mohammad esmaeeli

Rituximab is a novel therapy that can help patients with steroid-dependent or resistant nephrotic syndrome. The aim of this study was to evaluate the efficacy of rituximab in children with corticosteroid-dependent and resistant nephrotic syndrome and to determine the factors associated with its efficacy.

In this study, 40 children with corticosteroid-dependent or resistant nephrotic syndrome who were treated with rituximab in Dr. Sheikh Hospital, Mashhad, between 2014 and 2018 were enrolled. Patients with a history of hematuria, severe urinary tract infection, or secondary nephrotic syndrome were excluded.

The mean age of patients was 11.9 ± 5.04 years, and 55% were female. The most common underlying pathology of nephrotic syndrome was focal segmental glomerulonephritis(FSGS)(42.5%) followed by membranoproliferative glomerulonephritis(MPGN)and minimal change disease(MCD). Most of the participants (62.5%) were steroid-dependent and the rest (27.5%) were steroid resistant. Only 10% of the patients showed complications following rituximab administration and 57.5% went into complete remission. A negative family history and steroid-dependent nephrotic syndrome were significantly associated with a better treatment response. Moreover, patients with steroid-resistant nephrotic syndrome were more likely to have a positive family history, while factors associatedwith steroid response included underlying pathology, gender, and family history.

Rituximab can cause remission in more than half of the patients with steroid-resistant or dependent nephrotic syndrome. Moreover, the only factors that reduce response to rituximab are a history of corticosteroid resistance and a positive family history of nephrotic syndrome.

The relation between science and religion has been one of the most important disturbance of scientists in recent centuries. Expressing thus issue was started in west countries since renaissance seriously and it expanded to all countries even Islamic countries. Mesbah as a philosopher and an Islamic scientist chooses completion idea which is based on his basis; e.g.  philosophical foundations with reasonable relativity, paradigm acceptance which means thought basis, experience acceptance which means revelation and inspiration by innocent, monopoly on legitimacy acceptance with salvations pluralism and behavioral pluralism, general rules of conversation acceptance agreement and required backgrounds and transcendent wisdom. Also, Ian Barbour as a philosopher and a western physicist chooses compilation idea which is based on his basis; e.g.   science and religion methodology parallelisms, critical realism, paradigm acceptance which means mindset, religious experience, religious pluralism, philosophic hermeneutic and scanning philosophy. Mesbah leads to separate levels of knowledge in case of its value recognizing and to accept that knowledge is multilevel and is necessary to face systematically in phenomena analysis, but Barbour works on relativity.

Context: 

Different studies have been done so far on drug safety and efficacy in children with refractory nephrotic syndrome (NS). Ofatumumab might be an effective drug for this syndrome; however, the long-term effects and cost-effectiveness of ofatumumab treatment have not been comprehensively assessed.

This study aims to perform a systematic review of the efficacy and safety of ofatumumab in children with difficult-to-treat NS.

Study Selection: 

An electronic literature search was conducted to identify appropriate studies. The search key terms were as follows: (“nephrotic syndrome” OR “minimal change disease” OR “focal segmental glomerulosclerosis” OR “membranous”) AND (“Ofatumumab” or “CD20” or “Arzerra” or “HuMax-CD20”).

Data Extraction: 

Data were extracted from the articles according to the selection criteria by two independent reviewers.

A total of 83 potentially relevant articles were identified. Thirty-two articles were removed due to duplication. Then 26 more articles were excluded because they were book sections and review papers and therefore not relevant. Another 14 items were removed after reviewing the full text of selected papers because the topics did not fit our study subject. Finally, 11 studies were selected in our systematic review. The benchmark considered to assess the efficacy of ofatumumab in children with nephrotic syndrome in most of the studies was a complete remission rate.

In conclusion, our systematic review showed that ofatumumab may be an effective drug in refractory NS treatment in children and could bring down the use of steroids and immunosuppressants. However, further large randomized trials are suggested.

Introduction. Nocturnal enuresis is a condition, which can affectthe quality of life in children. The present study was designed toinvestigate the efficacy of low-dose imipramine combined withdesmopressin on treatment of patients with primary nocturnalenuresis who were defined as desmopressin non-responders.Methods. A randomized clinical trial was carried out on patientswith primary nocturnal enuresis. Forty children with enuresisranging from 5 to 12 years old were randomly divided into theintervention (n = 20) and control groups (n = 20). The subjects inthe intervention group were treated with desmopressin combinedwith 5 mg imipramine at bedtime, and those in the control groupwere given desmopressin alone. The patients were followed upweekly for one month. The number of wet nights was recorded.Results. Two individuals in the intervention and three individualsin the control group were excluded from the study. Our findingsindicated that the age and gender showed no significant difference.Furthermore, a significant better recovery in the enuresis wasobserved in 18 of 20 patients who were treated with combinationtherapy after 1 month (P < .05). In addition, the frequency ofrecovery was significantly higher (83.3%) in the intervention group,compared with the control group (29.4%).Conclusion. The analysis showed that low-dose imipramine is welltolerated in clinical practice and may represent a good short-termtreatment option in combination therapy where desmopressinalone is not efficient enough.

