Hepatic osteodystrophy in chronic cholestasis: evidence for a multifactorial etiology.


:Children with cholestatic liver disease have been thought to develop hepatic osteodystrophy resulting from vitamin D and calcium malabsorption, resulting in secondary hyperparathyroidism and osteomalacia or rickets. However, treatment with vitamin D has not always proven successful in improving the bone disturbance. The aim of our study was to determine the role of vitamin D deficiency in the pathogenesis of hepatic osteodystrophy. We studied five patients, three female and two male, ages 0.9-19 yr, with biopsy-proven chronic cholestatic liver disease and previously low serum levels of vitamin D despite oral intake of vitamin D preparations. Patients were admitted to the Clinical Research Center for 8 days for sunlight deprivation and ultraviolet light substitution and for determinations of serum 25-hyroxyvitamin D(25(OH)) D2 and -D3, osteocalcin, and type I collagen telopeptide (ICTP), the last two being markers of bone formation and resorption, respectively. Samples were taken on admission, at discharge, and 1 month later. Results demonstrated low serum levels of osteocalcin and normal circulating levels of ICTP. Admission serum 25(OH)D2 levels were uniformly low or undetectable and remained so. Admission levels of circulating 25(OH)D3 were normal or low and did not rise during ultraviolet light therapy or subsequent resumption of oral vitamin D therapy and remained low 1 month later. These results indicate that in the face of low-normal to low total 25(OH)D levels, the low osteocalcin and normal ICTP levels suggest that decreased bone formation and not increased bone resorption is the main determinant of bone loss in a subset of children with chronic cholestatic liver disease.


Pediatr Transplant


Klein GL,Soriano H,Shulman RJ,Levy M,Jones G,Langman CB





Has Abstract


2002-04-01 00:00:00














  • Association of mast cells and liver allograft rejection.

    abstract::MCs are important effector cells in a broad range of immune responses. Their role in liver allograft rejection is not clear. Twenty-one liver transplant recipients (mean age +/- s.d.; 10.2 +/- 4.1 yr) who experienced a rejection episode are included in this study. Biopsy specimens from normal livers (allograft biopsy ...

    journal_title:Pediatric transplantation

    pub_type: 杂志文章


    authors: Arikan C,Nart D,Kilic M,Yuksekkaya HA,Aydogdu S

    更新日期:2008-05-01 00:00:00

  • Reduced ATG-F dosage for induction in pediatric renal transplantation: a single-center experience.

    abstract::Rabbit antithymocyte globulin (ATG-F) is an extensively used induction agent. To our knowledge, no study to date has assessed reduced ATG-F dosage in children undergoing renal transplantation. This was a retrospective analysis of pediatric renal recipients in the Department of Kidney Transplantation, The First Affilia...

    journal_title:Pediatric transplantation

    pub_type: 杂志文章


    authors: Shang W,Feng G,Gao S,Wang Z,Pang X,Li J,Liu L,Feng Y,Xie H,Zhang S,Qiao B

    更新日期:2014-05-01 00:00:00

  • Recurrence of non-alcoholic steatohepatitis after liver transplantation in a 13-yr-old boy.

    abstract::Nonalcoholic steatohepatitis (NASH) is the most severe form of non-alcoholic fatty liver disease (NAFLD). The aim of our study was to highlight NASH as a rare but possible problem in children. We present a case of 13-yr-boy with a well-established diagnosis of liver cirrhosis secondary to NASH, who underwent orthotopi...

    journal_title:Pediatric transplantation

    pub_type: 杂志文章


    authors: Jankowska I,Socha P,Pawlowska J,Teisseyre M,Gliwicz D,Czubkowski P,Kalicinski P,Cielecka-Kuszyk J,Socha J

    更新日期:2007-11-01 00:00:00

  • High depression rates among pediatric renal replacement therapy patients: A cross-sectional study.

    abstract::Depression is common in pediatric chronic kidney disease (CKD) patients. Depression is associated with inferior long-term outcomes. There is a paucity of studies that evaluate depression and possible associated factors in children and adolescents requiring renal replacement therapy (RRT). Cross-sectional study using C...

