = 32) was addressed with either closed reduction (n = 8) or open reduction of the hip (n = 24). Eight hip dislocations were not addressed. The outcome of the hip and knee was evaluated.

Results: The clinical and radiological outcomes were better in children with ligamentous laxity than without laxity. Twenty-two children were community walkers. An orthosis was needed in eight children. The frequency of spontaneous reduction of unreduced dislocation of the hip was noted in three children (5/8 hips).

Conclusion: Outcome in combined dislocation of knee and hip is good in most cases with surgical interventions. The outcome is better in children with ligamentous laxity. Spontaneous reduction of the dislocated hips might be achieved after gaining knee flexion following knee surgery for congenital the knee in a few cases.
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http://dx.doi.org/10.1007/s00264-020-04759-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7679307PMC
December 2020

Inflammation and Erythropoiesis-Stimulating Agent Hyporesponsiveness: A Critical Connection.

Kidney Med 2020 May-Jun;2(3):245-247. Epub 2020 May 11.

Donald and Barbara Zucker School of Medicine at Hofstra, Great Neck, NY.

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http://dx.doi.org/10.1016/j.xkme.2020.05.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7380430PMC
May 2020

Orthopedic manifestation as the presenting symptom of acute lymphoblastic leukemia.

J Orthop 2020 Nov-Dec;22:326-330. Epub 2020 Jun 7.

Department of Orthopaedics, Kasturba Hospital, Kasturba Medical College, Manipal Academy of Higher Education, Manipal, India.

Introduction: The diagnosis of Acute lymphoblastic leukemia (ALL) is delayed due to vague presentation and normal hematological investigations.

Objective: The objectives were to identify the frequency of ALL cases presented to the orthopedic department and with normal hematological investigations.

Material And Methods: 250 consecutive ALL cases were retrospectively evaluated to identify cases with musculoskeletal manifestations, and laboratory investigations.

Results: Twenty-two patients (4- vertebral compression fractures, 12- joint pain, 6- bone pain), presented primarily to the orthopedic department. Six patients had a normal peripheral smear.

Conclusion: The primary physician should maintain a high index of suspicion despite a normal peripheral smear.
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http://dx.doi.org/10.1016/j.jor.2020.05.022DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7340973PMC
June 2020

COVID-19-Associated Kidney Injury: A Case Series of Kidney Biopsy Findings.

J Am Soc Nephrol 2020 Sep 13;31(9):1948-1958. Epub 2020 Jul 13.

Department of Pathology, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Manhasset, New York.

Background: Reports show that AKI is a common complication of severe coronavirus disease 2019 (COVID-19) in hospitalized patients. Studies have also observed proteinuria and microscopic hematuria in such patients. Although a recent autopsy series of patients who died with severe COVID-19 in China found acute tubular necrosis in the kidney, a few patient reports have also described collapsing glomerulopathy in COVID-19.

Methods: We evaluated biopsied kidney samples from ten patients at our institution who had COVID-19 and clinical features of AKI, including proteinuria with or without hematuria. We documented clinical features, pathologic findings, and outcomes.

Results: Our analysis included ten patients who underwent kidney biopsy (mean age: 65 years); five patients were black, three were Hispanic, and two were white. All patients had proteinuria. Eight patients had severe AKI, necessitating RRT. All biopsy samples showed varying degrees of acute tubular necrosis, and one patient had associated widespread myoglobin casts. In addition, two patients had findings of thrombotic microangiopathy, one had pauci-immune crescentic GN, and another had global as well as segmental glomerulosclerosis with features of healed collapsing glomerulopathy. Interestingly, although the patients had confirmed severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection by RT-PCR, immunohistochemical staining of kidney biopsy samples for SARS-CoV-2 was negative in all ten patients. Also, ultrastructural examination by electron microscopy showed no evidence of viral particles in the biopsy samples.

