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Article ; Online: Congenital nephrotic syndrome: is early aggressive treatment needed? Yes.

Hölttä, Tuula / Jalanko, Hannu

2020 Volume 35, Issue 10, Page(s) 1985–1990

Abstract: Congenital nephrotic syndrome (CNS) was primarily considered one disease entity. Hence, one treatment protocol was proposed in the beginning to all CNS patients. Today, with the help of gene diagnostics, we know that CNS is a heterogeneous group of ... ...

Abstract	Congenital nephrotic syndrome (CNS) was primarily considered one disease entity. Hence, one treatment protocol was proposed in the beginning to all CNS patients. Today, with the help of gene diagnostics, we know that CNS is a heterogeneous group of disorders and therefore, different treatment protocols are needed. The most important gene defects causing CNS are NPHS1, NPHS2, WT1, LAMB2, and PLCE1. Before active treatment, all infants with CNS died. It was stated already in the mid-1980s that intensive medical therapy followed by kidney transplantation (KTx) should be the choice of treatment for infants with severe CNS. In Finland, early aggressive treatment protocol was adopted from the USA and further developed for treatment of children with the Finnish type of CNS. The aim of this review is to state reasons for "early aggressive treatment" including daily albumin infusions, intensified nutrition, and timely bilateral nephrectomy followed by KTx at the age of 1-2 years.
MeSH term(s)	Humans ; Infant ; Infusions, Intravenous ; Kidney Transplantation ; Nephrectomy ; Nephrotic Syndrome/diagnosis ; Nephrotic Syndrome/genetics ; Nephrotic Syndrome/mortality ; Nephrotic Syndrome/therapy ; Nutritional Support/methods ; Serum Albumin, Human/administration & dosage ; Severity of Illness Index ; Survival Analysis ; Time-to-Treatment ; Treatment Outcome
Chemical Substances	Serum Albumin, Human (ZIF514RVZR)
Language	English
Publishing date	2020-05-06
Publishing country	Germany
Document type	Journal Article ; Research Support, Non-U.S. Gov't ; Review
ZDB-ID	631932-4
ISSN	1432-198X ; 0931-041X
ISSN (online)	1432-198X
ISSN	0931-041X
DOI	10.1007/s00467-020-04578-4
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Zs.A 2196: Show issues

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Article ; Online: A Retrospective Study of Long-Term Outcomes in 16 ABO-Incompatible Deceased Donor Pediatric Liver Transplants from a National Transplant Center at Helsinki University Hospital, Finland, 1987-2022.

Jahnukainen, Timo / Sareneva, Inna / Lauronen, Jouni / Ylinen, Elisa / Tainio, Juuso / Nordin, Arno / Hukkinen, Maria / Pakarinen, Mikko P / Jalanko, Hannu

Annals of transplantation

2024 Volume 29, Page(s) e941929

Abstract: BACKGROUND The use of ABO-incompatible liver transplants (ABO-ILTs) from deceased donors has become more common due to the shortage of available donor livers and increased transplant waiting times. This retrospective study from a national transplant ... ...

Abstract	BACKGROUND The use of ABO-incompatible liver transplants (ABO-ILTs) from deceased donors has become more common due to the shortage of available donor livers and increased transplant waiting times. This retrospective study from a national transplant center at Helsinki University Hospital, Finland, aimed to assess the long-term outcomes of ABO-incompatible deceased donor pediatric liver transplants between 1987 and 2022. MATERIAL AND METHODS Sixteen (9.5%) of the 169 pediatric liver transplantations were ABO-ILTs. The median age at transplantation was 5.0 (0.5-15.4) years. Reasons for ABO-ILTs were acute liver failure (18.75%), malignancy (12.5%), small body size and long waiting time (25%), and other reasons (43.75%). The median post-transplant follow-up time was 147 (0.72-353) months. Patient and graft survival and occurrence of surgical complications were compared to ABO-identical transplants, and anti-ABO antibody titers were analyzed. RESULTS The 1-, 3-, and 5-year patient survivals were comparable between the ABO-I and ABO-compatible groups, being 81.3%, 73.9%, and 73.9% (ABO-I) and 87.5%, 82.5%, 77.9% (ABO-compatible), respectively. Three patients with ABO-ILTs died of sepsis and multiorgan failure during the first 3 months after transplantation. The occurrence of biliary complications and early vascular thrombosis (<30 days after transplantation) did not differ significantly between recipients with an ABO-ILT vs ABO-compatible liver graft. CONCLUSIONS The findings from this study support findings from previous studies that outcomes after ABO-incompatible liver transplants in children were comparable to outcomes from ABO-identical liver transplants.
MeSH term(s)	Child ; Humans ; Child, Preschool ; Adolescent ; Liver Transplantation/methods ; Retrospective Studies ; Finland ; Blood Group Incompatibility ; ABO Blood-Group System ; Hospitals ; Graft Survival ; Graft Rejection ; Living Donors
Chemical Substances	ABO Blood-Group System
Language	English
Publishing date	2024-02-13
Publishing country	United States
Document type	Journal Article
ZDB-ID	1484710-3
ISSN	2329-0358 ; 1425-9524
ISSN (online)	2329-0358
ISSN	1425-9524
DOI	10.12659/AOT.941929
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Zs.A 5093: Show issues