    journal_title:Pediatric transplantation

    pub_type: 杂志文章


    authors: Rodriguez Cuellar CI,García de la Puente S,Hernández Moraria J,Bojórquez Ochoa A,Filler G,Zaltzman Grishevich S

    更新日期:2019-12-01 00:00:00

  • Rebound thymic hyperplasia after liver transplantation for a child with biliary atresia; a case report.

    abstract::The thymus gland possesses the ability to regrow in children leading to a newly developed anterior mediastinal mass. This condition may represent a rebound phenomenon during recovery from a stressful event such as post-chemotherapy and hence was described as RTH. RTH after LT has not been well documented. We are repor...

    journal_title:Pediatric transplantation



    authors: Wang TK,Kuo FY,Lee SY,Cheng YF,Elsarawy AM,Lin CC,Lin TL,Chen CL

    更新日期:2018-07-24 00:00:00

  • Unrelated HSCT in an adolescent affected by congenital erythropoietic porphyria.

    abstract::CEP is a rare inborn error of porphyrin-heme synthesis. Clinical manifestations can range from mild to severe and include erythrodontia, reddish-colored urine, and hemolytic anemia that can be mild or severe and may result in splenomegaly. Completely avoiding exposure to the sun is crucial. Attempts to reduce erythrop...

    journal_title:Pediatric transplantation

    pub_type: 杂志文章


    authors: Faraci M,Morreale G,Boeri E,Lanino E,Dallorso S,Dini G,Scuderi F,Cohen A,Cappelli B

    更新日期:2008-02-01 00:00:00

  • Comparison of generic tacrolimus and Prograf drug levels in a pediatric kidney transplant program: brief communication.

    abstract::A generic version of tacrolimus was approved for use in the USA in August 2009. These narrow therapeutic index generics are tested for bioequivalence only in adults. No data are available on generic tacrolimus levels in children with allografts. Four patients with stable renal allografts in our pediatric program were ...

    journal_title:Pediatric transplantation

    pub_type: 杂志文章


    authors: Abdulnour HA,Araya CE,Dharnidharka VR

    更新日期:2010-12-01 00:00:00

  • Variation in resource utilization in liver transplantation at freestanding children's hospitals.

    abstract::We sought to examine the relationship between liver transplant-related total cost, patient outcome, and hospital resource utilization at freestanding children's hospitals. Using the PHIS database, a retrospective study of 374 patients that underwent liver transplantation at 15 freestanding children's hospitals from Ju...

    journal_title:Pediatric transplantation

    pub_type: 杂志文章


    authors: Minneman JA,Grijalva JL,LaQuaglia MJ,Kim HB,Rangel SJ,Vakili K

    更新日期:2016-11-01 00:00:00

  • Cyclosporine-induced white and grey matter central nervous system lesions in a pediatric renal transplant patient.

    abstract::Major neurologic complications secondary to cyclosporine are well documented and are known to include confusion, cortical blindness, seizure, spasticity, paresis, ataxia and coma. Most previous reports attribute these to white matter central nervous system (CNS) lesions or white/grey matter border lesions. Many predis...

    journal_title:Pediatric transplantation

    pub_type: 杂志文章


    authors: Jeruss J,Braun SV,Reese JC,Guillot A

    更新日期:1998-02-01 00:00:00

  • Psychosocial predictors of medication adherence in pediatric heart and lung organ transplantation.

    abstract::Few studies have identified the psychosocial characteristics of those children and their families associated with future non-adherence to immunosuppressive medications following a heart or lung transplant. UNOS data and medical records information were used to test the association between patient and family psychosoci...

    journal_title:Pediatric transplantation

    pub_type: 杂志文章


    authors: Killian MO

    更新日期:2017-06-01 00:00:00

  • Outcomes of Pneumocystis jiroveci pneumonia infections in pediatric heart transplant recipients.

    abstract::PJP is known to cause significant morbidity and rarely death in immunosuppressed patients. The prevalence and outcomes of PJP in pediatric solid-organ transplant patients are not well established. This study utilizes data from the PHTS to establish the prevalence and outcome of PJP in pediatric heart transplant recipi...