Conclusions: The most common finding in our kidney biopsy samples from ten hospitalized patients with AKI and COVID-19 was acute tubular necrosis. There was no evidence of SARS-CoV-2 in the biopsied kidney tissue.
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http://dx.doi.org/10.1681/ASN.2020050699DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7461689PMC
September 2020

Roxadustat for Anemia in Patients with Chronic Kidney Disease.

N Engl J Med 2020 07;383(1):e3

Donald and Barbara Zucker School of Medicine at Hofstra-Northwell, Great Neck, NY

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http://dx.doi.org/10.1056/NEJMc1913712DOI Listing
July 2020

Does the timing of treatment affect complications of pediatric femoral neck fractures?

J Orthop 2020 Nov-Dec;22:207-212. Epub 2020 May 6.

Department of Orthopaedics, Kasturba Hospital, Kasturba Medical College, Manipal Academy of Higher Education, Manipal, India.

Objectives: Pediatric femoral neck fractures were reviewed to compare complications with the time of presentation.

Methods: 34 pediatric femoral neck fractures were studied to detect union time, avascular necrosis, premature physis fusion, and the neck-shaft angle. All complications were comparable between both groups.

Results: The mean union time was more in late presented group. Four children developed AVN. The neck shaft angle and pre mature fusion of growth plate were same in both groups.

Conclusions: The frequency of the complications except union time in pediatric neck femur fracture is comparable in early and late presented groups.
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http://dx.doi.org/10.1016/j.jor.2020.04.023DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7225590PMC
May 2020

Acute kidney injury in patients hospitalized with COVID-19.

Kidney Int 2020 07 16;98(1):209-218. Epub 2020 May 16.

Division of Kidney Diseases and Hypertension, Department of Medicine, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Great Neck, New York, USA. Electronic address:

The rate of acute kidney injury (AKI) associated with patients hospitalized with Covid-19, and associated outcomes are not well understood. This study describes the presentation, risk factors and outcomes of AKI in patients hospitalized with Covid-19. We reviewed the health records for all patients hospitalized with Covid-19 between March 1, and April 5, 2020, at 13 academic and community hospitals in metropolitan New York. Patients younger than 18 years of age, with end stage kidney disease or with a kidney transplant were excluded. AKI was defined according to KDIGO criteria. Of 5,449 patients admitted with Covid-19, AKI developed in 1,993 (36.6%). The peak stages of AKI were stage 1 in 46.5%, stage 2 in 22.4% and stage 3 in 31.1%. Of these, 14.3% required renal replacement therapy (RRT). AKI was primarily seen in Covid-19 patients with respiratory failure, with 89.7% of patients on mechanical ventilation developing AKI compared to 21.7% of non-ventilated patients. 276/285 (96.8%) of patients requiring RRT were on ventilators. Of patients who required ventilation and developed AKI, 52.2% had the onset of AKI within 24 hours of intubation. Risk factors for AKI included older age, diabetes mellitus, cardiovascular disease, black race, hypertension and need for ventilation and vasopressor medications. Among patients with AKI, 694 died (35%), 519 (26%) were discharged and 780 (39%) were still hospitalized. AKI occurs frequently among patients with Covid-19 disease. It occurs early and in temporal association with respiratory failure and is associated with a poor prognosis.
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http://dx.doi.org/10.1016/j.kint.2020.05.006DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7229463PMC
July 2020

Evolution of Legg-Calvé-Perthes disease following proximal femoral varus osteotomy performed in the avascular necrosis stage:a prospective study.

J Child Orthop 2020 Feb;14(1):58-67

Scottish Rite Hospital for Children, Department of Orthopaedic Surgery, UT Southwestern Medical Center, Dallas, Texas, United States.

Purpose: This prospective study was undertaken to describe patterns of fragmentation of the femoral epiphysis following a proximal femoral varus osteotomy (PFVO) done during stage I of LCPD and to assess the disease duration and outcome in each pattern.

Methods: A total of 25 children treated by a PFVO in stage I of LCPD were followed until healing. The MRI Perfusion Index, radiographic changes in the femoral epiphysis, disease duration and the Sphericity Deviation Score (SDS) at healing were documented. The reproducibility of classification of the pattern of fragmentation, estimation of disease duration and SDS were assessed. The duration of the disease and SDS in the patterns of fragmentation were compared.

