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Article ; Online: Diagnosis and management of hypernatraemia in children.

Zieg, Jakub

Acta paediatrica (Oslo, Norway : 1992)

2021 Volume 111, Issue 3, Page(s) 505–510

Abstract: Hypernatraemia is most commonly caused by excessive loss of solute-free water or decreased fluid intake; less often, the aetiology is salt intoxication. Especially infants, young children and individuals with a lack of access to water are at risk of ... ...

Abstract	Hypernatraemia is most commonly caused by excessive loss of solute-free water or decreased fluid intake; less often, the aetiology is salt intoxication. Especially infants, young children and individuals with a lack of access to water are at risk of developing hypernatraemia. Diagnosis is based on detailed history, physical examination and basic laboratory tests. Correction of hypernatraemia must be slow to prevent cerebral oedema and irreversible brain damage. This article reviews the aetiology, differential diagnosis and management of conditions associated with paediatric hypernatraemia. Distinguishing states with water deficiency from states with salt excess is important for proper management of hypernatraemic patients.
MeSH term(s)	Child ; Child, Preschool ; Diagnosis, Differential ; Hospitalization ; Humans ; Hypernatremia/diagnosis ; Hypernatremia/etiology ; Hypernatremia/therapy ; Infant ; Sodium Chloride
Chemical Substances	Sodium Chloride (451W47IQ8X)
Language	English
Publishing date	2021-11-11
Publishing country	Norway
Document type	Journal Article ; Review
ZDB-ID	203487-6
ISSN	1651-2227 ; 0365-1436 ; 0803-5253
ISSN (online)	1651-2227
ISSN	0365-1436 ; 0803-5253
DOI	10.1111/apa.16170
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Article ; Online: Eculizumab use in patients with pneumococcal-associated hemolytic uremic syndrome and kidney outcomes.

Konopásek, Patrik / Zieg, Jakub

Pediatric nephrology (Berlin, Germany)

2023 Volume 38, Issue 12, Page(s) 4209–4215

Abstract: Background: Streptococcus pneumoniae-associated hemolytic uremic syndrome (P-HUS) is a rare and severe disease. Only a few reports have been published about eculizumab use in P-HUS.: Methods: We analyzed demographic, clinical, and laboratory data of ... ...

Abstract	Background: Streptococcus pneumoniae-associated hemolytic uremic syndrome (P-HUS) is a rare and severe disease. Only a few reports have been published about eculizumab use in P-HUS. Methods: We analyzed demographic, clinical, and laboratory data of patients with P-HUS from our center. Results: The cohort consisted of 4 females and 3 males. All patients had pneumonia. Four were given eculizumab (days 1-3). The eculizumab group required a shorter duration of dialysis and mechanical ventilation (medians 20 vs. 28.5 and 30 vs 38.5 days, respectively) compared with the non-eculizumab group, but this was still much longer than normally reported; the thrombocytopenia resolution was similar in both groups (medians 10 vs. 8 days). Chronic kidney disease (CKD) was correlated with the duration of dialysis and mechanical ventilation duration at 1 year (r = 0.797, P = 0.032 and r = 0.765, P = 0.045) and last follow-up (r = 0.807, P = 0.028 and r = 0.814, P = 0.026, respectively); our scoring system showed even stronger correlations (r = 0.872, P = 0.011 and r = 0.901, P = 0.0057, respectively). The eculizumab group showed slightly better 1-year and last follow-up CKD stage (2.75 vs. 3, P = 0.879 and 2.5 vs. 3.67, P = 0.517). Conclusions: Despite the fact that the eculizumab group showed better outcomes, eculizumab does not seem to improve the course of P-HUS compared with previous reports. Kidney outcomes are strongly correlated with the duration of dialysis and mechanical ventilation duration. A higher resolution version of the Graphical abstract is available as Supplementary information.
MeSH term(s)	Male ; Female ; Humans ; Streptococcus pneumoniae ; Renal Dialysis ; Kidney ; Hemolytic-Uremic Syndrome/complications ; Hemolytic-Uremic Syndrome/drug therapy ; Renal Insufficiency, Chronic/complications ; Atypical Hemolytic Uremic Syndrome/complications ; Atypical Hemolytic Uremic Syndrome/drug therapy
Chemical Substances	eculizumab (A3ULP0F556)
Language	English
Publishing date	2023-06-12
Publishing country	Germany
Document type	Journal Article ; Research Support, Non-U.S. Gov't
ZDB-ID	631932-4
ISSN	1432-198X ; 0931-041X
ISSN (online)	1432-198X
ISSN	0931-041X
DOI	10.1007/s00467-023-06037-2
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Article ; Online: Heart Tumor in a Girl with Nephrotic Syndrome, a Possible Association or a Rare Coincidence?