    journal_title:Pediatric transplantation

    pub_type: 杂志文章


    authors: Ng B,Dipchand A,Naftel D,Rusconi P,Boyle G,Zaoutis T,Edens RE

    更新日期:2011-12-01 00:00:00

  • Tracking of blood pressure and its impact on graft function in pediatric renal transplant patients.

    abstract::We studied tracking of BP and its impact on GFR in 44 PRTP followed for 56 months. Three months PT 77% had elevated SBP percentile. First year SBP and DBP correlated positively with final values (p < 0.0001, 0.0002, respectively). Pretransplant and three month PT SBP correlated positively (p = 0.02). At one yr, SBP an...

    journal_title:Pediatric transplantation

    pub_type: 杂志文章


    authors: Silverstein DM,Leblanc P,Hempe JM,Ramcharan T,Boudreaux JP

    更新日期:2007-12-01 00:00:00

  • Acute lymphoblastic leukaemia: whom and when should we transplant?

    abstract::To date, the evidence upon which a decision is made to transplant a child with acute lymphoblastic leukaemia either in first or subsequent remission has rarely been based on randomized trial data. Modern era management of infection and graft-versus-host disease lessens risks but procedure-related deaths still remain h...

    journal_title:Pediatric transplantation

    pub_type: 杂志文章,评审


    authors: Eden OB

    更新日期:1999-01-01 00:00:00

  • Outcomes of Berlin Heart EXCOR® pediatric ventricular assist device support in patients with restrictive and hypertrophic cardiomyopathy.

    abstract::The outcomes of pediatric ventricular assist device support in patients with diastolic heart failure have not been well described. This study reviews the North American experience with Berlin Heart EXCOR® ventricular assist device implants in children with such physiology. The Berlin Heart clinical database was review...

    journal_title:Pediatric transplantation

    pub_type: 杂志文章


    authors: Su JA,Menteer J

    更新日期:2017-12-01 00:00:00

  • Plasmapheresis-resistant acute humoral rejection successfully treated with anti-C5 antibody.

    abstract::Even if kidney graft survival has improved during the last decades, sensitized pediatric patients are an emerging problem. We describe a 17-yr-old male who lost his first graft due to chronic rejection becoming hyperimmunized (CDC PRA 99.61%). A desensitization protocol based on high-dose IVIG, PP, and two Mabthera(®)...

    journal_title:Pediatric transplantation

    pub_type: 杂志文章


    authors: Ghirardo G,Benetti E,Poli F,Vidal E,Della Vella M,Cozzi E,Murer L

    更新日期:2014-02-01 00:00:00

  • Eight-year follow-up in pediatric living donor kidney recipients receiving alemtuzumab induction.

    abstract::Recipient lymphocytes are crucial for direct and indirect pathways of allorecognition. We proposed that the administration of alemtuzumab several weeks pretransplantation could eradicate peripheral lymphatic cells and promote donor-specific acceptance. This was a single-center, retrospective review of 101 consecutive ...

    journal_title:Pediatric transplantation

    pub_type: 杂志文章


    authors: Kaabak MM,Babenko NN,Shapiro R,Maschan AA,Zokoev AK,Schekaturov SV,Vyunkova JN,Dymova OV

    更新日期:2017-08-01 00:00:00

  • Augmented immunosuppression and PTCY-based haploidentical hematopoietic stem cell transplantation for thalassemia major.

    abstract::Alternate donor HSCT for thalassemia major from a matched unrelated donor or haploidentical family donor is a feasible therapeutic option in children with no matched family donor. Aggressive pretransplant immunosuppression, reduced toxicity conditioning, and PTCY result in excellent thalassemia-free survival. We descr...

    journal_title:Pediatric transplantation

    pub_type: 杂志文章


    authors: Vellaichamy Swaminathan V,Ravichandran N,Ramanan KM,Meena SK,Varla H,Ramakrishnan B,Jayakumar I,Uppuluri R,Raj R

    更新日期:2020-10-27 00:00:00

  • Neuroradiologic findings in children with renal transplantation under 5 years of age.