Results: Four patterns of fragmentation were noted, namely, typical fragmentation, bypassing fragmentation, abortive fragmentation and atypical fragmentation with horizontal fissuring. The reproducibility of classifying the pattern of fragmentation was moderate (Kappa: 0.48) while the reproducibility of other continuous variables was excellent. The Perfusion Index was less than 50% in every affected hip. The duration of the disease and SDS were lowest in children in whom the stage of fragmentation was bypassed but these differences were not statistically significant.

Conclusion: Following a proximal femoral osteotomy during stage I of LCPD the fragmentation stage may be bypassed partially or completely and the chances of a good outcome appear to be very good if fragmentation is bypassed.

Level Of Evidence: Level II Prognostic Study.
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http://dx.doi.org/10.1302/1863-2548.14.190153DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7043118PMC
February 2020

A rare case of rubber band syndrome of wrist with distal radius and ulna fracture.

J Orthop 2020 Jul-Aug;20:60-62. Epub 2020 Jan 10.

Department of Orthopedics, Kasturba Medical College, Manipal Academy of Higher Education, Manipal, Karnataka, 576104, India.

Rubber band syndrome (Dhaga Syndrome) is a very rare condition seen mostly among children in whom elastic bands worn around the wrist or forearm for religious or decorative purpose and its presence is overlooked by the parents. These forgotten bands slowly cuts through the skin and soft tissue underneath causing constriction and damage to the underlying structures. Circumferential constricting scar with discharging sinus is the cardinal feature. We report a case of rubber band syndrome in a 2 year old boy in which the constriction was severe enough to cause fracture of the distal radius and ulna.
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http://dx.doi.org/10.1016/j.jor.2020.01.010DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7000557PMC
January 2020

Hemiepiphysiodesis using 2-holed reconstruction plate for correction of angular deformity of the knee in children.

J Orthop 2020 Jul-Aug;20:54-59. Epub 2020 Jan 10.

Department of Orthopedics, Kasturba Medical College, Manipal Academy of Higher Education, Manipal, Karnataka, India.

Background: This study assesses the outcome of hemi epiphysiodesis with a 2-holed reconstruction plate using the tension band principle in angular deformities of the knee and variables influencing the outcome.

Methods: Prospective study from 2012 to 2016 included 23 children with coronal deformities of the knee joint. IMD, ICD, BMI, mLDFA, mMPTA, mTFA, Mechanical axis deviation, Perpendicular mechanical axis ratio, and screw angle measured. Correction and rate of correction compared with BMI, age, etiology, PMAR and MAD, deformity, a segment of bone and the screw angle.

Results: Total 65 segments operated in 23 children with 40 knees. Out of the total 65, 36 plates were kept in distal femur physis and 29 in the proximal tibial physis. Thirteen knees had genu varum and 27 had genu valgum. The mean age at the time of surgery was 11.25 months. The mean follow-up of 36 months and the average time for correction was 18.64 months. 77.5% correction achieved with ROC maximum in the initial 6 months. ROC in TFA per year were 7.8(femur), 5(tibia), and 12(femur with tibia). Complications included broken implant, screw back out, screw irritation and rebound phenomenon.

Conclusions: 2-holed reconstruction plate gives similar correction to that of the 8-plate within a comparable period without additional complications. It has the advantage of being cost effective and readily available. Age, BMI, type of deformity, amount of initial deformity, do not play a significant role in the outcome of correction. Screw placement may have a role in the final correction of the deformity.

Level Of Evidence: Level 3.
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http://dx.doi.org/10.1016/j.jor.2020.01.001DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7000426PMC
January 2020

Trends in the Proportions of Women Program Chairs, Moderators, and Speakers at American Society of Nephrology Kidney Week 2011-2019.

Clin J Am Soc Nephrol 2020 02 23;15(2):265-266. Epub 2020 Jan 23.

