Miroslav Živičnjak

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Verified

Miroslav verified their affiliation via an institutional email.

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• PhD
• scientist at Hannover Medical School

X-linked hypophosphatemia (XLH) is the most common inherited form of hypophosphatemic rickets. Children with XLH have an increased risk of obesity, which may promote high blood pressure, but data on blood pressure in XLH are inconclusive. We aimed to assess blood pressure and its determinants in pediatric XLH patients. We conduct a prospective, mul...

Background: Children with X-linked hypophosphatemia (XLH) present with rickets, leg deformities, and growth failure. Bone stability depends on balanced bone growth in both, length and width. Data on body proportions, including transverse body dimensions, in children with XLH treated with phosphate supplements and active vitamin D is lacking. Method...

Schimke immuno-osseous-dysplasia (SIOD) is an autosomal recessive systemic disease due to pathogenic variants in SMARCAL1 . Manifestations include nephrotic syndrome (NS), kidney failure, T-cell dysfunction, vaso-occlusive disease, and disproportionate short stature, a general feature of this disease. Here, we present a markedly different growth pa...

Context The pathophysiology of cystinosis-associated metabolic bone disease is complex. Objective We hypothesized a disturbed interaction between osteoblasts and osteoclasts. Design Binational cross-sectional multicenter study. Setting Hospital clinics. Patients One hundred and three patients with cystinosis (61% children) with chronic kidney d...

Background Infantile nephropathic cystinosis (INC) is a rare lysosomal storage disorder, mostly and often firstly affecting the kidneys, together with impaired disharmonious growth and rickets, eventually resulting in progressive chronic kidney disease (CKD). With the introduction of cysteamine therapy, most pediatric patients reach adulthood with...

Background: Infantile nephropathic cystinosis (INC) is a systemic lysosomal storage disease causing intracellular cystine accumulation, resulting in renal Fanconi syndrome, progressive kidney disease (CKD), rickets, malnutrition, and myopathy. An INC-specific disproportionately diminished trunk length compared to leg length poses questions regardi...

Context: Burosumab has been approved for the treatment of children and adults with X-linked hypophosphatemia (XLH). Real-world data and evidence for its efficacy in adolescents is lacking. Objective: To assess the effects of 12 months burosumab treatment on mineral metabolism in children (aged < 12 years) and adolescents (aged 12-18 years) with...

Mucopolysaccharidosis type I (MPS I) is an autosomal‐recessive metabolic disorder caused by an enzyme deficiency of lysosomal alpha‐l‐iduronidase (IDUA). Haematopoietic stem cell transplantation (HSCT) is the therapeutic option of choice in MPS I patients younger than 2.5 years, which has a positive impact on neurocognitive development. However, im...

The study conference of the German Society for Pediatric Nephrology (GPN), which has been held for almost 50 years, is the most important instrument of cooperative research in German pediatric nephrology. It has made a significant contribution to improving clinical care and strengthens the cooperation between the pediatric nephrology centers. Many...

Children with infantile nephropathic cystinosis (INC), an inherited lysosomal storage disease resulting in cystine accumulation in all body cells, are prone to progressive chronic kidney disease (CKD), impaired growth and reduced weight gain; however, systematic anthropometric analyses are lacking. In this prospective multicenter study we investiga...

Background Recombinant human growth hormone (rhGH) is frequently used for treatment of short stature in children with chronic kidney disease (CKD) prior to kidney transplantation (KT). To what extent this influences growth and transplant function after KT is yet unknown. Methods Post-transplant growth (height, sitting height, leg length) and clini...

Background Short stature is a frequent complication after pediatric kidney transplantation (KT). Whether the type of transplantation and prior treatment with recombinant human growth hormone (GH) affects post-transplant growth, is unclear. Methods Body height, leg length, sitting height, and sitting height index (as a measure of body proportions)...

Context: Children with nephropathic cystinosis (NC) show persistent hypophosphatemia, due to Fanconi syndrome, as well as mineral and bone disorders related to chronic kidney disease (CKD), but systematic analyses are lacking. Objective: To compare biochemical parameters of bone and mineral metabolism between children with NC and controls across...

Background: We recently showed that a 3-year growth hormone (GH) treatment improves linear growth in severely short children with X-linked hypophosphatemic rickets (XLH). It is unknown if GH therapy increases adult height in XLH patients. Methods: We carried out a follow-up analysis of a randomized controlled open-label GH study in short prepube...