Giggle incontinence, also known as “enuresis risoria”, is characterized by unexpected, involuntary, and complete bladder voiding in response to laughter. The cause is unknown; however, there are different assumptions based on several case studies. This research discusses the effectiveness of methylphenidate for giggle incontinence in children.

Fifteen girls who met the giggle incontinence criteria were randomly divided to two groups: group A (n=8 girls) and group B (n=7 girls). The participants in group A took Oxybutynin chloride and the patients in group B received methylphenidate for one month. The response of the two groups to drugs was assessed.

Group A included 8 girls (mean age: 7.7 years) and group B comprised 7 girls (mean age: 8.4 years). Only one patient in group A showed complete response to Oxybutynin chloride. No wetting was reported by six patients in group B.

Giggle incontinence is a rare form of incontinence. The pathophysiology is unfortunately unknown yet. Methylphenidate may be suggested for symptomatic relief compared to Oxybutynin chloride until the etiology of the disease is more clearly defined.Keywords: Giggle incontinence; Giggle micturition; Laughter incontinence; Enuresis.

In different tissues, the endothelin is produced by vascular endothelium. They are potent vasoconstrictor peptides. There is a little information about the role of endothelin in reflux nephropathy.
The aim of this study is to evaluate urinary levels of endothelin in patients with vesicoureteral reflux (VUR).
Patients and It was a cross-sectional study that conducted on 81 children who received voiding cystourethrogram (VCUG). Based on VCUG reports, patients were divided into two groups; with reflux (40 persons) and without reflux (41 persons). We got a urine sample from patients with mid-stream or urine bag method. The endothelin level was assessed with ELISA immunoassay. Data was analyzed using SPSS 16.
Based on VCUG reports, 40 patients (49.4%) had urinary reflux, of them 20 cases suffered from unilateral urinary reflux and others from bilateral. Of 40 patients with reflux, 23 cases (57.5% of reflux group) had kidney scar and seven individuals (17.5%) had abnormal kidney sonography. Of patients with urinary reflux, 13 cases (32.5%) had grad 1 urinary reflux, 8 cases (20%) grade 2, and 5 cases (12.5%) grade 3 and finally 14 cases (35%) grade 4. The UET-1 levels were significantly higher in VUR patients compared to the control group (P Urine endothelin-1 level can be considered as an alternative to VCUG for screening vesicourethral reflux.

Penile hair tourniquet syndrome (PHTS) is a rare clinical condition characterized by progressive penile strangulation caused by a piece of hair curled around sulcus corona. This clinical condition may result in some clinical and urgent complications including ischemia and gangrene of glans penis, urethra-cutaneous fistulae, and urethral transection. The case report is about a four year and six month old circumcised boy with a hair coil penile strangulation caused by strands of hair being wrapped around the sulcus corona.

Carnitine deficiency is commonly seen in dialysis patients. This study assessed the association dialysis and pediatric patient's characteristics with plasma carnitines levels. Plasma carnitine concentrations were measured by tandem mass spectrometry in 46 children on hemodialysis or peritoneal dialysis. The total carnitine, free carnitine (FC), and L-acyl carnitine (AC) levels of 40 µmol/L and less, less than 7 µmol/L, and less than 15 µmol/L were defined low, respectively. An FC less than 20 µmol/L and an AC/FC ratio greater than 0.4 were considered as absolute and relative carnitine deficiencies. The correlation between carnitines levels and AC/FC ratio and age, duration of dialysis, characteristics of dialysis, and blood urea nitrogen and serum albumin concentrations were assessed. Absolute carnitine deficiency, low total carnitine, and low AC concentrations were found in 66.7%, 82.6%, and 51% of the patients, respectively. All of the patients had relative carnitine deficiency. Carnitine measurements were not significantly different between the hemodialysis and peritoneal dialysis groups. More severe relative carnitine deficiency was found in those with lower blood urea nitrogen levels and those on peritoneal dialysis. No linear correlation was found between carnitine levels and age, duration of dialysis, characteristics of dialysis, serum albumin level, or blood urea nitrogen level. Absolute and relative carnitine deficiencies are common among children on dialysis. Patients with lower blood urea nitrogen levels and peritoneal dialysis patients are more prone to severe relative carnitine deficiency.