    abstract::Chronic renal failure (CRF) is known to have adverse effects on the neurodevelopmental outcome of affected children. Some of these effects can be ameliorated by transplantation (TX). The cause and nature of the underlying brain injury is not known. We performed a brain magnetic resonance imaging (MRI) study on a group...

    journal_title:Pediatric transplantation

    pub_type: 杂志文章


    authors: Valanne L,Qvist E,Jalanko H,Holmberg C,Pihko H

    更新日期:2004-02-01 00:00:00

  • Predictors of neuropsychological functioning and medication adherence in pediatric heart transplant recipients referred for neuropsychological evaluation.

    abstract::Children who undergo heart transplantation are at risk for long-term neurodevelopmental sequelae secondary to heart disease and its treatment. Detailed neuropsychological profiles in clinical sample status post-pediatric heart transplantation are sparse in the literature, and there is little information regarding pred...

    journal_title:Pediatric transplantation

    pub_type: 杂志文章


    authors: Wolfe KR,Kelly SL,Steinberg E,Pliego J,Everitt MD

    更新日期:2020-02-01 00:00:00

  • Complications of Cryptosporidium infection after pediatric liver transplantation: Diarrhea, rejection, and biliary disease.

    abstract:BACKGROUND:Cryptosporidium enteritis can be devastating in the immunocompromised host. In pediatric liver transplant recipients, infection may be complicated by prolonged carriage of the parasite, rejection, and biliary tree damage and fibrosis. Herein, we report on six patients and their long-term outcomes following c...

    journal_title:Pediatric transplantation

    pub_type: 杂志文章


    authors: Fischer RT,Day JC,Wasserkrug H,Faseler M,Kats A,Daniel JF,Slowik V,Andrews W,Hendrickson RJ

    更新日期:2020-12-01 00:00:00

  • Arterioportal fistulae following segmental liver transplantation in a child.

    abstract::We report a case of arterio-portal fistulae in a 12-month-old-child following a segmental liver transplantation. The fistula, probably the result of mass ligature of a vascular pedicle during back table allograft reduction, is to our knowledge the first such case reported. Diagnosed on the third post-operative day, th...

    journal_title:Pediatric transplantation

    pub_type: 杂志文章


    authors: Mieles LA,Waldman D,Orloff MS

    更新日期:2002-10-01 00:00:00

  • Cannulation of the aorta in organ donors with aortic and caval injuries at the time of procurement.

    abstract::Injuries sustained by major vessels during procurement pose a major threat to organ viability. Aortic and inferior vena cava lacerations produce rapid hemorrhage associated with hypoperfusion and ischemic damage. We describe a technique that will prevent such damage in the event of vascular mishaps. ...

    journal_title:Pediatric transplantation

    pub_type: 杂志文章


    authors: Molmenti EP,Molmenti H,Marubashi S,Sanchez EQ,Gogel B,Goldstein RM,Levy MF,Fasola CG,Gonwa TA,Klintmalm GB

    更新日期:2001-10-01 00:00:00

  • Impact of growth hormone treatment on a Belgian population of short children with renal allografts.

    abstract::We retrospectively analyzed the effects of recombinant human growth hormone (rhGH) in a Belgian population of 36 short children with renal allografts. Seven children were dropped from the growth study: 1 had skeletal dysplasia and in 6 cases rhGH was given for less than 1 yr (1 died, 1 developed genu valgum, 2 were no...

    journal_title:Pediatric transplantation

    pub_type: 杂志文章


    authors: Janssen F,Van Damme-Lombaerts R,Van Dyck M,Hall M,Schurmans T,Herman J,Hooghe L,Van Damme B

    更新日期:1997-11-01 00:00:00

  • Successful transplantation of a cadaveric kidney with post-infectious glomerulonephritis.

    abstract::This report describes a successful renal Tx in a patient with chronic renal failure, caused by dysplastic kidneys, who received a cadaveric kidney with post-infectious glomerulonephritis. Sequential renal biopsies were performed at 12 h before Tx, and at 1 h and on days 8 and 58 post-Tx. Post-operative hematuria disap...