Konopásek, Patrik / Stara, Veronika / Kočí, Martin / Zieg, Jakub

Klinische Padiatrie

2023

Title translation	Herztumor bei einem Mädchen mit nephrotischem Syndrom, ein möglicher Zusammenhang oder ein seltener Zufall?
Language	English
Publishing date	2023-11-16
Publishing country	Germany
Document type	Journal Article
ZDB-ID	120650-3
ISSN	1439-3824 ; 0300-8630
ISSN (online)	1439-3824
ISSN	0300-8630
DOI	10.1055/a-2183-8302
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Article: Practical recommendations for the management of children after kidney and liver transplantation.

Zieg, Jakub / Bauer, David / Krejčová, Vlasta / Gonsorčíková, Lucie

Casopis lekaru ceskych

2023 Volume 161, Issue 7-8, Page(s) 296–302

Abstract: The number of pediatric solid organ transplantations is growing. This therapy leads often to better quality of life but also brings some specific complications. Our review summarizes practical recommendations for long-time care of the children after ... ...

Title translation	Praktická doporučení pro péči o dětské pacienty po transplantaci ledviny a jater.
Abstract	The number of pediatric solid organ transplantations is growing. This therapy leads often to better quality of life but also brings some specific complications. Our review summarizes practical recommendations for long-time care of the children after kidney and liver transplantation. The knowledge of the issues related to transplantation is essential for the first contact physicians, whose cooperation with transplant centre contributes highly to adequate management of these children.
MeSH term(s)	Child ; Humans ; Liver Transplantation ; Quality of Life ; Kidney ; Organ Transplantation ; Physicians
Language	English
Publishing date	2023-02-06
Publishing country	Czech Republic
Document type	Review ; Journal Article
ZDB-ID	413441-2
ISSN	1805-4420 ; 0008-7335
ISSN (online)	1805-4420
ISSN	0008-7335
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Article ; Online: Fluid management in children with volume depletion.

Zieg, Jakub / Narla, Deepti / Gonsorcikova, Lucie / Raina, Rupesh

Pediatric nephrology (Berlin, Germany)

2023 Volume 39, Issue 2, Page(s) 423–434

Abstract: Volume depletion is a common condition and a frequent cause of hospitalization in children. Proper assessment of the patient includes a detailed history and a thorough physical examination. Biochemical tests may be useful in selected cases. Understanding ...

Abstract	Volume depletion is a common condition and a frequent cause of hospitalization in children. Proper assessment of the patient includes a detailed history and a thorough physical examination. Biochemical tests may be useful in selected cases. Understanding the pathophysiology of fluid balance is necessary for appropriate management. A clinical dehydration scale assessing more physical findings may help to determine dehydration severity. Most dehydrated children can be treated orally; however, intravenous therapy may be indicated in patients with severe volume depletion, in those who have failed oral therapy, or in children with altered consciousness or significant metabolic abnormalities. Proper management consists of restoring circulatory volume and electrolyte balance. In this paper, we review clinical aspects, diagnosis, and management of children with volume depletion.
MeSH term(s)	Child ; Humans ; Dehydration/diagnosis ; Dehydration/etiology ; Dehydration/therapy ; Fluid Therapy/adverse effects ; Water-Electrolyte Balance ; Physical Examination
Language	English
Publishing date	2023-07-14
Publishing country	Germany
Document type	Review ; Journal Article
ZDB-ID	631932-4
ISSN	1432-198X ; 0931-041X
ISSN (online)	1432-198X
ISSN	0931-041X
DOI	10.1007/s00467-023-06080-z
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Article ; Online: Cyclosporin A in combination with corticosteroids as a treatment for immunoglobulin A vasculitis nephritis in children.

Konopásek, Patrik / Krejčová, Vlasta / Švábová, Eva / Zieg, Jakub

Minerva pediatrics

2023

Abstract: Background: Immunoglobulin A vasculitis is the most common vasculitis in children. It is usually a self-limiting condition, and the long-term prognosis depends on the severity of renal involvement. Although cyclosporin A is not generally recommended for ...