Division of Kidney Diseases and Hypertension, North Shore University Hospital and Long Island Jewish Medical Center, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Great Neck, New York

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http://dx.doi.org/10.2215/CJN.08140719DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC7015095PMC
February 2020

Age- and gender-related reference ranges for thromboelastography from a healthy Indian population.

Int J Lab Hematol 2020 Apr 31;42(2):180-189. Epub 2019 Dec 31.

Manipal College of Nursing, Manipal Academy of Higher Education, Manipal, India.

Introduction: Thromboelastography (TEG) is a whole blood clotting assay largely used in major surgeries and trauma to monitor patients' in vivo hemostatic status. Standardization of kaolin-activated citrated whole blood thromboelastography is not done in the Indian population. This study primarily aims to derive reference ranges of kaolin-activated TEG for healthy volunteers in the Indian population. Secondarily, it aims to study the age- and gender-related hemostatic changes in the study population.

Methods: A total of 120 healthy volunteers were enrolled (55 adult males, 32 adult females, and 33 children). The volunteers were interviewed for any bleeding history or drug intake which affects coagulation. Kaolin-activated TEG was performed on citrated whole blood, and parameters including R-time, K-time, angle, MA, LY30, and CI were analyzed.

Results: Derived reference range for total volunteers irrespective of age and sex were as follows: R-time: 3.8-10.6, K-time: 1.2-3.1, angle: 44.9-72.0, MA: 41.2-64.5, LY30: 0-9.9, and CI: -3.7 to 3.4. Statistically significant difference was observed in different age and sex groups for R-time, K-time, and angle. About 40% of the volunteers had at least one abnormal parameter according to the manufacturer's reference range which decreased to 12.5% when the derived reference ranges were considered.

Conclusion: Gender- and age-related variances were observed in reference ranges of our population and which was also differed from the other ethnic population. Many of our healthy volunteers were categorized as coagulopathic when manufacturer's reference range was considered. So, it is important to derive the reference range of the target population before using the TEG into clinical practice.
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http://dx.doi.org/10.1111/ijlh.13148DOI Listing
April 2020

Biallelic variants p.Arg1133Cys and p.Arg1379Cys in COL2A1: Further delineation of phenotypic spectrum of recessive Type 2 collagenopathies.

Am J Med Genet A 2020 02 22;182(2):338-347. Epub 2019 Nov 22.

Center for Medical Genetics, University of Antwerp and Antwerp University Hospital, Antwerp, Belgium.

The phenotypic spectrum of Type 2 collagenopathies ranges from lethal achondrogenesis Type 2 to milder osteoarthritis with mild chondrodysplasia. All of them are monoallelic except for the two recent reports showing that biallelic variants in COL2A1 can cause spondyloepiphyseal dysplasia congenita in two children. Here we report two additional families with homozygous variants, c.4135C>T (p.Arg1379Cys) and c.3190C>T (p.Arg1133Cys) in COL2A1 resulting in two distinct skeletal dysplasia phenotypes of intermediate severity. Though all six patients from four families exhibit a spondylo-epimetaphyseal dysplasia, they demonstrate a wide variation in severity of short stature and involvement of epiphyses, metaphyses, and vertebrae. We hypothesize that the variants are likely to be hypomorphic, given the underlying mechanisms of disease causation for known heterozygous variants in COL2A1. With this report, we provide further evidence to the existence of autosomal recessive Type 2 collagenopathy.
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http://dx.doi.org/10.1002/ajmg.a.61414DOI Listing
February 2020

Vaccine-associated kidney diseases: A narrative review of the literature.

Saudi J Kidney Dis Transpl 2019 Sep-Oct;30(5):1002-1009

Division of Kidney Diseases and Hypertension, North Shore University Hospital and Long Island Jewish Medical Center, Zucker School of Medicine at Hofstra/Northwell, Great Neck, NY, USA.