    journal_title:Pediatric transplantation

    pub_type: 杂志文章


    authors: Mizuiri S,Shigetomi Y,Sugiyama K,Miyagi M,Hatano T,Tajima E,Aikawa A,Ohara T,Kawamura S,Hasegawa A

    更新日期:2000-02-01 00:00:00

  • Resistance to growth hormone in children with chronic liver disease.

    abstract::Malnutrition adversely affects mortality and morbidity before and after liver transplantation. Outcome might be improved if liver transplant recipients were in a better nutritional state at the time of transplantation. In this review, we will examine the potential use of GH and IGF-I to improve nutritional status in p...

    journal_title:Pediatric transplantation

    pub_type: 杂志文章,评审


    authors: Bucuvalas JC,Horn JA,Chernausek SD

    更新日期:1997-08-01 00:00:00

  • Renal injury and recovery in pediatric patients after ventricular assist device implantation and cardiac transplant.

    abstract:BACKGROUND:The use of ventricular assist devices (VADs) in children with heart failure may be of particular benefit to those with accompanying renal failure, as improved renal function is seen in some, but not all recipients. We hypothesized that persistent renal dysfunction at 7 days and/or 1 month after VAD implantat...

    journal_title:Pediatric transplantation

    pub_type: 杂志文章


    authors: Hollander SA,Cantor RS,Sutherland SM,Koehl DA,Pruitt E,McDonald N,Kirklin JK,Ravekes WJ,Ameduri R,Chrisant M,Hoffman TM,Lytrivi ID,Conway J

    更新日期:2019-08-01 00:00:00

  • Efficacy and safety of three times daily dosing of tacrolimus in pediatric kidney transplantation patients: A single-center comparative study.

    abstract:BACKGROUND/AIM:Several PK studies have shown that most pediatric patients may require higher doses on a mg/kg basis compared to adults to attain similar therapeutic trough concentrations. The aim of this study was to compare the efficacy and safety of three times daily to twice a day dosing of tacrolimus in pediatric k...

    journal_title:Pediatric transplantation

    pub_type: 杂志文章


    authors: Alabdulkarim Z,Al-Jedai A,Alkortas D,Alhasan K,Devol E

    更新日期:2020-09-01 00:00:00

  • Liver transplantation as a treatment option for three siblings with homozygous familial hypercholesterolemia.

    abstract::FH is a hereditary inherited disorder of cholesterol metabolism. Homozygous form of the disease associates severe form of atherosclerotic disease. Clinicians have been tried to inhibit the progression of the homozygous FH with medical and surgical treatment. We here present three siblings with homozygous FH who were s...

    journal_title:Pediatric transplantation

    pub_type: 杂志文章


    authors: Küçükkartallar T,Yankol Y,Kanmaz T,Topaloğlu S,Acarli K,Kalayoglu M

    更新日期:2011-05-01 00:00:00

  • Comparison of melphalan- And busulfan-based myeloablative conditioning in children undergoing allogeneic transplantation for acute myeloid leukemia or myelodysplasia.

    abstract:BACKGROUND:The optimal conditioning regimen for alloHCT in children with myeloid malignancies remains undefined. PROCEDURE:We performed a retrospective review of children undergoing alloHCT for AML and MDS over a 10-year period (2008-2018) at our institution, comparing the outcomes of recipients of either a myeloablat...

    journal_title:Pediatric transplantation

    pub_type: 杂志文章


    authors: Oshrine B,Adams L,Nguyen ATH,Amankwah E,Shyr D,Hale G,Petrovic A

    更新日期:2020-06-01 00:00:00

  • Monitoring and modulation of Epstein-Barr virus loads in pediatric transplant patients.

    abstract::A major risk faced by bone-marrow and solid organ transplant patients is the development of post-transplant lymphoproliferative disease or post-transplant lymphoma (PTLD). In pediatric transplantation, PTLD onset is often associated with a rapid rise in Epstein-Barr virus (EBV) load in peripheral blood mononuclear cel...

    journal_title:Pediatric transplantation

    pub_type: 杂志文章


    authors: Orentas RJ,Schauer DW Jr,Ellis FW,Walczak J,Casper JT,Margolis DA

    更新日期:2003-08-01 00:00:00