MMP-1 and -3 haplotype is associated with congenital anomalies of the kidney and urinary tract.

BACKGROUND: Congenital anomalies of the kidney and urinary tract (CAKUT) are a common cause of progressive chronic kidney disease that may lead to end-stage renal disease and renal replacement therapy in childhood. Altered expression or activity of matrix metalloproteinases (MMPs) have been found in CAKUT. The MMP-1, -3, and -8 polymorphisms studied here are located in the gene promoters and alter expression. Our aim was to investigate associations of MMP polymorphisms, solely and in haplotypes, with CAKUT in children. METHODS: A case-control study with 101 pediatric patients and 281 controls was performed. The MMP-1 (-1607 1G/2G), -3 (5A/6A), and -8 (-799 C/T) genotypes were determined by PCR-restriction fragment length polymorphism. RESULTS: We found statistically significant associations of MMP-3 5A/6A polymorphism (p < 0.0001) and 1G-(1607)-6A haplotype, with no preferences for MMP-8 -799C or T alleles, with CAKUT (OR = 2.93, 95 % CI 1.43-5.98, adjusted for gender, p = 0.003) and with obstructive uropathies in a subgroup of patients (OR = 4.57, 95 % CI 2.74-7.61, adjusted for gender, p < 0.0001). CONCLUSIONS: MMP-3 genotypes and MMP-3 and -1 haplotypes encompassing either MMP-8 -799C or T alleles were associated with CAKUT and obstructive uropathies in pediatric patients. Still, functional and association studies are needed to elucidate evident roles of MMPs in CAKUT.

Epidemiology of chronic kidney disease in children in Serbia.

BACKGROUND: The epidemiological information from well-defined populations regarding childhood chronic kidney disease (CKD), particularly those concerning non-terminal stages, are scanty. The epidemiology of CKD in children is often based on renal replacement therapy (RRT) data, which means that a considerable number of children in earlier stages of CKD are missed as they will reach end-stage renal disease (ESRD) in adulthood. Here, we report the basic epidemiological data on childhood CKD in Serbia, gathered over the 10-year period of activity of the Serbian Pediatric Registry of Chronic Kidney Disease. METHODS: Since 2000-09, data on incidence, prevalence, aetiology, treatment modalities and outcome of children aged 0-18 years, with CKD Stages 2-4 and CKD Stage 5, were collected by reporting index cases from paediatric centres. RESULTS: Three hundred and thirty-six children were registered (211 boys, 125 girls, male/female ratio 1.7). The median age at registration was 9.0 years [interquartile range (IQR) 3-13]. Median follow-up was 4.0 years (IQR, 1-9). The median glomerular filtration rate (GFR) at the time of the registration was 39.6 mL/min/1.73m(2) (IQR, 13.8-65.4). Median annual incidence of CKD 2-5 stages was 14.3 per million age-related population (p.m.a.r.p.), while those of CKD 2-4 or CKD 5 were 9.1 and 5.7 p.m.a.r.p., respectively. The median prevalence of CKD 2-5 was 96.1 p.m.a.r.p., 52.8 p.m.a.r.p. in CKD 2-4 and 62.2 p.m.a.r.p. in CKD 5. The main causes of CKD were congenital anomalies of kidney and urinary tract and hereditary nephropathies. Kidney survival was the worst in children with glomerular diseases and in those with advanced CKD. Haemodialysis was the most common first modality of RRT. Mortality rate was 4.5%, mainly due to cardiovascular and infectious complications. CONCLUSIONS: Epidemiology of paediatric CKD in Serbia is similar to that reported from developed European countries. The knowledge of the epidemiology of earlier stages of CKD is essential for both institution of renoprotective therapy and planning of RRT, a fact of paramount importance in countries with limited resources.

Antidiabetic effect of two different Ganoderma species tested in alloxan diabetic rats.