Background Children with chronic kidney disease are frequently born small for gestational age (SGA) and prone to disproportionately short stature. It is unclear how SGA affects growth after kidney transplantation (KTx). Methods Linear growth (height, sitting height, and leg length) was prospectively investigated in a cohort of 322 pediatric KTx rec...

Impairment of pubertal growth and sexual maturation resulting in reduced adult height is an significant complication in children suffering from chronic kidney disease (CKD). Delayed puberty and reduced pubertal growth are most pronounced in children with pre-existing severe stunting before puberty, requiring long-term dialysis treatment, and in tra...

Data for body mass index (BMI=weight/height2) from 4496 Croatian children (ages 6-19y) from the Zagreb growth study undertaken in 1997, have been used to obtain age- and sex-specific empirical percentiles. These have been compared with corresponding WHO/NCHS percentiles. There were no differences between these data sets for the 5th through the 50th...

Background and objectives: Poor linear growth is a frequent complication of CKD. This study evaluated the effect of kidney transplantation on age-related growth of linear body segments in pediatric renal transplant recipients who were enrolled from May 1998 until August 2013 in the CKD Growth and Development observational cohort study. Design, se...

Despite the relevant findings on non-average information processing rate (IPR) indicators-intelligence relation, and on age-related changes of some of these indicators during aging, the research on sex-specific age-related changes of these indicators during childhood and adolescence are lacking. In a transversal study, 1197 school children (598 gir...

We analyzed the impact of birth parameters and parental height on long-term growth outcome in children with chronic kidney disease (CKD) stage 3-5. Linear growth was prospectively investigated in 509 children, with a mean follow-up of 4.1 years. Growth outcome was categorized in (i) poor growth (PG): height standard deviation score (SDS) during fol...

Background: The attainment of normal growth and maturation remains a major challenge in the management of children and adolescents requiring renal replacement therapy (RRT). Methods: We compared growth and maturation in 384 German children with RRT who were followed between 1998 and 2009 with 732 children who were enrolled in the European Dialys...

When a known microimbalance affecting multiple genes is detected in a patient with syndromic intellectual disability, it is usually presumed causative for all observed features. Whole exome sequencing (WES) allows questioning this assumption. In this study of three families with children affected by unexplained syndromic intellectual disability, ge...

Children with X-linked hypophosphatemic rickets (XLH) are prone to progressive disproportionate stunting despite oral phosphate and vitamin D treatment. Our objective was to analyze the effects of GH treatment on stature and lengths of linear body segments in short children with XLH. A 3-yr randomized controlled open-label GH study in short prepube...

Grigull L, Sykora K‐W, Tenger A, Bertram H, Meyer‐Marcotty M, Hartmann H, Bültmann E, Beilken A, Zivicnjak M, Mynarek M, Osthaus AW, Schilke R, Kollewe K, Lücke T. Variable disease progression after successful stem cell transplantation: Prospective follow‐up investigations in eight patients with Hurler syndrome. Pediatr Transplantation 2011: 15: 8...

Children with X-linked hypophosphatemic rickets (XLH) are prone to severe stunting. A multicenter mixed-longitudinal study was conducted to assess age-related stature, sitting height, arm and leg length in XLH patients on continuous treatment with phosphate and calcitriol. Mean standard deviation scores (SDS) for all body dimensions were markedly r...

Low birth weight has been identified as a risk factor for chronic kidney disease (CKD). We analysed perinatal parameters taken from the National Birth Certificates of 435 children with CKD stages 3-5 of different aetiology and time of onset of CKD. Diseases were classified as congenital with onset of renal disease during fetal life (n = 260; 60%),...

Wachstum und Entwicklung unterliegen in Kindes-und Jugendalter einer geschlechtsspezifischen Kontrolle. Die Diagnostik und Therapie von pathologischem Wachstum setzt die genauen Kenntnisse des geschlechtsspezifischen Wachstums und der anthropometrischen Normwerte voraus. Die Unterschiede der statomotorischen Entwicklung von Mädchen und Jungen sind...

Unlabelled: Schimke immuno-osseous dysplasia (SIOD) is a rare autosomal-recessive multisystem disorder with disproportionate growth failure, impaired T cell function, and steroid-resistant nephrotic syndrome. Recently, we presented the typical anthropometric features of SIOD. We now present data on two siblings who were initially classified as suf...

Despite major progress in dialysis, nutrition and drug treatment in the past 20 years, growth of infants and toddlers with chronic kidney disease (CKD) remains a major challenge in paediatric nephrology. Our hypothesis is that early growth deficit is one of the most important factors for impaired final height in children with CKD, and we conclude t...