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Article ; Online: Pericardial Constriction and Myocardial Restriction in Pediatric Mulibrey Nanism: A Complex Disease With Diastolic Dysfunction.

Sarkola, Taisto / Lipsanen-Nyman, Marita / Jalanko, Hannu / Jokinen, Eero

CJC open

2021 Volume 4, Issue 1, Page(s) 28–36

Abstract: Background: Mulibrey nanism (MUL) is a rare condition with profound growth delay. Congestive heart failure is a major determinant of prognosis. The aim was to delineate pericardial constriction and myocardial functional abnormalities in a pediatric MUL ... ...

Abstract	Background: Mulibrey nanism (MUL) is a rare condition with profound growth delay. Congestive heart failure is a major determinant of prognosis. The aim was to delineate pericardial constriction and myocardial functional abnormalities in a pediatric MUL sample. Methods: A total of 23 MUL patients and 23 individually sex- and age-matched healthy control subjects were prospectively assessed in a cross-sectional study with echocardiography. Results: Clinical signs of heart failure were present in 7 MUL patients, with severe congestive heart failure in 2. Significant diastolic dysfunction, mainly related to constriction, was found in MUL patients without pericardiectomy (N = 18)-septal bounce, pronounced hepatic vein atrial reversal and right heart inflow-outflow variations, and decreased inferior vena cava collapse during respiration. The appearance of the pericardium was not different from that of control subjects. Longitudinal diastolic myocardial velocities were similar to those in control subjects, suggesting an absence of significant myocardial restriction. Right ventricular free wall longitudinal systolic strain and bilateral longitudinal myocardial systolic velocities were decreased in MUL patients, indicating mild biventricular systolic dysfunction. Myocardial motion abnormalities and persistent congestive heart failure were common (in 3 of 6) in MUL patients with a history of pericardiectomy. Cardiac dimensions were similar between MUL patients and control subjects when adjusting for body size, except for smaller biventricular volumes. Conclusions: MUL disease presents with significant constriction-related diastolic dysfunction and mild bilateral systolic dysfunction. Constriction-restriction assessments during follow-up could be of benefit in decision-making regarding pericardiectomy in MUL disease. Myocardial abnormalities were prevalent among MUL patients who had undergone pericardiectomy and are consistent with progression of myocardial disease in a significant proportion of patients.
Language	English
Publishing date	2021-08-30
Publishing country	United States
Document type	Journal Article
ISSN	2589-790X
ISSN (online)	2589-790X
DOI	10.1016/j.cjco.2021.08.012
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Article ; Online: Physical performance after pediatric solid organ transplantation.

Mäenpää, Heidi / Tainio, Juuso / Jalanko, Hannu / Arokoski, Jari / Jahnukainen, Timo

Pediatric transplantation

2021 Volume 26, Issue 2, Page(s) e14163

Abstract: Introduction: Low physical activity is a well-recognized problem in pediatric solid organ transplant recipients; however, little is known about the differences between transplant groups. Physical performance testing was performed in a cohort of ... ...