This study was designed to define total protein, phenol and flavonoid content as well as LC-MS/MS phenolic profile related to antioxidant and antidiabetic activity of ethanolic (EtOH) and water extracts of G. pfeifferi and G. resinaceum. G. resinaceum water extract possessed the highest ability to scavenge DPPH˙ and O2˙-, while the EtOH extract of the same species showed better activity on NO˙ related to other extracts. The highest level of bioactive compounds was determined generally in EtOH extracts. Antidiabetic action was evaluated by the oral glucose tolerance test (OGTT) and histological examination of pancreas and liver in normoglycemic and alloxan-induced diabetic animals. Histological examination of pancreatic tissue demonstrated that G. pfeifferi extracts have protective effects. To conclude, analysed extracts could be considered as a promising candidate for further research with the aim to promote antidiabetic activity, which is for the first time reported for G. pfeifferi.

Nonspecific perichondritis of the lumbar spine.

A teenage girl with soft tissue inflammation involving the L2-S1 region of the lumbar region is reported. Magnetic resonance (MR) imaging revealed high-signal lesions on T2-weighted images in association with contrast enhancement. Histology revealed nonspecific perichondritis with mononuclear inflammatory infiltration of the perichondrium and connective tissue. Six months after treatment, MR imaging was normal. No similar case of perichondritis with involvement of the lumbar region could be found in the current literature.

Growth in children with chronic kidney disease: 13 years follow up study.

BACKGROUND: Growth retardation is one of the most visible comorbid conditions of chronic kidney disease (CKD) in children. To our knowledge, published data on longitudinal follow-up of growth in pediatric patients with CKD is lacking from the region of South-East Europe. Herein we report the results from the Serbian Pediatric Registry of Chronic Kidney Disease. METHODS: The data reported in the present prospective analysis were collected between 2000 and 2012. A total of 324 children with CKD were enrolled in the registry. RESULTS: Prevalence of growth failure at registry entry was 29.3 %. Mean height standard deviation scores (HtSDS) in children with stunting and those with normal stature were -3.00 [95 % confidence interval (CI) -3.21 to -2.79] and -0.08 (95 % CI -0.22 to 0.05) (p < 0.001), respectively. Children with hereditary nephropathy had worse growth at registration (-1.51; 95 % CI -1.97 to -1.04, p = 0.008). Those with CKD stages 4 and 5 before registration had more chance to have short stature at registration than those with CKD stages 2 and 3 [odds ratio (OR) = 0.458, CI 0.268-0.782, p = 0.004]. Dialysis was an independent negative predictor for maintaining optimal stature during the follow-up period (OR = 0.324, CI = 0.199-0.529, p < 0.001), while transplantation was an independent positive predictor for improvement of small stature during follow-up (OR = 3.706, CI = 1.785-7.696, p < 0.001). CONCLUSION: Growth failure remains a significant problem in children with CKD, being worst in patients with hereditary renal disease. Growth is not improved by standard dialysis, but transplantation has a positive impact on growth in children.

Common variable immunodeficiency complicated with hemolytic uremic syndrome.

Common variable immunodeficiency is a primary immunodeficiency disease characterized by reduced serum immunoglobulins and heterogeneous clinical features. Recurrent pyogenic infections of upper and lower respiratory tracts are the main clinical manifestations of common variable immunodeficiency. Hemolytic uremic syndrome is a multisystemic disorder characterized by thrombocytopenia, microangiopathic hemolytic anemia, and organ ischemia due to platelet aggregation in the arterial microvasculature. This is one of the rare cases of patients diagnosed with common variable immunodeficiency, which was complicated by hemolytic uremic syndrome.

[Resistence of Escherichia coli, the most frequent cause of urinary tract infection in children, to antibiotics].