Abstract	Background: Immunoglobulin A vasculitis is the most common vasculitis in children. It is usually a self-limiting condition, and the long-term prognosis depends on the severity of renal involvement. Although cyclosporin A is not generally recommended for the management of moderate immunoglobulin A vasculitis nephritis, a few previous reports showed its efficacy. Our aim was to determine whether the treatment with cyclosporin A in combination with corticosteroids is safe and effective for moderate pediatric immunoglobulin A vasculitis nephritis. Methods: Nine children underwent treatment. Mean follow-up was 3.1±1.6 (1.4-5.8) years. Results: All children (seven females and two males) reached complete remission (65.8±27.6 [24-99]) days. No patient had relapse, one patient had slightly impaired kidney function (glomerular filtration rate 84.4 mL/min/1.73 m Conclusions: Cyclosporin A in combination with corticosteroids seems to be a safe and effective treatment for moderate immunoglobulin A vasculitis nephritis. More studies with cyclosporin A should be conducted to better determine the best therapeutic approach.
Language	English
Publishing date	2023-04-06
Publishing country	Italy
Document type	Journal Article
ZDB-ID	3062664-X
ISSN	2724-5780
ISSN (online)	2724-5780
DOI	10.23736/S2724-5276.23.07057-X
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Article: Cerebral sinovenous thrombosis in children with nephrotic syndrome: systematic review and one new case.

Konopásek, Patrik / Piteková, Barbora / Krejčová, Vlasta / Zieg, Jakub

Frontiers in pediatrics

2023 Volume 11, Page(s) 1207871

Abstract: Aim: The aim of this review is to provide clinicians with characteristics of children with nephrotic syndrome and cerebral sinovenous thrombosis (CSVT).: Methods: We have reviewed 37 articles of pediatric cases and provided 1 new case. PRISMA ... ...

Abstract	Aim: The aim of this review is to provide clinicians with characteristics of children with nephrotic syndrome and cerebral sinovenous thrombosis (CSVT). Methods: We have reviewed 37 articles of pediatric cases and provided 1 new case. PRISMA guidelines were followed. Results: Sixty-two patients were included in the review. CSVT was more common in males, usually occurred within 6 months of nephrotic syndrome onset and was found more often in outpatients. The superior sagittal sinus was the most common sinus affected. Non-contrast computed tomography was the most frequent radiologic study performed, with 30% of results negative for CSVT. Headache and vomiting were the most common symptoms while neurologic symptoms were less frequent. Anticoagulation treatment was strongly inconsistent throughout the literature. Thrombosis outcomes were favorable. The most common possible risk factors were corticosteroid treatment, proteinuria and hypoalbuminemia. Four children had a genetic predisposition diagnosed after thrombosis. No markers for anticoagulation prophylaxis seemed to be relevant for the majority of thrombosis occurring in outpatients. Conclusion: Prophylactic anticoagulation does not seem reasonable to prevent CSVT. Knowledge of nonspecific symptoms and of nephrotic syndrome being a state of hypercoagulation and early use of appropriate radiologic study seem to be of major importance.
Language	English
Publishing date	2023-08-24
Publishing country	Switzerland
Document type	Journal Article ; Review
ZDB-ID	2711999-3
ISSN	2296-2360
ISSN	2296-2360
DOI	10.3389/fped.2023.1207871
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Article: Case report: Severe hypertension-induced priapism in an infant with unrecognized autosomal recessive polycystic kidney disease.

Konopásek, Patrik / Ptáčníková, Natálie / Toni, Ledjona / Zieg, Jakub

Frontiers in pediatrics

2023 Volume 11, Page(s) 1216239

Abstract: Priapism is a urologic emergency requiring prompt management. There are three types of priapism: stuttering (intermittent), non-ischemic (high-flow/arterial), and ischemic (low-flow/veno-occlusive). Here, we present the first case of an infant with ... ...