Abstract	Introduction: Low physical activity is a well-recognized problem in pediatric solid organ transplant recipients; however, little is known about the differences between transplant groups. Physical performance testing was performed in a cohort of pediatric kidney, liver, and heart transplant recipients. Methods: Fifty-one patients (54.9% boys), including 17 liver, 20 kidney, 2 combined liver-kidney, and 12 heart transplant recipients, were tested at the median age of 11.5 (7.5-14.9) years. The results were compared with a control group, which consisted of 425 healthy schoolchildren. The physical performance test included six different tests of endurance, strength, flexibility, and speed. Results: The transplant recipients performed worse on most tests when compared with the control subjects (leg-lift test 42.0 vs. 44.9 repetitions, p = .002; repeated squatting 21.6 vs. 23.9 repetitions, p < .001; sit-up test 9 vs. 17 vs. 9 repetitions, p < .001, back extension 20 vs. 35 repetitions, p < .001; and shuttle run test 26.5 vs. 23.7 seconds, p < .001). None of the test results differed statistically significantly between the transplant groups. Conclusion: The physical performance of pediatric solid organ transplant recipients is lower than that of their healthy peers but do not differ between different transplant groups. More systematic rehabilitation programs and follow-up are needed.
MeSH term(s)	Adolescent ; Case-Control Studies ; Child ; Female ; Finland ; Heart Transplantation ; Humans ; Kidney Transplantation ; Liver Transplantation ; Male ; Organ Transplantation ; Physical Functional Performance ; Transplant Recipients
Language	English
Publishing date	2021-10-18
Publishing country	Denmark
Document type	Journal Article
ZDB-ID	1390284-2
ISSN	1399-3046 ; 1397-3142
ISSN (online)	1399-3046
ISSN	1397-3142
DOI	10.1111/petr.14163
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Zs.A 4947: Show issues

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Article ; Online: Male Sexual Function After Pediatric Kidney Transplantation-A Cross-sectional Nationwide Study.

Tainio, Juuso / Jahnukainen, Timo / Jalanko, Hannu / Jahnukainen, Kirsi

The journal of sexual medicine

2020 Volume 17, Issue 10, Page(s) 2104–2107

Abstract: ... dimensions of quality of life. Tainio J, Jahnukainen T, Jalanko H, et al. Male Sexual Function After ...

Abstract	Background: Data on adult sexual functioning after kidney transplantation (KTx) during childhood or adolescence are scarce. Aim: To assess the long-term sexual and psychosocial quality of life after pediatric KTx. Methods: 29 young men (median age 27.1 years) were examined 18.7 years (median) after KTx. 56 age-matched healthy men (median age 30.0 years) served as controls. Outcome: We studied the influence of sociodemographics, previous renal replacement therapy, current reproductive hormonal serum levels, testicular size, and data on several validated mental and physical questionnaires on participants' Derogatis Interview for Sexual Functioning self-report scores. Results: The KTx recipients had significantly poorer sexual functioning than their healthy peers. KTx men had less frequent sexual activity with a partner (P = .03) and poorer orgasms (P = .002) than the controls but no erectile dysfunction (P = .5). Clinical implications: Depressive symptoms, relationship status, and longer dialysis duration predicted poor adult sexual functioning in KTx recipients, whereas age at transplantation or at the time of the study did not. Strengths & limitations: This study contributes extended follow-up data to the very scarce literature on adult sexual functioning in pediatric KTx recipients. Relatively small population and low participation rate limit the comprehensive data interpretation in a population-based cohort of male KTx recipients. Conclusion: Sexual functioning is often impaired in young men after pediatric KTx, emphasizing the need for long-term monitoring of sexual health and sexuality as important dimensions of quality of life. Tainio J, Jahnukainen T, Jalanko H, et al. Male Sexual Function After Pediatric Kidney Transplantation-A Cross-sectional Nationwide Study. J Sex Med 2020;17:2104-2107.
MeSH term(s)	Adolescent ; Adult ; Child ; Cross-Sectional Studies ; Humans ; Kidney Transplantation/adverse effects ; Male ; Orgasm ; Quality of Life ; Sexual Behavior
Language	English
Publishing date	2020-07-21
Publishing country	Netherlands
Document type	Case Reports ; Research Support, Non-U.S. Gov't
ZDB-ID	2251959-2
ISSN	1743-6109 ; 1743-6095
ISSN (online)	1743-6109
ISSN	1743-6095
DOI	10.1016/j.jsxm.2020.06.011
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Zs.A 6417: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular ab Jg. 2022: Lesesaal (EG)
Zs.MO 505: Show issues