Immunization is one of the greatest public health achievements of the 20 century. Vaccines have enabled the eradication of deadly diseases and decreased the morbidity and mortality associated with various infections. Most vaccines are safe to administer and cause only minor side effects. Although very rare, various glomerular diseases and acute kidney injury have been reported following immunization with certain vaccines including influenza, pneumococcal, and hepatitis B vaccines. This review summarizes these rare renal complications that have been published in the literature. Physicians and other health-care providers administrating vaccines should be aware of these very rare but possible renal side effects.
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http://dx.doi.org/10.4103/1319-2442.270254DOI Listing
April 2020

Biosimilar Erythropoiesis-Stimulating Agents in Chronic Kidney Disease.

Adv Chronic Kidney Dis 2019 07;26(4):267-271

Division of Nephrology, Department of Medicine, Donald and Barbara Zucker School of Medicine at Hofstra/Northwell, Great Neck, NY. Electronic address:

Anemia is a well-known complication of advanced CKD and treatment with erythropoietin analogues (epoetin) remains a key component of management. Although biologic agents, including epoetin, play an extremely important role in the treatment of various medical conditions, their cost can be prohibitive. As a result, several biosimilar agents have now been approved by the U.S. Food and Drug Administration. Biosimilar epoetin has been used in Europe since 2007. In this article, we will review biosimilar development and focus on the first approved biosimilar epoetin in the United States, epoetin alfa-epbx.
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http://dx.doi.org/10.1053/j.ackd.2019.04.007DOI Listing
July 2019

Validation of Pediatric Self-Report Patient-Reported Outcomes Measurement Information System (PROMIS) Measures in Different Stages of Legg-Calvé-Perthes Disease.

J Pediatr Orthop 2020 May/Jun;40(5):235-240

Texas Scottish Rite Hospital for Children.

Objectives: Patient-reported outcomes (PRO) assessing health-related quality of life (HRQoL) are important outcome measures, especially in Legg-Calvé-Perthes disease (LCPD) where symptoms (pain and limping), activity restrictions, and treatments vary depending on the stage of the disease. The purpose of this study was to investigate the validity of the Patient-reported Outcomes Measurement Information System (PROMIS) for measuring HRQoL of patients with LCPD in various stages of the disease.

Methods: This is a multicenter validity study. Patients with LCPD between 4 and 18 years old were included and classified into modified Waldenström stages of disease: Early (1 or 2A), Late (2B or 3), or Healed (4). Seven PROMIS domains were collected, including Pain Interference, Fatigue, Mobility, Depression, Anger, Anxiety, and Peer Relationships. Convergent, discriminant, and known group validity was determined.

Results: A total of 190 patients were included (mean age: 10.4±3.1 y). All 7 domains showed the worst scores in patients in the Early stage (known group validity). Within each domain, all domains positively correlated to each other (convergent validity). Patients who reported more anxiety, depression, and anger were associated with decreased mobility and increased fatigue and pain. Peer relationships had no to weak associations with other domains (discriminant validity).

Conclusions: PROMIS has construct validity in measuring the HRQoL of patients in different stages of LCPD, suggesting that PROMIS has potential to serve as a patient-reported outcome tool for this population.

Level Of Evidence: Diagnostic level III study.
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http://dx.doi.org/10.1097/BPO.0000000000001423DOI Listing
October 2020

An emerging ribosomopathy affecting the skeleton due to biallelic variations in NEPRO.

Am J Med Genet A 2019 09 27;179(9):1709-1717. Epub 2019 Jun 27.

Department of Medical Genetics, Kasturba Medical College, Manipal, Manipal Academy of Higher Education, Manipal, India.