Abstract	Priapism is a urologic emergency requiring prompt management. There are three types of priapism: stuttering (intermittent), non-ischemic (high-flow/arterial), and ischemic (low-flow/veno-occlusive). Here, we present the first case of an infant with recurrent non-ischemic priapism as the first sign of severe hypertension. An 11-month-old infant was admitted to the hospital for high-flow priapism. On admission, he was found to have severe hypertension that required a combination of five antihypertensive drugs; abdominal ultrasound showed polycystic kidneys, splenomegaly, and a parenchymal liver lesion. The priapism resolved spontaneously and did not recur again after the initiation of antihypertensive treatment. Genetic analysis confirmed autosomal recessive polycystic kidney disease (ARPKD). We found no other explanation for the priapism, such as genital trauma, hematologic disease, or anything else. Decreased nitric oxide (NO) bioavailability seen in patients with hypertension seems to be the principal mechanism of hypertension causing priapism. This hypothesis is supported by animal models of genetically modified mice lacking nitric oxide synthase. The same mechanism is thought to be the genesis of priapism and other complications, such as pulmonary hypertension, in patients with sickle cell disease. We present a case of severe hypertension-associated priapism in a child with unrecognized ARPKD. The endothelial dysfunction with decreased NO bioavailability seen in patients with hypertension may be the principal pathogenic mechanism.
Language	English
Publishing date	2023-09-12
Publishing country	Switzerland
Document type	Case Reports
ZDB-ID	2711999-3
ISSN	2296-2360
ISSN	2296-2360
DOI	10.3389/fped.2023.1216239
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Article: Pathophysiology of Hyponatremia in Children.

Zieg, Jakub

Frontiers in pediatrics

2017 Volume 5, Page(s) 213

Abstract: Hyponatremia is a common electrolyte disorder in children. It is generally defined as plasma sodium of less than 135 mmol/l. Sodium homeostasis is essential for maintaining intravascular volume and is tightly linked to water balance. Plasma water volume ... ...

Abstract	Hyponatremia is a common electrolyte disorder in children. It is generally defined as plasma sodium of less than 135 mmol/l. Sodium homeostasis is essential for maintaining intravascular volume and is tightly linked to water balance. Plasma water volume is regulated mainly by the secretion of an antidiuretic hormone (ADH) and by the thirst mechanism. ADH is synthesized in the hypothalamus and stored in the posterior hypophysis. It binds to V2 receptors in the distal nephron and induces translocation of aquaporin water channels in the plasma membrane to retain water. There are two main types of receptors involved in the control of the body water balance-osmoreceptors and baroreceptors. Osmoreceptors reside in hypothalamus and respond to changes of extracellular fluid (ECF) osmolality. Baroreceptors are mechanoreceptors that sense blood pressure in the vessel wall. Response reflexes from baroreceptors influence sympathetic outflow, vessel tonus, and cardiac output. An increase of 1% of plasma osmolality may cause an increase in ADH levels, while the threshold of volume receptors for ADH secretion is higher. However, significant hypotension is a more potent stimulus for ADH secretion than increased osmolality. The main cause of pediatric hyponatremia is an abundance of free water. This may occur in hypovolemic children with low ECF volume, normovolemic patients with inappropriately increased ADH secretion, and also in hypervolemic individuals with decreased effective circulating volume and appropriately increased ADH levels. Proper understanding of the pathophysiology of hyponatremic states is essential for establishing the correct diagnosis and appropriate therapy.
Language	English
Publishing date	2017-10-16
Publishing country	Switzerland
Document type	Journal Article ; Review
ZDB-ID	2711999-3
ISSN	2296-2360
ISSN	2296-2360
DOI	10.3389/fped.2017.00213
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Article: Bartter and Gitelman syndromes.

Zieg, Jakub / Doležel, Zdeněk

Casopis lekaru ceskych

2021 Volume 161, Issue 3-4, Page(s) 131–134

Abstract: Bartter and Gitelman syndromes belong to salt-losing tubulopathies. These rare diseases may be associated with severe electrolyte disorders. Early identification of tubulopathies is essential for appropriate management. Progress in molecular genetics ... ...

Title translation	Bartterův a Gitelmanův syndrom.
Abstract	Bartter and Gitelman syndromes belong to salt-losing tubulopathies. These rare diseases may be associated with severe electrolyte disorders. Early identification of tubulopathies is essential for appropriate management. Progress in molecular genetics enabled the identification of genes and pathophysiologic mechanisms associated with these diseases. Here, we review etiology and diagnostics of these disorders from the light of current knowledge. Additionally, we discuss contemporary therapeutic approaches.
MeSH term(s)	Bartter Syndrome/diagnosis ; Bartter Syndrome/genetics ; Bartter Syndrome/therapy ; Gitelman Syndrome/diagnosis ; Gitelman Syndrome/genetics ; Gitelman Syndrome/therapy ; Humans
Language	English
Publishing date	2021-11-30
Publishing country	Czech Republic
Document type	Journal Article ; Review
ZDB-ID	413441-2
ISSN	1805-4420 ; 0008-7335
ISSN (online)	1805-4420
ISSN	0008-7335
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