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Book ; Thesis: Alpha-Fetoprotein

JALANKO, HANNU

EXPRESSION IN NORMAL DEVELOPMENT AND IN EXPERIMENTAL LIVER CARCINOGENESIS

1979

Size	43 S.
Document type	Book ; Thesis
Thesis / German Habilitation thesis	HELSINKI, UNIV., DISS., 1979
HBZ-ID	HT002693351
ISBN	951-99236-1-6 ; 978-951-99236-1-1
Database	Catalogue ZB MED Medicine, Health

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Article ; Online: Expression of fibrosis-related genes in liver allografts: Association with histology and long-term outcome after pediatric liver transplantation.

Voutilainen, Silja H / Kosola, Silja K / Lohi, Jouko / Jahnukainen, Timo / Pakarinen, Mikko P / Jalanko, Hannu

Clinical transplantation

2021 Volume 35, Issue 8, Page(s) e14373

Abstract: Background: Unexplained graft fibrosis and inflammation are common after pediatric liver transplantation (LT).: Objective: We investigated the graft expression of fibrogenic genes and correlated the findings with transplant histopathology and outcome. ...

Abstract	Background: Unexplained graft fibrosis and inflammation are common after pediatric liver transplantation (LT). Objective: We investigated the graft expression of fibrogenic genes and correlated the findings with transplant histopathology and outcome. Methods: Liver biopsies from 29 recipients were obtained at a median of 13.1 (IQR: 5.0-18.4) years after pediatric LT. Control samples were from six liver-healthy subjects. Hepatic expression of 40 fibrosis-related genes was correlated to histological findings: normal histology, fibrosis with no inflammation, and fibrosis with inflammation. Liver function was evaluated after a subsequent follow-up of 9.0 years (IQR: 8.0-9.4). Results: Patients with fibrosis and no inflammation had significantly increased gene expression of profibrotic TGF-β3 (1.17 vs. 1.02 p = .005), CTGF (1.64 vs. 0.66 p = .014), PDGF-α (1.79 vs. 0.98 p = .049), PDGF -β (0.99 vs. 0.76 p = .006), integrin-subunit-β1 (1.19 vs. 1.02 p = .045), α-SMA (1.12 vs. 0.58 p = .013), type I collagen (0.82 vs. 0.53 p = .005) and antifibrotic decorin (1.15 vs. 0.99 p = .045) compared to patients with normal histology. mRNA expression of VEGF A (0.84 vs. 1.06 p = .049) was lower. Only a few of the studied genes were upregulated in patients with both fibrosis and inflammation. The gene expression levels showed no association with later graft outcome. Conclusions: Altered hepatic expression of fibrosis-related genes is associated with graft fibrosis without concurrent inflammation.
MeSH term(s)	Allografts/pathology ; Child ; Fibrosis ; Humans ; Liver Cirrhosis/genetics ; Liver Cirrhosis/pathology ; Liver Transplantation
Language	English
Publishing date	2021-06-27
Publishing country	Denmark
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	639001-8
ISSN	1399-0012 ; 0902-0063
ISSN (online)	1399-0012
ISSN	0902-0063
DOI	10.1111/ctr.14373
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Zs.A 2204: Show issues

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Article ; Online: Long-term effects of paediatric kidney transplantation.

Holmberg, Christer / Jalanko, Hannu

Nature reviews. Nephrology

2016 Volume 12, Issue 5, Page(s) 301–311

Abstract: Renal transplantation in paediatric patients usually provides excellent short-term and medium-term results. Early diagnosis of chronic kidney disease and active therapy of end-stage renal disease before and after transplantation enables the majority of ... ...