Hyperhomocysteinemia is common in end stage renal diseases. We aimed to determine the prevalence of hyperhomocysteinemia in dialysis cases and define independent risk factors of the development of hyperhomocysteinemia. The total plasma homocysteine values were measured in 46 dialysis patients including 20[43.4 %] girls and 26[56.6 %] boys aged 1.6-25 [19.9±6.5] years based on two different reference values for children [age dependent] and adults [cut off point of 15 µmol/L]. Using the reference values for children, 26 cases [56.2 %] had hyperhomocysteinemia including 41.6% of CAPD and 2/3 of hemodialysis patients with no significant difference based on age, gender, duration and modality of dialysis, and dosage of folate supplement [p>0.05 for all]. Using a cut-off point of 15 µmol/L, hyperhomocysteinemia was reported in 30.4% of the patients including 11 hemodialysis and one CAPD [P=0.022], 10 out of 19 girls [52.6%%] and 4 out of 26 boys [15.4%] [p=0.063], but logistic regression analysis did not show any significant differences in the incidence rate of hyperhomocysteinemia according to the modality of dialysis and gender [P=0.998 and 0.137 respectively]. We found mild hyperhomocysteinemia as a common finding in dialysis patients; also, the prevalence of hyperhomocysteinemia was comparable in children and young adults. However, we noted that hemodialysis patients and females were more prone to more intense elevations of plasma homocysteine levels. We found that neither gender nor modality of dialysis played a role as risk factors for development of hyperhomocysteinemia in children and young adults.

Deficiencies of water soluble vitamins such as folate and vitamin B12 has been reported as etiologic factors of hyperhomocysteinemia. This study was conducted to find whether there is a correlation between serum levels of these vitamins and plasma total homocysteine (tHcy) levels. 19 hemodialysis subjects were enrolled. The study group comprised 52.6% girls and 47.4% boys aged 80-324 (204.7±78.4) months who were on dialysis from 1.5-153 (42.1±43.3) months ago. All patients were supplemented by folate and 15 cases were received oral vitamin B12.Folate serum levels <1.5 ng/ml were defined as low (deficiency).As for vitamin B12, levels < 120 pg/ml, 120-160 pg/ml were defined as deficient and borderline, respectively. Plasma Hcy levels of 5-15 µmol/L and > 15 µmol/L were defined as normal and hyperhomocysteinemia, respectively. The correlation between the serum levels of vitamins and plasma Hcy levels was checked by the Pearson correlation test and P-values <0.05 and r>0.7 indicated a good (significant) correlation. 13 patients (68.4%) had hyperhomocysteinemia whereas plasma tHcy levels were normal in 6 (31.6%). No patient had folate or vitamin B12 deficiency.There was no correlation between tHcy levels and serum vitamin B12 (P=0.621, r=1) and serum folate levels (P=0.571, r=1). Normal and even high serum levels of folate and vitamin B12 cannot prevent the occurrence of hyperhomocysteinemia in hemodialysis patients.

Total Hip Replacement (THR) is done comprehensively all over the world. Despite the effects of braces on control of THR complications، few studies have been performed on this subject. The purpose of this study was to evaluate immediate effect of hip abduction brace on mobility and functional balance of patients with THR. Eight men and nine women with THR participated in this study. Mobility and functional balance were assessed and compared with and without brace conditions by means of TUG، FR and Berg tests. A significantly higher TUG، FR and Berg scores were seen in brace condition compared to without brace situation (P < 0. 05). Hip abduction brace can increase mobility and functional balance in patients with THR.

Acute kidney injury (AKI) is characterized by a reversible increase in the blood concentration of creatinine and nitrogenous waste products and by the inability of the kidney to regulate fluid and electrolyte homeostasis appropriately. Hyperuricemia is a feature of several pathologies and requires an appropriate and often early treatment، owing to the severe consequences that it may cause. A rapid and massive raise of uric acid، mainly damage the kidney.. Rasburicase، compared to allopurinol، results in more rapid reduction of uric acid and prevent its accumulation in patients with hematologic malignancy with hyperuricemia and AKI. We evaluated the efficacy of rasburicase (0. 15 mg/kg) administered as single dose in 15 patient with AKI in sheikh hospital، Mashhad University of medical Sciences. Beside conventional therapy، infusion of Rasburicase in 50cc normal saline in30 minute intravenously was done and all adverse reaction was treated. Before injection of Rasburicase and 1st، 3nd and 7rd day after it urea، creatinine، uric acid and urine output were determined and compared. Within first 24 hour، the levels of serum uric acid in all patients decreased dramatically. there was a significant improvement in estimated GFR and urine output. Urea and creatinine level decreased from Day 0 to Day 7 but there was no difference between creatinine before treatment and 1st day. Rasburicase is effective for management of hyperuricemia and uremia in AKI Patients.

In the last two centuries, chronic kidney disease (CKD) and renal failure (RF) have been considered as the main medical problems which were fatal in many cases. Trace elements such as selenium, copper, and zinc are the components of biological enzymes which have a crucial role in decreasing reactive oxygen. The levels of these trace elements reduce in CKD patients. Close and careful nutritional support of children with CKD, particularly in the early stages of the disease, is necessary.