INTRODUCTION: Urinary tract infections (UTI) take the second place in the incidence of bacterial infection in children. Escherichia coli is a cause of infection in 85-90%. A periodic evaluation of the resistance to antimicrobial drugs has to be performed in each geographic region, since investigations confirmed that the resistance of bacteria causing UTI has been in progress. MATERIAL AND METHODS: A retrospective investigation has been performed, comprising the two time periods in the range of 10 years in order to identify the prevalence and resistance of the bacteria causing UTI in the patients treated at the Department of Nephrology of Institute for Child and Youth Health Care of Vojvodina. RESULTS: During the first investigated period from January 1996 up to December 1997, there were 163 urin analyses performed vs 134 urine analyses in the second period, starting from January 2006 to December 2007. In both periods, Escherichia coli, was the most frequent cause of UTI (82.1% in 1996/97 vs 86.50% in 2006/07). During this ten-year period, the resistance of Escherichia coli increased both to ampicillin (from 53% to 69% (p > 0.05) and to trimethoprim/sulfamethoxazole (34% vs 55%; p < 0.05) as well as to cephalexin (4% vs 36%, p < 0.05) which has been lately used in our region as a drug of choice in empiric therapy of the suspect UTI. DISCUSSION: There have been records on a slow increase of the Escherichia coli resistance to ceftazidim, gentamycin and nalidixic acid, but significant increase to ampicillin, trimethoprim/sulfamethoxazole and cephalexin. CONCLUSION: For the initial therapy of UTI in the Province of Vojvodina we recommend: perorally--ephalosporins I, II and III generation, and in case when the child is not capable to get therapy perorally, or in the case of highly febrile infant--ephalosporins III generation parenterally.

[Postnatal follow-up of children having antenatal hydronephrosis].

The application of antenatal ultrasound for the evaluation of congenital anomalies in early pregnancy has become a common practice. Hydronephrosis is the most common anomaly observed. Antenatal interventions are experimental and done in advanced centers. Though there are defined criteria of intervention, it is still not clear as to who will benefit. Most patients will have relatively benign course, however identification of those who will have potentially serious postnatal problems permits early intervention. This study gives recommendations for the postnatal examination and follow-up of the newborns having hydronephrosis diagnosed on antenatal ultrasound.

Idiopathic hypercalciuria associated with urinary tract infection in children.

The aim of this study was to evaluate the association between idiopathic hypercalciuria (IH) and urinary tract infection (UTI) in children. This prospective clinical study included 75 patients with UTI (without urinary tract malformations and lithiasis) and a control group of 30 healthy children. Of the total number of patients with UTI, 21% (n = 16/75) had IH, but only 7% (n = 2/30) with IH were reported in the control group (p < 0.05). Recurrent UTI affected 33% (n = 25/75) of patients , and in 67% (n = 50/75) of patients, UTI was diagnosed for the first time. In the group of patients with recurrent UTI, 44% (n = 11/25) had IH, but only 10% (n = 5/50) were reported in the group of patients with first-time UTI (p < 0.05). The results of multifactorial logistic regression analysis showed that clinical and laboratory parameters (recurrent UTI, dysuria, and microscopic hematuria) may predict the diagnosis of IH in 80% of patients and absence of IH in 87% of cases. In our opinion, IH is a major contributing factor to UTI, especially to recurrent UTI in children.

Splenic peliosis in the course of IgA nephropathy.

The immunoglobulin A (IgA) immunoregulation disorders lie at the basis of Henoch-Schönlein purpura nephritis and IgA nephropathy. Peliosis is the condition characterized by cystic formations within the parenchyma of solid organs filled with blood. The authors report a case of a girl presenting with hematuria occurring during the course of respiratory infections since her fifth year. Pathohistological examination was not performed at that time. At the age of 13, the girl was hospitalized for abdominal pain. Computed tomography examination showed the presence of multiple, relatively well-defined nodular formations located in the spleen parenchyma. Splenectomy was performed. Morphological finding completely corresponded to peliosis of the spleen, with the deposits of IgA in the lesions within it. A year and a half following the splenectomy, a typical clinical picture of Henoch-Schönlein purpura nephritis developed. Biopsy findings of the skin and kidneys detected deposits of IgA. This is the first case of a patient suffering from associated IgA and Henoch-Schönlein purpura nephritis complicated by splenic peliosis to be described in the world's literature.