Abstract	Renal transplantation in paediatric patients usually provides excellent short-term and medium-term results. Early diagnosis of chronic kidney disease and active therapy of end-stage renal disease before and after transplantation enables the majority of children to grow and develop normally. The adverse effects of immunosuppressive medication and reduced graft function might, however, hamper long-term outcomes in these patients and can lead to metabolic complications, cardiovascular disease, reduced bone health, and malignancies. The neurocognitive development and quality of life of paediatric transplant recipients largely depend on the primary diagnosis and on graft function. Poor adherence to immunosuppression is an important risk factor for graft loss in adolescents, and controlled transition to adult care is of utmost importance to ensure a continued normal life. In this Review, we discuss the outcomes and long-term effects of renal transplantation in paediatric recipients, including consequences on growth, development, bone, metabolic, and cardiovascular disorders. We discuss the key problems in the care of paediatric renal transplant recipients and the remaining challenges that should be the focus of future research.
MeSH term(s)	Age Factors ; Bone Density ; Child ; Child Development ; Cognition ; Graft Survival ; Human Growth Hormone/therapeutic use ; Humans ; Immunosuppressive Agents/adverse effects ; Kidney Failure, Chronic/etiology ; Kidney Failure, Chronic/therapy ; Kidney Transplantation/adverse effects ; Kidney Transplantation/mortality ; Kidney Transplantation/psychology ; Lymphoproliferative Disorders/etiology ; Metabolic Diseases/etiology
Chemical Substances	Immunosuppressive Agents ; Human Growth Hormone (12629-01-5)
Language	English
Publishing date	2016-05
Publishing country	England
Document type	Journal Article ; Research Support, Non-U.S. Gov't ; Review
ZDB-ID	2490366-8
ISSN	1759-507X ; 1759-5061
ISSN (online)	1759-507X
ISSN	1759-5061
DOI	10.1038/nrneph.2015.197
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Zs.A 6334: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 2021: Bestellungen von Artikeln über das Online-Bestellformular ab Jg. 2022: Lesesaal (EG)
Zs.MG 107: Show issues

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Article ; Online: Nephrin Trafficking beyond the Kidney--Role in Glucose-Stimulated Insulin Secretion in β Cells.

Lehtonen, Sanna / Jalanko, Hannu

Journal of the American Society of Nephrology : JASN

2015 Volume 27, Issue 4, Page(s) 965–968

MeSH term(s)	Animals ; Female ; Humans ; Insulin/metabolism ; Insulin Secretion ; Male ; Membrane Proteins/physiology ; Receptor, Insulin/physiology ; Signal Transduction/physiology ; TOR Serine-Threonine Kinases/physiology
Chemical Substances	Insulin ; Membrane Proteins ; TOR Serine-Threonine Kinases (EC 2.7.1.1) ; Receptor, Insulin (EC 2.7.10.1)
Language	English
Publishing date	2015-09-23
Publishing country	United States
Document type	Editorial ; Research Support, Non-U.S. Gov't ; Comment
ZDB-ID	1085942-1
ISSN	1533-3450 ; 1046-6673
ISSN (online)	1533-3450
ISSN	1046-6673
DOI	10.1681/ASN.2015080960
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Zs.A 3344: Show issues

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Article ; Online: Long-term Outcome of Kidney Transplantation in 6 Patients With Autoimmune Polyendocrinopathy-candidiasis-ectodermal Dystrophy.

Laakso, Saila / Kaijansinkko, Henna / Räisänen-Sokolowski, Anne / Jahnukainen, Timo / Kataja, Janne / Mäkitie, Outi / Helanterä, Ilkka / Jalanko, Hannu

Transplantation

2021 Volume 106, Issue 4, Page(s) e244–e246

MeSH term(s)	Humans ; Kidney Transplantation/adverse effects ; Mutation ; Polyendocrinopathies, Autoimmune/complications ; Polyendocrinopathies, Autoimmune/diagnosis ; Polyendocrinopathies, Autoimmune/genetics ; Transcription Factors/genetics
Chemical Substances	Transcription Factors
Language	English
Publishing date	2021-11-30
Publishing country	United States
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	208424-7
ISSN	1534-6080 ; 0041-1337
ISSN (online)	1534-6080
ISSN	0041-1337
DOI	10.1097/TP.0000000000003993
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Zs.A 488: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular Jg. 1995 - 2021: Lesesall (1.OG) ab Jg. 2022: Lesesaal (EG)
Zs.MO 509: Show issues

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