Cartilage hair hypoplasia (CHH), anauxetic dysplasia 1, and anauxetic dysplasia 2 are rare metaphyseal dysplasias caused by biallelic pathogenic variants in RMRP and POP1, which encode the components of RNAse-MRP endoribonuclease complex (RMRP) in ribosomal biogenesis pathway. Nucleolus and neural progenitor protein (NEPRO), encoded by NEPRO (C3orf17), is known to interact with multiple protein subunits of RMRP. We ascertained a 6-year-old girl with skeletal dysplasia and some features of CHH. RMRP and POP1 did not harbor any causative variant in the proband. Parents-child trio exomes revealed a candidate biallelic variant, c.435G>C, p.(Leu145Phe) in NEPRO. Two families with four affected individuals with skeletal dysplasia and a homozygous missense variant, c.280C>T, p.(Arg94Cys) in NEPRO, were identified from literature and their published phenotype was compared in detail to the phenotype of the child we described. All the five affected individuals have severe short stature, brachydactyly, skin laxity, joint hypermobility, and joint dislocations. They also have short metacarpals, broad middle phalanges, and metaphyseal irregularities. Protein modeling and stability prediction showed that the mutant protein has decreased stability. Both the reported variants are in the same domain of the protein. Our report delineates the clinical and radiological characteristics of an emerging ribosomopathy caused by biallelic variants in NEPRO.
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http://dx.doi.org/10.1002/ajmg.a.61267DOI Listing
September 2019

Increasing Medical Student Interest in Nephrology.

Am J Nephrol 2019 11;50(1):4-10. Epub 2019 Jun 11.

Division of Nephrology and Transplantation, Maine Medical Center, Portland, Oregon, USA.

Background: Interest in nephrology careers is declining, possibly due to perceptions of the field and/or training aspects. Understanding practices of medical schools successfully instilling nephrology interest could inform efforts to attract leading candidates to the specialty.

Methods: The American Society of Nephrology Workforce Committee's Best Practices Project was one of several initiatives to increase nephrology career interest. Board-certified nephrologists graduating medical school between 2002 and 2009 were identified in the American Medical Association Masterfile and their medical schools ranked by production. Renal educators from the top 10 producing institutions participated in directed focus groups inquiring about key factors in creating nephrology career interest, including aspects of their renal courses, clinical rotations, research activities, and faculty interactions. Thematic content analysis of the transcripts (with inductive reasoning implementing grounded theory) was performed to identify factors contributing to their programs' success.

Results: The 10 schools identified were geographically representative, with similar proportions of graduates choosing internal medicine (mean 26%) as the national graduating class (26% in the 2017 residency Match). Eighteen educators from 9 of these 10 institutions participated. Four major themes were identified contributing to these schools' success: (1) nephrology faculty interaction with medical students; (2) clinical exposure to nephrology and clinical relevance of renal pathophysiology materials; (3) use of novel educational modalities; and (4) exposure, in particular early exposure, to the breadth of nephrology practice.

Conclusion: Early and consistent exposure to a range of clinical nephrology experiences and nephrology faculty contact with medical students are important to help generate interest in the specialty.
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http://dx.doi.org/10.1159/000501058DOI Listing
August 2020

Management of the Knee Problems in Spastic Cerebral Palsy.

Indian J Orthop 2019 Jan-Feb;53(1):53-62

Department of Pediatric Orthopedics, Kasturba Medical College, Manipal Academy of Higher Education, Manipal, Karnataka, India.

Two common knee problems in cerebral palsy are increased knee flexion during stance phase and reduced knee flexion during the swing phase of gait. We reviewed the recent literature and based on that, we formed this review. Hamstring spasticity, quadriceps weakness, soleus weakness, and lever-arm dysfunction are few factors which lead to increased knee flexion during stance phase. Rectus spasticity diminishes knee flexion in the swing. Resulting gait-stiff knee gait interferes with ground clearance. Both gait patterns result into esthetically poor gait and increased energy consumption. Knee flexion gait may lead to pain in the knee. Natural history of knee flexion gait suggests deterioration over time. In the early stage, these gait abnormalities are managed by nonoperative treatment. Cases in which nonoperative measures fail or advance cases need surgical treatment. Various variables which are taken into consideration before selecting a particular treatment option are described. We also present an algorithm for decision-making. Nonsurgical options and surgical procedures are discussed.
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http://dx.doi.org/10.4103/ortho.IJOrtho_339_17DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6394172PMC
March 2019