In a cross-sectional study of growth, 5,260 healthy children of both sexes from Zagreb (Croatia) aged 2 to 18 years were measured. Six transversal body dimensions were studied: biacromial, transverse chest, antero-posterior chest, biiliocristal, bicondylar humerus and bicondylar femur diamters. A significant increase in body diameters has been obse...

The impact of chronological age on longitudinal body growth from early childhood through adolescence using detailed anthropometric methods has not yet been studied in children with chronic kidney disease (CKD). We have evaluated growth failure by measuring four components of linear growth: body height (HT), sitting height (SHT), arm length (AL) and...

Schimke-immuno-osseous dysplasia is a rare autosomal-recessive multisystem disorder with the main clinical features of disproportionate growth deficiency, defective cellular immunity, and progressive renal disease. It is caused by mutations of SMARCAL1, a gene encoding a putative chromatin remodeling protein of unknown function. Because a detailed...

Seventy-two pediatric kidney recipients of living related donors (LRD) and 145 of cadaveric donors (CAD) were analyzed for height standard deviation scores (Ht-SDS) and glomerular filtration rates (GFR) directly after transplantation and over the following 5 years. GFR was significantly higher immediately after transplantation in LRD compared with...

Data for body mass index (BMI=weight/height2) from 4496 Croatian children (ages 6-19y) from the Zagreb growth study undertaken in 1997, have been used to obtain age- and sex-specific empirical percentiles. These have been compared with corresponding WHO/NCHS percentiles. There were no differences between these data sets for the 5th through the 50th...

Results of the analysis of morphological status of cadets and pilots of the Croatian Air Force are presented, in relation to the morphological status of a sample of conscripts of the Croatian Army. The results of the morphological status of body built of the flier potential of the Croatian Air Force, i.e., the potential (cadets) and active fliers (...

The extent to which growth after renal transplantation differs between children with a living related donor graft (LRD) and those with a cadaveric donor graft (CAD) is unclear. We retrospectively studied growth in the 5 years after transplantation in 30 boys who received LRD and 21 who received CAD. Height was similar in both groups after transplan...

In a cross-sectional study of growth, 5,155 children (2,591 females, 2,564 males) from the town of Zagreb (Croatia) were measured. Four traits of linear dimensionality (stature, sitting height, arm and leg lengths) were studied in the age span of 3 to 18 years. A significant average annual increase of all four anthropometric parameters were observe...

We investigated cognitive-motor abilities in 303 (156 female) school children from Zagreb, Croatia, in the age span 10 to 14 years using a newly developed chronometrical reactionmeter system (CRD). The following tests were applied: CRD-311 (simple visual discrimination of signal location), CRD-324 (short-term memory actualisation), CRD-21 (simple c...

Recombinant human growth hormone (rhGH) therapy of growth retardation in chronic renal failure (CRF) has become well established. While there are ample data about its effectiveness in restoring longitudinal growth delay, data on complex anthropometry are scarce. Twenty-three children with CRF (6 after renal transplantation) were investigated using...

This study evaluated the extent of thyroid abnormalities in a remote iodine-deficient area of the Ivory Coast. Ultrasonography was used in detecting the presence of goiter. The overall prevalence rates of goiter were 64.7% among females and 53.3% among males. In children aged 6 to 15 years (n = 314), the prevalence of goiter was 62% regardless of s...

Changes in body morphology of anorectic girls during illness as well as during the treatment period were studied using data on 23 anthropometric traits. The sample consisted of 20 adolescent girls, aged 16.05 +/- 2.21 years. Girls with shorter duration of anorexia nervosa have larger amounts of fat and muscle tissue on the upper arm, as well as thi...

The morphological characteristics (20 anthropometric variables) of a total of 2,351 examinees (from the age of 18 to 90) were analyzed by a model of the principal components of the factor analysis. Four factors were extracted that explain 71.4% of the total variance. The factors-"general body voluminosity", "subcutaneous fat tissue", "longitudinal...

Growth channels phenomena in pubescence girls (aged 11.5 to 14.5 years) were used as a basis for a detailed analysis of the relation between the soft tissue growth component (related to growth channels) and menarcheal experience. The soft tissue component showed statistically significant differences between the growth channels (in all groups and at...

The structure of morphological/motor characteristics was analysed on a sample of seven-year old girls, who were measured by morphological measurements and motor tests, by means of the MORPHOTAX algorithm. Five morphological/motor taxonomic dimensions were established, i.e.: the first one differentating the subcutaneous fatty tissue and to a certain...